[Primary pulmonary arterial hypertension. Current considerations]. / Hipertensión arterial pulmonar primaria. Consideraciones actuales.

Primary pulmonary hypertension (PPH) is a rare, progressive and fatal condition. Because of nonspecific signs and symptoms, patients diagnosed with PPH are often present with a relatively advanced disease. Significant advances have been made in recent years. A revised nomenclature has been proposed, the pathobiology is now better defined, new risk factors have been identified, and the newer therapeutic strategies appear to be changing the natural history and prognosis of this disease.

Cystic angiomatosis of the bone appearing as intrathoracic lung masses.

A rare case of cystic angiomatosis of the bone (CAB) manifesting as bilateral intrathoracic masses is described. In 1996, a 47-year-old woman was referred to the chest clinic at St Boniface Hospital for evaluation of chronic cough, and her chest radiograph showed large, bilateral pleural-based intrathoracic masses. Ten years before, she had been diagnosed with CAB following bone biopsies of her hip and knee. Computed tomography revealed two chest wall masses with internal septations of bone. CAB is a rare, multicentric disease characterized by involvement of the vascular and lymphatic systems, producing diffuse cystic lesions in the skeleton. Although the ribs are frequently involved, the presentation with intrathoracic masses is uncommon.

[Development of a canine model of chronic progressive right ventricular hypertension]. / Desarrollo de un modelo canino de hipertensión ventricular derecha crónica progresiva.

INTRODUCTION: Several animal models of right ventricle hypertension (RVH) have been produced through pulmonary artery banding with linen, tygon or teflon. Nevertheless few devices attempting a progressive, step by step graduated chronic development of RVH have been reported. The present study describes the results in our animal model of chronic RVH. MATERIAL AND METHODS: We designed a software programmed to obtain hemodynamic data and installed a small occlusive hydraulic device (OHD) at the pulmonary artery trunk producing a raise in the right ventricular systolic pressure (RVSP); this pressure can be modified externally through the OHD. We studied 12 healthy mongrel dogs (18 to 28 kg of weight) in the course of 6 months. Hemodynamic measurements were performed at different RVSP at two months intervals; (Baseline, 40 mmHg and 60 mmHg). RESULTS: The software was useful to analyze several hemodynamic variables at each RVSP. At 60 mmHg, the end diastolic pressure of the right ventricle (RVEDP) increased from 4.2 +/- 0.4 mmHg to 13.2 +/- 1.1 mmHg, p < 0.000, accompanied with a fall in cardiac output adjusted to the dogs weight from 0.16 +/- 0.03 L/min/kg to 0.09 +/- 0.01 L/min/kg, p > 0.000. Also an increase of the end diastolic pressure of the left ventricle (LVEDP) from 7.4 +/- 0.8 mmHg to 16.3 +/- 2.8 mmHg, p < 0.000, was observed. RVSP was maintained in chronic condition and the intraclass correlation coefficient was 0.83, P < 0.005. CONCLUSIONS: Right ventricular chronic hypertension is created. The device is useful and reliable to maintain chronic increments of RVSP. The software permits a versatile analysis.