Cardiology

A baby is born at term in good condition, requiring no resuscitation at delivery. He is well after birth, breast-feeding well and is transferred to the postnatal ward with mum.

When he is reviewed at approximately 24 hours of age, he is noted to be cyanosed and is transferred to the special care baby unit. On arrival on special care, he is cyanosed with saturations of 79% in air, is mildly tachypnoeic with a respiratory rate of 62 breaths per minute and has a heart rate of 146 beats per minute.

On examination, his chest is clear and you can hear a loud systolic murmur.

A full blood count is performed which shows a haemoglobin of 18.9 g/dl with a white cell count of 5.3 109 and normal platelets. Oxygen is administered via nasal cannula which fails to improve his oxygen saturations.

Which of the following diagnoses are possible in this child? Choose three answers only.Ventricular septal defect (VSD) Incorrect

Transposition of the great arteries

Incorrect

Patent ductus arteriosus (PDA)

Incorrect

Pulmonary stenosis

Incorrect

Total Anomalous Pulmonary Venous Drainage (TAPVD)

Incorrect

Coarctation of the aorta Incorrect

Transposition of the great arteriesPulmonary stenosisTotal Anomalous Pulmonary Venous Drainage (TAPVD)Transposition of the great arteries is the commonest type of cyanotic heart disease in the newborn. It usually presents in the first few days of life. Examination is often normal but a systolic murmur may be heard if there is an associated VSD or pulmonary stenosis or a patent ductus arteriosus (PDA).

Severe pulmonary stenosis presents in the newborn period with cyanosis and an ejection systolic murmur. There is decreased blood flow to the lungs and early intervention is required if there is a critical decrease in flow.

Total Anomalous Pulmonary Venous Drainage (TAPVD) is a condition in which the pulmonary veins drain into the right atrium or its venous tributaries rather than into the left atrium. If this abnormal drainage is obstructed, the baby can present with heart failure or cyanosis in the newborn period. (Unobstructed TAPVD presents later.)

A murmur may be the presenting feature of a VSD in a newborn but the infant would not be cyanosed unless there were other abnormalities such as pulmonary atresia which would decrease pulmonary blood flow. Coarctation of the aorta and a PDA would likewise not present with cyanosis.

Match the following syndromes with the correct cardiac examination findings.

A Trisomy 21 B Noonans syndrome C Edwards syndrome D Di George syndrome E Williams syndrome F Pataus syndrome G HoltOram syndrome H Turners syndrome

Scenario 1

Incorrect

Normal S1 with fixed, widely split S2 and ejection systolic murmur

0 Your answerG Correct answerHoltOram syndrome These clinical signs are consistent with a secundum ASD, which is associated with HoltOram syndrome (absent radii and ASD Scenario 2

Incorrect

Boy with ejection systolic murmur radiating to the neck with carotid thrill

0 Your answerE Correct answerWilliams syndrome Williams syndrome is caused by a deletion on chromosome 7q coding for elastin. Typical features are stellate pattern in the iris, fish-shaped lips, cocktail-party speech and mental retardation. Cardiac features include peripheral pulmonary stenosis, VSD and ASD, but most notably supravalvular aortic stenosis whose signs are described above. Here I have stated boy so as to avoid confusion with Turners syndrome, which is also associated with aortic stenosis.Scenario 3

Incorrect

A cyanosed infant with normal S1, single S2 and long ejection murmur best heard at upper left sternal edge

0 Your answerD Correct answerDi George syndrome These signs are suggestive of tetralogy of Fallot (VSD, right ventricular outflow tract obstruction, right ventricular hypertrophy and overriding aorta), which is the most common congenital cyanotic heart condition. The murmur is due to turbulent flow in the right ventricular outflow tract and as the obstruction becomes more sever the murmur becomes softer and shorter. Di George syndrome is 22q microdeletion syndrome resulting in dysmorphogenesis of the 3rd and 4th pharyngeal pouches early in gestation and this leads to thymic and parathyroid hypoplasia and other abnormalities including congenital heart defects (also truncus arteriosus). Dysmorphic features include low set ears, hypertelorism and short philtrum. Affected children can have variable degrees of immunodeficiency. Scenario 4

Incorrect

Normal S1 with ejection click and ejection murmur best heard at the upper left sternal edge radiating to the back

0 Your answerB Correct answerNoonans syndrome Noonans syndrome is an autosomal dominant condition whose features include mild mental retardation, ptosis, webbed neck, shield chest and short stature. The most common cardiac manifestation is pulmonary stenosis but PDA, VSD, ASD and branch stenosis of the pulmonary arteries is also seen. The signs here are consistent with pulmonary valve stenosis.

Scenario 5

Incorrect

Acyanotic infant with soft ejection systolic murmur and weak femoral pulses

0 Your answerH Correct answerTurners syndrome These clinical features are suggestive of coarctation of the aorta, which can present as a duct-dependent circulation with collapse in the neonatal period. Older children may present with hypertension and a systolic murmur radiating to the back. The key feature is the weak or absent femoral pulses. Turners syndrome is monosomy of the X chromosome so only affects women. Girls can have short stature, webbed neck, shield chest, widely spaced nipples, cubitus valgus, congenital lymphoedema and ovarian dysgenesis. Up to 30% of affected girls have a bicuspid aortic valve and about 15% have coarctation of the aorta.

An 8-month-old boy presents to the Accident and Emergency Department with a 2-day history of runny nose and cough and today has had difficulty breathing, is lethargic and has vomited once. He has no significant past medical history and his immunisations are up to date.

On examination he looks unwell with delayed capillary refill of 45 s. Heart rate (HR) 180, respiratory rate (RR) 60, temperature 39 oC and he has increased work of breathing. Auscultation of his precordium reveals normal first and second heart sounds with a gallop audible and femoral pulses are both palpable. Chest and abdominal examination is unremarkable.

Chest X-ray (CXR) shows cardiomegaly but lung fields are clear. A 12-lead electrocardiogram (ECG) shows sinus tachycardia. He is becoming more lethargic and once iv access is obtained he received a fluid bolus of 10 ml/kg saline and bloods are taken for culture, FBC, U&E, C-reactive protein (CRP) and clotting.

What is the most likely diagnosis? PDA

VSD

Viral myocarditis Correct answer

Kawasakis disease

SVT

A diagnosis of viral myocarditis should be considered in a previously well child who presents with cardiac failure and cardiomegaly on CXR. Enterovirus is the commonest viral cause. There is a spectrum of severity with some children being asymptomatic and others presenting in failure with dysrhythmias. Neonates may present with fever, poor feeding, respiratory distress and poor pulses. A murmur may be present suggesting mitral regurgitation from dilatation of the valve annulus. A gallop rhythm may be heard. Blood tests may reveal raised inflammatory markers, acidosis and raised cardiac enzymes. Viral serology may be useful for definitive diagnosis. CXR will show cardiomegaly with pulmonary congestion and electrocardiogram (ECG) may have ST- and T-wave changes. Pericardial effusion may be revealed on echo. Treatment is supportive until function improves. Diuretics are generally indicated to treat failure and ECMO may be required as a bridge to cardiac transplant.A Pulmonary stenosis (PS) B Aortic incompetence C Coarctation D Ventricular septal defect E Hypertrophic obstructive cardiomyopathy (HOCM) F Atrioventricular septal defect (AVSD) G Truncus arteriosus H Mitral valve incompetence I Patent ductus arteriosus

For each of the following children select the most likely cardiac lesion associated with their condition. Each answer may be used once, more than once or not at all.Scenario 1

Incorrect

A 3-month-old baby with macroglossia, cardiomegaly, hypotonia and failure to thrive.

0 Your answerE Correct answerHypertrophic obstructive cardiomyopathy (HOCM)Glycogen storage disease type 2. This condition is fatal in the first year of life. It affects muscles, causing cardiomegaly, HOCM, hypotonia and thickened muscles (including the tongue). It is caused by decreased -glucosidase activity, leading to glycogen accumulation in cardiac and striated muscles.Scenario 2

Incorrect

An 8-year-old girl with ptosis, a high arched palate and a history of bleeding disorders and learning difficulties.

0 Your answerA Correct answerPulmonary stenosisNoonans syndrome is caused by a gene defect in chromosome 12 (usually sporadic). Phenotypically like Turners syndrome, but they tend to have right-sided heart defects (pulmonary stenosis, peripheral pulmonary stenosis). Bleeding disorders and intellectual impairment are also seen. Scenario 3

Incorrect

A 1-year-old boy with butterfly vertebrae who is seen regularly by the hepatologist for a history of cholestasis.

0 Your answerA Correct answerPulmonary stenosis Alagilles syndrome is also known as arterio-hepatic dysplasia. Hypoplasia of the intralobular bile ducts causes neonatal hepatic cholestasis. Other features include butterfly vertebrae, persistent posterior embryotoxon in the eye and pulmonary stenosis.Scenario 4

Incorrect

A neonate with intra-uterine growth retardation (IUGR), jaundice and hepatosplenomegaly, also has a purpuric rash on her skin.

0 Your answerI Correct answerPatent ductus arteriosus Congenital rubella syndrome. Other cardiac lesions seen in this condition include peripheral PS and ventricular septal defect (VSD). In addition to the above features, thrombocytopenia, cataracts, encephalitis, chorioretinitis and deafness are seen.Scenario 5

Incorrect

A 12-month-old girl with a history of neonatal tetany and recurrent infections.

