Dieulafoy's disease of the bronchus: rare but potentially fatal: a case report and a review of literature.

BACKGROUND: Dieulafoy's disease of the bronchus can cause massive and even fatal hemoptysis. Even though it is rare, it should be considered by physicians all over the world. This paper reports a case of bronchial Dieulafoy's disease and summarizes the data of similar cases reported in literature. METHODS: We report a case of bronchial Dieulafoy's disease (BDD) in Tunisia. We also present a review of literature related to BDD from 1995 to 2022 using the PubMed, Google Scholar, web of science and Chinese National Knowledge Infrastructure Databases. Clinical characteristics, chest imaging, bronchoscopic and angiographic findings were summarized. Treatment courses were identified as well as patients' outcome. RESULTS: We report the case of a 41-year-old man, so far in good health, presenting with massive hemoptysis. Bronchoscopy showed blood clots and a protruding lesion covered by mucosa with a white pointed cap at the entrance of the right upper lobe. Biopsies were not attempted. Embolization of bronchial artery was first realized and was not successful, with post procedure complications. Surgical intervention stopped the bleeding and pathological examination of the resected specimen confirmed Dieulafoy's disease of the bronchus. Ninety cases of BDD were reported from 1995 to 2022. The main symptom was hemoptysis. Chest imaging findings were not specific. The diagnosis of BDD was mainly based on the bronchoscopy, branchial angiography and pathological findings or surgical specimens. Bronchoscopy findings were mostly nodular or prominent lesions (52.4%). Twenty-eight patients underwent bronchoscopic biopsies, 20 had massive bleeding and 10 died. Bronchial angiography mainly showed tortuous and dilation of bronchial artery, and the lesions were mainly located in the right bronchus. Selective bronchial artery embolization (SBAE) was performed in 32 patients and 39 patients underwent surgery. CONCLUSION: To our knowledge, this is the first case of bronchial Dieulafoy's disease to be reported in Tunisia and North Africa. When the diagnosis is suspected, bronchoscopic biopsy should be avoided as it might lead to fatal hemorrhage. Selective bronchial artery embolization can stop the bleeding, but surgery can be required.

Artificial Intelligence-based CT Assessment of Bronchiectasis: The COPDGene Study.

Background CT is the standard method used to assess bronchiectasis. A higher airway-to-artery diameter ratio (AAR) is typically used to identify enlarged bronchi and bronchiectasis; however, current imaging methods are limited in assessing the extent of this metric in CT scans. Purpose To determine the extent of AARs using an artificial intelligence-based chest CT and assess the association of AARs with exacerbations over time. Materials and Methods In a secondary analysis of ever-smokers from the prospective, observational, multicenter COPDGene study, AARs were quantified using an artificial intelligence tool. The percentage of airways with AAR greater than 1 (a measure of airway dilatation) in each participant on chest CT scans was determined. Pulmonary exacerbations were prospectively determined through biannual follow-up (from July 2009 to September 2021). Multivariable zero-inflated regression models were used to assess the association between the percentage of airways with AAR greater than 1 and the total number of pulmonary exacerbations over follow-up. Covariates included demographics, lung function, and conventional CT parameters. Results Among 4192 participants (median age, 59 years; IQR, 52-67 years; 1878 men [45%]), 1834 had chronic obstructive pulmonary disease (COPD). During a 10-year follow-up and in adjusted models, the percentage of airways with AARs greater than 1 (quartile 4 vs 1) was associated with a higher total number of exacerbations (risk ratio [RR], 1.08; 95% CI: 1.02, 1.15; P = .01). In participants meeting clinical and imaging criteria of bronchiectasis (ie, clinical manifestations with ≥3% of AARs >1) versus those who did not, the RR was 1.37 (95% CI: 1.31, 1.43; P < .001). Among participants with COPD, the corresponding RRs were 1.10 (95% CI: 1.02, 1.18; P = .02) and 1.32 (95% CI: 1.26, 1.39; P < .001), respectively. Conclusion In ever-smokers with chronic obstructive pulmonary disease, artificial intelligence-based CT measures of bronchiectasis were associated with more exacerbations over time. Clinical trial registration no. NCT00608764 © RSNA, 2022 Supplemental material is available for this article. See also the editorial by Schiebler and Seo in this issue.

A histological and immunohistochemical study on the parabronchial epithelium of the domestic fowl's (Gallus gallus domesticus) lung with special reference to its scanning and transmission electron microscopic characteristics.

