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The Journal of Thoracic and... Apr 2022
Topics: Cardiac Catheterization; Extracorporeal Membrane Oxygenation; Humans; Postoperative Care; Pulmonary Artery; Stenosis, Pulmonary Artery; Sternotomy; Thoracotomy
PubMed: 34649719
DOI: 10.1016/j.jtcvs.2021.07.058 -
The European Respiratory Journal Dec 2023Peripheral pulmonary artery stenosis (PPS) refers to stenosis of the pulmonary artery from the trunk to the peripheral arteries. Although paediatric PPS is well...
BACKGROUND
Peripheral pulmonary artery stenosis (PPS) refers to stenosis of the pulmonary artery from the trunk to the peripheral arteries. Although paediatric PPS is well described, the clinical characteristics of adult-onset idiopathic PPS have not been established. Our objectives in this study were to characterise the disease profile of adult-onset PPS.
METHODS
We collected data in Japanese centres. This cohort included patients who underwent pulmonary angiography (PAG) and excluded patients with chronic thromboembolic pulmonary hypertension or Takayasu arteritis. Patient backgrounds, right heart catheterisation (RHC) findings, imaging findings and treatment profiles were collected.
RESULTS
44 patients (median (interquartile range) age 39 (29-57) years; 29 females (65.9%)) with PPS were enrolled from 20 centres. In PAG, stenosis of segmental and peripheral pulmonary arteries was observed in 41 (93.2%) and 36 patients (81.8%), respectively. 35 patients (79.5%) received medications approved for pulmonary arterial hypertension (PAH) and 22 patients (50.0%) received combination therapy. 25 patients (56.8%) underwent transcatheter pulmonary angioplasty. RHC data showed improvements in both mean pulmonary arterial pressure (44 40 mmHg; p<0.001) and pulmonary vascular resistance (760 514 dyn·s·cm; p<0.001) from baseline to final follow-up. The 3-, 5- and 10-year survival rates of patients with PPS were 97.5% (95% CI 83.5-99.6%), 89.0% (95% CI 68.9-96.4%) and 67.0% (95% CI 41.4-83.3%), respectively.
CONCLUSIONS
In this study, patients with adult-onset idiopathic PPS presented with segmental and peripheral pulmonary artery stenosis. Although patients had severe pulmonary hypertension at baseline, they showed a favourable treatment response to PAH drugs combined with transcatheter pulmonary angioplasty.
Topics: Adult; Female; Humans; Child; Stenosis, Pulmonary Artery; Hypertension, Pulmonary; Constriction, Pathologic; Pulmonary Artery; Familial Primary Pulmonary Hypertension; Pulmonary Arterial Hypertension
PubMed: 38061784
DOI: 10.1183/13993003.00763-2023 -
JACC. Cardiovascular Interventions Dec 2022
Topics: Humans; Pulmonary Arterial Hypertension; Pulmonary Artery; Treatment Outcome
PubMed: 36480985
DOI: 10.1016/j.jcin.2022.10.004 -
Scientific Reports May 2021For CT pulmonary angiograms, a scout view obtained in anterior-posterior projection is usually used for planning. For bolus tracking the radiographer manually locates a...
For CT pulmonary angiograms, a scout view obtained in anterior-posterior projection is usually used for planning. For bolus tracking the radiographer manually locates a position in the CT scout view where the pulmonary trunk will be visible in an axial CT pre-scan. We automate the task of localizing the pulmonary trunk in CT scout views by deep learning methods. In 620 eligible CT scout views of 563 patients between March 2003 and February 2020 the region of the pulmonary trunk as well as an optimal slice ("reference standard") for bolus tracking, in which the pulmonary trunk was clearly visible, was annotated and used to train a U-Net predicting the region of the pulmonary trunk in the CT scout view. The networks' performance was subsequently evaluated on 239 CT scout views from 213 patients and was compared with the annotations of three radiographers. The network was able to localize the region of the pulmonary trunk with high accuracy, yielding an accuracy of 97.5% of localizing a slice in the region of the pulmonary trunk on the validation cohort. On average, the selected position had a distance of 5.3 mm from the reference standard. Compared to radiographers, using a non-inferiority test (one-sided, paired Wilcoxon rank-sum test) the network performed as well as each radiographer (P < 0.001 in all cases). Automated localization of the region of the pulmonary trunk in CT scout views is possible with high accuracy and is non-inferior to three radiographers.
