Long term follow-up of systemic right ventricle

Purpose: to assess the long-term follow-up in patients with systemic right ventricle. Methods: Seventy-three patients with systemic right ventricle (SRV) evaluated in the outpatient clinic between January 2014 and September 2020 were enrolled in this study. Thirty-four patients had a transposition of the great arteries treated either with Mustard or Senning procedure and 39 patients had a congenitally corrected transposition of the great arteries (ccTGA). Results: Mean age at the first evaluation was 29.6 ± 14.2 years, 47.9% were female. NYHA class at the visit was as follows: I in 53 % of cases, II in 33 % of cases, III 11% of cases and IV in 3% of cases. The first echocardiographic evaluation testifies end diastolic/systolic area 20.1 ± 5.3 cm2/m2 respectively 12.9 ± 4.4cm2/m2 significantly more dilated was the SRV of ASO patients. Overall, systolic function was normal or slightly impaired. A quarter of patients had significant tricuspid regurgitation (sTR). Cardiac MRI (CMR) confirmed the dilatation of the SRV end diastolic RV volume = 117 ± 40ml/m2 a RV ejection fraction = 49 ± 12%. Late gadolinium enhancement (LGE) was presented 42.3% of cases. CPET showed a reduced exercise capacity. Survival free from adverse events was 98.6% at one year and 95.8% at 5-year follow-up without difference between the two groups. The most common adverse event during follow-up the presence of arrhythmia requiring hospitalisation (27.1%) followed by signs of heart failure requiring hospitalisation (12.3%). The presence of LGE at CMR together with lower exercise capacity, higher NYHA class and more dilated and/or hypokinetic RV predicted a poorer outcome. Conclusions: Long term follow-up of patients with systemic right ventricle is characterized by a high incidence of clinical events, prevalently arrhythmias and heart failure which cause most of the unscheduled hospitalisations.