Timing, Accessibility of Resources Key to Effective Treatment of Fulminant Myocarditis

Published today in the journal Circulation, researchers outlined the need for increased awareness and education of fulminant myocarditis (FM) among healthcare providers. FM is a rapidly developing rare condition that induces severe inflammation of the heart, which is attributed to an exceptionally high risk of death caused by cardiogenic shock, fatal arrhythmias, and multi-organ failure.

The condition is often caused by a viral infection, and it can also results from drug reactions and hematologic cancers. Because FM comes on suddenly and with significant severity, researchers stress that early recognition is vital for favorable outcomes of this former universally fatal condition that, historically, was usually diagnosed at autopsy.

Current treatment for all patients with FM involves some form of inotropic or mechanical circulatory support to maintain end-organ perfusion until transplantation or recovery. Some patients with specific subtypes of FM have responded to immunomodulatory therapy in addition to guideline-directed medical care.

While the increasing availability of circulatory support, orthotopic heart transplantation, and disease-specific treatments promote efficient care for patients with FM, significant morbidity and mortality have continued. The delay in diagnosis and initiation of circulatory support was referenced as key factors for this trend, as well as a lack of appropriately trained specialists to manage the condition.

In the scientific statement, researchers detail the resources necessary to manage the spectrum of FM:

• Extracorporeal life support, percutaneous and durable ventricular assist devices, and transplantation capabilities
• Specialists in advanced heart failure, cardiothoracic surgery, cardiac pathology, immunology, and infectious disease

Education of frontline providers who are most likely to encounter FM first was additionally highlighted by researchers, as their ability to uncover the condition at onset serves as essential in increasing timely access to appropriately resourced facilities. Finding the disease early in its development can prevent the severity of symptoms, which can cause multi-organ system failure, and promote tailored disease-specific therapies.

Leslie Cooper, MD, FAHA, vice chair of the Statement Writing Group, noted that fortunately the condition is rare and often seen in younger and healthier patients as opposed to critically ill patients, which assists FM diagnosis. “This is where there are the greatest opportunities: early diagnosis, rapid treatment and the ability of frontline clinicians to detect the subtle signs and symptoms of this serious condition,” said Cooper.

FM’s uncommonness, however, adds to the difficulty in studying the condition, as the authors of the statement said that most of the evidence for treatment of the condition stems from registries, case series, and case reports as opposed to standard randomized prospective placebo-controlled clinical trials.

“These are not guideline-based recommendations because the evidence to date is not strong enough to reach the rigor required to be classified as guidelines. Rather, these are considerations for the clinician to review that are based on our expert experience,” they said.

Reference

Kociol RD, Cooper LT, Fang JC, et al. Recognition and initial management of fulminant myocarditis: a scientific statement from the American Heart Association [published online January 6 2020]. Circulation. doi: 10.1161/CIR.0000000000000745.