Scimitar syndrome is a form of partial anomalous pulmonary venous drainage that is dramatically visible on plain chest radiography (CXR). In these individuals the entire venous drainage from the right lung enters a single anomalous large vein that descends to the inferior vena cava. This descending vein is visible on CXR as a curvilinear density along the right heart border and resembles the curved Turkish sword that gives the condition its name. Scimitar syndrome forms part of the large spectrum of associated conditions known as venolobar syndrome. These include right lung hypoplasia or sequestered segments of right lung, congenital heart disease and various others. We report the case of a young woman who presented incidentally, with a murmur, at 16 years of age. Full investigation including angiography showed a large atrial septal defect with right heart dilation and scimitar syndrome. She underwent surgical correction with uneventful and complete correction by baffling of the scimitar vein from its entry into the inferior vena to the left atrium through the enlarged atrial septal defect.
heart defects; congenital; scimitar syndrome
Horseshoe lung is a congenital pulmonary malformation that is usually associated with scimitar syndrome. This malformation consists of fusion of both pulmonary lobes from the posterobasal segments. The fusion appears in the retrocardiac area, in front of the esophagus and thoracic aorta. Pleural separation of pulmonary lobes distinguishes pseudohorseshoe appearance from a true horseshoe lung. Scimitar syndrome known as hypogenetic lung syndrome is a part of the congenital pulmonary venolobar syndrome. It is a partial anomalous pulmonary venous return with pulmonary hypoplasia. Scimitar vein is an anomalous drainage vessel between the right pulmonary lobe vessels and the inferior vena cava. The appearance of the vessel resembles Turkish scimitar; therefore, the syndrome is called scimitar syndrome. We hereby report a 61-year-old woman with adult form congenital scimitar syndrome and will describe the imaging findings of pseudohorseshoe lung appearance.
Congenital Abnormalities; Pulmonary Circulation; Pulmonary Atresia; Computed Tomography
Scimitar syndrome or pulmonary venolobar syndrome is a rare, complex, and variable malformation of the right lung characterized by an abnormal right sided pulmonary drainage into the inferior vena cava, malformation of the right lung, abnormal arterial supply, and sometimes cardiac malformation. Despite the varying degrees of pulmonary hypoplasia and pulmonary artery hypertension, about half of the patients with scimitar syndrome are asymptomatic or mildly symptomatic when the diagnosis is made. Neonates have severe symptoms and worse prognosis while older children come to light because of recurrent respiratory infections, heart murmur, or an abnormal chest radiograph.
Pulmonary Artery Atresia; Scimitar Syndrome; CT Angiography
The Scimitar syndrome is a rare congenital anomaly that consists in part of total or partial anomalous venous drainage of the right lung to the inferior vena cava (IVC). This descending vein is visible on CXR as a curvilinear density along the right heart border and resembles the curved Turkish sword that gives the condition its name. Scimitar syndrome forms part of the large spectrum of associated conditions known as venolobar syndrome. These include right lung hypoplasia or sequestered segments of the right lung, congenital heart disease and various others. Surgical approaches to the Scimitar syndrome have varied according to the anatomic and pathologic features presented in each case. Here we review the clinical signs and symptoms, diagnostic dilemmas, current medical and surgical managements of Scimitar syndrome.
anomalous pulmonary venous drainage
pulmonary venolobar syndrome
Scimitar syndrome is a rare but serious congenital condition that consists of anomalous pulmonary venous drainage of the right lung to the inferior vena cava. The appearance on chest radiography resembles a curved Turkish sword, or scimitar. Scimitar syndrome is associated with other anomalies, including hypoplasia of the right lung, dextroposition of the heart, anomalous systemic arterial supply to the right lung, and atrial septal defect. Clinical manifestations in infants include severe tachypnea and cyanosis. Adult patients may present with dyspnea and increased fatigue. Few adult cases have been reported in the medical literature. Classical surgical repair involves diversion of the scimitar venous flow into the left atrium with a baffle, requiring the use of cardiopulmonary bypass and deep hypothermic circulatory arrest. Herein, we report the case of a 42-year-old woman with 2 scimitar veins who underwent corrective surgery at our center without the use of cardiopulmonary bypass. We also comment on the importance of a patient's lung hypoplasia in the decision to repair the defect through a right thoracotomy.
