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A 28-year-old African American male with history of intermittent headaches presented to an outside hospital (OSH) with few days history of severe right lower extremity pain, swelling and dyspnea. Lower extremity venous ultrasound revealed a large occlusive deep vein thrombosis (DVT) extending from the right common femoral to the right popliteal vein. Chest Computed tomography angiogram (CT Angiogram) revealed a 5.9 cm mid ascending thoracic aortic aneurysm, a 3.2 cm pulmonary artery dilatation and no evidence of pulmonary embolus. He was started on anticoagulation with intravenous heparin and transferred to our institution for surgical management of the aortic aneurysm and evaluation for large vessel vasculitis.

Objectives The aim of this study was to evaluate the clinical impact of computed tomography (CT)–derived pulmonary artery dilatation (PAD) in patients undergoing transcatheter aortic valve replacement (TAVR). Background Several studies have reported an association between pulmonary hypertension and cardiovascular events, but the prognostic value of PAD in patients undergoing TAVR remains unclear. Methods The preprocedural computed tomographic studies of patients who underwent TAVR at Cedars-Sinai Medical Center between November 2013 and December 2017 were analyzed. Patients were divided into 2 groups according to the presence of PAD assessed on CT (pulmonary artery [PA] diameter ≥29 mm). The primary endpoint was all-cause mortality at 2 years. Results A total of 895 patients were included (mean age 81.3 ± 8.5 years), with a mean Society of Thoracic Surgeons score of 4.8%. The median PA diameter was 28.0 mm, and PAD was observed in 369 patients (41.2%). Compared with the no-PAD group, the PAD group had higher Society of Thoracic Surgeons scores and higher rates of atrial fibrillation, chronic kidney disease, and chronic obstructive pulmonary disease. The PAD group had higher 2-year all-cause mortality than the no-PAD group (28.9% vs 12.8%; P 36 mm Hg). Conclusions CT-derived PAD is a significant prognostic factor in patients undergoing TAVR.

Background Pulmonary artery (PA) dilatation is often seen in pulmonary hypertension (PH) and is considered a long-term consequence of elevated pressure. The PA dilates over time and therefore may reflect disease severity and duration. Survival is related to the stage of the disease at the time of diagnosis and therefore PA diameter might be used to predict prognosis. This study evaluates the outcome of patients with pulmonary arterial hypertension (PAH) and chronic thrombo-embolic pulmonary hypertension (CTEPH) and investigates whether PA diameter at the time of diagnosis is associated with mortality. Methods Patients visiting an outpatient clinic of a tertiary centre between 2004 and 2018 with a cardiac catheterisation confirmed diagnosis of PAH or CTEPH and a CT scan available for PA diameter measurement were included. PA diameter and established predictors of survival were collected (New York Heart Association (NYHA) class, N‑terminal pro-brain natriuretic peptide (NT-proBNP) level and 6‑min walking distance (6MWD)). Results In total 217 patients were included (69% female, 71% NYHA class ≥III). During a median follow-up of 50 (22–92) months, 54% of the patients died. Overall survival was 87% at 1 year, 70% at 3 years and 58% at 5 years. The mean PA diameter was 34.2 ± 6.2 mm and was not significantly different among all the diagnosis groups. We found a weak correlation between PA diameter and mean PA pressure ( r = 0.23, p Conclusion The prognosis of PAH and CTEPH is still poor. Known predictors of survival were confirmed, but PA diameter at diagnosis was not associated with survival in PAH or CTEPH patients.

