Renal Outcomes After Lung or Combined Heart-Lung Transplantation in Pulmonary Hypertension.

Renal impairment is considered a contra-indication for lung (LTX) or combined heart-lung (HLTX) transplantation due to increased mortality. We hypothesized that renal impairment in pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) is the result of reduced cardiac output and should be partly reversible after LTX. We performed a retrospective analysis in 67 consecutive PAH and CTEPH patients who underwent (H)LTX, to investigate the postoperative evolution of renal function in function of baseline renal function using a mixed model effect test.

Prediction of Left Ventricular Filling Pressures Using Natural Shear Waves: Incremental Value Over Conventional Echocardiography.

Background: Increased myocardial stiffness can lead to diastolic dysfunction, resulting eventually in increased left ventricular filling pressures (LVFPs). The clinical assessment of left ventricular (LV) diastolic function is challenging. Shear wave elastography is a novel method based on tracking shear waves, such as those induced by mitral valve closure (MVC), using high frame rate (HFR) echocardiography.

Management of chronic thromboembolic pulmonary hypertension.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a severe and progressive disease. Three treatment modalities are available: pulmonary endarterectomy, balloon pulmonary angioplasty, and pulmonary hypertension (PH) medical therapy. Both mechanical therapies may also be considered in patients with chronic thromboembolic pulmonary disease without PH.

Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension: Results of an International Multicenter Prospective Registry.

Background: Chronic thromboembolic pulmonary hypertension results from mechanical obstruction of major pulmonary artery lumina with fibrotic tissue. Main treatment has been pulmonary endarterectomy, a complex surgical procedure removing vascular obstruction. However, at least 40% of patients are not candidates for pulmonary endarterectomy because of technical inoperability, comorbidities, or limited access to surgery.

Endothelial Features Along the Pulmonary Vascular Tree in Chronic Thromboembolic Pulmonary Hypertension: Distinctive or Shared Facets?

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of pulmonary embolism, characterized by the presence of organized fibro-thrombotic material that partially or fully obstructs the lumen of large pulmonary arteries, microvasculopathy, and enlargement of the bronchial systemic vessels. The precise mechanisms underlying CTEPH remain unclear. However, defective angiogenesis and altered pulmonary arterial endothelial cell (PAEC) function may contribute to disease progression.

Effects of macitentan and selexipag across prognostic age groups in patients with pulmonary arterial hypertension.

Background: Age affects disease severity and patient outcomes in pulmonary arterial hypertension. This post-hoc analysis identified prognostic age groups and associated macitentan/selexipag treatment effects.

Methods: Randomized trials evaluated macitentan (SERAPHIN; NCT00660179) and selexipag (GRIPHON; NCT01106014) versus placebo (primary endpoint: time to morbidity/mortality [M/M]).

Mono and combination therapies in pulmonary arterial hypertension patients with comorbidities: A COMPERA analysis.

Aims: Pulmonary arterial hypertension (PAH) is often diagnosed in elderly patients with comorbidities. Although initial monotherapy is recommended for these patients, the value of combination therapy remains unclear. Here, we compare the efficacy of initial monotherapy and combination therapy in PAH patients with cardiovascular comorbidities.

Corrigendum to 'Pulmonary endarterectomy through inverted-T upper hemisternotomy'[JTCVS Techniques Volume 28, December 2024, Pages 65-72].

[This corrects the article DOI: 10.1016/j.xjtc.

Uncommon Causes of Pulmonary Hypertension With Associated Cardiomyopathy: Computed Tomography and Magnetic Resonance Imaging of Cardiothoracic Manifestations.

Pulmonary hypertension (PH) is a disease characterized by pathologically increased pressure in the pulmonary arteries, defined by a mean pulmonary arterial pressure (mPAP) >20 mmHg at rest measured with right heart catheterization (RHC). This definition encompasses pathologies with very different pathological backgrounds, ultimately resulting in PH. For this reason, the latter can be possibly (though seldom) accompanied by cardiomyopathies, pathologies characterized by a structural and functionally abnormal myocardium not secondary to coronary disease, hypertension, valvular disease, or congenital heart disease.

Cost-effectiveness of follow-up algorithms for chronic thromboembolic pulmonary hypertension in pulmonary embolism survivors.

Introduction: Achieving an early diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH) in pulmonary embolism (PE) survivors results in better quality of life and survival. Importantly, dedicated follow-up strategies to achieve an earlier CTEPH diagnosis involve costs that were not explicitly incorporated in the models assessing their cost-effectiveness. We performed an economic evaluation of 11 distinct PE follow-up algorithms to determine which should be preferred.

Pulmonary endarterectomy through inverted-T upper hemisternotomy.

Objective: We aimed to explore the feasibility of an inverted-T upper hemisternotomy approach for pulmonary endarterectomy (PEA) and report the results after 17 cases.

Methods: PEA was conducted through a 7-cm skin incision using an inverted-T upper hemisternotomy across the third intercostal spaces. Cardiopulmonary bypass (CPB) was established through central arterial and percutaneous femoral dual-staged venous cannulation.

The volume-outcome relationship for pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension.

Background: We conducted a volume-outcome meta-analysis of pulmonary endarterectomy procedures for chronic thromboembolic pulmonary hypertension to objectively determine the minimum required annual case load that can define a high-volume centre.

Methods: Three electronic databases were systematically queried up to 1 May 2024. Centres were divided in volume tertiles.

