A Case Report: Management of Systemic RV Failure on Adult Congenitally Corrected Transposition of Great Arteries (ccTGA) Complicating Symptomatic Bradycardia with Atrial Fibrillation Slow Ventricular Response (AFSVR)

Author : M.A. Sobirin, K. Rizky,U. Bahrudin, I. Uddin
Upload Date : 19-04-2018

Background Congenitally corrected transposition of great arteries (CCTGA) is a rare congenital heart disease with a poor prognosis in adult due to systemic right ventricle (RV) failure. The physiological, anatomic and complicated characteristics of an aged symptomatic patient are described.

Case Presentation A 50-year-old male patient was referred to dr. Kariadi Hospital (RSDK) with typical symptoms and clinical of acute decompensated heart failure (ADHF) and symptomatic bradycardia with atrial fibrillation slow ventricular response (AFSVR). Patient was given intravenous diuretics to reduce congestion and emergent transvenous temporary pacemaker (TPM) was inserted to improve hemodynamics. Diagnostic echocardiography showed congenitally corrected transposition of Great Arteries (ccTGA) without other congenital cardiac defects confirmed by cardiac MSCT, accompanied by decreased systemic right ventricle (RV) function and severe regurgitation of systemic atrioventricular (AV) valve (tricuspid). During cardiac intensive care follow up, other complications were emerged; bronchopneumonia, disseminated intravascular coagulation (DIC), as well as TPM loss capture with electrocardiogram showed AFSVR rhythm. Medical treatment of systemic RV heart failure was extrapolated to LV heart failure which comprised of ACE inhibitors, diuretics, and aldosterone antagonist. Intravenous antibiotics and vitamin K injections were also administered. The patient clinically and hemodynamically improved and stable then received permanent pacemaker implantation. In future, clinical and echocardiographic monitoring should be performed routinely for evaluation of RV function, regurgitation of systemic AV valves and ultimately best treatment approach for this patient.

KEYWORDS : Adult ccTGA, RV failure, medical treatment, cardiac pacemaker

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