congenital heart disease from the block (as in j-lo from the block, pun definitely intended!)

Congenital Heart Congenital Heart Disease from the Disease from the

BlockBlock(as in J-Lo from the block, pun definitely intended!)(as in J-Lo from the block, pun definitely intended!)

Premchand Anne, MD, MPHPremchand Anne, MD, MPHPGY IVPGY IV

9/1/20059/1/2005

ObjectivesObjectives

►Fetal and neonatal Fetal and neonatal circulationcirculation

►DADA►PathophysiologyPathophysiology

Left to Right shuntsLeft to Right shunts ObstructiveObstructive Valvular regurgitationValvular regurgitation Cyanotic congenital heart diseaseCyanotic congenital heart disease

Fetal CirculationFetal CirculationFetal Circulation: four Fetal Circulation: four

shuntsshunts PlacentaPlacenta

►Receives 55% of total COReceives 55% of total CO►Lowest vascular resistanceLowest vascular resistance

Ductus venosusDuctus venosus►From placenta: has 70% satsFrom placenta: has 70% sats►Highest POHighest PO22: umbilical vein : umbilical vein

(30)(30) Foramen ovaleForamen ovale

►1/3 of RA return goes to LA1/3 of RA return goes to LA Oxygenate brain and Oxygenate brain and

coronaries better (POcoronaries better (PO22=28)=28) Lower body: POLower body: PO22=24=24

Ductus arteriosusDuctus arteriosus►PA => Descending Aorta => PA => Descending Aorta =>

placentaplacenta

Neonatal CirculationNeonatal Circulation► Change in gas exchange from placenta to Change in gas exchange from placenta to

lungslungs Increase in systemic vascular resistance due to Increase in systemic vascular resistance due to

absence of placenta and closure of DA due to absence of placenta and closure of DA due to presence of increased POpresence of increased PO22

Reduction of PVR after lung expansion due to Reduction of PVR after lung expansion due to oxygen, increased LA return, fall in RA pressure oxygen, increased LA return, fall in RA pressure due to DA closure and increase in systemic due to DA closure and increase in systemic pressure => closure of foramen ovalepressure => closure of foramen ovale

Rapid initial fall, slower fall by 6-8 weeks and Rapid initial fall, slower fall by 6-8 weeks and then after 2 yearsthen after 2 years


►Fetal and neonatal circulationFetal and neonatal circulation

►DADA►PathophysiologyPathophysiology

Left to Right shuntsLeft to Right shunts Obstructive lesionsObstructive lesions Valvular regurgitationValvular regurgitation Cyanotic congenital heart diseaseCyanotic congenital heart disease

DA closureDA closure

►Within 10-15 hours of birthWithin 10-15 hours of birth► Postnatal increase in Oxygen is the strongest Postnatal increase in Oxygen is the strongest

stimulus for closure; premies don’t respond as stimulus for closure; premies don’t respond as well to oxygen.well to oxygen.

► PGEPGE22 decreases after birth due to loss of decreases after birth due to loss of placenta and increased pulmonary blood flow placenta and increased pulmonary blood flow to wash it off.to wash it off.

► Indomethacin closes PDAsIndomethacin closes PDAs►Maternal ingestion of ASA can cause Maternal ingestion of ASA can cause

Persistent Pulmonary Hypertension of the Persistent Pulmonary Hypertension of the Newborn (premature closure of DA and poor Newborn (premature closure of DA and poor development of arterioles)development of arterioles)

► PGEPGE11 keeps DA open. keeps DA open.

Pulmonary arteries and DA Pulmonary arteries and DA respond in opposite mannerrespond in opposite manner


►Fetal and neonatal circulationFetal and neonatal circulation►DADA►PathophysiologyPathophysiology

Left to Right shuntsLeft to Right shunts Obstructive lesionsObstructive lesions Valvular regurgitationValvular regurgitation Cyanotic congenital heart diseaseCyanotic congenital heart disease

Left to Right ShuntsLeft to Right Shunts

►ASDASD►VSDVSD►PDAPDA►ECDECD

Left to Right Shunts-ASDLeft to Right Shunts-ASD

ASDASD

►RA and RV dilatedRA and RV dilated►RV dilation => increased time for RV dilation => increased time for

repolarization => RBBB on EKGrepolarization => RBBB on EKG►NO CHF until 6-8 weeks when PVR NO CHF until 6-8 weeks when PVR

drops considerably.drops considerably.

