Dilated Pulmonary Artery Research Articles

Clinical, laboratory, and echocardiographic characteristics of critical multisystem inflammatory syndrome in children: a retrospective, observational study

ObjectiveMultisystem inflammatory syndrome in children (MIS-C) is a critical childhood disease that is associated with coronavirus disease (COVID-19). We aimed to describe the clinical, laboratory, and echocardiographic characteristics and outcome of critical MIS-C cases in Egyptian children during the first wave of the COVID-19 pandemic.DesignA retrospective, observational study.SettingA single-center tertiary pediatric intensive care unit (PICU).In Ain Shams university hospitals Cairo EgyptMethodsChildren admitted to the PICU diagnosed with severe MIS-C as per the Centers for Disease Control’s definition from June 23, 2020, to August 22, 2020, were included.ResultsThe patient’s mean age was 7.45 (interquartile range [IQR], 4.23) years, and the cause of PICU admission was hypotension and shock. All patients had a fever for 4.8 (IQR, 2.5) days before shock developed. Overall, 68% had a gastrointestinal manifestation, and 55.6% had a rash. Thirty-five of 45 patients had ≥ 4 elevated inflammatory markers. The cardiac troponin I level was elevated in 35 of 45 patients. The most common cardiac condition was valvulitis (tricuspid regurgitation, 29/45; mitral valve regurgitation, 28/45; pulmonary valve regurgitation, 5/45; atrial valve regurgitation, 4/45). Twenty-one patients had an impaired ejection fraction < 50%, and 17 patients had coronary dilatation. Six patients had pericardial effusion, 1 patient had dilated pulmonary arteries, and 6 patients (13.3%) died of their associated comorbidities. The mean PICU length of stay among patients with no associated comorbidities was 7 days.ConclusionsCritical cases of MIS-C had a spectrum of different cardiac conditions, with valvulitis being the most common one. The worst outcome occurred in patients with comorbidities and infants.

Coronary artery compression by dilated pulmonary artery in an early infantile case of tetralogy of Fallot with absent pulmonary valve: ventricular fibrillation as the initial symptom of myocardial ischaemia.

Coronary artery compression by a dilated pulmonary artery is a rare complication in patients with tetralogy of Fallot with absent pulmonary valve. We present a case in which this condition manifested at two months of age, with ventricular fibrillation as the initial symptom of myocardial ischaemia. It is important to recognise that this potentially fatal complication can occur in early infancy.

Salidroside ameliorates hypoxic pulmonary hypertension by regulating the two-pore domain potassium TASK-1 channel

BackgroundHypoxic pulmonary vasoconstriction (HPV) is a reflex constriction of vascular smooth muscle. This study aims to investigate the role of Salidroside (Sal) in pulmonary arterial dilatation and the potential mechanism of Sal regulating hypoxic pulmonary hypertension in vitro and in vivo. MethodsA rat model of hypoxic pulmonary hypertension (HPH) was constructed using hypoxic chamber. The effect of Sal on HPH were evaluated using vascular ring, whole cell patch-clamp, WGA staining, HE staining, and Sirius Scarlet staining assays. ResultsSal treatment alleviated the injury of acute hypoxia on pulmonary circulation in SD rats. Meanwhile, Sal treatment reduced the pulmonary vascular tone of acute hypoxia in a concentration-dependent manner, which was involved in the TWIK-related acid-sensitive potassium channel 1 (TASK-1) mediating diastolic effect. We found that Sal treatment significantly increased the TASK-1 current of pulmonary artery smooth muscle cells (PASMCs) in a concentration-dependent manner, as well as reversed the inhibitory effect of acute hypoxia on the TASK-1 current. Moreover, Sal treatment improved the TASK-1 current density, suppressed the proliferation, and enhanced the apoptosis of PASMCs in SD rats under continuous hypoxic condition. In addition, we found that the electrophysiological remodeling and pulmonary vascular remodeling of PASMCs were improved by the treatment of Sal through the regulation of TASK-1 channel. ConclusionsSal could alleviate HPH by restoring the function of TASK-1 channel, which may provide a novel method for the treatment of HPH.

