pulmonary artery stenosis

In systemic lupus erythematosus (SLE), cardiovascular complications are among the leading causes of death. Cardiovascular risk in SLE is even higher in the presence of antiphospholipid antibodies or secondary antiphospholipid syndrome (APS). The aim of this retrospective, single-center study was to investigate the occurrence of antiphospholipid antibodies and non-thrombotic cardiac manifestations in 369 SLE patients. We also assessed the clinical and laboratory characteristics of the patients to reveal the risk factors for cardiac manifestations...

OBJECTIVES: Preoperative intravenous epoprostenol therapy can cause thrombocytopaenia, which may increase the risk of perioperative bleeding during lung transplantation. This study aimed to determine whether lung transplantation can be safely performed in patients with epoprostenol-induced thrombocytopaenia. METHODS: From June 2008 to July 2022, we performed 37 lung transplants in patients with pulmonary arterial hypertension (PAH), including idiopathic PAH (n = 26), congenital heart disease-associated PAH (n = 7), pulmonary veno-occlusive disease (n = 3), and peripheral pulmonary artery stenosis (n = 1) at our institution...

We report the case of a 9-year-old male with severe congenital pulmonary valve stenosis referred to our centre for percutaneous valvotomy. On admission, trans-thoracic echocardiogram confirmed a unicuspid pulmonary valve with a peak/mean pulmonary valve gradient of 91/53 mmHg and a pulmonary annulus of 13.8 mm (-0.8 Z Score). It also showed an enlarged RV (RV/LV ratio 0,9). During cardiac catheterisation, an additional atrial septal defect (secundum) with significant left to right shunt (Qp/Qs > 2) was diagnosed, which was not amenable to percutaneous closure...

BACKGROUND: It is common for clinicians to use the pulmonary artery diastolic pressure (PADP) as a surrogate for the pulmonary capillary wedge pressure (PCWP). Here, we determine the validity of this relationship in patients with various phenotypes of cardiogenic shock (CS). METHODS AND RESULTS: In this analysis of the Critical Care Cardiology Trials Network registry, we identified 1,225 admissions with CS that received pulmonary artery catheters. Linear regression, Bland-Altman, and receiver operator characteristic analyses were performed to determine the strength of association between PADP and PCWP in patients with left-, right-, bi-ventricular, and other non-myocardial phenotypes of CS (e...

Congenitally corrected transposition of the great arteries (ccTGA) is a rare malformation with diverse morphology. We assessed features of fetuses with ccTGA and evaluated neonatal and pediatric outcomes. This was a retrospective review of fetuses with ccTGA at Birmingham Women's and Children's Hospital born from 2005 to 2019. Of thirty-six fetuses identified, six had unavailable prenatal data, one was postnatally diagnosed with isomerism and 29 fetuses were evaluated. ccTGA without associated cardiac lesions was found in 28% (8/29), ccTGA with significant VSD in 31% (9/29), ccTGA with pulmonary obstruction in 24% (7/29) and ccTGA with complex anomalies in 17% (5/29)...

Alagille syndrome is an autosomal-dominantinherited disease characterized by intrahepatic bile duct involvement, congenital heart disease, eye anomalies, skeletal and central nervous system involvement, kidney anomalies, and facial appearance. Liver transplant is the only treatment option for patients with end-stage liver disease and Alagille syndrome. Bilateral peripheral pulmonary artery stenosis is a contraindication for liver transplant due to high mortality, and the decision for liver transplant in patients with bilateral peripheral pulmonary artery stenosis is extremely challenging for anesthesiologists andtransplant surgeons...

Structural variants (SVs) pose a challenge to detect and interpret, but their study provides novel biological insights and molecular diagnosis underlying rare diseases. The aim of this study was to resolve a 9p24 rearrangement segregating in a family through five generations with a congenital heart defect (congenital pulmonary and aortic valvular stenosis and pulmonary artery stenosis), by applying a combined genomic analysis. The analysis involved multiple techniques, including karyotype, chromosomal microarray analysis (CMA), FISH, genome sequencing (GS), RNA-seq, and optical genome mapping (OGM)...

Cardiovascular risk factors are the cause of atherosclerotic disease, which can involve all the elastic and muscolo-elastic arteries. The etiopathogenesis of atherosclerosis is multifactorial since genetics, lifestyle, and comorbidities can be simultaneously involved. Clinical manifestations can be heterogeneous and include myocardial infarction, stroke, aortic aneurysms, renal artery stenosis, renal insufficiency, peripheral artery disease, etc. Currently, 70% of clinical events cannot be prevented with available drug therapy, statins included, and at least 10% of coronary events occur in apparently healthy individuals in the absence of major traditional risk factors...

The unguarded tricuspid valve is a rare and severe condition. When found in the fetus, they mostly undergo abortion or intrauterine death. The details of the fetal course in such cases are poorly understood. Here, we report a case of an unguarded tricuspid valve detected at 20 weeks of gestation who developed a complete atrioventricular block and survived in utero. The fetus also had pulmonary atresia with intact ventricular septum, Uhl's disease, hypoplastic right ventricle, noncompacted left ventricle, valvular aortic stenosis, and right coronary artery fistula to the right ventricle...

