Atrial septal defect in older adults: atypical radiographic appearances

Atrial septal defect (ASD) is the most common congenital cardiac anomaly manifested in adulthood. Clinical and radiographic features are well defined in patients less than 30 years of age. In older patients, however, the clinical symptoms are often atypical, and the auscultatory findings may be misleading. The radiographic manifestations in older patients have not been well described. In the current study, of 70 patients over the age of 50 years with proved ASD, 21 (30%) had atypical radiographic features, including apparently normal vascularity, left atrial enlargement, pulmonary venous hypertension, and pulmonary edema. In a control group of 70 younger patients with ASD, only 5.7% had atypical findings. The development of pulmonary venous hypertension and pulmonary edema in older patients was associated with smaller defects and a higher prevalence of mitral valve disease, left ventricular dysfunction, and pulmonary arterial hypertension than seen in older patients with typical radiographic findings.     

Imaging of medical disease of the newborn lung.

Surfactant deficiency remains the most common and serious pulmonary affliction of premature infants. Exogenous surfactant and corticosteroid administration have had a major impact in improving survival and morbidity in this disease with consequent alterations in the clinical and radiographic course. Chronic lung disease, although still common, is usually less severe than previously, especially in larger infants. Significant therapeutic strides in treating persistent pulmonary hypertension with or without meconium aspiration have been the use of high-frequency ventilators, inhaled nitric oxide, and ECMO. Prompt recognition and treatment of infection, atelectasis, and air leak whether primary or secondary are important to overall outcome. Less common pulmonary conditions, such as lymphangiectasia, pulmonary hypoplasia, and alveolar proteinosis, may initially masquerade as more common lesions. Persistent pulmonary hypertension is often the initial diagnosis and part of the clinical problem. Attention must be paid to the clinical and radiographic appearance and excursion of the thoracic cage because deformities and malformations may be the cause of respiratory problems.     

Pulmonary venoocclusive disease: case report and review of the literature.

A fatal case of pulmonary venoocclusive disease in a 4-year-old boy is reported, with emphasis on the radiologic features of this condition. The disease is characterized by fibrous intimal obliteration of the lumina of small pulmonary veins and venules leading to severe secondary pulmonary arterial hypertension. The clinical picture is usually indistinguishable from that of other forms of pulmonary arterial hypertnesion, but radiologic features of postcapillary pulmonary hypertension, in conjunction with a normal venoocclusive disease. The diagnosis is definitively established by lung biopsy.     

High-resolution computed tomographic imaging and pathologic features of pulmonary veno-occlusive disease: a review of three patients

Pulmonary veno-occlusive disease (PVOD) is a rare and severe form of pulmonary hypertension that is often difficult to differentiate from primary pulmonary hypertension. Differentiating these two entities before medical treatment is critical, as therapy commonly indicated for patients with primary pulmonary hypertension can be harmful and even fatal in patients with PVOD. In the setting of known pulmonary hypertension, computed tomography findings that are highly suggestive of PVOD include extensive, patchy centrilobular ground-glass opacities, ill-defined nodular densities, and interlobular septal thickening. Definitive diagnosis requires lung biopsy, demonstrating fibrous obliteration of the pulmonary venules and small veins of the lobular septa, with secondary medial hypertrophy of the pulmonary arteries. The purpose of this article is to review reported radiographic clues to the diagnosis of PVOD, as well as to illustrate these high-resolution computed tomography findings along with pathologic correlation.     

Pulmonary arterial hypertension: value of perfusion scintigraphy

Records of 34 patients with established causes of pulmonary arterial hypertension were retrospectively reviewed. The ventilation-perfusion scans were blindly classified according to standardized criteria as normal or high, low, or intermediate probability of pulmonary embolism as the cause of pulmonary arterial hypertension. Twelve of 13 patients with primary pulmonary hypertension had normal or low-probability scans, but the perfusion pattern was not helpful in distinguishing between histologic subtypes. All eight patients with large-vessel thromboembolic hypertension had high-probability scans; however, three of 13 patients with nonembolic secondary pulmonary hypertension also had high-probability scans. While a normal or low-probability scan excluded proximal pulmonary emboli as a cause of pulmonary hypertension, a high-probability scan may be associated with a variety of other nonembolic causes of secondary pulmonary hypertension.     

