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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 -
World Journal For Pediatric &... Jan 2017We describe the diagnosis and surgical repair of a five-month-old infant with a congenital discontinuous right pulmonary artery. Initial echocardiogram failed to show...
We describe the diagnosis and surgical repair of a five-month-old infant with a congenital discontinuous right pulmonary artery. Initial echocardiogram failed to show the right pulmonary artery and revealed systemic left pulmonary artery pressure based on the tricuspid regurgitation jet. Computed tomographic angiography confirmed the diagnosis of discontinuous right pulmonary artery. The right pulmonary artery appeared essentially normal in size, and there were no significant aortopulmonary collateral arteries. Using cardiopulmonary bypass and aortic transection, we created an anastomosis between the right and the main pulmonary arteries augmented anteriorly by a pericardial patch. Postoperative lung perfusion scan demonstrated balanced pulmonary blood flow to the lungs. Pulmonary hypertension resolved over three weeks in the postoperative period, an expected outcome in this age-group.
Topics: Cardiac Surgical Procedures; Computed Tomography Angiography; Echocardiography; Humans; Infant; Male; Pulmonary Artery; Pulmonary Atresia
PubMed: 26993757
DOI: 10.1177/2150135115618871 -
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 -
Journal of Cardiovascular... Sep 2018Pulmonary artery-derived ventricular arrhythmia is gradually being recognized, which in a clinical context is recognized as an arterial ectopic beat. Our study aimed to... (Review)
Review
Pulmonary artery-derived ventricular arrhythmia is gradually being recognized, which in a clinical context is recognized as an arterial ectopic beat. Our study aimed to provide new insights on the epidemiological characteristics, origin site, electrocardiogram (ECG) characteristics, intracardiac electrophysiological characteristics and radiofrequency catheter ablation (RFCA) strategies for pulmonary artery-derived ventricular arrhythmia. Patients with a distance between the origin site and the pulmonary valve of >10 mm have what is known as pulmonary trunk-derived ventricular arrhythmia, while patients with a distance between the origin site and the pulmonary valve of ≤10 mm have what is known as pulmonary sinus cusp-derived ventricular arrhythmia. It is very difficult to differentiate pulmonary artery-derived ventricular arrhythmia from right ventricular outflow tract-derived ventricular arrhythmia on ECGs as both share similar anatomical features, but pulmonary artery-derived ventricular arrhythmia shows obvious intracardiac electrophysiological characteristics. Currently, conclusions based on the epidemiological characteristics of pulmonary artery-derived ventricular arrhythmia, relationship between the origin site and the pulmonary valve, electrophysiological characteristics, and RFCA strategies are controversial and still need further study.
Topics: Catheter Ablation; Electrocardiography; Humans; Pulmonary Artery; Ventricular Fibrillation; Ventricular Premature Complexes
PubMed: 29864191
DOI: 10.1111/jce.13652 -
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 -
VASA. Zeitschrift Fur Gefasskrankheiten Aug 2018Although pulmonary embolism is the most common abnormality of the pulmonary artery, there is a broad spectrum of other congenital and acquired pulmonary arterial... (Review)
Review
Although pulmonary embolism is the most common abnormality of the pulmonary artery, there is a broad spectrum of other congenital and acquired pulmonary arterial abnormalities. Multiple imaging modalities are now available to evaluate these abnormalities of the pulmonary arteries. CT and MRI are the most commonly used cross-sectional imaging modalities that provide comprehensive information on several aspects of these abnormalities, including morphology, function, risk-stratification and therapy-monitoring. In this article, we review the role of state-of-the-art pulmonary arterial imaging in the evaluation of non-thromboembolic disorders of pulmonary artery.
Topics: Computed Tomography Angiography; Echocardiography; Humans; Magnetic Resonance Angiography; Predictive Value of Tests; Prognosis; Pulmonary Artery; Pulmonary Circulation; Tomography, Emission-Computed; Vascular Diseases; Vascular Malformations
PubMed: 29806799
DOI: 10.1024/0301-1526/a000709 -
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 -
Journal of Thoracic Imaging Nov 2016Coronary-pulmonary arterial fistulas (CPAFs) are rare coronary artery anomalies that have been described only in limited case reports. This study aims to evaluate the... (Review)
Review
PURPOSE
Coronary-pulmonary arterial fistulas (CPAFs) are rare coronary artery anomalies that have been described only in limited case reports. This study aims to evaluate the clinical presentation and imaging findings of CPAFs collected from 6 participating medical centers along with CPAFs reported in the literature, to discern any general trends present in CPAFs.
MATERIALS AND METHODS
A total of 25 cases of CPAF diagnosed by coronary computed tomography angiography were collected across 6 participating institutions. In addition, utilizing a PubMed literature search, 78 additional CPAF cases were obtained. The imaging findings and relevant clinical history were reviewed.
RESULTS
Of the 103 CPAF patients, 60 (63% of patients with sex known) were male, with ages ranging from newborn to 88 years (mean=46.1 y). The most common symptoms reported were chest pain (n=40, 39%) and dyspnea (n=26, 25%), with a murmur as the most common physical examination finding (n=38, 37%). The most common coronary artery of origin for a CPAF was the left main/left anterior descending (n=87, 84%), followed by the right coronary artery (n=39, 38%). The fistula most commonly terminated in the main pulmonary artery (n=92, 89%). Multiple CPAFs were present in 46 cases (45%). Coronary artery aneurysms were identified in 20 cases (19%). Pediatric CPAF cases were usually associated with pulmonary atresia with ventricular septal defect.
CONCLUSIONS
CPAFs are seen in a variety of clinical settings, from infants with advanced congenital heart disease to elderly patients who have undergone revascularization surgery. Although coronary artery fistulas have previously been described as rarely involving multiple coronary arteries, with the right coronary artery being most often involved, our series demonstrates that multiple fistulas are commonly present, with the most common pattern being between the left main/left anterior descending and the main pulmonary trunk.
Topics: Arterio-Arterial Fistula; Computed Tomography Angiography; Coronary Angiography; Coronary Vessel Anomalies; Coronary Vessels; Humans; Pulmonary Artery
PubMed: 27768631
DOI: 10.1097/RTI.0000000000000232