Prognostic Value of Transthoracic Impedance Cardiography, Amino-Terminal Pro-B-Type Natriuretic Peptide Levels, The Six-Minute Walk Test, and Chest X-Ray in Elderly Patients with Chronic Heart Failure: A Comparative Study in Lithuania.

BACKGROUND Chronic heart failure (CHF) is a complex clinical syndrome associated with frequent, recurrent, and long-term hospitalizations. This study from a single center in Lithuania aimed to evaluate outcomes in 87 elderly patients hospitalized with CHF. The methods used included comparing transthoracic impedance cardiography (ICG), amino-terminal pro-B-type natriuretic peptide (NT-proBNP) levels, the six-minute walk test (6MWT), and dilatation of the right pulmonary artery on chest X-ray (dRPAcXR). MATERIAL AND METHODS The study sample consisted of 87 patients (49 men and 38 women). All subjects underwent 6MWT and ICG in addition to the standard CHF tests. The median duration of the follow-up was 23 months. Data about patient outcomes were gathered from the National Medical Record Database. RESULTS By multivariate Cox proportional analysis, thoracic fluid content (TFC) ≥41.1 1/kΩ (hazard ratio [HR] 32.354, 95% confidence interval [CI] 2.758-379.488), NT-proBNP ≥332.0 pmol/L (HR 4.739, 95% CI 1.656-13.559), 6-minute walk distance (6MWD) ≤203.5 m (HR 3.975, 95% CI 1.002-15.770), and dRPAcXR (HR 5.555, 95% CI 1.714-18.005) were associated with a poor prognosis in CHF patients (all P<0.05). The correlations between ICG and 6MWD and other non-invasive diagnostic tests examined in this study were weak to moderate. CONCLUSIONS TFC ≥41.1 1/kΩ, NT-proBNP ≥332.0 pmol/L, 6MWD ≤203.5 m, and dRPAcXR had a combined prognostic value in predicting cardiovascular death in patients with CHF. Therefore, these parameters may be of value in the assessment of the diagnosis and prognosis in this patient cohort.

Discovery of a novel mutation F184S (c.551T>C) in GATA4 gene causing congenital heart disease in a consanguineous Saudi family

Background & aimCongenital heart disease (CHD) is the most common cause of non-infectious deaths in infants worldwide. However, the molecular mechanisms underlying CHD remain unclear. Approximately 30 % of the causes are believed to be genetic mutations and chromosomal abnormalities. In this study, we aimed to identify the genetic causes of CHD in consanguineous families. MethodsFourth-generation pedigrees with CHD were recruited. The main cardiac features of the patient included absence of the right pulmonary artery and a large dilated left pulmonary artery. To determine the underlying genetic cause, whole-exome sequencing was performed and subsequently confirmed using Sanger sequencing and different online databases to study the pathogenesis of the identified gene mutation. An in-silico homology model was created using the Alphafold homology model structure of GATA4 (AF-P43694-F1). The missense3D online program was used to evaluate the structural alterations. ResultsWe identified a deleterious mutation c.551T > C (p.Phe184Ser) in GATA4. GATA4 is a highly conserved zinc-finger transcription factor, and its continuous expression is essential for cardiogenesis during embryogenesis. The in-silico model suggested a compromised binding efficiency with other proteins. Several variant interpretation algorithms indicated that the F184S missense variant in GATA4 is damaging, whereas HOPE analysis indicated the functional impairment of DNA binding of transcription factors and zinc-ion binding activities of GATA4. ConclusionThe variant identified in GATA4 appears to cause recessive CHD in the family. In silico analysis suggested that this variant was damaging and caused multiple structural and functional aberrations. This study may support prenatal screening of the fetus in this family to prevent diseases in new generations.

