Pulmonary arterial hypertension associated with tetralogy of Fallot

International Heart Journal
Jun Yasuhara, Hiroyuki Yamagishi

Abstract

Pulmonary arterial hypertension (PAH) is a common postoperative complication in patients with congenital heart disease (CHD). Although the recent clinical classification of pulmonary hypertension divided PAH associated with CHD (PAH-CHD) into several subclasses, the anatomical and hemodynamic features of postoperative PAH-CHD vary enormously. Therefore, it is still difficult to obtain clinical evidence supporting the indication of pulmonary vasodilators for PAH-CHD. We often encounter patients with PAH occurring after surgical treatment of tetralogy of Fallot (TOF), especially patients with major aortopulmonary collateral arteries (MAPCAs). PAH might be caused by pulmonary agenesis, hypoplasia and/or thrombosis, inadequate closure of the ventricular septal defect, relief of the pulmonic stenosis, or an excessively large prior systemic-to-pulmonary shunt. Moreover, patients with TOF and MAPCAs who are diagnosed as inoperable because of the presence of PAH show similar hemodynamic and clinical features to patients with Eisenmenger syndrome. The MAPCAs in these patients usually show hypoplastic and abnormal arborization. Based on our experience, we believe that PAH-targeted therapies are effective in some patients with PAH occurri...Continue Reading

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Citations

Jul 6, 2018·Journal of the American Heart Association·Konstantinos DimopoulosMichael A Gatzoulis
Jan 12, 2020·Heart Failure Reviews·Anna S MuellerJonathan N Ginns
Mar 4, 2020·Trends in Cardiovascular Medicine·Meghan M Cirulis, Tim Lahm
May 1, 2021·Case Reports in Cardiology·Metri HaddadenDaniel Keena

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