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

• Published in: Journal of cardiovascular development and disease Vol. 10; no. 5; p. 219
• Main Authors: Ciuca, Cristina, Balducci, Anna, Angeli, Emanuela, Di Dio, Mariateresa, Assenza, Gabriele Egidy, Mariucci, Elisabetta, Ragni, Luca, Lovato, Luigi, Niro, Fabio, Gesuete, Valentina, Careddu, Lucio, Bartolacelli, Ylenia, Bulgarelli, Ambra, Donti, Andrea, Gargiulo, Gaetano Domenico
• Format: Journal Article
• Language: English
• Published: Switzerland MDPI AG 17-05-2023; MDPI
• Subjects: adults with congenital heart disease; Arrhythmia; atrial switch operation; Cardiovascular disease; Care and treatment; Congenital diseases; Congenital heart disease; congenitally corrected transposition of the great arteries; Genetic disorders; Heart; Heart failure; Heart surgery; Medical research; Medicine, Experimental; Mustard operation; Physiological aspects; Pulmonary arteries; Quality of life; Senning operation; Statistical analysis; systemic right ventricle; Transplantation; Veins & arteries; congenitally corrected transposition of the great arteries; adults with congenital heart disease; Mustard operation; systemic right ventricle; Senning operation; atrial switch operation
• ISSN: 2308-3425; 2308-3425
• DOI: 10.3390/jcdd10050219

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.
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).
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.
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.