A shape-supervised feature fusion U-Net for tubular structure segmentation

Accurate segmentation of tubular structures, such as blood vessels and bile ducts, is pivotal for clinical diagnosis and subsequent treatment. However, challenges arise from their unique structure and morphology. These structures are thin and elongated, and often exhibit complex branching patterns, making precise delineation difficult. Additionally, the intricate and often noisy backgrounds in medical images complicate the extraction of detailed and shape features. These challenges collectively hinder the effectiveness of automated segmentation methods. To address these issues, this paper introduces the shape-supervised feature fusion U-Net (SFU-Net), a novel approach that extends the traditional U-Net architecture. Our network incorporates a feature fusion module that effectively combines different semantic information in both the encoder and decoder paths, facilitating the capture of detailed geometric representations of tubular structures. Furthermore, a novel distance-based tubularity loss is designed to capture boundary information and supervise segmentation integrity by imposing shape constraints. We conducted comprehensive experiments on three tubular structure segmentation tasks in medical imaging, including the dilated biliary tree, hepatic vessel, and pulmonary artery. The experimental results illustrate that the proposed SFU-Net achieves the best performance in terms of accuracy and completeness, with Dice scores of 76.14%, 80.42%, and 83.79%, and HD95 values of 8.59 mm, 6.43 mm, and 4.31 mm across three datasets, respectively. Compared to traditional U-Net-based segmentation methods and other tubular segmentation approaches, our method exhibits superior performance, underscoring its effectiveness in medical image segmentation with tubular structures.

Living-donor lobar lung transplantation for pulmonary Langerhans cell histiocytosis complicated by extensive thrombi in central pulmonary arteries

BackgroundPulmonary Langerhans cell histiocytosis (PLCH) is a rare disorder characterized by the proliferation of Langerhans cells along the small airways, which causes nodular and cystic changes in the lung parenchyma. Lung transplantation can be a life-saving option for patients with severe respiratory failure or pulmonary hypertension. Herein, we present a case of successful lung transplantation in a patient with PLCH who developed unusually large thrombi in the central pulmonary artery.Case presentationA 47-year-old woman with 16-year history of PLCH with rapidly developing respiratory failure was admitted to our hospital for the evaluation of a lung transplant. Enhanced computed tomography revealed large thrombi in dilated central pulmonary arteries. Right heart catheterization revealed severe pulmonary hypertension, with a mean pulmonary artery pressure of 48 mmHg. The thrombi shrank markedly after 3 months of anticoagulation therapy. However, the respiratory status of the patient did not improve. We performed bilateral living-donor lobar lung transplantation with thrombectomy under extracorporeal membrane oxygenation for the remaining thrombi in the main pulmonary arteries. The dilated main pulmonary arteries of the recipient required direct plication for size mismatch. The patient survived in good condition for more than 2 years with no recurrence of thrombosis.ConclusionPreoperative anticoagulation therapy for massive thrombi in the pulmonary arteries was effective and led to safe lung transplantation.

Epidemiological analysis of pulmonary artery dilation prevalence in Moscow: automated computed tomography image analysis

Epidemiological analysis of pulmonary artery dilation prevalence in Moscow: automated computed tomography image analysis

Lung Involvement in Pulmonary Vasculitis: A Radiological Review.

Pulmonary vasculitis identifies a heterogeneous group of diseases characterized by inflammation, damage and necrosis of the wall of pulmonary vessels. The most common approach to classify vasculitis is according to etiology, therefore dividing them into primary and secondary, with a further sub-classification of primary vasculitis based on the size of the affected vessels (large, medium, and small). Pulmonary involvement is frequently observed in patients with systemic vasculitis and radiological presentation is not pathognomonic, but may vary between diseases. The main findings using high-resolution computed tomography (HRCT) include small vessel wall thickening, nodular lesions, cavitary lesions, reticular opacities, ground-glass opacities (GGO), consolidations, interlobular septal thickening, tracheobronchial stenosis, and aneurysmal dilatation of pulmonary arteries, with or without pleural effusion. Radiological diagnosis alone is difficult since signs and symptoms of lung vessel involvement are often non-specific and might overlap with other conditions such as infections, connective tissue diseases and neoplasms. Therefore, the aim of this review is to describe the most common radiological features of lung involvement in pulmonary vasculitis so that, alongside detailed clinical history and laboratory tests, a prompt diagnosis can be performed.

Chronic thromboembolic disease among patients undergoing surgical pulmonary embolectomy for acute pulmonary embolism.

