Cardiovascular Outcomes in Congenital Heart Disease

Congenital heart disease (CHD) represents a spectrum of structural cardiac anomalies present from birth, affecting nearly 1% of live births worldwide. Advances in surgical and interventional techniques have dramatically improved survival, yielding a growing population of adolescents and adults living with repaired or palliated defects. Long‐term cardiovascular outcomes now centre on late complications such as heart failure, arrhythmias, exercise intolerance and end‐organ sequelae. Patients with single‐ventricle physiology managed by the Fontan circulation are at particular risk of chronically elevated central venous pressures, leading to hepatic congestion, protein‐losing enteropathy and diminished cardiopulmonary fitness. Quality of life may be impaired by reduced exercise capacity, psychosocial stressors and the need for ongoing multidisciplinary surveillance. Contemporary management emphasises individualised risk stratification, non‐invasive monitoring of ventricular function and end‐organ health, and integration of lifestyle interventions to optimise functional status. Globally, the burden of CHD demands adaptation of care pathways to resource‐limited settings, highlighting the importance of scalable rehabilitation programmes and accessible diagnostic tools. Practical applications include structured cardiac rehabilitation, routine hepatobiliary assessment in Fontan patients and targeted therapies to preserve myocardial and end‐organ function over the life course.

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Cardiovascular Outcomes in Congenital Heart Disease publication trend

The graph below shows the total number of articles in cardiovascular outcomes in congenital heart disease across all publications each year (not limited to Nature Index journals).

Technical terms

Congenital heart disease (CHD): A range of structural heart defects present at birth affecting cardiac chambers, valves or vessels.

Fontan circulation: A palliative surgical configuration for single‐ventricle hearts that routes systemic venous return directly to the pulmonary arteries, bypassing a subpulmonary ventricle.

Cardiac rehabilitation: A structured programme of exercise training, education and psychosocial support aimed at improving cardiovascular function and quality of life.

Hepatic fibrosis: The accumulation of extracellular matrix proteins in the liver, often resulting from chronic congestion or inflammation and progressing to cirrhosis.

References

1. Early hybrid cardiac rehabilitation in congenital heart disease: the QUALIREHAB trial. European Heart Journal (2024).
2. Severity of Fontan-Associated Liver Disease Correlates with Fontan Hemodynamics. Pediatric Cardiology (2020).
3. Non-invasive Investigations for the Diagnosis of Fontan-Associated Liver Disease in Pediatric and Adult Fontan Patients. Frontiers in Cardiovascular Medicine (2017).