Pulmonary circulation
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Pulmonary circulation · Sep 2015
Never too old for congenital heart disease: sinus venosus atrial septal defect with anomalous pulmonary venous return in an octogenarian.
We report a rare case in an 86-year-old woman with symptoms of exercise intolerance, fatigability, worsening lower extremity edema, and normal ejection fraction on echocardiographic examination who presented with a presumptive diagnosis of heart failure with preserved ejection fraction (HFpEF). Hemodynamic studies revealed that she had normal left-sided filling pressures, mildly elevated pulmonary pressures with normal pulmonary vasculature resistance, and evidence of right ventricular dysfunction. ⋯ Contrast-enhanced multidetector cardiac computed tomography confirmed the presence of a patent foramen ovale, a sinus venosus atrial septal defect, and 3 anomalous pulmonary venous communications to the right atrium and superior vena cava. We hereby present one of the oldest diagnosed cases of sinus venosus defect with anomalous pulmonary venous return as a rare cause of recent-onset dyspnea, volume overload, and functional intolerance in an 86-year-old woman with an initial misdiagnosis of HFpEF.
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Pulmonary circulation · Sep 2015
Prevalence and hospital discharge status of human immunodeficiency virus-associated pulmonary arterial hypertension in the United States.
Pulmonary arterial hypertension (PAH) is a noninfectious complication of human immunodeficiency virus (HIV) infection that has gained in importance since the advent of antiretroviral therapy. HIV-associated PAH (HIV-PAH) has a higher prevalence than idiopathic PAH (IPAH), although the vascular pathology seen in HIV-PAH is virtually identical to that seen in IPAH. Initiating therapy for PAH at an early stage is associated with a better prognosis; however, because of the nonspecific symptoms associated with PAH, the diagnosis is often delayed. ⋯ To address this issue, we conducted a retrospective review of the National Hospital Discharge Survey data and the multiple-cause mortality data to determine the prevalence of HIV-PAH at hospital discharge and death. Using these large data sets, we observed that the prevalence of HIV-PAH among HIV-infected individuals at hospital discharge and death was significantly lower than the reported prevalence in the literature. In addition, we found that PAH was designated as the most common cause of mortality in patients with HIV-PAH.
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Pulmonary circulation · Jun 2015
Right ventricular remodeling in idiopathic and scleroderma-associated pulmonary arterial hypertension: two distinct phenotypes.
Patients with scleroderma (SSc)-associated pulmonary arterial hypertension (PAH) have worse survival than patients with idiopathic PAH (IPAH). We hypothesized that the right ventricle (RV) adapts differently in SSc-PAH versus IPAH. We used cardiac magnetic resonance imaging (cMRI) and hemodynamic characteristics to assess the relationship between RV morphology and RV load in patients with SSc-PAH and IPAH. ⋯ RVMI varies linearly with measures of RV load. After adjusting for multiple potential confounders, patients with SSc-PAH demonstrated significantly less RV hypertrophy with increasing PVR than patients with IPAH. This difference in adaptive hypertrophy may in part explain previously observed decreased contractility and poorer survival in SSc-PAH.
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Pulmonary circulation · Jun 2015
Acute effects of riociguat in borderline or manifest pulmonary hypertension associated with chronic obstructive pulmonary disease.
Riociguat is the first oral soluble guanylate cyclase stimulator shown to improve pulmonary hemodynamics in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension (PH). This pilot study assessed the impact of a single dose of riociguat on hemodynamics, gas exchange, and lung function in patients with PH associated with chronic obstructive pulmonary disease (COPD). Adults with COPD-associated borderline or manifest PH (pulmonary vascular resistance > 270 dyn·s·cm(-5), mean pulmonary artery pressure ≥ 23 mmHg, ratio of forced expiratory volume in 1 second [FEV1] to forced vital capacity < 70%, and partial pressure of oxygen and carbon dioxide in arterial blood > 50 and ≤ 55 mmHg, respectively) received riociguat 1 or 2.5 mg during right heart catheterization. ⋯ No relevant changes in lung function or gas exchange were observed. Single doses of riociguat were well tolerated and showed promising hemodynamic effects without untoward effects on gas exchange or lung function in patients with COPD-associated PH. Placebo-controlled studies of chronic treatment with riociguat are warranted.
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Pulmonary circulation · Mar 2015
The sexual dimorphism associated with pulmonary hypertension corresponds to a fibrotic phenotype.
Although female predominance in the development of all types of pulmonary hypertension (PH) is well established, many clinical studies have confirmed that females have better prognosis and higher survival rate than males. There is no clear explanation of why sex influences the pathogenesis and progression of PH. Using a rat angioproliferative model of PH, which closely resembles the primary pathological changes observed in humans, we evaluated the role of sex in the development and progression of PH. ⋯ The reduction in fibrosis in female rats correlated with increased expression of caveolin-1 and reduced endothelial nitric oxide synthase-derived superoxide. We conclude that, in the pathogenesis of PH, female sex is associated with greater remodeling of the pulmonary arteries but greater survival. Conversely, in males, the development of pulmonary and cardiac fibrosis leads to early and severe RV failure, and this may be an important reason for the lower survival rate among males.