This study aimed to assess the prevalence of chronic thromboembolic lesions in the pulmonary arteries among patients undergoing pulmonary embolectomy for acute pulmonary embolism and their impact on treatment outcomes. We conducted a retrospective, single-center analysis of consecutive patients undergoing emergency pulmonary embolectomy for acute pulmonary embolism between 2013 and August 2021. According to European Society of Cardiology guidelines, the diagnosis was based on clinical presentation, imaging studies and laboratory tests. Surgery was selected as the optimal treatment modality within the Pulmonary Embolism Response Team. Based on the intraoperatively identified chronic lesions patients were divided into two groups: acute only and acute/chronic. The analysis comprised history, laboratory and imaging studies, early and long-term mortality, and postoperative complications. We determined predictive factors for chronic thromboembolic lesions and risk factors for death. The analysis included 33 patients. Intraoperatively, 42% (14) of patients had chronic lesions. Predictive factors for these lesions are the duration of symptoms >1 week (OR=13.75), pulmonary artery dilatation >3.15 cm (OR=39.00) and right ventricle systolic pressure >52 mmHg (OR=29.33). No hospital deaths occurred in the acute only group and two in the acute/chronic group (0% vs. 14.3%; P=0.172). Risk factors for death are the duration of symptoms >3 weeks (HR=7.35) and postoperative use of extracorporeal membrane oxygenation (HR=7.04). Acute thromboembolic disease overlapping chronic clots is relatively common among patients undergoing pulmonary artery embolectomy. A detailed evaluation of the patient's medical history and imaging studies can identify these patients, as they require special attention when making treatment decisions. Surgical treatment in a center of expertise in pulmonary endarterectomy seems reasonable.

Quadricuspid pulmonary valve: A rare cause of pulmonary artery aneurysm.

A 17-year-old male presented to our hospital from an external center with complaints of chest pain and shortness of breath, referred with preliminary diagnoses of pulmonary stenosis and pulmonary artery dilation. Echocardiography revealed moderate pulmonary regurgitation and mild pulmonary stenosis. Coronary computed tomography (CT) angiography showed normal coronary arteries and no presence of a patent ductus arteriosus, but an aneurysmal dilation of up to 4 cm was observed in the pulmonary artery. The patient was found to have a quadricuspid (QPV) pulmonary valve, while the aortic valve appeared normal. No phenotypic anomalies or accompanying aortic or lung abnormalities were detected in this case. Our case represents the first in the pediatric age group diagnosed via CT scan. Our study contributes insights into the relationship between QPV anomaly and pulmonary artery aneurysm, aiding in the diagnosis and management of these rare conditions. Furthermore, our findings highlight the importance of evaluating the possibility of QPV in patients with pulmonary valve stenosis, regurgitation, or artery aneurysm, emphasizing the significance of coronary CT angiography in the diagnostic process.

A CASE OF PULMONARY HYPERTENSION ASSOCIATED WITH MYELOFIBROSIS: THERAPEUTICAL CHALLENGES FROM ACUTE TO CHRONIC SETTING

Abstract A 85–year–old female was transferred to our ICCU for acute heart failure from one of our spoke hospitals. The patient had a long history of chronic myelofibrosis, treated with fedratinib as a second–line agent after ruxolitinib failure. During a recent hospital admission, transthoracic echocardiography already showed signs of severe pulmonary hypertension (PH) with right ventricular (RV) dysfunction, so diuretic was started. On admission the patient presented showed consistent with acute on chronic kidney injury (serum Cr 3.2 mg/dl), stable anemia (Hb 9.2 mg/dl) and markedly elevated Nt–proBNP. A pulmonary CT–scan excluded acute pulmonary embolism and lung parenchymal alterations, with a clear dilation of main pulmonary artery. Transthoracic echocardiography (figure 1) confirmed precapillary PH phenotype diagnosis. Electrocardiogram (figure 2) showed sinus rhythm; incomplete RBBB, T wave inversion in V2 to V4. The patient was initially treated with high dose furosemide, low flow oxygen; but later she needed multiple cycles of inotropes, vasopressors and combined diuretic therapy (metolazone and acetazolamide) to stabilize the clinical setting. The patient also experienced episodes of paroxysmal high ventricular rate atrial flutter. After resolution of volume overload, a right heart catheterization was performed and findings were consistent with severe precapillary pulmonary hypertension: PA pressure 90/58/40 mmHg, normal PCWP, high PVR, mild cardiac index reduction. Due to critical clinical situation, Sildenafil 20 mg was started as an off–label therapy for PH (group 5). Subsequently, the patient experienced gradual but constant hemodynamic and clinical improvement and was discharged in NYHA class II. At 1 month control, patient was stable, echocardiogram revealed improvement in right ventricle dimension, systolic function and right atrial enlargement. At three months, patient is still in an intermediate risk class for PH, so we are evaluating a combination therapy (either endothelin receptor antagonist or prostanoids), taking into account anemia and thrombocytopenia. Take home message: PH can be a complication of hematological disorders as myelofibrosis, included in “mixed” forms (group 5) of PH. In our patient, after a challenging management for acute right heart failure, an off–label PDE–5 inhibitor stabilized the clinical scenario: more data are needed on PH drugs use in this setting where up to now therapeutic strategies are lacking.