Thoracic manifestations associated with advanced liver disease

Advanced liver disease and portal hypertension may produce various intrathoracic complications that involve the pleural space, lung parenchyma, and pulmonary circulation. Dyspnea and arterial hypoxemia are the common clinical symptoms and signs in patients with such complications. In these patients, intrathoracic complications most often develop during the course of hepatic disease, but a few patients may be seen first with respiratory symptoms or radiographic abnormalities. Therefore, radiologists should be made aware of these disorders that occur in patients with chronic liver disease. In this article, the authors describe and illustrate the clinical and imaging spectrum of thoracic abnormalities associated with advanced liver disease and portal hypertension.     

PVOD suggested by MDCT and clinical findings in a pregnant woman

Pulmonary hypertension secondary to pulmonary venoocclusive disease (PVOD) is increasingly recognized (Wagenvoort, Chest 69:82–86, [20]; Scully et al., N Engl J Med 308:823–834, [21]). The clinical presentation is usually progressive pulmonary hypertension. It should be kept in mind when there is pulmonary arterial hypertension, pulmonary edema, and a normal pulmonary artery wedge pressure. Importance of diagnosing this condition is to protect patient from fatal pulmonary edema when using prostacyclins that are effective for treatment of primary pulmonary hypertension. Herein, we present multidetector computed tomography findings of PVOD in a pregnant woman that presented with pulmonary hypertension.     

Pulmonary hypertension: Work in progress

Pulmonary arterial hypertension is a rare disorder defined by mean pulmonary artery pressures that exceed 25 mm Hg at rest or are greater than 30 mm Hg with exercise. The mortality rate is high for untreated patients, mainly as a result of progressive right heart dysfunction. Pulmonary arterial hypertension has been historically classified as primary pulmonary hypertension or pulmonary hypertension resulting from an underlying disease process. Ongoing research in the nuclear medicine field holds great promise for understanding the pathophysiologic pathways for this condition, as well as the monitoring of the continually evolving therapeutic options.     

Rupture of a solitary,erosive peripheral pulmonary artery aneurysm

Aneurysms of the pulmonary arteries and their branches are rare. Although they may occur as isolated congenital anomalies, they more frequently are associated with congenital heart disease and pulmonary arterial hypertension. Less common predisposing factors include primary pulmonary hypertension, collagen vascular disease, trauma, and pulmonary banding. Inflammatory or infectious contributing factors include chronic granulomatous infection, infective endocarditis, pneumonia, and the Hughes-Stovin and Behçet's syndromes. Chronic pulmonary arterial hypertension, due to an underlying left-to-right shunt, as well as a superimposed focal bacterial pneumonia were the etiologic factors in the development of the segmental pulmonary artery aneurysm in our patient. The rarity of this constellation of predisposing factors and the diagnostic dilemma posed by the findings in our patient led us to report our case and review the pertinent literature.     

先天性心脏病合并肺动脉高压分级及性质的评估

鉴别先天性心脏病合并肺动脉高压是动力型还是阻力型对治疗方案的选择和预后的评估均具重要意义。本文综述先天性心脏病合并肺动脉高压的临床分级、分期及肺动脉高压性质的判断,旨在为临床诊疗提供参考。     

Cardiac causes of pulmonary arterial hypertension: assessment with multidetector CT

The causes of pulmonary arterial hypertension (PAH) are diverse and include multiple congenital and acquired cardiac diseases as well as diseases primarily affecting the pulmonary vasculature, lung, pleura and chest wall. The traditional role of CT in evaluating PAH includes assessment of pulmonary vasculature and lung parenchyma with limited assessment of the heart. Advances in multidetector CT technology with improved spatial and temporal resolution now permit accurate delineation of cardiac morphology. CT pulmonary angiography (CTPA) is widely utilised in the workup of patients with suspected pulmonary vascular disease and can identify both pulmonary and cardiac causes. As the initial presentation for CTPA is often precipitated by nonspecific, unexplained symptoms and therefore undertaken by a general radiologist, it is important that a systematic approach to the interpretation of these studies, including cardiac evaluation, is routinely adopted. This paper reviews the CT evaluation in pulmonary hypertension with a particular focus on the cardiac causes, their subclassification into congenital systemic to pulmonary shunts and secondary to left heart disease, and their imaging features. It emphasises the use of a systematic approach to interpretation of CTPA examinations both in patients with known PAH and those with previously unsuspected disease.     

Eisenmenger syndrome and idiopathic pulmonary arterial hypertension: do parenchymal lung changes reflect aetiology?

