Unusual Variant of a Rare Constellation: A Left-sided Scimitar Syndrome with Connection to the Azygos Vein

Scimitar syndrome is a rare variant of partial anomalous pulmonary venous return. The syndrome consists of a right-sided abnormal lung segment that is directly supplied by arteries from the aorta and has an anomalous venous drainage to the inferior vena cava. Three cases of left-sided scimitar syndrome have been reported in the literature, but it has not yet been associated with venous drainage to the azygos vein. Herein we report a left-sided scimitar syndrome with anomalous venous drainage to the azygos vein.     

Scimitar syndrome--case report

Scimitar syndrome is a rare congenital anomaly. This syndrome is characterized by partial or complete anomalous pulmonary venous drainage of the right lung to the inferior vena cava. There is a characteristic abnormal radiographic shadow which descends along the right cardiac border (scimitar sign). We examined 71-year old woman with severe pulmonary hypertension due to a large shunt between pulmonary veins and right atrium. Other cause of pulmonary hypertension is atrial septum defect. Our patient required permanent pacemaker implantation for tachy-brady syndrome.     

Differential pulmonary flow in hypoplastic left heart syndrome.

We report a case of hypoplastic left heart syndrome associated with restrictive interatrial communication and partial anomalous pulmonary venous connection via a right lower pulmonary vein draining to the inferior vena cava. We found unequal pulmonary artery pressure and different pulmonary artery structure, with the right pulmonary artery being lower in pressure and more tortuous and dilated in its peripheral branches than the left. This was attributed to the variant degrees of pulmonary venous obstruction. The left pulmonary venous return was severely obstructed by the restrictive interatrial communication, whereas the anomalous right lower pulmonary vein drained into the inferior vena cava, with less obstruction. To the best of our knowledge, there has never before been a report of differential pulmonary flow associated with a partial anomalous pulmonary vein, in a case of hypoplastic left heart syndrome.     

Abnormal venous connection between the left upper pulmonary vein and the left brachiocephalic vein,associated with rheumatic combined valvular heart disease

Summary A case of partial anomalous pulmonary venous return (PAPVR) associated with mitral stenosis and aortic regurgitation is described. The diagnostic clue was radiocardiography using radioiodide serum albumin (RISA), our routine procedure before cardiac catheterization. The abnormal vessel connected with both the left upper pulmonary vein (PV) and the left brachiocephalic vein, without a stenotic lesion. Aortic valve replacement, open mitral commissurotomy, and simple ligation of the anomalous vein were successfully performed.     

Drenagem venosa pulmonar anômala em gestante identificada pela ressonância magnética cardíaca

Anomalous pulmonary venous return (APVR) is a rare cardiac anomaly defined as one or more pulmonary veins draining into a structure other than the left atrium, with venous return directly or indirectly to the right atrium. The most common form is partial APVR, in which one to three pulmonary veins drain into systemic veins or into the right atrium. We report the case of a woman diagnosed with partial APVR by magnetic resonance imaging during pregnancy.     

Diagnostic problems in visceral heterotaxia]

Due to the lack of uniformity in the criteria for formulating the diagnosis of the syndrome of heterotaxy, 12 cases with this abnormality were reviewed. The patients were selected on the following basis: symmetrical liver, changeable P waves in consecutive electrocardiograms, bronchial isomerism, hematological disturbances, anomalous relationship of the inferior vena cava and abdominal aorta, anomalous systemic and pulmonary venous drainage and complex cardiac malformations. The most frequent findings were: symmetrical liver, changeable P waves, anomalous systemic venous return, anomalies of the atrio-ventricular valves, particularly atrioventricular canal, aorto-cava juxtaposition, single atrium, anomalous pulmonary venous return, transposition of the great arteries and pulmonary stenosis. Less frequent anomalies were: atrial and ventricular septal defects, atrial isomerism, truncus arteriosus and partial distortion of the great arteries. The hematological disturbances as well as the radioisotope scanning of the liver and the spleen were of little help. Suggestions are given for the diagnosis of the syndrome and for the evaluation of present diagnostic procedures making necessary to use the data gathered with more precision.     