Three-Dimensional Rotational Angiography as Guidance for Percutaneous Patent Ductus Arteriosus (PDA) Device Closure: A Case Report

Background: Patent Ductus Arteriosus (PDA) is a prevalent congenital heart defect characterized by the persistence of an open arterial connection between the aorta and the pulmonary. Percutaneous PDA closure involves deploying occlusion devices through catheter- based procedures. Case Presentation: We report a 1-year and 6-month-old girl diagnosed with Patent Ductus Arteriosus, presented with persistent complaints of failed to thrive , shortness of breath and recurrent cough. Physical examination revealed a continuous murmur at the upper left sternal border, and the Echocardiography examination confirmed a PDA with size of 4-7 mm, with diastolic shortening and dilatation of the Main Pulmonary Artery (MPA). The successful percutaneous PDA device closure procedure was performed using Three-Dimensional Rotational Angiography (3DRA), following an attempt with Two-Dimensional Angiography, which failed to determine the actual defect size in the patient. Conclusions: Confirming the morphology and size of a PDA can be difficult due to its location between the aorta and pulmonary blood vessels, making it a challenging congenital anomaly to assess accurately. 3DRA precisely delineates the PDA structure located between the aortic and pulmonary blood vessels and acts as a guidance tool for percutaneous PDA device closure.

Understanding the Mechanisms of Main Bronchial Compression in Patients with Intracardiac Anomalies

BackgroundThe study focuses on vascular compression of the main bronchus in the aortopulmonary space, examining potential contributors within the same axial plane. Its goal is to uncover mechanisms of bronchial compression in patients with intracardiac anomalies and review surgical outcomes, aiming to enhance future results. MethodsThe morphology and topology of structures within the axial plane of the aortopulmonary space were objectively analyzed, including the sternum, ascending aorta, heart, pulmonary artery, descending aorta, and other relevant elements. Identified deviations from the normal configuration were systematically identified. Operative procedures included mobilizing and removing the compressing vessel, followed by suspending the airway wall to a rigid prosthesis (external stenting), vertebra, or ascending aorta. ResultsComputed tomography revealed potential factors contributing to bronchial stenosis, including anteriorly deviated descending aorta (20 patients), dilated pulmonary artery (6), cardiomegaly (12), flat chest (7), funnel chest (3), posteriorly deviated ascending aorta after arterial switch operation (3), low aortic arch (3), and aberrant subclavian artery (2). Kaplan-Meier analysis demonstrated operative survival rates of 96% at 1 year, 87% at 5 years, and 80% at 8-15 years. Ten-year follow-up computed tomography after external stenting procedure revealed the narrowest diameter of the stented bronchus as 94.4% of the reference. ConclusionsConsistent long-term airway patency was observed post-surgery. While the pulmonary artery and descending aorta exert direct compressive effects in most cases, various other potential mechanisms may contribute to bronchial compression. Identifying and addressing these factors through a multidisciplinary approach is crucial for sustaining bronchial patency and preventing complications.

A Case Report: A Successful Management of Extrinsic Compression of the Left Main Coronary Artery by a Dilated Pulmonary Artery in Patients with Pulmonary Arterial Hypertension

Extrinsic compression of the left main coronary artery (LMCA) by a dilated pulmonary artery can be life threatening in pulmonary arterial hypertension patients; however, it can be treated with percutaneous coronary intervention. In this case report, we presented a successful treatment with the stenting of the LMCA stenosis associated with extrinsic compression of LMCA by a dilated pulmonary artery.