The current study was designed to give complete histo-and immunohistochemical features of the parabronchial epithelium of domestic fowl's (Gallus gallus domesticus) lung with special reference to Scanning electron microscope (SEM) and mean transmission electron microscope (TEM) features. The lung exhibited variable-sized atrial openings encircled by exchange tissue zones. The parabronchial atrial chambers appeared as ovoid and polygonal-shaped that separated by the well-developed interatrial septum. The deep atrial lumens had blood vessels pierced by openings that represent the infundibula. The parabronchial blood capillaries meshwork was branched and exhibited ovoid-shaped air capillaries with numerous extravasated blood vessels. By TEM, there were several air capillaries and groups of squamous and endothelial respiratory cells and the squamous cells had oval nucleus with evenly distributed chromatin. The endothelial respiratory cells had few microvilli on their free surfaces. The parabronchial tubes opened into a group of widened atria that had smooth muscle bundles at the interatrial septa. The atrial chambers led to narrow infundibula. Moreover, the lining epithelium of parabronchi, atria, infundibula, and air capillaries was formed by simple squamous epithelium. Air capillary walls were lined by two types of respiratory cells (Types-I and II). Collagen fibers were concentrated within the tunica externa layers of the parabronchial blood vessels as well as, they were observed in CT interparabronchial septa. Immunohistochemically, the elastin immunoreactivity was detected around the parabronchial blood vessels, at the base of each parabronchial atria, and in the area encircling the alveolar-capillary walls. Our work concluded that there are a relation between the fowl's lifestyle and the surrounding environmental conditions.

Management of Significant Airway Bleeding during Robotic Assisted Bronchoscopy: A Tailored Approach.

Robotic assisted bronchoscopy represents a major turning point in bronchoscopic history. The management strategies to address significant airway bleeding in this "robotic era" are not well documented, and further guidance is required. We present a case report that exemplifies our approach and management strategy using a combined and simultaneous flexible/robotic bronchoscopy if this complication is encountered.

VATS right posterior segmentectomy with anomalous bronchi and pulmonary vessels: a case report and literature review.

BACKGROUND: Anatomic variation may increase the difficulty and risk of anatomic segmentectomy. The preoperative three-dimensional computed tomography bronchography and angiography (3D-CTBA) can provide a detailed model of the segmental structure, and contribute to precise and safe segmentectomy. CASE PRESENTATION: This is a case of anomalous bronchi and pulmonary vessels in the right upper posterior segment (RS2). Under the guidance of 3D-CTBA, anatomic RS2 segmentectomy was performed accurately and safely. The postoperative condition was uneventful. CONCLUSIONS: This rare case highlights the importance of 3D-CTBA to guild accurate segmentectomy with anatomic variation.

[A Study on the Authenticity of Preoperative Pulmonary Bronchial Angiography by DeepInsight Software].

BACKGROUND: Precise segmentectomy has become the first choice of surgical treatment for pulmonary nodules and early lung cancer, and the key and difficult point of the surgery lies in the precise location and resection of the lesion. DeepInsight is an auxiliary software for precise lung surgery jointly developed by our center and Neusoft Company, which can determine the precise anatomy of the lung and locate the location of lung lesions before operation. This study is to verify the authenticity and reliability of DeepInsight lung bronchial angiography assisted surgery. METHODS: In this study, 1,020 patients with pulmonary nodules <2.0 cm in diameter were included in the Department of Thoracic Surgery Jiangsu Provincial People's Hospital from August 1, 2016 to December 31, 2019. Computed tomographic angiography (CTA) was performed on all the included patients before surgery. The DeepInsight software was used to perform preoperative bronchial angiography on the operative side of the lung to identify the affected pulmonary segments, pulmonary arteries and pulmonary veins. Two thoracic surgeons independently assessed the visibility of the affected pulmonary vessels using the 5-point method, and the χ² test assessed the consistency between observers. In addition, virtual imaging and real anatomy of pulmonary vessels on the operative side were performed during the operation, and the involved pulmonary vessels were finally determined by 2 chief physicians of thoracic surgery. RESULTS: There were no statistically significant differences between the number and spatial anatomy of the vessels involved in the pulmonary virtual imaging using DeepInsight software before operation and the number of vessels involved during operation in 1,020 patients. And the consistency among observers is quite satisfactory. CONCLUSIONS: The DeepInsight software virtual imaging of pulmonary bronchial vessels can accurately reconstruct the actual pulmonary vessels and assist the completion of pulmonary segmental resection.

