Acute respiratory distress syndrome with transiently impaired left ventricular function and Torsades de Pointes arrhythmia unmasking congenital long QT syndrome in a 25-yr-old woman.
We report a case of recurrent episodes of Torsades de Pointes arrhythmia in the setting of transiently impaired left ventricular ejection fraction, acute respiratory distress syndrome, transient hypokalaemia and QT-prolonging drugs, in a previously healthy 25-yr-old female patient. In the course of the clinical and genetic work-up this patient was newly diagnosed with a mutation in KCNH2 encoding the alpha-subunit of the human repolarizing potassium channel I(Kr). This case report illustrates the multivariate nature of long-QT syndrome, and emphasizes the usefulness of a pharmacological test for repolarization abnormalities.
Hinterseer
M
M
Department of Medicine I, Klinikum Grosshadern, LMU München, Germany. martin.hinterseer@med.uni-muenchen.de
Irlbeck
M
M
Ney
L
L
Beckmann
B-M
BM
Pfeufer
A
A
Steinbeck
G
G
Kaab
S
S
D000328:Adult; D000900:Anti-Bacterial Agents / Q000009:adverse effects; D000935:Antifungal Agents / Q000009:adverse effects; D000072237:ERG1 Potassium Channel; D004452:Echocardiography / Q000379:methods; D004917:Erythromycin / Q000009:adverse effects; D051638:Ether-A-Go-Go Potassium Channels / Q000235:genetics; D005260:Female; D015725:Fluconazole / Q000009:adverse effects; D006801:Humans; D007008:Hypokalemia / Q000150:complications / Q000503:physiopathology; D008133:Long QT Syndrome / Q000150:complications / Q000151:congenital* / Q000503:physiopathology; D009154:Mutation / Q000235:genetics; D012008:Recurrence; D012128:Respiratory Distress Syndrome / Q000209:etiology* / Q000503:physiopathology; D016171:Torsades de Pointes / Q000150:complications* / Q000503:physiopathology; D016896:Treatment Outcome; D018487:Ventricular Dysfunction, Left / Q000150:complications* / Q000503:physiopathology
D000900:Anti-Bacterial Agents; D000935:Antifungal Agents; D000072237:ERG1 Potassium Channel; D051638:Ether-A-Go-Go Potassium Channels; C000606913:KCNH2 protein, human; D004917:Erythromycin; D015725:Fluconazole