Feasibility and safety of endomyocardial biopsy by lumenless pacing lead sheath-directed method during cardiac implantable electronic device implantation.

Background: Patients with an indication for a cardiovascular implantable electronic device (CIED) are complicated with special cardiomyopathy or other unspecified cardiac abnormalities and may need endomyocardial biopsy (EMB). However, EMB by a bioptome is usually avoided to reduce the risk of lead displacement in the CIED periprocedural period.

Objective: We aimed to assess the safety and feasibility of a novel approach for transvenous right ventricular (RV) EMB using the lead sheath method (L-S-M) during CIED implantation and compared it with the traditional bioptome method (T-B-M).

Clonal Hematopoiesis of Indeterminate Potential in Chronic Thromboembolic Pulmonary Hypertension: A Multicenter Study.

Background: The pathogenesis of chronic thromboembolic pulmonary hypertension (CTEPH) is multifactorial and growing evidence has indicated that hematological disorders are involved. Clonal hematopoiesis of indeterminate potential (CHIP) has recently been associated with an increased risk of both hematological malignancies and cardiovascular diseases. However, the prevalence and clinical relevance of CHIP in patients with CTEPH remains unclear.

Congenital thrombophilia in East-Asian venous thromboembolism population: a systematic review and meta-analysis.

Background: Various inherited traits contribute to the overall risk of venous thromboembolism (VTE). In addition, the epidemiology of thrombophilia in the East-Asian VTE population remains unclear; thus, we aimed to assess the proportion of hereditary thrombophilia via a meta-analysis.

Methods: Publications from PubMed, EMBASE, web of science, and Cochrane before December 30, 2022, were searched.

Percutaneous transluminal pulmonary angioplasty for Takayasu arteritis-associated pulmonary hypertension: A single-arm meta-analysis.

Background: The efficacy and safety of percutaneous transluminal pulmonary angioplasty (PTPA) for Takayasu arteritis-associated pulmonary hypertension (TA-PH) remain unclear.

Objectives: To examine the efficacy and safety of PTPA in TA-PH.

Methods: PubMed, Embase, and the Cochrane Central Register of Controlled Trials Library were searched from inception to August 18, 2022, for articles investigating the efficacy and safety of PTPA for TA-PH.

Time-Velocity Integral of Left Ventricular Outflow Tract Predicts Worse Long-Term Survival in Pulmonary Arterial Hypertension.

Background: The time-velocity integral of the left ventricular outflow tract (TVI) has been demonstrated to correlate with heart failure hospitalization and mortality, but the association of TVI with the severity and prognosis of pulmonary arterial hypertension (PAH) has not been evaluated.

Objectives: The aim of this study was to investigate the predictive value of baseline TVI in PAH.

Methods: A total of 225 consecutive patients with a diagnosis of incident PAH were prospectively studied and echocardiology-derived TVI was measured at enrollment followed by right heart catheterization examination within 48 hours.

Percutaneous Pulmonary Angioplasty for Patients With Takayasu Arteritis and Pulmonary Hypertension.

Background: Percutaneous transluminal pulmonary angioplasty (PTPA) is a treatment modality for chronic thromboembolic pulmonary hypertension, but whether it can be applied to Takayasu arteritis-associated pulmonary hypertension (TA-PH), another chronic obstructive pulmonary vascular disease, remains unclear.

Objectives: This study sought to investigate the efficacy and safety of PTPA for TA-PH.

Methods: Between January 1, 2016, and December 31, 2019, a total of 50 patients with TA-PH who completed the PTPA procedure (the PTPA group) and 21 patients who refused the PTPA procedure (the non-PTPA group) were prospectively enrolled in this cohort study.

Plasma metabolomics in the perioperative period of defect repair in patients with pulmonary arterial hypertension associated with congenital heart disease.

The quality of life and survival rates of patients with pulmonary arterial hypertension associated with congenital heart disease (CHD-PAH) have been greatly improved by defect-repair surgery and personalized treatments. However, those who survive surgery may remain at risk of persistent PAH, the prognosis may be considerably worse than those unoperated. Dynamic monitoring of clinical measures during the perioperative period of shunt correction is therefore indispensable and of great value.

Subendocardial Involvement as an Underrecognized Cardiac MRI Phenotype in Myocarditis.

Background Subendocardial late gadolinium enhancement (LGE) detected with cardiac MRI in myocarditis represents a diagnostic dilemma, since it may resemble myocardial ischemia. Purpose To explore and compare the histopathologic characteristics and clinical features and outcomes in patients with myocarditis with and without subendocardial involvement at cardiac MRI. Materials and Methods This retrospective study evaluated 39 patients with myocarditis pathologically proven by means of either endomyocardial biopsy or explant pathologic findings between 2015 and 2020.

