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Atrial arrhythmias (AA) are common in patients with pulmonary hypertension (PH) and contribute to morbidity and mortality. Given the growing PH population, understanding the pathophysiology, clinical impact, and management of AA in PH is important. AA occurs in PH with a 5-year incidence of 10% to 25%. AA confers a higher morbidity and mortality, and restoration of normal sinus rhythm improves survival and functionality. AA is thought to develop because of structural alterations of the right atrium caused by changes to the right ventricle (RV) due to elevated pulmonary artery pressures. AA can subsequently worsen RV function. Current guidelines do not provide comprehensive recommendations for the management of AA in PH. Robust evidence to favor a specific treatment approach is lacking. Although the role of medical rate or rhythm control, and the use of cardioversion and ablation, can be inferred from other populations, evidence is lacking in the PH population. Much remains to be determined regarding the optimal management strategy. We present here our institutional approach and discuss areas for future research. This review highlights the epidemiology and pathophysiology of AA in patients with PH, describes the relationship between AA and RV dysfunction, and discusses current management practices. We outline our institutional approach and offer directions for future investigation. [ABSTRACT FROM AUTHOR]

The sex differences in use, safety outcomes, and health-care resource use of patients with pulmonary embolism (PE) undergoing percutaneous pulmonary artery thrombectomy are not well characterized. What are the sex differences in outcomes for patients diagnosed with PE who undergo percutaneous pulmonary artery thrombectomy? This retrospective cross-sectional study used national inpatient claims data to identify patients in the United States with a discharge diagnosis of PE who underwent percutaneous thrombectomy between January 2016 and December 2018. We evaluated the demographics, comorbidities, safety outcomes (in-hospital mortality), and health-care resource use (discharge to home, length of stay, and hospital charges) of patients with PE undergoing percutaneous thrombectomy. Among 1,128,904 patients with a diagnosis of PE between 2016 and 2018, 5,160 patients (0.5%) underwent percutaneous pulmonary artery thrombectomy. When compared with male patients, female patients showed higher procedural bleeding (16.9% vs 11.2%; P <.05), required more blood transfusions (11.9% vs 5.7%; P <.05), and experienced more vascular complications (5.0% vs 1.5%; P <.05). Women experienced higher in-hospital mortality (16.9% vs 9.3%; adjusted OR, 1.9; 95% CI, 1.2-3.0; P =.003) when compared with men. Although length of stay and hospital charges were similar to those of men, women were less likely to be discharged home after surviving hospitalization (47.9% vs 60.3%; adjusted OR, 0.7; 95% CI, 0.50-0.99; P =.04). In this large nationwide cohort, women with PE who underwent percutaneous thrombectomy showed higher morbidity and in-hospital mortality compared with men. [ABSTRACT FROM AUTHOR]

Background: Pulmonary hypertension (PH) is a common complication of interstitial lung disease (ILD) and is associated with worse outcomes and increased mortality. Evaluation of PH is recommended in lung transplant candidates, but there are currently no standardized screening approaches. Trials have identified therapies that are effective in this setting, providing another rationale to routinely screen patients with ILD for PH.Research Question: What screening strategies for identifying PH in patients with ILD are supported by expert consensus?Study Design and Methods: The study convened a panel of 16 pulmonologists with expertise in PH and ILD, and used a modified Delphi consensus process with three surveys to identify PH screening strategies. Survey 1 consisted primarily of open-ended questions. Surveys 2 and 3 were developed from responses to survey 1 and contained statements about PH screening that panelists rated from -5 (strongly disagree) to 5 (strongly agree).Results: Panelists reached consensus on several triggers for suspicion of PH including the following: symptoms, clinical signs, findings on chest CT scan or other imaging, abnormalities in pulse oximetry, elevations in brain natriuretic peptide (BNP) or N-terminal pro-brain natriuretic peptide (NT-proBNP), and unexplained worsening in pulmonary function tests or 6-min walk distance. Echocardiography and BNP/NT-proBNP were identified as screening tools for PH. Right heart catheterization was deemed essential for confirming PH.Interpretation: Many patients with ILD may benefit from early evaluation of PH now that an approved therapy is available. Protocols to evaluate patients with ILD often overlap with evaluations for pulmonary hypertension-interstitial lung disease and can be used to assess the risk of PH. Because standardized approaches are lacking, this consensus statement is intended to aid physicians in the identification of patients with ILD and possible PH, and provide guidance for timely right heart catheterization. [ABSTRACT FROM AUTHOR]

