Features and Outcomes of Methamphetamine-associated Pulmonary Arterial Hypertension.

Rationale: Although amphetamines are recognized as "likely" agents to cause drug- and toxin-associated pulmonary arterial hypertension (PAH), (meth)amphetamine-associated PAH (Meth-APAH) has not been well described.

Objectives: To prospectively characterize the clinical presentation, histopathology, and outcomes of Meth-APAH compared with those of idiopathic PAH (iPAH).

Methods: We performed a prospective cohort study of patients with Meth-APAH and iPAH presenting to the Stanford University Pulmonary Hypertension Program between 2003 and 2015.

Drug Development and Biologics in Asthma. A New Era.

Considerable progress has been made toward developing targeted biological therapeutics for asthma, due in large part to a deeper understanding of asthma pathophysiology. This explosion of knowledge has revealed asthma to be a much more complex and heterogeneous entity than previously understood. The identification of particular asthma phenotypes with distinct pathophysiologic mechanisms has opened up a new era for patient populations not well served by current therapies, especially patients with severe asthma.

Future directions in idiopathic pulmonary fibrosis research. An NHLBI workshop report.

The median survival of patients with idiopathic pulmonary fibrosis (IPF) continues to be approximately 3 years from the time of diagnosis, underscoring the lack of effective medical therapies for this disease. In the United States alone, approximately 40,000 patients die of this disease annually. In November 2012, the NHLBI held a workshop aimed at coordinating research efforts and accelerating the development of IPF therapies.

Characteristics and outcome after hospitalization for acute right heart failure in patients with pulmonary arterial hypertension.

Background: Although much is known about the risk factors for poor outcome in patients hospitalized with acute heart failure and left ventricular dysfunction, much less is known about the syndrome of acute heart failure primarily affecting the right ventricle (acute right heart failure).

Methods And Results: By using Stanford Hospital's pulmonary hypertension database, we identified consecutive acute right heart failure hospitalizations in patients with PAH. We used longitudinal regression analysis with the generalized estimating equations method to identify factors associated with an increased likelihood of 90-day mortality or urgent transplantation.

Incidence, correlates, and consequences of acute kidney injury in patients with pulmonary arterial hypertension hospitalized with acute right-side heart failure.

Background: Though much is known about the prognostic influence of acute kidney injury (AKI) in left-side heart failure, much less is known about AKI in patients with pulmonary arterial hypertension (PAH).

Methods And Results: We identified consecutive patients with PAH who were hospitalized at Stanford Hospital for acute right-side heart failure. AKI was diagnosed according to the criteria of the Acute Kidney Injury Network.

A novel non-invasive method of estimating pulmonary vascular resistance in patients with pulmonary arterial hypertension.

Background: The assessment of pulmonary vascular resistance (PVR) plays an important role in the diagnosis and management of pulmonary arterial hypertension (PAH). The main objective of this study was to determine whether the noninvasive index of systolic pulmonary arterial pressure (SPAP) to heart rate (HR) times the right ventricular outflow tract time-velocity integral (TVI(RVOT)) (SPAP/[HR x TVI(RVOT)]) provides clinically useful estimations of PVR in PAH.

Methods: Doppler echocardiography and right-heart catheterization were performed in 51 consecutive patients with established PAH.

ERG evaluation of daily, high-dose sildenafil usage.

Purpose: Sildenafil can cause transient, mild ERG changes in healthy individuals taking large single doses. Although the drug was originally intended for intermittent use in erectile dysfunction, it has now been approved for chronic use in subjects with pulmonary arterial hypertension (PAH). The purpose of our study is to investigate possible ERG changes in subjects using large doses of sildenafil on a chronic daily basis.

The ethics of randomized clinical trials in pulmonary arterial hypertension.

Randomized clinical trials (RCTs) conducted during the past decade have shown that several therapies produce improvements in surrogate endpoints for patients with pulmonary arterial hypertension (PAH). Whether these therapies also influence clinical outcomes remains uncertain. These changes in the PAH landscape have raised several complex ethical issues regarding the conduct of RCTs in PAH, but to date, these issues have not been fully explored.

Health-related quality of life and patient-reported outcomes in pulmonary arterial hypertension.

Advances in our understanding of the basic pathophysiology of pulmonary arterial hypertension (PAH) has led to an expanding number of therapeutic options. The ultimate goals of therapy are to lengthen survival while improving symptoms and quality of life. A wealth of research in other conditions has established health-related quality of life (HRQoL) to be an important clinical endpoint.

Increased mortality in African Americans with idiopathic pulmonary arterial hypertension.

Background: Idiopathic pulmonary arterial hypertension (IPAH) is a progressive disorder that usually culminates in right ventricular failure and death without treatment.

Objective: To assess mortality trends by race and gender for idiopathic pulmonary arterial hypertension in the United States from 1994-1998.

Methods: The U.

Management strategies for patients with pulmonary hypertension in the intensive care unit.