0 Your answerG Correct answerTruncus arteriosusDi George syndrome is caused by sporadic chromosome 22q deletion. Clinical features are: face: hypertelorism, ante-Mongoloid slant, cleft lip and palate hypoparathyroidism: hypocalcaemia: neonatal tetany and seizures absent thymus: cell-mediated immunodeficiency: candidiasis, pneumonias etc cardiovascular system (CVS): tetralogy of Fallot, right-sided aortic arch, truncus arteriosus, interrupted aortic arch.A 3-month-old baby has a large VSD and is failing to thrive. He was born at term by NVD and a murmur was heard at his neonatal check. Echo revealed a large VSD with a moderate left to right shunt. He fed well and had no signs of failure on discharge at 1 week of age. His birthweight was 3.4 kg and his current weight is 4 kg. He is admitted to the ward with respiratory distress, tachypnoea and tachycardia and is awaiting repair of his cardiac lesion.

Which of the following are useful in management of this baby? Choose TWO options NG feeds Incorrect

-blockers Incorrect

Salbutamol nebulisers Incorrect

Furosemide and spironolactone Incorrect

Oxygen overnight Incorrect

NG feedsFurosemide and spironolactoneThis child has been re-admitted in heart failure as evidenced by tachypnoea, increased work of breathing, poor weight gain and probably sweating. Pulmonary congestion can also cause wheezing and an enlarged liver may be palpable on abdominal examination. Infants can be asymptomatic except for when active, in the case of babies when feeding and symptoms may become obvious as pulmonary vascular resistance falls.

Chest X-ray (CXR) would reveal increased cardiothoracic ratio and echo would show the VSD, left-to-right shunt and one can assess left ventricular function (expressed as fractional shortening percentage).

Diuretics are certainly helpful in the management of heart failure to relieve pulmonary congestion and alleviate symptoms. Increasing caloric intake is important and NG feeds are valuable to reduce the extra energy expended during feeding and to support increased work of breathing. Oxygen may be required to maintain saturations at the childs normal saturations but these will be lower as there is mixing of blood across the VSD. A 3-year-old girl is referred by her GP with a heart murmur. She was born at term by NVD with no neonatal problems and has been generally well, except for a couple of viral infections. Her immunisations are up to date and her growth is following the 25th centile.

On examination she is pink and well perfused with easily palpable peripheral pulses. Her chest is clear and she has normal first and second heart sounds with a continuous murmur heard best at the upper left sternal edge. The murmur does not radiate. Abdominal examination is unremarkable. Electrocardiogram (ECG) is within normal limits and echo reveals a small PDA with no evidence of hypertrophy.

What is the best definitive treatment option for this child? Diuretics

Surgical ligation of PDA via thoracotomy

Device closure of PDA via catheterisation Correct answer

No treatment required

Annual echo scan to monitor size of PDA

In utero, the ductus arteriosus serves to lead blood from the pulmonary arteries to the aorta so bypassing the fetal lungs. The duct will usually close shortly after birth but in some cases fails to do so and this more commonly happens in premature babies. If the duct remains patent, depending on the size of the duct and shunt, the infant can become symptomatic with features of heart failure and left-to-right shunt. Spontaneous closure of the duct is rare after infancy.

Small PDAs are generally asymptomatic but are closed, as the later risks of endocarditis and complications are present. Small PDAs can be closed by transcatheter device closure (usually a coil device for small ducts) and this also allows for catheter pressure measurements at the same time. The occlusion device is placed in the duct under X-ray guidance and the main risk of the procedure is a leak around the device but this is usually insignificant.

Diuretics can be useful in the management of PDA if the child is in failure so not in this case. Surgical ligation is still an option and can be performed thorascopically but catheter closure is less invasive. You are the neonatal registrar on call and your SHO asks you to review a baby on the postnatal ward. He is a term baby who is well with no concerns noted from either mum or the midwives. There is no antenatal history of note but mum has recently moved to the area and therefore there is no record of any antenatal scan results.

On a routine newborn check the baby was noted to have a loud, grade 3/6 systolic murmur which is best heard at the upper left sternal edge. The rest of the examination is normal with no other worrying features and the baby is feeding well.

An ECG is done which is within normal parameters for age.

The baby is reviewed by one of the cardiologists who performs an echo and makes a diagnosis of Tetralogy of Fallot.

Which of the following are features of this condition? Choose four answers only:Right ventricular hypertrophy Incorrect

Left ventricular hypertrophy

Incorrect

Ventricular septal defect (VSD)

Incorrect

Atrial septal defect (ASD)

Incorrect

Right ventricular outflow tract obstruction

Incorrect

Left ventricular outflow tract obstruction

Incorrect

Overriding aorta

Incorrect

Co-arctation of the aorta Incorrect

Right ventricular hypertrophyVentricular septal defect (VSD)Right ventricular outflow tract obstructionOverriding aortaTetralogy of Fallot is a morphological diagnosis with four main features. These are: VSD, right ventricular outflow tract obstruction (eg pulmonary stenosis), right ventricular hypertrophy and overriding aorta. It is usually asymptomatic and may be diagnosed by the presence of a murmur. Occasionally, in the presence of severe pulmonary stenosis, babies can present with cyanosis, in which case early intervention is usually indicated. In older infants and children, cyanotic spells can occur, usually precipitated by physical activity, and these may require treatment with oxygen, morphine, -blockers and putting the child in the knee-chest position to try and increase systemic venous return.

Classical features of right ventricular hypertrophy on ECG and a boot-shaped heart with oligaemic lung fields on chest X-ray are seen in older children.

You are asked to review a baby on the postnatal ward who the midwives suspect has Downs syndrome.

There is no antenatal history of note and routine scans were normal. The baby was born by spontaneous vaginal delivery and did not require any resuscitation at birth. Both parents are fit and well and have three other children who are all healthy with no medical problems.

On examination, you feel that the baby does have features of Downs syndrome, and after a long discussion with the parents, blood is sent for karyotyping which confirms the diagnosis. The rest of the examination is normal apart from a long, grade 3/6 systolic murmur heard all over the praecordium. All peripheral pulses are present and equal, blood pressure is normal in all limbs and there are no signs of respiratory distress.

You refer the baby to the cardiologists for review of the murmur.

What is the most common cardiac abnormality in babies with Downs syndrome? Choose one answer only: Tetralogy of Fallot Incorrect

Persistent ductus arteriosus (PDA)

Incorrect

Atrial septal defect (ASD)

Incorrect

Atrioventricular septal defect (AVSD)

Incorrect

Transposition of the great arteries

Incorrect

Hypoplastic left heart syndrome Incorrect

Atrioventricular septal defect (AVSD)Up to half of all children with Downs syndrome may have an underlying congenital cardiac defect and therefore these babies are routinely referred to paediatric cardiologists for assessment even if no murmur is heard.

The most common cardiac defect in Downs syndrome is an atrioventricular septal defect (previously called an endocardial cushion defect). PDA and Tetralogy of Fallot do also occur but are far less frequent.

Incorrect

If this lesion is left untreated, what is the major long-term problem that could result from this defect, and if present would cause cyanosis and deterioration in clinical condition? Choose one answer only:Heart failure

Hypertension

Pulmonary hypertension

Correct answer

Renal failure

Increasing size of the defect

Children with Downss syndrome and an uncorrected AVSD are at high risk of developing Eisenmengers syndrome. This is a condition in which there is increased pulmonary vascular resistance with the development of pulmonary hypertension and a reversal of blood flow through the AVSD from left-to-right to right-to-left. This occurs at an earlier age in children with large defects. Patients develop signs of pulmonary hypertension such as cyanosis, right ventricular hypertrophy with a parasternal heave and a loud pulmonary component to the second heart sound.Incorrect

In the presence of Eisenmengers syndrome and an AVSD, what is the most appropriate treatment? Choose one answer only:Medical management of the pulmonary hypertension Incorrect

Surgery to close the defect

Incorrect

Pulmonary artery banding

Incorrect

Patching of the defect via a cardiac catheter Incorrect

Medical management of the pulmonary hypertensionOnce pulmonary hypertension has developed, with reversal of the shunt and Eisenmengers syndrome, the defect is considered inoperable. Closing the AVSD after this point will not help the patient and will result in heart failure and further deterioration. The only treatment available is to try and improve the symptoms through medical management.A 3-year-old child had high fever for 2 weeks, with associated malaise and poor appetite. He had dark urine for the past 2 days. There was no dysuria or frequency of urination. There was no cough or runny nose or sore throat recently. There was no joint pain or swelling and there was no skin rash. Family members were healthy. There was no recent travel. On admission, body temperature was 39.5 oC. Blood pressure was 100/70 mmHg. Heart rate was 110/min. Physical examination showed mild pallor. Chest was clear. Apex was not displaced. There was no abnormal cardiac impulse. A grade 3/6 systolic murmur was heard at the left sternal border. Liver was 2 cm below costal margin and a spleen tip was felt.

Investigations results were as follows:White blood cell (WBC)17x 109/l

neutrophil14x 109/l

lymphocyte2x 109/l

haemoglobin10 g/dl

Urea5.3 mmol/l

creatinine40 mol/l

alanine aminotransferase (ALT)30 IU/l

aspartate aminotransferase (AST)40 IU/l

Urinalysis: white blood cell (WBC) 2/HPF, numerous dysmorphic red blood cells

Which THREE of the following investigations were the most important?

