CARDIOVASCULAR MEDICINE

Long term follow up of children with myocarditis treated by immunosuppression and of children with dilated cardiomyopathy

M Giulia Gagliardi¹, M Bevilacqua¹, C Bassano⁶, B Leonardi¹, R Boldrini², F Diomedi Camassei², A Fierabracci⁴, A G Ugazio³, G F Bottazzo⁵

¹ Departments of Cardiology and Cardiac Surgery, Ospedale Pediatrico Bambino Gesù, Scientific Institute, IRCCS, Rome, Italy
² Department of Pathology, Ospedale Pediatrico Bambino Gesù
³ Department of Paediatrics, Ospedale Pediatrico Bambino Gesù
⁴ Laboratory of Autoimmunity, Ospedale Pediatrico Bambino Gesù
⁵ Scientific Directorate, Ospedale Pediatrico Bambino Gesù
⁶ Department of Cardiac Surgery, Tor Vergata University, Rome, Italy

Correspondence to:
Correspondence to:
Dr Maria G Gagliardi
Dipartimento Medico-Chirurgico di Cardiologia, Ospedale Pediatrico Bambino Gesù, IRCCS, Piazza S Onofrio 4, 00165 Rome, Italy; gagliard{at}opbg.net

Objective: To describe the treatment and long term outcome after immunosuppressive treatment of children with myocarditis.

Methods and results: 114 patients with newly diagnosed dilated cardiomyopathy were divided into three groups, according to the histological pattern: group A, acute myocarditis; group B, borderline myocarditis; and group C, non-inflammatory cardiomyopathy. Groups A and B were treated with cyclosporine and prednisone in addition to conventional treatment. Survivors of the whole cohort were analysed for 13 year transplant-free survival and assessed for left ventricular function. Event-free survival at 13 years was 97 (3)% for group A, 70 (8)% for group B, and 32 (7)% for group C (p < 0.0001). It was 96 (4)% at one year and 83 (5)% at 13 years for the cumulated myocarditis group (A and B). Cardiac function recovered completely in 79% of survivors in group A, 64% in group B, and 36% in group C. The rate of complete recovery in the cumulated group (A and B) was 70%.

Conclusions: The high long term survival rate of this cohort of children with myocarditis is probably due to the effect of short term immunosuppression. This result differs from previously published series of conventionally treated children, whose survival probability at one year was about 60%.

Abbreviations: CHF, congestive heart failure; DCM, dilated cardiomyopathy; EMB, endomyocardial biopsy; LVEDV, left ventricular end diastolic volume indexed to body surface area; LVEF, left ventricular ejection fraction

Keywords: myocarditis; immunosuppression; idiopathic dilated cardiomyopathy

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