The outcomes of Fontan operation in patients with hypoplastic left heart syndrome (HLHS) versus other forms of univentricular heart

Abstract

Abstract Background Single ventricle physiology is a rare condition but is highly represented in clinical practice due to a high prevalence of late complications and comorbidities. The Fontan operation has undergone considerable modification over the past 5 decades and is now in a superior hydrodynamic form. Purpose The purpose was to identify the potential differences in outcomes of Fontan operation between patients with hypoplastic left heart syndrome (HLHS) and with other indications to this treatment. Methods and results The study comprised 74 patients (20±6 years) after the Fontan operation (26 females) divided based on the indication to this operation as a group with HLHS (19 patients) and the group without HLHS (54 patients). Patients with HLHS had significantly more often had a fenestration between the systemic venous return and the pulmonary venous atrium (89% vs 41%; p=0.001) which was closed in further observation in 89% in HLHS versus 39% in patients without HLHS, p=0.001. Regarding complications after Fontan operation, in HLHS group more often narrowings of pulmonary arteries requiring stenting occurred (74% vs 13%; p=0.001) and Fontan-associated liver disease (37% vs 9%, p=0.05). There were no differences in stenting of the lateral tunnel connection, fistulas, enteropathy, rhythm disturbances, peacemaker implantation, vascular complications, strokes between groups. HLHS patients more often were treated with antiplatelet drugs (26% vs 0, p=0.001), spironolactone (63% vs 23%, p=0.01) and angiotensin-converting-enzyme inhibitors (89% vs 44%, p=0.01). Conclusions HLHS patients have worse outcomes of Fontan operation. This group of patients more often needs fenestrations and regular controls towards pulmonary arteries narrowings and Fontan-associated liver disease. Funding Acknowledgement Type of funding sources: Public hospital(s). Main funding source(s): Polish Mother's Memorial Hospital - Research Institute