Blood culture Incorrect

Viral titre Incorrect

Chest X-ray Incorrect

Echocardiogram Incorrect

Ultrasound scan of abdomen Incorrect

Urine culture Incorrect

Erythrocyte sedimentation rate (ESR) Incorrect

Antinuclear antibody Incorrect

Antistreptolysin O titre

Incorrect

Blood cultureEchocardiogramErythrocyte sedimentation rate (ESR)The patient most likely had infective endocarditis, as suggested by prolonged fever with malaise, anorexia, hepatosplenomegaly and anemia. There was an underlying heart lesion manifested as a cardiac murmur, which was unlikely to be flow murmur because of its loud intensity. Complication of renal emboli or infarction had occurred as suggested by the presence of dysmorphic RBCs in the urine. The most important investigation was several sets of blood culture to aid diagnosis and to identify the infecting organisms. Echocardiogram was important to demonstrate the presence of vegetations and the underlying heart lesion. ESR was an important marker of inflammation to monitor response to treatment. Viral titre was not indicated since viral infection rarely persists with high fever for 2 weeks. Neutrophilia in this case indicated bacterial infection rather than viral infection. Chest X-ray is an adjunct in any cardiac condition, but is not an essential investigation in infective endocarditis. There was no sign of chest involvement in this case. Ultrasound of the abdomen could demonstrate hepatomegaly and splenomegaly but added little diagnostic information in this case. Urine culture was not indicated in this case since he did not have symptoms of urinary tract infection and there was no pyuria. Antinuclear antibody is a screening test for suspected systemic lupus erythaematosus. It is rare in boys of this age, though it can cause prolonged fever with hepatosplenomegaly, anemia and glomerulonephritis. Antistreptolysin O titre serves as evidence of recent Streptococcus pyogenes infection to aid diagnosis of post-streptococcal glomerulonephritis or acute rheumatic fever. However, these diagnoses were unlikely in this case. Post-streptococcal glomerulonephritis does not result in hepatosplenomegaly or heart murmur. Acute rheumatic fever was unlikely at this young age and there were not enough criteria, such as erythaema marginatum, subcutaneous nodules, arthritis or Sydenhams chorea.A 15-month-old girl is referred by her GP with a heart murmur. She is well and thriving and there are no concerns over her development. She was born at term by NVD and had no neonatal problems. She has had two chest infections in the last 5 months and received antibiotics for these. Her mother describes no episodes of breathlessness of cyanosis.

On examination she is comfortable with no respiratory distress. Auscultation revels normal first heart sound, fixed widely split second heart sound and a 2/6 ejection systolic murmur. Her chest is clear and abdominal exam is unremarkable. Chest X-ray (CXR) is within normal limits and electrocardiogram (ECG) reveals right-axis deviation.

What is the most likely diagnosis? Primum ASD

Secundum ASD Correct answer

VSD

PDA

Truncus arteriosus

The most likely diagnosis is a secundum ASD. This can present as an incidental finding or the child may have a degree of failure evident. Any increase in pulmonary blood flow from left to right shunts can predispose to chest infections.

ASD accounts for 6% of congenital heart lesions and secundum defects occur at the level of the foramen ovale. The signs outlined in the above case are classical of ASD with an ejection systolic murmur caused by increased flow across the right ventricular outflow tract because of the left to right shunt. The electrocardiogram (ECG) in a secundum defect will show RAD due to right ventricular hypertrophy, whereas a primum defect leads to LAD or superior axis on ECG. These defects can be repaired in early childhood usually with an autologous pericardial patch. What are the complications of coarctation of the aorta repair?

Choose THREE options Ischaemia of the spinal cord Incorrect

Pulmonary hypertension Incorrect

Systemic hypertension Incorrect

Thromboembolism Incorrect

Re-coarctation Incorrect

Ischaemia of the spinal cordSystemic hypertensionRe-coarctationCoarctations are repaired by either end-to-end anastomosis or subclavian flap repair. Systemic hypertension is not uncommon postoperatively but usually resolves without long-term treatment. There is a small incidence of re-coarctation (or residual constriction) and ischaemia of the spinal cord resulting in paralysis is a rare complication ( 1.5 cm diameter) Changes in the hands and feet (erythema, oedema and later desquamation.

Most of the blood results in this condition are non-specific but the platelet count can be high.

The diagnosis is important as approximately one-third of affected children develop coronary artery aneurysms (the risk of which can be lowered with appropriate treatment such as iv immunoglobulin and aspirin)

The following data were obtained during a cardiac catheterisation of a baby who was suspected of having a cardiac defect.

SiteOxygen saturations %

Left atrium 96

Left ventricle 96

Aorta96

Right atrium 74

Right ventricle74

Pulmonary artery 89

Which of the following is the most likely diagnosis?Ventricular septal defect

Pulmonary stenosis

Coarctation of the aorta

Atrial septal defect

Patent ductus arteriosus Correct answer

This is a patent ductus arteriosus. A step-up of oxygen saturation is seen between the right ventricle and the pulmonary artery, suggesting that oxygenated blood has been shunted from left to right at this point. (Also remember that an increase in pressure between two sites suggests a stenosis upstream.)Incorrect

Which of the following conditions are associated with this defect? TWO choices only.Congenital rubella syndrome Incorrect

Turners syndrome Incorrect

Noonans syndrome Incorrect

Maternal systemic lupus erythematosus (SLE) Incorrect

Williams syndrome Incorrect

Marfans syndrome Incorrect

Homocystinuria Incorrect

Downs syndrome Incorrect

Congenital rubella syndromeDowns syndromeTurners syndrome is associated with coarctation and aortic stenosis, while Noonans syndrome is associated with pulmonary stenosis and peripheral pulmonary stenosis. Supravalvular aortic stenosis and pulmonary stenosis are seen in Williams syndrome. Marfans syndrome is associated with aortic incompetence and aortic dissection.A 12-year-old girl is brought to the Accident and Emergency Department after collapsing at home while revising for exams. On arrival she is alert, heart rate (HR) 110, respiratory rate (RR) 18, Sats 99% and blood pressure (BP) 115/72. She cannot remember what happened and had no warning before the episode. Her parents witnessed the episode and say that she just blacked out and fell to the floor, cutting her head in the process and had no abnormal movements. She has had a couple of episodes in the past year of feeling dizzy and faint but has not collapsed like this. She is otherwise well with no past medical history of note and there is no significant family history.

Examination is unremarkable including full neurological exam. Electrocardiogram (ECG) shows sinus rhythm, normal axis, ST segments and T waves with QTc of 0.5 s.

What is the most likely diagnosis? RomanoWard syndrome

Atrial tachycardia

Aortic stenosis

Prolonged QT syndrome Correct answer

Hypertrophic obstructive cardiomyopathy (HOCM)

This is a history of cardiac syncope with no prodrome and the fact that she sustained an injury while falling supports a blackout episode (as opposed to gently fainting to the floor!). The previous episodes of near-syncope could also be related to this episode. The electrocardiogram (ECG) finding of corrected QT measurement of 0.5 s is prolonged (should be < 0.44 s) and supports the diagnosis of prolonged QT syndrome. This often presents later in childhood and can be provoked by exercise, stress or emotion. Forms can be genetic (AR, AD (Romano ward), Brugada syndrome) or associated with congenital deafness (JervellLangNielsen syndrome). Electrolyte abnormalities should be ruled out. Prolonged QT interval predisposes to ventricular tachyarrhythmias, which are the cause of sudden death; torsades de pointes are particularly common in this disease. Treatment involves -blockade and insertion of an implantable defibrillator. If children present in VT or VF they should be treated according to the APLS protocols.

Aortic stenosis can present with syncope but there would be abnormal findings on cardiovascular examination. HOCM may present with displaced apex beat and a heart murmur but one may see electrocardiogram (ECG) changes consistent with hypertrophy. You are asked to review a 2-hour-old baby who was born by emergency LSCS for fetal distress. Apgar scores were 71, 95, 910 and baby required two bag and mask breaths. The baby has remained tachypnoeic since birth with some respiratory distress and has not been able to feed yet. There are no risk factors for infection.

Heart rate (HR) is 230 bpm, respiratory rate (RR) 68 and Sats 95% in air. Temperature is 37.3 oC. On examination the baby has intercostal and subcostal recession and no murmurs audible. A 2-cm liver edge is palpable.

The baby is transferred to NICU for monitoring. Chest X-ray (CXR) shows no cardiomegaly or pulmonary plethora. A 12-lead electrocardiogram (ECG) reveals a narrow complex tachycardia at rate of 240 bpm.

Which of the following are useful in the management of neonatal supraventricular tachycardia (SVT)? Icepacks to face Incorrect

Verapamil Incorrect

Adenosine iv Incorrect

Propranolol iv Incorrect

Cardioversion dc Incorrect

Icepacks to faceAdenosine ivCardioversion dc

SVT can be difficult to diagnose in neonates as the symptoms are similar to other neonatal conditions but electrocardiogram (ECG) should be diagnostic. In older children it can present with palpitations, shortness of breath or even syncope. In neonates the SVT is usually a re-entrant meaning the electrical activity goes down through the atrio-ventricular node and then passes retrogradely up the accessory pathway.

Investigations should include a 12-lead ECG; check electrolytes (potassium, calcium and magnesium abnormalities can affect heart rhythm) and an Echo to rule out structural heart disease (particularly Ebsteins anomaly and cardiomyopathy, both associated with tachyarrhythmia).

Management is aimed at restoring sinus rhythm and depends on the clinical state of the infant. If the baby is not haemodynamically compromised then vagal manoeuvres can be tried. In babies this can be placing ice packs on the face for brief periods of time (the diving reflex). Adenosine is useful to determine the underlying pathway and is useful in terminating arrhythmia if an accessory pathway is present. Adenosine should be used under continuous electrocardiogram (ECG) monitoring and the trace marked when adenosine has been given. Dosing starts at 0.05 mg/kg and can be increased incrementally to 0.25 mg/kg. The drug should be given into a large vein if possible and flushed quickly as the half-life is very short. It is not unusual for the SVT to return once the adenosine has worn off. If the baby is unstable then they should be intubated and ventilated and synchronised DC cardioversion used (0.052 J/kg). Amiodarone is another anti-arrhythmic drug that can be used but requires loading doses. Maintenance treatment can be amiodarone or flecainide but most children can stop maintenance after 1 year of age, as 90% will have no further attacks. Verapamil is not used as it can cause profound hypotension.

