Congenital Heart Disease


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Congenital Heart Disease

  1. 1. Congenital Heart Disease• Evaluation of congenital heart disease requires o ECG, CXR, and hyperoxia test (measuring preductal (radial a.) arterial blood gas on room air and then repeating on FIO2 100%. Interpretation helps narrow diagnosis• Cyanotic Heart Disease (Ductal Independent Mixing Lesions)  TTTT o Truncus Arteriosus  Background: • Single arterial vessel, therefore complete mixing of systemic and pulmonic blood • usually has ventricular septal defect, 30% have right sided aortic arch • Associated with 22q11 microdeletion – DiGeorge’s syndrome may → hypocalcemia  Clinical Manifestations • Moderate Cyanosis, CHF develops in weeks, biventricular hypertrophy on ECG • Holosystolic Murmur at L sternal border • Single Loud S2 – b/c there’s only 1 valve  Treatment • Anticongestion = digoxin & diuretics • Surgical repair – close VSD, and make conduit b/n both aorta and pulm artery o d-Transposition of Great Arteries  Background: • Aorta arises from RV, Pulmonary A. arises from LV – operates in Parallel • #1 cyanotic HD presenting in neonates, 5% of all congenital heart disease • Most have intact ventricular septum • ASD, VSD, or PDA that allows mixing is required for survival  Clinical Manifestions • Cyanosis present from birth, Single Loud S2, Right ventricular heave, • If ventricular septum defect, then harsh holosystolic murmur lower left sternal border • Right ventricular hypertrophy seen, right axis deviation on ECG, Egg-shaped heart on X-ray  Treatment • PGE1 to keep PDA open • Rashkind balloon atrial septostomy → atrial mixing of blood • Surgical repair – switch back o Total Anomalous Pulmonary Venous Connection without Obstruction (TAPVC)  Background: • Rare lesion, where all pulmonary venous return is directed to the Right Atrium o May drain to: Supracardia: SVC (50%), Cardiac: Directly to RA (20%), Infradiaphragmatic: Portal or Hepatic (20%), Mixed (10%). • “Without obstruction” refers to the site where the anomalous vein enters a vessel – ie there is no constriction at this site. Therefore there is increased Pulmonary blood flow, mild Pulmonary HTN, and Moderate Cyanosis  Clinical Manifestations • Findings similar to Atrial septal defect • Right ventricular heave, wide and fixed split S2 • Systolic ejection murmur at Left upper sternal border • Cardiomegaly, increased pulmonary vasculature on CXR, • ECG shows right axis deviation and Right Ventricular Hypertrophy  Treatment • Rx of CHF • Surgically redirect vessels into Left Atrium Necessary in 1st month of Life• Cyanotic Congenital Heart Disease: Lesions with Ductal-Dependent Pulmonary Blood flow o Tricuspid Atresia
  2. 2.  Background: • No connection b/n RA and RV, leads to hypoplasia or absence of Right ventricle • 30% have transposition of great arteries, 90% have VSD (this allows blood to flow from the left ventricle to both the Pulm A. & Aorta • Most also have pulmonic stenosis • The only way for systemic blood to get to the Left side (and therefore the Pulm A. and Aorta is through a Patent Foramen Ovale or ASD  Clinical Manifestations • Cyanosis is severe, poor feeding, tachypnea over first 2 weeks of life • VSD → loud holosystolic murmur at left lower sternal border – if present • PDA → continuous murmur – if present • ECG – superior axis and Left Ventricular Hypertrophy  Treatment • PGE1 to keep PDA open to maintain Pulmonary flow • balloon atrial septostomy, if atrial defect not sufficient • Blalock-Taussig Shunt – Gor-Tex conduit b/n subclavian Artery and Pulmonary A.o Pulmonic Atresia with Intact Ventricular Septum  Background: • Rare defect with pulmonary valvular and infundibular atresia with right ventricular and tricuspid valve hypoplasia o ie. Basically the Right Ventricle is unable to produce any pulmonary outflow – so RV is hypertensive and have