APOLLO Summary on Pulmonary Vascular Disease Fellowship Training.

Pulmonary vascular disease (PVD), and in particular, pulmonary hypertension (PH), is a highly specialized area of medicine comprised of complex diagnostics, classification systems, risk assessment tools, and therapeutics, the correct application of which has been shown to impact patient outcomes. The PVD scientific and patient community recognizes the importance of standardization of care patterns and has thus implemented a clinical accreditation process for PH care centers across the United States. However, a similar standardization system is lacking in PVD sub-specialty provider training.

CTEPH: A Kaiser Permanente Northern California Experience.

Introduction: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare and life-threatening form of pulmonary hypertension and the only potentially curable form of the World Health Organization Pulmonary Hypertension classes. Thus, the prompt and accurate diagnosis of this condition is imperative. Despite widespread chronic symptoms following acute pulmonary embolism (PE), the condition is rarely considered, and an externally validated inexpensive diagnostic algorithm is lacking.

ISHLT consensus statement: Perioperative management of patients with pulmonary hypertension and right heart failure undergoing surgery.

Pulmonary hypertension (PH) is a risk factor for morbidity and mortality in patients undergoing surgery and anesthesia. This document represents the first international consensus statement for the perioperative management of patients with pulmonary hypertension and right heart failure. It includes recommendations for managing patients with PH being considered for surgery, including preoperative risk assessment, planning, intra- and postoperative monitoring and management strategies that can improve outcomes in this vulnerable population.

Amyloid cardiomyopathy in a large integrated health care system.

Background: Light Chain (AL) and transthyretin (ATTR) amyloidosis are the most common forms of amyloid cardiomyopathy. Population based studies describing the epidemiology and clinical features of amyloid cardiomyopathy are often based in tertiary medical centers and thus may be limited by referral bias.

Methods And Results: We performed a cohort study of 198 patients diagnosed and treated in the Kaiser Permanente Northern California health care system who had a confirmed diagnosis of cardiac amyloidosis between 2001 and 2016.

Performance of the REVEAL model in WHO Group 2 to 5 pulmonary hypertension: application beyond pulmonary arterial hypertension.

Background: The majority of patients with pulmonary hypertension (PH) have non-pulmonary arterial hypertension PH (non-PAH PH) or multifactorial PH. The REVEAL score was designed to predict 1-year survival in patients with pulmonary arterial hypertension (PAH) only. It is unknown whether this model is applicable to a more general population of PH patients.

Validation of the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL) pulmonary hypertension prediction model in a unique population and utility in the prediction of long-term survival.

Background: The Registry to Evaluate Early and Long-Term Pulmonary Arterial (PAH) Hypertension Disease Management (REVEAL) model was designed to predict 1-year survival in patients with PAH. Multivariate prediction models need to be evaluated in cohorts distinct from the derivation set to determine external validity. In addition, limited data exist on the utility of this model in the prediction of long-term survival.

Anesthesia and pulmonary hypertension.

Anesthesia and surgery are associated with significantly increased morbidity and mortality in patients with pulmonary hypertension due mainly to right ventricular failure, arrhythmias, postoperative hypoxemia, and myocardial ischemia. Preoperative risk assessment and successful management of patients with pulmonary hypertension undergoing cardiac surgery involve an understanding of the pathophysiology of the disease, screening of patients at-risk for pulmonary arterial hypertension, analysis of preoperative and operative risk factors, thorough multidisciplinary planning, careful intraoperative management, and early recognition and treatment of postoperative complications. This article will cover each of these aspects with particular focus on the anesthetic approach for non-cardiothoracic surgeries.

World Health Organization Pulmonary Hypertension group 2: pulmonary hypertension due to left heart disease in the adult--a summary statement from the Pulmonary Hypertension Council of the International Society for Heart and Lung Transplantation.

Pulmonary hypertension associated with left heart disease is the most common form of pulmonary hypertension encountered in clinical practice today. Although frequently a target of therapy, its pathophysiology remains poorly understood and its treatment remains undefined. Pulmonary hypertension in the context of left heart disease is a marker of worse prognosis and disease severity, but whether its primary treatment is beneficial or harmful is unknown.