Match the following maternal disorders and teratogens with the correct heart diseases.

A Rubella in pregnancy B Lithium C SLE D Phenytoin E Fetal alcohol syndrome F HHV6 in pregnancy G Aspirin

Scenario 1

Incorrect

Patent ductus arteriosus (PDA)

0 Your answerA Correct answerRubella in pregnancy Rubella in pregnancy causes congenital heart lesions in up to 35% of babies born to affected mothers. The two conditions associated with rubella are PDA and peripheral pulmonary stenosis and it is infection before 8 weeks gestation, which causes heart disease. Other features of congenital rubella include cataracts and deafness. Scenario 2

Incorrect

Atrial septal defect

0 Your answerE Correct answerFetal alcohol syndrome Up to 70% of children with fetal alcohol syndrome have cardiac lesions, which are variable. They include ASD, VSD, tetralogy of Fallot and coarctation of the aorta. Children also have a typical facies with long, featureless philtrum and developmental delay.

Scenario 3

Incorrect

Aortic stenosis

0 Your answerD Correct answerPhenytoin Phenytoin is teratogenic and causes the fetal hydantoin syndrome. Apart from craniofacial abnormalities and nail abnormalities cardiac lesions include aortic stenosis, pulmonary stenosis, coarctation of the aorta and PDA.

Scenario 4

Incorrect

Congenital heart block

0 Your answerC Correct answerSLE SLE and Sjgrens syndrome can affect the fetus causing congenital heart block. The cause is anti-Ro antibodies, which cross the placenta. Babies can be severely affected and born hydropic requiring urgent treatment. Management will depend on the stability of the baby; if the baby is haemodynamically unstable a chronotropic agent (isoprenaline) may be required. Implantable pacemakers are available but insertion is limited by the size of the infant.

Scenario 5

Incorrect

Ebsteins anomaly

0 Your answerB Correct answerLithium Lithium used to treat maternal bipolar disorder can cause Ebsteins anomaly. The anatomical lesion is an abnormal tricuspid valve whose leaflets are stuck to the ventricular wall. There is an ASD and the inlet of the right ventricle becomes arterialised. Infants are cyanosed and often do not thrive. They are prone to SVTs. The characteristic murmur is a long systolic murmur and also a diastolic murmur. Management depends on severity and age at presentation but neonates may require nitric oxide for pulmonary vasodilation or the ductus arteriosus opening. Neonatal presentation has a poor prognosis.

A 12-hour-old baby is found on the postnatal ward to be grunting and dusky. He was born at term by normal vaginal delivery (NVD) after an uneventful pregnancy with normal antenatal scans. On examination he has increased work of breathing with subcostal and intercostal recession, nasal flaring and grunting. He has a systolic murmur and 2-cm liver edge palpable. Capillary refill time is 3 s and he is alert. His heart rate (HR) is 190 and respiratory rate (RR) 68. Oxygen saturations are measured as 72%. He is transferred to the neonatal intensive care unit (NICU) where his perfusion becomes poor and respiratory effort reduced.

What are the immediate next steps in the management of this baby? (Choose TWO).Intubation and conventional ventilation Incorrect

High-flow oxygen Incorrect

High-frequency oscillation ventilation (HFOV) Incorrect

Inhaled nitric oxide Incorrect

Prostaglandin infusion Incorrect

Inotropic support Incorrect

Intubation and conventional ventilationProstaglandin infusion

This is a classical presentation of a baby with a duct-dependent cardiac lesion becoming symptomatic as the duct closes shortly after birth. Here the symptoms are respiratory distress, poor perfusion and cyanosis and cyanotic congenital heart disease must be considered despite the history of normal antenatal scans.

The initial management of this neonate begins with airway, breathing and circulation as any resuscitation would. It does not matter what the exact diagnosis is, as the management will be essentially the same, but duct-dependent lesions should be considered as this might influence commencing a prostin infusion. As the baby has poor perfusion and reduced respiratory effort it would be appropriate to intubate and ventilate the baby (airway and breathing) but HFOV would not be necessary at this stage. Prostin infusion may help to keep open a closing duct and buy time to allow full diagnosis and transfer to appropriate cardiac centre. Inhaled nitric oxide is not indicated in the initial management but may be of use if pulmonary hypertension is suspected. Inotropic support may be indicated but this would depend on blood pressure measurements and perfusion once ventilated. Theoretically high-flow oxygen is not advised in duct-dependent lesions as oxygen stimulates ductal tissue to contract and close the duct but often babies need FiO2 >21% to maintain saturations in the 70s. A 3-year-old boy is seen in the Accident and Emergency Department with a 6-day history of fever and irritability. He saw his GP 3 days previously, who prescribed a course of oral antibiotics for tonsillitis but his fever has continued and he has become more miserable. Today his mother noticed his hands were red and the skin was starting to peel and on direct questioning she remembers his eyes being quite red a few days earlier.

His temperature is 39.6 oC, heart rate (HR) 140, respiratory rate (RR) 36, Sats 97% with immediate capillary refill. His chest is clear with normal first and second heart sounds and no murmurs. Abdominal examination is unremarkable. His throat is red with slightly enlarged tonsils but no pus and a 2-cm cervical lymph node palpable on the left side of his neck. He has a blanching maculopapular rash over his trunk.

Blood results are as follows: Hb 11.0

WCC21.1

NQ16.8

platelets (Plt)500

clottingnormal

electrolytes and renal functionnormal

aspartate aminotransferase (AST)40

C-reactive protein (CRP) 198

chest X-ray (CXR) and electrocardiogram (ECG)unremarkable

What is the most likely diagnosis?Rheumatic fever

Kawasakis disease Correct answer

Infective endocarditis

HenochSchnlein purpura

Streptococcal infection

The most likely diagnosis is Kawasakis disease, a vasculitis illness also known as mucocutaneous lymph node syndrome. It is characterised by fever lasting more than 5 days, polymorphous rash, non-suppurative conjunctivitis, cervical lymphadenopathy and swelling and desquamation of the hands and feet. Children are also typically very miserable and irritable. Blood results can show raised white cell count (WCC) and C-reactive protein (CRP), transaminitis and after the second week (usually) rising platelet count which can reach >1000.

Treatment consists of intravenous immunoglobulin (either one dose or two depending upon response) and aspirin. Initially the aspirin dose is anti-inflammatory (30100 mg/kg per day) which then falls to 25 mg/kg per day once the fever has settled. This is continued depending on echo findings.

Echo scan should be performed in the 2nd or 3rd week of the illness and is to look for coronary artery aneurysms, one of the main complications of Kawasakis disease and now the leading cause of acquired heart disease in children in the western world. Patients who are unresponsive to intravenous immune globulin (IVIG) (temperature does not settle) are more likely to form aneurysms. If the echo is abnormal repeated scans would be required to determine if the lesion is static. Most aneurysms will regress within 2 years.

An alternative diagnosis could be streptococcal infection (toxic shock syndrome) with tonsillitis and desquamation with raised inflammatory marker and white cell count, but this child does fit the criteria for Kawasakis disease. A 10-year-old boy is seen in A&E with a minor injury to his ankle sustained while playing football. On doing his observations, the nurse looking after him notes his blood pressure to be 180/100 and you are asked to review him.

On history, he is otherwise well. He takes no regular medications and is normally active and plays football twice a week. His weight and height are within normal limits. There is no other past medical history of note but mum does mention that he had a urinary tract infection when he was younger which cleared up with antibiotics.

On examination, he looks well, his chest and cardiovascular examinations are normal and his abdomen is soft and non-tender with no palpable masses. You re-check his blood pressure and this is still raised with a systolic reading of 170 in both arms.

What is the most likely cause of the high blood pressure in this child? Choose one answer only:

Anxiety

Phaeochromocytoma

Neuroblastoma

Renal scarring

Correct answer

Co-arctation of the aorta

The most likely cause of high blood pressure in a child is secondary to another underlying condition. It is very important to re-check the readings, ensuring the correct cuff size is used and that the blood pressure is checked in both arms.

In a child with a history of a urinary tract infection, the most likely cause would be renal parenchymal disease, eg scarring secondary to reflux nephropathy. Therefore it would be important to check urea and electrolytes and perform a renal ultrasound and a DMSA scan in order to exclude this as the cause. Other investigations looking for other causes of secondary hypertension (eg catecholamine excess secondary to phaeochromocytoma or steroid excess due to Cushings syndrome) should also be performed.

Hypertension in a child can be asymptomatic or may present with proteinuria, headaches, seizures or cardiac failure. It is important to do a full examination looking specifically for abdominal masses and evidence of papilloedema in any child presenting with hypertension. A 10-year-old girl is admitted to the general paediatric ward with a fever and polyarthritis. She has had swelling of her ankles and wrists over the last week (the swelling is starting to improve) and yesterday developed redness and swelling of both knees as well.

On examination, there is limited movement of her knee and ankle joints due to pain. There is evidence of pericarditis on cardiovascular exam in the form of a pericardial friction rub and she has a temperature of 39 C. There is no rash at present though her mum does describe a rash which was present 2 weeks before which sounds like a macular papular rash with a darker border to the lesions.

