Publikationsserver der Universitätsbibliothek Marburg
Titel:Verlaufsuntersuchung von 154 Patienten mit dilatativer Kardiomyopathie mit oder ohne Inflammation und mit oder ohne Viruspersistenz im ersten Jahr nach Diagnosestellung
Autor:Schwalb, Birte
Weitere Beteiligte: Maisch, Bernhard (Prof. Dr.)
Veröffentlicht:2014
URI:https://archiv.ub.uni-marburg.de/diss/z2014/0681
DOI: https://doi.org/10.17192/z2014.0681
URN: urn:nbn:de:hebis:04-z2014-06817
DDC: Medizin
Titel (trans.):Natural history investigation of 154 patients with dilated cardiomyopathy with or without inflammatory aetiology and with or without viral persistence during their first year after date of diagnosis
Publikationsdatum:2014-10-21
Lizenz:https://rightsstatements.org/vocab/InC-NC/1.0/

Dokument

Schlagwörter:
Herzmuskelerkrankung, dilatative Kardiomyopathie, dilated cardiomyopathy

Zusammenfassung:
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 DCM sowohl in ihrer Gesamtheit als auch in ätiologischen Subgruppen analysiert wurden. Die Einteilung erfolgte in Anlehnung an die aktuelle Definition der European Society of Cardiology in 1)Familiäre Kardiomyopathie 2)Nicht familiäre Form als a)reine DCM mit 82 Patienten b)DCM mit Viruspersistenz mit 38 Patienten c)inflammatorische DCM bei 17 Patienten d)DCM mit Inflammation und Viruspersistenz mit 15 Patienten. Diese wurden einer einjährigen Verlaufsbeobachtung klinischer, echokardiographischer und hämodynamischer Parameter unterzogen. Mit einer Verbesserung der mittleren EF von 29,3 auf 43,8% (p<0,001), einer Verringerung des mittleren LVEDD von 67,8 auf 61,5mm (p<0,001) und einer Überlebensrate von 95,9% konnte ein sehr erfreulicher klinischer Verlauf nachgewiesen werden. Dieser Verlauf war wesentlich günstiger, als es nach älteren Angaben der Literatur zu erwarten gewesen wäre und bestätigt die positive Entwicklung des klinischen Verlaufes in neueren Studien. In unserer Untersuchung fanden sich sogar 12 Patienten, deren EF und LVEDD sich normalisierten. Neben der Darstellung des klinischen Verlaufes ging die Untersuchung der Frage nach, durch welche Faktoren die dargestellte Entwicklung bedingt sein kann. Ursächlich für den erfreulichen klinischen Verlauf und die niedrige Mortalität dürfte die korrekte Einhaltung einer leitliniengerechten medikamentösen Herzinsuffizienztherapie (98,7% der Patienten hatten einen Betablocker und/oder einen ACE-Hemmer/AT1-Antagonist erhalten) und die Durchführung einer ätiologiespezifischen Therapie bei 80% der Patienten sein. Des Weiteren hatten Patienten mit besonders schlechten Herzleistungen und weiteren leitlinien- bzw. empfehlungsgemäßen Parametern wie QRS-Verbreiterung und NYHA-Stadien ICDs und/oder CRTs erhalten. Bei vier der ICD-Patienten konnten arrhythmische Ereignisse adäquat therapiert werden. CRT-D-Patienten konnten ihre initial schlechtere Herzleistung an die der ICD-Patienten angleichen, so dass die rechtzeitige Versorgung dieser Patienten mit ICD und/oder CRT sicherlich ebenfalls zur Besserung der Herzleistung und Reduzierung der Mortalität beigetragen hat. Darüber hinaus spielt die enge Anbindung an unser Zentrum mit regelmäßigen Verlaufsuntersuchungen und die Sensibilisierung für die Erkrankung im Zusammenhang mit den Familienuntersuchungen eine große Rolle. Hierdurch konnten medikamentöse Therapieanpassungen vorgenommen werden und frühzeitige Indikationsstellungen zur Therapieausweitung wie ICD- bzw. CRT-Implantation erfolgen. In der Subgruppenanalyse konnten Patienten nachverfolgt werden, die sich in ihrem klinischen Verlauf unterschieden. Hinsichtlich der Ätiologie fanden wir bei Patienten mit Viruslast eine schlechtere kardiale Pumpfunktion, häufiger Linksschenkelblöcke und häufiger initiale kardiale Dekompensationen als bei DCM-Patienten anderer Ätiologie (mit Inflammation oder ohne Virusbefall). In der Literatur wird das Vorhandensein einer Viruslast wie auch einer Inflammation kontrovers diskutiert. Patienten mit einer familiären DCM hatten, wie auch in anderen Untersuchungen der Literatur beschrieben, einen tendenziell schlechteren klinischen Verlauf als Patienten mit einer sporadischen Form (EF bei Follow-up fDCM=40,7% vs. sDCM=44,6%, p=0,1945; LVEDD bei Follow-up fDCM=64,3mm vs. sDCM=60,7mm, p=0,007). Kardiale Dekompensationen im Untersuchungszeitraum betrafen Patienten, die bereits zum Einschlusszeitpunkt einen signifikant schlechteren LVEDD hatten (LVEDD 74,1mm vs. 67,3mm, p=0,0232). Dies waren deutlich jüngere Patienten und zu einem größeren Anteil Patienten mit einer familiären DCM. Ein lebensbeendendes oder potentiell lebensbeendendes Ereignis (Herztransplantation, Tod, ICD-Schock) trat bei Patienten auf, die bereits bei Untersuchungsbeginn eine tendenziell schlechtere EF, einen größeren LVEDD und ein höheres NYHA-Stadium hatten (EF 26,9% vs. 29,1%, n.s.; LVEDD 70,2mm vs. 67,5mm, n.s.; NYHA 2,6 vs. 2,4 n.s.). Der Anteil an Patienten mit viraler DCM war unter diesen Patienten fast doppelt so groß wie unter den ereignisfreien Patienten. Patienten mit einem LSB zum Einschlusszeitpunkt hatten tatsächlich eine schlechtere EF als Patienten mit normaler QRS-Breite (EF 25,2% vs. 30,2%, p=0,0026). Aber sogar diese Patienten konnten ihre EF im Laufe des Jahres signifikant verbessern. Insgesamt konnten wir einen günstigen Verlauf der DCM bei einer gut charakterisierten Patientengruppe im ersten Jahr nach Diagnosestellung feststellen. Damit konnte diese Untersuchung einen Beitrag zur Entwicklung der DCM unter optimaler medikamentöser und apparativer Therapie leisten.

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