ASDASD

Left to Right Shunts-VSDLeft to Right Shunts-VSD

VSDVSD

►LA and LV dilated in a small to moderate LA and LV dilated in a small to moderate VSD: does this make sense?VSD: does this make sense? Shunt occurs only during systole, where the Shunt occurs only during systole, where the

blood goes from LV to pulmonary arteryblood goes from LV to pulmonary artery There is increased pulmonary return to LA and There is increased pulmonary return to LA and

then to LV => dilationthen to LV => dilation►RV, LA, LV are all dilated in a large size RV, LA, LV are all dilated in a large size

VSD VSD ►Complication of large VSD=Eisenmenger’s Complication of large VSD=Eisenmenger’s

=> generalized cyanosis=> generalized cyanosis

VSDVSD

Left to Right Shunts-PDALeft to Right Shunts-PDA

PDAPDA

►Onset of CHF is similar to that of a VSDOnset of CHF is similar to that of a VSD►Complication of a large PDA=> Complication of a large PDA=>

Eisenmenger’s => differential Eisenmenger’s => differential cyanosis (lower body cyanosis): WHY?cyanosis (lower body cyanosis): WHY?

PDAPDA

ECDECD

►Endocardial cushion is responsible for Endocardial cushion is responsible for upper part of ventricular septum and upper part of ventricular septum and lower part of atrial septumlower part of atrial septum

►Absence leads to VSD, primum ASD, Absence leads to VSD, primum ASD, clefts in mitral and tricuspid valvesclefts in mitral and tricuspid valves

Left to Right Shunts-ECDLeft to Right Shunts-ECD

ECDECD

►Primum ASD = Secundum ASD; RA Primum ASD = Secundum ASD; RA and RV are dilated with widely split and RV are dilated with widely split and fixed Sand fixed S2 2 and systolic ejection and systolic ejection murmur at left upper sternal border. murmur at left upper sternal border.

►RBBB due to prolonged repolarizationRBBB due to prolonged repolarization►Obligatory shunt with LV -> RA lesionObligatory shunt with LV -> RA lesion

ECDECD



Left to Right shuntsLeft to Right shunts

Obstructive lesionsObstructive lesions Valvular regurgitationValvular regurgitation Cyanotic congenital heart diseaseCyanotic congenital heart disease

Obstructive LesionsObstructive Lesions

►Ventricular Outflow obstructionVentricular Outflow obstruction Aortic stenosisAortic stenosis Pulmonary stenosisPulmonary stenosis Coarctation of the aortaCoarctation of the aorta

►Stenosis of AV valvesStenosis of AV valves Mitral stenosisMitral stenosis Tricuspid stenosisTricuspid stenosis

Ventricular Outflow ObstructionVentricular Outflow ObstructionAS, PS, COAAS, PS, COA

►All three lesions produce the following:All three lesions produce the following: Ejection systolic murmurEjection systolic murmur Hypertrophy of the respective ventricleHypertrophy of the respective ventricle Post-stenotic dilatation is present with the Post-stenotic dilatation is present with the

obstruction at the valvular level; absent in obstruction at the valvular level; absent in subvalvular stenosissubvalvular stenosis

Aortic StenosisAortic Stenosis

►Murmur is loudest in Murmur is loudest in RUSBRUSB

► Loudness is Loudness is proportional to proportional to severityseverity

► LVHLVH

Pulmonary StenosisPulmonary Stenosis

►Murmur is loudest at LUSBMurmur is loudest at LUSB► Loudness is proportional to severityLoudness is proportional to severity► RVHRVH

Coarctation of AortaCoarctation of Aorta

► Ejection type SEM over the descending Ejection type SEM over the descending aorta, distal to COAaorta, distal to COA

►Often see Bicuspid aortic valvesOften see Bicuspid aortic valves►Delayed or absent pulses in LEDelayed or absent pulses in LE► Post stenotic dilation => figure-of-3 sign on Post stenotic dilation => figure-of-3 sign on

xrayxray► Lesion is juxtaductalLesion is juxtaductal► Symptomatic patients have a VSD and may Symptomatic patients have a VSD and may

see RVH and RBBB rather than LVHsee RVH and RBBB rather than LVH

A-V valve obstructionA-V valve obstructionMitral StenosisMitral Stenosis

►More often rheumatic than congenitalMore often rheumatic than congenital►Diastolic murmur due to pressure Diastolic murmur due to pressure

gradient between LA and LV; gradient between LA and LV; subsequently LA, Pulmonary veins, RV subsequently LA, Pulmonary veins, RV dilation; best at apexdilation; best at apex

►Pulmonary edema if hydrostatic > Pulmonary edema if hydrostatic > osmotic pressure; dyspnea with or osmotic pressure; dyspnea with or without exertion.without exertion.

