Predictors of paravalvular aortic regurgitation after surgery for Behcet’s disease-related severe aortic regurgitation

Hong-Mi Choi,
Hyung-Kwan KimEmail author View ORCID ID profile,
Sung-Ji ParkEmail author,
Hyun-Jung Lee,
Yeonyee E. Yoon,
Jun-Bean Park,
Yong-Jin Kim,
Goo-Young Cho,
In-Chang Hwang,
Dae-Won Sohn and
Jae K. Oh

Orphanet Journal of Rare Diseases201914:132

https://doi.org/10.1186/s13023-019-1083-8

Received: 3 March 2019
Accepted: 26 April 2019
Published: 10 June 2019

Abstract

Background

Behcet’s disease (BD)-related aortic regurgitation (AR) is known to be associated with paravalvular leakage (PVL) after successful aortic valve (AV) surgery. This study aimed to determine predictors of PVL after successful AV surgery in BD patients. We retrospectively collected data of 35 patients (42.1 ± 9.1 years, 27 men) who underwent surgery for severe BD-related AR at two tertiary centers. The diagnosis was established based on echocardiographic, surgical, and/or pathological findings in conjunction with the International Study Group criteria for BD. A total of 76 cases of AV surgery in 35 patients were analyzed.

Results

A median follow-up duration was 8.0 years (interquartile range, 5.4–14.3 years). PVL developed in 18 patients (51.4%) within 2 years after the first surgery. Six patients who met the diagnostic criteria for BD did not develop PVL, in whom 5 patients took immunosuppressive therapy (IST). However, 4 of 9 patients (44.4%) who did not meet the diagnostic criteria developed PVL, in whom four (44.4%) patients took IST. On multivariable analysis, postoperative IST and concomitant aortic root replacement (ARR) were two independent predictors for less PVL development (HR 0.38, 95% CI 0.17–0.89, p = 0.025 for postoperative IST; HR 0.17, 95% CI 0.08–0.36, p < 0.001 for concomitant ARR). Preoperative IST use did not determine PVL development (p = 0.75).

Conclusions

Postoperative, but not preoperative, IST and concomitant ARR were independent predictors of less development of PVL. Special attention is required for early diagnosis BD-related AR, especially in patients not satisfying the current diagnostic criteria.