Abstract 4042: Epidemiology of Inflammatory and Non-inflammatory Dilated Cardiomyopathy: Initial Assessement From the KNHI Demonstrate Underdiagnosis of Inflammatory and Familial Cardiomyopathy
Data of selected registries and clinical studies of tertiary referal centers postulate in dilated cardiomyopathy (DCM) a familial and genetic factors in 25 to 40% and for cardiac inflammation and viral pathogenesis up to 50% of pts. The German national competence net on heart failure (KNHI) data base represents the “real world” of the epidemiology in different forms of heart failure. Methods: In the KNHI data base 2376 heart failure pts with an ejection fraction (EF)/=117% by echo were enroled after coronary artery disease, valvular heart disease and hypertension were excluded. Results: 1036 pts (236 female, 800 male) were diagnosed DCM. Endomyocardial biopsies (EMB) were carried out in only 266 pts (26%) to inflammation and viral persistence. Familial DCM was recognized in 81 (7,8%) of all DCM pts only. An inflammatory dilated cardiomyopathy (DCMi) could be ascertained in 135 pts (50,75% of the biopsied pts). Pericardial effusion (PE) was assessed by echo in 79 (7,6%) of the DCM pts indicative for an inflammatory process. In all other 1340 heart failure pts a pericardial effusion was seen less frequently (3,1%, P <0,001). In pts, who did not undergo EMB (n=770), perimyocarditis was suspected in only 214 individuals (20,65%). Thus a substantial part of the pts, at least 30,15%, were deprived of a correct aetiologic diagnosis and consequently also of specific treatment. There was no gender difference for DCMi when compared to DCM (P=0,409), but pts with DCMi were 8,4 years younger than pts with DCMI without inflammation (P<0,001). A complete left bundle branch block (LBB) was diagnosed in 346 pts with DCM (33,4%) and 43 pts with DCMi (31,9%) to a similar extent but less frequently in only 17 out of the 81 pts with familial DCM (21%; P=0,014 vs. non familial) and in the residual heart failure pts (n=328, 24,5%). Atrial fibrillation was more frequent in 185 of the 1036 DCM pts (17,9%), when compared to 17 of 135 pts with DCMi (12,6%) and 11 of 81 pts with familial DCM (13,6%). Conclusion: Even in the, real life“ of a specialised heart failure consortium, the final etiopathological diagnosis of the underlying cause of DCM remains unresolved in 74% of pts. This underlines the urgent need for a complete diagnostic work-up including EMB.