AIM: To document the pulmonary vascular changes on thin-section computed tomography (CT) in patients with Eisenmenger syndrome and idiopathic pulmonary arterial hypertension, and to determine whether there is any correlation with pulmonary arterial pressures or the aetiology of pulmonary hypertension. MATERIAL AND METHODS: From the National Pulmonary Hypertension Database, we identified eight patients with idiopathic pulmonary arterial hypertension and 20 patients with Eisenmenger syndrome (secondary to a ventriculoseptal defect) who had also undergone contrast-enhanced thin-section CT. CT studies were reviewed for the presence of centrilobular nodules, mosaicism, neovascularity, and bronchial artery hypertrophy. Haemodynamic data were also reviewed. RESULTS: Centrilobular nodules, mosaicism, and neovascularity were seen in both patient groups (p>0.05). A significantly higher number of enlarged bronchial arteries were seen in patients with Eisenmenger syndrome. There was no correlation with pulmonary arterial pressures. CONCLUSION: Patients with idiopathic pulmonary arterial hypertension and Eisenmenger syndrome demonstrated similar pulmonary vascular changes on CT. These changes did not predict the underlying cause of pulmonary hypertension or its severity.     

The assessment of pulmonary arterial pressure by pulsed Doppler in patients with obstructive pneumopathy]

The severity of pulmonary arterial hypertension can be assessed by duplex-Doppler echocardiography, a subxiphoid approach and a general-purpose duplex device. Normally, the peak Doppler flow velocity occurs in midsystole and the flow profile is parabolic (bullet-like). In pulmonary arterial hypertension, changes in vascular compliance cause maximal acceleration of blood in early systole, with shortening of pulmonary acceleration time (AcT, or time to peak velocity). In the more severe cases, a midsystolic notching is visible, related to rapid deceleration of blood flow, followed by a brief secondary increase in velocity in the late systole. We studied 19 adult patients with chronic obstructive pulmonary disease with duplex-Doppler examination, with a subxiphoid approach and right heart catheterization. The study was diagnostic in all cases with Doppler recordings of good quality. An relationship was found between AcT and pulmonary mean or systolic arterial pressure at rest. An evident accurate prediction of pulmonary arterial pressure in emphysematous patients is possible by means of pulsed Doppler, also in case of low-level hypertension. We believe this method to be a simple and reliable adjunct to the non-invasive work-up of emphysematous patients and to represent a good alternative to the classical parasternal approach, which is often not feasible in these patients.     

Pulmonary artery dissection in long standing idiopathic pulmonary arterial hypertension: A case report

Pulmonary arterial dissection (PAD) is a rare and often lethal complication of chronic pulmonary arterial hypertension (PAH), which may occurs in patients with idiopathic pulmonary arterial hypertension (IPAH) and potentially in those with connective tissue disorders. While rare, sudden death often occurs secondary to acute cardiac tamponade, as the pulmonary artery dissects into the pericardium; this diagnosis is often made postmortem. Nevertheless, with the proliferation of multidetector computed tomography (MDCT) as a diagnostic test, patients may be identified very early after symptom onset, prompting rapid intervention with decreased morbidity and mortality. We report a case of IPAH complicated by pulmonary artery aneurysm (PAA) and PAD, diagnosed by CT pulmonary angiogram (CTPA), and treated with bilateral lung transplantation, pulmonic valve replacement, and re-anastomosis of the donor main PA to a pulmonary valve conduit.     

肺动脉造影对胸部疾病的诊断价值：（附12例分析）

本文报告12例肺动脉造影检查,其中先天性肺动脉发育异常者6例,原发性肺动脉高压4例,左上肺静脉瘤及白塞氏病各1例。胸片可见一侧肺不发育呈均匀致密影;一侧肺动脉不发育使肺体积变小;一侧肺动脉发育不良表现单侧透明肺;原发性肺动脉高压呈肺门动脉扩张;肺静脉瘤及白塞氏病形成肺门部圆形肿物。因缺少影像特征表现,仅根据胸片较难做出正确诊断。经肺动脉造影均可确诊。     

Assessment of pulmonary hypertension by CT and MR imaging

In the recent World Health Organization (WHO) classification the group of pulmonary arterial hypertension (PH) comprises the classic primary pulmonary hypertension and several conditions with definite or very high risk factors to develop pulmonary arterial hypertension. Therapeutic advances drive the need for a comprehensive pre-therapeutic evaluation for optimal treatment. Furthermore, follow-up examinations need to be performed to monitor changes in disease status and response to therapy. Up to now, the diagnostic imaging work-up of PH comprises mainly echocardiography, invasive right heart catheterization and ventilation/perfusion scintigraphy. Due to technical advances helical computed tomography (CT) and magnetic resonance imaging (MRI) became more important in the evaluation and for differential diagnosis of pulmonary arterial hypertension. Both modalities are reviewed and recommendations for clinical use are given.This work was supported by the German Research Council (DFG, FOR 474).     

