Complete AV Block, Underestimated and Highly Impacting Complication of Ebstein's Anomaly Surgery

Background: Ebstein's Anomaly (EA) is a rare congenital heart disease with an incidence of 0.5%. Throughout the last five decades, multiple surgical techniques for tricuspid valve (TV) surgery have been reported. Therefore, the aim of our study was to investigate outcome and risk factors for mortality and morbidity after surgery for EA.

Method: All patients (pts) with EA, included in the National Register for Congenital Heart Defects until initial query (March 2021) were enrolled in our retrospective study. All medical documents sent by hospitals, outpatient clinics, rehabilitation centers, or pts were included in our database. Of all participating institutions 32 hospitals performed TV surgeries in Germany (n = 26) and abroad (n = 6).

Results: All available data between 1973 and 2021 were reviewed. A total of 397 EA pts were enrolled (167 males and 230 females). Median age was 28 years. (2–79 years × 0.25 = 17 × 0.75 = 44). Also, 197 pts were subjected to TV surgery. Median age at first TV surgery was 13.3 years (0–61.9 years). Overall, 286 TV surgeries were performed (71.7% repair, 22.7% replacement, and 5.6% Starnes). A total of 71 pts underwent multiple procedures (maximum = 4). Median age of all TV surgeries was 14.3 years (0–63.8 years). Of 197 pts, 26 (13.2%) developed complete atrioventricular (AV) block requiring pacemaker implantation. Median age was 26.9 years (0.57–61.9 years). TV replacement has a 3.64 times higher risk of complete AV block than TV repair (p = 0.029). Age at first operation correlated with the incidence of complete AV block. Each increase in age by 10 years led to a 1.47 times higher incidence (p = 0,008). Cone reconstruction resulted in a slight reduction of complete AV block compared with monocusp repair (0.49x, p = 0,379). Moreover, 62% of pts, suffering complete AV block after TV surgery, underwent multiple procedures. Of these, 42% had previous TV surgery and 31% had to be reoperated (25% repair and 75% replacement). On long-term follow-up, 19% of pts with complete AV block died. Whereof 40% died within the first month after surgery.

Conclusion: Our registry analysis showed that after surgery for EA, a surprisingly high number of pts (13.2%) suffer from complete AV block which is a risk factor for early mortality. TV replacement has a higher risk for complete AV block compared with TV reconstruction. Higher patient age at the time of surgery is associated with complete AV block reflecting that the optimal timing has to be investigated.

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Artikel online veröffentlicht:
12. Februar 2022