The evolving landscape of combination therapy for pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) is a progressively fatal disease, and the goal in treatment is to prevent disease progression. The standard of care often involves medications from multiple therapeutic classes, and there has been significant interest both in the choice of agent as well as the timing of initiation. There is a growing body of support for starting multiple medications at the time of diagnosis, or 'upfront ', rather than using sequential addition to prevent clinical deterioration.

Early Diagnosis of Pulmonary Arterial Hypertension: A Plea for Help.

Pulmonary arterial hypertension is a devastating, progressive, and fatal disease. When diagnosed, evaluated, and treated early, patients can expect important decreases in disease progression, improvement in quality of life, and improvement in survival rates. Sadly, the disorder is often allowed to progress to New York Heart Association Functional Class or WHO functional class III or IV status before practitioners on the front lines think of the diagnosis or refer to a pulmonary hypertension center.

Right atrial pressure/pulmonary artery wedge pressure ratio: A more specific predictor of survival in pulmonary arterial hypertension.

Background: Pulmonary arterial hypertension (PAH) is a progressive, fatal disease. Current prognostic models are not ideal, and identifying more accurate prognostic variables is needed. The objective of this study was to evaluate the relative prognostic value of the right atrial pressure/pulmonary artery wedge pressure (RAP/PAWP) ratio in PAH patients.

Lupus-Associated Pulmonary Arterial Hypertension: Variable Course and Importance of Prompt Recognition.

We describe a critically ill young woman with systemic lupus erythematosus (SLE) presenting with circulatory shock, multiorgan dysfunction, and elevated right-sided heart pressures. She was found to have recurrent acute severe pulmonary arterial hypertension (PAH) in the setting of an SLE flare. Our report highlights the variable course that SLE-associated PAH can take in the same patient and the implications of this for instituting the most effective treatment approach with each episode.

Macitentan for the treatment of pulmonary arterial hypertension.

Macitentan is the most recently approved dual endothelin-receptor antagonist (ERA) for the treatment of symptomatic pulmonary arterial hypertension. Compared to other available ERAs, it demonstrates superior receptor-binding properties, with consequently improved tissue penetration, and a longer duration of action allowing for once-daily dosing. It has a favorable adverse-effect profile, with notably no demonstrable increase in the risk of hepatotoxicity or peripheral edema, but like other ERAs, it is potentially limited by significant anemia.

Pharmacologic therapy for pulmonary arterial hypertension in adults: CHEST guideline and expert panel report.

Objective: Choices of pharmacologic therapies for pulmonary arterial hypertension (PAH) are ideally guided by high-level evidence. The objective of this guideline is to provide clinicians advice regarding pharmacologic therapy for adult patients with PAH as informed by available evidence.

Methods: This guideline was based on systematic reviews of English language evidence published between 1990 and November 2013, identified using the MEDLINE and Cochrane Library databases.

Unresolved pulmonary embolism leading to a diagnosis of pulmonary artery sarcoma.

Pulmonary artery sarcomas (PAS) are rare tumors with a poor prognosis. They are often misdiagnosed as pulmonary embolism (PE) leading to futile anticoagulation treatment and delay in proper diagnosis. We present a case of a patient who was initially misdiagnosed and anticoagulated for presumed pulmonary embolism.

Standard nonspecific therapies in the management of pulmonary arterial hypertension.

Recent advances in pulmonary arterial hypertension (PAH) research have created a new era of PAH-specific therapies. Although these therapeutics have revolutionized PAH therapy, their innovation was predated by supportive but nonspecific medical therapies adapted from their use in more common cardiopulmonary diseases. These therapies include oxygen therapy, diuretics, digoxin, anticoagulation, and high-dose calcium channel blockers.

Diagnosis of pulmonary arterial hypertension.

Accurate diagnosis of pulmonary arterial hypertension can be challenging and often requires a high index of clinical suspicion. Use of a variety of noninvasive tests can help define the population of patients in whom invasive cardiac catheterization should be pursued. An understanding of the historical, physical exam, electrocardiographic, radiographic, and echocardiographic clues in the diagnosis is important.

Meningoencephalitis due to Gemella haemolysans.

Gemella haemolysans is an uncommon but described cause of invasive disease in humans. We report a case of meningitis due to G. haemolysans that did not grow in cerebrospinal fluid culture, demonstrating a potential role for direct 16S rRNA gene PCR and sequencing in culture-negative cerebrospinal fluid when bacterial meningitis is suspected.