Left main coronary artery compression in precapillary pulmonary hypertension.

Pulmonary hypertension (PH) is a progressive and invalidating condition despite available therapy. Addressing complications such as left main coronary artery compression (LMCo) due to the dilated pulmonary artery (PA) may improve symptoms and survival. Nevertheless, clear recommendations are lacking. The aim of this study is to analyze the prevalence, characteristics, predictive factors and impact of LMCo in a heterogenous precapillary PH population in a single referral center. Two hundred sixty-five adults with various etiologies of precapillary PH at catheterization were reviewed. Coronary angiography (CA) was performed for LMCo suspicion. Revascularization was performed in selected cases. Outcomes were assessed at a mean follow-up of 3.9 years. LMCo was suspected in 125 patients and confirmed in 39 (31.2%), of whom 21 (16.8%) had 50%-90% stenoses. Nine revascularizations were performed, with clinical improvement. The only periprocedural complication was a stent migration. LMCo was associated with PH etiology (p 0.003), occuring more frequently in congenital heart disease-associated PH (61.5% of all LMCo cases, 66.6% of LMCo ≥ 50%). Predictors of LMCo ≥50% were PA ≥ 37.5 mm (Sn 81%, Sp 74%) and PA-to-aorta ≥1.24 (Sn 81%, Sp 69%), with increased discrimination when considering RV end-diastolic area. LMCo ≥ 50% without revascularization presented clinical deterioration and worse survival (p 0.019). This analysis of a heterogeneous pre-capillary PH population provides LMCo prevalence estimation, predictive factors (PA size, PA-to-aorta, RV end-diastolic area and PH etiology) and long-term impact. While LMCo impact on survival is inconclusive, untreated LMCo ≥ 50% has worse prognosis. LMCo revascularization may be performed safely and with good outcomes.

The Mechanical Cause of Myocardial Infarction in Eisenmenger Syndrome: Unidimensional Left Main Compression

Eisenmenger syndrome may rarely present as acute coronary syndrome due to extrinsic left main compression by the dilated and hypertensive pulmonary artery. Though computed tomography (CT scan) has been the investigation of choice for diagnosis of this entity, high-definition intravascular ultrasound (HD-IVUS) may play a crucial role in diagnosis especially in sick patients with a characteristic finding of a “slit-like” ostia. Treatment of this “left main compression syndrome” with a drug eluting stent of high radial strength, evidenced angiographically as well as by HD-IVUS makes percutaneous coronary intervention (PCI) a preferred strategy to coronary artery bypass graft (CABG) in such high-risk patients.

Significance of Pulmonary Artery Dilatation in Lung Cancer Patients With Chronic Obstructive Pulmonary Disease Who Underwent Pulmonary Resection

BackgroundThe significance of pulmonary artery (PA) diameter in patients with non-small cell lung cancer (NSCLC) and chronic obstructive pulmonary disease (COPD) who undergo pulmonary resection has not been elucidated. MethodsData of 357 patients with NSCLC and COPD who underwent pulmonary resection were retrospectively reviewed. The main PA diameter, determined by preoperative computed tomography, relative to the body surface area (PBR), was used as an index of PA dilatation, and patients were divided into 2 groups using median values. The relationship between the PBR and short- and long-term outcomes was also analyzed. ResultsThe mean age was 70.8 years, and 82% of the patients were men. The median main PA diameter was 24 mm (range, 17-43 mm), and the median PBR was 14.5 (range, 10.4-28.6). Lobectomy or more was performed in 276 patients (78%) and sublobar resection in 81 patients (22%). The postoperative complication rates did not differ between the low- and high-PBR groups (33% vs 32%, P = .91). The relapse-free survival (RFS) and overall survival (OS) rates of the low-PBR group were significantly better than those of the high-PBR group (5-year RFS: 76% vs 59%, P = .0003; 5-year OS: 88% vs 72%, P = .0010). A multivariable analysis identified high PBR as a poor prognostic factor for both RFS and OS. ConclusionsPA dilatation was associated with poor long-term outcomes and was an independent poor prognostic factor for both RFS and OS in NSCLC patients with COPD who underwent pulmonary resection.

Vessel and Airway Characteristics in One-Year Computed Tomography-defined Rapid Emphysema Progression: SPIROMICS.

