Pulmonary circulation
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Pulmonary circulation · Sep 2015
Statement on pregnancy in pulmonary hypertension from the Pulmonary Vascular Research Institute.
Pregnancy outcomes in patients with pulmonary hypertension remain poor despite advanced therapies. Although consensus guidelines recommend against pregnancy in pulmonary hypertension, it may nonetheless occasionally occur. This guideline document sought to discuss the state of knowledge of pregnancy effects on pulmonary vascular disease and to define usual practice in avoidance of pregnancy and pregnancy management. ⋯ Further, we identified the current state of knowledge on the effects of sex hormones during pregnancy on the pulmonary vasculature and right heart and suggested areas for future study. There is currently limited evidence-based knowledge about both the basic molecular effects of sex hormones and pregnancy on the pulmonary vasculature and the best practices in contraception and pregnancy management in pulmonary hypertension. We have drafted 38 consensus-based recommendations to guide clinicians in these challenging topics, but further research is needed in this area to define best practices and improve patient outcomes.
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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.