Closing Arguments - Holding Court in PH: Should All Adult Fontan Patients Be Placed on PAH Group 1 Therapy?


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Patients with congenital heart disease are increasingly surviving into adulthood. For patients with single ventricle anatomy, pulmonary blood flow is dependent on central venous pressure with little or no ventricular support. Patients who have undergone Fontan surgery and progress to adulthood frequently develop increased pulmonary vascular resistance, which impacts ventricular function in a precapillary pulmonary hypertension phenotype. This court case will explore the central question should these patients be placed on Group 1 vasodilator therapy. This pro/con debate will place you, the audience, in the role of the juror to decide the outcome. Evidence is presented, and expert witnesses queried the pluses and minuses of this therapeutic approach to maintaining physical function and quality of life in Fontan patients.

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