Tetralogy of Fallot with absent pulmonary valve—When the ductus is present: A case of isolated branch pulmonary artery and review of literature

Sruti Rao, Hani K. Najm, Robert D. Stewart, Munir Ahmad, Francine Erenberg, Malek Yaman

Research output: Contribution to journalArticlepeer-review

Abstract

Tetralogy of Fallot/Absent Pulmonary Valve (TOF/APV) has been classically associated with the absence of a patent ductus arteriosus (PDA). We present a rare case of APV in TOF with a discontinuous left pulmonary artery (LPA) that was suspected during fetal echocardiogram. Postnatal echocardiogram confirmed the origin of a hypoplastic LPA from the PDA. Despite an aneurysmal (right pulmonary artery) (RPA), axial imaging demonstrated widely patent tracheobronchial system with no evidence of bronchial compression. Clinically, the child required only minimal respiratory support. Genetic testing was positive for 22 q11deletion, commonly associated with this lesion. Surgery consisted of unifocalization of the discontinuous LPA with placement of a valved pulmonary homograft during complete repair of this lesion. Our case highlights the importance of prenatal detection, to aid in the prompt initiation of prostaglandins so as to ensure early rehabilitation of the left lung. Inability to visualize one of the branch pulmonary arteries (PA's) and a PDA on fetal echocardiogram in TOF/APV must raise suspicion for an eccentric branch PA with ductal origin.

Original languageEnglish (US)
Pages (from-to)996-1000
Number of pages5
JournalEchocardiography
Volume36
Issue number5
DOIs
StatePublished - May 2019
Externally publishedYes

Keywords

  • congenital heart disease
  • echocardiography
  • fetal echocardiography
  • pediatric echocardiography
  • tetralogy of Fallot

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Cardiology and Cardiovascular Medicine

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