Acyanotic Heart Disease

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Acyanotic Heart Disease

  1. 1. Acyanotic Heart Disease Board Review
  2. 2. Content Specs <ul><li>Major clinical findings in: VSD, ASD, PDA and AS </li></ul><ul><li>Importance of PDA in the presentation of hypoplastic left heart syndrome and in coarctation of the aorta </li></ul><ul><li>Initial management of a premature infant with PDA </li></ul><ul><li>Immediate (eg, referral) and long-term (eg, frequent BP measurements) management in a patient with CoA </li></ul><ul><li>Expected natural history of ventricular septal defect and Bicuspid AV </li></ul><ul><li>Understand the management of severe PS </li></ul><ul><li>Understand the risks for pulmonary vascular obstructive disease </li></ul><ul><li>(Eisenmenger) in patients with untreated large left-to-right shunt </li></ul><ul><li>lesions with pulmonary hypertension (eg, large VSD, AV septal defect, large PDA). </li></ul>
  3. 3. Acyanotic Heart Lesions <ul><li>Left to Right Shunting---extra volume in right side of heart--increased pulmonary blood flow---increased cardiac work load. </li></ul><ul><li>Obstructive Lesions---stenosis of valve or vessel---pressure rises behind obstruction and distal blood flow reduces/absent. </li></ul><ul><li>Decreased systemic perfusion and shock can be caused by obstructive lesions </li></ul><ul><li>Rarely present at birth </li></ul><ul><li>Mostly leads to CHF </li></ul>
  4. 4. ASD <ul><li>10% of all CHD, F>M </li></ul><ul><li>RV compliance lower than LV, leads to L to R shunting. Increased blood flow through ASD,enlarge RA, RV and inc. Pul blood flow. </li></ul><ul><li>CF: Most infants and children are asymptomatic. </li></ul><ul><li>Fatigue and SOB </li></ul><ul><li>Palpitations and atrial dysrythmias </li></ul><ul><li>Recurrent pul infections </li></ul><ul><li>Paradoxical Image- stroke, organ damage </li></ul><ul><li>Systolic murmur- inc flow across PV </li></ul><ul><li>Wide fixed split S2 </li></ul>
  5. 5. Diagnosis and Management <ul><li>RV enlargement on CXR </li></ul><ul><li>RV ↑/RBBB on EKG </li></ul><ul><li>Surgery at age 2-5 </li></ul><ul><li>Natural History: </li></ul><ul><li>If not treated may lead to Atrial dysrrythmias and heart failure. </li></ul><ul><li>Small secondum ASD may close spontaneously during first year of life </li></ul><ul><li>Associated with Holt-Oram syndrome </li></ul>
  6. 6. VSD <ul><li>25% of all CHD </li></ul><ul><li>Degree of shunt depends upon the size of shunt and pressure difference b/n SVR and PVR. With age PVR decreases, increase bld flow to rt and then to pulmonary artery. </li></ul><ul><li>With large VSD, pul vessels are exposed to systemic pressure, leading to pulmonary hypertension and pulmonary vascular disease. </li></ul><ul><li>Large and moderate defects may present in first few weeks of life. </li></ul><ul><li>CF: tachypnea, dyspnea, FTT, systolic murmur at LLSB for small VSD, systolic/diastolic murmur if large VSD, pounding heart, frequent resp infections, CHF mostly with large VSD </li></ul>
  7. 7. VSD <ul><li>Complications: CHF, FTT, Endocarditis, Arrhythmia, PAH, Eisenmenger </li></ul><ul><li>CXR- Cardiomegaly </li></ul><ul><li>EKG- LVH </li></ul><ul><li>Natural History and Management: </li></ul><ul><li>20-80% closes spontaneously </li></ul><ul><li>No Abx prophylaxis required for dental procedures </li></ul><ul><li>Digoxin, diuretics and ACE inhibitors for afterload reduction </li></ul><ul><li>Small VSD, mostly medical, 15-50% closes spontaneously </li></ul><ul><li>Surgery early in life </li></ul><ul><li>Soft nipples to ease stress of feeding </li></ul><ul><li>Eisenmenger: Reversal of shunt to Rt to left, progressive cyanosis and exercise intolerance. Polycythemia, coagulopathy and high risk pregnancy. </li></ul>
  8. 8. AV Canal/Endocardial Cushion Defect <ul><li>Contiguous atrial and ventricular SD with abnormal AV valves. </li></ul><ul><li>Mostly associated with ostium primum defects, MR </li></ul><ul><li>Left to rt shunt across atrial/ventricular defect—pul hypertension and inc. PVR. </li></ul><ul><li>CHF and pulmonary infections in infancy with complete AV canal defects. </li></ul><ul><li>Diuretics for vol. overload </li></ul><ul><li>Treatment- usually surgical. </li></ul>
