Congenital heart disease and pulmonary hypertension.
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2012-07
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Many patients with congenital heart disease and systemic-to-pulmonary shunts develop pulmonary arterial hypertension (PAH), particularly if the cardiac defect is left unrepaired. A persistent increase in pulmonary blood flow may lead to obstructive arteriopathy and increased pulmonary vascular resistance, a condition that can lead to reversal of shunt and cyanosis (Eisenmenger syndrome). Cardiac catheterization is crucial to confirm diagnosis and facilitate treatment. Bosentan is the only medication to date to be compared with placebo in a randomized controlled trial specifically targeting congenital heart disease-associated PAH. Lung transplantation with repair of the cardiac defect or combined heart-lung transplantation is reserved for recalcitrant cases.
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Gupta, Vedant, Adriano R Tonelli and Richard A Krasuski (2012). Congenital heart disease and pulmonary hypertension. Heart Fail Clin, 8(3). pp. 427–445. 10.1016/j.hfc.2012.04.002 Retrieved from https://hdl.handle.net/10161/11010.
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Richard Andrew Krasuski
Dr. Richard Krasuski is an Adjunct Professor of Medicine and Pediatrics at Duke University Medical Center. He was formerly the Director of the Adult Congenital Heart Center, Director of Hemodynamic Research, and the Medical Director of the CTEPH Program at Duke. He is considered a thought leader in the fields of pulmonary hypertension and congenital heart disease. His research focus is in epidemiologic and clinical studies involving patients with pulmonary hypertension and patients with congenital heart disease. He is involved in multiple multicenter studies through the Alliance for Adult Research in Congenital Cardiology (AARCC). He has also helped to develop multiple research databases in these patient populations. He is Co-PI in the EPIPHANY Study examining the impact of medical and transcatheter interventions on RV-PA coupling in patients with chronic thromboembolic pulmonary hypertension. Over his career he has mentored over 100 students, residents and fellows and has published over 350 peer reviewed articles, book chapters and meeting abstracts. He is also the Chief Editor of Advances in Pulmonary Hypertension and on the editorial boards of >20 leading medical journals. He has been an invited speaker at over 100 medical centers worldwide.
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