Double-Inlet single ventricle associated with transposition of the great arteries, situs inversus, and criss-cross heart in an unrepaired adult: A case report
- 1Escuela Superior Politécnica de Chimborazo, Riobamba, Ecuador. Escuela Superior Politécnica de Chimborazo Escuela Superior Politécnica de Chimborazo Riobamba Ecuador
- 2Hospital General Puyo, Puyo, Ecuador. Hospital General Puyo Puyo Ecuador
- 3Universidad de las Américas, Quito, Ecuador. Universidad de las Américas Universidad de las Américas Quito Ecuador
Abstract
The single ventricle represents a group of complex congenital heart diseases characterized by the mixing of oxygenated and deoxygenated blood, chronic cyanosis, and ventricular volume overload. The combination of a double-inlet single ventricle with transposition of the great arteries, situs inversus, and a criss-cross heart is extremely rare, reported only in isolated cases in the literature. Most of these patients require early palliative interventions, such as the bidirectional Glenn procedure and eventual Fontan circulation, to optimize oxygenation and reduce ventricular overload. We present the case of a 26-year-old man with a history of cyanotic congenital heart disease, scheduled for surgery at age 12 but managed conservatively due to high surgical risk. Survival into adulthood without definitive surgical correction is likely due to a favorable hemodynamic balance conferred by pulmonary stenosis, which limits pulmonary overcirculation and protects the dominant ventricle. Currently, the patient remains clinically stable, classified as New York Heart Association functional class II, with no recent episodes of cardiovascular decompensation, hospitalizations, or documented thromboembolic events. This case highlights the anatomical rarity and underscores the importance of specialized follow-up in adults with complex univentricular congenital heart disease who have not undergone corrective surgery.
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BibTeX
@article{vargaslucio2026doubleinlet,
title = {{Double-Inlet single ventricle associated with transposition of the great arteries, situs inversus, and criss-cross heart in an unrepaired adult: A case report}},
author = {Brayan Vargas-Lucio and Jaime Bravo-Alvarado and Sebastián Gaibor-Gaibor and Erick Vargas-Lucio and Alex Almache-Palacios},
journal = {Archivos Peruanos de Cardiología y Cirugía Cardiovascular},
year = {2026},
month = sep,
doi = {10.47487/apcyccv.v7i3.613},
url = {https://doi.org/10.47487/apcyccv.v7i3.613},
note = {CC BY-NC 4.0},
}
RIS
TY - JOUR
TI - Double-Inlet single ventricle associated with transposition of the great arteries, situs inversus, and criss-cross heart in an unrepaired adult: A case report
AU - Brayan Vargas-Lucio
AU - Jaime Bravo-Alvarado
AU - Sebastián Gaibor-Gaibor
AU - Erick Vargas-Lucio
AU - Alex Almache-Palacios
PY - 2026
DA - 2026/09/26
JO - Archivos Peruanos de Cardiología y Cirugía Cardiovascular
DO - 10.47487/apcyccv.v7i3.613
UR - https://doi.org/10.47487/apcyccv.v7i3.613
AB - The single ventricle represents a group of complex congenital heart diseases characterized by the mixing of oxygenated and deoxygenated blood, chronic cyanosis, and ventricular volume overload. The combination of a double-inlet single ventricle with transposition of the great arteries, situs inversus, and a criss-cross heart is extremely rare, reported only in isolated cases in the literature. Most of these patients require early palliative interventions, such as the bidirectional Glenn procedure and eventual Fontan circulation, to optimize oxygenation and reduce ventricular overload. We present the case of a 26-year-old man with a history of cyanotic congenital heart disease, scheduled for surgery at age 12 but managed conservatively due to high surgical risk. Survival into adulthood without definitive surgical correction is likely due to a favorable hemodynamic balance conferred by pulmonary stenosis, which limits pulmonary overcirculation and protects the dominant ventricle. Currently, the patient remains clinically stable, classified as New York Heart Association functional class II, with no recent episodes of cardiovascular decompensation, hospitalizations, or documented thromboembolic events. This case highlights the anatomical rarity and underscores the importance of specialized follow-up in adults with complex univentricular congenital heart disease who have not undergone corrective surgery.
SN - 2708-7212
ER -