Rev Esp Cardiol
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Pulmonary arterial hypertension frequently arises in patients with congenital heart disease. The vast majority present with congenital cardiac shunts. Initially these may manifest as left-to-right (i.e. systemic-to-pulmonary) shunts. ⋯ Postoperative pulmonary hypertension, both in the immediate period after surgical repair and during long-term follow-up, remains a real therapeutic challenge. The clinical situation of a single ventricle with Fontan circulation also presents difficulties when pulmonary vascular lesions are present. This article reviews pulmonary hypertension associated with congenital shunts and discusses a number of the specific problems encountered.
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Echocardiographic evaluation of right ventricular systolic function remains subject to debate because of the anatomic peculiarities of the right ventricle. Several methods have been developed for quantifying right ventricular systolic function. The aim of this study was to determine the reliability and reproducibility of a number of echocardiographic parameters by evaluating their inter-observer and intra-observer variability. ⋯ The most reliable and reproducible echocardiographic parameters for evaluating right ventricular systolic function in patients with isolated mitral valvulopathy were the TAPSE and the tricuspid annular peak systolic velocity.