婴幼儿先天性心脏病的外科手术与围术期处理

目的总结婴幼儿先天性心脏病的外科手术经验。方法自2000年1月至2004年12月共为133例,年龄5￣36个月,体重6￣15kg的先天性心脏病患儿施行外科手术治疗。全组非体外循环动脉导管结扎术29例;体外循环手术104例,包括室间隔缺损、继发孔房间隔缺损、肺动脉瓣狭窄、部分型房室间隔缺损、部分型肺静脉异位引流、法乐四联症、房间隔缺损并右室流出道梗阻和其他复合畸形。结果全组术后住院死亡6例,死亡率4.5%。术后主要并发症包括低心排综合征、呼吸衰竭、心律失常、肺部感染、出血等。死亡原因包括重度低心排综合征、严重室性心律失常、肺动脉高压危象等。结论心外科、心儿科、麻醉科等人员的密切配合,心内、外科镶嵌治疗成为婴幼儿心脏外科手术成功的重要保证。     

Scimitar syndrome with bicuspid aortic valve. A case report of cross-sectional non- invasive imaging allowing a complete anatomical and functional assessment

Scimitar syndrome is a variant of partial anomalous pulmonary venous return with an aberrant vein, the Scimitar vein, draining the right lung to the inferior vena cava instead of the left atrium, resulting in a left-to-right shunt. The classic frontal radiographic finding, designated as “the scimitar sign”, is of a scimitar (a Turkish sword) shaped density along the right cardiac border. The diagnosis can be made by echocardiography, and cardiac catheterisation remains the gold standard to assess the left-to-right shunt. However, the place of multimodal cardiac imaging by computed tomography and magnetic resonance imaging is increasing. We report the case of a 26 year-old man presenting with chest pain during a brief panic attack, in whom scimitar syndrome was associated with a bicuspid aortic valve, a clinical association rarely reported in the literature. CT and MRI cardiac imaging was as accurate as echocardiography and hemodynamics, particularly for shunt quantification.     

Comparative echocardiographic features of conditions presenting with symptomatic pulmonary hypertension and right ventricular hypertrophy in early infancy.

Echocardiographic dimensions in infants (less than 2 months) with pulmonary hypertension, associated with coarctation of the aorta in 21, total anomalous venous return in 10, and left-to-right shunts in 14 were compared with 10 infants with respiratory distress syndrome, seven with transient tachypnoea of the newborn, 20 normal children, and with each other. Distinguishing features of total anomalous pulmonary venous return were very significantly lower left atrial and left ventricular dimensions. Right ventricular enlargement was maximal in patients with coarctation of the aorta and total anomalous pulmonary venous return. Left ventricular end-diastolic dimension in coarctation of the aorta was not significantly different from normal but the left ventricular end-systolic dimension was significantly lower, suggesting a hypercontractile left ventricle. Similar findings were observed in patients with left-to-right shunts. M-mode echocardiograms are hence valuable in differentiating conditions which may present with intractable congestive cardiac failure in the newborn and also distinguishing them from common respiratory disease of the neonate.     

Platypnea-orthodeoxia syndrome combined with multiple congenital heart anomalies

A 71-year-old woman was admitted for paralysis on the left side of her body. She developed dyspnea and hypoxemia after admission. Although pulmonary embolism was suspected, hypoxemia and dyspnea occurred repeatedly in spite of anticoagulation therapy. Transesophageal echocardiography revealed a patent foramen ovale (PFO), an atrial septal aneurysm (ASA), and a right-to-left shunt that appeared in an upright position. She was diagnosed with platypnea-orthodeoxia syndrome. Moreover, cardiac catheterization showed congenital anomalies, such as unroofed coronary sinus, partial anomalous pulmonary venous return and persistent left superior vena cava. Simple surgical closure of the ASA and PFO improved all of her symptoms.     

Partial left anomalous pulmonary venous return: a diagnostic dilemma

Two adults were seen with left partial anomalous pulmonary venous return with intact atrial septum. Both cases had remained undiagnosed despite medical attention since childhood, including previous cardiac catheterization in one patient. Noninvasive tests, indocyanine green dye injections, pulmonary artery angiography, and venous sampling for oximetry high in the superior vena cava above the entry of the innominate vein will help establish the diagnosis of this disorder.     