BACKGROUND: Breathlessness is common in the population and can be related to a range of medical conditions. We aimed to evaluate the burden of breathlessness related to different medical conditions in a middle-aged population. METHODS: Cross-sectional analysis of the population-based Swedish CArdioPulmonary bioImage Study of adults aged 50-64 years. Breathlessness (modified Medical Research Council [mMRC] ≥ 2) was evaluated in relation to self-reported symptoms, stress, depression; physician-diagnosed conditions; measured body mass index (BMI), spirometry, venous haemoglobin concentration, coronary artery calcification and stenosis [computer tomography (CT) angiography], and pulmonary emphysema (high-resolution CT)...

Pulmonary hypertension is a condition characterised by elevated pulmonary arterial pressures secondary to various aetiologies; the most common ones are left heart diseases. Similarly, an association between thyroid diseases and pulmonary hypertension has been reported in some cases, but the pathophysiological relationship has not been fully elucidated. Etiological investigation is an important step in the management of pulmonary hypertension and determines the appropriate treatment. In this report, we present a case of severe pulmonary hypertension in a 57-year-old woman, in which mixed aortic valve disease and hypothyroidism were involved...

KEY CLINICAL MESSAGE: Before valvular interventions, echocardiography, especially the TEE or the ventilation/perfusion scan, should be performed to detect silent PTE and set a more accurate treatment and surgical plan. ABSTRACT: Pulmonary hypertension (PH) is a progressive and critical disease that can be caused by mitral stenosis (MS). Some of these patients present with disproportionate PH, which is an uncommon phenomenon and is considered a challenging diagnostic and treatment process...

BACKGROUND: Noonan syndrome (NS) is relatively common but poorly recognized. We aimed to describe the phenotypic and genotypic spectrum of NS in a Chinese cohort. METHOD: The study retrospectively investigated consecutive pediatric patients who presented at the Guangdong cardiovascular institute between 2018 and 2020 with confirmed known NS-relevant mutations determined by exome sequencing. Dates of genetic testing, Age, sex, institution of genetic testing, mutated gene (related to NS) and its classification, heterozygosity, and parental origin were identified from the sequencing reports...

BACKGROUND: Hybrid interventions have become a common option in the management for a variety of patients with congenital heart disease. In this review we will discuss the data that has driven decision making about hybrid interventions to date. METHODS: The existing literature on various hybrid approaches was reviewed and summarized. In addition, the key tenants to creating a successful hybrid program within a congenital heart center are elucidated. RESULTS: Hybrid strategies for single ventricle patients, pulmonary atresia with intact ventricular septum, branch pulmonary artery stenosis, and muscular ventricular septal defect closure have important benefits and limitations compared to traditional approaches...

BACKGROUND: Pulmonary artery stenosis endangers people's health. Quantitative pulmonary pressure ratio (QPPR) is very important for clinicians to quickly diagnose diseases and develop treatment plans. OBJECTIVE: Our purpose of this paper is to investigate the effects of different degrees (50% and 80%) of pulmonary artery stenosis on QPPR. METHODS: An idealized model is established based on the normal size of human pulmonary artery. The hemodynamic governing equations are solved using fluid-structure interaction...

Background: Excellent outcomes of right ventricle to pulmonary artery conduits with polytetrafluoroethylene (PTFE) valves have been reported. The purpose of this study was to analyze the short-term results of our handmade PTFE bicuspid valved conduit (VC) for right ventricular outflow tract reconstruction. Methods: Between September 2019 and May 2023, bicuspid PTFE-VC was implanted in 17 patients at a median age of 2.5 years (range, 3 months to 13.6 years). The PTFE-VC was fashioned from a commercially available PTFE tube graft (14 mm in three patients, 16 mm in three patients, 18 mm in one patient, 20 mm in three patients, and 22 mm in seven patients) and 0...

Chronic thromboembolic pulmonary hypertension (CTEPH) is a pulmonary vascular disease characterized by an insidious onset, progressive deterioration, and poor prognosis. It is distinguished by the thrombotic organization within the pulmonary arteries, leading to vascular stenosis or occlusion. This results in a progressive increase in pulmonary vascular resistance and pulmonary arterial pressure, ultimately leading to right heart failure. In recent years, balloon pulmonary angioplasty (BPA) has emerged as an effective treatment option for patients ineligible for pulmonary endarterectomy (PEA)...

Objective: To summarize and analyze the clinical features, treatment, and prognosis of pulmonary artery stenosis post-lung transplantation. Methods: A 62-year-old male patient was admitted to the hospital with a cough and chest tightness of over a year's duration, which had worsened in the last two months, leading to the diagnosis of idiopathic pulmonary fibrosis. The clinical data were observed and reviewed post-left allograft single lung transplantation. Literature searches were conducted using the keywords "lung transplantation" "stenosis, pulmonary artery" and "postoperative complications" in CNKI, Wanfang Medical Network, and PubMed databases up to December 2022...

OBJECTIVES: We aimed to review the outcomes of treating incidentally encountered asymptomatic airway stenosis during open-heart surgery conservatively without the use of tracheoplasty. METHODS: Between January 2002 and October 2022, 25 patients were incidentally diagnosed with tracheal stenosis during open-heart surgery. Intraoperative bronchoscopy and/or laryngoscopy revealed tracheal stenosis; however, this was not consistent with the findings of the preoperative computed tomography...

Currently, there is no specific medication approved for the treatment of valvular heart disease per se. Except for secondary mitral valve insufficiency and tricuspid valve insufficiency in pulmonary hypertension, drug therapy for higher-grade valvular heart disease is limited to diuretic therapy for symptom control. Conservative therapy for comorbidities and potential heart failure can be beneficial regardless of the specific valve lesion. In cases of aortic valve stenosis or insufficiency, controlling arterial hypertension is important...