WEB SITE FEATURE
Cardiovascular surgical procedures/methods; dyspnea/etiology; heart atria/abnormalities/surgery; heart defects, congenital; pulmonary veins/abnormalities/surgery; patient selection; radiography, thoracic; scimitar syndrome/complications/diagnosis/physiopathology/surgery; treatment outcome
Pulmonary embolism due to hydatid disease is an unusual condition resulting from the rupture of a hydatic heart cyst or the opening of liver hydatidosis into the venous circulation. A 78-year old male patient complaining of dyspnea, cough and severe chest pain was admitted to our emergency department. A multidetector computed tomography of the chest revealed the presence of multiple nodules in both lungs especially in left and multiple hypodense filling defect in left main pulmonary artery and its branches. In addition, coronal reformatted multidetector computed tomography images also showed two hypodense cystic parenchymal masses on the left lobe of the liver with a cystic embolus in the right atrium. Pulmonary embolism should be kept in mind in patients who have hepatic hydatidosis if suddenly chest pain and dyspnoea occurs, especially in regions where hydatidosis is endemic.
Pulmonary embolism; Rupture; Echinococcosis; Hepatic; Multidetector computed tomography; Aged people; Case report; Turkey
Three cases of a congenitally misplaced segment of lung are described. In one case the abnormal segment was identified at thoracotomy; it extended from an origin in the right upper lobe across the midline into the left hemithorax. The remaining two patients, with identical radiographic features, were identified in a review of 24 cases of bronchovascular malformation. In all three patients the crossover segment was associated with a variant of the scimitar syndrome: right lung hypoplasia with dextrocardia, aberrant systemic arterial supply to the right lower lobe, and anomalous venous drainage of the whole of the right lung, and in two patients with eventration of the right diaphragm.
A case of an asymptomatic 32-year-old male with a complex congenital pulmonary vein varix is reported herein. Chest X-ray incidentally revealed a tubular opacity passing from the periphery of the left lingula to the mediastinum. ECG gated multidetector computed tomography showed the opacity to be a vessel emptying into the left atrium via the left superior pulmonary vein. In addition, a second vascular structure was noted within the posterior mediastinum that was emptying into the same pulmonary vein. These findings were also confirmed by magnetic resonance imaging, 4D magnetic resonance angiography and invasive arterial angiography. Based on multimodality imaging findings the diagnosis of complex congenital pulmonary venous varix with posterior mediastinal extension was established.
pulmonary vein varix; varix; mediastinum; venous structure; mediastinal tissue
Anomalous pulmonary venous drainage to the inferior vena cava is a rare congenital cardiac defect. Oxygenated blood from the right lung enters the right atrium resulting in a left-to-right shunt. Because the radiographic shadow of the anomalous vein resembles a curved saber, this defect has been called the “scimitar” deformity. From 1958 through June 30, 1975, 11 patients underwent surgical correction of this anomaly at our institution with 10 survivors. Diagnosis was made by routine roentgenography of the chest in all but one patient. Eight patients had total correction which consisted of implanting the anomalous vein into the right atrium, opening the interatrial septum and applying a patch graft as a baffle to direct pulmonary venous blood into the left atrium. Five patients had associated cardiac defects which were also repaired. Three patients underwent pneumonectomy and all survived. The only operative death occurred in a 5-year-old female with an atrial septal defect and endocardial cushion defect. We believe the existence of a large left-to-right shunt justifies surgical intervention. The prognosis appears to depend upon the presence of other cardiac or pulmonary anomalies.
