Branch Pulmonary Artery Stenosis

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: 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: The optimal conduit for right ventricle to pulmonary artery (RV-PA) reconstruction does not exist. Reinterventions are common due to conduit stenosis and endocarditis. Tailoring conduit choice according to patients' characteristics could improve the outcomes. The study aimed to compare graft-related events (infective endocarditis, transcatheter pulmonary valve replacement (PVR), transcatheter conduit dilatation, surgical conduit replacement, and transcatheter pulmonary branch intervention for RV-PA reconstruction using bovine jugular vein, aortic homograft, and porcine-valved conduits...

BACKGROUND: Brachycephalic dogs with pulmonary stenosis are known to have a higher incidence of concurrent coronary artery abnormalities than non-brachycephalic breeds, which increases risk when performing balloon valvuloplasty. The use of ECG-gated CT angiography has been reported for the evaluation of coronary arteries in normal dogs and dogs with pulmonary stenosis. The purpose of this study was to report findings of coronary artery origination and morphology of main branches using ECG-gated CT angiography in brachycephalic dogs with pulmonary stenosis...

Takayasu arteritis (TA) is a rare systemic vasculitis of the aorta and its primary branches, which usually occurs in young women. Due to its insidious onset and lack of specific symptoms, this disease can be easily misdiagnosed or missed. Approximately 50 ​% of the patients having TA with pulmonary artery involvement develop pulmonary hypertension (PH). The 3-year survival rate among patients with TA-related PH is lower than that among patients with TA alone. Early balloon pulmonary angioplasty (BPA) can improve the clinical symptoms and survival of patients with stable TA...

OBJECTIVES: Left pulmonary artery (LPA) or bifurcation stenoses at Fontan palliation can be very challenging to treat and may also require cardioplegia and aortic transection. Moreover, the low pressure of Fontan circulation and the bulkiness of the aorta increase the risk of a patch angioplasty collapse. Pre-Fontan LPA stenting of stenotic LPAs overcomes those drawbacks therefore the present study aimed to evaluate its advantageous impact on Fontan surgery. METHODS: A multicentre retrospective analysis was performed on 304 consecutive Fontan patients...

BACKGROUND: Branch pulmonary artery stenosis is common after surgical repair in patients with biventricular CHD and often requires reinterventions. However, (long-term) effects of percutaneous branch pulmonary artery interventions on exercise capacity, right ventricular function, and lung perfusion remain unclear. This review describes the (long-term) effects of percutaneous branch pulmonary artery interventions on exercise capacity, right ventricular function, and lung perfusion following PRISMA guidelines...

Tetralogy of Fallot with pulmonary stenosis has a diverse clinical spectrum with the degree of right ventricular outflow tract obstruction (RVOTO) and size of the branch pulmonary arteries driving clinical management. Optimal surgical management involves consideration of patient clinical status and degree and location (subvalvar, valvar, and supravalvar) of RVOTO. Timing of repair requires multidisciplinary decision-making and complete surgical repair with relief of RVOTO by either transannular patch or valve sparing repair techniques...

BACKGROUND: Treatment of pulmonary artery (PA) stenosis in congenital heart disease is associated with adverse outcomes. The aim of this retrospective cohort study was to compare outcomes after surgical patch augmentation of PA stenosis in patients with biventricular congenital heart disease using different patch materials. METHODS: We identified all patients from our institutional congenital heart disease database who underwent patch augmentation for PA stenosis on the main pulmonary artery (MPA) or PA branches between 2012 and 2018...

BACKGROUND: SwiftNINJA (Merit Medical Systems, USA) is a novel steerable microcatheter intended for coronary and peripheral vascular interventions. We evaluate and report the first use of SwiftNINJA in pediatric catheterization of congenital heart defects (CHDs). METHODS: We performed a retrospective clinical data review of children with CHDs in whom SwiftNINJA was used during cardiac catheterization between April 2022 and June 2023. Utility, application, and standard safety were described comprehensively...

BACKGROUND: Hybrid aortic arch repair with debranching of the supra-aortic vessels carries a high risk of cerebral infarction and fatal complications associated with side clamping of the ascending aorta. A previous study had reported the "real chimney" technique, a novel method for clamp-free total debranching thoracic endovascular aortic repair with the ascending aortic sleeve banding with dacron. In this study, we aim to build upon this foundation by presenting our experience with the early and midterm outcomes of this technique...

