Verlaufsuntersuchung von 154 Patienten mit dilatativer Kardiomyopathie mit oder ohne Inflammation und mit oder ohne Viruspersistenz im ersten Jahr nach Diagnosestellung

Diese Untersuchung zeigte den klinischen Verlauf von 154 Patienten mit dilatativer Kardiomyopathie im ersten Jahr nach Diagnosestellung. Für diese Fragestellung war von Bedeutung, dass in diesem vom BMBF im Kompetenznetz Herzinsuffizienz und von Eurogene geförderten Projekt die 154 Patienten einer...

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Bibliographic Details
Main Author: Schwalb, Birte
Contributors: Maisch, Bernhard (Prof. Dr.) (Thesis advisor)
Format: Dissertation
Published: Philipps-Universität Marburg 2014
Innere Medizin
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Table of Contents: This investigation showed the natural history of 154 patients with dilated cardiomyopathy during their first year after date of diagnosis. We detected a favorable outcome with an improvement of EF from 29.0 to 43.8% (p<0.001), an improvement of LVEDD from 67.7 to 61.5mm (p<0.001) and a one-year-survival rate of 95.9%. This development was more favorable than we would have expected from older referenced literature. We even detected 12 patients with a normalization of EF and LVEDD. In addition to the presentation of the natural history we discussed, which factors were responsible for the favorable outcome and found as a major factor medical treatment in accordance to the medical guidelines (98.7% of the patients had a therapy with beta blockers and/or ACE-inhibitors/AT1-reuptake-inhibitors). In addition the specific therapy according to the aetiology (taken by 80% of the patients with a viral or inflammatory etiology) was also responsible for the favorable outcome. Interestingly, especially women and patients with a familial DCM decided to take an aetiology specific therapy. Furthermore, patients with a reduced cardiac output and other additional parameters like longer QRS duration or higher NYHA state had an implanted ICD and/or CRT. Four ICD-patients suffered from an arrhythmic event and got a sufficient antiarrhythmic treatment. CRT-D-patients were able to improve their reduced cardiac output to the same level of ICD-patients. This means that an early implantation of ICD and/or CRT is also responsible for the improvement of the cardiac outcome and the decrease of the mortality. We also found that the bond between our institution and the patient with regular visitations and examinations and the education of the disease in the context of familial examinations played a role for the outcome of the disease. We could adapt the medical treatment on short notice and could find the indication for an implantation of an ICD and/or CRT in time. Moreover, we identified subgroups of patients with different natural histories. As for the aetiological subgroups, patients with a viral disease had a worse ejection fraction, more frequently left bundle branch blocks, and more frequently cardiac decompensations when compared to patients with other aetiologies. Patients with a familial DCM had a worse natural history than patients with a sporadic DCM, as also described in the literature (EF at follow-up of patients with fDCM 40.7% vs. patients with sDCM 44.6%, p=0.1945; LVEDD at follow-up of patients with fDCM 64.3mm vs. patients with sDCM 60.7mm, p=0.007). Patients who suffered from a cardiac decompensation during the time of investigation had a significantly larger LVEDD at the date of inclusion (LVEDD 74,1mm vs. 67,3mm, p=0,0232). These patients were younger and had more often a familial DCM. A life ending or near life ending event (heart transplantation, death, ICD-shock) was observed in patients who had a worse EF, a larger LVEDD, and a higher NYHA state at the date of inclusion (EF 26.9% vs. 29.1%, n.s., LVEDD 70.2mm vs. 67.5mm, n.s., NYHA state 2.6 vs. 2.4, n.s.). In this group of patients, those with viral disease was twice as large when compared to patients without major cardiac events. Patients with a left bundle branch block at the date of inclusion had a worse EF than patients with normal QRS-duration (EF 25.2% vs. 30.2%, p=0.0026); although even these patients could also improve their EF during the time of investigation significantly. Altogether we detected a favorable outcome of the natural history of DCM for a precisely characterized group of patients during their first year after diagnosis. Therefore, this investigation could make a significant contribution to the knowledge of the natural history of DCM under ideal medical treatment and pacemaker therapy, heart transplantation or implantation of an artificial heart.