Pulmonary endarterectomy. Initial report in a cardiology hospital / Endarterectomía pulmonar. Reporte inicial en un hospital de cardiología

Background: Currently the options for treatment of chronic thromboembolic pulmonary hypertension can be pulmonary endarterectomy, pulmonary angioplasty and pharmacological treatment. Objective: To show the feasibility of performing pulmonary endarterectomy in a cardiology hospital. Methods: From December 2013 to June 2014 a serie of consecutive cases was studied according to the guidelines of the Fifth World Symposium of Pulmonary Hypertension. Its antecedents, clinical characteristics, functional class, hemodynamics, exercise capacity were defined in pre and post-operative conditions. Results: Three cases, two males with A + blood group and one female O + with presence of antiphospholipid antibodies; the three patients with prior history of pulmonary embolism, obese, with dyspnea and syncope; preoperative systolic pulmonary pressures were 60, 50, 59 mm Hg, and post-operative 43, 33, 21 mm Hg; functional class III/IV vs. I/IV; walked meters 320, 266, 252 vs. 480, 527, 0, respectively. One patient died, not related to surgery, due to multiple organ failure 40 days after surgery. Conclusions: Pulmonary endarterectomy is a feasible procedure with clinical and hemodynamic improvement.

Introducción: actualmente, las opciones de tratamiento de la hipertensión pulmonar tromboembólica crónica pueden ser la endarterectomía pulmonar, la angioplastía pulmonar y el tratamiento farmacológico. Objetivo: mostrar la factibilidad de realizar la endarterectomía pulmonar en un hospital de cardiología. Métodos: de diciembre del 2013 a junio del 2014 se estudió una serie de casos consecutivos de acuerdo con las guías del 5to Simposio Mundial sobre Hipertensión Pulmonar. Se indagaron sus antecedentes, características clínicas, clase funcional, hemodinámica y capacidad de ejercicio, en condiciones pre- y posoperatorias. Resultados: se operaron tres pacientes, dos varones con grupo sanguíneo A+ y una mujer O+ con presencia de anticuerpos antifosfolípidos; los tres pacientes tenían antecedentes de embolia pulmonar previa, obesos, con disnea y síncope; las presiones pulmonares sistólicas preoperatorias fueron de 60, 50, 59 mm Hg, respectivamente, y posoperatorias de 43, 33, 21 mm Hg; clase funcional III/IV frente a I/IV; metros caminados 320, 266, 252 frente a 480, 527 y cero, respectivamente. Un paciente falleció por falla orgánica múltiple a 40 días del posoperatorio, el desenlace no estuvo relacionado con la cirugía. Conclusiones: la endarterectomía pulmonar es un procedimiento factible de realizar con mejoría clínica y hemodinámica.

Identification of genomic copy number variations in lung benign metastasizing leiomyomatosis.

OBJECTIVES: Lung metastasizing leiomyomatosis (LML) is an infrequently diagnosed pathology developed after sexual maturation, commonly preceded by uterine myomas. Symptoms can include difficulties to breathe, cough, dyspnea and pain, because of mechanical obstruction exerted by expanding local growing leiomyomas. Lung leiomyomas are normally detected by imaging studies, but nowadays the precise diagnosis demands histological characterization of biopsies obtained from the affected tissues. The purpose of the present study was to determine the presence of genomic alterations in circulating cells of LML. METHODS: Immunohistochemical characterization of a lung biopsy extracted by thoracoscopy was performed. Pathologic proliferative smooth muscle cells were observed in a major lung metastasizing nodule, with a growing pattern similar to a uterine myoma. The presence of cellular linages different to smooth muscle cells was discarded by testing the presence of a battery of molecular markers. Also, a normal karyotype was determine by GTG-banding cytogenetic study, but a high density microarray analysis revealed six submicroscopic chromosomal regions displaying genomic abnormalities: microduplications were detected on chromosomes 4, 14, 17 and 22; and microdeletions on chromosomes 8 and 10. CONCLUSION: This study remarks the relevance of submicroscopic chromosomal analysis of unusual pathologic conditions such as Benign Metastasizing Leiomyomatosis. This propitiate a better understanding of the molecular basis on the development of the pathology, in order to reckon on minimally invasive diagnostic methods, and to design appropriate treatments.