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, 8. Pulmonary hypertension. (7) CT scan obtained with a lung window setting at the level of the upper lobes in a 75-year-old man shows marked enlargement of the pulmonary arteries (arrowheads) in relation to the bronchi . (8) CT scan obtained with a lung window setting in a 65-year-old woman shows a mosaic perfusion pattern, with increased diameters of vessels in areas of hyperattenuation (arrows) and sharp tapering of peripheral vessels in areas of hypoattenuation (arrowheads).
Source publication
Various congenital and acquired anomalies may affect the pulmonary arteries in adult patients. Congenital anomalies (proximal interruption, anomalous origin of the left pulmonary artery [pulmonary artery sling], and idiopathic dilatation of the pulmonary trunk) are usually found incidentally at chest radiography or computed tomography (CT). Acquire...
Contexts in source publication
Context 1
... patients affected by pulmonary hyperten- sion, the caliber of the most peripheral vessels is abruptly diminished because of vasoconstriction. This feature is not always visible at CT, although it is sometimes observed indirectly as a mosaic perfusion pattern on thin-section CT images (25) (Fig 8). ...
Context 2
... pattern of attenuation-patchy areas in which attenuation is either increased or decreased-may be seen in the lung parenchyma on thin-section CT scans when vascular disease, infiltrative lung disease, or airways disease is present (28). Areas of increased lung attenuation may be attributable to blood flow redistribution, if the size and number of ves- sels in those areas are increased in comparison with those in areas of normal or decreased attenu- ation (Fig 8) and if there is no air trapping (28). Features that help to distinguish mosaic attenua- tion due to vascular abnormalities from mosaic attenuation caused by airway anomalies are en- largement of the central pulmonary arteries (which indicates pulmonary hypertension) and dilatation of the bronchi (which indicates airway abnormality). ...
Citations
... Chest X-ray remains the best initial test of choice [11]. It determines the site of bleeding in 45 to 65% of the cases and the cause in 25% to 35% [12]. The next step has been a subject of debate as to whether bronchoscopy is better than CT in determining the cause of hemoptysis; however, recent studies demonstrate the superiority of CT [10]. ...
... Therefore, it is often safer to postpone the bronchoscopy until a definitive diagnosis is made [10]. A radiologic definition based on CT states that pulmonary artery hypertension is diagnosed when the pulmonary artery diameter is more than 28 mm [12]. In our case, this is conclusively proven by a pulmonary artery diameter of 38 mm [13]. ...
... Recent studies have shown that acute presentations, like in this case, if treated surgically, present with more complications in the long term [12]. Studies have shown that a surgical approach is also associated with high operative mortality (>15%) [9]. ...
We report a rare case of a 24-year-old male with a rare anatomic variant of patent ductus arteriosus (PDA). The patient presented with symptoms of productive cough with recurrent and severe bouts of hemoptysis and grade I dyspnea. There were no prior episodes reported. The patient was vitally stable with bilateral clubbing. On cardiopulmonary auscultation, a prominent parasternal heave, loud P2, and right lung crepitus were noted. A complete blood count revealed an elevated hemoglobin and RBC count. An ECG revealed sinus tachycardia and right ventricle (RV) strain. ECHO confirmed these findings, as dilated right atrium (RA) and RV, mild tricuspid valve regurgitation (TR), and severe pulmonary hypertension were noted. CT of the chest demonstrated multiple ground glass opacities, right lung consolidation, and volume loss suggestive of right-sided pneumonia with atelectasis. CT also proved the presence of PDA and an anomalous origin of the right pulmonary artery from the right ascending aorta, causing compression of the right main bronchus. We show the clinical and radiological findings and discuss the implications and approach to this rare congenital cardiovascular malformation, as well as how a patient-centered approach is necessary for its management.
... Dual-energy CT may be performed in addition to a standard CTPA to produce iodine perfusion maps with high levels of agreement with V′/Q′ scintigraphy [36]. Other vascular abnormalities that may be associated with different forms of PH include calcification, aneurysms and wall thickening in vasculitis, pulmonary arteriovenous malformations in patients with hereditary haemorrhagic telangiectasia and peripheral systemic-to-pulmonary shunts in patients with CHD [37]. The PA should also be assessed for the presence of a persistent aortic connection ( persistent ductus arteriosus (PDA)). ...
