management of congenital heart disease in infants
DESCRIPTION
PRESENTATION FOR NON CARDIOLOGISTSTRANSCRIPT
MANAGEMENT OF CONGENITAL HEART DISEASE IN INFANTS
Dr Syed RazaConsultant Cardiologist
Objectives
• Prevalence• Common congenital heart defects• Diagnostic Workup• Management
Size Of The Problem !
• Commonest birth defect• 33% of all birth defects• In US alone 35,000 babies are born with heart
defects every year.• Prevalence of 8 per 1000 births. • 1 million grown up adults with heart defects
Size of The Problem!
• Incidence is higher in pre-term infants. • Can be as high as 10-15% in infants who
have a parent with CHD• 5-10% association with abnormal
karyotyping.• Females - more prone to have PDA and ASD• Males - more prone to have valvular aortic
stenosis, coarctation of the aorta, TOF and Transposition of the Great Vessels
• Maternal rubella is associated with PDA
Other Associated Birth Defects (TRAVEL)
• T – Tracheoesophageal fistula • R – Renal anomalies • A – Anal atresia • V – Vertebral anomalies• E – Esophageal atresia• L – Limb defects
How to deal with the problem?
• Family physicians must know how to screen for cardiac defects in infants.
• Must be competent to stabilize the infant and work as a team with Paediatric Cardiologist and Cardiac surgeon.
• Be able to provide psychosocial support to the family.
CLASSIFICATION
• – Acyanotic - Left-to-right shunt - oxygenated to un
oxygenated blood
– Cyanotic - Right-to-left shunt - deoxygenated blood to oxygenated blood
– Hemodynamic and Blood Flow Patterns • Increased pulmonary flow• Decreased pulmonary flow• Obstruction to blood flow (out of the heart)
Common Types of CHD
ACYANOTICVentricular septal defect (Commonest –
Membranous type)Atrial septal defect Atrioventricular canal defect Pulmonary stenosis,Patent ductus arteriosus Aortic stenosis andCoarctation of the aorta
Types of CHD – contd:
CYANOTIC
Tetralogy of Fallot Transposition of the great arteries.
Heart Anatomy
Patent Ductus Arteriosus
Atrial Septal Defect
Ventricular Septal Defect
Hypoplastic Left Heart Syndrome
Coarctation of the Aorta
Tetralogy of Fallot
Tetrology of Fallot
Transposition of the Great Arteries
Problems Associated :
• Acyanotic CHD Congestive Cardiac Failure
• Cyanotic CHD Hypoxia
History Taking
• Must be careful. Some of the symptoms are very subtle.
• Obtain a detailed prenatal history including exposure to infections (cytomegalovirus, toxoplamosis, rubella or varicella), medication usage, drug and alcohol use, nutrition and exposure to radiation.
Associated Risk Factors
• Diabetes • Family history of CHD • Alcohol• Exposure to drugs such as Phenytoin,
Warfarin, Lithium, Thalidomide etc.• First-trimester rubella exposure (PDA)• Residence at high altitudes (PDA)• Karyotype abnormalities
How to suspect CHD?
• Feeding problems• Fatigue and excessive sweating• Severe growth impairment• Recurrent chest infection
CLINICAL SIGNS
• Signs of poor growth• Tachypnea• Sub costal recession• Tachycardia• Cyanosis• Peri orbital oedema• Murmur
MURMUR
• The presence or absence of a heart murmur is unreliable
• Murmur at birth that disappears before baby’s discharge from hospital – likely PDA
• Murmur is not present in many severe forms of CHD, such as tricuspid atresia, Coarctation of the aorta and Transposition of the great vessels.
• The intensity of the murmur is unrelated to the severity of the abnormality.
Pulmonary vs Cardiac Cyanosis
Suspicion of cyanosis should be confirmed by pulse oximetry.
Pulmonary disorders - administration of 100 percent oxygen usually increases the arterial oxygen saturation to at least 95 percent. Cyanotic CHD - oxygen saturation will only increase to 80 to 85 percent.
Heart Failure Signs
Presence of third heart sound
Lung crepitations
Oedema – late sign (Peri-orbital before leg)
Hepatomegaly
Ascitis
INVESTIGATIONS
• ECG- a normal ECG does not exclude the presence of CHD if index of suspicion is high.
• Chest radiographs - cardiomegaly and increased pulmonary vascular markings.
A serious underlying heart defect is unlikely if ECG and CXR are normal.
Investigations : contd
• Echocardiogram - primary diagnostic test.• Phonocardiogram - heart sounds are recorded and
displayed as a diagram• MRI - used to evaluate heart structure, size or blood
flow
• Lab tests - Hb and Hct (polycythemia), • ESR (rheumatic fever, Kawasaki disease,
myocarditis), • ABG (presence of a right to left shunt), • Clotting times (PT, PTT) and platelet count, • Na, K, digoxin level
DIAGNOSIS
• Studies suggest that 30 to 60 percent of congenital heart defects can be detected prenatally by high resolution four-chamber transvaginal echocardiogram.