Tricuspid Regurgitation. • Dependent on PDA for survival • In some cases Coronary perfusion is dependent on Right Ventricle! Therefore decreasing RV hypertension can lead to MI and death  Clinical Manifestations • Extremely cyanotic, tachypneic, Tricuspid Regurgitation murmur on left sternal border • Continuous PDA murmur • ECG – LV hypertrophy, leftward axis  Treatment • PGE1 to keep PDA open to maintain Pulmonary flow • Must check coronary arteries before surgery via catheterization • If coronaries not RV dependent, then RV to pulmonary artery connection is madeo Tetralogy of Fallot  Background: • #1 cause of Cyanosis presenting during 3rd week of life • 4 defects: VSD, Right ventricular outflow obstruction, Right ventricular hypertrophy, and “overriding” large aorta  Clinical Manifestations • Cyanotic due to right to left shunting across VSD and decreased Pulmonary flow • “Tet Spells” periodic cyanosis and agitation – caused be increase in RV outflow tract resistance, increasing the R → L shunt. Last minutes to hours, may resolve or progress to death • exam: RV heave felt, Systolic ejection murmur in L upper sternum due to RV outflow obstruction • PDA – cont murmur, VSD – holosystolic murmur at Left lower sternal border • ECG – shows RA dilation, RV hypertrophy • 25% have right sided arch  Treatment • “Tet-spells” – Rx by decreasing R → L shunting, by increasing systemic vascular resistance, and dec pulm vascular resistance
  3. 3. o Supplimental O2, vagal maneuvers, morphine sulfate, vasoconstrictors, beta- blockers and volume administration, also bring the child’s knees to their chest increasing systemic resistance and decreasing the shunt from Right to Left. o pull knees of child to chest – decreases preload, increases systemic resistence o Ebstein’s Anomaly  Background • Extremely rare, where leaflets are displaced into RV cavity • Results in hypoplasia of RV, tricuspid regurg and/or stenosis • 80% have Patent Foramen Ovale, with R → L shunt • Massively dilated RA • Associated with Wolff-Parkinson-White (WPW) syndrome  Clinical Manifestations • Cyanosis and CHF in first few days of life • Widely fixed S2 and Tricuspid Regurg at L lower sternal border • ECG shows Right Bundle Branch Block • Delta Waves due to WPW syndrome • CXR – MASSIVE cardiomegaly, with enlarged RA  Treatment • PGE1 to keep PDA open to maintain Pulmonary flow • CHF therapy with Digoxin and diuretics • Surgical repair of Tricuspid has had poor results• Cyanotic Congenital Heart Disease: Lesions with Ductal Dependent Systemic Blood Flow o Hypoplastic Left Heart Syndrome (HLHS)  Background • #1 cause of death from congenital HD during 1st month • Syndrome with: Hypoplasia of LV, Aortic Valve stenosis or atresia, Mitral Valve stenosis or atresia and hypoplasia of ascending aortic with coarctation of aorta. • All combine to reduce flow through the Left side • Causes L → R shunt at Atrial level & R → L shunt at Ductus Arteriosus  Clinical Manifestations • As ductus close → CHF, moderate cyanosis, tachypnea, pulmonary rales and hepatomegaly • Poor or absent peripheral pulses • S3 and Loud single S2  Treatment • PGE1 to keep PDA open to maintain flow to the Aorta from right side • Norwood palliation: restores unobstructed systemic blood flow o Interrupted Aortic Arch  Background • Aortic arch is not complete and ends in: Type A: beyond L subclavian, Type B: b/n L subclavian and L common carotids, Type C: b/n L common carotids and brachiocephalic arteries • All flow distal to the dead-end must come from PDA (R → L) • Also associated with DiGeorge’s syndrome due to 22q11 microdeletion  Clinical Manifestations • Pulmonary edema • Presentation similar to Critical Coarctation of Aorta (below)  Treatment • PGE1 to keep PDA open to maintain systemic flow • Surgery to reanastomose aortic segments o Total Anomalous Pulmonary Venous Connection with Obstruction
