DOI: 10.5176/2382-5669_CCMR13.31
Authors: Jerelyn Adviento MD, Angelo Dave Javier MD, Raul Jara MD, Nelson Abelardo MD
Abstract:
Pulmonary Artery Hypertension (PAH) in Adult Congenital Heart Disease is a debilitating and progressive disease, which leads to decreased functional capacity, right ventricular failure, and is often associated with early mortality. This cross sectional study sought to determine the prevalence of PAH among Adult Congenital Heart Disease patients seen at the Philippine General Hospital Congenital Heart Disease ( PATCHED) Out-Patient Clinic from August 2011- January 2013. To describe their clinical, echocardiographic and management profiles. All patients > 18 years old, diagnosed with Congenital Heart Disease, Repaired or unrepaired with clinical and echocardiographic signs of PAH, with or without overt signs of heart failure were included. Among a total of 95 ACHD patients, 56{6e6090cdd558c53a8bc18225ef4499fead9160abd3419ad4f137e902b483c465} (n=54) have PAH. 22{6e6090cdd558c53a8bc18225ef4499fead9160abd3419ad4f137e902b483c465} have eisenmengerization. Mean age was 33 (Range: 19-78years old) with female predominance of 69{6e6090cdd558c53a8bc18225ef4499fead9160abd3419ad4f137e902b483c465}. Unrepaired atrial septal defect ( 42{6e6090cdd558c53a8bc18225ef4499fead9160abd3419ad4f137e902b483c465}) was the most frequent etiology. Most are symptomatic with 13{6e6090cdd558c53a8bc18225ef4499fead9160abd3419ad4f137e902b483c465} of the patients under NYHA III – IV. The mean pulmonary artery pressure was 51 mmhg by transthoracic echocardiography. 21{6e6090cdd558c53a8bc18225ef4499fead9160abd3419ad4f137e902b483c465} showed RV dilatation and dysfunction. Digoxin, diuretics and oxygen were the mainstay therapy. Use of Sildenafil were noted among 5{6e6090cdd558c53a8bc18225ef4499fead9160abd3419ad4f137e902b483c465} of the patients. Only 9{6e6090cdd558c53a8bc18225ef4499fead9160abd3419ad4f137e902b483c465} of the patients underwent cardiac surgery. This data showed that the prevalence of PAH in Adult congenital heart disease is rising. Patients with unrepaired defects are the most at risk of its complications. These patients are mostly confined to conservative medical therapy as the use of new drugs are limited due to high cost and applicability. At present there is no standardized treatment for this condition and a general policy of non- intervention to avoid destabilization of the balanced physiology is recommended
Keywords: pulmonary artery hypertension, adult congenital heart disease
Updating...