Takotsubo Cardiomyopathy Triggered by Acute Intermittent Porphyria

Background Takotsubo cardiomyopathy (TC) is a reversible condition characterized by myocardial akinesis due to catecholamine-mediated myocardial stunning. Acute intermittent porphyria (AIP) is associated with a rise in catecholamine, which could trigger TC. This study aims to evaluate patients with porphyria-triggered TC. Methods Data from the National Inpatient Sample (NIS) was used to study the prevalence rate and clinical outcome of porphyria-triggered TC among patients with TC. Results Overall, 32,500 cases were admitted between 2012 and 2016 with TC. The rates of smoking, hypertension, hyperlipidemia, and diabetes mellitus were 28%, 54%, 45%, and 23%, respectively. Six and three percent had cardiogenic shock and cardiac arrest, respectively. The overall inpatient mortality was 5.4%. Out of 32,500 patients with takotsubo cardiomyopathy, only three of these cases were found to have porphyria. Patients with porphyria were not significantly different in the baseline health characteristics from patients without porphyria. Additionally, there were no significant differences in the inpatient clinical outcomes between patients with porphyria vs. patients without porphyria.  Conclusion TC triggered by porphyria is a rare disease. Patients with this disease have an excellent short-term prognosis. Beta-blocker medications might be effective in these patients to reduce the risk of recurrence. Further prospective studies are needed to test the effectiveness of beta-blocker in reducing the recurrence of TC.