►Loud S1 due to widely parted MV leaflets Loud S1 due to widely parted MV leaflets at onset of systole due to prolongation of at onset of systole due to prolongation of diastolediastole

►Dilated LA leads to A-fibDilated LA leads to A-fib

A-V valve obstructionA-V valve obstructionTricuspid StenosisTricuspid Stenosis

►Rare and usually congenitalRare and usually congenital►Dilation and hypertrophy of RADilation and hypertrophy of RA► If severe, can lead to hepatomegaly If severe, can lead to hepatomegaly

and JVD.and JVD.►(+) mid diastolic murmur(+) mid diastolic murmur



Left to Right shuntsLeft to Right shunts Obstructive lesionsObstructive lesions

Valvular regurgitationValvular regurgitation Cyanotic congenital heart diseaseCyanotic congenital heart disease

Valvular RegurgitationValvular Regurgitation

►Mitral regurgitationMitral regurgitation►Tricuspid regurgitationTricuspid regurgitation►Aortic regurgitationAortic regurgitation►Pulmonary regurgitationPulmonary regurgitation

Mitral RegurgitationMitral Regurgitation

►Volume overload of the LA and LV with Volume overload of the LA and LV with LVH and LAH on EKGLVH and LAH on EKG

►Regurgitant systolic murmur at the apexRegurgitant systolic murmur at the apex►(+) S3 due to rapid early diastole due to (+) S3 due to rapid early diastole due to

fluid overload of LAfluid overload of LA►Pulmonary hypertension occurs Pulmonary hypertension occurs

occasionally due to dampening of occasionally due to dampening of pressure by dilated LApressure by dilated LA

Mitral RegurgitationMitral Regurgitation

Tricuspid RegurgitationTricuspid Regurgitation

►RV and RA enlargeRV and RA enlarge►RAH and RVH with RBBB on EKGRAH and RVH with RBBB on EKG►Systolic regurgitant murmur with S3 in Systolic regurgitant murmur with S3 in

tricuspid area.tricuspid area.►Pulsatile liver and neck veins; reflects Pulsatile liver and neck veins; reflects

right atrial pressure during systole.right atrial pressure during systole.

Aortic RegurgitationAortic Regurgitation

►Overload of LVOverload of LV►LV enlargement on xray and LVH on LV enlargement on xray and LVH on

ECGECG►Wide pulse pressure and bounding Wide pulse pressure and bounding

peripheral pulse due to rapid drop in peripheral pulse due to rapid drop in BP in the aorta due to leakageBP in the aorta due to leakage

►High pitched decrescendo murmur at High pitched decrescendo murmur at the apexthe apex

►AUSTIN FLINT MURMUR due to mitral AUSTIN FLINT MURMUR due to mitral valve flutter during diastole (blood in valve flutter during diastole (blood in opposite directions)opposite directions)

Aortic RegurgitationAortic Regurgitation

Pulmonary RegurgitationPulmonary Regurgitation

►RV enlargement and prominent PA RV enlargement and prominent PA segmentsegment

►The direction of regurgitation is to the The direction of regurgitation is to the RV, aka along Left sternal border and RV, aka along Left sternal border and diastolic.diastolic.



Left to Right shuntsLeft to Right shunts Obstructive lesionsObstructive lesions Valvular regurgitationValvular regurgitation

Cyanotic congenital heart Cyanotic congenital heart diseasedisease

Cyanotic Congenital Heart Cyanotic Congenital Heart LesionsLesions

Cyanotic Congenital Heart Cyanotic Congenital Heart LesionsLesions

►Complete Transposition of the Great Complete Transposition of the Great Arteries (TGA)Arteries (TGA)

►Persistent Truncus Arteriosus and Persistent Truncus Arteriosus and single ventriclesingle ventricle

►Tetralogy of FallotTetralogy of Fallot►Tricuspid atresiaTricuspid atresia►Pulmonary AtresiaPulmonary Atresia►Total Anomalous Pulmonary Venous Total Anomalous Pulmonary Venous

ReturnReturn

Complete Transposition of the Complete Transposition of the Great ArteriesGreat Arteries

►D-TGA is the most common cyanotic lesionD-TGA is the most common cyanotic lesion►Aorta from RV and Pulmonary artery from Aorta from RV and Pulmonary artery from

LVLV►Normally, aorta is under and over the RPANormally, aorta is under and over the RPA► In TGA, aorta is to the right of the RPA In TGA, aorta is to the right of the RPA

because of opening to the RV, hence D-TGAbecause of opening to the RV, hence D-TGA► In L-TGA, aorta is to the left of the PA and In L-TGA, aorta is to the left of the PA and

congenitally corrected TGAcongenitally corrected TGA►A PFO is normally present in D-TGAA PFO is normally present in D-TGA

TGATGA

►Newborn is cyanotic, with metabolic Newborn is cyanotic, with metabolic acidosis, detrimental to myocardiumacidosis, detrimental to myocardium

►Leads to CHF in the first week of life.Leads to CHF in the first week of life.►Associated with hypoglycemiaAssociated with hypoglycemia►Consider TGA if CYANOSIS, CHF WITH Consider TGA if CYANOSIS, CHF WITH

CXR FINDINGS, AND NO MURMURCXR FINDINGS, AND NO MURMUR►Must do BALLOON ATRIAL Must do BALLOON ATRIAL

SEPTOSTOMY (RASHKIND PROCEDURE) SEPTOSTOMY (RASHKIND PROCEDURE) to increase mixing, if only PFO.to increase mixing, if only PFO.

