Shota Kawai¹, Takashi Kido^1,*, Yuta Teguri¹, Koji Miwa¹, Tomomitsu Kanaya¹, Yoichiro Ishii², Hisaaki Aoki², Futoshi Kayatani², Sanae Tsumura¹

Congenital Heart Disease, Vol.18, No.4, pp. 399-411, 2023, DOI:10.32604/chd.2023.042243

Abstract Objectives: We sought to determine the long-term outcomes and mortality-associated factors after systemic-to-pulmonary artery shunt (SPS) in patients with heterotaxy syndrome. Methods: We retrospectively analyzed all patients with a functional single ventricle and heterotaxy syndrome who underwent SPS at our center from January 2001 to April 2022. Results: This study involved 84 patients. Restrictive pulmonary blood flow requiring early modulation was presented in 34 (40%) patients. Compared with patients without restrictive pulmonary blood flow (N = 50 [60%]), the postnatal survival of these 34 patients was significantly lower at 10 years (log rank: p = 0.04), but the statistical significance… More >

Helena Staehler¹, Carina Hopfner², Masamichi Ono^1,*, Jürgen Hörer¹

Congenital Heart Disease, Vol.17, No.1, pp. 25-30, 2022, DOI:10.32604/CHD.2022.018366

Abstract We report a very rare case of successful intracardiac correction in a patient with heterotaxy syndrome. The cardiac malformations included dextrocardia, double outlet right ventricle, pulmonary stenosis, interrupted inferior vena cava, hemiazygos continuation and total anomalous pulmonary venous return. One-stage correction was performed. The atrial procedure consisted of intra- and extraatrial rerouting of the anomalous systemic and pulmonary venous return. The hepatic veins were detached and diverted to the left atrium via an extracardiac conduit. The correction of the double outlet right ventricle was accomplished by intraventricular redirection of the blood flow from the left ventricle to the aorta. The… More >

Christopher R. Broda, Katherine B. Salciccioli, Keila N. Lopez, Peter R. Ermis, Douglas S. Moodie, Heather A. Dickerson

Congenital Heart Disease, Vol.14, No.6, pp. 885-894, 2019, DOI:10.1111/chd.12856

Abstract Background: Heterotaxy syndrome (HS) is a condition in which the thoracoabdominal organs demonstrate an abnormal lateral arrangement and is often associated with con‐ genital heart disease (CHD). Little is known about the adult HS population with CHD.
Objective: To describe the outcomes and sociodemographics of the adult CHD popu‐ lation with HS.
Methods: Records of patients 18 years of age or older with diagnoses of both CHD and HS at Texas Children's Hospital from 1964 to 2018 were reviewed.
Results: Sixty‐two patients metinclusion criteria.Median agewas 22.7 [IQR19.6‐30.0] years; 26 (42%) were female; and 13 (21%) of patients had a gap… More >