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Congenital Heart DiseasesIISamer Abbas, MDCardiology UlC/Christ02/27/2008ClassificationJ Cyanosis with low pulm flow:-TOF-Ebstien Anamoly■ Hypoplastic of RV -Tricuspid atresia? Pulm atresiaJ Cyanosis with high pulm flow:-TGA-Double outlet V.? Double inlet V-TAPVD.Eisenmenger SyndromeIn 1897 Victor Eisenmenger published a paper entitled Congenital Defects of the Ventricular Septum.”In 1958, Paul Wood summarized Eisenmengers 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 anddigitalis, but collapsed and died suddenly on November 13 following a large hemoptysisEisenmenger SyndromeEl Large left to right shunt:-severe pulmonary vascular disease leading to shunt reversalU Initial reversible changes:一 Medial hypertrophy of the pulmonary vasculature-Intimal proliferationEisenmenger SyndromeLI Progressive irreversible changes:一 plexiform lesions一 necrotizing arteritisEl As the increased PVR approaches or exceeds the SVR the shunt is reversed.LI As R to L shunting develops cyanosis appears.n Most patients will develop exertional dyspnea and impaired exercise tolerance.Eisenmenger Syndrome3 Palpitations occur in 50% of patients一 A. fib/flutter in 40%-VTin 10%)3 Hemoptysis in ~20%n PE, angina, syncope, endocarditis ~10%[3 Signs of PHTN一 RV heave, palpable P2, and right sided S4LI Pulmonary ejection click and a soft scratchy SEM一 d/t dilated pulmonary trunk3 High pitched decrescendo diastolic murmur (Graham-Steele) audible in most patientsn Usually no peripheral edema until R HF ensuesEisenmenger SyndromeEl ECG shows RVH, RAE and RAD LI Atrial arrhythmias may be presentEisenmenger Syndrome[1 CXR reveals prominent central pulmonary arteries and decreased vascular markings (pruning) of the peripheral vesselsEisenmenger
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