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1/4. Precoronary stenosis after stage I palliation for hypoplastic left heart syndrome.

    We report a patient with stenosis of the native ascending aorta after palliation of hypoplastic left heart syndrome and aortic atresia. We describe the approach to diagnosis, temporary support with extracorporeal membrane oxygenation, and successful surgical reintervention. Stenosis of the native ascending aorta is an important, potentially reversible cause of acute, early postoperative ventricular dysfunction.
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2/4. pulmonary artery banding before Norwood procedure.

    Described here is the use of bilateral pulmonary artery banding as a means to achieve effective restoration of adequate systemic blood flow before a Norwood procedure in two newborns with hypoplastic left heart syndrome who presented after birth with a severe imbalance of Qp/Qs and multiorgan system dysfunction despite usual pharmacologic and ventilatory strategies.
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3/4. Diminutive fetal left ventricle at mid-gestation associated with persistent left superior vena cava and coronary sinus dilatation.

    In a fetus with a small left ventricle diagnosed at mid-gestation, a persistent left superior vena cava connected to a dilated coronary sinus was detected. Although the size of the mitral annulus appeared to be normal, opening of the mitral valves was restricted in diastole. Echocardiographic follow-up showed no significant growth of the left ventricle and termination of pregnancy was carried out at 31 weeks at the parents' request. In addition to the prenatal findings, postmortem examination revealed a small mitral annulus with abnormal insertion of the mitral valve chordae tendineae. We hypothesized that abnormal venous return to a dilated coronary sinus may have led to mitral valve dysfunction and hypoplasia. On the other hand the left ventricular hypoplasia we observed may have involved a global abnormality of the left-sided cardiac structures.
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4/4. Reversal of fenestration flow during ventricular systole in Fontan patients in junctional or ventricular paced rhythm.

    Sinus node dysfunction is relatively common in patients with Fontan palliation for single ventricle congenital heart disease, and such patients often are in junctional rhythm or may have pacemaker systems for bradycardia. Because the physiologic determinants of left atrial pressure play a major role in determining pulmonary blood flow and therefore cardiac output in Fontan patients, the loss of atrioventricular (AV) synchrony in junctional rhythm or demand ventricular pacing in these patients might be expected to influence cardiac output. We report two cases of Fontan patients with the absence of AV synchrony that resulted in reversal of flow through the Fontan fenestration during ventricular systole. In both cases, restoration of AV synchrony by atrial pacing resulted in the elimination of retrograde fenestration flow, increased cardiac output, and improved clinical status.
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