Objective: Pulmonary hypertension may be encountered in the intensive care unit in patients with critical illnesses such as acute respiratory distress syndrome, left ventricular dysfunction, and pulmonary embolism, as well as after cardiothoracic surgery. Pulmonary hypertension also may be encountered in patients with preexisting pulmonary vascular, lung, liver, or cardiac diseases. The intensive care unit management of patients can prove extremely challenging, particularly when they become hemodynamically unstable.

Active bacterial myocarditis: a case report and review of the literature.

Bacterial myocarditis (BM) is an uncommon cause of infectious myocarditis. BM is usually seen in the context of overwhelming sepsis or as part of a specific bacterial syndrome. The definitive diagnosis of bacterial myocarditis requires biopsy or morphologically proven active myocarditis with evidence of bacterial invasion or positive tissue cultures.

Current therapies for pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) is a progressive disease of the pulmonary vasculature, characterized by relentless deterioration and death. Patients with PAH are known to be at increased risk for anesthetic complications and surgical morbidity and mortality. However, outcomes in patients have improved with the recent development of new drug therapies.

Pulmonary nocardiosis in a heart transplant patient: case report and review of the literature.

Pulmonary infection with Nocardia is an uncommon but serious infection found in immunocompromised patients. We describe a rapidly progressive pulmonary nocardiosis in a heart transplant patient. We then review the common clinical features of Nocardia infection in transplant recipients, outlining the challenges in its diagnosis and management.

Surgical and interventional therapies for pulmonary arterial hypertension.

Surgical and interventional therapies for pulmonary arterial hypertension (PAH) in appropriately selected patients have the potential to dramatically improve or, in some cases, cure PAH. These include atrial septostomy, a palliative procedure or bridge to transplantation in patients with refractory right heart failure, pulmonary thromboendarterectomy for pulmonary hypertension associated with chronic thromboembolic disease, and closure of congenital systemic-pulmonary shunts in patients with PAH but without significant pulmonary vascular disease. Lung transplantation should be considered for patients with all forms of PAH who demonstrate advanced or progressive disease.

Lung transplantation: a decade of experience.

Background: Over the past 3 decades, the field of lung transplantation has been refined. However, many barriers exist that limit long-term success. The purpose of this study was to review a single institution's long-term experience with single and double lung transplantation and to assess the effect of different immunosuppressive therapies on outcomes.

Health-related quality of life in patients with pulmonary arterial hypertension.

Study Objectives: Patients with pulmonary arterial hypertension (PAH) often present with dyspnea and severe functional limitations, but their health-related quality of life (HRQOL) has not been studied extensively. This study describes HRQOL in a cohort of patients with PAH.

Design: Cross-sectional study.

Prognosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines.

Although idiopathic pulmonary arterial hypertension is perceived as a progressive disease with a uniformly poor outcome, the natural history of disease is heterogeneous, with some patients dying within months of diagnosis and others living for decades. The course of the disease has also been altered by advances in medical therapies. The outcome of patients with other types of pulmonary arterial hypertension (PAH) has been less well characterized.

Surgical treatments/interventions for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines.

While considerable advances have been achieved in the medical treatment of pulmonary arterial hypertension (PAH) over the past decade, surgical and interventional approaches continue to have important roles in those patients for whom medical therapy is unavailable or has been unsuccessful. These techniques include pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension, thoracic transplantation, and atrial septostomy. This chapter will provide evidence-based recommendations for the selection and timing of surgical and interventional treatments of PAH for physicians involved in the care of these complex patients.

Matrix metalloproteinase inhibition decreases ischemia-reperfusion injury after lung transplantation.

Increased microvascular permeability and extravasation of inflammatory cells are key events of lung ischemia-reperfusion (IR) injury. The purpose of this study was to investigate the role of matrix metalloproteinases (MMP) in IR-induced alveolar capillary membrane disruption after experimental lung transplantation. We used a rat model of lung orthotopic transplantation (n = 86) with a prolonged cold ischemic phase.

Weight gain after lung transplantation.

Background: Weight gain is frequently observed after lung transplantation, but the magnitude, predictors and implications of weight gain after lung transplant are unknown.

Methods: This retrospective cohort study included 826 lung transplant recipients randomly selected from 12 international transplant centers. We included adult patients with available weight data at baseline and 1 year post-transplant.

PG490-88, a derivative of triptolide, attenuates obliterative airway disease in a mouse heterotopic tracheal allograft model.

The current treatment of obliterative bronchiolitis in lung transplant recipients is sub-optimal. Triptolide is a novel immunosuppressant that has a mechanism of action distinct from currently available immunosuppressants, including induction of T-cell apoptosis, blockade of fibroblast proliferation/maturation and inhibition of transforming growth factor-beta (TGF-beta) mRNA production. We hypothesized that triptolide may be helpful in blocking obliterative airway disease in lung transplant recipients.