Recurrent Hemoptysis: A Bronchial Dieulafoy's Lesion in a Pediatric Patient.

OBJECTIVE: This paper presents a case of a bronchial Dieulafoy's lesion in a pediatric patient with recurrent hemoptysis. CASE REPORT: A 11-year old female presented multiple times with dry cough and hemoptysis to an outside hospital, each time leading to a diagnosis of epistaxis and subsequent discharge. When she arrived to our tertiary center with heavy hemoptysis and no evidence of epistaxis, the patient was urgently taken to the operating room by both the otolaryngology and pediatric pulmonology services. Active bleeding from a Dieulafoy's lesion on the right lower bronchus was found and selective embolization of two tortuous arteries was subsequently performed. The patient was discharged in stable condition without recurrence of hemoptysis over the last two months. CONCLUSION: While rare, especially in pediatric patients, bronchial Dieulafoy's lesions may cause severe hemoptysis and should be considered in the differential diagnosis when the etiology for hemoptysis is unclear.

Bronchovascular bundle thickening on CT as a predictor of survival and brain metastasis in patients with stage IA peripheral small cell lung cancer.

AIM: To determine if bronchovascular bundle (BVB) thickening on pretreatment computed tomography (CT) images helps predict survival in patients with peripheral small cell lung cancer (pSCLC) ≤3 cm. MATERIALS AND METHODS: The pretreatment CT examinations of 79 histopathologically proven pSCLC ≤3 cm (TNM stage I, 21; II, 13; III, 22; IV, 23) were reviewed retrospectively. The CT characteristics of the nodule and associated findings, including BVB thickening, were evaluated. Progression-free survival (PFS), overall survival (OS), and brain metastasis-free survival were compared with the presence of BVB thickening using Kaplan-Meier and Cox regression analysis. RESULTS: Among the 79 patients, 34 (43%) had BVB thickening. BVB thickening was prevalent in patients with mediastinal lymph node metastasis (50.9% versus 22.7%; p=0.024) and distant metastasis (60.9% versus 35.7%; p=0.049). Out of the 21 patients with TNM stage IA disease, the 16 patients (76.2%) without BVB thickening showed better PFS, OS, and brain metastasis-free survival (mean, 1,762 versus 483 days; p=0.019: 2,243 versus 1,328 days; p=0.038: 2,274 versus 1,287 days; p=0.038, respectively). Multivariate Cox regression analysis showed that the absence of BVB thickening (hazard ratio [HR], 7.806; 95% CI, 1.241-49.091; p=0.029) and surgery (HR, 0.075; 95% CI, 0.008-0.746; p=0.027) were independent and useful prognostic factors for PFS. CONCLUSIONS: BVB thickening was found more frequently in patients with advanced-stage pSCLC ≤3 cm, and the PFS was more favourable in patients without BVB thickening, with a similar tendency to that of OS and brain metastasis-free survival, in stage IA pSCLC.

Delineation of the healthy rabbit lung by immunohistochemistry - a technical note.

Investigation and studies of pulmonary diseases and injuries require pre-clinical animal models. The rabbit lung model is widely used and allows for a diverse set of readouts. Among them, histology and immunohistochemistry are of invaluable merit because qualitative and quantitative information about tissue morphology and composition can be easily obtained. In this technical note, we performed several histological and immunohistochemical stainings in the rabbit healthy naïve lung tissue. Overnight formalin fixation with subsequent paraffin embedding was compared to cryopreservation with a subsequent 10-minute formalin fixation prior to staining. Antigen retrieval (AR) for paraffin embedded sections proved to enhance the corresponding signals compared to analogous staining without AR. Advantages and disadvantages of chromogenic versus immunofluorescence stainings were discussed. In addition, several morphological structures, such as the intrapulmonary bronchus with its mucosal folds, the pulmonary artery, the alveoli and the lymph nodes, were stained with various stainings at the same site in order to give a comprehensive picture of their composition. Besides Haematoxylin&Eosin and Elastica van Gieson staining, collagen I, collagen III, fibronectin, α-SMA, ki-67 and protease-activated receptor-2 (PAR-2) immunohistochemistry was performed. Collagen I, collagen III and fibronectin expression was positive at the outer rim of the pulmonary arteries, while the inner rim was collagen III positive. Moreover, the fibronectin staining in the intrapulmonary bronchus showed an opposite trend when compared to the collagen III staining. The alveoli exhibited PAR-2 expression, while PAR-2 was not expressed in lymph nodes of the healthy rabbit lung.

