Broken heart syndrome triggered by acute intermittent porphyria is rare

Incidence of the heart condition estimated to be 1 in 10,000

Steve Bryson, PhD avatar

by Steve Bryson, PhD |

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Acute intermittent porphyria (AIP) doesn’t appear to be strongly associated with Takotsubo cardiomyopathy, a temporary condition marked by stress-induced heart muscle weakness, a study shows.

Despite the potential connection between the two conditions, the incidence of the heart condition triggered by AIP is estimated to be one in 10,000. The study, “Takotsubo Cardiomyopathy Triggered by Acute Intermittent Porphyria,” was published in Cureus.

AIP is the most common form of acute porphyria, a group of genetic disorders marked by disruptions in the production of a molecule called heme that helps transport oxygen in the body. As a result, porphyrins and other heme precursor molecules accumulate in tissues and organs, eventually reaching toxic levels and causing symptoms.

The disorder has been associated with a rise in the levels of catecholamines, hormones such as adrenaline that are produced by the adrenal glands in response to stress or excitement.

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1 in 10,000 cases of Takotsubo cardiomyopathy tied to AIP

Catecholamines can also trigger Takotsubo cardiomyopathy, sometimes called stress-induced cardiomyopathy or broken heart syndrome, a reversible condition marked by temporary heart muscle weakness. A feature of acute porphyria is severe abdominal pain, which can trigger the heart condition.

Because of their possible connection, researchers at the Taibah University-College of Medicine, Saudi Arabia, investigated the prevalence of AIP-triggered Takotsubo cardiomyopathy. Data were collected from the Healthcare Cost and Utilization Project (HCUP), a large, publicly available inpatient database in the U.S.

Between 2012 and 2016, 32,500 people with Takotsubo cardiomyopathy were identified. They had a median age of 68 and most (85%) were women.

Among them, 28% were smokers, 54% had high blood pressure, 45% had elevated fatty molecule levels in the bloodstream, and 23% had diabetes. Heartbeat irregularities were detected in 20%, chronic kidney disease in 13%, and sepsis in 11%. Overall inpatient mortality was 5.4%.

Three people with Takotsubo cardiomyopathy had porphyria. There were no overall health characteristic differences between those with or without porphyria when they entered the database. There were also no differences in clinical outcomes between those with or without porphyria.

AIP-associated Takotsubo cardiomyopathy could be treated with a combination of hemin, a medicine that eases AIP attack symptoms, and beta-blockers, which block the action of adrenaline, the researchers said.

“This study found that [Takotsubo cardiomyopathy] triggered by porphyria is a rare disease, with an estimated incidence of 1 in 10,000 patients,” the researchers wrote. “The study also found that these patients had a good prognosis, with no inpatient deaths.”