The relationship between pulmonary artery pressure and pulmonary artery diameter in pulmonary hypertension

The pulmonary arteries dilate in response to many factors, principally increased pressure and flow. In patients who have pulmonary arterial hypertension but no increase in flow, we have compared main pulmonary artery size at computed tomography with pulmonary haemodynamic data obtained during right heart catheterisation. In patients with primary pulmonary hypertension and chronic thromboembolic pulmonary hypertension, dilatation correlated with raised pulmonary vascular resistance and reduced cardiac output but not with mean arterial pressure. In patients with chronic lung disease no correlations were shown though a trend between raised pressure and size was observed. We speculate that pulmonary artery compliance is an important factor which determines the degree of dilatation in response to raised pressure. Estimations of pressure cannot be made from measurements of pulmonary artery size without knowledge of the underlying lung disease.     

Review article: Effects of radiographic contrast media on the lung

The pulmonary adverse effects of intravascular use of water soluble radiographic contrast media (RCM) include bronchospasm, pulmonary oedema and increase in the pulmonary arterial blood pressure (Ppa). Symptomatic bronchospasm is rare but subclinical increase in airways resistance is common after intravascular injection of RCM. Experimental studies have demonstrated that the low osmolar ionic dimer ioxaglate can induce significant bronchospasm in comparison with other types of RCM. Histamine and endothelin, which are potent bronchoconstrictors and released in response to the administration of RCM, do not seem to mediate the bronchospastic effect of RCM. Pretreatment with corticosteroids or antihistamine does not appear to prevent RCM induced bronchospasm, but the administration of beta(2) adrenergic agonist can abolish this adverse effect. RCM induced pulmonary oedema can be secondary to endothelial injury causing an increase in the permeability of the microcirculation. It may also occur in patients with incipient cardiac failure, when large doses of RCM particularly of the high osmolar type are used. A rise in Ppa induced by RCM seems to be secondary to an increase in pulmonary vascular resistance through direct effects on the pulmonary circulation. Low osmolar non ionic monomers induce the least changes in the pulmonary circulation and should be the contrast media of choice for intravascular use in patients with pulmonary hypertension. The mechanisms responsible for the effects of RCM on airway resistance and pulmonary circulation remain unclear. Intrabronchial administration of high osmolar water soluble RCM is dangerous and can induce severe bronchial irritation and pulmonary oedema. Low osmolar RCM are well tolerated by the lungs following aspiration with minimal histological reaction.     

Chronic pulmonary embolism in children

Chronic pulmonary embolism with pulmonary hypertension in children is rarely diagnosed clinically; literature review yielded only 17 recorded cases. To demonstrate the radiographic features as well as to encourage the diagnostic consideration of chronic pulmonary embolism in children, this report focuses on three additional children with chronic pulmonary embolism. Of these 20 total cases, only two were not catheter-related; 17 patients had emboli as a complication of ventriculoatrial shunting, and one had emboli secondary to indwelling venous hyperalimentation. Analysis of the information available on the 20 cases revealed the following radiographic features: cardiomegaly (19 cases), large central pulmonary arteries with rapid distal tapering (15 cases), oligemia (five cases), "infiltrate" (three cases), and effusion (two cases). With increasing use of central catheterization as treatment for children with chronic illness, the incidence of chronic pulmonary embolism will likely increase; therefore, clinical diagnosis should reflect this increase. The radiologist in particular should be aware of the clinical and radiologic features of chronic pulmonary embolism in children.     

Pulmonary artery hypertension in chronic obstructive lung disease. The place of morphometric studies in thoracic radiography

Standard biplane chest X-rays were tested for the validity of morphometric criteria in the diagnosis of pulmonary artery hypertension. Twenty-seven patients suffering from chronic obstructive lung disease were examined and compared with a control group without cardiopulmonary disease. The diameter of the right and left pulmonary artery, pulmonary conus and the hilar-to-thoracic ratio were significantly increased in patients with chronic obstructive lung disease (p less than 0,0001). Measurement of the right pulmonary artery was 19.7 +/- 3.9 mm compared to 13.6 +/- 1.2 mm of the control group; mean hilar thoracic index was 0.35 compared to 0.31. Thus if the width of the descending branch of the right pulmonary artery was above 16 mm, pulmonary arterial hypertension was suggested, with a specificity of almost 100%, although the sensitivity of the diagnosis was only 59%. The mean pulmonary arterial pressure obtained by right heart catheterization correlated poorly with the morphometric criteria obtained.