Pulmonary hypertension (PH) is defined by the presence of a mean pulmonary arterial pressure >20 mmHg. Current guidelines describe five groups of PH with shared pathophysiological and clinical features. In this paper, the first of a series covering all five PH classification groups, the clinical, radiological and pathological features of pulmonary arterial hypertension (PAH) will be reviewed. PAH may develop in the presence of associated medical conditions or a family history, following exposure to certain medications or drugs, or may be idiopathic in nature. Although all forms of PAH share common histopathological features, the presence of certain pulmonary arterial abnormalities, such as plexiform lesions, and extent of co-existing pulmonary venous involvement differs between the different subgroups. Radiological investigations are key to diagnosing the correct form of PH and a systematic approach to interpretation, especially of computed tomography, is essential.
... However, recent innovation of computed tomography (CT) enables non-invasive diagnosis of SA-PAF prior to invasive procedure. Furthermore, SA-PAF is often misdiagnosed as either arteriovenous malformation or pulmonary embolism in CT, which are often seen as an aberrant pulmonary artery filling defect (1,15). The reported CT findings of SA-PAF are either nodular or diffuse soft tissue opacity in the lung periphery abutting the pleura, or hypertrophy of intercostal or other systemic arteries connected to peripheral pulmonary arteries (16). ...
Background
Systemic artery to pulmonary artery fistula (SA-PAF) is an uncommon disease which is often incidentally diagnosed during evaluation of hemoptysis patients. The aim of our study was to describe the cases of SA-PAF in our institution and to report the correlating clinical and radiological findings.
Methods
We reviewed 231 chest computed tomography (CT) scans performed in our institution due to hemoptysis from January 2020 to February 2023. In patients diagnosed with SA-PAF had their electronic medical records and CT images analyzed.
Results
In 231 patients, 19 (8.2%) of them had SA-PAF findings which was characterized by a peripheral nodular soft tissue opacity in the subpleural lung and traceable vascular structure in continuity with one or more peripheral pulmonary artery branches in CT. Etiology of each patient was categorized as either congenital (7, 36.8%), and acquired (12, 63.2%). The origins of SA-PAFs were 16 intercostal, two anterior mediastinal, and one costocervical artery. Eight of 19 patients did not show any associated intralobar imaging abnormalities, while bronchiectasis, cellular bronchiolitis, centrilobular emphysema, and pleura effusion were observed in 11 patients.
Conclusions
SA-PAF is a benign vascular anomaly which is frequently overlooked when evaluating hemoptysis by either clinician or radiologists but is an important factor in the differential diagnosis of patients with hemoptysis.
... The current gold standard of UAPA diagnosis is conventional computed tomography pulmonary angiography (CTA), as this has the advantage of highlighting spatial orientation and extent of peripheral blood vessels. UAPA is also sometimes referred to as unilateral interruption of the pulmonary artery since, classically, on CTA, it will show the beginning of the pulmonary artery branch with a blind end while the distal pulmonary arterial tree is maintained [4,8]. Other nonspecific common radiographic findings of UAPA include volume loss to the ipsilateral lung with overinflation and mediastinal shift. ...
Congenital absence of the left pulmonary artery remains a rarely reported anomalous condition and is even less commonly seen in conjunction with a right-sided aortic arch. While most cases are identified during prenatal fetal ultrasonography and require early childhood intervention, some asymptomatic cases can go unrecognized until incidentally detected on chest imaging as an adult. This case details a 31-year-old male with a congenital absence of the left pulmonary artery and right-sided aortic arch with subsequent atretic and fibrotic lung, all found on imaging during admission for acute alcoholic hepatitis.
... TTE is an important tool for the diagnosis of central PAA but has a limited role in evaluating peripheral PAAs. Ce-CT scan is the gold standard imaging tool to evaluate the morphology and the location of PAAs and to differentiate it from other vascular pathologies [5]. PAAs may be associated with pulmonary valve pathologies, as shown by Reisenauer et al. that the most common pulmonary valve pathology associated with PAA is pulmonary valve regurgitation [6]. ...
Pulmonary artery aneurysm (PAA) is a rare disorder with a difficult diagnosis and debated management in literature due to the limited number of cases. Even if the definitive treatment of PAA is surgery, consistent guidelines still need to be developed to help surgeons determine when intervention is appropriate. We report a case of a 77-year-old female diagnosed with central PAA measuring 61 mm at contrast-enhanced computerized tomography scan which was treated surgically. The patient underwent a successful elective complete pulmonary root replacement with a Medtronic Freestyle (Medtronic Inc, Minneapolis, MN) porcine root. Postprocedural recovery and follow-up at 12 months were uneventful.
