School of Medicine and Health Sciences Poster Presentations

Title

Surgical Outcomes of Patients with Heterotaxy Syndrome

Document Type

Poster

Abstract Category

Cardiology/Cardiovascular Research

Keywords

cardiology, heterotaxy, outcomes

Publication Date

Spring 5-1-2019

Abstract

Heterotaxy syndrome may be defined as a spectrum of abnormally discordant organ situs resulting from aberrant left-right axis determination in the thoracic and abdominal cavities. The abnormal degree of thoracic and abdominal visceral symmetry results in complex intracardiac abnormalities. In cases in which only one ventricle is capable of supporting the circulation, patients with heterotaxy syndrome will undergo univentricular palliation. The aim of this study is to compare the outcomes of heterotaxy syndrome patients at Children's National Hospital who received either univentricular palliation or full biventricular repair. We performed a descriptive analysis of patients with heterotaxy syndrome who underwent cardiac surgical repair at Children's National Hospital from January 2008 to December 2017. Diagnosis of heterotaxy syndrome was confirmed based on echocardiography. Patients who had undergone previous cardiac surgeries at outside hospitals were excluded from analysis. Patients were classified as either univentricular or biventricular according to their functional anatomy after cardiac repair. Univentricular patients were further divided according to stage of repair as follows: stage I (PA band, BT shunt, Norwood), stage II (Bidirectional Glenn operation, Kawashima), and stage III (Fontan operation). Data collection was performed via retrospective chart review. Seventy-nine patients with heterotaxy syndrome were included in the study (35 males, 34 females). Fifty-three patients underwent single ventricle palliation and 26 underwent complete biventricular repair. Of those receiving single ventricle palliation, 44 patients underwent stage I repair, 38 patients underwent stage II repair, and 21 patients underwent stage III repair. Genetic abnormalities were found in 22% of the cohort (17/79), occurring in 17% of univentricular patients (9/53) and 31% of biventricular patients (8/26). 30-day morality for univentricular patients at each stage was as follows: stage I=16% (7/44), stage II= 2% (1/38), stage III=0% (0/21). 30-day mortality for biventricular patients was 19% (5/26). Median follow-up days (interquartile range) from time of cardiac surgery for univentricular patients was 649 days (129-1642 days) and biventricular patients was 228 days (30-659 days). Long term survival was 66% in univentricular patients (35/53) and 62% in biventricular patients (16/26). Outcomes of heterotaxy patients undergoing cardiac surgery at Children's National Hospital are comparable to other centers. Further research of heterotaxy patients should be performed in both univentricular and biventricular groups to improve outcomes in the current era. Identifying risk factors that result in negative outcomes in this complex group should be the priority of future studies.

Open Access

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Presented at Research Days 2019.

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Surgical Outcomes of Patients with Heterotaxy Syndrome

Heterotaxy syndrome may be defined as a spectrum of abnormally discordant organ situs resulting from aberrant left-right axis determination in the thoracic and abdominal cavities. The abnormal degree of thoracic and abdominal visceral symmetry results in complex intracardiac abnormalities. In cases in which only one ventricle is capable of supporting the circulation, patients with heterotaxy syndrome will undergo univentricular palliation. The aim of this study is to compare the outcomes of heterotaxy syndrome patients at Children's National Hospital who received either univentricular palliation or full biventricular repair. We performed a descriptive analysis of patients with heterotaxy syndrome who underwent cardiac surgical repair at Children's National Hospital from January 2008 to December 2017. Diagnosis of heterotaxy syndrome was confirmed based on echocardiography. Patients who had undergone previous cardiac surgeries at outside hospitals were excluded from analysis. Patients were classified as either univentricular or biventricular according to their functional anatomy after cardiac repair. Univentricular patients were further divided according to stage of repair as follows: stage I (PA band, BT shunt, Norwood), stage II (Bidirectional Glenn operation, Kawashima), and stage III (Fontan operation). Data collection was performed via retrospective chart review. Seventy-nine patients with heterotaxy syndrome were included in the study (35 males, 34 females). Fifty-three patients underwent single ventricle palliation and 26 underwent complete biventricular repair. Of those receiving single ventricle palliation, 44 patients underwent stage I repair, 38 patients underwent stage II repair, and 21 patients underwent stage III repair. Genetic abnormalities were found in 22% of the cohort (17/79), occurring in 17% of univentricular patients (9/53) and 31% of biventricular patients (8/26). 30-day morality for univentricular patients at each stage was as follows: stage I=16% (7/44), stage II= 2% (1/38), stage III=0% (0/21). 30-day mortality for biventricular patients was 19% (5/26). Median follow-up days (interquartile range) from time of cardiac surgery for univentricular patients was 649 days (129-1642 days) and biventricular patients was 228 days (30-659 days). Long term survival was 66% in univentricular patients (35/53) and 62% in biventricular patients (16/26). Outcomes of heterotaxy patients undergoing cardiac surgery at Children's National Hospital are comparable to other centers. Further research of heterotaxy patients should be performed in both univentricular and biventricular groups to improve outcomes in the current era. Identifying risk factors that result in negative outcomes in this complex group should be the priority of future studies.