Patients with Congenital Heart Disease more likely to develop Immune Deficiency Syndromes

This study published in European Heart Journal by Gerhard-Paul Diller and colleagues aimed to provide population-based data on the prevalence and clinical significance of immune deficiency syndromes (IDS) associated with congenital heart disease (CHD). The research utilised administrative data from the German Health System to assess the prevalence of increased susceptibility to infection (ISI) or confirmed IDS in CHD patients and compared these findings to an age-matched non-congenital control group. Additionally, the study explored the prognostic significance of IDS by analyzing all-cause mortality and freedom from emergency hospital admissions.

Key Findings:

● High Prevalence: Among the 54,449 CHD patients included in the study, 27.5% had increased susceptibility to infection (ISI), and 5.6% had documented IDS. This contrasted with a prevalence of 2.9% for IDS in the age-matched general population.

● Significant Impact: Over an observation period of 394,289 patient-years, 3,824 CHD patients died, and 31,017 experienced combined events of all-cause mortality or emergency hospital admission.

● Predictors of Mortality: On multivariable analysis, the presence of ISI or documented IDS emerged as independent predictors of all-cause mortality. Patients with IDS had a 1.77 times higher risk of mortality, while those with ISI had a 2.14 times higher risk.

● Higher Risk of Hospitalization: Both ISI and confirmed IDS were associated with a significantly higher risk of emergency hospital admissions during follow-up.

Implications:

These findings highlight several crucial implications for CHD patients:

● Common Immune Deficiency: Limited immune competence is prevalent in CHD patients, making them more susceptible to infections.

● Increased Risk: IDS in CHD patients is linked to an elevated risk of morbidity and mortality, emphasising the importance of early detection and intervention.

● Screening and Collaboration: There is a need for structured IDS screening in CHD patients, along with collaboration with immunology specialists. Identifying immune deficiencies can lead to tailored therapeutic interventions.

● Tailored Prophylaxis: Future studies should investigate whether IDS patients might benefit from intensified antibiotic shielding or tailored prophylaxis.

In summary, this research underscores the significance of addressing immune deficiency syndromes in the CHD population. Early detection and appropriate interventions can help improve the outcomes and overall health of individuals living with congenital heart disease.

Reference:

Diller, G.-P., Lammers, A. E., Fischer, A., Orwat, S., Nienhaus, K., Schmidt, R., Radke, R. M., De-Torres-Alba, F., Kaleschke, G., Marschall, U., Bauer, U. M., Roth, J., Gerß, J., Bormann, E., & Baumgartner, H. (2023). Immunodeficiency is prevalent in congenital heart disease and associated with increased risk of emergency admissions and death. European Heart Journal, 44(34), 3250–3260. https://doi.org/10.1093/eurheartj/ehad029