TGATGA

►Small PFO: rashkind procedureSmall PFO: rashkind procedure►Large VSD: RVH with LV and LA Large VSD: RVH with LV and LA

dilationdilation►Corrective proceduresCorrective procedures

Mustard or Senning ProcedureMustard or Senning Procedure Jatene SwitchJatene Switch

Persistent Truncus Arteriosus Persistent Truncus Arteriosus and Single Ventricleand Single Ventricle

► Single arterial blood vessel arises from heart Single arterial blood vessel arises from heart in TA, along with a large VSD; PAs come off in TA, along with a large VSD; PAs come off the TAthe TA

► In single ventricle, both AV valves empty into In single ventricle, both AV valves empty into the single ventricle, with the aorta or PA the single ventricle, with the aorta or PA coming off the rudimentary ventricle.coming off the rudimentary ventricle.

► SimilaritiesSimilarities Complete mixing of systemic pulmonary venous Complete mixing of systemic pulmonary venous

blood in the ventricleblood in the ventricle Pressures in both ventricles are identicalPressures in both ventricles are identical Level of oxygen saturation in systemic circulation Level of oxygen saturation in systemic circulation

is dependent on the magnitude of pulmonary blood is dependent on the magnitude of pulmonary blood flowflow

Tetralogy of FallotTetralogy of Fallot

►4 main findings:4 main findings: VSDVSD Overriding Aorta (not always present)Overriding Aorta (not always present) Pulmonic stenosisPulmonic stenosis RVH (secondary PS)RVH (secondary PS)

►With mild PS, shunt is left to right, With mild PS, shunt is left to right, leading to PINK TOF, LV and RV leading to PINK TOF, LV and RV pressures are samepressures are same

►With severe PS, the shunt is right to left, With severe PS, the shunt is right to left, with PBF from PDAwith PBF from PDA


►Murmur is the superimposition of PS Murmur is the superimposition of PS murmur on top of the VSD murmurmurmur on top of the VSD murmur

► In cyanotic TOF, severe PS produces In cyanotic TOF, severe PS produces Right to left shunt at the ventricular Right to left shunt at the ventricular level and systolic pressures are equal level and systolic pressures are equal in LV, RV and aortain LV, RV and aorta

►Extreme TOF is in pulmonary atresia Extreme TOF is in pulmonary atresia where there is complete R to L shunt, where there is complete R to L shunt, and complete arterial desaturationand complete arterial desaturation

Tetralogy of Fallot-TET spellTetralogy of Fallot-TET spell

► hypoxic spell consists ofhypoxic spell consists of Hyperpnea=increased systemic venous Hyperpnea=increased systemic venous

returnreturn Worsening cyanosis=causes hyperpneaWorsening cyanosis=causes hyperpnea Disappearance of heart murmurDisappearance of heart murmur

► May cause deathMay cause death► Provoked by anything decreasing SVR Provoked by anything decreasing SVR

such as crying, defecation, and such as crying, defecation, and increased physical activity => vicious increased physical activity => vicious cyclecycle

► Treatment: Treatment: MSO4 abolishes hyperpneaMSO4 abolishes hyperpnea Pick up in knee chest position Pick up in knee chest position NaHCO3 to decrease acidosisNaHCO3 to decrease acidosis Add O2Add O2

Tricuspid AtresiaTricuspid Atresia

►Tricuspid valve and a portion of RV do Tricuspid valve and a portion of RV do not existnot exist

►RA return goes through PFO or ASD to RA return goes through PFO or ASD to LA due to increased RA pressure LA due to increased RA pressure =>dilation of RA, and dilation of LA =>dilation of RA, and dilation of LA and LV due to increased volumeand LV due to increased volume

►PA gets blood from LV to remnant RV PA gets blood from LV to remnant RV by VSD with decreased PA by VSD with decreased PA flow=>cyanosisflow=>cyanosis

►CXR: decreased pulmonary vascular CXR: decreased pulmonary vascular markings, dilated RA and LVmarkings, dilated RA and LV

Tricuspid Tricuspid AtresiaAtresia

Pulmonary AtresiaPulmonary Atresia

►PDA is the source of blood to lungsPDA is the source of blood to lungs►Systemic return => RA =>LA => LV Systemic return => RA =>LA => LV

=> aorta =>PDA => lungs =>LA=> aorta =>PDA => lungs =>LA►RV normally hypoplastic; if normal, RV normally hypoplastic; if normal,

expect Tricuspid regurgexpect Tricuspid regurg►Rapid deterioration of clinical status if Rapid deterioration of clinical status if

DA closes; give PGE1 to keep openDA closes; give PGE1 to keep open

Total Anomalous Pulmonary Total Anomalous Pulmonary Venous Return (TAPVR)Venous Return (TAPVR)

