8Changes after birth:Shift of blood flow for gas exchange, from the placenta to the lungs.Closure of ductus venosus :no placentaincrease in pulmonary blood flowfunctional closure of foramen ovale (increased LA pressure)Closure of ductus arteriosus (^O2)
9Newborn Cardiac Exam Vital signs: RR, HR, BP respiratory effort color palpate point of maximal impulse (PMI) of heartpalpate pulses of all extremitiesauscultate
10Case 1: 2 day old infant2 day old baby girl is being examined for discharge physical.Vitals: HR:120 RR:40 BP: r arm 75/40Gen : low hairline, webbed neck, edemetous dorsum of feet
11Case 1: cardiac exam Normal PMI, no thrill No hepatosplenomegally unable to palpate pulses in lower extremitiesBP’s all four extremities: both legs systolic BP lower 40/30
12Case 1: Auscultation S2 splits normally ejection click systolic ejection murmur III/VI at the URSB and LLSBsystolic murmur radiates to the backearly diastolic decrescendo murmur at 3LICS
13Case 1:What do you think is the congenital defect?
16Coarctation of Aorta (COA) Incidence: 8% of all congenital heart defectsTurner’s syndrome: 30% have COAPreductal: 40% associated with other cardiac defects,symptomatic early in lifePostductal: less likely to have symptoms early
17COA EKG: LVH (but may be normal in 20%) X-rays: heart size may be normal or slightly enlargedRib notching in older childrenEcho: can see narrowingBicuspid aortic valveDoppler:disturbed flow
18COA complicationsCOA can cause CHF, HTN (intracranial bleeding,hypertensive encephalopathy)Bicuspid aortic valve: stenosis or regurgLV failureinfective endocarditis
19Preductal COA in Newborn 80% of infants with preductal COA develop CHF by 3 months of age!Symptoms of CHF: poor feeding, poor weight gain, dyspnea in first 2-6 weeks of life.
20Case 2: Two week old with murmur Two week old infant, who you saw as a newborn with normal exam, now is noted to have a heart murmur.PMI LSB, not hyperdynamicpulses equal all extremitiesNo HSM
21Case 2: Cardiac examGrade III/VI holosystolic murmur at LLSB
22Case 2: What would you like to do next? EKG: LVH,LAHBlood pressures all four extremitiesXray: look for cardiomegally and increase in pulmonary vascularityoxygenationEcho: VSD
23Ventricular Septal Defect (VSD) The most common form of congenital heart disease: 20-25%may be located in different anatomical locationsmay be associated with many other cardiac defects ( in many cases essential for survival)may be small or large (can cause CHF)
24Case 3: One day old infant One day old infant with tachypnea and cyanosisGen: cyanoticRR:65, HR 140, BP 40/20pulse ox on RA: less than 80
25Case 3: Respiratory and Cardiac Lungs clear, no retractions, RR rapidCardiac: PMI at LSBpulses palpable all extremitiesS2 single and loud. No heart murmur.
26What would you like to do? ABG: before oxygenationGive 100% O2 and then repeat ABGEKG, 4 extremity BPCXR
27Results of ABG Before O2: PH: 7.15,PaCO2 30 and Pa O2 40 After 100% O2: PH: 7.12, PaCO2 25 and PaO2 50
29Transposition of Great Arteries 5% of all congenital heart defectsAorta arises anteriorly from RV, PA arises posteriorly from LVdefects (VSD,ASD,PDA) that permit mixing of the two circulations are necessary for survival
30TGA: ABG:hypoxemia is unresponsive to O2 EKG: RVH X-rays: egg-on-a-string silhouettecardiomegally with increased pulmonary vascularityEcho: PA from LVassociated anomalies:VSD,ASD,PDA
31Immediate Treatment Prostaglandin E1 to reopen PDA Oxygen cardiology/surgery referral
32DDX of Cyanotic Heart Dz Transposition of the Great ArteriesTetralogy of FallotTotal Anomalous Pulmonary Venous ReturnTricuspid AtresiaPulmonary AtresiaTruncus Arteriosus
33Other congenital cyanotic defects Ebstein’s anomalysingle ventricleDouble-outlet right ventricle (depends on associated defects…if cyanotic or not)
40TOF complications Hypoxic spells growth retardation with severe cyanosisbrain abscess and CVAinfective endocarditispolycythemia
41Hypoxic Spell ( “TET Spell” Paroxysm of hyperpnea (rapid and deep)irritability and prolonged cryincreasing cyanosisdecreased intensity of heart murmur(may lead to limpness, convulsion,CVA or death)
42Treatment of “TET Spell” Knee-chest (squat )positionmorphine sulfatetreat acidosisoxygenation
43Total Anomalous Pulmonary Venous Return One percent of all congenital heart defectsDefect: no direct communication between the pulmonary veins and the left atrium (they can drain: supracardiac,cardiac,infracardiac or mixed)
52Pulmonary Atresia: findings PE: S2 is singlemurmur of PDAEKG: normal axis,LVHXray: decreased pulmonary vascularityEcho:atretic pulmonary valve and hypoplastic RV
53Pulmonary AtresiaProstaglandin E1cardiac surgery
54Truncus Arteriosus Less than 1% of all congenital heart Dz Only a single arterial trunk leaves the heart and gives rise to the pulmonary, systemic and coronary circulationslarge VSD is always present
55Truncus Arteriosus PE: cyanosis wide pulse pressure and bounding pulsesharsh VSD murmur LSBEKG:normal axis,LAHXrays: cardiomegally and increased pulm vascularity50% R aortic archEcho:single great artery,VSD
57Hypoplastic Left Heart Hypoplastic left heart syndrome refers to underdevelopment of the left side of the heart.This syndrome may include:Small aorta: This is the major blood vessel from the left ventricle to the body.
58Hypoplastic Left Heart May Include:Aortic valve atresia (absence): This valve normally opens and closes to let blood flow from the left ventricle to the aorta. When atresia is present, there is no connection between the left ventricle and aorta, and no forward blood flow.Mitral valve stenosis or atresia: This valve normally opens and closes to let blood flow between the left atrium and left ventricle. Stenosis causes little blood flow; atresia causes no blood flow. Either atresia or stenosis may be present.