Purpose In vitro fertilization with intracytoplasmic sperm injection (IVF-ICSI) is generally regarded as an indication for fetal echocardiography due to a reported increased risk of congenital abnormalities including cardiac anomalies. In this study we evaluated the utility of fetal echocardiography after anatomic survey in an experienced center. Methods This was a retrospective case review of in vitro fertilization with intracytoplasmic sperm injection pregnancies who had echocardiography in our institution from January 1996 to October 2010. Results Records from 85 mothers and 110 fetuses were identified. During anatomic survey, six cardiac anomalies were identified, including four ventricular septal defects, one pulmonary-aortic disproportion, and one post-valvular pulmonary artery dilatation. At fetal echocardiography, two of the four ventricular septal defects were confirmed and an additional three were identified. The most common cardiac anomaly seen in our population of IVF-ICSI pregnancies was ventricular septal defect, which was identified in 3.6% of all cases in the neonatal period. Of ventricular septal defects identified in the prenatal period, 71% resolved before birth. Conclusion In experienced centers, a fetal echocardiography may not be necessary if the 3 vessels tracheal view of the heart is evaluated and the heart is evaluated carefully for a ventricular septal defect.

Pulmonary artery dilatation comprises a heterogeneous group of disorders. Early diagnosis is important as the presentation may be incidental, chronic, or acute and life threatening depending upon the etiology. Cross-sectional imaging plays an important role, with CT pulmonary angiography being regarded as the first line investigation in the evaluation of pulmonary artery pathologies. Moreover, effects of pulmonary artery lesions on proximal and distal circulation can also be ascertained with the detection of associated conditions. Special attention should also be given to the left main coronary artery and the trachea-bronchial tree as they may be extrinsically compressed by the dilated pulmonary artery. In context of an appropriate clinical background, CT pulmonary angiography also helps in treatment planning, prognostication, and follow-up of these patients. This review mainly deals with imaging evaluation of the pulmonary arterial dilatations on CT with emphasis on the gamut of etiologies in the adult as well as pediatric populations.

In clinical practice, the presence of wheezing generally indicates an airway disease. In rare circumstances, adjacent mediastinal structures may compress the tracheobronchial tree leading to obstructive physiology. Compression of the tracheobronchial region by an enlarged pulmonary artery (PA) is exceedingly rare. We present here a case of pulmonary hypertension, where the enlarged PA resulted in obstructive lung physiology with a relevant review of the literature.

Background Computed tomography pulmonary angiography (CTPA) is the gold standard for the diagnosis of pulmonary embolism (PE). However, contrast is contraindicated in some patients. The purpose of this study was to determine the diagnostic accuracy of unenhanced multidetector CT (MDCT) for diagnosis of central PE using CTPA as the gold standard. Methods The records of patients with suspected PE seen between 2010 and 2013 were retrospectively reviewed. Inclusion criteria were an acute, central PE confirmed by CTPA and non-enhanced MDCT before contrast injection. Patients with a PE ruled out by CTPA served as a control group. MDCT findings studied were high-attenuation emboli in pulmonary artery (PA), main PA dilatation > 33.2 mm, and peripheral wedge-shaped consolidation. Receiver operating characteristic (ROC) analysis was used to determine the sensitivity and specificity of unenhanced MDCT to detect PE. Wells score of all patients were calculated using data extracted from medical records prior to imaging analysis. Results Thirty-two patients with a PE confirmed by CTPA and 32 with a PE ruled out by CTPA were included. Among the three main MDCT findings, high-attenuation emboli in the PA showed best diagnostic performance (Sensitivity 72.9%; Specificity 100%), followed by main PA dilatation > 33.2 mm (sensitivity 46.9%; specificity 90.6%), and peripheral wedge-shaped consolidation (sensitivity 43.8%; specificity 78.1%). Given any one or more positive findings on unenhanced MDCT, the sensitivity was 96.9% and specificity was 71.9% for a diagnosis of PE in patients. The area under the curve (AUC) of a composite measure of unenhanced MDCT findings (0.909) was significantly higher than that of the Wells score (0.688), indicating unenhanced MDCT was reliable for detecting PE than Wells score. Conclusions Unenhanced MDCT is an alternative for the diagnosis of acute central PE when CTPA is not available.

Aortopulmonary window is a rare congenital heart defect. Left main coronary artery extrinsic compression by an enlarged pulmonary artery is a rare complication and a potential cause for chest pain and sudden cardiac death in patients with pulmonary hypertension. Here, we present the case of a 14-year-old boy with a large aortopulmonary window who was planned for a device closure, but during the procedure, he developed ST-T segment changes while the device was being deployed, and hence the procedure was abandoned. The boy subsequently underwent a successful surgical closure thereafter.

