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

Amna Qasim, Chelsea B. Johnson, Muhammad A. Aly, Ashraf Aly

Research output: Contribution to journalReview article

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.

Original languageEnglish (US)
Pages (from-to)E121-E126
JournalAJP Reports
Volume9
Issue number2
DOIs
StatePublished - Jan 1 2019

Fingerprint

Hypoplastic Left Heart Syndrome
Prenatal Diagnosis
Aortic Valve
Heart Ventricles
Norwood Procedures
Fontan Procedure
Heart Atria
Mitral Valve
Pulmonary Artery
Pregnant Women
Pregnancy

Keywords

  • absent aortic valve
  • fetal echocardiography (ECHO)
  • hypoplastic left heart syndrome

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Obstetrics and Gynecology

Cite this

Prenatal Diagnosis and Successful Palliation of Absent Aortic Valve with Hypoplastic Left Heart Syndrome : A Case Report and Review of Literature. / Qasim, Amna; Johnson, Chelsea B.; Aly, Muhammad A.; Aly, Ashraf.

In: AJP Reports, Vol. 9, No. 2, 01.01.2019, p. E121-E126.

Research output: Contribution to journalReview article

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N2 - 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.

AB - 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.

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