Pericardiocentesis in Severe Pulmonary Arterial Hypertension Guided by a Pulmonary Artery Catheter.

Patients, often with underlying rheumatologic disease, may present with pericardial effusions in the setting of pulmonary hypertension (PHTN). Pericardial drainage in PHTN is associated with significant morbidity and mortality. We describe a patient with PHTN who developed cardiac tamponade that was managed safely and effectively with pulmonary artery catheter-guided pericardiocentesis.

Pulmonary Vasodilator Therapy in Severe Pulmonary Hypertension Due to Chronic Obstructive Pulmonary Disease (Severe PH-COPD): A Systematic Review and Meta-Analysis.

Chronic obstructive pulmonary disease-associated pulmonary hypertension (PH-COPD) results in a significant impact on symptoms, quality of life, and survival. There is scant and conflicting evidence about the use of pulmonary hypertension (PH) specific therapy in patients with PH-COPD. PubMed, OVID, CINAHL, Cochrane, Embase, and Web of Science were searched using various MESH terms to identify randomized controlled trials (RCTs) or observational studies investigating PH-specific therapies in patients with severe PH-COPD, defined by mean pulmonary artery pressure (mPAP) of more than 35 mm Hg or pulmonary vascular resistance (PVR) of more than 5 woods units on right heart catheterization.

Approach to the hospitalized patient with pulmonary arterial hypertension.

Purpose Of Review: Hospitalization in pulmonary arterial hypertension (PAH) patients is an important clinical worsening event significantly associated with subsequent mortality. Furthermore, irrespective of the cause of hospitalization, the overall outcome is closely related to the severity of the right ventricular (RV) dysfunction. Therefore, understanding the pathophysiology of pulmonary hypertension and RV failure is paramount in successfully managing PAH patients requiring hospitalization.

Undiagnosed ventricular septal defect with resultant Eisenmenger syndrome presenting with diplopia.

Ventricular septal defect (VSD) is the most common congenital heart lesion among children. In most cases, however, it is identified and corrected in childhood, before long-term sequelae such as pulmonary hypertension develop. In this case report, we present a young man with an undiagnosed VSD with consequent Eisenmenger syndrome who initially presented to medical attention with diplopia found to be caused by cerebral infarcts.

Delphi consensus recommendation for optimization of pulmonary hypertension therapy focusing on switching from a phosphodiesterase 5 inhibitor to riociguat.

Dual combination therapy with a phosphodiesterase-5 inhibitor (PDE5i) and endothelin receptor antagonist is recommended for most patients with intermediate-risk pulmonary arterial hypertension (PAH). The RESPITE and REPLACE studies suggest that switching from a PDE5i to a soluble guanylate cyclase (sGC) activator may provide clinical improvement in this situation. The optimal approach to escalation or transition of therapy in this or other scenarios is not well defined.

Advances in the management of pulmonary arterial hypertension.

The management of pulmonary arterial hypertension (PAH) has significantly evolved over the last decades in the wake of more sensitive diagnostics and specialized clinical programs that can provide focused medical care. In the current era of PAH care, 1-year survival rates have increased to 86%-90% from 65% in the 1980s, and average long-term survival has increased to 6 years from 2.8 years.

Cor Pulmonale from Concomitant Human Immunodeficiency Virus Infection and Methamphetamine Use.

• HIV and frequent methamphetamine use are two known risk factors for PAH development. • HIV and methamphetamine are both associated with higher rates of mortality in PAH. • Echocardiography is an effective noninvasive modality for assessing PAH severity.

COVID-19 in Pulmonary Artery Hypertension (PAH) Patients: Observations from a Large PAH Center in New York City.

Information on outcomes of COVID-19 in pulmonary arterial hypertension (PAH) patients is limited to a few case series and surveys. Here, we describe our experience at a large Pulmonary Hypertension Center in New York City at the height of the pandemic. We performed a retrospective chart review of eleven consecutive PAH patients who were diagnosed with SARS-CoV-2 infection.

Imaging of Pulmonary Hypertension: Pictorial Essay.

Pulmonary hypertension (PH) is an end result of a diverse array of complex clinical conditions that invoke hemodynamic and pathophysiological changes in the pulmonary vasculature. Many patients' symptoms begin with dyspnea on exertion for which screening tests such as chest roentgenograms and more definitive noninvasive tests such as CT scans are ordered initially. It is imperative that clinicians are cognizant of subtle clues on these imaging modalities that alert them to the possibility of PH.

Clinical and hemodynamic benefit of macitentan and riociguat upfront combination in patients with pulmonary arterial hypertension.

In this open-label study, we evaluated the effect of upfront macitentan and riociguat combination in newly diagnosed pulmonary arterial hypertension (PAH) patients. In 15 consecutive PAH patients, we collected clinical and hemodynamic data at baseline, visit 1 (median 4 months) and visit 2 (median 12 months). Survival and transplantation status were analyzed over 36 months.

Exercise-induced pulmonary hypertension by stress echocardiography: Prevalence and correlation with right heart hemodynamics.

Objectives: The aim of this study was to determine the prevalence of exercise-induced pulmonary hypertension (EIPH) in consecutive subjects referred for stress echocardiography for chest pain or shortness of breath and correlate echocardiographic diagnosis of EIPH with hemodynamics at right heart catheterization (RHC).

Background: Elevated pulmonary pressure can lead to significant morbidity and mortality. EIPH by ehocardiography has been described in patients with connective tissue disease.

Pulmonary Hypertension Due to Common Respiratory Conditions: Classification, Evaluation and Management Strategies.