Background/Aim: Dilatation of the main pulmonary artery (mPA) is a common incidental finding in chest imaging and often leads to consultation. The aim of this study was to determine the prevalence of mPA dilatation in a coronary artery CT angiography (CCTA) population. Patients and Methods: The study investigated 985 consecutive patients scheduled for diagnostic CCTA. The transverse axial diameter of the mPA was measured. The prevalence of mPA dilatation was estimated using different reference values (Framingham Heart Study: 28.9 mm for males and 26.9 mm for females, Bozlar: 29.5 mm for both genders and Karazincir: 32.6 mm for males and 31.9 mm for females). Results: The patient mean age was 53.0±9.7 years (66.5% were women). Body surface area (BSA) correlated moderately with the mPA diameter (r=0.423, p

Aortopulmonary Window is a rare congenital heart defect comprising 0.1-0.2% of the total spectrum. Spontaneous closure of this defect is unknown and survival into childhood and adult life is rarely seen. Severe pulmonary artery hypertension develops and causes dilatation of the pulmonary artery. Left main coronary artery extrinsic compression by an enlarged pulmonary artery is a rare complication and a potential cause for chest pain and sudden cardiac death in patients with pulmonary hypertension.

CITATIONSimonson J, Greenberg H, Talwar A. Pulmonary artery dilatation and obstructive sleep apnea. J Clin Sleep Med. 2020;16(4):647.

Idiopathic pulmonary hypertension (PH) is a rare disease defined by the mean artery pulmonary pressure (mPAP) of at least 25 mmHg, pulmonary artery wedge pressure ≤ 15 mmHg and a pulmonary vascular resistance > 3 Wood units in the absence of other causes of precapillary pulmonary hypertension. Pulmonary artery (PA) dilation is a common finding in PH, but only a small proportion of patients with PH had a diameter >40 mm. We present a patient with a severe PA dilatation already at the time of diagnosis.

Right ventricular failure is a leading cause of mortality in patients with pulmonary arterial hypertension (PAH). However, up to 25% of such patients die unexpectedly, without warning signs of hemodynamical decompensation. We previously documented that pulmonary artery (PA) dilatation significantly increases the risk of those deaths. Some of them may be due to dissection of PA resulting in cardiac tamponade. However, direct confirmation of this mechanism is difficult as most of such deaths occur outside hospitals. We present 4 patients with severe PAH and PA dilatation in whom PA dissection has been confirmed. Three patients had IPAH, one had PAH associated with congenital heart disease. All patients had mean pulmonary artery pressure (PAP) > 50 mmHg at diagnosis and dissection occurred late in the course of apparently well controlled disease (6 to 14 years). Several clinical elements were common to our patients - high systolic PAP, long lasting PH, progressive dilatation of PA to more than 50 mm with chest pain prior to dissection. However, clinical course followed three different patterns: sudden death due to cardiac tamponade, hemopericarditis caused by blood leaking from dissected aneurysm with imminent but not immediate cardiac tamponade, or chronic asymptomatic PA dissection. Indeed, two of our patients are alive and on lung transplantation waiting list for more than 2 years now. Further research is needed to suggest optimal management strategies for patients with stable PAH but significantly dilated proximal pulmonary arteries or confirmed PA dissection depending on the clinical presentation and expected outcome.

An 18-month-old female Holstein Friesian heifer presented with a history of stunted growth and a recent onset of anorexia; she presented with cyanosis and eventually died. A postmortem examination revealed obstruction of the right ventricular outflow tract, ventricular septal defect, overriding aortic root, right ventricular hypertrophy, and an atrial septal defect, indicating a pentalogy of Fallot (POF). In addition to POF, the heifer also had pulmonary artery dilatation, although she did not present with patent ductus arteriosus. This heifer had the longest lifespan among the Holstein cattle reported to have POF, which may be secondary to delayed pulmonary obstruction due to deformation of one of the pulmonary valves.

