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Introduction: Peripheral pulmonary arterial stenosis is poorly recognized in the adult population that it can cause pulmonary hypertension, but it is likely that it is misdiagnosed as idiopathic pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. Case description: 16-year-old female, in functional class II, grade II systolic murmur pulmonary focus, saturation 96%. Chest tomography: left peribronchovascular consolidation. Catheterization: diffuse peripheral pulmonary stenosis, perfusion defect in the left lobe vasculature, probable thrombosis vs atresia, PMAP 78mmHg. Preserved biventricular systodiastolic function. Perfusion lung scan: pulmonary thromboembolism. Methodology: The Boolean method was used to search for MESH terms "segmental arteries" "pulmonary" [and] "stenosis" in the database with repositories of indexed arbitrary scientific evidence (PubMed) and not indexed (ScholarGoogle). Discussion Patients with this disease usually have systemic pressures of the right ventricle secondary to obstruction in the lobar, segmental, and subsegmental branches. Small segmental artery stenting, particularly in patients with congenital diffuse peripheral pulmonary stenosis, has been less successful. Conclusions: In recent years, peripheral pulmonary artery surgery has been associated with low mortality and a reduction in the RV / Ao pressure ratio of almost 50%. The current treatment of these patients remains somewhat controversial and poorly documented since there are places where they cannot be approached surgically. |
