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Therrien J et al Pulmonary valve replacement in adults late after repair of tetralogy of Fallot: are we operating too late J Am Coll Cardiol 2000 Nov 1;36(5):1670 5 [PMID: 11079675] Warnes CA The adult with congenital heart disease: born to be bad J Am Coll Cardiol 2005 Jul 5;46(1):1 8 [PMID: 15992627]
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with a QRS width of greater than 180 msec The width of the QRS corresponds to the RV size, and in many patients, the QRS width actually decreases following repair of the pulmonary insufficiency
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Echocardiography/Doppler usually establishes the diagnosis by noting the unrestricted (large) VSD, the RV infundibular stenosis, and the enlarged aorta In patients who have had tetralogy of Fallot repaired, echocardiography/Doppler also provides data regarding the amount of pulmonic regurgitation, RV and LV function, and the presence of aortic regurgitation Cardiac MRI and CT can quantitate both the pulmonary insufficiency and the RV volumes In addition, cardiac MRI and CT can identify whether there is either a native pulmonary arterial branch stenosis or a stenosis at the distal site of a prior Blalock or other shunt Cardiac catheterization is sometimes required to document the degree of pulmonic regurgitation because noninvasive studies depend on velocity gradients Pulmonary angiography demonstrates the degree of pulmonic regurgitation, and RV angiography helps assess any postoperative outflow tract aneurysm
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PATENT DUCTUS ARTERIOSUS
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Rare in adults Adults with small or moderate size patent ductus arteriosus are usually asymptomatic, at least until middle age Widened pulse pressure; loud S2 Continuous murmur over left pulmonary area; thrill common Echocardiography/Doppler is helpful, but the lesion is best visualized by MRI, CT, or contrast angiography
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The embryonic ductus arteriosus allows shunting of blood from the PA to the aorta in utero The ductus arteriosus normally closes immediately after birth so that pulmonary blood flows to the pulmonary arteries Failure to close normally results in a persistent shunt connecting the left PA and aorta, usually near the origin of the left subclavian artery Prior to birth, the ductus is kept patent by the effect of circulating prostaglandins; in the neonate, a patent ductus can often be closed by administration of a prostaglandin inhibitor such as indomethacin The effect of the persistent left-to-right shunt on the pulmonary circuit is dependent on the size of the ductus If large enough, pulmonary hypertension (Eisenmenger physiology) may occur A small ductus may be well tolerated until adulthood
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Prognosis & Treatment
A few patients with just the right amount of pulmonic stenosis enter adulthood without having had surgery However, most patients have had surgical repair of tetralogy of Fallot, including VSD closure, resection of infundibular muscle, and insertion of an outflow tract patch Many have a transannular patch resulting in pulmonic regurgitation Patients should be monitored to ensure the RV volume does not increase Low-pressure pulmonic regurgitation is difficult to diagnose except during cardiac catheterization due to the fact that the RV diastolic pressures tend to be high and the pulmonary arterial diastolic pressure is low This means there is little gradient between the PA and the RV in diastole, so that there may be little murmur or evidence for turbulence on color flow Doppler If the RV begins to enlarge, it must be assumed that this is due to pulmonic regurgitation until proven otherwise If an anomalous coronary is present, then an extracardiac conduit around it from the RV to the PA may be necessary By 20-year follow-up, reoperation is needed in about 10 15%, not only for severe pulmonic regurgitation but also for residual infundibular stenosis Usually the pulmonary valve is replaced with a pulmonary homograft, though a porcine bioprosthetic valve is also suitable Cryoablation of tissue giving rise to arrhythmias is sometimes performed at the time of reoperation Branch pulmonary stenosis may be percutaneously opened by stenting All patients require endocarditis prophylaxis
Atik FA et al Long-term results of correction of tetralogy of Fallot in adulthood Eur J Cardiothorac Surg 2004 Feb;25(2): 250 5 [PMID: 14747122] Shinebourne EA et al Tetralogy of Fallot: from fetus to adult Heart 2006 Sep;92(9):1353 9 [PMID: 16908723]
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