Patients With Hypoplastic Left Heart Syndrome Have a Shorter Superior Vena Cava

Area of Science:

• Congenital Heart Surgery
• Pediatric Cardiology
• Thoracic Surgery

Background:

• Hypoplastic left heart syndrome (HLHS) treatment often involves the Fontan pathway and Glenn procedure.
• The superior vena cava (SVC) length is a critical factor in performing the Glenn procedure.
• Previous studies have not extensively compared SVC length in HLHS patients.

Purpose of the Study:

• To investigate the hypothesis that the superior vena cava (SVC) is shorter in patients with HLHS.
• To compare SVC length between HLHS patients and those with other congenital heart diseases.
• To determine the potential impact of SVC length on the Glenn procedure in HLHS.

Main Methods:

• Study included patients with HLHS, ventricular septal defects (VSD), and pulmonary atresia with intact ventricular septum (PA/IVS).
• The effective superior vena cava ratio (ESCVR) was measured as a proxy for SVC length.
• ESCVR was defined as the distance from the inferior border of the left brachiocephalic vein to the superior surface of the right pulmonary artery relative to height.

Main Results:

• The median ESVCR was significantly lower in HLHS patients (12.54 mm/m) compared to VSD (17.96 mm/m) and PA/IVS (18.46 mm/m) groups.
• Statistical analysis confirmed a significant difference in ESVCR between HLHS and the other groups (P < .002).
• These findings indicate a shorter SVC in HLHS patients.

Conclusions:

• Patients with HLHS exhibit a relatively shorter superior vena cava (SVC).
• This anatomical variation may present challenges during the Glenn procedure for HLHS patients.
• Further consideration of SVC length is important for surgical planning in HLHS.