CRISSCROSS HEART WITH TRANSPOSITION: PHYSIOLOGICALLY UNCROSSED

TOPIC: Cardiovascular Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Transposition of the great arteries (TGA) is characterized by ventriculo-arterial discordance. Crisscross atrioventricular valves (CCAV) are complex arrangements with streaming venous inflows without mixing into superior-inferior ventricles. Rarely do patients age well early definitive surgery. The Rastelli operation closes ventricular septal defect (VSD) via intracardiac baffle that directs blood from left ventricle to aorta; an extracardiac conduit right pulmonic artery (RV-PA) also placed [1]. CASE PRESENTATION: A 39 year old female d-TGA, large outlet VSD, secundum atrial septum (ASD) and superiorly-inferiorly oriented ventricles CCAV underwent pulmonary band (PAB) at 1 but no other interventions. Prior (with #26 synergraft hybrid #22 RV-PA conduit) closure ASD, she was NYHA FC 3-4, had persistent hypertension (PH) (transpulmonary gradient 30 mm Hg/ PVR 6.2 Woods units on selexipag, tadalafil oxygen; desaturated a 6 minute walk test (6MWT) 71% 40%) systemic RV pressure, chronic cyanosis, pedal edema exercise intolerance. She operating room outflow tract obstruction, mild stenosis residual shunting visualized. Post-op, remained cyanotic oxygen PH subjectively improved energy edema. found have multiple small muscular VSDs after discharge transcatheter saturation energy. Now 41, her most recent 6MWT 90% air. DISCUSSION: Delayed surgical palliations for congenital heart disease high risk procedures being major factor. With despite PAB delayed repair restore septated technique then closed technique, performed in unusual plane interventricular septum. Improved saturation, tolerance quality life noted off macitentan. Her higher level functioning remarkable given patients' status thought depend underlying hemodynamic outcomes rather than anatomic details let alone amide complexity associated staged TGA [2]. While still limits her, palliation ultimately imbued objective subjective improvements this patient. CONCLUSIONS: Invasive high-risk can be lead significant improvements. Further investigations late interventions those warranted. REFERENCE #1: GC. new approach "anatomic" transposition arteries. Mayo Clin Proc 1969;44: 1–12. #2: Hoffman P, Szymanski Lubiszewska B, Rózanski J, Lipczynska M, Klisiewicz A. hearts adults: echocardiographic evaluation natural history. J Am Soc Echocardiogr. 2009 Feb;22(2):134-40. DISCLOSURES: No relevant relationships David Briston, source=Web Response KC Chan, Jeffy Jacob, Todd Roth, Frank Scholl,