During the second annual Houston Shock Symposium, Parvathi Mudigonda, MD, a cardiology fellow at the University of Cincinnati, presented a case study on a patient with giant cell myocarditis (GCM). The patient had a successful recovery with left ventricular support alone.
GCM, Dr. Mudigonda explained, is a rare cause of fulminant myocarditis often associated with infectious and autoimmune diseases. In addition to fulminant myocarditis, other clinical features of GCM include cardiogenic shock, biventricular failure, ventricular arrhythmias, and heart block. Without transplantation, it is often fatal.
Giant cells “are very large cells with a large amount of cytoplasm and peripherally clustered nuclei either in a horseshoe shape or circumferentially,” Dr. Mudigonda said.
“When histiocytes, which are tissue macrophages, are overwhelmed trying to phagocytose their target, they basically call for backup and fuse together,” she said. GCM may be mistaken for sarcoid, but in GCM, the amount of necrosis is significantly greater than the poorly formed granulomas.
The present patient was a 62-year-old black female regularly seeing her primary care provider, with mild comorbidities including type 2 diabetes mellitus, hyperlipidemia, morbid obesity, and borderline hypertension. The patient is a former smoker who now uses an electronic cigarette. The patient presented at the onset of flu season with symptoms including new onset of cough, wheezing, exertional dyspnea, orthopnea, and severe fatigue. Her primary doctor treated her with a Zpak and steroid taper. The initial treatment was ineffective, and within hours of presenting to an outside hospital with new onset heart failure, the patient progressed to fulminant cardiogenic shock. An echocardiography revealed severe biventricular dysfunction with mild left ventricular hypertrophy (LVH). She was eventually transferred to Dr. Mudigonda’s institution, with worsening shock and rising troponin.
“She was started on dual inotrope support with milrinone and dobutamine, and she had some marginal improvement for about 36 hours,” Dr. Mudigonda said. He then said she progressed to refractory cardiogenic shock; her urine output began tapering, and her Lactate elevated.
The patient’s presentation indicated that she may have had fulminant myocarditis, and she received intravenous high dose steroids on day three. A multidisciplinary discussion regarding serious treatment options “eventually boiled down to either [extracorporeal membrane oxygenation {ECMO}] or [left ventricular assist device {LVAD}],” Dr. Mudigonda said. “Given the low likelihood of improvement within the next ten days, and even lower chance of obtaining a donor heart, the decision was made to proceed directly to durable LVAD support.” On day four, the patient received a HeartMate II.
After it was confirmed that the patient had GCM, she began receiving cyclosporine in addition to the 40 mg daily dose of prednisone she was already taking. During recovery, she sustained severe right ventricular failure, which improved significantly in one month after treatment with pulmonary vasodilators. Within two months, left ventricular function normalized.
“What was initially a bridge to transplantation became a bridge to recovery,” Dr. Mudigonda said.
The discovery of a renal mass changed the course of immunosuppression to 20 mg daily of prednisone. But today, six months later, the patient is alive and home, doing well in cardiac rehab with a good quality of life. Current discussions focus on removing her renal mass.
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