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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 -
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 -
Current Cardiology Reports Sep 2019Sympathetic overactivity plays an important role in the progression of pulmonary arterial hypertension (PAH). The purpose of this review is to illustrate localization of... (Review)
Review
PURPOSE OF REVIEW
Sympathetic overactivity plays an important role in the progression of pulmonary arterial hypertension (PAH). The purpose of this review is to illustrate localization of pulmonary arterial sympathetic nerves, the key steps of pulmonary artery denervation (PADN) procedure, and to highlight clinical outcomes.
RECENT FINDINGS
Sympathetic nerves mostly occurred in the posterior region of the bifurcation and pulmonary trunk. Emerging preclinical data provided the potential of PADN for PAH. PADN, produced at bifurcation area, improved a profound reduction of pulmonary arterial pressure and ameliorated clinical outcomes with an exclusive ablation catheter. The application of PADN in the patients of PAH or combined pre-capillary and post-capillary PH (CpcPH) improved the hemodynamic parameters and increased 6MWD. Sympathetic overactivity aggravates PAH. PADN is a promising interventional treatment for PAH and CpcPH. Additional clinical trials are warranted to confirm the efficacy of PADN.
Topics: Denervation; Hemodynamics; Humans; Hypertension, Pulmonary; Pressoreceptors; Pulmonary Arterial Hypertension; Pulmonary Artery; Sympathectomy; Sympathetic Nervous System; Treatment Outcome
PubMed: 31486924
DOI: 10.1007/s11886-019-1203-z -
Current Cardiology Reports Nov 2019Pulmonary arterial hypertension (PAH) leads to progressive increases in pulmonary vascular resistance (PVR), right heart failure, and death if left untreated. This... (Review)
Review
PURPOSE OF REVIEW
Pulmonary arterial hypertension (PAH) leads to progressive increases in pulmonary vascular resistance (PVR), right heart failure, and death if left untreated. This review will summarize and discuss recent updates in the classification and management of patients with PAH.
RECENT FINDINGS
PAH requires careful hemodynamic assessment and is defined by a mean pulmonary artery pressure > 20 mmHg with normal left-sided filling pressures and a PVR ≥ 3 Wood units. Most patients with PAH require targeted pharmacotherapy based on multiparametric risk stratification. Significant improvements in clinical outcome have been realized through the approval of 14 unique pharmacotherapeutic options. The latest clinical recommendations provide the updated hemodynamic definition and clinical classification as well as evidence-based treatment recommendations. An important change is the focus on initial upfront combination therapy for most patients with PAH. Structured follow-up and escalation of treatment for those not achieving low-risk status is paramount.
Topics: Algorithms; Biomarkers; Cardiovascular Agents; Disease Progression; Echocardiography; Heart Failure; Hemodynamics; Humans; Practice Guidelines as Topic; Pulmonary Arterial Hypertension; Pulmonary Artery; Risk Assessment
PubMed: 31758342
DOI: 10.1007/s11886-019-1235-4 -
The International Journal of... Jul 2019The purpose of this review article is to provide a brief overview of pulmonary artery aneurysms and pseudoaneurysms, to discuss the classifications of these conditions,... (Review)
Review
The purpose of this review article is to provide a brief overview of pulmonary artery aneurysms and pseudoaneurysms, to discuss the classifications of these conditions, review the role of imaging and discuss management in affected patients.
Topics: Aneurysm; Aneurysm, False; Computed Tomography Angiography; Hemodynamics; Humans; Predictive Value of Tests; Pulmonary Artery; Pulmonary Circulation; Risk Factors; Treatment Outcome
PubMed: 31190207
DOI: 10.1007/s10554-019-01547-3 -
Clinical Imaging Nov 2022A wide spectrum of pathology, both congenital and acquired, can affect the pulmonary arteries. While some of these are commonly seen in everyday clinical practice, some... (Review)
Review
A wide spectrum of pathology, both congenital and acquired, can affect the pulmonary arteries. While some of these are commonly seen in everyday clinical practice, some are rare. These entities may be discovered incidentally at imaging for other reasons in an asymptomatic patient, however patients may go on to develop symptoms over the course of their lifetime. Although an enlarged pulmonary artery can be visualized on chest X-ray (CXR), for the most part, CXR is insensitive for detecting abnormalities of the pulmonary arteries. Contrast-enhanced chest CT (CECT) is a better test to evaluate the pulmonary arteries as it is readily available, quick to perform, able to provide multiplanar reformatted images, and noninvasive. CECT is not only able to assess the lumen and wall of the pulmonary artery, but also provides a detailed evaluation of the entire thorax, including the heart, mediastinal structures, and lungs, often times picking up associated findings, and is the mainstay for evaluating disorders of the pulmonary vasculature. MRI allows for detailed evaluation of the vessel wall which can be especially helpful in cases where malignancy or vasculitis are suspected, and is also able to provide useful physiologic data such as quantification of flow. It is important for the radiologist to be aware of the many conditions which affect the pulmonary arteries, as some may require urgent treatment. This article will review normal pulmonary artery anatomy and physiology, as well as the various imaging findings of pulmonary vascular pathologies.
