A Misdiagnosed Case of Double Outlet Right Atrium Associated With Hypoplastic Right Ventricle

Irene Raso; Andrea Quarti; Massimo Chessa; Domenica Paola Basile; Antonio Saracino; Angelo Fabio D’Aiello; Claudia Raineri; Mario Carminati; Alessandro Giamberti

doi:10.1177/2150135119894292

A Misdiagnosed Case of Double Outlet Right Atrium Associated With Hypoplastic Right Ventricle

Irene Raso, Andrea Quarti, Massimo Chessa, Domenica Paola Basile, Antonio Saracino, Angelo Fabio D’Aiello, Claudia Raineri, Mario Carminati, Alessandro Giamberti

Houston Methodist

Research output: Contribution to journal › Article › peer-review

Abstract

Double outlet right atrium (DORA) is a rare congenital heart disease in which the right atrium opens into both ventricles. The reduced leftward motion of the interventricular septum causes a malalignment between the atrial and the ventricular septum at the cardiac crux, which is the pathognomonic feature of this heart defect. We describe a case of significant exertional desaturation in an adult patient who was diagnosed with DORA, restrictive right ventricle, and anomalous tricuspid valve. Subsequently, the patient underwent one-and-a-half ventricular palliation.

Original language	English (US)
Pages (from-to)	358-360
Number of pages	3
Journal	World Journal for Pediatric and Congenital Heart Surgery
Volume	11
Issue number	3
DOIs	https://doi.org/10.1177/2150135119894292
State	Published - May 1 2020

ASJC Scopus subject areas

Surgery
Pediatrics, Perinatology, and Child Health
Cardiology and Cardiovascular Medicine

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10.1177/2150135119894292

Cite this

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title = "A Misdiagnosed Case of Double Outlet Right Atrium Associated With Hypoplastic Right Ventricle",

abstract = "Double outlet right atrium (DORA) is a rare congenital heart disease in which the right atrium opens into both ventricles. The reduced leftward motion of the interventricular septum causes a malalignment between the atrial and the ventricular septum at the cardiac crux, which is the pathognomonic feature of this heart defect. We describe a case of significant exertional desaturation in an adult patient who was diagnosed with DORA, restrictive right ventricle, and anomalous tricuspid valve. Subsequently, the patient underwent one-and-a-half ventricular palliation.",

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T1 - A Misdiagnosed Case of Double Outlet Right Atrium Associated With Hypoplastic Right Ventricle

AU - Raso, Irene

AU - Quarti, Andrea

AU - Chessa, Massimo

AU - Basile, Domenica Paola

AU - Saracino, Antonio

AU - D’Aiello, Angelo Fabio

AU - Raineri, Claudia

AU - Carminati, Mario

AU - Giamberti, Alessandro

PY - 2020/5/1

Y1 - 2020/5/1

N2 - Double outlet right atrium (DORA) is a rare congenital heart disease in which the right atrium opens into both ventricles. The reduced leftward motion of the interventricular septum causes a malalignment between the atrial and the ventricular septum at the cardiac crux, which is the pathognomonic feature of this heart defect. We describe a case of significant exertional desaturation in an adult patient who was diagnosed with DORA, restrictive right ventricle, and anomalous tricuspid valve. Subsequently, the patient underwent one-and-a-half ventricular palliation.

AB - Double outlet right atrium (DORA) is a rare congenital heart disease in which the right atrium opens into both ventricles. The reduced leftward motion of the interventricular septum causes a malalignment between the atrial and the ventricular septum at the cardiac crux, which is the pathognomonic feature of this heart defect. We describe a case of significant exertional desaturation in an adult patient who was diagnosed with DORA, restrictive right ventricle, and anomalous tricuspid valve. Subsequently, the patient underwent one-and-a-half ventricular palliation.

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A Misdiagnosed Case of Double Outlet Right Atrium Associated With Hypoplastic Right Ventricle

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