Fontan-like circulation as a criterion for heart transplantation in arrhythmogenic right ventricular dysplasia

Arrhythmogenic right ventricular dysplasia (ARVD) is often associated with progressive right ventricular dysfunction. Although heart transplantation (HTx) is suggested in these patients, indication and optimal timing for listing can be challenging.

The study comprises four patients (two male, range: 37-56 years) with advanced ARVD who were considered for HTx. Standard inclusion criteria for HTx listing such as clinical signs, New York Heart Association (NYHA) classification (II-III), and N-terminal pro-brain natriuretic peptide (NT-proBNP) (3672 ± 1407 pg/ml) were heterogeneous and did not add unequivocally to decision making. In all patients, though, right heart hemodynamics revealed Fontan-like circulation (FLC) with equilibrated pressure tracings between the right atrium (16 ± 4 mmHg) and the pulmonary artery (16 ± 5 mmHg). In this condition, the pulmonary blood flow can be regarded as nearly non-pulsatile, as it is passive and propelled by the transpulmonary gradient and intrathoracic pressure alterations produced by breathing to the left atrium. Based on these findings, all patients were listed for HTx and were finally successfully transplanted.

In patients with ARVD, evidence of FLC may serve as an additional criterion for HTx. This applies particularly to patients who do not clearly fulfill standard transplant criteria and to patients with electrical instability.