Cardiology in the young
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Cardiology in the young · Feb 2004
Long-term survival in children with atrioventricular septal defect and common atrioventricular valvar orifice in Sweden.
The survival for patients with atrioventricular septal defect has improved markedly over the last decades and, during the same period, the survival of children with Down's syndrome has also increased. The aim of our study was to investigate long-term survival in patients having atrioventricular septal defect with common valvar orifice, but without associated significant congenital heart defects, in the setting of Down's syndrome, comparing the findings to those in chromosomally normal children with the same malformation. ⋯ Survival in uncomplicated atrioventricular septal defect with common atrioventricular valvar orifice has greatly increased, and surgical correction is now equally successful in patients with Down's syndrome and chromosomally normal patients, and for both genders. Death in connection with surgery is no longer the major threat, and focus must now be on long-term follow-up.
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The neonate with functionally univentricular physiology presents unique challenges to the cardiac team. An integrated approach that applies working knowledge of cardiac anatomy, cardiopulmonary physiology, and basic principles of intensive care is essential to guide management of each individual patient. This requires cooperative and constructive involvement of a surgical, medical, nursing and respiratory care team experienced in the management of such patients. In the neonate with this physiology, systemic oxygen delivery is optimized by manipulating pulmonary and systemic resistances, augmenting total cardiac output, and utilizing strategies for ventilation that preserve optimal pulmonary recruitment.