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Congenital Heart Diseases II

Congenital Heart Diseases II. Samer Abbas, MD Cardiology UIC/Christ 02/27/2008. Cyanosis with low pulm flow: - TOF - Ebstien Anamoly - Hypoplastic of RV - Tricuspid atresia - Pulm atresia. Cyanosis with high pulm flow: TGA Double outlet V. Double inlet V TAPVD.

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Congenital Heart Diseases II

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  1. Congenital Heart DiseasesII Samer Abbas, MD Cardiology UIC/Christ 02/27/2008

  2. Cyanosis with low pulm flow: - TOF - Ebstien Anamoly - Hypoplastic of RV - Tricuspid atresia - Pulm atresia Cyanosis with high pulm flow: TGA Double outlet V. Double inlet V TAPVD. Classification

  3. Eisenmenger Syndrome • In 1897 Victor Eisenmenger published a paper entitled “Congenital Defects of the Ventricular Septum.” • In 1958, Paul Wood summarized Eisenmenger’s accounts: “The patient was a powerfully built man of 32 who gave a history of cyanosis and moderate breathlessness since infancy. He managed well until January of 1894 when dyspnea increased and edema set in. Seven months later he was admitted to the hospital in a state of heart failure……He improved with rest and digitalis, but collapsed and died suddenly on November 13 following a large hemoptysis”

  4. Eisenmenger Syndrome • Large left to right shunt: • severe pulmonary vascular disease leading to shunt reversal • Initial reversible changes: • Medial hypertrophy of the pulmonary vasculature • Intimal proliferation

  5. Eisenmenger Syndrome • Progressive irreversible changes: • plexiform lesions • necrotizing arteritis • As the increased PVR approaches or exceeds the SVR the shunt is reversed. • As R to L shunting develops cyanosis appears. • Most patients will develop exertional dyspnea and impaired exercise tolerance.

  6. Eisenmenger Syndrome • Palpitations occur in >50% of patients • A. fib/flutter in 40% • VT in 10%) • Hemoptysis in ~20% • PE, angina, syncope, endocarditis ~10% • Signs of PHTN • RV heave, palpable P2, and right sided S4 • Pulmonary ejection click and a soft scratchy SEM • d/t dilated pulmonary trunk • High pitched decrescendo diastolic murmur (Graham-Steele) audible in most patients • Usually no peripheral edema until R HF ensues

  7. Eisenmenger Syndrome • ECG shows RVH, RAE and RAD • Atrial arrhythmias may be present

  8. Eisenmenger Syndrome • CXR reveals prominent central pulmonary arteries and decreased vascular markings (pruning) of the peripheral vessels

  9. Eisenmenger Syndrome • Large variation in life expectancy in adults with Eisenmenger syndrome • Rate of survival among patients with Eisenmenger syndrome is: • 80% at 10 years • 77% at 15 years • 42% at 25 years • Recent study of 109 adults revealed following as independent predictors of mortality: • Age at presentation • Supraventricular arrhythmias • Poor NYHA functional class (III or IV)

  10. Eisenmenger Syndrome • Pregnancy is discouraged due to high maternal (50%) and fetal (60%) mortality. • CVA may occur secondary to paradoxical emboli • Cerebral abscesses • Avoid intravascular volume depletion, heavy exertion, high altitudes, and use of vasodilators • IV epoprostenol may be beneficial in decreasing PVR • Phlebotomy with isovolumic replacement is recommended for patients with moderate to severe symptoms of hyperviscosity and an elevated hematocrit >65% • Prevention of iron deficiency is important • Supplemental oxygen reduces episodes of dyspnea • Lung transplantation (with repair of cardiac defect) or heart/lung transplantation is an option

  11. Tetralogy of Fallot • Etienne-Louis Arthur Fallot made the first published bedside diagnosis that was proven at post-mortem in 1888 and called the condition of ‘maladie bleue’.

  12. Epidemiology • Prevalence varies from 0.26-0.48/1000 live births. • Exact incidence of TOF with PS versus pulmonary atresia or absent pulmonary valve is not known as most studies lump them together. • Most recent study is the BWIS (Baltimore –Washington Infant Study) -Incidence 0.33/1000 live births -Fifth most common defect overall (6.8% of all CHD) -Most common cyanotic CHD -TOF with PS 0.26/1000 live births (5.4% of all CHD) -Suggestion of male predominance ( p=NS)

  13. Genetics • Syndromes associated with TOF: • DiGeorge’s • Velocardiofacial • Alagille’s • Trisomy 21/18/13 • Cat Eye • San Luis Valley • Mutations of Nkx 2.5 may be associated with TOF.