• Foetal echocardiography with outflow-tract views can be particularly helpful in detecting anomalies of the great arteries.
• SCAN USUALLY PERFORMED AT 18-22 WEEKS
Cardiac Catheterization
• Can be either diagnostic or interventional– Pressures in the heart can be measured– CO can be evaluated– Blood samples can be obtained and tested (O2
sat)– Contrast can be injected to study blood flow,
vessels and chambers– Balloon angioplasty can be performed to
stretch stenosed areas or blockages in vessels– Can study electrical activity of the heart
MRI
• Provides structural details when echocardiogram images are not optimum.
• Magnetic resonance imaging provides excellent anatomic evaluation particularly if repair is contemplated.
• Yields even more information than angiography/catheterization.
TREATMENT AIMS
• 1) improving cardiac function
• 2) removing accumulated fluid and sodium
• 3)decrease cardiac demands
• 4) improve tissue O2
• 5) Improve nutrition and hydration
• 6)Prevention of infection
• 7) Patient and family support
TREATMENT OPTIONS
• MEDICATION• CLOSURE DEVICE THERAPY• SURGERY• IMPROVING GENERAL WELL BEING
Heart Failure Management
• Diuretics - Lasix, spironalactone, thiazides (may need K replacement)
• Improve contractility – Digoxin. • Decrease afterload - hydralazine
(vasodilator), nifedipine (Ca channel blocker), nipride (vasodilator), or captopril (ACE inhibitor)
Heart Failure Management: Cont’d
Improve oxygenation - administer O2 as needed by hood, mask or nasal prongs.
Adequate nutrition is also important may need six to eight small meals daily rather than 3 large meals, may need tube feedings
Important Points about Care of the Child with a Heart Defect
• Parents taking a child with a heart defect home should have discharge planning which includes name/number of the specialist and all emergency numbers.
• Instruction in what to do if child becomes cyanotic, CPR training.
• Should not allow a child with a cyanotic heart defect or severe aortic stenosis to cry for extended periods of time.
CLOSURE DEVICE : AMPLATZ
CLOSURE DEVICE
BALOON VALVULOPLASTY
BALOON VALVULOPLASTY
SURGERY
Nutrition Requirements
• Adequate nutrition is extremely important in infants with CHD
• Infants who are unable to gain sufficient weight with breast feeding, supplementation may be provided:
Options include a formula with a high caloric density, nocturnal enteral feeding or continuous 24-hour feeding with a nasogastric or duodenal tube
Nutrition Requirements: Contd.
• Don’t usually have to restrict sodium intake (regulates water balance)
• Need vitamin supplements and maybe an iron supplement
Prophylaxis Against Bacterial Endocarditis: ACC/AHA Guidelines
• Patients who have complex cyanotic congenital heart disease (e.g., single-ventricle states, transposition of the great arteries, tetralogy of Fallot). (Level of Evidence: C)• Patients with surgically constructed systemic pulmonary shunts or conduits. (Level of Evidence: C)• Patients with congenital cardiac valve malformations, particularly those with bicuspid aortic valves.
Infective Endocarditis Prophylaxis
• Completely repaired congenital heart defect with prosthetic material or device, whether placed by surgery or by catheter intervention, during the first 6 months after the procedure. (Level of Evidence: B)
Prophylaxis Not Recommended
• Patients with isolated secundum atrial septal
defect. (Level of Evidence: C)• Patients 6 or more months after successful surgical or percutaneous repair of atrial septal defect, ventricular septal defect, or patent ductus arteriosus. (Level of Evidence: C)
VACCINATION
Pneumococcal vaccine is recommended at two years of age
Influenza vaccine should be given yearly beginning at age six months.
MMR at 12 months instead of 15
Psychological Issues
• The diagnosis of CHD is cause for much distress amongst family members
• Parents experience shock, denial, guilt, anger, despair or confusion on learning that their infant has a cardiac defect.
• Greater stress if requires surgical intervention.
Effect of High Demand of Child’s Medical Treatment and Care
• Parents and care givers forced to give up jobs and careers
• Easy fatigability• Distress and irritability• No leisure time• Loss of resources• Depression
Handling Psychological Stress
• Reinforcement of the positive, normal attributes of the child helps the family see the child as an individual with many of the same needs as healthy children
• Consultation with a mental health professional may help to cope with the challenge.
General Physician’s Role
Important link between family and specialist Physician should provide correct information
and clear any doubts and misconceptions.Provide reassurance and maintain positive
attitude.Educate the family .Provide moral supportArrange for support group and psychological
help.
THANK YOU
ANY QUESTIONS ?