... We consider PV flow splits in the ranges (left/right) 40-50%/50-60% to compare differences in LA hemodynamics between the even split [7,17,24,37,66,67] and equal velocity inflow conditions [19,21,34,68,69] often used in simulation studies, and the more physiological uneven splits caused by the right lung being larger than the left lung. However, multiple factors can alter the PV flow split outside of the range we studied, including common ones like changes in body position [41] and less common ones like abnormalities in the pulmonary artery or veins [70,71], or lung resection surgeries [72]. Additionally, body position can also affect superior/inferior PVs flow split, whose effect would be interesting to study in future work. ...
Disruptions to left atrial (LA) blood flow, such as those caused by atrial fibrillation (AF), can lead to thrombosis in the left atrial appendage (LAA) and an increased risk of systemic embolism. LA hemodynamics are influenced by various factors, including LA anatomy and function, and pulmonary vein (PV) inflow conditions. In particular, the PV flow split can vary significantly among and within patients depending on multiple factors. In this study, we investigated how changes in PV flow split affect LA flow transport, focusing on blood stasis in the LAA, using a high-fidelity patient-specific computational fluid dynamics (CFD) model. We analyzed LA anatomies from eight patients with varying atrial function, including three with AF and either a LAA thrombus or a history of TIAs. Using four different flow splits (60/40\% and 55/45\% through right and left PVs, even flow rate, and same velocity through each PV), we found that flow patterns are sensitive to PV flow split variations, particularly in planes parallel to the mitral valve. Changes in PV flow split also had a significant impact on blood stasis and could contribute to increased risk for thrombosis inside the LAA, particularly in patients with AF and previous LAA thrombus or a history of TIAs. Our study highlights the importance of considering patient-specific PV flow split variations when assessing LA hemodynamics and identifying patients at increased risk for thrombosis and stroke.
... CTA is an important tool for both detection and follow-up of PAAs as it provides the size, location, and characteristics of aneurysms such as saccular or fusiform aneurysm types. Another advantage of CTA is being helpful to reveal the underlying etiology of an aneurysm [15]. ...
Aneurysms of the pulmonary artery are uncommon vascular pathologies that are associated with congenital structural cardiac anomalies, pulmonary hypertension, vasculitis, neoplasm, iatrogenic, and infection. PAAs are commonly asymptomatic and accidentally diagnosed, however, if symptomatic, clinical features are generally non-specific and depend on the etiology of PAA. CT pulmonary angiography remains the gold standard imaging modality and other diagnostic imaging tests include transthoracic echocardiography and right heart catheterization. Definitive treatment of PAA is surgery, however, conservative management with close monitoring should be practiced in patients with poor surgical candidates or surgery is unlikely to improve survival. Here, we report a case of pulmonary artery aneurysm secondary to congenital pulmonary valve stenosis as well as a brief review of the literature regarding pulmonary artery aneurysms.
... Earlier, the term "absence of pulmonary artery" was widely used for this condition. But, the recent consensus in scientific literature states that as the intrapulmonary vascular network is still intact, the term "interruption of the pulmonary artery" is preferred over "absence of pulmonary artery" [3]. ...
... On plain radiographs, hypoplasia of the lung can present as elevation of the diaphragm with an ipsilateral mediastinal shift indicating volume loss in the hemithorax [3]. Compensatory hyperinflation of the opposite lung with herniation into the smaller hemithorax can also be noted. ...
... On CT scans, the mediastinal portion of the affected pulmonary artery may be completely absent or may terminate within 1 cm of its origin [3]. Anastomosis of the transpleural collateral vessels with the peripheral branches of the pulmonary artery can be seen as serrated thickening of the pleura and subpleural parenchyma on CT scans [3]. ...
Introduction
Proximal interruption of pulmonary artery (PIPA) is a congenital anomaly presenting with aberrant termination of the pulmonary artery at the hilum. It results in a variety of radiological and clinical manifestations. Clinically, isolated PIPA can be asymptomatic till late adulthood or can present with dyspnoea, chest discomfort, hemoptysis and recurrent infections. PIPA can be associated with multiple cardiovascular anomalies such as tetralogy of Fallot (TOF), ventricular septal defects (VSD), and scimitar syndrome. We present a spectrum of cases with both isolated proximal interruption of the pulmonary artery and cases associated with other cardiovascular abnormalities. Typical chest radiographs and chest contrast-enhanced computed tomography (CECT) findings are discussed and demonstrated in detail. Proper and early diagnosis is a crucial step as it can significantly modify the treatment choice, thereby reducing morbidity.
Objective
To document the different presentations of the proximal arrest of pulmonary arteries, to document associations with cardiovascular and pulmonary manifestations, and to elaborate on and demonstrate the various radiological imaging findings.
