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The multisystem nature of isomerism: left isomerism complicated by Abernethy malformation and portopulmonary hypertension

Part of: Ventricular Septal Defect (VSD) Cardiac Morphology

Published online by Cambridge University Press:  18 March 2021

Megan L. Ringle Open the ORCID record for Megan L. Ringle [Opens in a new window] ,
Rohit Loomba Open the ORCID record for Rohit Loomba [Opens in a new window] ,
John C. Dykes ,
Danyal Khan Open the ORCID record for Danyal Khan [Opens in a new window] ,
David Schidlow  and
Gil Wernovsky
Megan L. Ringle*
Affiliation:
Division of Neonatal and Developmental Medicine, Lucile Packard Children’s Hospital, Stanford University School of Medicine, Palo Alto, CA, USA
Rohit Loomba
Affiliation:
Division of Cardiology, Advocate Children’s Hospital, Chicago, IL, USA Division of Cardiology, Chicago Medical School, Chicago, IL, USA
John C. Dykes
Affiliation:
Division of Cardiology, Lucile Packard Children’s Hospital, Stanford Uiversity School of Medicine, Palo Alto, CA, USA
Danyal Khan
Affiliation:
Department of Pediatric Cardiology, Nicklaus Children’s Hospital, Miami, FL, USA
David Schidlow
Affiliation:
Department of Cardiology, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA
Gil Wernovsky
Affiliation:
Divisions of Cardiac Critical Care and Pediatric Cardiology, Children’s National Hospital, Washington, DC, USA
*
Author for correspondence: M. L. Ringle, MD, MPH, Division of Neonatal and Developmental Medicine, Lucile Packard Children’s Hospital, Stanford University School of Medicine, 750 Welch Rd, Suite 315, Palo Alto, CA 94304, USA. Tel: 650-724-9954; Fax: 650-725-6581. E-mail: meganringle@gmail.com
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Abstract

Isomerism, also referred to as “heterotaxy” is a complex set of anatomic and functional perturbations. One of the most obvious manifestations of isomerism is the disturbance of organ arrangement, such that the thoracic organs are no longer asymmetric on the left and right. We report the case of a 14-year-old female in whom exercise-induced dyspnea led to a late diagnosis of left isomerism complicated by Abernethy malformation and portopulmonary hypertension. A comprehensive evaluation revealed two anatomic left lungs and hyparterial bronchi, bilateral left atria, an interrupted inferior caval vein with azygos continuation, multiple spleens, sinus node dysfunction, hepatic hypertrophy with focal nodular hyperplasia, and absence of the portal vein. Pulmonary vasodilator therapy was initiated resulting in clinical improvement. This case exhibits unique features including a late diagnosis of isomerism with Abernethy malformation and portopulmonary hypertension. The patient’s presentation, medical workup, and future treatment emphasise the importance of multidisciplinary care in children with complex multisystem disease. We review the multiple cardiac and extracardiac manifestations of isomerism.

Keywords

Isomerismheterotaxyatrial isomerismAbernethy syndrome
Type
Review
Information
Cardiology in the Young , Volume 31 , Issue 4 , April 2021 , pp. 532 - 540
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Copyright
© The Author(s), 2021. Published by Cambridge University Press

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