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MEFV mutation carriage as possible predisposition factor for the development of Post Pericardiotomy Syndrome (PPS)


PPS is a syndrome, which manifests with pleuropericardial inflammation and occurs in about 15-20% of patients undergoing surgery, involving the pleura, pericard or both. The pathogenesis of the syndrome is not yet fully understood. Carriage of MEFV mutations may explain the occurrence of this syndrome, which largely overlaps with FMF, in only part of the operated population.


To determine whether MEFV mutation carriage may precipitate PPS or affect its phenotype.


86 patients who underwent cardiac surgery were studied, 45 of whom developed PPS (study group) and 41 have not (control group). Demographic data (gender, age, region of residence, ethnic origin) and type of surgery were collected. The severity of PPS was evaluated, based on a predefined scale. Genetic analysis determining carriage of one of the three most common MEFV gene mutations (M694V, V726A, E148Q) was performed.


The rate of women was higher in the PPS group (p=0.001). No significant differences were found between the 2 groups with regards to the rate of mutation carriage. Subgroup analysis for age, ethnic origin and gender also failed to yield significant results. The severity of the PPS in carriers was lower compared to non carriers.


Carriage of MEFV mutations does not predispose for the development of PPS. However carriage of MEFV mutations does affect PPS phenotype (P<0.05).

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This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver ( applies to the data made available in this article, unless otherwise stated.

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Dechtman, I., Ben-Zvi, I., Yael, S. et al. MEFV mutation carriage as possible predisposition factor for the development of Post Pericardiotomy Syndrome (PPS). Pediatr Rheumatol 13 (Suppl 1), P76 (2015).

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