Pulmonary hypertension (PH) due to chronic respiratory disease and/or hypoxia is classified as World Health Organization (WHO) Group III pulmonary hypertension. The patients most commonly encountered in clinical practice with group III PH include those with chronic obstructive lung disease (COPD), diffuse parenchymal lung disease, and sleep-disordered breathing. The purpose of this review is to outline the variable clinical significance of pulmonary hypertension in the most common pulmonary disease states and how a clinician may approach the management of these patients.

Early Observations on the Use of Riociguat in a Large, Metropolitan Pulmonary Arterial Hypertension/Chronic Thromboembolic Pulmonary Hypertension Treatment Center.

Introduction: Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are rare, life-threatening diseases in which chronically elevated pressure in the pulmonary arteries results in vascular remodeling and right heart failure. Treatment goals are to improve patient functioning, exercise capacity, and symptoms; delay disease progression; normalize the right ventricular function; and, ultimately, improve survival. Therapeutic management centers on the affected physiologic pathways and includes endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and prostacyclins.

Successfully treated calcific uremic arteriolopathy: two cases of a high anion gap metabolic acidosis with intravenous sodium thiosulfate.

Calcific uremic arteriolopathy (CUA) is a rare and potentially fatal disorder of calcification involving subcutaneous small vessels and fat in patients with renal insufficiency. We describe the successful use of intravenous sodium thiosulfate (STS) for the treatment of CUA in two patients. The first case was complicated by the development of a severe anion gap metabolic acidosis, which was accompanied by a seizure.

A retrospective study on the effects of pulmonary rehabilitation in patients with pulmonary hypertension.

A retrospective analysis of 23 patients was undertaken to evaluate the outcome of pulmonary rehabilitation (PR) in patients with pulmonary hypertension (PH) over a preceding 6-year time frame. Chart review and data analyses were undertaken evaluating pulmonary arterial hypertension pharmacotherapy versus the same therapy with the addition of PR. Analysis included 23 patients who had a mean pulmonary artery pressure of 36.

A multicenter, retrospective study of patients with pulmonary arterial hypertension transitioned from parenteral prostacyclin therapy to inhaled iloprost.

Pulmonary arterial hypertension (PAH) is characterized by progressive increases in pulmonary vascular resistance, leading to right heart failure and death. Guidelines recommend customization of treatment, necessitating the development of effective strategies for transitioning patients among treatments. In this study, we characterized our experience with patient transitions from parenteral prostacyclin to inhaled iloprost.

Endpoints for clinical trials of sarcoidosis.

Over the past few years an increasing number of prospective controlled sarcoidosis treatment trials have been completed. Unfortunately, these studies utilize different endpoints making comparisons between studies difficult. At the recent World Association of Sarcoidosis and other Granulomatous disease (WASOG) meeting, a session was dedicated to the evaluation of clinical endpoints for various disease manifestations.

Sarcoidosis-associated pulmonary hypertension: assessment and management.

Pulmonary hypertension (PH) is a recognized complication of sarcoidosis, with increased morbidity and poor prognosis. Sarcoidosis-associated pulmonary hypertension (SAPH) is typically seen in advanced cases, with pulmonary fibrosis, destruction and obliteration of the pulmonary vasculature, and chronic hypoxemia. PH can, however, occur in the absence of pulmonary fibrosis, suggesting alternative pathophysiological mechanisms.

Prevalence and correlates of septal delayed contrast enhancement in patients with pulmonary hypertension.

Using cardiac magnetic resonance, the presence of myocardial delayed contrast enhancement (DCE) has been described in the ventricular septum at the level of the right ventricular insertion points in patients with pulmonary hypertension (PH). The aim of this study was to investigate the prevalence, extent, and correlates of this finding. Septal DCE was evaluated in 55 patients with known or suspected PH of various causes.

Pulmonary hypertension: accuracy of detection with left ventricular septal-to-free wall curvature ratio measured at cardiac MR.

Purpose: To retrospectively evaluate the accuracy and reproducibility of the cardiac magnetic resonance (MR) imaging-derived left ventricular septal-to-free wall curvature ratio for prediction of the right ventricular systolic pressure (RVSP) in patients clinically known to have or suspected of having pulmonary hypertension (PH), with same-day right-side heart catheterization (RHC) as the reference standard.

Materials And Methods: Institutional review board approval was received for this HIPAA-compliant study. Sixty-one patients clinically known or suspected of having PH underwent cardiac MR and RHC on the same day.

Pulmonary arterial hypertension: noninvasive detection with phase-contrast MR imaging.

Purpose: To retrospectively identify pulmonary arterial (PA) flow parameters measured with phase-contrast magnetic resonance (MR) imaging that allow noninvasive diagnosis of chronic PA hypertension (PAH).

Materials And Methods: The study was HIPAA compliant and was approved by the institutional review board; a waiver of informed consent was obtained. Fifty-nine patients (49 female patients; mean age, 46 years; range, 16-85 years) known to have or suspected of having PAH underwent breath-hold phase-contrast MR imaging and right-sided heart catheterization (RHC).

Pulmonary artery sarcoma misdiagnosed as chronic thromboembolic pulmonary hypertension.

Pulmonary artery sarcomas are rare neoplasms of the pulmonary artery that are often confused with chronic thromboembolic disease, as both diseases have similar presentations. In patients with presumed chronic thromboembolic pulmonary hypertension, certain clinical and imaging characteristics may suggest the alternative diagnosis of pulmonary artery sarcoma. In this article we present a case of a man initially diagnosed with chronic thromboembolic pulmonary hypertension, but who was later found to have pulmonary artery sarcoma.