A Behçet’s Disease Patient with Postoperative Perivalvular Leakage: A Case Report

Q: What is the clinical evidence from this study?

Study design: Case Report. Population: Cardiovascular Behçet's disease with severe aortic valve regurgitation and postoperative paravalvular leakage (n=1). Intervention: Aortic root replacement (Bentall procedure) with perioperative methylprednisolone and immunosuppressive therapy vs. Initial root-preserving aortic sinus repair and isolated aortic valve replacement without prior inflammation control. Primary outcome: Resolution of paravalvular leakage and interventricular septal dissection with long-term echocardiographic stability.

Q: What does this research mean for the field?

Aortic valve root replacement combined with perioperative immunosuppressive therapy can successfully resolve paravalvular leakage and left ventricular wall dissection following initial valve replacement in patients with cardiac Behçet's disease. Novelty: ClaimNovelty.INCREMENTAL. Consensus alignment: ConsensusAlignment.NEUTRAL.

Xinyu Pu; Liya Wei

doi:10.29328/journal.jcicm.1001060

November 6, 2026Journal of Clinical Intensive Care and MedicineOpen Access

A Behçet’s Disease Patient with Postoperative Perivalvular Leakage: A Case Report

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Why the study?

Behcet's disease is a rare chronic recurrent vasculitis, and this report aims to increase clinicians' awareness to enable timely diagnosis and treatment.

Key Points

To describe the diagnosis, surgical complications, and secondary management of a young female patient presenting with aortic valve involvement from Behçet's disease.
Clinical and histopathological evaluation of a 24-year-old female presenting with severe aortic regurgitation who underwent initial aortic valve replacement and repair.
Postoperative monitoring and subsequent aortic root replacement (Bentall procedure) accompanied by perioperative pulse methylprednisolone therapy.
Following initial root-preserving repair and aortic valve replacement, the patient developed a paravalvular leak and interventricular septal dissection 1.5 years postoperatively.
Following a secondary Bentall procedure combined with perioperative pulse methylprednisolone to suppress vascular inflammation, routine clinical follow-up remained uneventful through 2024.

Study Design

Type

Case Report (n=1)

Multicenter

No

Structured PICO

P

Population

1 female patient (age 24) with cardiovascular Behçet's disease and severe aortic regurgitation, followed for 7 years from initial emergency valve surgery in 2017 through reoperation and routine follow-up in 2024.

I

Intervention

Initial emergency surgery (aortic sinus aneurysm repair with double-layer autologous pericardial patch, mitral valve repair, aortic valve replacement, tricuspid valve repair using DEVAGE technique) followed by warfarin 6 mg/day, thalidomide 25 mg every night, cyclophosphamide for 0.5 years (total dosage ~10 g), and azathioprine 100 mg/day; subsequent redo surgery 2 years later consisting of ventricular septal aneurysm resection, aortic root replacement (Bentall procedure with 23# Regent valve and 32# VESCUTEK prosthetic vessel), and aortic valve paravalvular leakage repair with perioperative methylprednisolone 1000 mg/day for 3 days.

O

Outcome

Postoperative clinical stability and echocardiographic resolution of paravalvular leakage and left ventricular/interventricular septal dissection through 2024.

Limitations

Single-patient case report, so findings cannot be generalized to all patients with cardiovascular Behçet's disease
Limited microbiological investigation details available to definitively exclude infective endocarditis
Incomplete records regarding the exact duration of immunosuppressive therapy and the relationship between inflammatory activity and postoperative complications
The second surgical intervention was performed at an external hospital, limiting access to detailed operative and postoperative records
Describes the clinical course of a single patient; findings cannot be generalized to all patients with cardiovascular Behçet's disease
Limited available information regarding the microbiological investigations used to exclude infective endocarditis
Precise duration of immunosuppressive therapy and the relationship between inflammatory disease activity and postoperative complications could not be fully established from clinical records
Second surgical intervention was performed at another hospital, limiting access to detailed operative and postoperative records
Longer-term observations beyond 2024 are needed to assess the durability of the surgical outcome and risk of recurrent cardiovascular complications

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Implication

This case report highlights that in patients with cardiovascular Behçet's disease requiring aortic valve surgery, performing an aortic root replacement (Bentall procedure) alongside perioperative immunosuppressive therapy prevents recurrent paravalvular leakage and ventricular dissection compared to simple valve replacement.

Abstract

Behcet’s disease, a rare chronic recurrent vasculitis, affects various arteries and veins. A young female with aortic valve - involved Behcet’s disease had aortic valve replacement. Paravalvular leakage then occurred and quickly progressed to left ventricular wall dissection, leading to aortic valve root replacement. Current routine follow - up shows no significant issues. This article aims to boost clinicians’ awareness of Behcet’s disease for timely diagnosis and treatment of more patients.

Discussion

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Cite This Study

Pu et al. (2026) conducted a case report in Cardiovascular Behçet's disease with severe aortic valve regurgitation and postoperative paravalvular leakage (n=1). Aortic root replacement (Bentall procedure) with perioperative methylprednisolone and immunosuppressive therapy vs. Initial root-preserving aortic sinus repair and isolated aortic valve replacement without prior inflammation control was evaluated on Resolution of paravalvular leakage and interventricular septal dissection with long-term echocardiographic stability.

synapsesocial.com/papers/6af3463ebdf2424ebd04f843 https://doi.org/10.29328/journal.jcicm.1001060

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A Behçet’s Disease Patient with Postoperative Perivalvular Leakage: A Case Report

Key Points

Study Design

Structured PICO

Limitations

Ask about this paper

Implication

Abstract

Discussion

Cite This Study

A Behçet’s Disease Patient with Postoperative Perivalvular Leakage: A Case Report

Key Points

Study Design

Structured PICO

Limitations

Ask about this paper

Implication

Abstract

Discussion

Cite This Study

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