title: The Triply Twisted Heart: Cyanosis in an Adult With Situs Inversus, Levocardia, Double Outlet Right Ventricle, and Malposition of the Great Arteries.
abstract: We present a case of a 19-year-old female presenting with cyanosis since birth. The major anomaly demonstrated was a "triply twisted heart" with a balanced physiology, allowing her to survive into adulthood. Non-invasive imaging was done using 2D and real-time 3D (or 4D) echocardiography with multi-slice imaging from 4D volume datasets. Findings were confirmed using cardiac magnetic resonance imaging (MRI). A segmental approach revealed atrial and visceral situs inversus, levocardia, atrioventricular discordance, and ventriculoarterial discordance. Both the aorta and pulmonary artery were malposed and arise from the right ventricle (double outlet right ventricle or DORV). There was also a complete atrioventricular septal defect (CAVSD) associated with a functional single atrium and a functional univentricle (single ventricle). Other findings include a severe pulmonic stenosis (PS), preserved right and left ventricular systolic function, and a normal pulmonary arterial pressure. She also had a persistent left superior vena cava (SVC) that drains into the morphologic right atrium, while the right-sided SVC drains into the morphologic left atrium. A multidisciplinary team deemed that management be palliative. She is on regular follow-up at our clinics for non-invasive monitoring. To our knowledge, this is the first reported case in an adult with this combination of anomalies.
authors.affiliation: Section of Cardiology, Department of Medicine, University of the Philippines, Philippine General Hospital, Philippines.
authors.forename: Jaime Alfonso M
authors.initials: JA
authors.lastname: Aherrera
authors.affiliation: Section of Cardiology, Department of Medicine, University of the Philippines, Philippine General Hospital, Philippines.
authors.forename: Jose Donato A
authors.initials: JD
authors.lastname: Magno
authors.affiliation: Section of Cardiology, Department of Medicine, University of the Philippines, Philippine General Hospital, Philippines.
authors.forename: Celia Catherine C
authors.initials: CC
authors.lastname: Uy
authors.affiliation: Section of Cardiology, Department of Medicine, University of the Philippines, Philippine General Hospital, Philippines.
authors.forename: Lauro L
authors.initials: LL
authors.lastname: Abrahan
authors.affiliation: Department of Cardiology, St. Luke's Medical Center, Global City, Philippines.
authors.forename: Helga F Sta
authors.initials: HF
authors.lastname: Maria
authors.affiliation: Section of Cardiology, Department of Medicine, University of the Philippines, Philippine General Hospital, Philippines.
authors.forename: Rodel R
authors.initials: RR
authors.lastname: Buitizon
authors.affiliation: Section of Cardiology, Department of Medicine, University of the Philippines, Philippine General Hospital, Philippines.
authors.forename: Raul D
authors.initials: RD
authors.lastname: Jara
keywords: Atrioventricular septal defect; Malposition of the great arteries; Situs inversus