  9. 9. PDA <ul><li>Increase pulmonary bld flow </li></ul><ul><li>Assoc. with Prematurity & </li></ul><ul><li>congenital Rubella </li></ul><ul><li>CF: </li></ul><ul><li>Large: Systolic murmur, bounding pulses, CHF, usually req surgery </li></ul><ul><li>Small: Continuous murmur, indomethacin in premmies </li></ul><ul><li>PDA maintain systemic bld supply in CoA and Hypoplastic left heart </li></ul><ul><li>In term infant PDA is d/t deficiency of endothelial layer and vascular media but PDA in premature infant is d/t hypoxia and immaturity. PDA persisting >1 wk in term infant require medical/surgical management. In premature infants spontaneous closure may occur. </li></ul>
  10. 10. Coarctation <ul><li>Systemic bld flow obstruction, 98% time just below origin of Lt SCA and Ductus. </li></ul><ul><li>Presents with shock like picture in first few days of life d/t closing ductus </li></ul><ul><li>In first few days of life, PDA widens the juxtaductal aorta to provide temporary relief. </li></ul><ul><li>With severe CoA, RVH provide blood to descending aorta, femoral puses are palpable, ductal RT to Lt shunt present with differential cyanosis. </li></ul><ul><li>M:F::2:1 </li></ul><ul><li>Murmur at infrascapular area </li></ul><ul><li>Cardiac asthma d/t inc pul bld flow, LVH </li></ul><ul><li>4 extremity BP </li></ul><ul><li>EKG: LVH </li></ul><ul><li>CXR: Rib notching </li></ul><ul><li>MRI: better visualization </li></ul>
  11. 11. Coarctation <ul><li>Treatment: </li></ul><ul><li>Severe CoA, require PGE 1 in early infant life. </li></ul><ul><li>Balloon angioplasty- dissection later </li></ul><ul><li>Surgery is usual Tx </li></ul><ul><li>Post-op rebound hypertension require medical management. </li></ul><ul><li>Recoarctation may occur, require careful monitoring and revision. </li></ul><ul><li>Natural History: Persistent Hypertension and coronary artery disease if left untreated. </li></ul><ul><li>Associated with Turner’s, Bicuspid AV </li></ul><ul><li>May present with shock and diminished systemic perfusion, pulmonary congestion with decreased filling of failing left heart. Tx with ionotropes. </li></ul>
  12. 12. Bicuspid Aortic Valve/AS <ul><li>Mostly an incidental finding </li></ul><ul><li>Calcification of bicuspid valve in later life </li></ul><ul><li>leads to stenosis/insufficiency or aortic root dilatation. </li></ul><ul><li>Usually systolic murmur with opening snap </li></ul><ul><li>Treatment: </li></ul><ul><li>Stenosis: valvuloplasty, surgery </li></ul><ul><li>Insufficiency: surgery and vasodilators </li></ul><ul><li>Dilatation: ACE inhibitors </li></ul><ul><li>Associated with Turner’s, Williams, CoA </li></ul>
  13. 13. Pulmonary Stenosis <ul><li>Usually associated with ASD, symptoms d/t dominant defect. Valvular dysplasia with Noonan. </li></ul><ul><li>Hypertrophy of Rt ventricle with increased RV pressure </li></ul><ul><li>Arterial oxygen saturation remain normal even in severe stenosis unless Rt to Lt shunt through ASD/VSD/PDA exists. </li></ul><ul><li>Mild to moderate PS is usually asymptomatic. </li></ul><ul><li>Critical pulmonary stenosis: Severe PS in neonate leading to Rt to Lt shunting through PFO- cyanosis and CHF. </li></ul><ul><li>Treatment with balloon valvuloplasty </li></ul>
  14. 14. Ductal dependent Lesions <ul><li>Critical CoA, interrupted aortic arch, critical AS, HLHS </li></ul><ul><li>CF: Single and loud S2, increase in right ventricular activity, abnormal postductal SpO2. Decreased femoral pulses often are not present at this stage because the ductus usually is large. </li></ul><ul><li>If critical left heart obstruction is suspected, room air pulse oximetry in the lower extremities (postductal saturation) may be helpful. A value above 96% or 97% rules out a completely ductal-dependent left heart obstruction (hypoplastic left heart or interrupted aortic arch). High saturations, however, can occur in the setting of coarctation of the aorta. </li></ul><ul><li>The closure of the ductus arteriosus cause decreased systemic blood flow, oliguria, acidosis, pulmonary edema, and heart failure. As the cardiac output decreases, retrograde blood flow from the ductus into the common coronary artery (ascending aorta) results in decreased right and left coronary artery blood flow, leading to myocardial ischemia, ventricular dysfunction, and death. </li></ul><ul><li>The clinical presentation of left heart obstructive disease may mimic sepsis; the infant exhibits tachypnea, mottled gray skin, and poor perfusion, with decreased peripheral and central pulses. A high serum BNP concentration may prove to be a cardiac-specific marker of heart failure in this setting. </li></ul><ul><li>Fluid resuscitation, respiratory and ionotropic support. PGE1 infusion should be started before echocardiographic diagnosis. </li></ul>

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