Pulmonary artery dissection is a rare and extremely dangerous disease with high mortality rates. It is one of the most serious complications of chronic pulmonary hypertension. It may be related to chronic pulmonary hypertension and pulmonary artery dilatation. Early diagnosis of pulmonary dissection is particularly important because of its high mortality. Once the symptoms worsen or severe deterioration of the disease occurs, imaging examination should be performed promptly for early diagnosis and timely treatment.

Idiopathic dilatation of the pulmonary artery (IDPA) is a rare congenital heart disease. It has been described for almost one hundred years, and numerous definitions have been proposed. The IDPA diagnostic criteria have not been updated for years. Secondary to primary disease, pulmonary artery aneurism was recognised as a lethal defect; however, long-term follow-up of patients with IDPA has not been well researched. Thus, indications to medical or surgical treatment are not evidence based. Here, we present a rare case of a 54-year-old patient with IDPA, who remained under observation for 36 years without surgical intervention.

Background Pulmonary artery (PA) dilatation is usually observed in patients with pulmonary hypertension (PH), but a PA aneurysm (PA diameter > 40 mm) is rare. The difference between characteristics of patients with and those without progression of PA diameter remains poorly understood. We assessed the changes in PA diameter in patients with PH coexisting with and without a PA aneurysm. Methods We investigated the changes in PA diameter by multi-detector computed tomography performed twice with an interval of more than one year in 44 patients with PH. Seventeen patients had a PA aneurysm and 27 patients did not have a PA aneurysm at baseline. Results The median follow-up period was 3.6 years. All patients received medical or invasive treatment for PH. At baseline, main PA diameters were 52 ± 15 mm in patients with a PA aneurysm and 33 ± 3 mm in patients without a PA aneurysm. Mean PA pressure was higher in patients with a PA aneurysm than in those without a PA aneurysm (61 ± 15 mmHg vs. 51 ± 16 mmHg, p = 0.04). At follow-up, mean PA pressure significantly decreased in both patients with a PA aneurysm (44 ± 11 mmHg) and patients without a PA aneurysm (41 ± 18 mmHg). Main PA diameter significantly increased in patients with a PA aneurysm (65 ± 28 mm, change ratio: 23.3%), while it did not increase in patients without a PA aneurysm (32 ± 3 mm, change ratio: −3.1%). Conclusions PA dilatation progressed in patients with a PA aneurysm despite treatment of PH. The progression of PA dilatation is independent of reduction of PA pressure by PH treatment.

Extrinsic compression of the left main coronary artery (LMCA) secondary to pulmonary artery dilatation is a rare syndrome. Most cases of pulmonary artery hypertension but no atherosclerotic risk factors rarely undergo coronary angiography, and hence, diagnoses are seldom made and proper management is often delayed in these patients. We describe a patient that presented with pulmonary hypertension, clinical angina, and extrinsic compression of the LMCA by the pulmonary artery, who was treated successfully by percutaneous coronary intervention. Follow-up coronary angiography showed patent stent in the LMCA in the proximity of the dilated main pulmonary artery. This case reminds us that coronary angiography and percutaneous coronary intervention should be considered in pulmonary hypertension patients presenting with angina or left ventricular dysfunction.