Unilateral primary pulmonary hypoplasia is rare in adulthood (UPHA); it is characterized by a decreased number of bronchial segmentation and decreased/absent alveolar air space. Classical chest X-ray may be confusing, and the biological tests are unspecific. We present a case of UPHA in a 60-year-old female, smoker, with 3 term normal deliveries, who presented with late recurrent pneumonias and bronchiectasis-type symptomathology, arterial hypertension, and obesity. Chest X-rays revealed opacity in the left lower pulmonary zone, an apparent hypoaerated upper left lobe and left deviation of the mediastinum. Preoperatory multidetector computer tomography (MDCT) presented a small retrocardiac left lung with 5-6 bronchial segmentation range and cystic appearance. After pneumonectomy the gross specimen showed a small lung with multiple bronchiectasis and small cysts, lined by hyperplasic epithelium, surrounded by stromal fibrosclerosis. We concluded that this UPHA occurred in the 4–7 embryonic weeks, and the 3D MDCT reconstructions offered the best noninvasive diagnosis.
To evaluate the patterns of pulmonary venous drainage into the left atrium and to determine the frequency of each variant of pulmonary venous anatomy.
Materials and methods:
After institutional review board approval (No. 09JUL011148), 300 studies of thoracic multidetector computed tomography were retrospectively reviewed for the anatomical features of the pulmonary vein and its drainage pattern into the left atrium. The percentage of each pattern was calculated.
The anatomy of pulmonary venous drainage in 300 patients (150 male and 150 female, mean age 60.16 years) showed some variation. In the right pulmonary vein, the most common drainage pattern was two ostia (90.33%), followed by three to five ostia (6.33%) and a single ostium (3.33%). There were one or two separate middle lobe vein ostia in groups of more than two openings. On the left side, there were two patterns; a single venous ostium (59%) was much more common than two ostia (41%). In both right and left pulmonary veins, there were five cases (2 male, 3 female) that had a single pulmonary venous ostium, bilaterally. However, there were only 17 cases (5.67%), out of 300 enrolled in this study, that had bilateral pulmonary venous ostial variations.
A classification system to succinctly describe pulmonary venous drainage patterns was developed. In left-sided drainage, a single left pulmonary ostium was the most common variation. The right-sided venous drainage varied more in both number and pattern than those of the left side; nevertheless, bilateral pulmonary venous ostial variation was not frequently found.
Multidetector CT; pulmonary vein; left atrium
A 58-year-old female patient presented to the hospital with hearing loss. In the chest radiography obtained before her ear surgery, volume decrease in the right hemithorax, elevation of the right diaphragm, and increase of ventilation in the right lung were detected. At the thorax CT-CT angiography, hypoplasia of the main pulmonary artery and its branches and arteriovenous malformation localized in the middle lobe of the right lung were detected. Thus, diagnosis of Swyer-James-Macleod syndrome associated with right lung middle lobe hypoplasia and arteriovenous malformation was made. This kind of association has not been reported earlier, so we are presenting it in the light of the literature knowledge.
Anomalous origin of the coronary artery from the opposite sinus of Valsalva and a course of that artery between the ascending aorta and the pulmonary artery is a rare congenital anomaly. It can cause myocardial ischemia, syncope, and sudden cardiac death in young people. Herein, we report the case of a 24-year-old man who was brought to our hospital after cardiac arrest due to ventricular fibrillation. Emergent coronary angiography revealed that the left coronary artery was normal; however, the right coronary artery originated at the left sinus of Valsalva. After admission, the patient was treated with mild therapeutic hypothermia for 48 hours and had a favorable neurologic recovery. Subsequent 16-slice multidetector computed tomography revealed that the right coronary artery arose from the left main coronary artery, took an intramural course, and was severely compressed between the ascending aorta and the pulmonary artery. The patient underwent direct implantation of the anomalous artery into the correct aortic sinus. Histologic specimens from the proximal end of the right coronary artery showed an intramural segment with intimal fibrous thickening, fragmentation and random arrangement of the elastic fiber, degeneration of the medial smooth-muscle cells, and an increase in the medial stromal substance. Postoperatively, repeat coronary angiography with provocation testing for coronary spasm revealed no myocardial ischemic change. The patient recovered uneventfully. We found that cardiac multidetector computed tomography was useful in evaluating the cause of the sudden cardiac arrest, identifying the anomalous coronary artery, and helping to guide the surgical decisions.
