Abstract

Within the last few decades the pre-natal echocardiographic diagnosis of congenital heart defects has made substantial progresses, particularly for the identification of complex malformation. “Functionally” univentricular hearts categorize a huge variety of heart malformations. Since no one of the patients with these congenital heart defects can ever undergo a bi-ventricular type of repair, early recognition and decision-making from the neonatal period are required in order to allow for appropriate multiple-step diagnostic and treatment procedures, either of interventional cardiology and/or surgery, on the pathway of “univentricular” heart. In the literature strong disagreements exist about the potential impact of the pre-natal diagnosis on the early and late outcomes of complex congenital heart defects. This review of the recent reports has been undertaken to better understand the impact of pre-natal diagnosis in “functionally” univentricular hearts taking into consideration the following topics: pre-natal screening, outcomes and survival, general morbidity, neurologic and developmental consequences, pregnancy management and delivery planning, resources utilization and costs/benefits issues, ethical implications, parents counseling, and interruption of pregnancy versus treatment.

Highlights

  • Since no one of the patients with these congenital heart defects can ever undergo a bi-ventricular type of repair, early recognition and decision-making from the neonatal period are required in order to allow for appropriate multiple-step diagnostic and treatment procedures, either of interventional cardiology and/or surgery, on the pathway of “univentricular” heart

  • In the literature strong disagreements exist about the potential impact of the pre-natal diagnosis on the early and late outcomes of complex congenital heart defects

  • LITERATURE REVIEW The reported studies have been analyzed, taking into consideration the impact of the pre-natal diagnosis on the following topics: “Functionally” univentricular hearts categorize a huge variety of heart malformations, including but not limited to mitral atresia, tricuspid atresia, hypoplastic right or left heart, pulmonary atresia with intact ventricular septum and hypoplastic right ventricle, aortic atresia, double outlet right ventricle with hypoplastic right or left ventricle, complete atrio-ventricular septal defect with unbalanced ventricles, double inlet right or left ventricle

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Summary

Antonio Francesco Corno*

Reviewed by: Yves Durandy, Centre Chirurgical Marie Lannelongue, France Federico Gutierrez-Larraya, Hospital Universitario La Paz, Spain. Within the last few decades the pre-natal echocardiographic diagnosis of congenital heart defects has made substantial progresses, for the identification of complex malformation. “Functionally” univentricular hearts categorize a huge variety of heart malformations. In the literature strong disagreements exist about the potential impact of the pre-natal diagnosis on the early and late outcomes of complex congenital heart defects. This review of the recent reports has been undertaken to better understand the impact of pre-natal diagnosis in “functionally” univentricular hearts taking into consideration the following topics: pre-natal screening, outcomes and survival, general morbidity, neurologic and developmental consequences, pregnancy management and delivery planning, resources utilization and costs/benefits issues, ethical implications, parents counseling, and interruption of pregnancy versus treatment

INTRODUCTION
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