►Defect: Pulmonary veins drain into RADefect: Pulmonary veins drain into RA►Andy, you know this to be yet another Andy, you know this to be yet another

prob!prob!►ASD is usually present for RA => LA flowASD is usually present for RA => LA flow►Three kinds:Three kinds:

Supracardiac: drain into SVCSupracardiac: drain into SVC Cardiac: drain into RA; Andy, the last one is: Cardiac: drain into RA; Andy, the last one is: Infracardiac: drain into hepatic vein, portal Infracardiac: drain into hepatic vein, portal

vein, or IVCvein, or IVC

Total Anomalous Pulmonary Total Anomalous Pulmonary Venous Return (TAPVR)Venous Return (TAPVR)

►Normally, consider obstructive vs Normally, consider obstructive vs nonobstructive; infracardiac is nonobstructive; infracardiac is obstructive to venous returnobstructive to venous return

► In nonobstructive: volume overload of In nonobstructive: volume overload of RV due to small ASD. +RBBB. RV due to small ASD. +RBBB.

► In obstructive: pulmonary venous In obstructive: pulmonary venous hypertension and secondary RA and hypertension and secondary RA and RV hypertension.RV hypertension.

Infracardiac confluence

Supracardiac and cardiac confluences

Question-1-2005Question-1-2005You are evaluating a 4-year-old healthy girl at her annual health You are evaluating a 4-year-old healthy girl at her annual health

supervision visit. You note clear breath sounds, strong pulses, supervision visit. You note clear breath sounds, strong pulses, a quiet precordium, and a murmur. Your partner noted a a quiet precordium, and a murmur. Your partner noted a murmur at last year’s visit.murmur at last year’s visit.

Of the following, the finding MOST consistent with the Of the following, the finding MOST consistent with the diagnosis of an innocent murmur isdiagnosis of an innocent murmur is

► continuous machinery murmur under the left claviclecontinuous machinery murmur under the left clavicle

► harsh systolic murmur at the right upper sternal borderharsh systolic murmur at the right upper sternal border

► high-pitched systolic murmur in the back between the high-pitched systolic murmur in the back between the scapulaescapulae

► low-pitched,long, diastolic murmur in the left axillalow-pitched,long, diastolic murmur in the left axilla

► low-pitched,vibratory systolic murmur at the left sternal low-pitched,vibratory systolic murmur at the left sternal borderborder

Question-1Question-1You are evaluating a 4-year-old healthy girl at her annual health You are evaluating a 4-year-old healthy girl at her annual health

supervision visit. You note clear breath sounds, strong pulses, supervision visit. You note clear breath sounds, strong pulses, a quiet precordium, and a murmur. Your partner noted a a quiet precordium, and a murmur. Your partner noted a murmur at last year’s visit.murmur at last year’s visit.

Of the following, the finding MOST consistent with the Of the following, the finding MOST consistent with the diagnosis of an innocent murmur isdiagnosis of an innocent murmur is

► continuous machinery murmur under the left claviclecontinuous machinery murmur under the left clavicle

► harsh systolic murmur at the right upper sternal borderharsh systolic murmur at the right upper sternal border

► high-pitched systolic murmur in the back between the high-pitched systolic murmur in the back between the scapulaescapulae

► low-pitched,long, diastolic murmur in the left axillalow-pitched,long, diastolic murmur in the left axilla

► low-pitched,vibratory systolic murmur at the left sternal low-pitched,vibratory systolic murmur at the left sternal borderborder

Question-2Question-2► You are evaluating a 16-year-old boy for preparticipation You are evaluating a 16-year-old boy for preparticipation

sports screening. The boy states that his older brother was sports screening. The boy states that his older brother was diagnosed with a seizure disorder and died suddenly during diagnosed with a seizure disorder and died suddenly during high school track practice. He also has a younger sister who high school track practice. He also has a younger sister who has a history of syncope.has a history of syncope.

Before approving him for sports participation, which of the Before approving him for sports participation, which of the following tests must be performed? following tests must be performed?

► computed tomography of the headcomputed tomography of the head

► electrocardiographyelectrocardiography

► electroencephalographyelectroencephalography

► genetic testing for ion channel abnormalitiesgenetic testing for ion channel abnormalities

► tilt table testtilt table test

Question-3Question-3► A 750-g infant who was born at 27 weeks’ gestation was weaned A 750-g infant who was born at 27 weeks’ gestation was weaned

successfully from the ventilator on postnatal day 3. Two days later, successfully from the ventilator on postnatal day 3. Two days later, the infant has bounding pulses, tachypnea, and a new murmur. the infant has bounding pulses, tachypnea, and a new murmur. Echocardiography confirms the diagnosis of patent ductus arteriosus. Echocardiography confirms the diagnosis of patent ductus arteriosus. Hemoglobin is 13 g/dL (130 g/L). Electrolytes, creatinine, and Hemoglobin is 13 g/dL (130 g/L). Electrolytes, creatinine, and platelets are within normal imits.platelets are within normal imits.