You do some routine blood tests which show:

Hb11.6

WCC16.3

platelets458

CRP67

ESRis raised at 42

On further questioning, her mother mentions that they moved to the UK from Zimbabwe 3 years previously and that she had a similar illness shortly before they moved but this resolved spontaneously. They went back to Zimbabwe to visit relatives approximately 6 weeks ago.

What is the most common long-term cardiac problem resulting from this condition? Choose one answer only.Aortic stenosis

Mitral regurgitation

Tricuspid stenosis

Mitral stenosis

Correct answer

Constrictive pericarditis

The features described would fit with a diagnosis of acute rheumatic fever. This is caused by an abnormal immune response to a preceding Group A beta-haemolytic streptococcus (Streptococcus pyogenes) infection. It is more common in developing countries. Rheumatic fever is diagnosed based on the finding of clinical criteria (Jones criteria). Major criteria include pancarditis, polyarthritis, erythema marginatum, Sydenhams chorea and subcutaneous nodules. Minor criteria include fever, polyarthralgia, history of rheumatic fever, raised acute phase reactants (ESR and CRP) and prolonged PR interval on ECG. Two major or one major and two minor criteria are required to make the diagnosis as well as evidence of previous Group A strep infection.

The most common long-term problem is mitral stenosis resulting from scarring and fibrosis of the valve. More rarely, aortic and tricuspid valve disease can occur. Valve disease can develop up to 5 to 10 years after an attack of acute rheumatic fever. Symptoms do not usually develop until later in life. Prophylaxis against further attacks of rheumatic fever is recommended after an initial episode (penicillin).

An anxious mother brought a 4-week-old baby girl to a general practitioner complaining that she had been feeding poorly in the past 1 week. Her antenatal and perinatal course was uneventful. Pre-discharge examination on day 2 of life was entirely normal. On physical examination, the body temperature was 39 oC and the child had profuse sweating. Respiratory rate was 60/min. Heart rate was 180/min. Breath sounds were normal but there were crepitations heard occasionally. All peripheral pulses were easily palpable. Apex beat was located at the 6th intercostal space in mid-axillary line. There was a strong parasternal impulse. The second heart sound was split and a grade 2/6 systolic murmur was heard at left sternal border.

Which of the following was the most likely diagnosis? Tetralogy of Fallot

Ventricular septal defect (VSD) Correct answer

Patent ductus arteriosus

Myocarditis

Pneumonia

Ventricular septal defect was the most compatible diagnosis with the clinical description. A normal newborn examination could not exclude a congenital heart disease. The heart murmur of ventricular septal defect and heart failure symptoms might not be obvious in the first few days of life when the pulmonary arterial pressure was still high. With progressive fall of pulmonary arterial pressure, heart failure symptoms would become more obvious and the child might have poor feeding, tachypnoea, displaced apex, basal lung crepitations and hepatomegaly. The strong parasternal impulse could be caused by pulmonary hypertension due to high pulmonary blood flow from left to right shunt through the large VSD. Fever in a child with congenital heart disease raised the possibility of infective endocarditis, which should be considered in this patient. A strong parasternal impulse is also present in patients with Tetralogy of Fallot. But it usually causes cyanosis without heart failure symptoms and signs, unless it is the type of pink Fallot with large VSD and mild pulmonary stenosis, which has symptoms and signs sometimes indistinguishable from a large VSD. But pink Fallot is certainly rarer than a large VSD. Patent ductus arteriosus can present as heart failure and give easily palpable pulses. However, the murmur is usually continuous unless it is a small-sized ductus, which may only have murmur detectable in the systolic phase. However, a small ductus should not present with heart failure symptoms and signs. Acute myocarditis could cause heart failure symptoms and signs as in this patient, as well as the fever. However, it could not account for the heart murmur. Similarly, while pneumonia might cause fever and tachypnoea and basal crepitations, it could not account for the displaced cardiac apex and murmur. Nevertheless, pneumonia can complicate most congenital heart diseases with haemodynamic disturbances, such as a large VSD.Incorrect

What THREE of the following investigations were the most important in this case?Echocardiogram

Incorrect

Cardiac catheterisation

Incorrect

Chest X-ray

Incorrect

Blood culture

Incorrect

Virus study

Incorrect

Chromosomal analysis Incorrect

EchocardiogramChest X-rayBlood culture Echocardiogram was the single most important investigation to define the underlying structural heart defects. Chest X-ray was an adjunct to aid diagnosis of congenital heart disease. It was of higher importance in this case since the child had fever with lung crepitations. The child might have pneumonia complicating the ventricular septal defect. A chest X-ray also helped to assess the degree of left to right shunt by visualizing the amount of pulmonary vasculature. Blood culture was an important investigation of possible infective endocarditis in this case. Cardiac catheterisation was not urgently needed in this case. It might be performed if operative repair of the VSD was considered at a later stage after fever settled. Virus study is more important in cases of myocarditis, although the fever in this patient might have been caused by a virus. This was of lower importance here, since viral identification probably would not help the management in this setting. Chromosomal analysis is worth considering in patients with congenital heart disease. But it is of secondary importance for patient management and frequently has poor yield if there is no other associated dysmorphic features or other malformations.

Choose the most common genetic association for each of the conditions described:

A Downs syndrome B Williams syndrome C Di George syndrome D Noonans syndrome E Marfans syndrome F PraderWilli syndrome

Scenario 1

Incorrect

A baby is born with an antenatal diagnosis of a chromosome abnormality, initially suspected by the finding of increased nuchal translucency on ultrasound. She has a complete AVSD (atrioventricular septal defect) on cardiac echo.

0 Your answerA Correct answerDowns syndromeDowns syndrome is often associated with congenital heart disease and there is a 30% incidence of heart defects in these babies. The most common condition is a complete AVSD (also called an endocardial cushion defect).

Scenario 2

Incorrect

A baby has a truncus arteriosus diagnosed antenatally. After birth, he is noted to have lymphopenia, hypocalcaemia and a cleft palate as well as characteristic facial features. FISH testing of chromosomes shows a deletion on chromosome 22.

0 Your answerC Correct answerDi George syndromeDi George syndrome is caused by a deletion on chromosome 22 (22q11.2 deletion). It results in abnormal development of the third and fourth pharyngeal pouches during intrauterine life. Cardiac associations can include trucus arteriosus, interrupted aortic arch, Tetralogy of Fallot and VSD. Hypocalcaemia can occur and usually presents in the first few days or weeks of life. There is thymic hypoplasia leading to lymphopaenia. Characteristic facial features include hypertelorism, low-set ears, antimongoloid slant to eyes, micrognathia, carp-shaped mouth and cleft palate.

Scenario 3

Incorrect

A 14 year old boy presents with classical clinical and echo features of mitral valve prolapse with mitral regurgitation which was diagnosed after a murmur was heard by his GP. He is tall, as are other members of his family and his only medical history of note is a spontaneous pneumothorax one year ago.

0 Your answerE Correct answerMarfans syndromeMarfans syndrome is an autosomal dominant condition which results in a defect in the formation of collagen. Patients are tall and may have features of joint laxity, arachnodactyly, pectus excavatum, kyphoscoliosis and high arched palate. Lens dislocation and myopia may occur. Cardiac associations include aortic and mitral valve regurgitation as well as a dilated aortic root with the possibility of aortic dissection.

Which of the following conditions are associated with pulmonary stenosis? Choose TWO options only.Pompes disease Incorrect

Alagilles syndrome Incorrect

Friedreichs ataxia Incorrect

Turners syndrome Incorrect

Marfans syndrome Incorrect

Homocystinuria Incorrect

Fetal alcohol syndrome Incorrect

Williamss syndrome Incorrect

Alagilles syndromeWilliamss syndromePulmonary stenosis is seen in Noonans, Williams and Alagilles syndromes. Some other associations include: Pompes disease and Friedreichs ataxia: hypertrophic obstructive cardiomyopathy (HOCM) Turners syndrome: aortic stenosis, coarctation Marfans syndrome: aortic dissection, aortic incompetence.A 4-month-old infant has undergone an atrioventricular septal defect (AVSD) repair and pre-operatively was in failure with a large left to right shunt and failure to thrive. Shortly after return from theatre his blood pressure and oxygen saturations fall consistent with a pulmonary hypertensive crisis.

What is the management? Sedation paralysis Incorrect

Increase FiO2 Incorrect

Inhaled nitric oxide Incorrect

Dopamine infusion Incorrect

Prostaglandin infusion Incorrect

Ventilate in air Incorrect

Sedation paralysisIncrease FiO2Inhaled nitric oxide Children who have a large left to right shunt are at risk of pulmonary hypertension postoperatively, so a 4-month-old baby with AVSD, who was clinically in failure and not thriving, is at risk. Pulmonary hypertensive crises are caused by a change in pulmonary vascular resistance leading to reduce pulmonary blood flow. This manifests clinically as desaturation followed by hypotension. If the right atrial pressure was being measured this would also be seen to rise.

If a child is ventilated they should be put into 100% oxygen and hand bagged so they can be hyperventilated to promote pulmonary vasodilation. Sedation is also important and paralysis can also be useful. Inhaled nitric oxide is a potent pulmonary vasodilator and may be useful if crises persist. Pre-oxygenation in 100% oxygen and sedation before stimulation is recommended (eg before physiotherapy or suctioning).

Although hypotension is a result of pulmonary hypertension dopamine infusion is probably not necessary for this reason (may be needed for myocardial dysfunction) and prostaglandin is not indicated.