Topics: Adult; Aged; Aged, 80 and over; Angiography; Deep Learning; Female; Humans; Image Processing, Computer-Assisted; Male; Middle Aged; Phantoms, Imaging; Pulmonary Artery; Radiation Dosage; Retrospective Studies; Tomography, X-Ray Computed
PubMed: 33986402
DOI: 10.1038/s41598-021-89647-w -
Nagoya Journal of Medical Science Feb 2019Pulmonary hypertension (PH) is a hemodynamic state that is characterized by a resting mean pulmonary artery pressure ≧ 25 mmHg. The common forms of PH are pulmonary... (Review)
Review
Pulmonary hypertension (PH) is a hemodynamic state that is characterized by a resting mean pulmonary artery pressure ≧ 25 mmHg. The common forms of PH are pulmonary arterial hypertension (PAH), chronic thromboembolic pulmonary hypertension (CTEPH), PH caused by left-heart disease, and PH due to lung disease. Previously regarded as untreatable, the treatment of PAH has dramatically advanced since the introduction of the drug epoprostenol in 1999, with three-year survival rates improving from 30%-40% to over 85%. Drugs available for the specific treatment of PAH include endothelin-receptor antagonists, phosphodiesterase type 5 inhibitors, soluble guanylate cyclase stimulators, prostacyclin analogs, and prostacyclin-receptor agonists. In the past decade, management and treatment of CTEPH have also improved. While pulmonary endarterectomy used to be the only option for the treatment of CTEPH, newer treatments include a soluble guanylate cyclase stimulator, which has proven to be an efficacious targeted therapy. Other cases benefit from balloon pulmonary angioplasty.
Topics: Animals; Endarterectomy; Endothelin Receptor Antagonists; Humans; Hypertension, Pulmonary; Pulmonary Artery; Pulmonary Embolism
PubMed: 30962652
DOI: 10.18999/nagjms.81.1.19 -
Biomedicine & Pharmacotherapy =... Sep 2020Pulmonary arterial hypertension (PAH) is a progressive disease with limited effective therapies that can be lethal in the terminal stage. Since the universal use of... (Review)
Review
Pulmonary arterial hypertension (PAH) is a progressive disease with limited effective therapies that can be lethal in the terminal stage. Since the universal use of traditional PAH-specific drugs affecting the nitric oxide pathway, endothelin pathway and prostacyclin pathway, major advances to treat this devastating condition have been made. For example, it has been recently demonstrated that PAH is associated with various dysfunctional genes and molecular signaling pathways, abnormal metabolism and endocrine systems, inflammation and immune dysfunction. Numerous descriptions of novel mechanisms in PAH have been presented, and some new drugs targeted to ease, reverse or cure PAH have been developed at the laboratory-level and/or tested in clinical trials. There are also some investigated drugs that have shown promising efficacy and safety in animal models but have failed in human beings. Inspiringly, immunotherapies also seem to be valuable therapies for PAH patients. In this review, we update the advances in investigational pharmacotherapy and immunotherapy of PAH. Hopefully this work can help promote more non-invasive treatment for PAH patients.
Topics: Animals; Antihypertensive Agents; Arterial Pressure; Humans; Immunosuppressive Agents; Immunotherapy; Molecular Targeted Therapy; Pulmonary Arterial Hypertension; Pulmonary Artery; Signal Transduction; Vaccines
PubMed: 32559622
DOI: 10.1016/j.biopha.2020.110355 -
Anatomical Record (Hoboken, N.J. : 2007) Feb 2023Ductus arteriosus is a muscular artery in fetal circulation, spanning from the bifurcation of the pulmonary trunk to the aortic arch, shunting blood directly from...
Ductus arteriosus is a muscular artery in fetal circulation, spanning from the bifurcation of the pulmonary trunk to the aortic arch, shunting blood directly from pulmonary circulation into systemic circulation thus by-passing the fluid-filled lungs. Postnatally, it changes name to the ligamentum arteriosum (LA), when a cascade of anatomical and physiological processes leads to its closure. Though the LA has generally been considered as a fibrosed remnant of the ductus arteriosus, anecdotal and contradictory reports still describe the LA as a small muscular artery. We hypothesized the likelihood of contractile muscular elements retainment in this so-called ligament. To investigate this, mediastinum of wild-type mouse, pig, and human LA were subjected to routine and special histological staining, single-immunolabeling, electron microscopy (mouse and pig only), and tension recording of explanted pig LA in organ bath experiments. Contrary to a canonical ligament, the LA was mainly made up of α-smooth muscle actin-positive cells in all three species, confirmed by routine and special histological staining as well as transmission electron microscopy. Myocytes within the LA contracted in response to exogenous noradrenalin (NA). NA-induced precontracted LA relaxed upon administration of the α1-adrenergic blockers (prazosin and tamsulosin). Though the LA does not function in its original capacity as fetal shunt, it is clearly not a passive structure, and may be described as muscular and contractile. The contractile abilities of LA myocytes may act on the two great vessels to which it is attached causing a change in their distensibility.
