A rare case of primitive right atrium angio-sarcoma with favorable outcome, in a young female: case report and literature review / Angiosarcoma primitivo de la aurícula derecha en una mujer joven, con respuesta favorable al tratamiento: reporte de un caso y revisión de la literatura

Primary malignant cardiac tumors are extremely rare, but their associated mortality is very high. The clinical presentation is oñen variable and nonspecific; by the time symptoms appear, the tumor has usually evolved to a large intracardiac and paracardiac mass causing considerable hemodynamic involvement, regional invasión and distant dissemination. We report a 26 year-old female with a right atrium angiosarcoma with a surprisingly favorable outcome at 2 years follow-up after treatment with a combination of neoadjuvant chemotherapy and surgical resection. She presented with chest tightness, shortness of breath at effort, atypical chest pain, palpitations, asthenia, weight loss and profuse perspiration. Physical examination and irnaging diagnostic procedures, identified a pericardial effusion and a pathologic cardiac mass. The diagnosis of a malignant angiosarcoma was suggested afier computed tomography transthoracic and transesophageal echocardiography It was confirmed at surgery by a biopsy followed by histopathology and immunohistochemistry.

Los tumores cardíacos malignos son extremadamente raros y su mortalidad es muy alta. El cuadro clínico es variable e inespecífico: frecuentemente cuando aparecen síntomas ya existe una gran masa intracardíacayparacardíaca que causa compromiso hemodinámico grave, además de invasión locorregionaly diseminación a distancia. Comunicamos un caso especial de una mujerjoven que tuvo una evolución sorprendentemente favorable después de un tratamiento combinado con quimioterapia neoadyuvante y resección quirúrgica. La paciente presentó opresión precordial, disnea de esfuerzo, dolor torácico atípico, palpitaciones, astenia, baja de peso y sudoración profusa. El examen físico y exámenes complementarios, especialmente de imágenes, identificaron un derrame pericárdico y una masa cardíaca patológica. La tomografía computada y la ecocardiografía, particularmente la transesofágica, sugirieron el diagnóstico de angiosarcoma maligno, que se confirmó en una intervención quirúrgica que incluyó una biopsia con estudios histopatológicos e inmunohistoquímicos. Se discuten las alternativas del diagnóstico y del tratamiento, y se revisan la epidemiología y los recursos terapéuticos actuales en la literatura. Este caso ilustra la utilidad de la ecocardiografía transesofágica, para el diagnóstico de estos tumores y se plantea que el uso de nuevas alternativas quimioterapias asociadas a la extirpación quirúrgica pueden mejorar la sobrevida que, en nuestra paciente, alcanza dos años libre de síntomas.

Conventional radiology and right heart catheterization in estimating primary pulmonary hypertension and pulmonary hypertension secondary to left-sided valvular disease.

AIM: to estimate the comparative value of conventional radiology and cardiac catheterization in establishing the diagnosis and severity of primary and secondary pulmonary hypertension. We also tried to achieve some correlations between the radiological and invasive parameters. METHODS: we performed a retrospective study over an 11-years time period, analyzing data from a group of 14 patients diagnosed with primary pulmonary hypertension compared to a matched group of 20 patients suffering from pulmonary hypertension secondary to mitral and aortic valvular disease. All the patients had undergone conventional radiology (chest X-ray) and catheterization of the right heart cavities and pulmonary artery. RESULTS: we detected significantly elevated pulmonary artery pressures and resistances in the primary pulmonary hypertension group compared to secondary hypertension patients. Cardiac output values were much lower in the primary pulmonary hypertension individuals compared with secondary pulmonary hypertension in left-sided valvular disease. The pulmonary artery arch diameter, the diameter of the right descending pulmonary artery and the value of the arterio-bronchial ratio were similar in the two groups. CONCLUSION: in both groups, we found a statistically significant positive correlation between the values of the pulmonary artery vascular resistance and the diameter of the right descending pulmonary artery, which are very specific parameters in the diagnosis of pulmonary hypertension. In the primary pulmonary hypertension group we identified a significant inverse correlation between the diameter of the right descending pulmonary artery and the values of cardiac output. This finding was not confirmed in the secondary group.