Classification of pulmonary hypertension.

Pulmonary hypertension (PH) can develop in association with many different diseases and risk factors, and its presence is nearly always associated with reduced survival. The prognosis and management of PH is largely dependent upon its underlying etiology and severity of disease. The combination of clinical and hemodynamic classifications of PH provides a framework for the diagnostic evaluation of PH to establish a final clinical diagnosis that guides therapy.

Furthering the link between the sarcomere and primary cardiomyopathies: restrictive cardiomyopathy associated with multiple mutations in genes previously associated with hypertrophic or dilated cardiomyopathy.

Mutations in genes that encode components of the sarcomere are well established as the cause of hypertrophic and dilated cardiomyopathies. Sarcomere genes, however, are increasingly being associated with other cardiomyopathies. One phenotype more recently recognized as a disease of the sarcomere is restrictive cardiomyopathy (RCM).

Transplantation in adults with congenital heart disease.

Considerable progress in pediatric cardiac surgery has led to more patients with congenital heart disease surviving into adulthood. However, progressive cardiopulmonary dysfunction often occurs late after palliative or corrective surgeries to the point where transplantation becomes the only treatment option. Adult congenital heart disease represents a growing population of patients being referred for heart, lung, and combined heart-lung transplantation.

Increased CD62e(+) endothelial microparticle levels predict poor outcome in pulmonary hypertension patients.

Background: Endothelial and leukocytes-derived microparticles (EMPs and LMPs, respectively) are increased in patients with pulmonary hypertension (PH). We hypothesized that the levels of circulating EMPs and LMPs could predict outcome in these patients.

Methods: Patients undergoing right heart catheterization for untreated pre-capillary PH were eligible for the study.

Effect of pulmonary hypertension on clinical outcomes in advanced heart failure: analysis of the Evaluation Study of Congestive Heart Failure and Pulmonary Artery Catheterization Effectiveness (ESCAPE) database.

Background: Pulmonary hypertension has been shown to predict hospitalizations and mortality in patients with heart failure. We aimed to define the prevalence of mixed pulmonary hypertension (MPH; mean pulmonary artery pressure > or = 25 mm Hg, pulmonary capillary wedge pressure >15 mm Hg, and pulmonary vascular resistance > or = 3 Wood units), identify clinical predictors of MPH, and determine whether MPH predicts adverse outcomes in patients hospitalized with severe heart failure.

Methods: This is a subgroup analysis of patients assigned to pulmonary artery catheter placement in the Evaluation Study of Congestive Heart Failure and Pulmonary Artery Catheterization Effectiveness (ESCAPE) trial.

Analytic reviews: cardiogenic shock with preserved systolic function: a reminder.

Although cardiogenic shock, whether acute or chronic, most frequently results from depressed left ventricular systolic function, the same syndrome can occur in patients with preserved systolic function. The etiologies and the pathophysiology of the syndrome of cardiogenic shock with preserved ejection fraction are distinctly different from those with reduced ejection fraction. The therapeutic approaches are also different.

The clinical pharmacology of intranasal l-methamphetamine.

Background: We studied the pharmacology of l-methamphetamine, the less abused isomer, when used as a nasal decongestant.

Methods: 12 subjects self-administered l-methamphetamine from a nonprescription inhaler at the recommended dose (16 inhalations over 6 hours) then at 2 and 4 (32 and 64 inhalations) times this dose. In a separate session intravenous phenylephrine (200 microg) and l-methamphetamine (5 mg) were given to define alpha agonist pharmacology and bioavailability.

Circulating endothelial microparticle levels predict hemodynamic severity of pulmonary hypertension.

Rationale: Circulating microparticles (MPs) are submicron membrane fragments shed from damaged or activated vascular cells. Endothelial MPs are a biological marker of dysfunctional endothelium. Vascular remodeling and endothelial dysfunction are involved in pulmonary hypertension (PH).