A baby is born in good condition at term and requires no resuscitation at birth. There are no antenatal concerns and he is transferred to the postnatal ward with mum. You are called to review him at approximately 12 hours of age because he is feeding poorly, has started grunting and according to the midwives is a strange colour.

On arrival he is slightly tachypneic with mild subcostal recessions but is not grunting at present. He is noticeably cyanosed and therefore you immediately transfer him to special care. His saturations are 75% in air on arrival on the special care baby unit but they improve to 80% with 100% oxygen.

On examination, he is mildly tachypnoeic with recessions. His chest is clear. On auscultation you can hear a systolic murmur all over the praecordium.

What should your first steps in management be? Choose two answers only.Start iv antibiotics Incorrect

Check blood glucose

Incorrect

Start iv fluids

Incorrect

Start an infusion of Prostin

Incorrect

Give oxygen

Incorrect

Do an ECG Incorrect

Check blood glucoseStart an infusion of ProstinIn any unwell newborn with grunting and respiratory signs, it is important to check a blood glucose. This can be done on a capillary sample and is a relatively straightforward thing to correct if abnormal. It should therefore be included in your initial assessment.

In this baby there are signs of cyanosis which does not correct significantly with oxygen treatment. You are suspecting a cardiac condition in this case. Babies who become cyanosed shortly after birth due to cardiac causes possibly have a duct-dependent cardiac lesion and may worsen significantly once the patent ductus arteriosus closes (thereby effectively decreasing blood flow to the lungs further). In these babies it is important to try and keep the duct open and this is done with the use of prostaglandin E (Prostin).

An ECG is usually normal and unhelpful in this setting. Antibiotics and iv fluids may be indicated but are usually unnecessary and there is the risk of causing fluid overload in such babies. However, iv 10% dextrose is often used, especially in babies with low blood sugars or who are unwell. Oxygen is of no significant benefit in these babies.

Incorrect

A chest X-ray is performed which shows clear lung fields and a normal heart shadow with possibly a slightly narrowed upper mediastinum.

What is the most likely diagnosis?

Hypoplastic left heart dyndrome

Ventricular septal defect (VSD)

Tetralogy of Fallot

Coarctation of the aorta

Transposition of the great arteries Correct answer

Transposition of the great arteries is the commonest type of cyanotic heart disease in the newborn and the most likely diagnosis in a cyanosed neonate with narrowing of the upper mediastinum on chest x-ray. A murmur suggests a coexistent VSD or pulmonary stenosis or may be due to a patent ductus arteriosus. The diagnosis is made by echocardiography which shows the aorta arising from the right ventricle and the pulmonary artery from the left.

Neither a VSD nor coarctation of the aorta would present with cyanosis in a neonate but would more commonly present with heart failure later in infancy.

Tetralogy of Fallot usually presents in infancy and causes cyanosis if there is severe right ventricular outflow tract obstruction. Chest X-ray here shows a boot-shaped heart with pulmonary oligaemia.

Hypoplastic left heart syndrome presents with heart failure rather than cyanosis.

A 9-month-old baby with tetralogy of Fallot is admitted to hospital with increased hypercyanotic spells.

How do you manage spelling? Choose FOUR options. 100% oxygen Incorrect

Dopamine infusion Incorrect

-blockers Incorrect

Draw legs up to chest Incorrect

Morphine Incorrect

100% oxygen-blockersDraw legs up to chestMorphine Tetralogy of Fallot is a complex cyanotic congenital heart disease comprising of VSD, right ventricular outflow tract obstruction (RVOTO), overriding aorta and right ventricular hypertrophy. The condition results in reduced blood flow to the lungs and presentation is variable depending on the lesion and size of the pulmonary arteries. If pulmonary arteries are small then infants can present early with cyanosis. Correction involves either a definitive repair or a two-stage procedure; BlalockTaussig shunt early to ensure adequate pulmonary blood flow followed by definitive repair (VSD closed and transannular patch repair of the RVOTO). Postoperative complications include right ventricular dysfunction and dysrhythmias.

Spelling is caused by spasm of the infundibular muscle which causes shunting of blood from right to left across the VSD. This leads to increased cyanosis and hypotension. Management of spells involves 100% oxygen, drawing the legs up to the chest to increase systemic venous return and -blockers, generally propranolol. Morphine can be useful to reduce any pain and calm the baby as cyanosis worsens as the baby becomes more upset. Match the following clinical scenarios with the relevant arrhythmia.

A Junctional ectopic tachycardia (JET) B Sinus arrhythmia C WolffParkinsonWhite syndrome D First degree heart block E SVT F Torsade de pointes G Prolonged QT syndrome H Complete heart block

Scenario 1

Incorrect

A neonate is born with a heart rate of 60 bpm but is haemodynamically stable. Electrocardiogram (ECG) shows P waves dissociated from QRS complexes

0 Your answerH Correct answerComplete heart block A heart rate of 60 bpm in a neonate is pathological but this baby is haemodynamically stable so cardiac massage is not appropriate. If the P waves bear no relation to QRS complexes this is complete heart block and a common cause of congenital complete heart block is maternal SLE.

Scenario 2

Incorrect

A 14-year-old girl arrives in the Accident and Emergency Department following an overdose. Her electrocardiogram (ECG) shows polymorphic ventricular tachycardia with QRS complexes of varying amplitudes

0 Your answerF Correct answerTorsade de pointes This is a description of torsades de pointes, a polymorphic form of VT where complexes change in amplitude and the QRS axis twists around the isoelectric line. It can spontaneously terminate but can degenerate into VT or VF causing sudden death. Causes include tricyclic antidepressant overdose, hypocalcaemia, hypomagnesaemia and prolonged QT syndrome.

Scenario 3

Incorrect

A child is being investigated for episodes of palpitations. Electrocardiogram (ECG) reveals slightly shortened PR interval and waves

0 Your answerC Correct answerWolffParkinsonWhite syndrome WolffParkinsonWhite syndrome is caused by an accessory pathway between the atria and ventricles. Symptoms include episodes of SVT, palpitations, chest pain and dizziness. Characteristic electrocardiogram (ECG) findings are a wave (a slurring of the Q upstroke), short PR interval and wide QRS complexes. Accessory pathways can be treated with ablation.

Scenario 4

Incorrect

A neonate is born with a heart rate of 60 bpm but is haemodynamically stable. Electrocardiogram (ECG) shows P waves dissociated from QRS complexes

0 Your answerH Correct answerComplete heart block A heart rate of 60 bpm in a neonate is pathological but this baby is haemodynamically stable so cardiac massage is not appropriate. If the P waves bear no relation to QRS complexes this is complete heart block and a common cause of congenital complete heart block is maternal SLE.

Scenario 5

Incorrect

A 10-month-old boy returns from theatre following AVSD repair. After a few hours his heart rate (HR) increases to 190 bpm and regular rhythm. A 12-lead electrocardiogram (ECG) reveals QRS complexes of normal morphology but dissociated from P waves (atrio-ventricular dissociation)

0 Your answerA Correct answerJunctional ectopic tachycardia (JET) This is a classical history and findings of JET. It may occur following cardiac surgery; infants at higher risk are those who have had a right ventriculotomy such as atrio-ventricular septal defect (AVSD) repair and Fallots repair. The rhythm is temperature sensitive, which is why keeping these children cool, can help control the tachycardia. There is atrio-ventricular (AV) dissociation and the ventricular rate exceeds the atrial rate (so rate generally between 170200 bpm) with normal QRS complexes and often retrograde P waves. Management involves correcting any electrolyte imbalances, cooling, temporary pacing via pacing wires placed during the operation and amiodarone.

Select ONE condition that most closely matched the description.

A Small ventricular septal defect B Large ventricular septal defect C Mitral regurgitation D Mitral stenosis E Aortic stenosis and regurgitation F Pulmonary stenosis and regurgitation G Patent ductus arteriosus H Atrial septal defect

Scenario 1

Incorrect

A 3-month-old boy presented with progressive shortness of breath for 2 months and poor feeding. Weight gain was poor. Physical examination showed mild subcostal recession. Chest was clear. Pulse was normal. A right ventricular impulse was felt. Apex beat was located at the 6th intercostal space in mid-clavicular line. The second heart sound was split and there was a grade 3/6 systolic murmur over the left sternal border.

0 Your answerB Correct answerLarge ventricular septal defect The patient had symptoms of heart failure resulting from a large ventricular septal defect. The displaced apex indicated left ventricular volume overload. The right ventricular impulse was due to pulmonary hypertension secondary to increased pulmonary blood flow. The murmur of VSD is typically pansystolic. Small ventricular septal defect does not result in heart failure symptoms or displaced apex. Mitral regurgitation can result in pulmonary venous congestion causing respiratory distress. It also causes left ventricular volume overload with displaced apex and a pansystolic murmur. But the murmur should be best heard at the apex rather than the left sternal border.

Scenario 2

Incorrect

An asymptomatic 3-year-old girl was told to have a heart murmur by a general practitioner. Physical examination showed a strong pulse. Apex was located at the 5th intercostal space in mid-clavicular line. There was no parasternal heave. Heart sounds were split and there was a grade 3/6 systolic murmur and grade 2/6 diastolic murmur over the left sternal border.

0 Your answerG Correct answerPatent ductus arteriosus The child likely had a patent ductus arteriosus, as suggested by the strong pulse. It typically causes a continuous machine-like murmur. However, sometimes there may be a short split between the systolic and diastolic phase, resulting in both systolic and diastolic murmurs. Aortic stenosis and regurgitation can result in both systolic and diastolic murmurs. However, the pulse should not be strong because of aortic stenosis. Pulmonary stenosis and regurgitation can also result in both systolic and diastolic murmurs. However, the pulse should be normal and a right ventricular impulse should be felt.