Material and methods
All the cases that were reported with proximal interruption of pulmonary artery on the CECT studies conducted between 2019 and 2022 at a tertiary care hospital in Telangana, India. The demographic data, clinical presentation, chest radiographs, and chest CECT were collected retrospectively. Data analysis was done using Microsoft Excel 2019 to calculate descriptive statistics. A total of 22 cases were identified of which three cases were excluded of as they were previously operated and 19 cases were taken as the study population.
Results
Nineteen patients were included in the study. Demographic details, clinical history, CECT, and chest radiographs were collected wherever available. The majority of the cases belonged to the ≤ 10 yrs age group with the most common clinical presentation being a previous diagnosis of tuberculosis or recurrent upper respiratory tract infections. The predominant findings on chest radiographs were deviation of the trachea to the affected side, volume loss in the ipsilateral lung field, and compensatory hyperinflation of the contralateral lung field. On the CECT chest, the main findings were interrupted pulmonary artery, hypoplastic lung fields with bronchiectasis, or ground glassing. Associated cardiovascular and pulmonary malformations were identified with notable cases: TOF, right-sided aortic arch and scimitar syndrome. Their typical imaging findings have been elucidated and discussed in detail.
Conclusions
Patients with recurrent respiratory infections or hemoptysis having hypoplastic lung field with hyperinflation of the contralateral lung on chest radiographs should be evaluated for pulmonary artery interruptions. Chest CECT allows evaluation of the bronchial tree and lung parenchyma at the same time which helps distinguish pulmonary interruption from conditions such as Swyer-James-Macleod syndrome, pulmonary hypoplasia, thromboembolism and arteritis. Cases with PIPA can also be associated with cardiovascular and pulmonary anomalies such as TOF, partial anomalous pulmonary venous connection (PAPVC), and VSD. The knowledge of these associations is essential as they can influence the mode of treatment and can help reduce the long-term morbidity and mortality associated with the condition.
... The CT approach to diagnosis of PH begins with identifying an enlarged pulmonary artery diameter greater than 29 mm, which is usually larger than that of ascending aorta at the same level [16] . The diameter must be measured in the axial plane at the bifurcation, orthogonal to the long axis of the pulmonary artery [17] . Transthoracic two dimensional doppler echocardiography is the first line modality for diagnosis of PH [18] . ...
... Pulmonary artery stenosis (PAS) is a congenital or acquired malformation that may involve the main, branched, lobar, segmental, or distal levels of the pulmonary arterial tree. 1 Being a heterogeneous condition, PAS could manifest from either intrinsic lesions or extrinsic compression attributable to a series of diseases, including Takayasu arteritis, fibrosing mediastinitis, and congenital heart diseases. [2][3][4][5] Although the aetiology of PAS varies, similarities have been observed in its pathophysiology and clinical manifestations. ...
Aims
The prognosis is poor for patients with pulmonary artery stenosis‐associated pulmonary hypertension (PAS‐PH). Identifying predictors of prognosis in PAS‐PH is crucial to preventing premature death, which has rarely been investigated. We aimed to explore the cardiopulmonary exercise testing (CPET) parameters to predict the prognosis of these patients.
Methods
We prospectively included all patients with PAS‐PH who underwent CPET between September 2014 and June 2021 in Fuwai Hospital (ClinicalTrials.gov ID: NCT02061787). The primary outcome was clinical worsening, including death, rehospitalization for heart failure, or deterioration of PH.
Results
Seventy‐two patients were included in this study. A median of 2‐year follow‐up revealed that 18 (25%) patients experienced clinical worsening. The 1‐year, 3‐year, and 5‐year event‐free survival rates were 92.5%, 81.7%, and 62.7%, respectively. Patients with clinical worsening demonstrated significantly worse baseline haemodynamics and poorer exercise capacity than their counterparts. Multivariable Cox regression identified that peak O2 pulse could independently predict clinical worsening [hazard ratio: 0.344, 95% confidence interval (CI) 0.188–0.631, P < 0.001], outperforming other parameters. Peak O2 pulse correlated with PH severity. Incorporating peak O2 pulse into the simplified 2015 European Society of Cardiology/European Respiratory Society risk stratification improved the accuracy for predicting clinical worsening (pre vs. post area under the curve: 0.727 vs. 0.846, P < 0.001; net reclassification index: 0.852, 95% CI 0.372–1.332, P < 0.001; integrated discrimination index 0.133, 95% CI 0.031–0.235, P = 0.011).
Conclusions
The prognosis is poor for PAS‐PH, and exercise intolerance and ventilation inefficiency are commonly observed. Peak O2 pulse independently predicted the prognosis of these patients. A low peak O2 pulse identified patients at high risk of clinical deterioration and served for risk stratification of PAS‐PH.