Of the following, the MOST appropriate initial management strategy Of the following, the MOST appropriate initial management strategy for this infant is for this infant is

► intravenous indomethacinintravenous indomethacin

► oxygen therapy at an Fio2 of 1.0oxygen therapy at an Fio2 of 1.0

► surgical ligation of the ductus arteriosussurgical ligation of the ductus arteriosus

► transcatheter closure of the ductus arteriosustranscatheter closure of the ductus arteriosus

► transfusion with packed red blood cellstransfusion with packed red blood cells

Question-4Question-4► During the physical examination of an otherwise healthy 2-During the physical examination of an otherwise healthy 2-

month-old infant, you note a harsh grade 3/6 holosystolic month-old infant, you note a harsh grade 3/6 holosystolic murmur that is low-pitched and heard best over the lower left murmur that is low-pitched and heard best over the lower left sternal border.sternal border.

Of the following, the diagnosis MOST consistent with these Of the following, the diagnosis MOST consistent with these auscultatory findings is auscultatory findings is

► aortic stenosisaortic stenosis

► atrial septal defectatrial septal defect

► patent ductus arteriosuspatent ductus arteriosus

► tetralogy of Fallottetralogy of Fallot

► ventricular septal defectventricular septal defect

Question-5Question-5► Your assistance is sought by a resident who is preparing a Your assistance is sought by a resident who is preparing a

presentation for her colleagues on the differential diagnosis of presentation for her colleagues on the differential diagnosis of stroke in pediatrics. You point out that certain patients who have stroke in pediatrics. You point out that certain patients who have cardiovascular pathology may be at increased risk for cardiovascular pathology may be at increased risk for cerebrovascular accident.cerebrovascular accident.

Which of the following cardiac conditions is MOST likely to be Which of the following cardiac conditions is MOST likely to be associated with a cerebrovascular accident? associated with a cerebrovascular accident?

► congestive heart failurecongestive heart failure

► constrictive pericardial diseaseconstrictive pericardial disease

► cyanotic congenital heart diseasecyanotic congenital heart disease

► Kawasaki diseaseKawasaki disease

► rheumatic feverrheumatic fever

Question-6Question-6► You are evaluating a 7-day-old infant because of poor feeding, tachypnea, You are evaluating a 7-day-old infant because of poor feeding, tachypnea,

and lethargy. According to his mother, the symptoms began 24 hours and lethargy. According to his mother, the symptoms began 24 hours earlier and have progressed throughout the day. Findings on physical earlier and have progressed throughout the day. Findings on physical examination include a respiratory rate of 80 breaths/min, heart rate of 180 examination include a respiratory rate of 80 breaths/min, heart rate of 180 beats/min, and blood pressure of 65/40 mm Hg. The infant is cool, beats/min, and blood pressure of 65/40 mm Hg. The infant is cool, mottled, and pale. There are no murmurs, but there is a gallop. You mottled, and pale. There are no murmurs, but there is a gallop. You palpate a pulse in the right brachial region but cannot palpate a femoral palpate a pulse in the right brachial region but cannot palpate a femoral pulse. You discuss your diagnosis with the parents, who want to know the pulse. You discuss your diagnosis with the parents, who want to know the immediate plan and possible long-term complications.immediate plan and possible long-term complications.Of the following, the MOST appropriate answer to the parents inquiry is Of the following, the MOST appropriate answer to the parents inquiry is

► immediate treatment for aortic coarctation; risk for chronic hypertensionimmediate treatment for aortic coarctation; risk for chronic hypertension► immediate treatment for aortic stenosis; risk for aortic valve replacementimmediate treatment for aortic stenosis; risk for aortic valve replacement► immediate treatment for cardiomyopathy; risk for cardiac transplantationimmediate treatment for cardiomyopathy; risk for cardiac transplantation► immediate treatment for double aortic arch; risk for tracheomalacia immediate treatment for double aortic arch; risk for tracheomalacia ► immediate treatment for hypoplastic left heart syndrome; risk for right immediate treatment for hypoplastic left heart syndrome; risk for right

ventricular dysfunctionventricular dysfunction

Question-7Question-7► You are evaluating a 3-day-old infant brought to the emergency You are evaluating a 3-day-old infant brought to the emergency

department for lethargy. The pregnancy, labor, and delivery were department for lethargy. The pregnancy, labor, and delivery were uncomplicated, and the baby was discharged from the hospital uncomplicated, and the baby was discharged from the hospital yesterday. On physical examination, the heart rate is 180 beats/min, yesterday. On physical examination, the heart rate is 180 beats/min, the respiratory rate is 80 breaths/min, and the blood pressure is the respiratory rate is 80 breaths/min, and the blood pressure is 50/30 mm Hg. The infant is pale and mottled and has cool 50/30 mm Hg. The infant is pale and mottled and has cool extremities and weak distal pulses.extremities and weak distal pulses.

