Outcomes of Ebstein's Anomaly Patients Treated with Tricuspid Valvuloplasty or Tricuspid Valve Replacement

Abstract

Background:The incidence of Ebstein's anomaly is extremely low, and except for the Mayo Clinic, no cardiac center has reported on a sufficient number of patients. The aim of our study was to report the outcomes of Ebstein's anomaly patients treated with tricuspid valvuloplasty (TVP) or tricuspid valve replacement (TVR).Methods:TVP or TVR was performed in 245 patients from July 2006 to April 2016. We reviewed patients' records and contacted patients via outpatient service and over the telephone.Results:The mean follow-up time was 43.6 ± 32.6 months, and 224 (91.4%) patients underwent follow-up. The mean operative age was 31.2 ± 15.7 years. TVR was performed in 23 patients, and TVP was performed in 201 patients. The 30-day mortality rate was 1.3%, and the overall survival rate was 97.9% at 5 and 10 years. The early mortality rate of the TVP group was lower than that of the TVR group (0.5% vs. 8.7%, P = 0.028), and the overall mortality rate of the TVP group was lower than that of the TVR group, without statistical significance (1.0% vs. 8.7%). After propensity score matching, the rates of mortality and New York Heart Association class ≥ III were lower in the TVP group than those in the TVR group without statistical significance. Seven patients with Type B Wolff-Parkinson-White (WPW) syndrome underwent one-stage surgery, and arrhythmias disappeared. Six patients suffered from episodes of left ventricular outflow tract obstruction (LVOTO) during surgery. Severe LVOTO could be treated with reoperation of the atrialized right ventricle.Conclusions:Ebstein's anomaly patients treated with TVP or TVR can experience optimal outcomes with midterm follow-up. However, TVP should be the first-choice treatment. Optimal outcomes can be obtained from one-stage operation in patients with Type B WPW syndrome. Severe LVOTO during surgery might be related to improper operation of the atrialized right ventricle.

Results of intervention and surgical treatment of Wolff-Parkinson- White syndrome in patients with Ebstein's anomaly

“…Несмотря на имеющиеся технические трудности и высокий процент (7-30%) случаев возникновения рецидивов тахиаритмий, процедура РЧА ДПП выполнима, безопасна и эффективна у больных с аномалией Эбштейна и синдромом ВПУ и, по мнению ряда авторов, является первым этапом лечения в данной группе больных [15,16]. Применение новых методов картирования и аблации с использованием новых типов катетеров упрощает хирургическое вмешательство, позволяет значительно улучшить результаты РЧА, снизить количество рецидивов и тем самым добиться стабильных результатов в отдаленные сроки наблюдения [17][18][19]. Главным достоинством РЧА является относительная безопасность, минимальная травматичность и короткий реабилитационный период после операции.…”

Left heart matters in Ebstein’s anomaly: the importance of a closer follow up—a case report

“…Although the condition exhibits typical characteristics, each affected patient has different morphological and haemodynamic features, resulting in different natural histories. 1 Age of presentation and symptomatology depend on the anatomic and functional severity of the tricuspid valve abnormality as well as the presence of associated anomalies such as septal defects, and the arrhythmia burden. 2 The combination of EA and an atrial septal defect or patent foramen ovale is found in 60–70% of cases.…”

“… 2 The combination of EA and an atrial septal defect or patent foramen ovale is found in 60–70% of cases. 1–3 Wolff–Parkinson–White syndrome occurs in 10–29% cases, and results in supraventricular tachycardia in at least 15% of these. 1 …”

“… 1–3 Wolff–Parkinson–White syndrome occurs in 10–29% cases, and results in supraventricular tachycardia in at least 15% of these. 1 …”

“… 6 Li et al . 1 reported a large series of 245 patients who underwent tricuspid valve surgery. Six were found to have LVOTO at the time of surgery, none of whom had signs of it preoperatively.…”