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Long Term Follow-Up of Patients with Systemic Right Ventricle and Biventricular Physiology: A Single Centre
Cristina Ciuca1, Anna Balducci1, Emanuela Angeli2
1Pediatric Cardiology and Adult Congenital Heart Disease Program, Department of Cardio-Thoracic and Vascular Medicine, IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40138 Bologna, Italy.
Insights
Adults with a systemic right ventricle (SRV) face frequent clinical events like arrhythmias and heart failure. Long-term monitoring is crucial for managing these congenital heart disease cases.
Area of Science:
- Cardiology
- Adult Congenital Heart Disease
- Cardiac Surgery
Background:
- Congenital heart disease (CHD) is increasingly diagnosed in adults.
- Systemic right ventricle (SRV) associated CHD cases have poorer prognoses.
- Understanding long-term outcomes in SRV patients is critical.
Purpose of the Study:
- To evaluate the long-term clinical outcomes and adverse events in adult patients with SRV.
- To compare outcomes between different etiologies of SRV (transposition of the great arteries vs. congenitally corrected transposition).
Main Methods:
- Retrospective analysis of 73 SRV patients evaluated between 2014-2020.
- Included patients with transposition of the great arteries (atrial switch) and congenitally corrected transposition (ccTGA).
- Follow-up data on clinical events, hospitalizations, and survival were analyzed.
Main Results:
- High incidence of clinical events, primarily arrhythmias (27.1%) and heart failure (12.3%).
- Six-year event-free survival was 90%; no significant difference between SRV groups.
- Late gadolinium enhancement (LGE), lower exercise capacity, higher NYHA class, and RV dilation/hypokinesis predicted poorer outcomes.
Conclusions:
- Long-term follow-up of SRV patients is marked by significant clinical events, mainly arrhythmias and heart failure.
- These events lead to frequent unscheduled hospitalizations.
- Despite event incidence, overall survival remains high, but risk factors for adverse outcomes require attention.
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.
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