Scenario 3

Incorrect

A 10-year-old child had occasional palpitations. Physical examination showed a normal pulse. Apex beat was located at the 6th intercostal space in mid-clavicular line. There was no parasternal heave. Heart sounds were split and there was a grade 3/6 systolic murmur over the apex.

0 Your answerC Correct answerMitral regurgitation The patient most likely had mitral regurgitation. Palpitation might be secondary to left atrial enlargement and associated atrial fibrillation. The apex was displaced due to left ventricular volume overload. A pansystolic murmur best heard at the apex radiating to axilla is classical.

Select ONE condition that most closely matched the description.

A Infective endocarditis B Acute myocarditis C Pericarditis D Cardiac rhabdomyoma E Acute rheumatic fever F Chronic rheumatic heart disease G Kawasaki disease

Scenario 1

Incorrect

A 4-year-old girl presented with fever for 5 days, with generalised maculopapular rash and swollen hands and feet. Cardiac apex was located at the 5th intercostal space at mid-clavicular line. Heart sounds were normal without murmur.

0 Your answerG Correct answerKawasaki disease Fever for 5 days with generalised rash and swollen extremities are most compatible with Kawasaki disease. Although infective endocarditis might give rise to rash rarely, it should not cause swollen extremities. The rash related to acute rheumatic fever is classically erythraemia marginatum, not generalised maculopapular rash. Acute rheumatic fever does not cause extremity swelling, but arthritis is a feature.

Scenario 2

Incorrect

A 7-year-old boy presented with fever for 2 days, with nausea, vomiting and lethargy. Cardiac apex was located at the 6th intercostal space at mid-clavicular line. Heart sounds were normal without murmur.

0 Your answerB Correct answerAcute myocarditis Acute myocarditis was the most likely diagnosis in this scenario. Nausea and/or vomiting is almost a constant feature of acute myocarditis. The patient also had evidence of heart failure as suggested by a displaced apex. Infective endocarditis was less likely as there was no cardiac murmur or underlying congenital heart disease. Acute pericarditis was also not likely since a 7-year-old child should be able to complain of chest pain, which is common in case of acute pericarditis. Acute rheumatic fever is extremely rare in this age and there was no other sign suggesting this diagnosis.Scenario 3

Incorrect

A 10-year-old girl presented with fever for 2 days and anterior chest pain and lethargy. Cardiac apex was located at the 6th intercostal space at mid-clavicular line. Heart sounds were muffled without murmur.

0 Your answerC Correct answerPericarditis Among the given choices, acute pericarditis was most likely to give rise to fever with chest pain. The muffled heart sounds were due to pericardial effusion associated with pericarditis. Acute myocarditis usually does not cause chest pain or muffled heart sounds. Kawasaki disease may cause ischemic chest pain in the presence of coronary artery complications such as stenosis or thrombosis of an aneurysm. But this usually does not occur in the acute stage with short course of fever. Chronic rheumatic heart disease and cardiac rhabdomyoma are usually painless.

Scenario 4

Incorrect

A 9-year-old girl presented with fever for 10 days, with malaise and anorexia. Cardiac apex was located at the 5th intercostal space at mid-clavicular line. Heart sounds were normal and there was a systolic murmur at left sternal border.

0 Your answerA Correct answerInfective endocarditis Infective endocarditis is the most likely cause of prolonged fever, malaise, anorexia and a heart murmur due to underlying congenital heart disease. Fever of acute myocarditis or acute pericarditis is usually of shorter duration and heart murmur is not a feature in these conditions. Fever in Kawasaki disease can be quite protracted but again heart murmur is uncommon.

Scenario 5

Incorrect

A 6-month-old boy presented with poor feeding for 4 weeks, with irritability. Cardiac apex was located at the 5th intercostal space at mid-clavicular line. Heart sounds were normal and there was a diastolic murmur at left sternal border.

0 Your answerD Correct answerCardiac rhabdomyoma Cardiac rhabdomyoma was the most likely diagnosis in this case. It is associated with tuberous sclerosis and tends to occur in infancy with spontaneous resolution in about half of the patients as they grow up. Rhabdomyomata are commonly located near atrioventricular valve causing functional mitral or tricuspid stenosis, leading to a diastolic murmur. Chronic rheumatic heart disease has never been reported in such a young infant, though it is associated with heart murmur without fever. Fever is a constant feature of Kawasaki disease. Acute pericarditis or myocarditis does not usually cause heart murmur.

A 4-year-old boy is referred to paediatric outpatients after a GP heard a murmur during a consultation for an upper respiratory tract infection. You review him in the clinic.

He was born at term with no antenatal problems and routine health checks have been normal. He is generally fit and well but suffers from occasional wheezy episodes, especially when he has a cold. He is on no medications and has no known allergies. There is no family history of heart disease.

On examination, he looks well and is on the 50th centiles for both height and weight. He is pink with saturations of 99% in air. His peripheral pulses are all palpable with normal volumes. His chest is clear but on auscultation of his heart you can hear a murmur localised to the pulmonary area. His heart sounds are normal and his apex is palpable in the fifth intercostal space in the midclavicular line. His abdomen is soft and non-tender and his liver is not palpable.

You diagnose an innocent murmur.

What are the features of this type of murmur? Choose four answers only:Systolic

Incorrect

Diastolic

Incorrect

Grade 12/6 in intensity

Incorrect

Varies with posture

Incorrect

Does not vary with posture

Incorrect

Radiates to other positions

Incorrect

Localised to one position Incorrect

Harsh Incorrect

Systolic Grade 1-2/6 in intensityVaries with postureLocalised to one positionInnocent murmurs are very commonly heard in children. The murmur described above could possibly be a pulmonary flow murmur.

Innocent murmurs are always systolic. They are soft with a musical or vibratory quality, are localised and poorly conducting, vary with posture and occur in a child with an otherwise normal cardiovascular examination. They are present in the presence of fever (causing a high output state) but may be heard at any time. The ECG and chest X-ray are normal in these children. No further investigations are usually necessary, although a cardiac echo may be done if available to exclude other causes and to provide reassurance for parents.

A 13-year-old boy is seen in the general paediatric clinic with the complaint of being unable to play in his football matches. He complains of shortness of breath when running as well as chest pain during matches. His mum tells you that he has had these symptoms on and off over the last few years but they have got worse recently and are now interfering with his daily activities. He is otherwise well with no past medical history of note and he is on no current medications. There is no family history of any heart disease and he has two younger siblings who are well.

On examination, he looks well, is pink and well perfused. His chest is clear with no audible wheeze. On cardiovascular examination, his pulses are regular with normal volumes, he has a grade 3/6 ejection systolic murmur heard at the upper left sternal border which seems to radiate into the neck. His abdomen is soft and non-tender with no palpable liver.

What is the most likely cause of his symptoms? Choose one answer only:

Asthma

Aortic stenosis

Correct answer

Hypertrophic cardiomyopathy

Aortic regurgitation

Ventricular septal defect

Aortic stenosis may be an isolated lesion or may be associated with other conditions such as mitral valve abnormalities or with co-arctation of the aorta. It may present with heart failure in the neonatal period (babies may be dependent on blood flow across the ductus arteriosus in order to maintain systemic blood flow and they therefore develop heart failure as the ductus closes). Later in childhood it may present with an asymptomatic murmur or, if severe, with reduced effort tolerance, chest pain and syncope.

Signs on examination are a small volume pulse, a soft aortic component to the second heart sound and an ejection systolic murmur, heard maximally at the aortic area and radiating to the carotids. A carotid thrill may also be present. An ECG may show signs of left ventricular hypertrophy.

If intervention is required, balloon or surgical valvotomy is performed. In neonates or children requiring early treatment, aortic valve replacement is often necessary at a later stage.

Asthma is unlikely in this child with a clear chest on examination and no past history of asthma. The other conditions listed may all present with a murmur but aortic stenosis is the most likely cause in this situation.

Which of the following cardiac lesions are associated with Williams syndrome? Choose TWO answers only.Aortic stenosis Incorrect

Atrial septal defect Incorrect

Ventricular septal defect Incorrect

Hypertrophic obstructive cardiomyopathy (HOCM) Incorrect

Aortic incompetence Incorrect

Supravalvular aortic stenosis Incorrect

Patent ductus arteriosus Incorrect

Pulmonary stenosis (PS) Incorrect

Supravalvular aortic stenosisPulmonary stenosis (PS)Williams syndrome is associated with pulmonary stenosis, peripheral PS and supravalvular aortic stenosis. Williams syndrome is caused by a microdeletion on chromosome 7. Sufferers have characteristic facial features: prominent lips, open mouth, blue eyes (so-called elfin facies). They present with idiopathic hypercalcaemia in the first 2 years of life and failure to thrive. They are also over-friendly (cocktail party personality), with learning difficulties.Which of the following can cause a prolonged QT interval in an electrocardiogram (ECG)? Choose ONE answer only.

Hypercalcaemia

Hyperthyroidism

Hyponatraemia

Fever

Hypocalcaemia Correct answer

Causes of a prolonged QT interval include: hypothyroidism, hypocalcaemia, hypomagnesaemia, hypothermia severe rheumatic carditis drugs: amiodarone, tricyclic antidepressants congenital: Romano-Ward syndrome, Jervell-Lange-Nielson syndrome (also associated with sensory-neural deafness).A 12-year-old girl is admitted from A&E with a fever of 40 C and feeling generally unwell.

