CC BY-NC-ND 4.0 · AJP Rep 2019; 09(02): e121-e126
DOI: 10.1055/s-0038-1677480
Case Report
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Prenatal Diagnosis and Successful Palliation of Absent Aortic Valve with Hypoplastic Left Heart Syndrome: A Case Report and Review of Literature

1  Department of Pediatrics, University of Texas Medical Branch, Galveston, Texas
,
Chelsea B. Johnson
1  Department of Pediatrics, University of Texas Medical Branch, Galveston, Texas
,
Muhammad A. Aly
1  Department of Pediatrics, University of Texas Medical Branch, Galveston, Texas
,
2  Division of Pediatric Cardiology, University of Texas Medical Branch, Galveston, Texas
› Author Affiliations
Further Information

Publication History

12 September 2018

02 December 2018

Publication Date:
09 April 2019 (online)

Abstract

Introduction Congenital absence of the aortic valve leaflets is a rare association with hypoplastic left heart syndrome (HLHS).

Case A 37-year-old pregnant woman was referred for fetal evaluation of possible HLHS at 22 weeks of gestation. The fetal echocardiogram (ECHO) was remarkable for a hypoplastic left atrium, nearly atretic mitral valve, small left ventricle, and a hypoplastic aortic valve with severe aortic insufficiency. A female infant was born at term and postnatal ECHO confirmed the above findings. In addition, there was complete absence of the aortic valve leaflets. The patient underwent Norwood's procedure at day 5 of life with atrial septectomy, over-sewing of the aortic valve annulus, and a 4 mm Sano's shunt between the right ventricle and the main pulmonary artery. She tolerated this surgery well and subsequently underwent a bidirectional Glenn's procedure at 8 months of life.

Conclusion Prenatal diagnosis of absent aortic valve should be suspected in the presence of severe aortic insufficiency in the fetal ECHO. Early postnatal intervention is critical as those patients are likely to deteriorate quickly. The over-sewing of the aortic valve may be important to prevent coronary steal and myocardial hypoperfusion which could potentially be detrimental.

Ethical Approval

This article does not contain any studies with human participants performed by any of the authors.


Informed Consent

Informed consent was waived for this study since no identifying information was included.