Orphanet J Rare Dis. 2019;14(1):132.

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

, , , , , , , , , ,

제1저자 최홍미 · 교신저자 김형관, 박성지

PMID 31182113 PMC6558675 DOI 10.1186/s13023-019-1083-8 IF 3.6 순환기내과

소속 (6)

Cardiovascular Center, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Gyeonggi-do, South Korea.
Division of Cardiology, Cardiovascular Center, Department of Internal Medicine, Seoul National University College of Medicine, Seoul, South Korea. cardiman73@gmail.com.
Division of Cardiology, Department of Medicine, Cardiovascular Imaging Center, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea. tyche.park@gmail.com.
Division of Cardiology, Cardiovascular Center, Department of Internal Medicine, Seoul National University College of Medicine, Seoul, South Korea.
Division of Cardiology, Department of Medicine, Cardiovascular Imaging Center, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea.
Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, USA.

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.

Keywords

Aortic regurgitationAortic valve surgeryBehcet’s diseaseParavalvular leakage

Figures

**Fig. 1** Representative case of Behcet’s disease-related aortic regurgitation. A 36-year-old male presented with Behcet’s disease-related severe aortic regurgitation. Pseudoaneurysm (asterisk) and interventricular septal dissection (arrow) between the right and left coronary cusps were clearly shown on transesophageal echocardiography (a, b), in the surgical field (c) and on computed tomography (**d-f**). R, right coronary cusp; L, left coronary cusp; N, non-coronary cusp; LCA, left coronary artery; RCA, right coronary artery

**Fig. 2** Summary of clinical outcomes of 35 patients with Behcet’s disease-related aortic regurgitation. Of the 35 patients who underwent aortic valve (AV) surgery, 18 developed paravalvular leakage (PVL) within 2 years after surgery. One patient died, and 7 patients survived without PVL. Along with 9 patients who had PVL at 2 years after surgery, 27 patients underwent the second surgery for PVL. Two patients died before the third surgery, and 7 developed PVL within 2 years. Of the 12 patients who underwent the third surgery, 4 developed PVL within 2 years after surgery and 2 developed PVL 2 years after surgery. Three patients died before the fourth surgery and 3 patients underwent the fourth surgery. One patient died, and 1 survived without PVL, and 1 developed PVL after 2 years but survived without surgery. A total of 76 AV surgeries were performed, and 29 PVLs developed within 2 years after AV surgery. * Including one heart transplantation. † The patient has survived with medical treatment 10 years after the fourth surgery

**Fig. 3** Temporal trends of outcomes according to the year of the first surgery. With time, there was a decreasing trend in (a) mean number of open heart surgeries (OHS) per patient, (b) paravalvular leakage (PVL) development, (c) all-cause mortality, and (d) cardiac mortality. Please note that clinical outcome has markedly improved since 2008, when unique echocardiographic findings suggesting severe Behcet’s disease-related aortic regurgitation were clinically introduced

**Fig. 4** Kaplan-Meier estimates of freedom from paravalvular leakage (PVL) stratified by (a) aortic root replacement (ARR) and (b) postoperative immunosuppressive therapy (IST). The incidence of PVL was significantly higher in patients who did not undergo postoperative IST or concomitant ARR

그림 출처: PubMed Central 공개 논문 (PMC6558675)

Conflict of interest statement

Prof. Hyung-Kwan Kim reports research grants from Actelion, Handok Pharmaceuticals, Dae-Woong Pharmaceuticals, Yuhan Pharmaceuticals and Norvatis.

그 밖의 정보

출판 형태: Journal Article
저널: Orphanet journal of rare diseases · eISSN 1750-1172
전자 출판: 2019-06-10
MeSH: Aortic Valve Insufficiency / mortality / physiopathology · Behcet Syndrome / mortality / physiopathology · Echocardiography · Female · Humans · Kaplan-Meier Estimate · Male · Proportional Hazards Models · Retrospective Studies · Risk Factors · Treatment Outcome
참고문헌: 23편