title: Peripartum cardiomyopathy presenting with syncope due to Torsades de pointes: a case of long QT syndrome with a novel KCNH2 mutation.
abstract: Peripartum cardiomyopathy (PPCM) is a cardiomyopathy of unknown cause that occurs in the peripartum period. We report a case of PPCM presenting with syncope 1 month after an uncomplicated delivery. Electrocardiography showed Torsades de pointes (TdP) and QT interval prolongation. Echocardiography showed left ventricular systolic dysfunction and endomyocardial biopsy showed myocyte degeneration and fibrosis. Administration of magnesium sulfate and temporary pacing eliminated recurrent TdP. Genetic analyses revealed that recurrent TdP occurred via electrolyte disturbance and cardiac failure due to PPCM on the basis of a novel mutation in KCNH2, a gene responsible for inherited type 2 long QT syndrome.
authors.affiliation: Department of Cardiology, National Hospital Organization, Kure Medical Center and Chugoku Cancer Center, Japan.
authors.forename: Orie
authors.initials: O
authors.lastname: Nishimoto
authors.forename: Morihiro
authors.initials: M
authors.lastname: Matsuda
authors.forename: Kei
authors.initials: K
authors.lastname: Nakamoto
authors.forename: Hirohiko
authors.initials: H
authors.lastname: Nishiyama
authors.forename: Kazuya
authors.initials: K
authors.lastname: Kuraoka
authors.forename: Kiyomi
authors.initials: K
authors.lastname: Taniyama
authors.forename: Ritsu
authors.initials: R
authors.lastname: Tamura
authors.forename: Wataru
authors.initials: W
authors.lastname: Shimizu
authors.forename: Toshiharu
authors.initials: T
authors.lastname: Kawamoto
mesh_terms: D000328:Adult; D002304:Cardiac Pacing, Artificial; D009202:Cardiomyopathies / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D000072237:ERG1 Potassium Channel; D004562:Electrocardiography; D051638:Ether-A-Go-Go Potassium Channels / Q000235:genetics*; D005260:Female; D005355:Fibrosis; D006801:Humans; D008133:Long QT Syndrome / Q000175:diagnosis* / Q000235:genetics* / Q000503:physiopathology; D008278:Magnesium Sulfate / Q000627:therapeutic use; D009154:Mutation / Q000235:genetics*; D032383:Myocytes, Cardiac / Q000473:pathology; D058725:Peripartum Period; D013575:Syncope / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D016171:Torsades de Pointes / Q000150:complications* / Q000503:physiopathology / Q000628:therapy; D016896:Treatment Outcome; D018487:Ventricular Dysfunction, Left / Q000503:physiopathology
chemical_list: D000072237:ERG1 Potassium Channel; D051638:Ether-A-Go-Go Potassium Channels; C000606913:KCNH2 protein, human; D008278:Magnesium Sulfate