IL-13 receptor α2-arginase 2 pathway mediates IL-13-induced pulmonary hypertension.

Although previous literature suggests that interleukin (IL)-13, a T-helper type 2 cell effector cytokine, might be involved in the pathogenesis of pulmonary hypertension (PH), direct proof is lacking. Furthermore, a potential mechanism underlying IL-13-induced PH has never been explored. This study's goal was to investigate the role and mechanism of IL-13 in the pathogenesis of PH.

Targeted approaches to the treatment of pulmonary hypertension.

Pulmonary arterial hypertension is a progressive and incurable disease. Over the past two decades, significant advances have been made in understanding and thus managing this disease. Multiple therapeutic options are currently available and optimizing the treatment of pulmonary arterial hypertension has become complex.

Pregnancy and pulmonary hypertension.

When pulmonary hypertension (PH) occurs in pregnancy, physiologic stress can overwhelm an already strained right ventricle resulting in right ventricular failure and death. Mortality remains unacceptably high (25%-30%). Patients with PH should be counseled to avoid pregnancy.

Chronic thromboembolic pulmonary hypertension.

Chronic thromboembolic pulmonary hypertension is one of the few forms of pulmonary hypertension that is surgically curable. It is likely underdiagnosed and must be considered in every patient presenting with pulmonary hypertension to avoid missing the opportunity to cure these patients. This article discusses the epidemiology, risk factors, natural history, diagnosis, and preoperative evaluation of patients with this disorder.

Hemothorax due to rupture of pulmonary arteriovenous malformation: an interventional emergency.

Spontaneous hemothorax as a result of a ruptured pulmonary arteriovenous malformation (PAVM) is a life-threatening event and requires immediate interventional therapy. We present two patients who survived following emergent embolization. Definitive thoracentesis was delayed until embolization was performed.

Endothelin receptor blockade in the management of pulmonary arterial hypertension: selective and dual antagonism.

Various treatments approved by the United States Food and Drug Administration for the management of pulmonary arterial hypertension (PAH) target three of the many pathways implicated in the development of PAH: prostacyclin-, endothelin-1 (ET-1)-, and nitric oxide-mediated pathways. The objectives of this manuscript are to provide background information on the role of ET-1 in the pathogenesis of PAH, to provide theoretical considerations for the advantages and disadvantages of dual vs single endothelin receptor antagonists (ERAs) for the management of PAH, and to describe the clinical study results from randomized, double-blind, placebo-controlled trials for the various ERAs. ET receptors (ET(A) and ET(B)) have different densities and distributions throughout the body and are dynamically regulated, such that blockade of ET(A) and ET(B) receptors may have different results in normal vs pathological conditions.

Adult respiratory distress syndrome after treatment with pegylated interferon alpha-2a and ribavirin.

Pulmonary manifestations of interferon (IFN) use are a rare but well known complication seen with both standard and pegylated interferon alpha-2b (pegIFNalpha-2b) forms of the agent. These are generally of modest intensity and reversible. We report the first case of fulminant adult respiratory distress syndrome (ARDS) associated with pegylated interferon alpha-2a (pegIFNalpha-2a) and ribavirin use for hepatitis C, complicated by subsequent and ultimately fatal sepsis and multiorgan failure.

Pulmonary hypertension in older adults.

Pulmonary hypertension is a frequently encountered problem in older patients. True idiopathic pulmonary arterial hypertension can also be seen and requires careful exclusion in older patients. Institution of therapies must be tempered with an appreciation of individual comorbidities and functional limitations that may affect patients' ability to comply and benefit from the complex treatments available for pulmonary arterial hypertension.

Radiology-Pathology Conference: pulmonary hyalinizing granuloma associated with lupus-like anticoagulant and Morvan's Syndrome.

Pulmonary hyalinizing granulomata are rare, noninfectious, fibrosing lesions of the lung, which can mimic metastatic disease radiographically. Their etiology is unknown, but they may be caused by an exaggerated immune response. We report the radiology, long clinical course, and pathology of a patient with pulmonary hyalinizing granuloma who presented with initially asymptomatic pulmonary nodules.

Diagnosis of pulmonary arterial hypertension.

Accurate diagnosis of pulmonary arterial hypertension is a challenging and complex process that requires a high index of clinical suspicion from even the most astute clinician. This article discusses the use of a variety of noninvasive tests that can help define the population of patients in whom invasive cardiac catheterization should be pursued. It points out the vagaries and limitations of electrocardiography and the radiographic and echocardiographic clues to the diagnosis.