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, usually associated with pulmonary venolobar syndrome, is a rare congenital anomaly involving the fusion of the postero-basal segments of the right and left lungs across the midline. The fused segment or the isthmus lies posterior to the pericardium and anterior to the aorta. The associated pulmonary venolobar syndrome involves anomalous systemic arterial supply and anomlaous systemic venous drainage of the right lung. With the advent of MDCT imaging, we can diagnose this rare condition as well all its associated anomalies non-invasively. Volume-rendered techniques greatly simplify the complex anatomy and provide easy understanding of the same.
Horse-shoe lung; scimitar syndrome; volume rendered reconstructions
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
Scimitar syndrome is a rare and complex congenital anomaly characterized by partial or complete anomalous pulmonary venous return from the right or left lung into the inferior vena cava, through drainage into the hepatic vein, right atrium or left atrium. The syndrome is commonly associated with hypoplasia of the right lung and right pulmonary artery. We present an 11-year-old female with atypical and rare type of scimitar syndrome. The girl has had cough for 2 months before admission, without fever or abnormalities on medical examination. X-ray films revealed inflammatory and atelectatic changes with mediastinal shift to the right. CT and CT angiography – hypoplasia of the right lung with no visible interlobar fissures. No areas of consolidation in the pulmonary parenchyma. Mediastinum shifted to the right. Single wide venous vessels draining the upper part of the right lung entering the superior vena cava. In our patient, clinical symptoms are mild, but a thorough physical examination could have helped diagnose the syndrome earlier.
scimitar syndrome; pediatric; 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
A 54-year-old man with right aortic arch underwent left lower lobectomy and lingular segmentectomy, followed by complete pneumonectomy, for refractory nontuberculous mycobacterial infection. Three months after the pneumonectomy, he developed acute respiratory distress. Computed tomography showed an excessive mediastinal shift with an extremely narrowed bronchus intermedius and right lower bronchus compressed between the right pulmonary artery and the right descending aorta. Soon after the nearly obstructed bronchus intermedius was observed by bronchoscopy, he began to exhibit frequent hypoxic attacks, perhaps due to mucosal edema. Emergent surgical repositioning of the mediastinum and decompression of the bronchus was indicated. After complete adhesiolysis of the left thoracic cavity was performed, to maintain the proper mediastinal position, considering the emergent setting, an open wound thoracostomy was created and piles of gauze were inserted, mildly compressing the heart and the mediastinum to the right side. Thoracoplasty was performed three months later, and he was eventually discharged without any dressings needed. Mediastinal repositioning under thoracostomy should be avoided in elective cases because of its extremely high invasiveness. However, in the case of life-threatening postpneumonectomy syndrome in an emergent setting, mediastinal repositioning under thoracostomy may be an option to save life, which every thoracic surgeon could attempt.
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
Transfusion-related acute lung injury (TRALI) is an underdiagnosed and underreported syndrome which by itself is the third leading cause of transfusion-related mortality. The incidence of TRALI is reported to be 1 in 2000 to 5000 transfusions. When combined with uncontrollable bleeding, survival is unachievable. We report the case of a 25-year-old man, who underwent open heart surgery as an infant to correct his congenital heart disease in association with right pulmonary artery atresia. He presented with hemoptysis secondary to aspergilloma and required a pneumonectomy of the nonfunctional right lung. During pneumolysis, significant bleeding occurred from the superior vena cava. The patient required a blood transfusion and was placed on cardiopulmonary bypass to control the bleeding. Simultaneous occurrence of severe pulmonary edema and retroperitoneal bleeding were noted. Approximately 8 L of frothy edema fluid were drained from the only functional left lung starting ~15 minutes after the transfusion and lasting for several hours until the end of the case. It most likely represented TRALI syndrome. Increasing abdominal girth and poor volume return to the pump were consistent with and pathognomonic for retroperitoneal bleeding. Though primary surgical bleeding in the chest was controlled successfully and a pneumonectomy performed without further difficulty, we were unable to separate the patient from cardiopulmonary bypass due to the inability to oxygenate. As a result, we could not reverse the anti-coagulation which potentially exacerbated the retroperitoneal bleeding. After multiple unsuccessful attempts the patient succumbed. This ill-fated case demonstrates the quandary of obtaining vascular access for emergency cardiopulmonary bypass while in the right thoracotomy position. It may be beneficial to have both the femoral artery and vein cannulated before positioning a patient in a lateral decubitus position. In addition, early direct access to the right atrium may obviate a need for femoral venous cannulation. Also, adult extracorporeal membrane oxygenation may be indicated if faced with such a severe pulmonary edema without ongoing hemorrhage.
