eISSN: 1897-4295
ISSN: 1734-9338
Advances in Interventional Cardiology/Postępy w Kardiologii Interwencyjnej
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vol. 16
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Transcatheter balloon atrial septostomy in thoraco-omphalopagus conjoined twins

Gurumurthy Hiremath
Lerraughn M. Morgan
Martina Richtsfeld
Mojca Konia
Daniel Saltzman
Matthew Ambrose

Department of Pediatrics, Division of Pediatric Cardiology, University of Minnesota, Masonic Children’s Hospital, Minneapolis, MN, USA
Department of Pediatric Cardiology, Valley Children’s Hospital, Fresno, CA, USA
Department of Pediatric Anesthesia, University of Minnesota, Masonic Childrens Hospital, Minneapolis, USA
Department of Pediatric Surgery, University of Minnesota, Masonic Children’s Hospital, Minneapolis, USA
Adv Interv Cardiol 2020; 16, 2 (60): 209–212
Online publish date: 2020/06/23
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Conjoined twinning is a rare congenital anomaly with a reported incidence of 1 in 50,000-200,000 births [1, 2]. Fusion at the thoracic level (thoracopagus) is the most common variety (40% of cases) followed by the abdomen (omphalopagus, 32%) [1]. Cardiac anomalies are common in these patients and range from shared pericardium to fused atria and ventricles [1]. Congenital heart disease (CHD) is common in addition to cardiac fusion in these children [3]. Perinatal outcomes are determined by the severity of cardiac involvement. Overall survival for conjoined twins is dismal at 25% [4]. Survival after successful separation for a conjoined twin with fused hearts is even more rare, with reported survival of 10% if fused at the atrial level and 0% if fused at the ventricular level [3, 5].
Cardiac catheterization has been used in diagnosis of CHD in these patients [3]. We report a case of thoraco-omphalopagus twins with atrial fusion, where the index twin had CHD and required transcatheter balloon atrial septostomy (BAS) at 3 months of age prior to surgical separation. Balloon atrial septostomy played a key role in medical management and successful cardiac surgery.

Case report

There was prenatal diagnosis of thoraco-omphalopagus conjoined twinning of two female fetuses with union from just cranial to the manubria, to just caudal to a common umbilicus, with a shared liver but otherwise separate intraabdominal and intrathoracic contents. Fetal echocardiography and post-natal transthoracic echocardiography identified separate cardiac masses with a shared pericardial sac. In this case, the index twin (twin B) had levocardia with a levoposed cardiac apex, tricuspid atresia with dextro-transposition of the great vessels and a large bulboventricular foramen, and secundum atrial septal defect. The co-twin (twin A) had dextroversion of the heart, but otherwise normal cardiac structure. In addition, there was a small vermiform vascular communication between the right atrial appendage of the index twin and the dextroverted/anterior left atrium of the co-twin. Flow across this tiny connection could only be demonstrated consistently well in the fetal echocardiograms. The twins were born by Caesarean section at 33 + 6/7 weeks gestational age.
The hemodynamics in the conjoined pair were complex. Using a combination of transthoracic echocardiography and CT angiography, arterial communications between the twins in the hepatic artery...

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