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Coronary angiography; coronary vessel anomalies/diagnosis/physiopathology/surgery; death, sudden, cardiac/prevention & control; replantation; risk assessment; sinus of Valsalva/abnormalities/surgery; treatment outcome
The objective of the present paper is to describe the clinical and computed tomography features of 'explosive pleuritis', an entity first named by Braman and Donat in 1986, and to propose a case definition. A case report of a previously healthy, 45-year-old man admitted to hospital with acute onset pleuritic chest pain is presented. The patient arrived at the emergency room at 15:00 in mild respiratory distress; the initial chest x-ray revealed a small right lower lobe effusion. The subsequent clinical course in hospital was dramatic. Within 18 h of admission, he developed severe respiratory distress with oxygen desaturation to 83% on room air and dullness of the right lung field. A repeat chest x-ray, taken the morning after admission, revealed complete opacification of the right hemithorax. A computed tomography scan of the thorax demonstrated a massive pleural effusion with compression of pulmonary tissue and mediastinal shift. Pleural fluid biochemical analysis revealed the following concentrations: glucose 3.5 mmol/L, lactate dehydrogenase 1550 U/L, protein 56.98 g/L, amylase 68 U/L and white blood cell count 600 cells/mL. The pleural fluid cultures demonstrated light growth of coagulase-negative staphylococcus and viridans streptococcus, and very light growth of Candida albicans. Cytology was negative for malignant cells. Thoracotomy was performed, which demonstrated a loculated parapneumonic effusion that required decortication. The patient responded favourably to the empirical administration of intravenous levofloxacin and ceftriaxone, and conservative surgical methods in the management of the empyema. This report also discusses the patient's rapidly progressing pleural effusion and offers a potential case definition for explosive pleuritis. Explosive pleuritis is a medical emergency defined by the rapid development of a pleural effusion involving more than 90% of the hemithorax over 24 h, which causes compression of pulmonary tissue and mediastinal shift to the contralateral side.
Explosive pleuritis; Pleurisy; Pleuritis; Pneumonia
Hypogenetic lung syndrome is more popularly known as a scimitar syndrome (SS). It is a rare developmental lung malformation which almost always occurs on the right side. The two most constant features of this syndrome are anomalous pulmonary venous return into systemic circulation, most frequently via inferior vena cava (IVC), and lung hypoplasia.
We are reporting such a case illustrating most typical and some uncommon features on chest radiograph and multislice computer tomography (MSCT) of chest. Focal herniation of liver through a diaphragmatic defect presenting as an ovoid soft tissue mass in right lower paraspinal region on chest X ray mimicking sequestration is an interesting but rare finding.
Dextrocardia; pulmonary hypoplasia; sequestration
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly associated with very high mortality during infancy. We report a 35-year-old female patient with ALCAPA initially visualized by echocardiography. She visited outpatient department presenting with intermittent chest discomfort for 3 weeks. Transthoracic echocardiography showed left coronary artery arising from main pulmonary artery and abundant septal color flow Doppler signals. Transesophageal echocardiography clearly revealed markedly dilated and tortuous right coronary artery showing windsock appearance. Multidetector computed tomography and coronary angiography enabled visualization of anomalous left coronary artery originating from left side of main pulmonary trunk. After treadmill exercise test which showed ST-segment depression presenting inducible myocardial ischemia, patient underwent direct re-implantation of the anomalous coronary artery into the aorta without any complication.
Coronary vessel anomalies; Echocardiography; Computed tomography
Reliable, noninvasive approaches to the diagnosis of pulmonary hypertension in idiopathic pulmonary fibrosis are needed. We tested the hypothesis that chest computed tomography-determined extent of pulmonary fibrosis and/or main pulmonary artery diameter can be used to identify the presence of pulmonary hypertension in patients with advanced idiopathic pulmonary fibrosis.
Cross-sectional study of 65 patients with advanced idiopathic pulmonary fibrosis with available right-heart catheterization and high-resolution chest computed tomography. An expert radiologist scored ground-glass opacity, lung fibrosis, and honeycombing in the computed tomography images on a scale of 0-4. These scores were also summed into a total profusion score. The main pulmonary artery was measured at its widest dimension on the supine full chest sequence. At this same level, the widest aorta diameter was measured.
