Hereditär verlängertes QT-Intervall (Romano Ward Syndrom) im geburtshilflichen Management

Zusammenfassung

Bei einer 28jährigen I Para mit Romano Ward Syndrom und Mißverhältnis wurde eine Schnittentbindung durchgeführt. Die Patientin war bis dahin beschwerdefrei und unbehandelt geblieben. Die potentielle Gefahr des Syndroms liegt in anfallsweise auftretenden Herzrhythmusstörungen, Synkopen und Herzstillstand. Das vitale Neugeborene zeigte ventrikuläre Extrasystolen, ebenfalls ein verlängertes QT-Intervall. Die Fallbeobachtung wird zum Anlaß genommen, das Krankheitsbild und geburtshilflich-anästhesiologische Probleme zu diskutieren.

Abstract

In the Romano-Ward syndrome, an inherited form of abnormally prolonged QT duration is a matter for concern. The frequency-corrected QT_c value in the ECG amounts to more than 0,44 s. Persons suffering from Romano-Ward syndrome are vitally endangered by attacks of arrhythmia, syncope and cardiac arrest which are triggered or aggravated by stress or certain drugs. In obstetrical anaesthesia, attention should be paid to the foetus, which, due to the genetic dominance, will have a 50% probability of suffering from 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 Caesarean section was performed. The QT_c values were pathologically prolonged (0.47 s; 0.48-0.63 s) during the pre- and intraoperative period. Haemodynamic 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 supraventriculär extra systoles occurred, and the QT_c duration increased to 0.51 s.