Although long neglected, the right side of the heart (RH) is now widely accepted as a pivotal player in heart failure (HF) either with reduced or preserved ejection fraction. The chronic overload of the pulmonary microcirculation results in an initial phase characterized by right ventricular (RV) hypertrophy, right atrial dilation, and diastolic dysfunction. This progresses to overt RH failure when RV dilation and systolic dysfunction lead to RV-pulmonary arterial (RV-PA) uncoupling with low RV output. In the context of its established relevance to progression of HF, clinicians should consider assessment of the RH with information from clinical assessment, biomarkers, and imaging. Notably, no single parameter can predict prognosis alone in HF. Assessments simultaneously should encompass RV systolic function, pulmonary pressures, an estimation of RV-PA coupling, and RH morphologic features. Despite a large volume of evidence indicating the relevance of RH function to the clinical syndrome of HF, evidence-based management strategies are lacking. Targeting RH dysfunction in HF should be an objective of future investigations, being an unmet need in the current management of HF. [ABSTRACT FROM AUTHOR]

Sweet's Syndrome (SS), also known as acute febrile neutrophilic dermatosis, is one of several cutaneous inflammatory disorders classified as neutrophilic dermatoses. Respiratory complications are described in <50 cases in the literature,1 without prior report of lung transplantation (LT). This article explains the clinical course of the first patient to receive LT for pulmonary SS and presents evidence suggesting recurrence of the primary lung disease in the allograft. [ABSTRACT FROM AUTHOR]

Background: The most important determinant of long-term survival in patients with idiopathic pulmonary fibrosis is the right ventricular (RV) adaptation to the increased pulmonary vascular resistance. Our aim was to explore RV contractile reserve during stress echocardiography in early-stage IPF.Methods: Fifty early-stage patients with IPF and 50 healthy control patients underwent rest and stress echocardiography, including RV two-dimensional speckle tracking echocardiography. At peak exertion, blood gas analysis and spirometry were also assessed.Results: At rest, RV diameters were mildly increased in IPF; however, although RV conventional systolic function indexes were similar between the IPF and control groups, RV global longitudinal strain and RV lateral wall longitudinal strain (LWLS) were significantly reduced in the IPF cohort. During physical exercise, patients with IPF showed a reduced exercise tolerance with lower maximal workload (P < .01), level of oxygen saturation (P < .001), and peak heart rate (P < .01). Systolic and diastolic BP values were similar in both groups. Systolic pulmonary artery pressure (PAPs) increase (ΔPAPs) during exertion was higher in IPF vs healthy subjects (P < .0001); RV LWLS increase (ΔRV LWLS) during exercise was lower in patients with IPF vs control patients (P < .00001). By multivariable analysis, RV LWLS at rest and ΔRV LWLS were directly related to peak exertion capacity, PAPs, and blood oxygen saturation level (Spo2; P < .0001). Δ RV LWLS was directly related to diffusion lung carbon monoxide (P < .0001).Conclusions: RV myocardial dysfunction is already present at rest in early-stage IPF and worsens during exertion as detected by two-dimensional speckle-tracking echocardiography. The RV altered contractile reserve appears to be related to reduced exercise tolerability and impaired pulmonary hemodynamic. [ABSTRACT FROM AUTHOR]

COUNTERPOINT: Should Initial Combination Therapy Be the Standard of Care in Pulmonary Arterial Hypertension? As mentioned, in the AMBITION trial, the only positive randomized controlled trial to date studying up-front combination therapy, 31% of patients in the monotherapy arm had a "clinical failure" event.[1] In other words, 69% of patients on monotherapy met criteria for "clinical success"! In conclusion, although up-front combination therapy may be overall more beneficial than monotherapy in newly diagnosed patients, this approach does not necessarily need to be applied to all patients with PAH. 15 G. Simonneau, L.J. Rubin, N. Galiè, Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomized trial. [Extracted from the article]