Of the following, the MOST likely cardiac diagnosis is Of the following, the MOST likely cardiac diagnosis is

► atrioventricular septal defectatrioventricular septal defect

► critical aortic stenosiscritical aortic stenosis

► large ventricular septal defectlarge ventricular septal defect

► tetralogy of Fallottetralogy of Fallot

► transposition of the great arteriestransposition of the great arteries

Question-8Question-8► A 10-year-old girl had recent pharyngitis with culture-proven group A A 10-year-old girl had recent pharyngitis with culture-proven group A

StreptococcusStreptococcus. She was noncompliant with antibiotic therapy. She now . She was noncompliant with antibiotic therapy. She now presents with fever to 102°F (38.9°C), a heart rate of 120 beats/min, presents with fever to 102°F (38.9°C), a heart rate of 120 beats/min, and a respiratory rate of 24 breaths/min. She has no murmurs or and a respiratory rate of 24 breaths/min. She has no murmurs or gallop rhythm. She has a nonpruritic, macular rash that appears as a gallop rhythm. She has a nonpruritic, macular rash that appears as a serpiginous, erythematous circle surrounding normal skin. She also serpiginous, erythematous circle surrounding normal skin. She also has an erythematous, warm, swollen left knee and right ankle.has an erythematous, warm, swollen left knee and right ankle.

Of the following, the MOST appropriate diagnostic study for this girl isOf the following, the MOST appropriate diagnostic study for this girl is

► chest radiographychest radiography

► echocardiographyechocardiography

► left knee and right ankle radiographyleft knee and right ankle radiography

► rheumatoid factorrheumatoid factor

► skin biopsy of the rashskin biopsy of the rash

Question-9Question-9► You are evaluating a 4-month-old girl in your office whom you You are evaluating a 4-month-old girl in your office whom you

know to have tetralogy of Fallot. Her mother informs you that the know to have tetralogy of Fallot. Her mother informs you that the infant has had fever, diarrhea, and poor feeding in the last 24 infant has had fever, diarrhea, and poor feeding in the last 24 hours. On physical examination, you note cyanosis of the hours. On physical examination, you note cyanosis of the extremities and perioral area, tachypnea, hyperpnea, and a heart extremities and perioral area, tachypnea, hyperpnea, and a heart rate of 180 beats/min. You do not hear a murmur.rate of 180 beats/min. You do not hear a murmur.

Of the following, the MOST appropriate management strategy is to Of the following, the MOST appropriate management strategy is to ► ► administer antipyretics for feveradminister antipyretics for fever

► encourage oral intake of fluidsencourage oral intake of fluids

► order echocardiographyorder echocardiography

► place her in the knee-chest position with oxygenplace her in the knee-chest position with oxygen

► reassure her mother because the murmur is gonereassure her mother because the murmur is gone

Question-10-2004Question-10-2004► A 14-year-old boy complains of fatigue, weight loss, and night A 14-year-old boy complains of fatigue, weight loss, and night

sweats over 2 months. His parents noted the recent onset of sweats over 2 months. His parents noted the recent onset of generalized swelling of the face and neck that has a dusky generalized swelling of the face and neck that has a dusky color.color.

Of the following, the MOST useful diagnostic test for this boy Of the following, the MOST useful diagnostic test for this boy is is


► computed tomography of the sinusescomputed tomography of the sinuses

► cranial computed tomographycranial computed tomography

► serum antinuclear antibody testserum antinuclear antibody test

► tuberculin skin testtuberculin skin test

Question-11Question-11► A 16-year-old girl who has systemic lupus erythematosus has A 16-year-old girl who has systemic lupus erythematosus has

been vomiting for 1 day after having vague abdominal been vomiting for 1 day after having vague abdominal discomfort associated with anorexia for 3 days. Physical discomfort associated with anorexia for 3 days. Physical examination reveals tachycardia, with a heart rate of 130 examination reveals tachycardia, with a heart rate of 130 beats/min. All peripheral pulses diminish in strength when she beats/min. All peripheral pulses diminish in strength when she inhales.inhales.

Of the following, the MOST important study to obtain initially is Of the following, the MOST important study to obtain initially is

► abdominal computed tomographyabdominal computed tomography


► electrocardiographyelectrocardiography

► erythrocyte sedimentation rateerythrocyte sedimentation rate

► upper gastrointestinal radiographic seriesupper gastrointestinal radiographic series

Question-12Question-12► An 11-year-old girl has a 1-week history of dyspnea, malaise, and An 11-year-old girl has a 1-week history of dyspnea, malaise, and

fatigue. She developed vomiting after 24 hours of feeling abdominal fatigue. She developed vomiting after 24 hours of feeling abdominal fullness and discomfort. Physical examination reveals a blood fullness and discomfort. Physical examination reveals a blood pressure of 85/50 mm Hg, tachypnea, rales, hepatomegaly, and no pressure of 85/50 mm Hg, tachypnea, rales, hepatomegaly, and no cardiac murmur. The heart rate by auscultation is 120 beats/min. cardiac murmur. The heart rate by auscultation is 120 beats/min. Palpation of the pulses reveals a regular rate of 60 beats/min.Palpation of the pulses reveals a regular rate of 60 beats/min.

