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Updated: Aug 16, 2026

Left Atrial Stenosis Induced Pulmonary Venous Arterialization and Group 2 Pulmonary Hypertension in Rat
08:34

Left Atrial Stenosis Induced Pulmonary Venous Arterialization and Group 2 Pulmonary Hypertension in Rat

Published on: November 18, 2018

Incomplete atrioventricular septal defect with hypoplastic left ventricle and left atrioventricular valve stenosis

M Nishimura1, M Yamagishi, K Fujiwara

  • 1Department of Pediatric Cardiovascular Surgery, Children's Research Hospital, Kyoto Prefectural University of Medicine, Kawaramachi, Hirokoji, Kamigyo-ku, Kyoto 602-8566, Japan.

The Japanese Journal of Thoracic and Cardiovascular Surgery : Official Publication of the Japanese Association for Thoracic Surgery = Nihon Kyobu Geka Gakkai Zasshi
|May 18, 2001
PubMed

Insights

This study reports a successful biventricular repair in an infant with a hypoplastic left ventricle and atrioventricular septal defect. Apex-ward left ventricular morphology predicted good postoperative function despite small end-diastolic volume.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease Surgery
  • Cardiac Morphology

Background:

  • Incomplete atrioventricular septal defect (AVSD) with a hypoplastic left ventricle (HLV) presents complex surgical challenges.
  • Left atrioventricular valve (LAVV) stenosis, often due to anomalous papillary muscles, further complicates management.

Observation:

  • A 2-month-old infant exhibited an incomplete AVSD, HLV, and LAVV stenosis caused by a solitary papillary muscle.
  • Echocardiography revealed marked right ventricular dilatation and a diminutive left ventricle occupying the apex.
  • Cardiac catheterization indicated a significant pulmonary-to-systemic flow ratio (3.61) and altered ventricular volumes.

Findings:

  • Successful biventricular repair was achieved through longitudinal division of the solitary papillary muscle and ostium primum closure with bovine pericardium.
  • Postoperative assessment demonstrated appropriate left ventricular function.
  • The left ventricle's apical position was a key factor in its ability to handle volume load post-repair.

Implications:

  • Apex-ward left ventricular morphology is a crucial predictor of successful biventricular repair outcomes in HLV with AVSD.
  • This case highlights the feasibility of biventricular repair even with severely reduced preoperative left ventricular end-diastolic volume.
  • Surgical strategies targeting papillary muscle anomalies can restore adequate left ventricular performance in complex congenital heart defects.

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