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Cardiovascular Disease |

Double Inlet Single Ventricle With Transposition of the Great Arteries and Situs Inversus With Dextrocardia in an Adult Without Surgical Correction: Challenges and Triumphs

Ntesi Asimi, MD; Jane Wilcox, MD; Sherif Afifi, MD
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Northwestern University, Chicago, IL


Chest. 2015;148(4_MeetingAbstracts):51A. doi:10.1378/chest.2263884
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Abstract

SESSION TITLE: Cardiovascular Disease Case Report Posters

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Single ventricle abnormality represents approximately 1% of all congenital cardiac defects, with an incidence of 0.05-0.1:10’000 live births [1]. With current medical and surgical advances, more patients are surviving to adulthood. Acute decompensation in adults is a common cause of death. New corrective approaches should be entertained to support the failing single ventricular hearts [2, 3]

CASE PRESENTATION: We present a case of a 61 year old female with unrepaired cyanotic congenital heart disease. Her defect is significant for double inlet single ventricle with left looping and transposition of great arteries and situs inversus with dextrocardia, with somewhat “protected” pulmonary bed secondary to pulmonary stenosis (PS). The patient has done well, despite developing atrial fibrillation and worsening atrioventricular valve regurgitation and complete heart block. TEE confirmed well preserved systolic function with EF 54%, severe PS and severe biatrial enlargement. Right and Left catheterization notable for within normal limits coronary flow and high normal pulmonary artery pressures. Cardiopulmonary testing is significant for abnormal cardiovascular response - inability to augment stroke volume and increased oxygen consumption with submaximal exercise response. Multidisciplinary decision was made to proceed with epicardial pacemaker (EPM). After uneventful minimal inferior medial sternotomy approach for placement and uncomplicated CTICU recovery our patient is undergoing evaluation for orthotopic heart transplant

DISCUSSION: The goal of EPM placement was to improve the patient’s choronotropic incompetence and respectively functional capacity. After initial post-operative recovery, patient noted improved functional status and oxygen saturation in high to low 70s

CONCLUSIONS: The uncorrected complex adult cyanotic cardiac pathology offers multifactorial array of challenges to the treating team. Understanding of the anatomy and pathological complications, as well as the physiological adaptation of each patient’s condition, is essential for the optimum management of these intricate disorders

Reference #1: EK Steinberger,C Ferencz,CA Loffredo.Infants With Single Ventricle:A Population-Based Epidemiological Study.Teratology.2002;65:103-115

Reference #2: Rossano JW,Chang AC.Perioperative management of patients with poorly functioning ventricles in the setting of the functionally univentricular heart.Cardiol Young.2006;16 (S1):47-54

Reference #3: H Vyas, DJ Hagler.Double Inlet Left Ventricle.Cur Treat Opt Card Med.2007;9:391-398

DISCLOSURE: The following authors have nothing to disclose: Ntesi Asimi, Jane Wilcox, Sherif Afifi

No Product/Research Disclosure Information


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