Fractal analysis of concurrently prepared latex rubber casts of the bronchial and vascular systems of the human lung.

Fractal geometry (FG) is a branch of mathematics that instructively characterizes structural complexity. Branched structures are ubiquitous in both the physical and the biological realms. Fractility has therefore been termed nature's design. The fractal properties of the bronchial (airway) system, the pulmonary artery and the pulmonary vein of the human lung generates large respiratory surface area that is crammed in the lung. Also, it permits the inhaled air to intimately approximate the pulmonary capillary blood across a very thin blood-gas barrier through which gas exchange to occur by diffusion. Here, the bronchial (airway) and vascular systems were simultaneously cast with latex rubber. After corrosion, the bronchial and vascular system casts were physically separated and cleared to expose the branches. The morphogenetic (Weibel's) ordering method was used to categorize the branches on which the diameters and the lengths, as well as the angles of bifurcation, were measured. The fractal dimensions (DF) were determined by plotting the total branch measurements against the mean branch diameters on double logarithmic coordinates (axes). The diameter-determined DF values were 2.714 for the bronchial system, 2.882 for the pulmonary artery and 2.334 for the pulmonary vein while the respective values from lengths were 3.098, 3.916 and 4.041. The diameters yielded DF values that were consistent with the properties of fractal structures (i.e. self-similarity and space-filling). The data obtained here compellingly suggest that the design of the bronchial system, the pulmonary artery and the pulmonary vein of the human lung functionally comply with the Hess-Murray law or 'the principle of minimum work'.

Localized Bronchial Hyperemia in Cases of Iatrogenic Hemoptysis: Clinical Presentations and Pathophysiological Mechanisms.

Hemoptysis is a frequently encountered symptom in many clinical settings, and etiologic diagnosis can sometimes prove challenging. Bronchoscopy may not promptly reveal the source or the cause of bleeding and few reports have focused so far on the abnormalities of bronchial mucosa vasculature that may unveil the underlying pathophysiology. In this special feature article, we present a series of cases presenting with hemoptysis after angiographic interventions in the thoracic vessels. Localized hyperemia and vascular dilatations in the bronchial mucosa observed during bronchoscopy as unique findings became clues enabling the correct diagnosis and management. We suggest the relevant pathophysiological mechanisms and discuss the available published experience on similar clinical entities.

Bilateral Lobar Transplants Using One Donor for Two Small-Sized Recipients.

Cadaveric lobar lung transplantation is an alternative for patients whose chest cavities have small dimensions. We present here a case where 1 donor was used for bilateral lobar transplantations in 2 high-risk patients. Coordination between the graft preparation at the back table and the 2 concomitant lung transplant teams was necessary to minimize the ischemic injury of the grafts and to plan for adequate vascular and bronchial cuffs for both implantations.

Exercise Intolerance in Heart Failure: Central Role for the Pulmonary System.

We propose that abnormalities of the pulmonary system contribute significantly to the exertional dyspnea and exercise intolerance observed in patients with chronic heart failure. Interventions designed to address the deleterious pulmonary manifestations of heart failure may, therefore, yield promising improvements in exercise tolerance in this population.

Bronchial-arterial-circulation-sparing Lung Preservation: A New Organ Protection Approach for Lung Transplantation.

BACKGROUND: Compromised microvasculature resulting from disrupted bronchial arterial circulation appears to trigger chronic lung allograft dysfunction. Maintaining the microvasculature throughout the transplant process could improve the long-term health of transplanted lungs. We recently developed a bronchial-arterial-circulation-sparing (BACS) lung preservation approach and tested whether this approach would decrease microvascular damage and improve allograft function. METHODS: The lungs of Lewis rats were procured using either the BACS approach, where the bronchial and pulmonary arteries were synchronously perfused; a conventional approach, where only the pulmonary artery was perfused; or a conventional approach with a prostaglandin flush. After 4 hours of cold ischemia, physiologic function and vascular tone of the grafts were evaluated during ex vivo lung perfusion (EVLP), and microvasculature damage was assessed using 2-photon microscopy analysis. Lung function was compared after transplant among the groups. RESULTS: After 4 hours of cold ischemia, the BACS group exhibited significantly higher adenosine triphosphate levels and lower expression of phosphorylated myosin light chain, which is essential for vascular smooth muscle contraction. On EVLP, the BACS and prostaglandin groups showed lower pulmonary vascular resistance and less arterial stiffness. BACS attenuated microvasculature damage in the lung grafts when compared with conventional preservation. After transplantation, the lungs preserved with the BACS approach exhibited significantly better graft function and lower expression of phosphorylated myosin light chain. CONCLUSIONS: Our data suggest that BACS lung preservation protects the dual circulation inherent to the lungs, facilitating robust microvasculature in lung grafts after transplantation, leading to better posttransplant outcomes.

