@inproceedings{8758517,
  abstract     = {{Cardiac isomerism or ‘isomerism of the atrial appendage’ is found in about 0,4-2% of congenital heart defects and is associated with a complex spectrum of anomalies. We reviewed isomerism cases from the last 20 years at a single institution.

Methods
Retrospective review of 48 patient records (Jan 2000-Dec 2020). Case selection was based on echocardiographic findings suggesting cardiac isomerism. Definitive diagnosis was made post-mortem or peri-operatively by identifying symmetrical morphological left or right atrial appendages.

Results
Left atrial isomerism (LAI) was diagnosed in 24 (10 confirmed), right atrial isomerism (RAI) in 24 cases (18 confirmed). Prenatal diagnosis was made in 33 cases (17 LAI; 16 RAI). Unconfirmed cases consist of 5 terminations of pregnancy were no autopsy was performed and 15 live-born patients who did not undergo cardiac surgery or autopsy.

In LAI, 21 cases (87,5%) have anomalous systemic venous return, being the only cardiovascular abnormality present in 10 patients. Conduction or rhythm abnormalities were present in 11 cases (45,8%). In RAI, only complex univentricular heart defects were found with a large proportion of AVSD, transposition of the great arteries and pulmonary stenosis/atresia. Splenic function is disturbed in 86% of all live-born patients. While other extra-cardiac defects are more incidental, malrotation is more prevalent in this cohort (>10%) than in the general population.

After exclusion of termination of pregnancy (n=11) and patients lost to follow-up (n=2), overall survival for isomerism patients is 60%, with a median time to follow-up of 9y. Compared to RAI, survival rates in patients with LAI are much higher:  84% (median follow-up 6y) compared to 31% (median follow-up 12ys). However, for those LAI patients that need univentricular palliation (n=3), survival rates are low (33%) and similar to RAI. Main causes of death are primary cardiac failure, abstinence of life-prolonging care and severe sepsis. 

Conclusion
Survival rates for patients with cardiac isomerism are low for those who need univentricular palliation. These results can be used when counseling parents. Additional pathology like (functional) asplenia and malrotation add to the burden of disease.}},
  author       = {{Mets, Gilles and Bové, Thierry and De Wilde, Hans and Francois, Katrien and Martens, Thomas and Muiño Mosquera, Laura and Panzer, Joseph and Roets, Ellen and Van Oostrum, Noortje and Vandekerckhove, Kristof and De Groote, Katya}},
  booktitle    = {{AEPC Annual meeting 2022, Abstracts}},
  keywords     = {{Isomerism}},
  language     = {{eng}},
  location     = {{Geneva, Switzerland}},
  title        = {{Cardiac isomerism : a 20 year retrospective cohort at a single institution}},
  year         = {{2022}},
}