Congenital severe peripheral pulmonary artery stenosis is usually associated with pulmonary atresia, ventricular septal defect and major aortopulmonary collateral artery. Complications mainly related to respiratory system frequently occur after peripheral pulmonary artery patch augmentation with or without uniforcalization. Pulmonary hemorrhage and airway bleeding can be fatal. Airway compression from surrounding structures such as reconstructed central pulmonary artery, pulmonary hypertension, ventilation/perfusion mismatch, and pulmonary ischemia reperfusion injury are also life-threating complications...

PURPOSE: To investigate the imaging characteristics and prognostic factors for the long-term survival of Behcet's disease (BD) with arterial involvement. METHODS: In this retrospective study, BD patients with arterial involvement were identified from January 2003 to January 2020. Arterial lesions were detected by ultrasonography, traditional arteriography, and/or computed tomography angiography (CTA). Cox proportional hazards regression analyses were performed to identify the prognostic factors...

BACKGROUND: Elastin-driven genetic diseases are a group of complex diseases driven by elastin protein insufficiency and dominant-negative production of aberrant protein, including supravalvular aortic stenosis (SVAS) and autosomal dominant cutis laxa. Here, a Chinese boy with a novel nonsense mutation in the ELN gene is reported. CASE PRESENTATION: We report a 1-year-old boy who presented with exercise intolerance, weight growth restriction with age, a 1-year history of heart murmur, and inguinal hernia...

Absent pulmonary valve with tricuspid atresia or tricuspid stenosis (APV-TA/TS) is an extremely rare congenital heart defect associated with significant morbidity and mortality. Compared to Tetralogy of Fallot with Absent Pulmonary Valve Syndrome, branch pulmonary arteries are not typically significantly dilated. We present the case of a newborn male prenatally diagnosed APV-TA with intact ventricular septum (IVS) and nearly discontinuous branch pulmonary arteries, the surgical strategy employed, and the salient hemodynamic factors considered in the medical decision-making...

To assess the feasibility and outcome of stent strut dilation after arterial duct stenting with associated branch pulmonary artery (BPA) stenosis. Stenting of arterial duct in infants with duct-dependent pulmonary circulation is technically challenging. The presence of BPA stenosis is a relative contraindication for stent implantation. Infants with duct-dependent pulmonary circulation and associated BPA stenosis were assessed either by transthoracic echocardiogram alone or additional computerized tomography angiogram when required...

A 9-month-old intact male Ragdoll cat was presented for evaluation of a left-sided systolic murmur that was first auscultated during examination for a newly developed cough. Transthoracic echocardiography revealed a narrowed left pulmonary artery and an increase in flow velocities at the level of the narrowing, consistent with left pulmonary artery branch stenosis. The right pulmonary artery appeared normal. Balloon angioplasty was performed and successfully reduced pressure gradient across the stenosis. The patient continues to do well 14 months after the procedure...

BACKGROUND: Takayasu arteritis (TAK) is a chronic inflammatory arteritis. It most often affects non-specific large vessel progressively, such as the aorta and its branches. The diagnosis in TAK is typically delayed. Isolated pulmonary artery involvement in Takayasu arteritis is uncommon. Owing to its rarity, the diagnosis is challenging and requires an integrated approach comprising clinical and imaging findings. In order to facilitate early diagnosis of TAK for clinicians, wider use of non-invasive imaging is impacting this...

Takayasu's arteritis is a chronic large vessel vasculitis that mainly affects the aorta and its major branches such as brachiocephalic, carotid, subclavian, vertebral, renal, coronary and pulmonary arteries. It most commonly occurs in female at child bearing age and female to male ratio is 8:2. Initial presentations of Takayasu's arteritis may be nonspecific like fever, malaise, weakness, fatigue, arthralgia, myalgia and weight loss but in advanced stage there may be features of vascular inflammation like segmental stenosis, occlusion, dilatation and/or aneurysm...

Alagille syndrome (ALGS) is a multisystem disease with high variability in clinical features. ALGS is predominantly caused by pathogenic variants in the Notch ligand JAG1. An iPSC line, NCHi011-A, was generated from a ALGS patient with complex cardiac phenotypes consisting of pulmonic valve and branch pulmonary artery stenosis. NCHi011-A is heterozygous for a single base duplication causing a frameshift in the JAG1 gene. This iPSC line demonstrates normal cellular morphology, expression of pluripotency markers, trilineage differentiation potential, and identity to the source patient...