Long-term experience with the one-and-a-half ventricle repair for simple and complex congenital heart defects

The one-and-a-half ventricle repair (1.5VR) is a surgical alternative to Fontan circulation or high-risk biventricular repair in patients with complex congenital heart disease (CHD) with a hypoplastic right ventricle (RV). We report our 25 years of experience to evaluate whether the degree of anatomical complexity of the CHD can affect long-term outcomes.

This is a retrospective study including patients undergoing 1.5VR between 1994 and 2018. Clinical records and operative reports were reviewed. Follow-up data were collected from all survivors at the last evaluation or by phone contact.

Twenty-nine patients underwent 1.5VR [median age: 3.5 years, interquartile range (IQR) 0.8-7.8]. Fifteen patients had 'simple' (i.e. confined to right heart lesions) anatomical characteristics (48.3%); the median tricuspid valve annulus z-score was -3.5 (IQR -6.2 to +3.6). There were no operative deaths. The median hospital stay was 21 days (IQR 10-33), with postoperative complications in 21 patients (75.8%). At a median follow-up of 13.2 years (IQR 3.2-25.6 years; completeness 96.6%), there were 3 late deaths. There was no significant difference in survival and in freedom from adverse events between simple and complex anatomy groups. However, stress test findings showed a tendency towards a higher maximum oxygen uptake in the simple group (P = 0.055).

The 1.5VR strategy is an effective low-risk surgical option for patients with CHD with hypoplastic RV. When it is planned from birth, late outcomes can be satisfactory. In our experience, the presence of associated major cardiac anomalies was not associated with either worse early or long-term outcomes and functional status.