Chest computed tomography-determined fibrosis score, ground-glass opacity score, honeycombing score, total profusion score, diameter of the main pulmonary artery, and the ratio of the pulmonary artery to aorta diameter did not differ between those with and without pulmonary hypertension. There was no significant correlation between mean pulmonary artery pressure and any of the chest computed tomography-determined measures.
High-resolution chest computed tomography-determined extent of pulmonary fibrosis and/or main pulmonary artery diameter cannot be used to screen for pulmonary hypertension in advanced idiopathic pulmonary fibrosis patients.
pressure, pulmonary artery; hypertension, pulmonary; pulmonary fibrosis; high-resolution chest computed tomography; diagnosis
We report on the case of a 68-year-old male patient with the history of right pneumonectomy due to bronchial carcinoma, who was referred for aortic valve replacement due to severe calcified aortic stenosis. Pre-operative chest X-ray and computed tomography (CT) revealed an unusually pronounced mediastinal shift to the right. Despite this unusual anatomy, we decided to perform surgery using the right anterolateral thoracotomy following thorough pre-operative planning using 3D-volume rendering of the CT data-set. This approach yielded excellent exposure of the aortic root and the ascending aorta, respectively. Following an uneventful operative and post-operative course the patient could be discharged on post-OP day 6.
Although only occasionally described for aortic valve replacement a right anterolateral thoracotomy may represent a valuable surgical approach, particular in patients with unusual anatomy, e.g. a mediastinal right-shift. However, thorough pre-operative planning, i.e. using visualization and planning techniques such as 3D-volume rendering should be mandatory as it provides information crucial to facilitate surgical steps and thus, may help avoid severe surgical complications.
Pneumonectomy; Aortic valve; Thoracotomy; Minimally invasive surgical procedures
We present a 27-year-old female patient admitted with an anterior mediastinal mass. She complained of chest discomfort and hemoptysis which began seven months prior. She had given birth five months prior. Thoracic X-ray showed an anterior mediastinal mass. Thorax computed tomography (CT) confirmed a well-defined anterior mediastinal mass with 13 × 12 cm diameter, extending to the right hemithorax. It was composed of cystic spaces and discrete areas like soft tissue and fat. Serum Ca 19-9 level was elevated. CT features were consistent with a mature teratoma. During median sternotomy, the tumor revealed adhesions to the right lung and the right subclavian artery. Histologically, the tumor was diagnosed as a mature teratoma. Estrogen and progesterone receptors were detected to be positive in the resected tissue. We conclude that alterations in hormone levels during pregnancy might be the cause of rapid tumor growth which leads to hemoptysis.
We present a case of pulmonary vein (PV) stenosis after radio-frequency (RF) ablation, in which a hemodynamic change in the pulmonary artery was similar to that of congenital PV atresia on time-resolved contrast-enhanced magnetic resonance angiography (TR-MRA). A 48-year-old man underwent RF ablation due to atrial fibrillation. The patient subsequently complained of hemoptysis, dyspnea on exertion, and right chest pain. Right PV stenosis after catheter ablation was diagnosed through chest computed tomography and lung perfusion scan. Pulmonary TR-MRA revealed the pulmonary artery via systemic arterial collaterals and draining systemic collateral veins. On a velocity-encoded cine image, the flow direction of the right pulmonary artery was reversed in the diastolic phase and the left pulmonary artery demonstrated continuous forward flow throughout the cardiac cycle. These hemodynamic changes were similar to those seen in congenital unilateral PV atresia.
Pulmonary vein; Radiofrequency catheter ablation; Complication; Hemodynamics; Magnetic resonance angiography
We present a woman having congenital anomalies of the inferior vena cava and partial anomalous pulmonary venous return from the right lung with atrial septal defect in a 38-year-old. Congenital anomalies of inferior vena cava are rare. They are seen more often in young males. If there are not other anomalies, they are latent for a long time. Peripheral venous thrombosis, chronic venous insufficiency, dyspnea and fatigue are often the first symptoms of these anomalies. Surgical repair of atrial septal defect with partial anomalous pulmonary venous return include provision of durably unobstructed systemic and pulmonary venous pathways, closure of the atrial septal defect, and avoidance of arrhythmias. The diagnosis has been determined by compression ultrasonography with color doppler assessment, multidetector computed tomography angiography and echocardiography.