A Personalized Pulmonary Circulation Model to Non-Invasively Calculate Fractional Flow Reserve for Artery Stenosis Detection.

Objective: Fractional Flow Reserve (FFR) is regarded as a fundamental index to assess pulmonary artery stenosis. The application of FFR can increase the accuracy of detection of pulmonary artery stenosis. However, the invasive examination may carry a number of physiological risks for patients.

Clinical features and survival in Takayasu's arteritis-associated pulmonary hypertension: a nationwide study.

Aims: This study aimed to assess the clinical characteristics and long-term survival outcome in patients with Takayasu's arteritis-associated pulmonary hypertension (TA-PH).

Methods And Results: We conducted a nationally representative cohort study of TA-PH using data from the National Rare Diseases Registry System of China. Patients with pulmonary artery involvement who fulfilled the diagnostic criteria of Takayasu's arteritis and pulmonary hypertension were included.

Radiofrequency Catheter Ablation of Supraventricular Tachycardia in Patients With Pulmonary Hypertension: Feasibility and Long-Term Outcome.

Background: Supraventricular tachycardia (SVT) occurs commonly and is strongly correlated with clinical deterioration in patients with pulmonary hypertension (PH). This study aimed to investigate the feasibility and long-term outcome of radiofrequency catheter ablation (RFCA) in PH patients with SVT.

Materials And Methods: Consecutive PH patients with SVT who were scheduled to undergo electrophysiological study and RFCA between September 2010 and July 2019 were included.

Association Between Anticoagulation Outcomes and Venous Thromboembolism History in Chronic Thromboembolic Pulmonary Hypertension.

The association between anticoagulation outcomes and prior history of venous thromboembolism (VTE) in chronic thromboembolic pulmonary hypertension (CTEPH) has not been established. This study aimed to compare the efficacy and safety of anticoagulation treatment in CTEPH patients with and without prior history of VTE. A total of 333 CTEPH patients prescribed anticoagulants were retrospectively included from May 2013 to April 2019.

Rare variants in MTHFR predispose to occurrence and recurrence of pulmonary embolism.

Background: Rare genetic variants play a critical role in unprovoked pulmonary embolism (PE). However, the known risk genes only account a small proportion of patients with PE. The objective of this study was to investigate the relationship between the rare variants of gene encoding methylenetetrahydrofolate reductase (MTHFR) and the initiation and long-term clinical outcomes of PE.

Alteration of Extracellular Superoxide Dismutase in Idiopathic Pulmonary Arterial Hypertension.

Superoxide dismutases (SODs) are an important family of antioxidant enzymes that modulate reactive oxygen species levels. It is largely unknown which SOD isoform(s) change in idiopathic pulmonary arterial hypertension (IPAH) patients. A total of 133 consecutive adult IPAH patients who underwent bone morphogenetic protein receptor type 2 ( genetic counseling were enrolled in this prospective study.

Derivation of an induced pluripotent stem cell line (PUMCHi003-A) from a patient with pulmonary arterial hypertension carrying heterozygous mutation in PTGIS gene.

Pulmonary arterial hypertension (PAH) is a rare but severe illness associated with mutations in the PTGIS gene. The single nucleotide variants may lead to the impairment of the endothelial cells functions, resulting in proliferation of the smooth muscle cells and occlusion of the pulmonary arterioles. We derived an induced pluripotent cell line from a PAH patient with heterozygous PTGIS c.

Spermine promotes pulmonary vascular remodelling and its synthase is a therapeutic target for pulmonary arterial hypertension.

Pathological mechanisms of pulmonary arterial hypertension (PAH) remain largely unexplored. Effective treatment of PAH remains a challenge. The aim of this study was to discover the underlying mechanism of PAH through functional metabolomics and to help develop new strategies for prevention and treatment of PAH.

Association of Rare PTGIS Variants With Susceptibility and Pulmonary Vascular Response in Patients With Idiopathic Pulmonary Arterial Hypertension.

Importance: Idiopathic pulmonary arterial hypertension (IPAH) is a fatal disease with high heritability; however, the bone morphogenetic protein receptor 2 (BMPR2) gene only accounts for 17% of IPAH. The genetic basis of IPAH needs further investigation.

Objective: To identify novel IPAH susceptibility genes other than BMPR2.

Germline mutation causes idiopathic pulmonary arterial hypertension.

Background: Idiopathic pulmonary arterial hypertension (IPAH) is a rare disease with high heritability. Although several predisposing genes have been linked to IPAH, the genetic aetiology remains unknown for a large number of IPAH cases.

Methods: We conducted an exome-wide gene-based burden analysis on two independent case-control studies, including a total of 331 IPAH cases and 10 508 controls.