Rationale: Rates of emphysema progression vary in chronic obstructive pulmonary disease (COPD), and the relationships with vascular and airway pathophysiology remain unclear. Objectives: We sought to determine if indices of peripheral (segmental and beyond) pulmonary arterial dilation measured on computed tomography (CT) are associated with a 1-year index of emphysema (EI; percentage of voxels <-950 Hounsfield units) progression. Methods: Five hundred ninety-nine former and never-smokers (Global Initiative for Chronic Obstructive Lung Disease stages 0-3) were evaluated from the SPIROMICS (Subpopulations and Intermediate Outcome Measures in COPD Study) cohort: rapid emphysema progressors (RPs; n = 188, 1-year ΔEI > 1%), nonprogressors (n = 301, 1-year ΔEI ± 0.5%), and never-smokers (n = 110). Segmental pulmonary arterial cross-sectional areas were standardized to associated airway luminal areas (segmental pulmonary artery-to-airway ratio [PAARseg]). Full-inspiratory CT scan-derived total (arteries and veins) pulmonary vascular volume (TPVV) was compared with small vessel volume (radius smaller than 0.75 mm). Ratios of airway to lung volume (an index of dysanapsis and COPD risk) were compared with ratios of TPVV to lung volume. Results: Compared with nonprogressors, RPs exhibited significantly larger PAARseg (0.73 ± 0.29 vs. 0.67 ± 0.23; P = 0.001), lower ratios of TPVV to lung volume (3.21 ± 0.42% vs. 3.48 ± 0.38%; P = 5.0 × 10-12), lower ratios of airway to lung volume (0.031 ± 0.003 vs. 0.034 ± 0.004; P = 6.1 × 10-13), and larger ratios of small vessel volume to TPVV (37.91 ± 4.26% vs. 35.53 ± 4.89%; P = 1.9 × 10-7). In adjusted analyses, an increment of 1 standard deviation in PAARseg was associated with a 98.4% higher rate of severe exacerbations (95% confidence interval, 29-206%; P = 0.002) and 79.3% higher odds of being in the RP group (95% confidence interval, 24-157%; P = 0.001). At 2-year follow-up, the CT-defined RP group demonstrated a significant decline in postbronchodilator percentage predicted forced expiratory volume in 1 second. Conclusions: Rapid one-year progression of emphysema was associated with indices indicative of higher peripheral pulmonary vascular resistance and a possible role played by pulmonary vascular-airway dysanapsis.

Pulmonary Artery Dilatation Due to Pressure or Volume Overload in Congenital Heart Disease.

Background: Pulmonary artery dilatation is described mostly in association with pulmonary hypertension. Patients/Methods: Study analysis: 60 patients with pulmonary arterial hypertension in congenital heart disease (PAH-CHD); 64 with repaired tetralogy of Fallot/pulmonary regurgitation (rTOF/PR); and 80 healthy (NORMAL). Measured were: main pulmonary artery (MPA) diameter and MPA/ascending aorta (Ao asc) ratio, by echocardiography (ECHO) and computer tomography or magnetic resonance imaging (CT/MRI). Results: In MPA diameter, significant differences between PAH-CHD, rTOF/PR, and NORMAL were found (median): 37 vs. 27 vs. 21 mm (p < 0.0001). In MPA/Ao asc ratio, there was a difference between PAH-CHD and NORMAL (median): 1.3 vs. 0.8 (p < 0.0001), but not between rTOF/PR and NORMAL: 0.74 vs. 0.8 (p = 0.3). Significant MPA dilatation (>40 mm) was present: in PAH-CHD, 35% (ECHO) and 76.9% (CT/MRI) of patients, while in rTOF/PR, 3.1% (ECHO) and 7.8% (CT/MRI). Severe MPA dilatation (>50 mm) occurred only in PAH-CHD: 16.7% (ECHO) and 31.4% (CT/MRI), while not in rTOF/PR. There was a significant correlation between ECHO and CT/MRI measurements, but ECHO was underestimated in all parameters. Conclusions: MPA dilatation due to pressure overload is more frequent and more severe; volume overload also leads to MPA dilatation but is less severe. The MPA/Ao asc ratio is not reliable for MPA dilatation estimation in rTOF/PR.