Dilated cardiomyopathy syndrome: nosological diagnosis as the basis of differential treatment
AbstractAim – to develop a comprehensive clinical and mor- phological approach to the nosological diagnosis and differential treatment of dilated cardiomyopathy (DCM). The trial enrolled 130 patients with DCM (mean age 46,89±12,50 years, 41 females), the mean LVEDD 6,6±0,9 cm, EF 29,2±10,7%, systolic pulmonary artery pressure (SPAP) 44,8±16,5 mm Hg. The control group consisted of patients with non-inflammatory heart disorders (EDD<6,0 cm, EF > 50%) who underwent openheart surgery (n=35, mean age 56,94±10,51 years) as well as healthy volunteers and patients with gastrointestinal disorders without heart disease (n=16, mean age 36,63±8,71 years). We performed viral serologic testing and the detection of anti-heart antibodies. 42 (32,3%) patients underwent morphological examination of the myocardium with PCR analysis. Having compared the morphological examination and noninvasive cardiac testing data we developed the algorithm of nosological diagnosis: isolated myocarditis was diagnosed in 63%, its combination with genetic disorders in 17%, genetic cardiomyopathy in 9%, in 11% the state was assessed as a primary (idiopathic) cardiomyopathy. The anti-heart antibodies level had the high- est correlation with the biopsy data. Based on individual indications the patients received basic treatment for myocarditis (acyclovir/ganciclovir, intravenous immunoglobulin (IVIG), hydroxychloroquine 200 mg/day, methylprednisolone 32 [20, 40] mg/day, azathioprine 108,3±34,2 mg/day, the duration of steroid treatment was 6 [4, 13] months). The efficacy of antiherpetic therapy (up to PCR negative blood) was 79,3%. Only as a result of the immunosuppressive therapy EF has sig- nificantly increased, left ventricular size and SPAP have decreased. Compared to untreated patients with myo- carditis, mortality and hospitalization frequency per year were significantly lower (13,8 and 31,6%, р=0,032, RR 0,63, 95% CI 0,37–1,05 and 0,69 и 1,29, р=0,001 re- spectively) with initially somewhat larger EF and lower NYHA class of heart failure. The risk of death of viruspositive patients with inflammatory cardiomyopathy is 1,95 times higher than that of virus-negative patients (95% CI 1,36–2,79, mortality 33,3 и 8,3%, p<0,01) despite the absence of the significant baseline differences. At the same time steroid therapy of both virus-positive and virus-negative patients has led to a prominent EF increase, left ventricular size and SPAP reduction and to decreasing trend of mortality risk. Mortality in patients with dilated cardiomyopathy was 20,8% with the average follow-up of 12,0 [5, 22] months. The integrated clinical and morphological approach allows us to make a nosological diagnosis in most patients with DCM and conduct effective antiviral and immunosuppressive therapy in patients with diagnosed myocarditis. Immunosuppressive therapy is warranted both in virus-negative and viruspositive patients with a high degree of immune activity after maximum possible suppression of the viral infection.
Keywords:dilated cardiomyopathy, myocarditis, endomyocardial biopsy, anti-heart antibodies, PCR diagnosis, immunosuppressive therapy
Clin. Experiment. Surg. Petrovsky J. – 2014. – N 1. – Р. 29–41.