Transfusion; lung injury; pulmonary atresia; retroperitoneal hematoma
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
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
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.
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
The clinical, radiological, electrocardiographic, and haemodynamic findings in 7 patients with the syndrome of congenital absence of the pulmonary valve with ventricular septal defect are reported. Findings included a pansystolic murmur and a low-pitched delayed diastolic murmur at the left sternal border with no pulmonary valve closure sound, an rsR′ pattern in V1 on the electrocardiogram, and a chest x-ray showing a large heart and gross dilatation of the main, left or right pulmonary arteries but normal or reduced vascularity of the peripheral lung fields. Cardiac catheterization showed a left-to-right shunt at ventricular level in every case, though in one the shunt was bidirectional. Despite right ventricular systolic pressures at least 75 per cent of systemic level in every patient, the pulmonary artery pressure was invariably normal. Pulmonary stenosis was shown to exist at infundibular level, at valve ring level, or at both. Selective right ventricular angiography was diagnostic: the main and right or left pulmonary arteries were aneurysmally dilated and there was an abrupt transition in calibre between these vessels and their immediate branches; enlargement of the right pulmonary artery caused distortion and downward displacement of the left atrium in 4 patients; in 2 there was a flat `diaphragm' in the pulmonary valve position.
The diagnosis was confirmed in 3 patients by necropsy. The pulmonary valve was represented by at most a rudimentary fringe of tissue. Histological examination of the pulmonary arteries in one patient showed a correlation between the dilatation of the artery and the structure of its wall.
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.
WEB SITE FEATURE
Coronary angiography; coronary vessel anomalies/diagnosis/physiopathology/surgery; death, sudden, cardiac/prevention & control; replantation; risk assessment; sinus of Valsalva/abnormalities/surgery; treatment outcome
Scimitar syndrome is a congenital heart disease characterized by an abnormal drainage of the right lung into the inferior vena cava, the right atrium or a variety of venous connections from the anomalous pulmonary vein to a systemic vein. This left-to-right shunt induces pulmonary hypertension and is an indication for surgical repair in cases of a history of recurrent pneumonia or significant left-to-right shunting. A corrective approach, which consists of rerouting the anomalous pulmonary flow to the left atrium, is usually performed. Complications of scimitar repair are stenosis, thrombosis and occlusion of the scimitar vein and its deviation.
This case report describes a 53-year-old Caucasian woman with known scimitar syndrome, undergoing surgical repair due to invaliding symptoms of dyspnoea, and presenting with postoperative Budd–Chiari syndrome due to anomalous drainage of her right hepatic vein into the left atrium. It is an interesting cause of liver pathology caused by Budd–Chiari that never has been described before.
This case report emphasizes the importance of a thorough preoperative evaluation, and the importance of antecedents in newly presenting pathology. It is an interesting cause of a known hepatic syndrome, the Budd–Chiari syndrome. This case report is of interest to many specialties, including Hepatology, Cardiology, Radiology and Cardiovascular Surgery. It exposes a new interesting anatomic variation of the scimitar syndrome with significant postoperative implications.
Budd–Chiari syndrome; Hepatic congestion; scimitar syndrome
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
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
Four pediatric patients with congenital coronary arteriovenous fistula (CAVF) were reported to remind pediatric practitioners and cardiologists of its diagnosis and management.
Materials and Methods
Four pediatric patients with congenital CAVF from June 1999 to November 2007 were included in this retrospective study. Study modalities included reviews of patients' profiles of clinical features, chest radiograph, Doppler echocardiography, cardiac catheterization with angiography, myocardial perfusion scan, and computed tomography.
All 4 patients were symptomatic. The clinical symptoms and signs were feeding problem, continuous murmur, tachycardia, tachypnea, cardiomegaly, and exertional chest pain. Myocardial enzyme was elevated in 1 patient. Echocardiography showed dilatation of the coronary artery in all 4 patients, and traced down its origin in 3 and drainage in 4. The fistulas originated from the right coronary artery in 2 patients and left coronary artery in 2, and were drained into the right ventricle in 2, right atrium in 1, and pulmonary artery in 1. Single left coronary artery was found in 1 patient. The pulmonary-to-systemic blood flow ratios ranged from 1.2 to 2.5. Transcatheter coil occlusion was successfully performed in 4 patients through a coaxial delivery system. The symptoms and signs of congestive heart failure and myocardial ischemia disappeared after the procedure.
