Adolescence to Adulthood: Safely Transitioning the Adolescent With Pulmonary Arterial Hypertension

Advances in Pulmonary Hypertension

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关键词: Cardiology;    Pulmonology;    Rheumatology;    Pediatrics;    Psychosocial Issues;    Lifestyle Issues;    Social Work;    WHO Group 1- Pulmonary Arterial Hypertension;    WHO Group 3- Pulmonary hypertension owing to lung disease;    /or hypoxia;    Pulmonary Function Tests;    Surgical Interventions;    symptoms;    early childhood;    Adolescence;    Support;    Associated Diseases;    Patent Ductus Arteriosus;    bronchiolitis obliterans;    cystic fibrosis;    hematologic disorder;    Medication Adherence;    Nonadherence;    Spirometry;    Transplantation;    endothelial dysfunction;    patient-reported outcomes;    Ethics;    Education;    Classification;   

DOI  :  

学科分类：医学（综合）

来源: Pulmonary Hypertension Association

【 摘 要 】

In my experience, this patient falls into the worst category of POPH because she no longer has the classic high cardiac output state as seen in most POPH patients. With the PVR of ~ 12 WU and dysfunctional RV, I would certainly favor an IV prostacyclin to start and titrate weekly and perhaps add an endothelin receptor antagonist such as ambrisentan if hemodynamics are not improved as desired. Try to avoid a beta blocker for variceal prophylaxis. Whether liver transplantation can ever be considered is problematic at this point.

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