Fatal pulmonary hemorrhage in a horse during bronchoalveolar lavage - single case report.

BACKGROUND: Pulmonary hemorrhage is a rare cause of death in horses. Hemorrhage within the respiratory tract has many causes, including mycosis of the guttural pouch, invasive procedures causing serious trauma to nasal conchae, or lung biopsy. We report on a rare case of a fatal pulmonary hemorrhage in a horse after a severe cough during bronchoalveolar lavage. To the best of our knowledge, this is the first report of spontaneous hemorrhage in a horse during bronchoalveolar lavage. CASE PRESENTATION: A 21-year-old mare which belonged to the didactic herd of The Faculty of Veterinary Medicine underwent BAL procedure for training purposes. Clinical examination prior to the procedure did not reveal any abnormalities and the horse had been classified as healthy. The horse was sedated with 0.01 mg/kg of detomidine and 0.01 mg/kg of butorphanol. The silicon BAL catheter was passed through the nasal passage into the trachea and then into the bronchus. Before catheter was wedged, the mare began to cough heavily and massive haemorrhage from mouth and nostrils occurred. Despite fluid therapy, shock occurred within 15 min and the mare was euthanized. Upon necropsy, site of hemorrhage was identified in the left lobar caudal bronchi, from a large blood vessel running directly beneath the bronchial wall. Upon histology, a chronic lympho-plasmocytic inflammatory process in left bronchi was identified. Moreover, Masson's trichrome staining revealed severe, perivascular fibrosis. CONCLUSION: Although BAL is a relatively safe procedure, and such complications should be treated as extremely rare, this case indicates that, in some individuals with specific subclinical problems, even mild physical force such as a cough can lead to rupture of the artery.

[Bronchoscopy and Interventional Pulmonology].

Recent advance in bronchology and interventional pulmonology contributes to improve the quality of patient care in thoracic surgery. Narrow band imaging (NBI) combined with high definition bronchoscopy clearly visualize the aberrant tumor vessels on the mucosa of endobronchial malignancy. It helps the preoperative surgical planning for the clear bronchial margin during the surgery. The index of hemoglobin (IHb) show the approximate amount of hemoglobin within the mucosal surface and we use the IHb for the evaluation of local circulation of bronchial anastomosis after bronchoplasty. Endobronchial ultrasound-guided transbronchial needle aspiration( EBUS-TBNA) is now the best 1st test for the mediastinal staging in patients with lung cancer. The enough anatomical knowledge and adequate training of EBUS-TBNA is important to achieve high diagnostic yield with safety. Endobronchial occlusion therapy using endobronchial Watanabe spigot (EWS) is a novel therapeutic approach for the patients with persistent air leakage and bronchial fistula. We use the EWS for the empyema cases with pleural fistula in combination with negative pressure wound therapy. In addition, EWS can be applicable for the refractory broncho-gastric tube fistula in patients with esophageal cancer. The hybrid stent which was fully covered metallic stent is approved in Japan in 2014. This novel stent provide the more chance of stent insertion not only for the patients with airway stenosis but also for the broncho-esophageal fistula.

Efficacy and safety of endobronchial ultrasound-guided transbronchial needle aspiration through the pulmonary arteries for the diagnosis of left hilar lesions.

INTRODUCTION: Endobronchial ultrasonography (EBUS) is an endoscopic method that aids needle aspiration to see the bronchial wall and adjacent tissues with an ultrasound probe. Pulmonary arteries are rarely present between the bronchus wall and the tissue. In this case, it was necessary to make a selection between invasive processes and transbronchial needle aspiration (TBNA) through the pulmonary artery. There are few case reports about the safety of TBNA through the pulmonary artery. We aimed to present the results of EBUS guided TBNA through the pulmonary arteries. MATERIALS AND METHODS: The data on four cases (three men) in whom EBUS guided TBNA was performed through the pulmonary artery between August 2010 and December 2015 were reviewed retrospectively. Procedures were conducted under local anesthesia and conscious sedation. For TBNA, 22-gauge needles were used. Cases were monitored for 24 hour after the procedures. Antibiotic prophylaxis and onsite cytopathology were not used. RESULT: All lesions existed were on the left hilar localization. Two of the diagnosed cases were carcinoma and one was the granulomatous lymphadenitis. We were not able to diagnose the last case. No complication was observed in any cases during the procedure. CONCLUSIONS: EBUS guided TBNA through the pulmonary arteries at left hilar lesions is safe. The rate of diagnoses from the tissues obtained is high. No special preparation is needed for the cases have no the pulmonary hypertension.

