Search

Infants with an unbalanced atrioventricular septal defect (AVSD) frequently present with comorbidities that may have an impact on their medical course and outcome. This study aimed to assess outcomes and explore possible prognostic indicators for patients undergoing surgical palliation for an unbalanced AVSD. The medical records of all infants presenting to the authors’ institution with an unbalanced AVSD over a 5-year period were retrospectively reviewed for assessment of outcomes and comorbidities. The study group consisted of 44 patients with an overall survival rate of 51% for the entire follow-up period. The majority of these patients (88%) underwent single-ventricle palliation, with an 83% rate of survival to initial hospital discharge and an overall long-term survival rate of 50%. The midterm outcome was significantly worse than that for a cohort of hypoplastic left heart syndrome patients undergoing single-ventricle palliation during the same period ( P = 0.03). In addition, 30% of the patients required either repair or replacement of their systemic atrioventricular valve at initial palliation or during subsequent follow-up evaluation. Of the patients with an unbalanced AVSD, 75% had associated congenital anomalies. In conclusion, infants with an unbalanced AVSD are a high-risk population with diminished midterm survival compared with palliated patients who have more classic forms of hypoplastic heart syndromes. This may be due to the higher incidence of both severe atrioventricular valve regurgitation and important associated congenital anomalies. [ABSTRACT FROM AUTHOR]

Abstract: Hypoplastic left heart syndrome encompasses a diverse group of heart defects that share the common feature of underdevelopment of the left heart. There are multiple anatomic substrates involved in the development of hypoplastic left heart syndrome, as well as variants of classic hypoplastic left heart, that may signify unique risks and benefits. Additionally, other functional and anatomic cardiac findings as well as extracardiac anomalies are frequently associated with hypoplastic left heart syndrome and have significant impact on outcome. Through a careful, systematic approach, fetal echocardiography can detect many of the anatomic and functional details that contribute to outcomes. Detailed fetal ultrasound and fetal karyotype will diagnose many of the non-cardiac lesions that can be found with hypoplastic left heart syndrome. Parents must also be counseled of risk factors that may not be detected in utero. Knowledge of the specific diagnostic features that can affect outcome allows the fetal cardiologist to obtain all the necessary details, and provides the most accurate assessment of probable outcome in each individual patient. [Copyright &y& Elsevier]

There has traditionally been less concern regarding coronary anomalies with left-sided congenital heart lesions such as hypoplastic left heart syndrome (HLHS) or Shone complex than with other lesions. However, coronary anomalies in this setting can profoundly affect surgical intervention, particularly when surgical repair involves the ascending aorta. We describe four patients with congenital left-sided heart lesions in which left coronary artery (LCA) anomalies substantially affected intervention and outcome. In the first two cases, the coronary anomalies were not identified prospectively and resulted in surgical injury directly to the coronary or to its surrounding region. In the latter two cases, successful identification of the coronary anomaly preoperatively allowed for modification of surgical technique and/or intervention. We conclude that detailed coronary artery assessment should be part of the routine echocardiographic evaluation of congenital left-sided heart lesions that require surgery. [ABSTRACT FROM AUTHOR]

Although most prenatally diagnosed pulmonary sequestrations (PS) are asymptomatic, large lesions are associated with pleural effusions and pulmonary hypoplasia. We present the first reported case of a prenatally diagnosed giant extralobar pulmonary sequestration that required the ex utero intrapartum treatment (EXIT) procedure with resection and extracorporeal membrane oxygenation (ECMO). We discuss the compelling rationale for performing EXIT-resection-ECMO in the setting of a large thoracic mass and anticipated severe respiratory failure at birth. Copyright © 2009 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]