Pediatric Cardiology

Biography

Biography: Paloma Manea

Abstract

MASS phenotype associates skeletal features, one of them being temporo-mandibular disorder (TDM). Apparently an innocent condition, TDM affects life quality, provoking symptoms like headache, neck and shoulder pain, dizzeness, tinitus and even deafness. Connective tissue in fibrilinopathies has the same abnormalities in the mitral valve, as well as in temporo-mandibular joint and this affirmation has been proved by multiple studies during last decade.
We’ve selected 46 children, diagnosed with MASS phenotype, aged between 5-17 years, with a predominance of females (70%). They were monithorized for 24 months, quarterly, using cardio logical and dental examination; Ghent revised criteria, electrocardiogram, echocardiogram and magnetic resonance imaging (MRI) for temporo-mandibular joint. 40% of the patients (18 of 46) associated TMD, proved by dental examination and MRI. 6 of these 18 patients with TMD revealed a severe dysfunction of this joint and all of them increased their dyspnea (as symptom) and their mitral regurgitation, pulmonary pressure values ,during 24 months observation.
As an invasive investigation of mitral degeneration(biopsy) isn’t the best option for deciding the surgical moment , we consider that non-invasive assessment of temporo-mandibular joint, in this fibrillinopathy, could be a useful prognosis marker for mitral deterioration(if abnormalities of connective tissue are similar). We’ ve made an adequate and in time selection of those 6 patients with cardiac surgical indication, so we reffered them to the cardiac surgeon and we’ve made this decision accounting the following: worsening dyspneea, supported by augmented mitral regurgitation, pulmonary pressure values and a non-conventional, but very precise marker of connective tissue deterioration: MRI of temporo-mandibular joint affected by dysfunction.