Review article

Mechanical Circulatory Support for Patients With Fulminant Myocarditis: The Role of Echocardiography To Address Diagnosis, Choice of Device, Management, and Recovery

Fulminant myocarditis (FM) is an inflammation of the myocardium characterized by progressive acute heart failure leading to cardiogenic shock that develops over several hours. In this article, we present a case of a female patient with acute fulminant lymphocytic myocarditis who was successfully treated with biventricular MCS.

Ventricular assist devices are electromechanical pumps used for circulatory support of patients with severe heart failure. Current ventricular assist device therapy provides a broad spectrum of support systems ranging from short-term to long-term devices. Echocardiography is an ideal modality to monitor patients undergoing mechanical circulatory support because it is less invasive, widely available and can be performed at the bedside. Both long-term and short-term devices require a careful echocardiographic management before implant, during surgical procedure and during postoperative course. Perioperative echocardiographic examination is performed to assess cardiac abnormalities that could lead to postoperative complications, to evaluate the surgical result and to determine reasons of postoperative haemodynamic compromises. Cannulae visualisation, patent forame ovale (PFO), air in cardiac chambers, aortic and or mitral regurgitation, right-ventricular function, left-ventricular unloading and filling, tamponade and pump failure are the most important issues discussed in this review.

Acute fulminant myocarditis is a life-threatening disease in children. A limited number of reports suggest that mechanical circulatory support (MCS) may be used to successfully bridge children with acute fulminant myocarditis to recovery or transplantation. We evaluated the effectiveness of MCS in children with myocarditis and identified risk factors associated with adverse outcomes.

Between 2001 and 2009, 16 children were treated for myocarditis at our institution; each child received MCS provided by extracorporeal membrane oxygenation, ventricular assist device(s), or both. Of these patients, 75% (12/16) survived: 7 recovered ventricular function, and 5 underwent successful orthotopic heart transplantation. In patients who were bridged to recovery, mean left ventricular ejection fraction significantly improved from initiation to termination of MCS (20 ± 9.3% to 62 ± 5%; P = .0004). Viral pathogens were detected in 11 patients by polymerase chain reaction, and viral presence was associated with death or need for transplantation (P = .011). Upon histologic analysis, absence of viral infection and lack of myocardial inflammation were associated with recovery (P values .011 and .044, respectively).

In children with acute fulminant and persistent myocarditis, MCS is a life-saving treatment strategy, particularly in the absence of viral infection.

Myocarditis can be totally asymptomatic or can manifest with chest pain syndromes, ranging from mild persistent chest pain of acute myopericarditis to severe symptoms that mimic acute myocardial infarction. About 60% of patients may have antecedent arthralgias, malaise, fevers, sweats, or chills consistent with viral infections 1 to 2 weeks before onset. Here, we report a postpartum young woman who developed postural hypotension as the first manifestation of fulminant myocarditis with initially acute “cold and dry” right-sided heart failure and cardiogenic shock. Common causes of postural hypotension include volume depletion, medications, diabetes, alcohol, infection, and varicose veins as well as dysautonomic syndromes. Fulminant myocarditis can cause cardiogenic shock. Myocardial inflammation more frequently affects localized areas of the left ventricle free wall, rarely right ventricle (RV). However, predominant RV involvement with acute right-sided heart failure and low cardiac output syndrome can be easily overlooked due to lack of typical heart failure signs. On reviewing medical literatures, we had found no report regarding the RV involvement with acute right-sided heart failure as the initial presentation of fulminant myocarditis.