Of the following, the MOST likely diagnosis is acute Of the following, the MOST likely diagnosis is acute

► hepatitishepatitis

► lobar pneumonialobar pneumonia

► myocarditismyocarditis

► pancreatitispancreatitis

► pericarditispericarditis

Question-13Question-13► Two weeks after having a perimembranous ventricular septal defect Two weeks after having a perimembranous ventricular septal defect

repaired, a 4-year-old child presents with anorexia and occasional repaired, a 4-year-old child presents with anorexia and occasional vomiting. On physical examination, there is pallor of the lips, and the vomiting. On physical examination, there is pallor of the lips, and the pulses are difficult to feel. The auscultated heart rate is 140 pulses are difficult to feel. The auscultated heart rate is 140 beats/min, but the heart rate by radial pulse is only 70 to 80 beats/min, but the heart rate by radial pulse is only 70 to 80 beats/min. There is no palpable radial pulse during the inspiratory beats/min. There is no palpable radial pulse during the inspiratory phase of respiration.phase of respiration.

Of the following, the intervention that is MOST likely to be effective is Of the following, the intervention that is MOST likely to be effective is ► direct current cardioversiondirect current cardioversion

► intravenous methylprednisoloneintravenous methylprednisolone

► pericardiocentesispericardiocentesis

► thoracentesisthoracentesis

► transvenous cardiac pacingtransvenous cardiac pacing

Question-14Question-14► An asymptomatic 4-year-old girl has long, spidery fingers and An asymptomatic 4-year-old girl has long, spidery fingers and

a pectus carinatum deformity. Her height is at the 50th a pectus carinatum deformity. Her height is at the 50th percentile and weight is at the 5th percentile. Cardiac percentile and weight is at the 5th percentile. Cardiac auscultation reveals a systolic click that occurs later in systole auscultation reveals a systolic click that occurs later in systole with squatting and earlier with standing.with squatting and earlier with standing.

Of the following, the MOST likely finding on echocardiography Of the following, the MOST likely finding on echocardiography would bewould be bicuspid aortic valvebicuspid aortic valve Ebstein anomaly of the tricuspid valveEbstein anomaly of the tricuspid valve idiopathic hypertrophic subaortic stenosisidiopathic hypertrophic subaortic stenosis mitral valve prolapsemitral valve prolapse sinus of Valsalva aneurysmsinus of Valsalva aneurysm

Question-15Question-15► A newborn who has cyanosis has pulse oximetry values of A newborn who has cyanosis has pulse oximetry values of

95% in head hood oxygen at 100% Fio2. Arterial blood gas 95% in head hood oxygen at 100% Fio2. Arterial blood gas from the right radial artery shows: pH, 7.34; Po2, 65 torr; and from the right radial artery shows: pH, 7.34; Po2, 65 torr; and Pco2, 38 torr.Pco2, 38 torr.

Of the following, the MOST likely diagnosis is Of the following, the MOST likely diagnosis is

► coarctation of the aorta with bicuspid aortic valvecoarctation of the aorta with bicuspid aortic valve

► double-inlet single ventricle with mild pulmonic valve stenosisdouble-inlet single ventricle with mild pulmonic valve stenosis

► pulmonary atresia with restrictive ductus arteriosuspulmonary atresia with restrictive ductus arteriosus

► transposition of the great vessels with restrictive foramen transposition of the great vessels with restrictive foramen ovaleovale

► ventricular septal defect with mild pulmonic valve stenosisventricular septal defect with mild pulmonic valve stenosis

Question-16Question-16

► Which of the following laboratory findings is either a Which of the following laboratory findings is either a major or minor Jones criterion for the diagnosis of major or minor Jones criterion for the diagnosis of acute rheumatic fever? acute rheumatic fever?

► decreased PR interval by electrocardiographydecreased PR interval by electrocardiography

► decreased serum complementdecreased serum complement

► decreased serum haptoglobindecreased serum haptoglobin

► elevated antistreptolysin O titerelevated antistreptolysin O titer

► elevated C-reactive proteinelevated C-reactive protein

Jones CriteriaJones Criteria

►Major JONESMajor JONES JointsJoints Obvious as in CarditisObvious as in Carditis NodulesNodules Erythema marginatumErythema marginatum Sydenham’s ChoreaSydenham’s Chorea

►Minor criteriaMinor criteria Elevated acute phase reactantsElevated acute phase reactants Increased PR intervalIncreased PR interval ArthralgiasArthralgias feverfever

ReferencesReferences

congenital heart disease from the block (as in j-lo from the block, pun definitely intended!)

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