Question 3: What do you predict will be the clinical outcome in this patient?
Answer: The patient had DCM with severe left ventricular dysfunction, and an histological diagnosis of an infection-negative chronic autoimmune DCM that has per se a dismal prognosis. She did not respond to standard heart failure therapy with Ace-inhibitors, beta-blockade was contraindicated due to asthma and she was put on ivabradine 10 mg daily. After few months she developed intolerance to ACE-inhibitors (cough) and was put con losartan 100 mg per day. Female gender, left ventricular dysfunction at presentation, and autoimmune pathogenesis are all negative prognostic features in myocarditis (2,3,4,6). Her daughter had been successfully treated years before her mother with immunosuppressive therapy for severe peri-partum autoimmune myocarditis with cardiogenic shock. Based on these considerations, in keeping with a previous study showing beneficial response to azathioprine and steroids in inflammatory DCM with increased HLA expression on EMB (5) and with the expert indications of the Myocarditis Position statement (3), the patient was put on dual immunosuppressive therapy (prednisone 1 mg/kg/day and azathioprine 2 mg/kg/day) as an inpatient, then discharged on such therapy and received cardiological and immunological follow-up as an ambulatory patient. Consideration of ICD implant as primary prevention was delayed by 6 months, pending potential response to immunosuppression and recovery of LV function. Indeed LVEF at 6 months was improved up to 44% and ICD was not placed. Holter monitoring did not reveal significant supraventricular or ventricular repetitive arrhythmia. Immunosuppression was well tolerated. She is alive and well at 34 months follow-up, in NYHA I, has recovered a normal LV function on echocardiography. She is currently on a low dose well-tolerated maintenance immunosuppressive therapy (prednisone 2.5 mg every other day, mainly for asthma control, but azathioprine was stopped at 24 months follow-up). She is also on regular, life-long, combined immunological and cardiological follow-up. This is a case of infection-negative chronic autoimmune familial DCM in an elderly patient with an optimal response to mild (dual) immunosuppressive therapy, in spite of the dismal prognosis of this disease entity (2,3,6). This case supports the view of an early biopsy-proven diagnosis for safe, patient-tailored, etiology-specific therapy of autoimmune acute and chronic forms of myocarditis/DCM (3).
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