Diagnosis of congenital CAVF could be achieved by appreciation of continuous murmur over area unusual for the ductus, and by scrupulous examination of echocardiography as well as angiography of the coronary artery through which coaxial transcatheter coil occlusion could be performed successfully.
Coronary arteriovenous fistula; transcatheter coil occlusion; coaxial delivery system; doppler; echocardiography; cardiac catheterization; angiography; myocardial ischemia
Thoracoscopic lobectomy for congenital pulmonary airway malformation has been indicated from the neonatal period to adolescence. However, it is difficult to approach the pulmonary artery for lobectomy in congenital lung malformations with incomplete or absent interlobar fissures. Multidetector computed tomographic images and computed tomography pulmonary angiography gave us helpful information before the operation. We performed thoracoscopic lobectomy for congenital pulmonary airway malformations with absent interlobar fissures and adhesions in accordance with information from multidetector computed tomographic images.
A 14-year-old Japanese girl received a diagnosis of congenital pulmonary airway malformation when she presented with pneumonia. Using multidetector computed tomography and three-dimensional reconstruction provides meticulous characterization of the anatomy in pediatric patients. We confirmed that her left A4+5 artery arose from her left pulmonary artery medial to A6. Her left pulmonary artery was divided just proximal to the A6 origin before the lobes were separated safely. We took advantage of using a stapler to divide the fissureless thick parenchyma. Perioperative diagnosis was congenital cystic adenomatoid malformation.
We used preoperative multidetector computed tomography to outline the bronchovascular anatomy and guide hybrid video-assisted thoracoscopic surgery for a congenital cystic adenomatoid malformation in a fissureless left lung.
Congenital cystic adenomatoid malformation of lung; Hybrid VATS; Thoracic surgery
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.
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.
Partial anomalous venous connection (PAPVC) is a rare congenital heart disease where the blood flow from one or more pulmonary veins (but not all) returns to the right atrium or systemic venous circulation and is often associated with a sinus venosus atrial defect (SVD). Transthoracic echocardiography (TTE) can provide limited information for this anomaly and the diagnosis of this congenital defect has been a clinical challenge.
We report here a case of a 75-year-old female with adult-onset pulmonary arterial hypertension (PAH), hypoxemia and right-sided chamber dilatation. The diagnosis of PAPVC was made incidentally by multidetector computed tomographic angiography (MCTA) that was performed to exclude pulmonary embolism. In this type of PAPVC, the atrial septum is intact, the right upper pulmonary vein (RUPV) connects to the superior vena cava (SVC), and the SVC overrides across the atrial septum and has bi-atrial connection, all of which are clearly manifested by MCTA.
This case indicates the need to exclude a PAPVC and SVD in unexplained pulmonary hypertension, and MCTA is a reliable non-invasive imaging technique with high resolution and wide anatomic coverage. The case also demonstrates that the coexisting SVD with PAPVC is an anomalous venous connection instead of atrial septal defect (ASD) and its key feature is the overriding of SVC or IVC across the intact atrial septum.
Sinus venosus atrial defect (SVD); Partial anomalous pulmonary venous connection (PAPVC); Pulmonary hypertension
Cor triatriatum sinistrum is a rare congenital disorder defined as a division of the left atrium by a diaphragmatic membrane resulting in two left atrial chambers. The membranous division of the atrium can be partial or complete and can affect either atrium, with involvement of the right atrium referred to as cor triatriatum dexter. The presence of fenestrations within the membrane allows for communication and forward passage of blood into the true atrium. Absence of fenestrations leads to early symptomatic engorgement of the lungs. We report the case of a young adult male presenting with recurrent hematemesis due to variceal bleeding. On CT imaging the patient was found to have cor triatriatum sinistrum, with a vertical membrane resulting in total obstruction of the pulmonary venous drainage on the right, with normal pulmonary venous drainage on the left. There was extensive pulmonary-systemic arterial collateralization to the right lung suggesting retrograde filling of the right pulmonary artery with effective flow reversal in the right lung.