Cardiovascular manifestations in hospitalized patients with hemochromatosis in the United States

Background: Heart complications are the main cause of morbidity and mortality in hemochromatosis, but the liver is the main site for iron deposition in these patients. Large multicenter studies have described cardiovascular (CV) manifestations in patients with secondary hemochromatosis. However, the overall prevalence and risk of CV manifestations in patients with hemochromatosis at the population level are unknown Objective: To examine the prevalence and risk of CV manifestations in patients with hemochromatosis. Methods: A retrospective cohort from the National Inpatient Sample database between 2012 and 2014 was studied. We identified hemochromatosis using ICD-9-CM diagnostic codes. CV manifestations were defined by the presence of conduction disorders, arrhythmias, congestive heart failure (CHF), pulmonary hypertension, and non-ischemic cardiomyopathy (NISCM). Results: Of the 63,846,188 weighted hospitalizations that met the inclusion criteria, 64,590 (0.1%) had hemochromatosis and 13,200,000 (20.7%) had one or more CV manifestations. Of those with hemochromatosis, 5.3% had primary and 94.7% had secondary hemochromatosis. 27.8% of all hemochromatosis patients had one or more CV manifestations, 16% cardiac arrhythmias, 10.6% supraventricular arrhythmias (SVA), 0.8% ventricular arrhythmias, 9.3% CHF, 7.4% pulmonary hypertension, 4.2% NISCM, 2% conduction disorders, and 0.4% cardiac arrest. SVA (14.6% vs 10.4%, P < 0.001) was more prevalent in primary hemochromatosis compared to secondary while pulmonary hypertension (7.7% vs 2.6%, P < 0.001) was more prevalent in secondary hemochromatosis compared to primary. In multivariate modelling, only the adjusted odds of composite CV manifestations (odds ratio [OR] 1.24, 95% confidence interval [CI]: 1.03-1.48, P < 0.05) and SVA (OR 1.59, 95% CI: 1.28-1.96, P < 0.001) were significantly higher in patients with primary hemochromatosis compared with patients without hemochromatosis. In patients with secondary hemochromatosis, the adjusted odds of composite CV manifestations (OR 1.84, 95% CI: 1.74-1.95, P < 0.001), CHF (OR 1.46, 95% CI: 1.35-1.57, P < 0.001), conduction disorder (OR 1.52, 95% CI: 1.33-1.73, P < 0.001), pulmonary hypertension (OR 4.43, 95% CI: 3.97-4.94, P < 0.001), SVA (OR 1.39, 95% CI: 1.29-1.48, P < 0.001), and NISCM (OR 1.98, 95% CI: 1.79-2.20, P < 0.001) were significantly higher compared with patients without hemochromatosis. Conclusion: Supraventricular arrhythmias, congestive heart failure, and pulmonary hypertension were the most common CV disorders in hemochromatosis patients. Risk-adjusted burden of supraventricular arrhythmias was significantly higher in primary and secondary hemochromatosis while patients with secondary hemochromatosis had a higher risk of congestive heart failure, pulmonary hypertension, conduction disorders, and non-ischemic cardiomyopathy.

Automated summary

The study found that supraventricular arrhythmias, congestive heart failure, and pulmonary hypertension were the most common cardiovascular disorders in patients with hemochromatosis. There were differences in the risk of certain cardiovascular diseases between patients with primary and secondary hemochromatosis.