Long Term Follow-Up of Patients with Systemic Right Ventricle and Biventricular Physiology: A Single Centre Experience

Abstract

Background: A progressively increasing prevalence of congenital heart disease (CHD) in adulthood has been noticed in recent decades; CHD cases with a systemic right ventricle have a poorer outcome.

Methods: Seventy-three patients with SRV evaluated in an outpatient clinic between 2014 and 2020 were enrolled in this study. Thirty-four patients had a transposition of the great arteries treated with an atrial switch operation; 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; 48% of the patients were female. The NYHA class at the visit was III or IV in 14% of the cases. Thirteen patients had at least one previous pregnancy. In 25% of the cases, complications occurred during pregnancy. Survival free from adverse events was 98.6% at one year and 90% at 6-year follow-up without any difference between the two groups. Two patients died and one received heart transplantation during follow-up. The most common adverse event during follow-up was the presence of arrhythmia requiring hospitalization (27.1%), followed by heart failure (12.3%). The presence of LGE together with lower exercise capacity, higher NYHA class and more dilated and/or hypokinetic RV predicted a poorer outcome. Quality of life was similar to the QoL of the Italian population.

Conclusions: Long-term follow-up of patients with a systemic right ventricle is characterized by a high incidence of clinical events, prevalently arrhythmias and heart failure, which cause most of the unscheduled hospitalizations.

Keywords: Mustard operation; Senning operation; adults with congenital heart disease; atrial switch operation; congenitally corrected transposition of the great arteries; systemic right ventricle.

Figure 1

Survival free from adverse events during the study: Overall, one-year survival free from adverse events was 98.6% while 6-year survival free from adverse events was 90%.

Figure 2

Quality of life evaluation: The SF-36 is a generic multi-item questionnaire comprising 36 questions in eight domains: physical functioning (PF) 83.8 ± 19.8; role physical (RP) 89.2 ± 29.9; bodily pain (BP) 90.3 ± 21.1, general health (GH) 67.8 ± 22, vitality (VT) 74 ± 16, social functioning (SF) 86.2 ± 17.8, role emotional (RE) 92.3 ± 25.5, and mental health (MH) 78.7 ± 15.9.