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Published on: September 5, 2011
Conjoined hearts in thoracopagus twins
R Thomas Collins1, Paul M Weinberg, Peter J Gruber
1Division of Cardiology, Arkansas Children's Hospital, Little Rock, AR 72202, USA. rtcollins@uams.edu
Insights
Separating conjoined hearts in thoracopagus twins is extremely difficult due to severe cardiac abnormalities. Most conjoined twins have major congenital heart disease, often with single ventricles, leading to poor outcomes and suggesting palliative care.
Area of Science:
- Cardiology
- Pediatric Surgery
- Medical Genetics
Background:
- Conjoined twins present complex surgical challenges, particularly when sharing cardiac structures.
- Thoracopagus twins with conjoined hearts require specialized evaluation to determine surgical feasibility.
Purpose of the Study:
- To identify cardiac abnormalities in conjoined twins that impede successful surgical separation.
- To analyze the outcomes of thoracopagus conjoined twins with conjoined hearts.
Main Methods:
- Retrospective review of consecutive thoracopagus conjoined twins with conjoined hearts.
- Analysis of autopsy, surgical findings, and clinical reports from 1980-2008.
- Evaluation of cardiac anatomy, including presence of major congenital heart disease and single-ventricle physiology.
Main Results:
- Nine sets of conjoined twins were reviewed (mean gestational age 33.8 weeks).
- Major congenital heart disease affected 94.4% of hearts; 72.2% had single-ventricle physiology.
- Total anomalous pulmonary venous return occurred in 39% of cases, with high mortality.
Conclusions:
- The inability to separate conjoined and single ventricles results in a poor clinical outcome for thoracopagus twins.
- Surgical nonintervention and palliative care should be strongly considered for these complex cases.
- Understanding specific cardiac structural abnormalities is crucial for managing conjoined twins.
Abstract:
This study aimed to identify the anatomic and pathologic structural cardiac abnormalities in conjoined twins and to focus on those that have prevented the successful separation of conjoined hearts. A retrospective review was undertaken to examine consecutive cases of thoracopagus conjoined twins with conjoined hearts evaluated at The Children's Hospital of Philadelphia from 1 January 1980 through 6 October 2008. The records included autopsy and surgical findings as well as clinical reports. The study group included nine sets of conjoined twins with a mean gestational age at birth of 33.8 ± 5.5 weeks. Three twin pairs were stillborn. Five twin pairs died afterward. One pair died of cardiopulmonary failure. The median age at death was 22 days (range, 0-345 days). Major congenital heart disease was present in 94.4% (17/18) of the hearts, and 72.2% (13/18) of the hearts had single-ventricle physiology. Total anomalous pulmonary venous return occurred in 39% (7/18) of the cases. The clinical outcome for thoracopagus twins with conjoined hearts remains poor because of inability to separate conjoined and single ventricles. Surgical nonintervention and palliative care should be strongly considered for these patients.
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