We report a very rare case of severe pulmonary arterial hypertension in a patient with neurofibromatosis type 1, and discuss the pathology, pathogenesis, current pulmonary hypertension classification system, and outcomes of pulmonary arterial hypertension in patients with neurofibromatosis type 1.

OBJECTIVE Pulmonary hypertension (PH) has been described in hemodialysis (HD) patients and has been associated with increased morbidity and mortality. Our objective was to determine the prevalence of pulmonary hypertension in patients on regular hemodialysis. METHODS This cross sectional study was conducted in Department of Nephrology, Liaquat National Hospital Karachi from April 2013 to Marc...

INTRODUCTION Pulmonary arterial hypertension (PAH) is a serious and often fatal complication of systemic lupus erythematosus (SLE). Because the diagnosis of PAH often is made years after symptom onset, early diagnostic strategies are essential. Doppler echocardiography currently is considered the noninvasive screening test of choice for evaluating pulmonary hypertension. AIM Screening for asy...

The severity of pulmonary hypertension was evaluated by right cardiac catheterization in 89 patients with stable chronic obstructive pulmonary disease, both at rest and during maximum treadmill exercise. Thirty-one patients were found to have pulmonary hypertension at rest, defined as a mean pulmonary arterial pressure of 20 mm Hg or more. Although the remaining 58 patients had normal mean pulm...

Neonates with pulmonary atresia and intact interventricular septum (PAIVS) do not have pulmonary vascular disease secondary to their heart abnormality. Persistent pulmonary hypertension of the newborn has not been described in association with this condition. The case is reported of a female neonate born with PAIVS, who preoperatively had no clinical evidence or any risk factors for persistent ...

Pulmonary hemodynamics were studied in 11 patients with hepatic cirrhosis who had undergone surgical portal decompression several years earlier. Four of the patients had both clinical and physiological evidence of pulmonary hypertension; two others had mild, subelinical pulmonary hypertension. Autopsy examination of two patients who had clinical evidence of pulmonary hypertension revealed thick...

RATIONALE Pulmonary arterial hypertension is characterized by endothelial dysfunction, impaired bone morphogenetic protein receptor 2 (BMPR2) signaling, and increased elastase activity. Synthetic elastase inhibitors reverse experimental pulmonary hypertension but cause hepatotoxicity in clinical studies. The endogenous elastase inhibitor elafin attenuates hypoxic pulmonary hypertension in mice,...

Primary pulmonary hypertension is a disorder with limited treatment options and poor outcome. We demonstrated a beneficial role of sildenafil, a phosphodiesterase 5 inhibitor, in a patient with primary pulmonary hypertension. After the initiation of sildenafil the pulmonary artery pressure decreased from 40/20mmHg to 16/6mmHg, while mean pulmonary arterial systolic pressure decreased from 25mmH...