What is the Survival Rate in Prenatally Detected Cantrell’s Pentalogy?

Author:

Pośpiech-Gąsior Katarzyna1,Słodki Maciej23,Respondek-Liberska Maria24

Affiliation:

1. 1. Perinatology Clinic of the Collegium Medicum, Jagiellonian University, Krakow

2. 2. Department of Prenatal Cardiology, Polish Mother’s Memorial Hospital Research Institute, Lodz, Poland

3. 3. Institute of Health Sciences, The State School of Higher Professional Education in Płock, Poland

4. 4. Medical University of Lodz, Department of Diagnoses and Prevention Fetal Malformations

Abstract

Abstract Cantrell’s pentalogy is a congenital defect characterized by uncompleted fusion of the anterior chest wall, resulting in an extrathoracic location of the heart. Ultrasound diagnosis during the first trimester of prenatal life is possible, and termination of pregnancy is usually chosen by pregnant women. We analysed 57 fetuses: 56 from literature and one additional recent case from our institute (from 2016) to evaluate what was the survival rate reported after prenatal diagnosis, including the possibility to terminate the pregnancy, intrauterine deaths and neonatal deaths. We found 10 survivors - 18% since 1984. Despite dismal prognosis of fetal ectopia cordis, there is a chance for postnatal survivorship probably due to evolving anatomical structures, not only in the first trimester of pregnancy but also during the following weeks of prenatal life.

Publisher

Walter de Gruyter GmbH

Subject

General Medicine

Reference31 articles.

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3. 3. Khoury MJ, Cordero JF, Rasmussen S. Ectopia cordis, midline defects and chromosome abnormalities: an epidemiologic perspective. Am J Med Genet. 1988;30:811–817.

4. 4. Respondek-Liberska M, Janiak K, Kaczmarek P, Borowski D, Czichos E. The significance of cardiography in the diagnosis of cardiac ectopia of the fetus. Ginekol Pol. 1998;69:139-144. Polish.

5. 5. Kaplan LC, Matsouka R, Gilbert EF, Opitz JM, Kurnit DM. Ectopia cordis and cleft sternum: evidence for mechanical teratogenesis following rupture of the chorion or yolk sac. Am J Med Genet. 1985;21:187–202.

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