Topics: Animals; Mice; Humans; Swine; Aorta, Thoracic; Ductus Arteriosus, Patent; Ductus Arteriosus; Pulmonary Artery; Ligaments
PubMed: 36054486
DOI: 10.1002/ar.25058 -
EuroIntervention : Journal of EuroPCR... May 2013Pulmonary arterial hypertension (PAH) is a group of diseases related to progressively increasing pulmonary vascular resistance, a high incidence of right ventricular... (Review)
Review
Pulmonary arterial hypertension (PAH) is a group of diseases related to progressively increasing pulmonary vascular resistance, a high incidence of right ventricular failure and premature death. Only a limited number of pharmaceutical therapies have proven to be beneficial for PAH. These therapies improve symptoms, exercise capacity, and haemodynamics; however, the clinical relevance of these effects has been challenged. Therefore, the effect of currently approved treatment options remains inconclusive. Conversely, several new drugs for various aetiologies and clinical stages are expected to provide significant advances for the treatment of PAH. Moreover, percutaneous pulmonary artery denervation treatment may lead to a new therapeutic orientation in patients with PAH. The aim of this review is to present the new developments in PAH treatment, provide a brief overview of future directions in the field and discuss the potential future prospects of these innovative therapies.
Topics: Animals; Arterial Pressure; Autonomic Denervation; Catheter Ablation; Familial Primary Pulmonary Hypertension; Humans; Hypertension, Pulmonary; Hypoglycemic Agents; Pulmonary Artery; Treatment Outcome
PubMed: 23732148
DOI: 10.4244/EIJV9SRA25 -
Archives of Cardiovascular Diseases 2013Unilateral absence of pulmonary artery (UAPA) is a rare malformation that can present as an isolated lesion or may be associated with other congenital heart defects.... (Review)
Review
Unilateral absence of pulmonary artery (UAPA) is a rare malformation that can present as an isolated lesion or may be associated with other congenital heart defects. UAPA is often associated with other congenital cardiovascular anomalies, such as tetralogy of Fallot, atrial septal defect, coarctation of aorta, right aortic arch, truncus arteriosus and pulmonary atresia. Diagnosis of UAPA is very difficult and is based on taking a complete medical history, physical examination and imaging examinations. Clinical symptoms include exercise intolerance, haemoptysis and recurrent respiratory infections. Adult patients with UAPA are often asymptomatic. There is no consensus regarding the treatment for UAPA. The therapeutic approach should be based on symptoms of the patient, pulmonary artery anatomy and associated aortopulmonary collaterals. Treatment options for these patients include partial or total pneumonectomy, closure of selected collateral arteries not solely responsible for pulmonary blood flow or a primary versus staged pulmonary artery anastomosis. This review summarizes pathophysiology, symptomatology and current diagnosis and treatment of this disease.
Topics: Diagnostic Imaging; Heart Defects, Congenital; Humans; Physical Examination; Pneumonectomy; Predictive Value of Tests; Pulmonary Artery; Treatment Outcome; Vascular Surgical Procedures
PubMed: 23938302
DOI: 10.1016/j.acvd.2013.05.004 -
PloS One 2020In congenital heart malformations with pulmonary stenosis to atresia an abnormal lateral ductus arteriosus to left pulmonary artery connection can lead to a localised...
OBJECTIVES
In congenital heart malformations with pulmonary stenosis to atresia an abnormal lateral ductus arteriosus to left pulmonary artery connection can lead to a localised narrowing (pulmonary ductal coarctation) or even interruption We investigated embryonic remodelling and pathogenesis of this area.
MATERIAL AND METHODS
Normal development was studied in WntCre reporter mice (E10.0-12.5) for neural crest cells and Nkx2.5 immunostaining for second heart field cells. Data were compared to stage matched human embryos and a VEGF120/120 mutant mouse strain developing pulmonary atresia.
RESULTS
Normal mouse and human embryos showed that the mid-pharyngeal endothelial plexus, connected side-ways to the 6th pharyngeal arch artery. The ventral segment formed the proximal pulmonary artery. The dorsal segment (future DA) was solely surrounded by neural crest cells. The ventral segment had a dual outer lining with neural crest and second heart field cells, while the distal pulmonary artery was covered by none of these cells. The asymmetric contribution of second heart field to the future pulmonary trunk on the left side of the aortic sac (so-called pulmonary push) was evident. The ventral segment became incorporated into the pulmonary trunk leading to a separate connection of the left and right pulmonary arteries. The VEGF120/120 embryos showed a stunted pulmonary push and a variety of vascular anomalies.
SUMMARY
Side-way connection of the DA to the left pulmonary artery is a congenital anomaly. The primary problem is a stunted development of the pulmonary push leading to pulmonary stenosis/atresia and a subsequent lack of proper incorporation of the ventral segment into the aortic sac. Clinically, the aberrant smooth muscle tissue of the ductus arteriosus should be addressed to prohibit development of severe pulmonary ductal coarctation or even interruption of the left pulmonary artery.
Topics: Animals; Aorta; Ductus Arteriosus; Homeobox Protein Nkx-2.5; Humans; Mice; Mice, Inbred C57BL; Neural Crest; Pulmonary Artery; Pulmonary Atresia; Vascular Endothelial Growth Factor A
PubMed: 32413023
DOI: 10.1371/journal.pone.0228478