Solitary fibrous tumor of the pleura is a rare, usually benign, and slow-growing neoplasm. Complete surgical resection for giant tumor of the pleura is challenging because of poor exposure and a large blood supply. We report the case of a giant hypervascular fibrous tumor that filled nearly the entire left hemithorax and anterior mediastinum, and its preoperative management.
A 59-year-old woman presented to us with exertional dyspnea and chest pain. A chest radiograph showed the right hemithorax completely opaque and a mediastinal shift to the left hemithorax. A tomography scan of the thorax showed a giant mass that almost completely filled the right hemithorax and compressed the mediastinum to the left. Because of excessive bleeding during dissection, the operation was terminated after a biopsy specimen was obtained. The biopsy was diagnosed as a benign fibrous tumour. A thoracic computed tomography angiogram showed that the mass was supplied by multiple intercostal arteries as well as an aberrant artery that branches off the celiac trunk in the subdiaphragmatic region. Due to the many arteries that needed to be embolized, the final decision was to control the bleeding following resection by inducing total circulatory arrest with the help of cardiopulmonary bypass. The bleeding could not be controlled under cardiopulmonary bypass and the patient’s death was confirmed.
We report this case to emphasize the necessity of preoperative embolization; the use of cardiopulmonary bypass and total circulatory arrest is not a valid alternative method to control the bleeding.
benign; intrathoracic; fibrous; giant tumor; benign tumors; solitary fibrous; tumors; therapy
A 30-year-old male, carpenter by profession, presented with a history of dry cough and progressive shortness of breath for two months along with right-sided chest pain for one and a half months. The clinico-radiological picture was suggestive of right-sided massive pleural effusion. Computed tomography (CT) scan of the thorax showed a huge mediastinal mass occupying the entire right hemithorax with very small amount of pleural effusion. CT-guided fine needle aspiration cytology and tru-cut biopsy from the mass both revealed small round-cell tumour, possibly small cell carcinoma of the lung. However, on immunohistochemistry tumour cells expressed Mic-2 and it was consistent with a diagnosis of primitive neuroectodermal tumour.
Ewing's sarcoma; immunohistochemistry; mediastinal tumour; primitive neuroectodermal tumour
The present series describes a group of adults with left-to-right shunts including partial anomalous pulmonary venous return (PAPVR) and/or an atrial septal defect (ASD) evaluated with ECG-gated 128-slice multidetector computed tomography (MDCT). PAPVR is defined as a left-to-right shunt where one or more, but not all, pulmonary veins drain into a systemic vein or the right atrium. PAPVR involving the right upper pulmonary vein can be associated with a sinus venosus ASD. The presence, course, number of anomalous veins and associated cardiovascular defects can be reliably observed by 128-slice MDCT angiography.
Partial anomalous pulmonary venous return (PAPVR); Atrial septal defect (ASD); Multidetector computed tomography (MDCT)
Partial anomalous pulmonary vein connection (PAPVC) is a rare congenital abnormal cardiac defect involving the pulmonary veins draining into the right atrium (RA) directly or indirectly by venous connection. Ninety percent of PAPVCs are accompanied by atrial septal defect (ASD). To our knowledge, there is no previous report of PAPVC with ventricular septal defect (VSD) without ASD in Korea, and in this paper, we report the first such case. A 2-day-old girl was admitted into the Chonnam National University Hospital for evaluation of a cardiac murmur. An echocardiogram revealed perimembranous VSD without ASD. She underwent patch closure of the VSD at 5 months of age. Although the VSD was completely closed, she had persistent cardiomegaly with right ventricular volume overload, as revealed by echocardiography. Three years later, cardiac catheterization and chest computed tomography revealed a PAPVC, with the right upper pulmonary vein draining into the right SVC. Therefore, correction of the PAPVC was surgically performed at 3 years of age. We conclude that it is important to suspect PAPVC in patients with right ventricular volume overload, but without ASD.
Partial anomalous pulmonary venous connection; Ventricular heart septal defect; Atrial heart septal defect