Efficacy and safety of selexipag in patients with inoperable or persistent/recurrent CTEPH (SELECT randomised trial).

Background: SELECT was the first global randomised controlled trial of selexipag with standard of care in patients with inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension.

Methods: SELECT was a multicentre, randomised, double-blind, placebo-controlled, parallel-group, group-sequential, phase 3 study (ClinicalTrials.gov: NCT03689244).

Incidence and clinical course of chronic thromboembolic pulmonary hypertension with or without a history of venous thromboembolism in Denmark.

Background: A considerable number of patients with chronic thromboembolic pulmonary hypertension (CTEPH) lack a history of venous thromboembolism (VTE).

Objectives: We aimed to examine the annual incidence and prevalence of CTEPH in Denmark and to compare the rates of VTE, bleeding, and mortality between CTEPH patients with and without a history of VTE.

Methods: The Danish National Patient Registry covering all Danish hospitals was used to identify all CTEPH cases between 2009 and 2018, based on combinations of discharge diagnoses using International Classification of Diseases, 10th Revision codes for CTEPH and relevant diagnostic and/or therapeutic interventions.

Worldwide CTEPH Registry: Long-Term Outcomes With Pulmonary Endarterectomy, Balloon Pulmonary Angioplasty, and Medical Therapy.

Background: The European Chronic Thromboembolic Pulmonary Hypertension (CTEPH) registry, conducted between 2007 and 2012, reported the major impact of pulmonary endarterectomy (PEA) on the long-term survival of patients with CTEPH. Since then, 2 additional treatments for inoperable CTEPH have become available: balloon pulmonary angioplasty (BPA), and an approved oral drug therapy with the guanylate cyclase stimulator riociguat. The current registry aimed to evaluate the effect of these new therapeutic approaches in a worldwide context.

Chronic thromboembolic pulmonary disease.

Chronic thromboembolic pulmonary hypertension is a complication of pulmonary embolism and a treatable cause of pulmonary hypertension. The pathology is a unique combination of mechanical obstruction due to failure of clot resolution, and a variable degree of microvascular disease, that both contribute to pulmonary vascular resistance. Accordingly, multiple treatments have been developed to target the disease components.

Optimisation of detecting chronic thromboembolic pulmonary hypertension in acute pulmonary embolism survivors: the InShape IV study.

Introduction: Chronic thromboembolic pulmonary hypertension (CTEPH) is often diagnosed late in acute pulmonary embolism survivors: more efficient testing to expedite diagnosis may considerably improve patient outcomes. The InShape II algorithm safely rules out CTEPH (failure rate 0.29%) while requiring echocardiography in only 19% of patients but may be improved by adding detailed reading of the computed tomography pulmonary angiography diagnosing the index pulmonary embolism.

Shared and Distinct Genomics of Chronic Thromboembolic Pulmonary Hypertension and Pulmonary Embolism.

Chronic thromboembolic pulmonary hypertension involves the formation and nonresolution of thrombus, dysregulated inflammation, angiogenesis, and the development of a small-vessel vasculopathy. We aimed to establish the genetic basis of chronic thromboembolic pulmonary hypertension to gain insight into its pathophysiological contributors. We conducted a genome-wide association study on 1,907 European cases and 10,363 European control subjects.

Angiogenesis in Chronic Thromboembolic Pulmonary Hypertension: A Janus-Faced Player?

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare form of pulmonary hypertension characterized by the presence of organized thrombi that obstruct pulmonary arteries, ultimately leading to right heart failure and death. Among others, impaired angiogenesis and inflammatory thrombosis have been shown to contribute to the progression of CTEPH. In this review, we summarize the 2-faced nature of angiogenesis in both thrombus formation and resolution in the context of CTEPH and highlight the dual role of angiogenesis and neovascularization in resolving venous thrombi.

Clinical-radiological-pathological correlation in chronic thromboembolic pulmonary hypertension.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare and potentially life-threatening complication of acute pulmonary embolism. It is characterised by persistent fibro-thrombotic pulmonary vascular obstructions and elevated pulmonary artery pressure leading to right heart failure. The diagnosis is based on two steps, as follows: 1) suspicion based on symptoms, echocardiography and ventilation/perfusion scan and 2) confirmation with right heart catheterisation, computed tomography pulmonary angiography and, in most cases, digital subtraction angiography.

Preexisting Chronic Thromboembolic Pulmonary Hypertension in Acute Pulmonary Embolism? A Case Report and Discussion.

A 61-year-old male presented with New York Heart Association class II breathlessness. Three years earlier, he had presented with a swollen leg, had received a diagnosis of deep vein thrombosis on ultrasound and of low-risk acute pulmonary embolism, and had been discharged on a direct oral anticoagulant after 8 hours. The patient also had a history of thyroidectomy and was on levothyroxine substitution.

Pulmonary arterial hypertension drugs can partially restore altered angiogenic capacities in -silenced human lung microvascular endothelial cells.

Mutations in the () gene and signaling pathway impairment are observed in heritable and idiopathic pulmonary arterial hypertension (PAH). In PAH, endothelial dysfunction is currently handled by drugs targeting the endothelin-1 (ET-1), nitric oxide (NO), and prostacyclin (PGI) pathways. The role of angiogenesis in the disease process and the effect of PAH therapies on dysregulated angiogenesis remain inconclusive.