She is a known cardiac patient and had a mitral valve replacement for severe mitral regurgitation 2 months previously. Since her operation, however, she has been fine and has been coping well at home over the month. Over the last 2 days she has developed a runny nose and a cough and has been complaining that the wound from her operation is quite sore. She has mild shortness of breath although she thinks this may be improving slightly.

On examination, she is pyrexial and has mild conjunctival pallor. She is not clubbed and there is no peripheral oedema. She has a respiratory rate of 40 breaths per minute and scattered crackles in her chest bilaterally. Her heart sounds are normal and she has a soft, grade 12 out of 6 systolic murmur heard maximally in the mitral area. Her abdomen is soft and non-tender with no masses and a liver palpable 1 cm below the costal margin. Her wound is quite red and tender to palpation. You do some routine blood tests.

The results are as follows:Hb9.7

WCC 20.3

neutophils 18

platelets564

Na 143

K 4.8

urea 3.5

creatinine64

CRP14

What should your choice of treatment at this point be? Choose one answer only:Treat with iv antibiotics for a lower respiratory tract infection

Treat with iv antibiotics for a wound infection

Give oral antibiotics

Start empiric antibiotics for bacterial endocarditis

No treatment Correct answer

Bacterial endocarditis should be considered in any patient with a prostethic heart valve (or with congenital heart disease or previous cardiac surgeries) who presents with a persistent fever. The risk is greatest in lesions with turbulent blood flow or in patients with prosthetic valves. Clinical signs include pallor, fever, changing cardiac murmurs, arthraligia or arthritis, haematuria and the rarely seen peripheral stigmata of splinter haemorrhages, Janeway lesions and Oslers nodes.

Echocardiography is used to look for the presence of vegetations on the cardiac valves.

In these patients it is vitally important to establish the diagnosis before starting on antibiotic treatment as treatment for endocarditis is a prolonged, usually six week, course of intravenous antibiotics and it is important to establish the identification of the infecting organism to ensure appropriate cover. Therefore empiric antibiotics should not be started and positive culture results should be awaited. Incorrect

If this patient had required a dental extraction in the preceding weeks it would have been important to provide adequate prophylaxis against endocarditis in the form of antibiotics before the procedure.

What is the most appropriate antibiotic choice in these situations? Choose one answer only: Penicillin Incorrect

Ciprofloxacin

Incorrect

Flucloxacillin

Incorrect

Amoxicillin

Incorrect

Ceftazidime

Incorrect

Trimethoprim Incorrect

AmoxicillinAntibiotics prophylaxis for infective endocarditis is required for most children with unoperated or postoperative congenital heart lesions. Some children for example, a closed PDA or repaired ASD dont need long-term prophylaxis and therefore local guidelines and policies should be followed. Dental procedures carry the risk of systemic bacteraemia and therefore need antibiotic cover before the procedure. Prophylaxis is also needed for certain ENT, genito-urinary or gastrointestinal procedures.

The usual recommended antibiotic is amoxicillin orally, or if allergic or had recently received penicillin, then clindamycin. High-risk patients receive a further dose after the procedure (local policies and recommendations should be used).

The mother of a 3-year-old child with Tetralogy of Fallot noticed that he was very irritable for the past 5 min and turned deeply blue. On admission, body temperature was 37 oC. Blood pressure was difficult to be measured since the child was very irritable. Heart rate was 180/min. Physical examination showed no significant heart murmur.

Which THREE of the following treatments were most likely to be effective in this case? Oxygen supplement Incorrect

Dopamine infusion Incorrect

Morphine bolus Incorrect

Bicarbonate bolus Incorrect

ATP bolus Incorrect

Intubation and mechanical ventilation Incorrect

Oxygen supplementMorphine bolusBicarbonate bolus The situation described was hypercyanotic spell in-patient with Tetralogy of Fallot. Effective treatments include a kneechest position, oxygen supplement, propranolol, morphine, bicarbonate and systemic vasoconstrictors.Select the most appropriate from the following options:A Ventricular septal defect (VSD) B Innocent murmur C Patent ductus arteriosus (PDA) D Aortic stenosis E Mitral stenosis F Aortic regurgitation G Venous hum

Scenario 1

Incorrect

A well six-year-old girl has a short, soft (grade 1-2/6) systolic murmur which seems to vary with respiration. It is localised to the left sternal edge with no radiation.

0 Your answerB Correct answerInnocent murmurInnocent murmurs are also known as physiological or flow murmurs and are commonly heard in normal children with no underlying heart disease. They are soft, systolic, short in duration with a musical or vibratory quality. They are localised to one area of the praecordium and tend to vary with respiration or posture.

Scenario 2

Incorrect

A 6-month-old baby has a murmur diagnosed by his GP. It is a continuous murmur, best heard under the left clavicle.

0 Your answerC Correct answerPatent ductus arteriosusA patent ductus arteriosus (PDA) is commonly found in preterm infants but can also be present in older children. It is a continuous murmur, best heard in the left infraclavicular region, grade 24 out of 6 in intensity. There may be associated features of bounding pulses and an active praecordium.

Scenario 3

Incorrect

A 6-week-old baby is referred to clinic with a murmur diagnosed by his GP on routine check. On examination he looks well but you can feel a thrill and hear a loud, grade 4/6 systolic murmur, well localised at the lower left sternal edge.

0 Your answerA Correct answerVentricular septal defect (VSD)A VSD is the commonest form of congenital heart disease. It can present with heart failure at around 6 weeks of age or as an asymptomatic murmur at any age. It presents as a systolic murmur, grade 25/6 in intensity, often well localised at the lower left sternal border. A thrill may be present. Management depends on the size of the defect and the amount of flow between the ventricles.

A 6-month-old girl with tetralogy of Fallot is about to undergo repair. Of note in her past medical history she was admitted to the Special Care Baby Unit (SCBU) for observation, as she was jittery. Blood tests at that time revealed hypocalcaemia but she has been well since. On examination she is alert and well perfused but looks cyanosed. Saturations are 86% in air. She has hypertelorism, slightly low set ears and is thriving. Auscultation reveals a 3/6 ejection systolic murmur at the right sternal edge and there are no signs of failure.

Which of the following testsmight be helpful to establish an underlying diagnosis?Choose TWO options. Lymphocyte subsets Incorrect

FISH for 22q microdeletion Incorrect

Order irradiated blood products

Incorrect

Airway assessment Incorrect

Thrombophilia screen Incorrect

Lymphocyte subsetsFISH for 22q microdeletion Tetralogy of Fallot can be associated with Di George syndrome (22q microdeletion syndrome), as can other cardiac defects such as truncus arteriosus and double aortic arch. 22q11 microdeletion results in dysmorphogenesis of the 3rd and 4th pharyngeal pouches early in gestation and this leads to thymic and parathyroid hypoplasia and other abnormalities including congenital heart defects. Neonates can present with hypocalcaemia as a result of underdeveloped parathyroid glands. Dysmorphic features include low set ears, hypertelorism and short philtrum. The immune defect associated with Di George (affecting ~25% of children with 22q11 deletion) is primarily a T-cell defect but the severity is variable and in some mildly affected children can correct with time.

FISH is the genetic test for 22q microdeletion and would be valuable in pre-operative work-up, as would lymphocyte subset (LSS). LSS are useful as they guide whether leucodepleted irradiated blood products are required for surgery to prevent graft versus host disease due to cell-mediated immunodeficiency associated with the syndrome. Irradiated blood products can be ordered when the results are known. There are no specific airway or thrombophilia problems associated with Di George syndrome.

An ex-26 week premature baby is now 5 weeks old on the NICU and weighs 1.6 kg. Her course on NICU has been complicated by an episode of NEC (not requiring surgery) and two episodes of sepsis. She has just started tolerating enteral feeds but remains on some TPN.

Although it has been possible to wean the ventilator settings the baby has failed extubation twice, being unable to remain stable on CPAP. Examination reveals a pink and well-perfused baby with a long line in situ. Heart rate (HR) is 150 bpm and Sats 94% in air. On auscultation of the chest one can hear transmitted sounds and a heart murmur audible in systole and diastole. Echo scan shows a large patent ductus arteriosus and some volume loading of the left atrium.

What treatment options are available for this baby? Choose THREE optionsSurgical ligation Incorrect

Diuretics Incorrect

Indometacin Incorrect

Captopril Incorrect

Sildenafil Incorrect

Surgical ligationDiureticsIndometacin PDA is more common in premature infants and if the PDA is large with a left to right shunt it can prevent babies weaning from the ventilator (as in this case). It can also increase the risk of NEC and chronic lung disease. The shunt is at the arterial level between the pulmonary artery and aorta. Abnormal ductal tissue can result in the duct failing to contract and close, but in the majority of cases the duct will close spontaneously. Echo is diagnostic and will give an idea of how large the shunt is and whether the left side of the heart has become enlarged.

Management can be conservative or surgical. Fluid restriction and diuretics are used as pulmonary blood flow is increased if the duct is large. Indometacin (a prostaglandin synthetase inhibitor) encourages ductal constriction and is usually given as a short course. Ibuprofen is also being used now. Sometimes surgical ligation of the duct through a left thoracotomy is necessary to allow weaning of ventilation.

Captopril is an angiotensin-converting enzyme (ACE) inhibitor used in some children following complex congenital heart disease repair and sildenafil is an oral pulmonary vasodilator. Neither drug is indicated in this case. A 5-year-old girl with Turner syndrome is referred to the cardiology outpatient clinic following the detection of a murmur by her GP.

She is currently well, is growing well and there are no parental concerns at present. She has no shortness of breath on exertion, does not complain of chest pain and there are no other sympt