Congenital heart disease associated pulmonary arterial hypertension.
Pulmonary arterial hypertension associated with congenital heart disease, although common (15%-30%) in all-comers with congenital heart disease, is variable in terms of clinical manifestations, severity of associated pulmonary arterial hypertension, and response to therapy and outcomes (depending on lesion anatomy, pulmonary circulation flows and pressures, and presence and timings of surgeries). Evaluation includes imaging and catheterization. Surgical or another anatomic correction may be desirable after rigorous preinterventional assessment. Patients who are not surgical candidates or who fail to improve early or late after surgery may have the potential to respond to idiopathic pulmonary arterial hypertension therapies. Lung or heart/lung transplantation remains an option for selected recalcitrant patients.
Landzberg
Michael J
MJ
Boston Adult Congenital Heart and Pulmonary Hypertension Group, Children's Hospital, Brigham and Women's Hospital, Beth Israel Deaconess Medical Center, Harvard University, Boston, MA 02115-5724, USA. michael.landzberg@cardio.chboston.org
D006348:Cardiac Surgical Procedures / Q000009:adverse effects; D004541:Eisenmenger Complex / Q000188:drug therapy / Q000503:physiopathology; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications* / Q000601:surgery; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular / Q000503:physiopathology; D012307:Risk Factors; D015398:Signal Transduction / Q000502:physiology