MASS phenotype and oral abnormalities

    Authors

    Abstract

    **Introduction**: MASS phenotype is a particular type of fibrillinopathy, the following Ghent criteria being present: Z score of aortic root < 3(child) / 2 (adult); systemic score <5; mitral valve prolapsed (M); borderline aorta dilation(A); skin striae (S) and skeletal features (S). (1) The goal of the study was to reveal correlations between oral abnormalities and cardiac pathology in MASS phenotype, for a better approach and therapy of cardiac involvement. **Patients and Methods**: Our study included 28 patients, with 2 years monitorization, aged between 5 and 48 years, with MASS phenotype. Every 6 months, cardiological and dental examination, laboratory findings, electrocardiogram and transthoracic echocardiogram were done. As concerning patients symptoms, the following were predominant: atypical chest pain, 1st class dyspnea and palpitations. Mild and moderate mitral regurgitation were usually seen on echocardiography, as mild or moderate pulmonary hypertension; 1 patient required mitral valve reconstruction, due to his severe mitral valve regurgitation and severe pulmonary hypertension. The most common oral anomalies were the following: dental implantation abnormalities, periodontal disease, maxillary protrusion and temporomandibular joint dysfunction. **Results**: The study revealed a direct correlation between mitral regurgitation severity and oral modifications. The strongest connection was between temporomandibular joint dysfunction and mitral valve prolapsed (r= 0.728, p=0.03), concordant with previous studies. This observation sustains a previous affirmation: fibrillin deficiency inside mitral valve, in fibrillinopathies, is the same as fibrillin deficiency in temporomandibular joint. **Conclusion**: Oral anomalies, inside MASS phenotype, could be an outcome marker for cardiovascular evolution in this disease.

    Keywords

    MASS phenotype, mitral regurgitation, oral abnormalities

    DOI

    https://doi.org/10.15836/ccar2018.378

    Literature

    1. Rippe M, De Backer J, Kutsche K, Mosquera LM, Schüler H, Rybczynski M, et al. Mitral valve prolapse syndrome and MASS phenotype: Stability of aortic dilatation but progression of mitral valve prolapse. Int J Cardiol Heart Vasc. 2016 Jan 21;10:39–46. https://doi.org/10.1016/j.ijcha.2016.01.002
    Cardiologia Croatica
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    MASS phenotype and oral abnormalities

    Extended Abstract
    Issue11-12
    Published
    Pages378
    PDF via DOIhttps://doi.org/10.15836/ccar2018.378
    MASS phenotype
    mitral regurgitation
    oral abnormalities

    Authors

    Paloma Horjinec*ORCID"Gr.T.Popa" University of Medicine and Pharmacy, Iasi, Romania
    Francisc Ludvig HorjinecORCID"Promedicanon" Office, Iasi, Romania

    *Correspondence email: maneacpaloma@yahoo.com

    Abstract

    **Introduction**: MASS phenotype is a particular type of fibrillinopathy, the following Ghent criteria being present: Z score of aortic root < 3(child) / 2 (adult); systemic score <5; mitral valve prolapsed (M); borderline aorta dilation(A); skin striae (S) and skeletal features (S). (1) The goal of the study was to reveal correlations between oral abnormalities and cardiac pathology in MASS phenotype, for a better approach and therapy of cardiac involvement. **Patients and Methods**: Our study included 28 patients, with 2 years monitorization, aged between 5 and 48 years, with MASS phenotype. Every 6 months, cardiological and dental examination, laboratory findings, electrocardiogram and transthoracic echocardiogram were done. As concerning patients symptoms, the following were predominant: atypical chest pain, 1st class dyspnea and palpitations. Mild and moderate mitral regurgitation were usually seen on echocardiography, as mild or moderate pulmonary hypertension; 1 patient required mitral valve reconstruction, due to his severe mitral valve regurgitation and severe pulmonary hypertension. The most common oral anomalies were the following: dental implantation abnormalities, periodontal disease, maxillary protrusion and temporomandibular joint dysfunction. **Results**: The study revealed a direct correlation between mitral regurgitation severity and oral modifications. The strongest connection was between temporomandibular joint dysfunction and mitral valve prolapsed (r= 0.728, p=0.03), concordant with previous studies. This observation sustains a previous affirmation: fibrillin deficiency inside mitral valve, in fibrillinopathies, is the same as fibrillin deficiency in temporomandibular joint. **Conclusion**: Oral anomalies, inside MASS phenotype, could be an outcome marker for cardiovascular evolution in this disease.

    Literature

    1. 1.
      Rippe M, De Backer J, Kutsche K, Mosquera LM, Schüler H, Rybczynski M, et al. Mitral valve prolapse syndrome and MASS phenotype: Stability of aortic dilatation but progression of mitral valve prolapse. Int J Cardiol Heart Vasc. 2016 Jan 21;10:39–46.DOI