Topics: Humans; Magnetic Resonance Imaging; Pulmonary Artery; Thorax; Tomography, X-Ray Computed; Vasculitis
PubMed: 36067656
DOI: 10.1016/j.clinimag.2022.08.018 -
Heart & Lung : the Journal of Critical... 2019Pulmonary artery dissection (PAD) is considered to be a rare condition with a very high mortality. Since a comprehensive review on PAD has not yet been done, we analysed... (Review)
Review
Pulmonary artery dissection (PAD) is considered to be a rare condition with a very high mortality. Since a comprehensive review on PAD has not yet been done, we analysed all the available reports on PAD. In this analysis and review we searched the databases; Medline, PubMed Central, Directory of Open Access Journals, Google Scholar using the search term "Pulmonary Artery Dissection" with no language restrictions. In the 150 cases of PAD reported from 1842 to June 2018, the average age at diagnosis was 44.8 years with a male to female ratio of 1.1:1. Diagnosis was made in 49.3% of the males in the third and fourth decades, and 55.4% of the females in the fifth and sixth decades. The primary underlying causes were pulmonary hypertension and heart diseases, both congenital (mainly PDA) and acquired. The commonest clinical presentations were dyspnoea and chest pain. The best investigation of diagnosis was CT scan. The pulmonary trunk was the site of dissection in 72.5%. Surgical treatment, or medical management followed by surgery, had the best success rates. The overall survival rate which was 10.9% up to the year 2000, increased to 59.3% thereafter. If PAD was diagnosed ante-mortem, 70.5% survived. Haemopericardium / cardiac tamponade was seen at autopsy in 84.2%. PAD is not as rare, nor as fatal as believed, and with a high index of suspicion and appropriate investigations, an early diagnosis of PAD can be made and successful treatment instituted.
Topics: Aortic Dissection; Cardiac Tamponade; Dyspnea; Echocardiography; Humans; Magnetic Resonance Imaging, Cine; Pulmonary Artery; Tomography, X-Ray Computed
PubMed: 30910250
DOI: 10.1016/j.hrtlng.2019.02.007 -
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 -
Brazilian Journal of Cardiovascular... Mar 2022Patient diagnosed with common arterial trunk, submitted to pulmonary artery banding in another center and lost to clinical follow-up. Referred to our center at four...
CLINICAL DATA
Patient diagnosed with common arterial trunk, submitted to pulmonary artery banding in another center and lost to clinical follow-up. Referred to our center at four years old, extremely cyanotic. Chest radiography: Cardiomegaly; attenuated peripheral vascular markings. Electrocardiography: Right ventricular hypertrophy. Echocardiography: Common arterial trunk, but it was not possible to analyze all the structures. Computed tomography angiography: Van Praagh type A4 common arterial trunk. Extremely hypoplastic right and left pulmonary arteries.
DIAGNOSIS
Association of aortic arch interruption type A is uncommon and should be considered.
OPERATION
Debanding of pulmonary arteries allowing for possible future complete repair.
Topics: Aorta, Thoracic; Child, Preschool; Echocardiography; Heart Defects, Congenital; Humans; Pulmonary Artery; Truncus Arteriosus, Persistent
PubMed: 35072407
DOI: 10.21470/1678-9741-2021-0008 -
The Journal of Thoracic and... Aug 2022
Topics: Coronary Vessel Anomalies; Heart; Humans; Morphogenesis; Pulmonary Artery
PubMed: 34666912
DOI: 10.1016/j.jtcvs.2021.08.084