Current opinion in pediatrics
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Curr. Opin. Pediatr. · Oct 2009
ReviewAssessment of pulmonary vasoreactivity in children with pulmonary hypertension.
With the current advance in understanding and treatment of pulmonary arterial hypertension in children, pulmonary vasoreactivity testing would navigate the treatment option. An inclusive review of the milestone studies and also recent literature over the last few years on the pulmonary vasoreactivity testing in children will provide the update on various available pulmonary vasodilator agents, markers related to vasoreactivity response, the implication of the testing result on child management and outlook for the long-term outcome. ⋯ The precise definition of pulmonary vasoreactivity testing remains debatable, particularly in children with pulmonary hypertension related to congenital heart defect. Defining the responder, in order to navigate the treatment option, is frequently dictated by institutional experience and facilities. Meanwhile, the criteria for responder in children with idiopathic pulmonary artery hypertension are reasonably consistent. In general, responders seem to have less severe disease and better prognosis.
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Curr. Opin. Pediatr. · Oct 2009
Surgical advances in the treatment of adults with congenital heart disease.
This article reviews three common abnormalities requiring surgical treatment in adults with congenital heart disease (CHD): Ebstein's anomaly, the failing Fontan, and pulmonary valve disease. ⋯ Surgery for Ebstein's anomaly can now be offered to older patients at low risk and with good late outcome. The operation includes tricuspid valve repair or replacement and frequent concomitant procedures such as atrial septal defect closure, arrhythmia surgery (the maze procedure), and coronary artery bypass grafting. Fontan conversion involves: conversion of the previously created atriopulmonary connection to a total cavopulmonary artery, extracardiac Fontan; arrhythmia surgery, typically with a modified biatrial maze procedure along with placement of an antitachycardia, dual-chamber pacemaker with steroid-eluting epicardial leads in all patients; and concomitant procedures to treat associated lesions. The need for PVR is increasing for many adults with CHD. In the past, residual defects such as chronic pulmonary regurgitation following repair of tetralogy of Fallot were considered benign. Recent evidence suggests that pulmonary regurgitation causes significant morbidity, producing right ventricular dilatation and dysfunction, exercise intolerance, arrhythmias, and sudden death. Multiple options exist for PVR including several recent developments such as PVR with a man-made polytetrafluoroethylene bicuspid valve and percutaneous PVR.
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Curr. Opin. Pediatr. · Oct 2009
ReviewThe adult with congenital heart disease: medical and surgical considerations for management.
The purpose of the present review is to outline some of the challenges of surgical and medical management in the adult with congenital heart disease (CHD). ⋯ Adults with CHDs present difficult challenges in the preoperative, intraoperative, and postoperative setting. Regional centers of excellence with congenitally-trained cardiac surgeons, cardiologists, and other medical subspecialists are required to optimize outcome.