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Introduction: Pectus excavatum (PE) and carinatum (PC) are common in Marfan syndrome (SM) and similar syndromes (SS). Patients can evolve without symptoms. In some there is depression, social adjustment disorders, pulmonary and cardiovascular symptoms in which there is controversy about their relationship with the structural damage of the thorax., Objective: To assess the prevalence of the type of thoracic deformity in patients with MS and SS in a historical and current cohort and to analyze the clinical, pulmonary and cardiovascular impact., Method: Prospective study. Subjects who met the Ghent criteria and who had a complete clinical record, an echocardiogram and/or magnetic resonance imaging, computed tomography and respiratory function tests were included., Results: Of a total of 338 patients with MS and SS, 112 cases with thoracic deformity were detected, the prevalence of PE and PC in SM 13.6 and 12.4, respectively, was lower in SS. There is compression and displacement of lung and right cardiac cavities by PE and the correlation between the Haller Index and the increased PASP is 44 (p = 0.009)., Conclusions: The prevalence of PE and PC in SM and SS is high, which impacts on lung function and cardiovascular damage, requires corrective management of the thoracic deformity and not only implies for aesthetic purposes., (Copyright: © 2018 SecretarÍa de Salud.)