All patients were receiving background iTRE at the time of randomization to ESU or placebo (PBO), and background oral PAH therapy was allowed (e.g. endothelin receptor antagonists and/or phosphodiesterase-5 inhibitors/soluble guanylate cyclase stimulators but no other prostanoids). Research Grant relationship with United Therapeutics Please note: $5001 - $20000 Added 03/14/2019 by Hubert Ford, source=Web Response, value=Grant/Research Support Research grant relationship with United therapeutics Please note: $20001 - $100000 Added 06/20/2019 by Shelley Shapiro, source=Web Response, value=Grant/Research Support. [Extracted from the article]

Exercise stress testing of the pulmonary circulation for the diagnosis of latent or early-stage pulmonary hypertension (PH) is gaining acceptance. There is emerging consensus to define exercise-induced PH by a mean pulmonary artery pressure > 30 mm Hg at a cardiac output < 10 L/min and a total pulmonary vascular resistance> 3 Wood units at maximum exercise, in the absence of PH at rest. Exercise-induced PH has been reported in association with a bone morphogenetic receptor-2 gene mutation, in systemic sclerosis, in left heart conditions, in chronic lung diseases, and in chronic pulmonary thromboembolism. Exercise-induced PH is a cause of decreased exercise capacity, may precede the development of manifest PH in a proportion of patients, and is associated with a decreased life expectancy. Exercise stress testing of the pulmonary circulation has to be dynamic and rely on measurements of the components of the pulmonary vascular equation during, not after exercise. Noninvasive imaging measurements may be sufficiently accurate in experienced hands, but suffer from lack of precision, so that invasive measurements are required for individual decision-making. Exercise-induced PH is caused either by pulmonary vasoconstriction, pulmonary vascular remodeling, or by increased upstream transmission of pulmonary venous pressure. This differential diagnosis is clinical. Left heart disease as a cause of exercise-induced PH can be further ascertained by a pulmonary artery wedge pressure above or below 20 mm Hg at a cardiac output < 10 L/min or a pulmonary artery wedge pressure-flow relationship above or below 2 mm Hg/L/min during exercise. [ABSTRACT FROM AUTHOR]

Humbert and Lau[1] make a rational argument for up-front combination therapy in pulmonary arterial hypertension (PAH); however, the data supporting up-front combination therapy in incident PAH are derived from a single randomized controlled trial (AMBITION [A Study of First-Line Ambrisentan and Tadalafil Combination Therapy in Subjects With Pulmonary Arterial Hypertension (PAH)]),[2] whereas there is a wealth of data supporting up-front monotherapy and subsequent sequential combination therapy to achieve treatment goals.[[3]] We can also agree that the majority of patients (>two-thirds) in the pooled monotherapy arm in the AMBITION study did not have a morbidity/mortality event; similarly, a majority of patients (>70%) in the up-front combination therapy arm did not meet criteria for a "satisfactory clinical response". Thus, one possible approach to incident PAH would be up-front parenteral prostacyclin monotherapy and the sequential addition of other PAH therapy based on clinical reassessment and treatment goals. The current treatment algorithm for PAH recommends up-front combination therapy with an endothelin receptor antagonist and phosphodiesterase type 5 inhibitor. [Extracted from the article]

B PURPOSE: b Patients with pulmonary fibrosis (PF) and concomitant pulmonary hypertension (PH) are known to have more severe hypoxemia compared to those without PH. Of patients with PF and severe PAH, IPS was most common in CTD (23%) followed by IPF (14%) and HP (9%). B CONCLUSIONS: b In patients with end-stage PF, concomitant severe PAH is strongly associated with the presence of IPS and occurs most commonly in CTD related fibrosis. [Extracted from the article]

Though it is most commonly reported in renal transplant recipients and predominately affects the skin, KS has also been reported in heart, liver, bone marrow, and lung transplant recipients. Of these nine patients, three had isolated endobronchial KS, five had isolated intrapulmonary KS, and one patient had both endobronchial and intrapulmonary involvement. B CONCLUSIONS: b Although KS in non-HIV, lung transplant recipients is exceedingly rare, the increasing number of solid organ transplants will likely increase the prevalence of pulmonary KS. [Extracted from the article]