Surgical treatment of the cardiac manifestations of relapsing polychondritis: Overview of 33 patients identified through literature review and the mayo clinic records

Chadi Dib, Sherif E. Moustafa, Martina Mookadam, Kenton J. Zehr, Clement Michet, Farouk Mookadam

Research output: Contribution to journalArticle

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Abstract

OBJECTIVES: To analyze the cardiac findings that necessitate surgery in patients with relapsing polychondritis (RP) and to compare our results to cases in the literature. MATERIAL AND METHODS: A systematic overview of the literature was completed with the addition of cases of RP from the Mayo patient population that necessitated cardiac surgery. RESULTS: Thirty-three patients were identified (25 from the literature and 8 from the Mayo patient population). Nine patients (27%) were female, 22 (67%) were male, and sex was not stated for 2 patients (6%). The patient age ranged from 17 to 69 years (mean ± SD, 42.5±15.7 years). At operation, 30 patients (91%) had aortic regurgitation, 21 (64%) had aortic root disease, and 13 (39%) had mitral regurgitation. The most common surgical procedure performed was aortic valve replacement in 12 patients (36%). The most common complications were death in 12 patients (36%) and prosthetic valve dehiscence in 4 patients (12%). CONCLUSIONS: Cardiac involvement is more prominent in the male population and requires more invasive procedures. Aortic valve replacement with composite graft replacement of the ascending aorta along with coronary artery ostial reimplantation should be considered in these patients. Postsurgical valvular complications include prosthetic dehiscence, paravalvular leakage, mediastinitis, and heart failure, and these complications are associated with postoperative corticosteroid therapy. Initiation of second-line immunosuppressive therapy should be substituted for corticosteroids after cardiac surgery.

Original languageEnglish (US)
Pages (from-to)772-776
Number of pages5
JournalMayo Clinic Proceedings
Volume81
Issue number6
DOIs
StatePublished - 2006

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Relapsing Polychondritis
Therapeutics
Aortic Valve
Thoracic Surgery
Adrenal Cortex Hormones
Population
Mediastinitis
Aortic Diseases
Aortic Valve Insufficiency
Replantation
Mitral Valve Insufficiency
Immunosuppressive Agents
Aorta

Keywords

  • AR = aortic regurgitation
  • AVR = aortic valve replacement
  • MR = mitral regurgitation
  • RP = relapsing polychondritis
  • TTE = transthoracic echocardiography

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Surgical treatment of the cardiac manifestations of relapsing polychondritis : Overview of 33 patients identified through literature review and the mayo clinic records. / Dib, Chadi; Moustafa, Sherif E.; Mookadam, Martina; Zehr, Kenton J.; Michet, Clement; Mookadam, Farouk.

In: Mayo Clinic Proceedings, Vol. 81, No. 6, 2006, p. 772-776.

Research output: Contribution to journalArticle

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N2 - OBJECTIVES: To analyze the cardiac findings that necessitate surgery in patients with relapsing polychondritis (RP) and to compare our results to cases in the literature. MATERIAL AND METHODS: A systematic overview of the literature was completed with the addition of cases of RP from the Mayo patient population that necessitated cardiac surgery. RESULTS: Thirty-three patients were identified (25 from the literature and 8 from the Mayo patient population). Nine patients (27%) were female, 22 (67%) were male, and sex was not stated for 2 patients (6%). The patient age ranged from 17 to 69 years (mean ± SD, 42.5±15.7 years). At operation, 30 patients (91%) had aortic regurgitation, 21 (64%) had aortic root disease, and 13 (39%) had mitral regurgitation. The most common surgical procedure performed was aortic valve replacement in 12 patients (36%). The most common complications were death in 12 patients (36%) and prosthetic valve dehiscence in 4 patients (12%). CONCLUSIONS: Cardiac involvement is more prominent in the male population and requires more invasive procedures. Aortic valve replacement with composite graft replacement of the ascending aorta along with coronary artery ostial reimplantation should be considered in these patients. Postsurgical valvular complications include prosthetic dehiscence, paravalvular leakage, mediastinitis, and heart failure, and these complications are associated with postoperative corticosteroid therapy. Initiation of second-line immunosuppressive therapy should be substituted for corticosteroids after cardiac surgery.

AB - OBJECTIVES: To analyze the cardiac findings that necessitate surgery in patients with relapsing polychondritis (RP) and to compare our results to cases in the literature. MATERIAL AND METHODS: A systematic overview of the literature was completed with the addition of cases of RP from the Mayo patient population that necessitated cardiac surgery. RESULTS: Thirty-three patients were identified (25 from the literature and 8 from the Mayo patient population). Nine patients (27%) were female, 22 (67%) were male, and sex was not stated for 2 patients (6%). The patient age ranged from 17 to 69 years (mean ± SD, 42.5±15.7 years). At operation, 30 patients (91%) had aortic regurgitation, 21 (64%) had aortic root disease, and 13 (39%) had mitral regurgitation. The most common surgical procedure performed was aortic valve replacement in 12 patients (36%). The most common complications were death in 12 patients (36%) and prosthetic valve dehiscence in 4 patients (12%). CONCLUSIONS: Cardiac involvement is more prominent in the male population and requires more invasive procedures. Aortic valve replacement with composite graft replacement of the ascending aorta along with coronary artery ostial reimplantation should be considered in these patients. Postsurgical valvular complications include prosthetic dehiscence, paravalvular leakage, mediastinitis, and heart failure, and these complications are associated with postoperative corticosteroid therapy. Initiation of second-line immunosuppressive therapy should be substituted for corticosteroids after cardiac surgery.

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