  4. 4.  Background – see above – • Presence of Severe Pulmonary Edema differentiates from w/o obstruction  Clinical Manifestations • TAPVC w/ obstruction presents with extreme cyanosis, tachypnea, and dyspnea • RV heave, narrowly split S2, Ventricular gallop (S3) • ECG – R axis deviation, RA dilation, RVH • CXR – pulmonary edema  Treatment • PGE1 should not be given – as pulmonary flow is already overflowed • Management of CHF and Pulmonary edmema • Surgical redirection of aberrant vessels into left atrium• Acyanotic Congenital Heart Disease o Atrial Septal Defects  Background • 3 types: o Ostium secundum: midportion of atrial septum o Ostium primum: defect in low atrial septum o Sinus venosus: defect at junction of RA and SVC or IVC • Usually results in L → R shunting, increasing Pulm blood flow  Clinical Manifestations • Usually asymptomatic • RV heave often present • Systolic ejection murmur in Pulmonic area • S2 wide and constantly split  Treatment • Ostium Secundum usually spontaneously close • Ostium Primum and sinus venosus require surgical closure o Ventricular Septal Defects  Background • #1 congenital defect, 26% of all • 5 types: conoventicular, muscular, inlet, conoseptal hypoplasia, malalignment  Clinical Manifestations • Small shunt has no symptoms • Larger, gives growth failure, CHF, chronic lower respiratory infection • With Eisenmenger physiology (R → L) presents with SOB, dyspnea, chest pain, and cyanosis. • Smaller defect = louder murmur • If pulmonary vascular obstruction → RV heave, ejection click, short systolic ejection murmur, diastoloic murmur of pulmonary valve insufficiency, Loud S2  Treatment • Most close without intervention • Rx CHF • May require surgery if large o Common Atrioventricular Canal  Background • Endocardial cushion defect • Deficiency of both atrial and ventricular septa and of Mitral and tricuspid valves • Severity varies widely depeding on extend of defect. • Approx 1/3 of Down’s syndrome have this; • L → R shunt at atrial and ventricular level  Clinical Manifestations
  5. 5. • Pulmonary HTN may develop • Presents with tachypnea, dyspnea, and poor feeding • Blowing holosystolic murmur at L lower sternal border due to VSD and Fixed wide S2 due to ASD • ECG – right axis deviation, RA dilation, LA dilatation  Treatment • Surgical repairo Patent Ductus Arteriosus  Background • 10% of Congenital heart disease, 2:1 female, higher in premies, • connects Pulmonary a to Aorta just distal to Left Subclavian a.  Clinical Manifestations • Small – no symptoms • Larger L → R: CHF, slow growth, repeated Lower Resp tract infections • Reversal to R → L: result of high pulm vasc resistance, causes SOB, dyspnea and Cyanosis • Continuous “machinery murmur”  Treatment • Indomethacin closes PDA by decreasing PGE1 levels • Usually closes by 1 month, if not surgery is requiredo Coarctation of the Aorta  Background • Constriction of aorta, usu decending at insertion site of ductus. • If in female – consider Turner’s syndrome • Aortic valve is Bicuspid in 80%,  Clinical Manifestations • Weak femoral pulses relative to upper • If severe may rely on PDA • Systolic ejection murmur at Apex • Visualized with Echo  Treatment • Balloon angioplasty or surgical end-to-end anastomosiso Aortic Stenosis  Background • Rigid thinkened valvular tissue → LV hypertrophy  Clinical Manifestations • Presents with cardiac collapse or harsh systolic ejection murmur at right upper sternal border and is preceded by ejection click • If severe may depend on PDA • On CXR see poststenotic dilatation of aorta • May see inverted T waves  Treatment • Open surgical valvotomy or by balloon valvuloplastyo Pulmonic Stenosis  Background • Pulmonary commissures are fused  Clinical Manifestations • May cause dyspnea on exertion and angina, • Ejection click varies with inspiration • Harsh ejection murmur at L upper sternal border  Treatment
  6. 6. • Balloon valvuloplasty