A Multifaceted Approach to Pulmonary Hypertension in Adults With Congenital Heart Disease.

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2018-09

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Abstract

Advances in the management of congenital heart disease (CHD) in children have resulted in growing numbers of adults with CHD. Pulmonary arterial hypertension related to CHD (PAH-CHD) is a common complication, affecting up to 10% of patients; and can arise even after successful and complete defect repair, with severe and potentially fatal consequences. Careful work-up in these patients is essential, particularly hemodynamic assessment, and can help define the most appropriate therapeutic approach. Management can be challenging, but the therapeutic armamentarium is continually expanding and now includes surgical, transcatheter and medical options. Timely correction of defects along with early treatment with advanced medical therapies appears to improve quality of life and possible even improve survival. Interestingly most studies of PAH-CHD have focused on its most severely afflicted patients, those with Eisenmenger Syndrome, making it less certain how to manage PAH-CHD of milder degrees. This review summarizes our current understanding of PAH-CHD and emphasizes the need for close follow-up in specialized centers of care where close collaboration is common practice.

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10.1016/j.pcad.2018.07.017

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Fathallah, Mouhammad, and Richard A Krasuski (2018). A Multifaceted Approach to Pulmonary Hypertension in Adults With Congenital Heart Disease. Progress in cardiovascular diseases, 61(3-4). pp. 320–327. 10.1016/j.pcad.2018.07.017 Retrieved from https://hdl.handle.net/10161/17950.

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Krasuski

Richard Andrew Krasuski

Professor of Medicine

Dr. Richard Krasuski is Director of the Adult Congenital Heart Center at Duke University Medical Center, the Director of Hemodynamic Research, and the Medical Director of the CTEPH Program. 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 upcoming 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 80 students, residents and fellows and has published over 300 peer reviewed publications, book chapters and meeting abstracts. He is also the Chief Editor of Advances in Pulmonary Hypertension and on the editorial boards of several leading medical journals.


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