title: Congenital heart disease and pulmonary hypertension: pharmacology and feasibility of late surgery.
abstract: Pulmonary arterial hypertension (PAH) with increased pulmonary vascular resistance (PVR), previously termed pulmonary vascular obstructive disease or pulmonary vascular disease is a frequent complication of congenital heart disease (CHD).While there have been advances in the medical treatments available for classic Eisenmenger syndrome patients who are not suitable for repair, the sub-group of patients with moderate sized congenital systemic to pulmonary shunts and mild to moderately elevated PVR remains challenging. With the development of targeted medical treatments for pulmonary arterial hypertension (PAH), the concept of a combined medical and interventional/surgical approach for patients with PAH associated with CHD (APAH-CHD) has emerged. Careful evaluation and an understanding of the predominant physiologic features will help guide the management of these complex patients and whether late surgical repair is feasible.
authors.affiliation: Columbia University College of Physicians and Surgeons, New York, NY 10032, USA. esb14@columbia.edu
authors.forename: Erika B
authors.initials: EB
authors.lastname: Rosenzweig
authors.forename: Robyn J
authors.initials: RJ
authors.lastname: Barst
mesh_terms: D006348:Cardiac Surgical Procedures / Q000379:methods*; D005240:Feasibility Studies; D006330:Heart Defects, Congenital / Q000150:complications / Q000188:drug therapy / Q000601:surgery; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000209:etiology / Q000601:surgery; D011379:Prognosis; D013997:Time Factors; D014656:Vascular Surgical Procedures / Q000379:methods*; D014665:Vasodilator Agents / Q000627:therapeutic use*
chemical_list: D014665:Vasodilator Agents