title: [Hereditary prolonged QT interval (Romano-Ward syndrome) in a female patient with non-elective cesarean section].
abstract: In the Romano-Ward syndrome, an inherited form of abnormally prolonged QT duration is the matter of concern. The frequency-corrected QTc value in the ECG amounts to more than 0.44 s (upper mean value: 0.44 s). Persons suffering from Romano-Ward syndrome are vitally endangered by attacks of arrhythmia, syncope, and cardiac arrest that are triggered or aggravated by stress or certain drugs. In obstetrical anesthesia, attention should be paid to the fetus, which, due to the genetic dominance, will have a 50% probability of having the disorder. A 28-year-old para I was admitted with cervical dilation on her predetermined date of delivery. Romano-Ward syndrome had been diagnosed 10 years earlier; the patient had been free of complaints and had received no treatment. Due to cephalopelvic disproportion and the cardiac syndrome, a primary cesarean section was performed. Following premedication and provision of a beta-blocker, defibrillator, and drugs for resuscitation, intubation anesthesia with methohexital, nitrous oxide, enflurane, and succinylcholine was carried out. The QTc values were pathologically prolonged (0.47 s; 0.48-063 s) during the pre- and intraoperative period. Hemodynamic irregularities did not occur in the perioperative period. The newborn was vital (3,830 g, 50 cm; Apgar scores 10/10/10; umbilical cord pH 7.29). Directly postpartum isolated supraventricular extrasystoles occurred, and the QTc duration increased to 0.51 s. Simultaneous registration of the ECG and phonocardiogram showed premature occurrence of the second cardiac sound, which is typical of Romano-Ward syndrome.
authors.affiliation: Institut für Anästhesiologie, Kinderklinik im Klinikum Bamberg.
authors.forename: J
authors.initials: J
authors.lastname: Plötz
authors.forename: H
authors.initials: H
authors.lastname: Heidegger
authors.forename: R
authors.initials: R
authors.lastname: von Hugo
authors.forename: H
authors.initials: H
authors.lastname: Grohmann
authors.forename: K H
authors.initials: KH
authors.lastname: Deeg
mesh_terms: D000328:Adult; D000769:Anesthesia, Inhalation; D000773:Anesthesia, Obstetrical; D002585:Cesarean Section; D005260:Female; D006801:Humans; D008133:Long QT Syndrome; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular
vernacular_title: Hereditär verlängertes QT-intervall (Romano-Ward-Syndrom) bei einer Patientin mit nicht-elektiver Sectio caesarea.