Introduction: Giant cell myocarditis (GCM) is known as a rare, rapidly progressive, and frequently fatal myocardial disease in young and middle-aged adults. The most common early manifestations are heart failure, ventricular arrhythmias, and atrioventricular block, but GCM may also disguise as an acute myocardial infarction and rarely presents as an unexpected sudden cardiac death. The diagnosis of GCM rests fully on microscopy of the heart muscle and even in experienced centers >4 in 10 cases may escape detection until autopsy or cardiac transplantation
Case report: This case presents a 64-year old female with insignificant medical history who developed syncope while on holiday as a result of newly documented intermittent 3rd degree AV block. No chest pain or shortness of breath was reported before the event. Laboratory findings showed elevated levels of hsTnT (1086 ng/l, range 0–14) and BNP (2164 ng/l, range 10–155). Echocardiography revealed borderline left ventricle ejection fraction (LV EF 50–55%); coronary angiography revealed no pathology. Suspicion of myocarditis was raised and cardiac MRI and pacemaker implantation were considered. Progression of LV systolic dysfunction was documented (EF 45%) on the 4th post-admission day (PAD). One day after, implantation of 2D-PM was performed (persistent AV block). Subsequent progression of LV systolic dysfunction (EF 30%, diffuse hypokinesis) requiring levosimendan administration was reported on the 7th PAD. Endomyocardial biopsy revealed acute myocarditis with giant-cell granulomas. The progression of low cardiac output with incessant ventricle tachycardia required LVAD Levitronix CentriMag implantation on the 8th PAD, even after combined immunosuppressive therapy and inotropic support. On the 17th PAD, conversion to LVAD Heart Mate II was performed. The patient was indicated for radiofrequency ablation (RFA) for incessant ventricular tachycardia on the 60th PAD. Due to severe aortic regurgitation, which developed during the acute illness after LVAD implantation, TAVI was performed (transfemoral implantation with an EvolutR 23 mm valve) on the 138th PAD. 6 months after the initial signs of fulminant myocarditis, the patient underwent heart transplantation (HTx); 2 months later, after surviving a complicated postoperative course, she was repatriated to her country of origin in a healthy condition.
Summary: We report a rare case of GCM in a 64-year old, previously healthy female, complicated by rapid development of progressive acute heart failure requiring the comprehensive care of our heart team. Using a broad spectrum of therapeutic approaches (2D-PM, LVAD Levitronix, LVAD Heart Mate II, RF ablation, TAVI) (Image 1), the patient successfully underwent HTx.