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Updated: Apr 7, 2026
09:22
Chronic Thromboembolic Pulmonary Hypertension and Assessment of Right Ventricular Function in the Piglet
Published on: November 4, 2015
12.7K
Hypoplastic Left Heart Syndrome With Right Ventricle Compression
Tai Fuchigami1, Masahiko Nishioka1, Kazuhiro Takahashi2
1Department of Pediatric Cardiovascular Surgery, Okinawa Prefectural Nanbu Medical Center & Children's Medical Center, Arakawa, Okinawa prefecture, Japan.
The Annals of Thoracic Surgery
|July 5, 2015
Summary
This study presents a novel 2-stage Norwood-Glenn procedure for a neonate with aortic atresia-mitral stenosis (AA-MS) and hypoplastic left heart syndrome (HLHS). The approach successfully managed right ventricular (RV) load, leading to a positive patient outcome.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Surgical Innovation
Background:
- Hypoplastic left heart syndrome (HLHS) with aortic atresia-mitral stenosis (AA-MS) presents complex hemodynamic challenges.
- Suprasystemic left ventricular (LV) pressure and septal deviation can compromise right ventricular (RV) function.
Observation:
- A male neonate with the AA-MS variant of HLHS exhibited a shifted intraventricular septum and a compressed RV.
- The patient required management of high RV volume load and output to maintain stroke volume.
Findings:
- A 2-stage Norwood-Glenn (NW-G) procedure was employed, including bilateral pulmonary artery banding (bPAB) on day of life (DOL) 4.
- Percutaneous balloon dilation of the bPAB was performed on DOL 58, followed by the NW-G procedure at 3 months without peripheral pulmonary hypoplasia.
Implications:
- This staged surgical approach effectively managed RV volume load in a complex HLHS case.
- The successful outcome suggests the potential of this modified NW-G strategy for similar pediatric cardiac conditions.
- This case highlights innovative management for neonates with severe congenital heart defects.
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