Hypoplastic Left Heart Syndrome Is Not a Predictor of Worse Intermediate Mortality Post Fontan

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Predictors of outcome after the Fontan operation: is hypoplastic left heart syndrome still a risk factor?

OBJECTIVE This study was undertaken to evaluate factors contributing to a decrease in early mortality and morbidity after the Fontan procedure between January 1, 1992, and December 31, 1999. METHODS Outcomes evaluated were early survival, duration of pleural effusions, and duration of hospitalization. Potential predictors evaluated included anatomic diagnosis, presence of a common atrioventri...

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Myocardial contractile response to dobutamine in hypoplastic left heart syndrome post-Fontan

Methods Prospective data from anesthetized patients with HLHS undergoing clinical XMR were analyzed. A fluid filled MR compatible catheter placed in the systemic RV recorded pressures at rest, with dobutamine infusion at 10 mcg/kg/ min and at 20 mcg/kg/min. Simultaneous cine short axis stacks of the ventricle were performed. Pressure-volume loops were constructed. End-systolic pressure-volume r...

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Hypoplastic left heart syndrome

Hypoplastic left heart syndrome (HLHS) refers to the abnormal development of the left-sided cardiac structures, resulting in obstruction to blood flow from the left ventricular outflow tract. In addition, the syndrome includes underdevelopment of the left ventricle, aorta, and aortic arch, as well as mitral atresia or stenosis. HLHS has been reported to occur in approximately 0.016 to 0.036% of...

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Hypoplastic left heart syndrome is heritable.

OBJECTIVES This study sought to determine the size of the genetic effect (heritability) in families identified by a hypoplastic left heart syndrome (HLHS) proband. BACKGROUND Hypoplastic left heart syndrome is a severe form of cardiovascular malformation (CVM), and it remains a leading cause of infant mortality and childhood morbidity. Familial clustering of HLHS and bicuspid aortic valve (BA...

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Hypoplastic left-heart syndrome.

Hypoplastic left heart syndrome may be accurately diagnosed during fetal life. Prenatal diagnosis provides the opportunity for parents to make an informed choice about their options, including surgery, nonintervention postnatally or termination of pregnancy. Short to medium term survival continues to improve for a condition that was previously invariably lethal. There continues to be a signific...

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ژورنال

عنوان ژورنال: The Annals of Thoracic Surgery

سال: 2017

ISSN: 0003-4975

DOI: 10.1016/j.athoracsur.2017.08.032