Echocardiographic Screening for Pulmonary Hypertension in Congenital Heart Disease: JACC Review Topic of the Week

Konstantinos Dimopoulos, Robin Condliffe, Robert M R Tulloh, Paul Clift, Rafael Alonso-Gonzalez, Radwa Bedair, Natali A Y Chung, Gerry Coghlan, Samantha Fitzsimmons, Alessandra Frigiola, Luke S Howard, Petra Jenkins, Damien Kenny, Wei Li, Simon T MacDonald, Colm McCabe, James J Oliver, Mark S Spence, Gergely V Szantho, Kate von Klemperer, Dirk G Wilson, Stephen J Wort
Journal of the American College of Cardiology 2018 December 4, 72 (22): 2778-2788
Echocardiography is the mainstay in screening for pulmonary hypertension (PH). International guidelines suggest echocardiographic parameters for suspecting PH, but these may not apply to many adults with congenital heart disease (ACHD). PH is relatively common in ACHD patients and can significantly affect their exercise capacity, quality of life, and prognosis. Identification of patients who have developed PH and who may benefit from further investigations (including cardiac catheterization) and treatment is thus extremely important. A systematic review and survey of experts from the United Kingdom and Ireland were performed to assess current knowledge and practice on echocardiographic screening for PH in ACHD. This paper presents the findings of the review and expert statements on the optimal approaches when using echocardiography to assess ACHD patients for PH, with particular focus on major subgroups: patients with right ventricular outflow tract obstruction, patients with systemic right ventricles, patients with unrepaired univentricular circulation, and patients with tetralogy of Fallot with pulmonary atresia.

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Lauren Shearer

A good overview of the complex interaction between anatomy and echocardiography. A high degree of prior knowledge is nessecary and a background in congenital heart disease essential to interpret these tests; nonetheless the paper supports the practice of adapting PH screening to the patient, and advocates the appropriate considerations


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