Recently, with the collaborative efforts of the Department of Cardiology, the Department of Rheumatology, ICU and other departments, the team led by Professor Miao Qi from the Department of Cardiac Surgery of PUMCH successfully performed a difficult re-operation on a patient with paravalvular leak due to Behcet’s disease. This, once again, brings hope to a family in distress.
The patient Mr. Hu is 51 years old this year. He was usually in good health and often took physical exercises after work until the end of last year, when he suddenly suffered chest tightness as severe as to the degree of suffocation. Work-up in the local hospital revealed severe aortic regurgitation, which means that the aortic valve, one of the “taps” in the heart, doesn’t close properly, causing blood to leak back and causing symptoms that must be eliminated with surgery.
Soon after the diagnosis, Mr. Hu underwent aortic valve replacement surgery in the local hospital, and the prior symptoms completely disappeared after the operation. But after a month, the suffocation-like symptom came back even worse and was accompanied by erratic heart beats. The detail he mentioned about “recurrent oral ulcers” caught the attention of doctors. It turned out that his severe aortic regurgitation was caused by vascular inflammatory changes attributable to Behcet’s disease, an autoimmune disease.
Behcet’s disease often presents with recurrent ulcers both in oral cavity and genital area, and a special type of skin rash. Patients often complain difficulties of wound healing after unwary cuts. Mythically, this disease may involve the aortic valve in some patients, if not all, which presented either acute laceration of valvular structure as like Mr. Hu’s, or more commonly seen chronic dilation of aortic root. If not discerned before the valve operation, the fate of implanted prosthetic valve is almost inevitably destined to severe paravalvular leak due to the inherent inflammatory processed brought by Behcet’s disease. Such inflammation usually not only impedes the fusion of the sewing tissue with the surrounding tissue which consequently leads to paravalvular leak, but also affects the heart conduction system, and results in erratic heart beats or even heart block which mandate pace maker insertion.
The local doctor told Mr. Hu that re-operation for patients with Behcet’s disease was extremely difficult and highly risky. This dealt a heavy blow to Hu’s family who had just made peace through the first operation. However, Hu’s paravalvular leak progressed very quickly, and in just half a month, the regurgitation progressed from mild to moderate to severe. If re-operation could not be carried out as soon as possible, he may suffer a sudden death any minute due to severe heart failure, aortic valve prolapse and obstruction and cardiac arrest, among others.
As a last resort, Hu came to Director Miao Qi through several rounds of referring. After being admitted to the hospital, Hu’s condition was very unstable. In addition to paravalvular leak, he also suffered from a series of symptoms such as heart failure, pneumonic infection, and arrhythmia. The bedside monitor sent off alerts almost non-stop. On one hand, the operation was urgent; on the other hand, it came with inevitably high odds of risk and even death. This dilemma posed a huge challenge to Dr Miao Qi’s team.
Considering Hu’s complex conditions and many complications, the team, as PUMCH was accustomed to, organized a multidisciplinary consultation that gave Hu comprehensive and professional advice. While multi-departments were racing against time for the consultation, the Department of Cardiac Surgery quickly completed all pre-operation evaluations and preparations for the patient.
On March 16, 2022, Hu was finally in an operation-ready state. However, unexpected difficulties happened almost as a routine during such operations. There was a rupture in the aortic root which was undetectable by the preoperative imaging modalities. And to make things worse, the inflammation wreaked havoc on the adjacent tissue and tremendously distorted the contour aortic root. Even if the new aortic valve could be sutured to the valve annulus, Hu still may not be able to make through the operation due to intractable massive bleeding at the aortic root. Even if he survived the operation by luck, the likelihood of recurrent paravalvular leak after surgery would still be very high.
Considering that the patient’s valve annulus had sunk into the left ventricular outflow tract, Dr. Miao skillfully reconstructed the outflow tract to provide a sufficiently strong anchoring zone for the new prosthetic valve and vascular grafts, and then successfully completed the aortic root and coronary artery reconstruction. The whole team brought out the best of their resolution, patience and skill to complete the operation after 8 hours of hard working.
Thanks to the efforts of the ICU medical staff, the tracheal intubation was removed for Hu on the same day of the operation and he was transferred back to the cardiac surgery ward on the next day. Gao Peng and Yang Deyan from the Department of Cardiology treated Hu’s arrhythmia by placing a pacemaker, clearing the last obstacle for his recovery. On the 12th day after the operation, Hu’s was discharged with his symptoms fully relieved.
Patients whose cardiovascular system is compromised by immune system diseases such as Behcet’s disease, Takayasu arteritis, and systemic lupus erythematosus often have complex conditions, which pose serious challenges to many cardiac surgeons. Tapping into PUMCH’s multidisciplinary strength, the Department of Cardiac Surgery has accumulated rich experience in the diagnosis and treatment of cardiac diseases combined with immune system diseases, which greatly reduces the risk of postoperative paravalvular leak in patients with Behcet’s disease.
Professor Miao Qi was operating on the patient.
Correspondent: Liu Jianzhou and Zhao Yanxue
Reporter: Hong Chengwei
Picture courtesy: Sun Liang
Translator: Liu Haiyan
Editor: Liu Xingrong and Wang Yao