A bstract Background Tetralogy of Fallot and absent pulmonary valve (TOF/APV) is associated with significant pulmonary artery dilatation and airway compression. Methods We performed a retrospective review of 73 consecutive patients who underwent repair for TOF/APV between January 2005–August 2015. Mean age was 6.4 ± 5.6 years (28 days–22 years). The right ventricular outflow tract (RVOT) was reconstructed using varied techniques. Freedom from RVOT gradients and re-operation was studied. Results There were four (5.5%) early deaths, two each in infants and older children. Median ICU stay was 2 days (range, 1–12 days). Mean ICU stay for, infants, children and adults, was 6.5 ± 6.04, 2.75 ± 2.45, and 2.33 ± 1.03 days, respectively (p = 0.0762). Median hospital stay was 6 days (range, 4 to 15 days). Mean hospital stay for, infants and children and adults was 7 ± 2, 6.75 ± 2.39, and 6.33 ± 1.63 days, respectively (p = 0.325). Mean follow up was 65 ± 36.6 months (median 56 months, range 7–126 months). On follow up echocardiography, 14 (21.21%) had no pulmonary regurgitation. 21 (31.81%) had mild PR patients, 8 (12.12%) moderate PR and 19 (28.78%) had severe PR. There were five (7.5%) reoperations. Five and ten-year survival was 95% ± 2.12 and 92.3% ± 3.45 respectively. Freedom from RVOT reoperation was 93 ± 2.62% and 89 ± 3.87% at 5 and 10 years. Conclusions In contrast to children and adults with TOF/APV, infants carry significant early mortality. But the mid-term outcome for patients who survive the initial repair of TOF/APV is acceptable. However, these patients require constant surveillance and irrespective of the methods of RVOT management, the reoperation rates are expected to be high as more of these patients survive into adulthood.

Dilatation of the pulmonary artery may lead to the compression of adjacent structures. Of those, the extrinsic compression of the left main coronary artery is the most worrisome. We present the case of a 48-year-old woman who was diagnosed with pulmonary artery dilatation due to severe, thromboembolic pulmonary hypertension. She also had angina and coronary angiography revealed a 70% ostial stenosis of the left main coronary artery. The presence of this isolated lesion in a young woman without risk factors for atherosclerosis suggests extrinsic compression of the left main coronary artery by the dilated pulmonary artery as the likely mechanism. The patient underwent direct stenting of the left main coronary stenosis with a good result. J Cardiovasc Med 9:747–750 Q 2008 Italian Federation of Cardiology.

This chapter will discuss the auscultatory manifestations of pulmonary regurgitation (PR). PR can be caused by a variety of conditions (e.g. pulmonary hypertension, pulmonary valve trauma, and pulmonary artery dilatation), and may be difficult to differentiate from aortic regurgitation. The chapter will also discuss the prognostic implications of pulmonary regurgitation.

A doença de Behçet pode ser acompanhada de manifestações pulmonares, às vezes letais se não tratadas adequadamente. Descreve-se o caso de um homem de 53 anos com história de episódios de aftas orais e febre havia vários anos, dois quadros febris prolongados nos últimos dois anos, uveíte tratada com corticóide havia 21 meses, hemoptise havia um mês e emagrecimento de 7kg. Ao exame físico apresentava aftas orais, pseudofoliculite e turvação visual. Os estudos radiológicos evidenciaram dilatação aneurismática de artéria pulmonar. Tratado com corticóide, foi feita lobectomia inferior esquerda e o exame anatomopatológico foi compatível com arterite de artéria pulmonar. O paciente foi tratado com clorambucil por oito meses e mantém-se bem, decorridos 18 meses da cirurgia.

An 80 year old clergy, presented with a 5-day history of left-sided chest pain which was non-radiating but had cough productive of white sputum. No history of haemoptysis, dyspnoea, orthopnoea or paroxysmal nocturnal dyspnoea was elicited. However, a history of smoking 2 cigarettes per day for 10 years was present. He is a known hypertensive on medications,also being managed for diverticulosis and Benign Prostatic Hypertrophy.Respiratory rate was 26cycles per minute with crepitations in the left mid-and lower lung zones. Bilateral pedal oedema was found but the cardiovascular and gastrointestinal systems were normal. Requests for an ultrasound, a chest X-rayCXR and Contrast Enhanced computerised tomography CECT of the chest were made. High resolution CECT of the chest was performed with a 64-slice Toshiba CT. Multiplanar images in axial, coronal and sagittal planes were acquired at 1mm cuts in pre-and post-contrast series. Images depicting the abnormality are shown (A, B, C,D). No further invasive workup like a biopsy was indicated following imaging. Prior to the CECT, bronchogenic CA was considered in view of the long standing smoking history. The role of Computerised tomography in the evaluation of chest symptoms has been emphasised in these images.

