Prognostic Relevance of Thyroid Disorders in Adults With Congenital Heart Disease

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Adults with congenital heart disease (ACHD) are frequently affected by thyroid diseases (TDs). However, the clinical relevance of TD in ACHD remains unknown. We aimed to describe the prevalence of TD in the ACHD population and to ascertain whether TD are associated with worse outcome. Patients with ACHD >18 years attending our tertiary center for a day-case between 2014 and 2019 were included. Clinical data between patients’ first visit and December 2020 were collected. Primary end point was a combination of death, hospitalization for heart failure (HF), and new-onset of arrhythmic events. Secondary end points were each part of the primary outcome as separate end points. A total of 495 patients with ACHD (32.2 [24.5 to 45.6] years; 54% women) were included. Median follow-up was 9.4 (4.5 to 13.1) years. The prevalence of TD was 30%. TD group showed worse clinical status, as demonstrated by N-terminal pro b-type natriuretic peptide values (243.5 [96.5 to 523] vs 94 [45 to 207] pg/ml, p <0.001) and New York Heart Association class (27% vs 13% in class III to IV, p <0.0001) with higher incident rate of adverse events at follow-up (4.45 [3.43 to 5.69] % vs 1.29[0.94 to 1.75] % per person-year, p <0.001). TD were independently associated with higher risk of death (hazard ratio [HR] 4.1, p = 0.009), arrhythmic events (HR 3.8, p <0.0001), and hospitalization for HF (HR 8.02, p <0.0001). There was a fourfold increased risk of primary end point in the TD group even after propensity score matching for clinical variables including age, gender, disease complexity, physiological stage, previous palliative surgery, ventricular function, pulmonary arterial hypertension, cyanosis, and presence of systemic right ventricle (HR 4.47, p <0.0001). In conclusion, TD are predictive of adverse outcome in the ACHD population. Routine screening of thyroid function during follow-up in this population may be helpful to identify those with higher risk of death, arrhythmias, and HF.

Section snippets

Methods

Data on consecutive patients with ACHD, >18 years, attending our tertiary center between 2014 and 2019 were retrospectively collected. The study population was divided in 2 groups according to the history of TD at baseline, defined as clinical manifest or subclinical (abnormal serum thyroid stimulating hormone [TSH] level) thyroid dysfunction including iatrogenic disease after thyroidectomy, goiter, and thyroid malformation. Electronic records were reviewed for clinical data between patients’

Results

A total of 495 patients (32.2 [24.5 to 45.6] years, 54% women) were included. Baseline clinical details are summarized in Table 1. The majority (414 = 84%) had moderate/complex disease. A total of 151 patients (30.5%) had a diagnosis of TD (Table 2). Of them, 115 were on replacement therapy (76%) and 7 were on suppressive treatment (5%) at last clinical assessment. Laboratory data of thyroid function obtained on an outpatient basis at last assessment were available for 73 patients. A total of

Discussion

TD were highly prevalent in our ACHD population and were related to increased risk of adverse outcome even after stratification for genetic disorders and statistical adjustment for baseline differences between groups. Thyroid hormones influence the cardiovascular system with effects on cardiac contractility, heart rate, peripheral vascular smooth muscle, and blood volume.1 A growing number of studies shed light on the association of even subclinical TD with the risk of death, HF, and AA in the

Conclusion

TD are highly prevalent in patients with complex ACHD and are associated with a fourfold increased risk of adverse outcome after propensity score matching accounting for baseline clinical differences and established risk factors for cardiac events. Our results support routine screening for TD during follow-up of patients with ACHD, which may allow to identify patients at higher risk of events.

Disclosures

The authors have no conflicts of interest to declare.

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  • Flavia Fusco and Giancarlo Scognamiglio contributed equally to the manuscript.

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