Tricuspid valve dysplasia with severe tricuspid regurgitation: Fetal pulmonary artery size predicts lung viability in the presence of small lung volumes

A. T. Nathan, B. S. Marino, T. Dominguez, S. Tabbutt, S. Nicolson, D. D. Donaghue, T. L. Spray, J. Rychik

Research output: Contribution to journalArticlepeer-review

7 Scopus citations

Abstract

Congenital tricuspid valve disease (Ebstein's anomaly, tricuspid valve dysplasia) with severe tricuspid regurgitation and cardiomegaly is associated with poor prognosis. Fetal echocardiography can accurately measure right atrial enlargement, which is associated with a poor prognosis in the fetus with tricuspid valve disease. Fetal lung volumetric assessments have been used in an attempt to predict viability of fetuses using ultrasonogram and prenatal MRI. We describe a fetus with tricuspid dysplasia, severe tricuspid regurgitation, right atrial enlargement and markedly reduced lung volumes. The early gestational onset of cardiomegaly with bilateral lung compression raised the possibility of severe lung hypoplasia with decreased broncho-alveolar development. Use of fetal echocardiography with measurement of pulmonary artery size combined with prenatal MRI scanning of lung volumes resulted in an improved understanding of this anomaly and directed the management strategy towards a successful Fontan circulation.

Original languageEnglish (US)
Pages (from-to)101-105
Number of pages5
JournalFetal Diagnosis and Therapy
Volume27
Issue number2
DOIs
StatePublished - Mar 2010

Keywords

  • Lung volumes
  • Pulmonary artery size
  • Tricuspid dysplasia

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Embryology
  • Radiology Nuclear Medicine and imaging
  • Obstetrics and Gynecology

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