雑誌掲載版, 65歳男.脳外科入院中に不整脈を指摘された.自覚症状はなく受診時には整脈であったが,第2肋間胸骨左縁にLevine II/VIの収縮期雑音とII音の固定性分裂を聴取し,胸部X線像上左第2弓の突出を認めた.心臓超音波検査では心房中隔欠損(ASD)と右心系の拡大を認め,胸部CT検査では左無名静脈から分岐した左異常肺静脈を認め,肺動脈幹は径50mm,左肺動脈は径36mmと拡張した.心臓カテーテル検査では主肺動脈圧は軽度高値のみで,右室と肺動脈間,両心房間に圧較差を認めた.カテーテルは下大静脈,右房,上大静脈,左無名静脈を経て左異常肺静脈に到達し,左異常肺静脈等で酸素飽和度の高値を示し,左肺動脈造影にて左異常肺静脈は左無名静脈に還流していた.左→右シャント率は32%,Qp/Qsは1.4であった.以上より部分肺静脈還流異常(PAPVC),ASD及び肺動脈弁狭窄(PS)と診断した.自覚症状がなく,不整脈も軽度であり明らかな右心不全を認めず,更にQp/Qsが1.4であることから経過観察とした

雑誌掲載版, 無症状で胸部X線像上著明な肺動脈拡張を呈し,その背景に標題の先天性心疾患が存在していた65歳男性の症例を経験した.著明な肺動脈拡張の原因を考察する上で興味深い症例と思われたので報告した

We report an aged female with a femoral fracture who developed acute pulmonary thromboembolism leading to pulmonary infarction. A 71-year-old woman came to our hospital complaining of right coxalgia that had persisted for one week. The chest x-ray findings showed an infiltrating shadow in the right middle lung field with prominent cardiomegaly. Enhanced chest CT scanning revealed consolidation of the same area and pulmonary artery dilatation, and lung perfusion scintigraphy showed a defect in the right middle lung field. Pulmonary angiography detected obliteration of right middle lobe arteries that seemed to have contributed to the development of pulmonary thromboembolism. These findings suggested pulmonary infarction secondary to pulmonary thromboembolism. Anti-coagulant and fiblinolytic therapy were performed. A recent chest film and perfusion scan 2 weeks later showed disappearance of the defect and scarring of the pulmonary infarct. PE caused by cardiac failure and obesity as the underlying disease for long time and hemoconcentration. The pathogenesis of pulmonary infarction may be caused by pulmonary congestion due to cardiac failure.

Pulmonary hypertension (PH) often leads to dilatation of the pulmonary artery (PA), which can be measured on chest computed tomography (CT). While the predictive capability of PA dilatation is useful to distinguish PH (mean PA pressure ≥25 mmHg) from normal (mean PA pressure ≤ 20 mmHg), CT characteristics of borderline PH (mean PA pressure 21-24 mmHg) have not been described. We aimed to investigate whether patients with borderline PH already show PA dilatation and to assess the diagnostic accuracy of PA dilatation for borderline PH diagnosis. Between April 2003 and September 2008, consecutive symptomatic patients with a mean PA pressure below 25 mmHg on right heart catheterization who had a chest CT available were retrospectively included. PA diameters from chest CT were correlated with hemodynamic measurements and analyzed with respect to their accuracy of predicting borderline PH. Main PA diameters were significantly larger in 26 patients with borderline PH compared with 52 patients without PH (3.16 ± 0.53 vs. 2.78 ± 0.43 cm, P = 0.001). The main PA diameter on CT correlated with mean PA pressure (r = 0.496, P < 0.001) and pulmonary vascular resistance (r = 0.445, P < 0.001), and predicted borderline PH with sensitivity, specificity, negative and positive predictive values of 77%, 62%, 84%, and 50%, respectively, using a cutoff ≥2.9 cm. This first systematic investigation of CT parameters in symptomatic patients with mean PA pressures less than 25 mmHg could show that, even in patients with borderline PH, significant PA dilatation can be present, which was related to PA pressure and pulmonary vascular resistance. This can be useful for identification of patients with borderline PH for further study and to prompt further diagnostic work-up of possible underlying diseases.

