Mildly dilated congestive cardiomyopathy. Use of prospective diagnostic criteria and description of the clinical course without heart transplantation.

Abstract

Prognosis in classically described dilated congestive cardiomyopathy has been reported to be related to ventricular size. Mildly dilated congestive cardiomyopathy (MDCM) has been defined as end-stage heart failure of unknown etiology (New York Heart Association class IV, left ventricular ejection fraction less than 30%), occurring with neither typical hemodynamic signs of restrictive myopathy nor significant ventricular dilatation (less than 15% above normal range). The present study includes follow-up in 12 nontransplant patients. In the first 4 months after diagnosis, two patients improved and are living, and two showed cardiac dilation and clinical deterioration and died. Six of the remaining eight with persistent MDCM died (four with intractable heart failure and two, sudden deaths) without change in ventricular size before death, despite medical therapy over 20±8 months. Eight comparable transplanted patients with persistent MDCM demonstrated improved total survival by life table analysis (p<0.05). A family history of congestive cardiomyopathy was found in nine of 16 patients (56%) with persistent MDCM. Nontransplant patients were older (p<0.02), but other findings were similar in the two groups. Endomyocardial biopsies available in 14 of 16 cases showed little or no myofibrillar loss in spite of severe hemodynamic impairment. The degree of myofibrillar loss did not correlate with hemodynamic parameters but showed good correlation with left ventricular size, that is, five of six patients with no myofibrillar loss had normal ventricular size, whereas all eight patients with mild myofibrillar loss had mild cardiomegaly (p<0.002).Our current experience suggests a somewhat variable but negative prognosis after prospective diagnosis of MDCM, with poor survival in patients with persistence of the original diagnostic features during follow-up. Preservation of heart size in MDCM is probably related to lack of significant myofibrillar loss. Thus, irrespective of heart size or myofibrillar preservation on biopsy, heart transplantation should be strongly considered in MDCM if signs of severe cardiac dysfunction persist despite therapy. (Circulation 1990;81:506-517) V entricular dilation is considered the earliest and most distinguishing morphologic feature of congestive cardiomyopathy, which is often called "dilated cardiomyopathy."1-5 Lack of significant ventricular dilation is typically found in hypertrophic and in restrictive cardiomyopathies.1,24-8 Restrictive cardiomyopathy has also been termed "nondilated cardiomyopathy" by some authors to emphasize its morphological appearance and differentiate it from dilated

Long-Term Prognosis of Fulminant Myocarditis Rescued by Percutaneous Cardiopulmonary Support Device

“…Previously reported echocardiographic manifestations of fulminant myocarditis include global and regional systolic and diastolic dysfunction 2,5,6,10-12,14 and lack of LV dilatation. 2,5,6,13,14 Felker et al reported that 14 patients with fulminant myocarditis presented with non-dilated, thickened, hypocontractile ventricles in the acute phase, but showed significant improvement in LV contraction 6 months after discharge, 14 and their findings are generally consistent with ours, although none of their patients was treated with PCPS.…”

Changes in myocardial cytoskeletal intermediate filaments and myocyte contractile dysfunction in dilated cardiomyopathy: an in vivo study in humans

“…Some investigators have found histological changes that appear to be related to left ventricular haemodynamics and prognosis,12 while others have failed to show such a correlation 213 14 Nevertheless, in recent years specimens obtained at endomyocardial biopsy have proved useful in exploring the cellular and molecular abnormalities of the failing human myocardium in vivo 1516 …”

Prognostic Significance of Non-Dilated Left Ventricular Size and Mitral Regurgitation in Patients With Dilated Phase of Hypertrophic Cardiomyopathy

“…12,19) On the other hand, several investigators have reported that the prognosis of DCM patients with mild LV dilatation is variable and that end-stage heart failure occurs in a subgroup of patients with DCM despite mild LV dilatation. [20][21][22][23] We previously reported that DCM patients with mild LV dilatation have two important features: one group of patients with early and mild DCM may stabilize or improve through the natural course or with appropriate medi- 71.0 ± 20.6 (n = 5) 64.5 ± 20.6 (n = 15) 0.546 E/Ea septal 12.0 ± 3.3 (n = 2) 15.9 ± 7.0 (n = 7) 0.479 Laboratory data BNP, pg/mL 700.1 ± 532.5 (n = 3) 328.5 ± 307.1 (n = 10) 0.144 Creatinine, mg/dL 1.03 ± 0.49 (n = 3) 0.93 ± 0.16 (n = 10) 0.753 Sodium, mmol/L 140.7 ± 3.2 (n = 3) 139.9 ± 2.5 (n = 11) 0.667 Total bilirubin, mg/dL 1.30 ± 1.13 (n = 2) 0.72 ± 0.22 (n = 11) 0.599…”