CARDIOPULMONARY DYSFUNCTIONS IN PATIENTS WITH FIBRODYSPLASIA OSSIFICANS PROGRESSIVE

Abstract

Fibrodysplasia ossificans progressiva (PFO) is a rare and inherited genetic disorder that affects the connective tissue, progressing to a debilitating condition over time. The diagnosis is based on clinical evaluation and imaging tests, however, currently, there are no therapies capable of modifying its evolution, and the management is focused on symptom control. This study carried out a literature review, using a qualitative approach to synthesize scientific evidence, with the objective of analyzing the characteristics of FOP, broadening the understanding of its pathogenesis and supporting coping strategies. Data collection was carried out in sources such as PubMed, Scielo and EBSCO, covering publications from the last ten covering the period from 2014 to 2024. The results showed a predominance of international and national publications conducted by medical researchers, clarifying a restricted and limited national scientific production, which reinforces the need for greater encouragement of research that subsidizes clinical practice and improves the quality of life of patients. The literature analysis showed that the clinical picture ranges from ectopic bone formation to severe cardiorespiratory complications, with risk of fatal evolution. These findings highlight the complexity of the disease and the urgency of therapeutic and multidisciplinary advances to mitigate its negative impact and poor prognosis in these cases.