We report the case of a patient 20-year-old woman with Dyspnea and chest pain associated with moderate effort, with no heart disease previously known. The assessments showed dilatation of main trunk and branches of pulmonary artery with severe pulmonary regurgitation, mild pulmonary hypertension, mild dilation and moderate right ventricle hypertrophy; that underwent surgery for severe pulmonary insufficiency and to surgically cure pulmonary aneurysm. The intraoperative review found an Agenesis of the septal leaflet of the pulmonary valve and pulmonary artery aneurysm. A change of pulmonary valve and plasty aneurysm was performed.Key words: pulmonary artery aneurysm, pulmonary valve agenesis, echocardiography resumen Reportamos el caso de una paciente mujer de 20 años con disnea y dolor torácico asociado a esfuerzo moderado, sin enfermedad cardiaca conocida, previamente. Las evaluaciones demostraron dilatación de tronco principal y ramas de arteria pulmonar con regurgitación pulmonar severa, hipertensión pulmonar leve, dilatación leve e hipertrofia moderada de ventrículo derecho; sometida a cirugía por insuficiencia pulmonar severa y cura quirúrgica de aneurisma pulmonar. El hallazgo intraoperatorio fue agenesia del velo septal de la válvula pulmonar y aneurisma de arteria pulmonar y sus ramas. Se realizó un cambio de válvula y plastía del aneurisma.
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