Bronchovascular injury associated with clinically significant hemoptysis after CT-guided core biopsy of the lung: Radiologic and histopathologic analysis.

OBJECTIVE: To evaluate bronchovascular injuries as the causative occurrence for clinically significant hemoptysis after percutaneous transthoracic needle biopsy (PTNB). MATERIALS AND METHODS: We included 111 consecutive patients who experienced hemoptysis after cone beam CT (CBCT)-guided PTNB from January 2014 through January 2017. Clinically significant hemoptysis was defined as hemoptysis causing hemodynamic instability or oxygen desaturation greater than 10% of baseline. The lesion characteristics were evaluated on CT images. The penetration of bronchovascular structures along the trajectory of the introducer needle and potential penetration at the firing of the biopsy gun were assessed on CBCT images. The cutting injury of bronchovascular structures was histopathologically assessed in biopsy specimens. The associated factors for clinically significant hemoptysis were assessed using logistic regression analyses. RESULTS: Seventeen patients (15.3%; 95%CI, 9.7%-23.2%) had clinically significant hemoptysis. On univariate analysis, the open bronchus sign (P = .004), nodule consistency (P = .012), potential penetration of a pulmonary vessel or bronchus 1 mm or larger at firing (P = .008 and P = .038, respectively), and a cutting injury of a pulmonary vessel 1 mm or larger (P = .007) or a bronchial structure (P = .041) were associated with clinically significant hemoptysis. Multivariate analysis found the following significant associated factors: potential penetration of a pulmonary vessel 1 mm or larger at firing (OR, 3.874; 95%CI, 1.072-13.997; P = .039) and cutting injury of a pulmonary vessel 1 mm or larger (OR, 6.920; 95%CI, 1.728-27.711; P = .006) or a bronchial structure (OR 4.604; 95%CI, 1.194-17.755; P = .027). CONCLUSION: Potential penetration and cutting injury of bronchovascular structures 1mm or larger at firing were independently associated with clinically significant hemoptysis after PTNB.

Evaluation of the CT imaging findings in patients newly diagnosed with chronic thromboembolic pulmonary hypertension.

PURPOSE: The aim of this study was to evaluate the vascular and parenchymal CT imaging findings, including vessel and cardiac chamber diameter measurements, in patients newly diagnosed with chronic thromboembolic pulmonary hypertension (CTEPH). The CT imaging findings were correlated with hemodynamic measurements and patient outcome. METHODS: Vascular and parenchymal CT findings were assessed on retrospectively ECG-gated MDCT angiography scans in 76 patients newly diagnosed with CTEPH. The diameters of the right and left ventricle (dRV, dLV), the right and left atrium (dRA, dLA), the ascending aorta (dAA), the right and left pulmonary arteries (drPA, dlPA), and the main pulmonary artery (dPA) were measured on axial CT scans. The CT imaging findings were correlated with demographic and hemodynamic data and adverse patient outcome due to right heart failure (RHF). RESULTS: The majority of patients showed chronic PE, mosaic perfusion, disparity in segmental vessel size, parenchymal densities, bronchial dilatation, and bronchial collaterals in CT. Mean pulmonary artery pressure (mPAP) was not significantly different in patients with and those without chronic PE, mosaic perfusion, disparity in segmental vessel size, parenchymal densities, bronchial dilatation, and bronchial collaterals. Mean PAP showed significant correlations with the CT metrics of dRV/dLV ratio, dRA, dRV, dPA and dPA/dAA ratio, but no correlation with the central thrombi score. By backward linear regression, the dPA/dAA ratio independently correlated with mPAP. Patients who died of RHF tended to have a higher frequency of exclusively chronic peripheral PE and greater dRV/dLV ratios on presenting CT scans compared with survivors. CONCLUSION: The majority of patients newly diagnosed with CTEPH show vascular signs of chronic PE, mosaic perfusion, parenchymal densities, disparity in segmental vessel size, bronchial dilatation, and bronchial collaterals on presenting CT scans. Particularly CTEPH patients with exclusively chronic peripheral PE and increased dRV/dLV ratios seem to be at risk of adverse outcome due to RHF.