The left-main coronary artery extrinsic compression due to enlarged pulmonary artery has been described in several case series. Ortner's syndrome is also a rare condition in some cardiovascular disorders. There have been no reports about these two rare conditions in the same patient. Hence, we report a very rare case of an Eisenmenger patient with severe pulmonary hypertension and dilated pulmonary artery which has compressed the left main coronary artery, severely narrowing it, and the left laryngeal recurrent nerve with subsequent Ortner's syndrome and brief literature review.

Objectives: Pulmonary arterial dilatation is considered a consequence of chronic pulmonary hypertension (PH), but despite its relatively common detection, its prevalence and prognostic impact have not yet been systematically investigated. The aim of the study was to investigate these factors in a relatively large cohort of severe PH patients. Methods: One hundred and forty-one consecutive patients diagnosed with PH were monitored for a mean of 957 days. Data including functional class, exercise capacity, invasive hemodynamics and pulmonary artery (PA) echo/CT scan measurement were performed and outcomes prospectively collected. Results: PA dilatation is a common feature, present in the 76.6% of cases in this cohort of severe PH patients. Survival at 1, 2 and 3 years was 83, 71 and 58%, respectively. On univariate analysis, the baseline variables associated with a poor outcome were related to pulmonary arterial hypertension associated with connective tissue disease (CDT-PAH), New York Heart Association (NYHA) functional class, 6-min walk test and right atrial pressure. On multivariate analysis only CDT-PAH and NYHA functional class remained independently associated with poor survival. Conclusions: PA dilatation is commonly detected in severe PH patients and is not associated with an increased risk of death.

We describe an infant presenting with contractures of the fingers, a large ventricular septal defect (VSD), and severe pulmonary artery dilatation. He had clinical and echocardiographic features of both neonatal or infantile Marfan syndrome (MFS) and congenital contractural arachnodactyly. After surgical VSD closure, the aortic root developed progressive dilatation while the size of pulmonary artery returned to normal limits. Eventually the diagnosis of MFS was confirmed by DNA analysis.

A pulmonary artery aneurysm is defined as pulmonary artery dilatation >4 cm. Pulmonary artery aneurysms are rare findings and are usually not associated with chest pain. This is the first report of a case of pulmonary artery aneurysm causing chest pain in a patient with Noonan’s syndrome. Chest X-ray showed cardiomegaly and a prominent main pulmonary artery segment and CT confirmed a pulmonary artery aneurysm with a pulmonary artery root measuring 5.6 cm. The patient underwent pulmonary valvuloplasty as well as pulmonary arterioplasty to repair the pulmonary artery aneurysm, and experienced subsequent relief of chest pain. In conclusion, pulmonary artery aneurysm is a rare complication in patients with Noonan’s syndrome and can be the cause of chest pain in such patients.

Although several techniques for the correction of tetralogy of Fallot (TOF) with absent pulmonary valve have been described, the method of choice is controversial. Symptomatic infants, in particular, have a poor prognosis because of severe central pulmonary artery dilatation and bronchial compression. An alternative technique for primary repair of TOF with absent pulmonary valve syndrome is suggested. Apart from correction of TOF, this approach includes translocation of the pulmonary artery anterior to the aorta and away from the airways. This technique has a potential to reduce or eliminate bronchial compression by the pulmonary artery. Insertion of valve homograft with anterior and/or posterior plication of the pulmonary artery is considered especially in symptomatic newborns and infants. The intermediate-term functional outcomes have been encouraging, with zero mortality events even in the youngest age group of patients and with the disappearance of respiratory symptoms in the majority of them.

Background Pulmonary Regurgitation (PR) accompanying with pulmonary artery dilatation is one of the most common complications after pulmonary valve stenosis repair during the late follow up period. PR is well tolerated first, but later it becomes the reason of the right ventricular dilatation and the morbid effects of it. Decision in the time of performing PVR is vitally important because severe PR ends up with RV volume overload and PVR is shown to be capable in reducing it and ameliorating the systolic activity afterwards. On the other hand PVR should have been performed as late as possible because of its limited time of life.