  14. Morphology • Ventricular Septal Defect • 80% of cases fibrous continuation between the mitral, tricuspid, and aortic valves • 20% cases there is a muscular rim around the defect. • Rarely subarterial defect due to hypoplasia of the outlet septum. • The pulmonary valve annulus tends to be hypoplastic or atretic.

  15. Morphology • Overriding Aorta • Degree of override can vary from an exclusive connection of the right ventricle to an exclusive connection to the left ventricle. • Pulmonary Stenosis: • Infundibular • valvular

  16. Morphology • Concentric right ventricular hypertrophy: • Secondary to RV outflow obstruction

  17. Associated Lesions • Pulmonary valvar stenosis • Pulmonary atresia • Absence of pulmonary valve leaflets • pulmonary insufficiency in utero and marked dilatation of the pulmonary trunk. • Right aortic arch • Patent foramen ovale • Atrial Septal Defect ( Pentalogy of Fallot) • Anomalous origin of the LAD from the RCA.

  18. On Exam • Cyanosis of varying degrees • Clubbing • Accentuated RV impulse • Second heart sound usually single and loud. If mild PS with normal valve apparatus S2 normally split. • Pulse pressure may be wide in the presence of aortopulmonary collaterals or PDA.

  19. On Exam • Systolic Murmur: • Upper left sternal border, crescendo-decrescendo or plateau • Intensity inversely proportional to severity of the right to left shunt • In PS with intact septum murmur directly proportional to the severity of stenosis • Murmur decreases in intensity during hypercyanotic spells • Diastolic Murmur: • If AI concomitantly heard in TOF with APV syndrome

  20. Tetralogy of Fallot

  21. Tetralogy of Fallot

  22. Catheterization

  23. Surgical repair • Closure of VSD • Relieving the RVOTO • Pulmonary valvotomy • Resection of infundibular muscle • RVOT or subannular patch • Transannular patch • only when PV annulus is restrictive • Pulmonary valve implantation • usually in adolescents and adults • Extracardiac conduit between the RV and PA • Angioplasty or patch augmentation of the central pulmonary arteries • Closure of ASD or PFO

  24. Mechanical problems Significant Pulmonary regurgitation RV Dilatation Restrictive RV Residual RVOTO Aneurysmal dilatation of the RVOT Residual VSD AR with or without root dilatation LV dysfunction Infective endocarditis Complications After Repair

  25. Complications after repair Electrical Problems • Heart block: RBBB, Bifascicular.Complete heart block is rare. • Supraventricular arrythmia (A.flutter/A.fib) • Relatively common. A.flutter-SCD,1:1. • NSVT: Significance uncertain, ? SCD • VT • SCD

  26. Risks for SCD/VT • Older age at repair • Male gender • Worse heart failure NYHA Class • Chronic RV volume overload • Presence of PR • QRS duration >180 msec • Transient complete heart block lasting more than 3 days after repair.

  27. Management of the complications • Significant PR and RV dilatation • Pulmonic valve replacement with homograft or porcine bioprosthesis • Timing of surgery is debated. Selection of asymptomatic patient ?criteria. • Might TV annuloplasty if concomitant moderate to severe TR. • Restrictive RV • Diuretics in the post-operative period. • Residual RVOTO • Indicated if RVSP>2/3 systemic pressure. • Surgery or balloon angioplasty with stenting.

  28. Management of complications • Aneurysmal dilatation of the RVOT • Residual VSD: • Surgical correction if Qp/Qs>2:1 or 1.5-2:1 with LV dilatation or dysfunctionor H/O paradoxical emboli • Dilatation of aortic root: • Aortic root replacement when ascending aorta>55 mm and/or in the presence severe AI.

  29. Management of the Complications • Heart block: • Patients with bifascicular block with prolonged PR (>0.20 msec) should have holter or EPS. If true trifascicular block is documented then pacemaker. • SVT • Correction of target residual hemodynamic lesions. • RF ablation for a.flutter or reentrant tachycardia either percutaneously or during surgery. • Biatrial maze for a.fib. • Anti-arrhythmic meds /antitachycardia pacemakers can be used as adjunctivetools.

  30. Management of Complications • VT • Correction of the hemodynamic lesions • RF ablation of VT focus • Role of AICD (?) • Antiarrhythmic meds for symptomatic patients. • SCD • Resuscitated SCD-AICD • Role of prophylactic AICD (?)

  31. Fontan procedure-requiring patient • Tricuspid atresia • Hypoplastic left ventricle • Double inlet ventricle • Isomerism

  32. Tricuspid atresia

  33. Tricuspid Atresia

  34. On Echo

  35. Hypoplastic Left Heart

  36. Double Inlet Ventricle

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