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J Thorac Cardiovasc Surg 1997;114:131-132
© 1997 Mosby, Inc.

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AORTIC VALVE REPLACEMENT IN RELAPSING POLYCHONDRITIS

Paris, France

From the Department of Thoracic and Cardiovascular Surgery, Hopital Bichat, Paris, France.

Received for publication Oct. 21, 1996 accepted for publication Nov. 4, 1996. Address for reprints: Loïc Lang-Lazdunski, MD, Service de Chirurgie Thoracique et Cardiovasculaire, Hopital Bichat, 46 rue Henri Huchard, 75877 Paris cedex 18, France.

Relapsing polychondritis is a rare inflammatory multiorgan disorder affecting cartillaginous structures and other connective tissues. More than 600 cases have been reported to date in the literature. Serious cardiovascular complications appear in about 25% of patients with relapsing polychondritis, the most frequent being aortic or mitral regurgitation (11.1%) and aortic aneurysms (6.1%). We report the first Bentall-type operation in a patient with relapsing polychondritis and review the literature to point out the factors that influenced morbidity and mortality of patients requiring aortic valve replacement to determine the ideal surgical procedure for patients with relapsing polychondritis.

Case report.

A 39-year-old man with relapsing polychondritis (RP) for 4 years and requiring permanent corticoid and immunosuppressive therapy for severe bilateral scleritis was admitted to our institution for recent shortness of breath. Severe aortic regurgitation associated with moderate aneurysmal dilatation of the ascending aorta (46 mm in diameter) were diagnosed, and the patient was referred to our department for surgical correction.

He underwent operation on February 24, 1995. The surgical procedure consisted of a Bentall-type operation with a No. 25 mechanical valvular prosthesis, with the coronary arteries being reimplanted with the use of the button technique. The postoperative course was uneventful, and the patient was discharged home on day 30. Histologic examination of the aortic root showed acute panaortitis lesions: the intima was thickened, and the media showed extensive loss and fragmentation of elastic fibers. There were also perivascular mononuclear cell infiltrates in the outer media and in the adventitia. The aortic valve was thickened and contained inflammatory mononuclear cell infiltrates.

Eighteen months after the operation, the patient was without symptoms with a regimen of prednisone 25 mg/day and a permanent immnunosuppressive therapy. Doppler echocardiography showed a well-functioning aortic prosthesis, no paraprosthetic leak, no false aneurysm, and a remaining thoracic aorta of normal caliber.

Discussion.

Although the first aortic valve replacement in a patient with RP was reported in 1967, the ideal surgical procedure for such patients still remains to be determined. An extensive review of the literature reported by us in 1995 disclosed only 21 cases of aortic valve replacement, associated with mitral valve replacement in six and supracoronary graft replacement of the ascending aorta in three. ¹ All patients had aortic insufficiency related to aortic root enlargement (77.7%) or exclusive valve involvement (22.3%) or both mechanisms. All patients with aortic root involvement exhibited macroscopic or microscopic signs of aortitis. Six patients had an aneurysm of the ascending aorta within a mean 2.2-year postoperative delay. Three patients underwent reoperation for aortic prosthesis dehiscence with an operative mortality of 66.6%; the three others died. In these patients, there is no doubt that corticosteroid therapy contributed to aortic tissue fragilization that resulted in suture leakage and prosthesis dehiscence. Also, two additional patients probably died of a ruptured ascending aorta within a 2-year postoperative delay (sudden death, no autopsy obtained). ¹

Consequently, we recommend a prophylactic Bentall-type operation in all patients with RP requiring aortic valve replacement. This procedure carries a minimal risk of morbidity/mortality and has proven to be safe and efficient in other diseases with aortic involvement, such as Marfan syndrome. ^2-5 Furthermore, we believe that the coronary button reimplantation technique associated with the exclusion technique for the aortic anastomoses is the most reliable method for prevention of bleeding and late false aneurysm formation at suture lines because all diseased aortic tissue is eliminated. ⁵ This option may prevent precoce or late anastomosis dehiscence and thus avoid further high-risk reoperations in fragile immunosuppressed patients.

References

1. Lang-Lazdunski L, Hvass U, Paillole C, Pansard Y, Langlois J. Cardiac valve replacement in relapsing polychondritis. A review. J Heart Valve Dis 1995;4:227-35.[Medline]
   
2. Gott VL, Pyeritz RE, Cameron DE, Greene PS, McKusik VA. Composite graft repair of Marfan aneurysym of the ascending aorta. Results in 100 patients. Ann Thorac Surg 1991;52:38-45.[Abstract]
   
3. Cohn L, Rizzo RJ, Adams DH, et al. Reduced mortality and morbidity for ascending aortic aneurysm resection regardless of cause. Ann Thorac Surg 1996;62:463-8.[Abstract/Free Full Text]
   
4. Lewis CTP, Cooley DA, Murphy MC, Talledo O, Vega D. Surgical repair of aortic root aneurysms in 280 patients. Ann Thorac Surg 1992;53:38-46.[Abstract]
   
5. Kouchoukos NT, Wareing TH, Murphy SF, Perrillo JB. Sixteen-year experience with aortic root replacement. Results in 172 operations. Ann Surg 1991;214:308-20.[Medline]
   

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This Article Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Ulrik Hvass Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Lang-Lazdunski, L. Articles by Hvass, U. Search for Related Content PubMed PubMed Citation Articles by Lang-Lazdunski, L. Articles by Hvass, U.