Introduction: The use of crack cocaine in the United Kingdom has risen dramatically over the last decade, leading to an increase in serious respiratory complications, which can often go undiagnosed for years. Here we report a case of a 29-year-old male admitted to the emergency department with a left mandibular fracture and chest pain after a cocaine binge and subsequent assault. Case Report: An abnormal chest radiograph taken as part of a trauma series led to further investigations elucidating the eventual diagnosis of pulmonary artery dilatation likely secondary to cocaine. Conclusion: To our knowledge, this is the first case reporting pulmonary artery dilatation secondary to cocaine in the absence of pulmonary hypertension. The patient was treated conservatively with outpatient right-heart catheterization and serial transthoracic echocardiography to monitor for emerging pulmonary hypertension. We briefly discuss pulmonary artery dilatation and the effects of cocaine on the pulmonary vasculature.

A 70-year-old male diagnosed with idiopathic pulmonary artery dilatation at 23. The patient was asymptomatic until the age of 68, when he started with exertional dyspnoea and was found to have a chronic pulmonary artery dissection with …

A 31-year-old female presented with tricuspid valve insufficiency, atrial septal defect, pulmonary valve stenosis, and corrected transposition of the great arteries with situs inversus. She underwent successful ring annuloplasty of the tricuspid valve, pericardial patch repair of the atrial septal defect, pulmonary commissurotomy, and correction of poststenotic pulmonary artery dilatation.

Contrast-enhanced dynamic computed tomography (CT) with table incrementation between scans was used to evaluate the hila in 100 patients without hilar abnormality and 15 patients with hilar pathology. This method of hilar evaluation was found to be superior to conventional CT because hilar vascular structures could be clearly opacified. Opacification of normal hilar vessels permitted accurate detection of hypovascular hilar masses, some of which were very small, nonlobular, and invisible using conventional CT. The technique was also found to be useful in evaluating the hila in patients with pulmonary artery dilatation or pulmonary opacification contiguous with hilar borders. We recommend that contrast-enhanced dynamic incremental CT be used for hilar investigation in patients referred for hilar CT examination unless there is a specific contraindication to contrast medium administration.

Atrial septal defect is associated with increased diameter and pulsation of the pulmonary arteries. The high pulmonary flow rate produces turbulence and a murmur. Intraluminal pressure is normal and vessel wall elastic properties may be altered as in poststenotic dilatation. Pulmonary artery angiograms in 6 normal patients, 9 with pulmonary stenosis and 10 with atrial septal defect were recorded on film, projected and traced. Arterial diameters at maximum systole and diastole were measured and, with intraluminal pressures, 2 points on the elastic diagram were calculated. These points lay on the initial linear portion of the graph so that a line through them to zero wall tension gave the resting radius (R o ) Each radius value (R) was normalized by dividing by the corresponding R 0 . By plotting wall tension versus strain (R/R 0 ), a regression line was obtained for normals significantly different from that for poststenotic dilatation ( P P 4 dyne/cm; poststenotic dilatation, 1.00 x 10 4 dyne/cm; and atrial septal defect, 0.89 x 10 4 dyne/cm. Increased pulmonary artery distensibility extends more peripherally in atrial septal defect, thus producing "hilar dance." A case of Marfan's syndrome showed similar changes.

The classic findings of a patent ductus arteriosus in a young individual are familiar to most physicians. However, it has been appreciated that several factors, principally pulmonary hypertension or pulmonary artery dilatation in the absence of appreciable hypertension, may significantly alter the classic image and lead to the erroneous diagnosis of ventricular septal defect. In 10 surgically proved cases a patent ductus arteriosus was the sole malformation and so closely simulated a septal defect that extracorporeal circulation was initially considered for each of the patients. Division of the ductus resulted in a complete cure in all 10 patients. Those findings of value in differential diagnosis are analyzed.