Abnormal drainage of inferior vena cava to left atrium together with a partial abnormal pulmonary venous drainage to right atrium in the presence of atrial septal defect

The direct abnormal drainage of the inferior vena cava (IVC), while rare, is well‐recognized anomaly of systemic venous drainage. It has been reported both in isolation and in association with other cardiac defects. This is a case of an abnormal drainage of IVC into left atrium (LA) together with partial abnormal pulmonary venous drainage to the right atrium (RA) and atrium septal defect (ASD).     

Fetal and Neonatal Imaging and Strategy of Primary Neonatal Heart Transplantation in Hypoplastic Left Heart with Ebstein's Anomaly

We present the anatomic constellation of mitral stenosis/aortic atresia variant of hypoplastic left heart syndrome, Ebstein's anomaly, and partial anomalous pulmonary venous return, an exceeding rare congenital heart defect. Prenatal echocardiography led to concern about the capacity of the right ventricle to increase cardiac output with lung expansion and pulmonary arterial runoff at birth, prompting the precaution of extracorporeal membrane oxygenator standby at delivery. Stage I palliation was not attempted, and control of pulmonary arterial blood flow was achieved with pulmonary artery banding, allowing sufficient ongoing hemodynamic stability. Orthotopic cardiac transplantation, repair of hypoplastic aortic arch, and primary sutureless repair of left pulmonary veins was performed, using dual‐site arterial cannulation and continuous mild hypothermic cardiopulmonary bypass. We discuss how this unique echocardiographic anatomy influenced the surgical decision and point out how it guided therapy toward a strategy of primary transplantation rather than standard staged surgical palliation.     

Left‐sided Partial Anomalous Pulmonary Venous Communication in a Patient with Turner's Syndrome

Congenital defects of the aortic valve and thoracic aorta are well recognised in patients with Turner's syndrome. Anomalous pulmonary venous connections that were previously considered rare in patients with Turner's syndrome have been detected in up to one in ten patients with the advent of cardiac magnetic resonance imaging. We describe an instance of partial anomalous pulmonary venous drainage in a patient with Turner's syndrome, discuss the clinical indicators of these anomalies, and briefly mention the management options in such patients.     

Total anomalous pulmonary venous return with associated patent ductus arteriosus. Two cases with successful correction.

A large patent ductus arteriosus associated with total anomalous pulmonary venous return results in a unique hemodynamic pattern, and long-term survival has been rare. Two patients, aged five and 18 years, underwent successful surgical repair of supracardiac total anomalous pulmonary venous return and an associated large patent ductus arteriosus. The association of the Noonan syndrome in the 18-year-old woman is of additional interest.     

Unusual abnormal pulmonary venous return

We describe a case of a middle-aged woman, known with situs inversus totalis. She was suffering from progressive dyspnoea. With different imaging techniques we came to the correct diagnosis, a partial abnormal pulmonary venous return, which was surgically confirmed. Although the structure was complex, the treatment was simple.     

Pulmonary artery sling associated with total anomalous pulmonary venous return. A rare case report

An infant with a supracardiac type of total anomalous pulmonary venous return associated with pulmonary artery sling is reported. Two-dimensional echocardiography can provide an accurate diagnosis of these two rare types of congenital cardiac lesion prior to esophagography and cardiac catheterization.     

One plug may not be enough: a novel technique for the occlusion of high-flow vascular connections: combined AGA vascular plug II and coil occlusion of a sequester artery in a patient with Scimitar Syndrome

A 5-year-old male presented at birth with tachypnea and abnormal chest x-ray. He was diagnosed with Scimitar Syndrome in the neonatal period by ultrasound, which revealed partial anomalous pulmonary venous return (PAPVR) of the right pulmonary vein to the inferior vena cava, as well as dextroversion of the heart, hypoplastic right pulmonary artery, and right lung hypoplasia. Due to the large shunt volume, the patient exhibited signs of congestive heart failure with dilatation of the left atrium and left ventricle. The patient underwent cardiac catheterization, which confirmed the presence of PAPVR as well as a large aorto-pulmonary collateral connecting the sequester to the descending aorta. Due to the high flow within the sequester artery a combined "sandwich" technique was used with two AGA vascular plugs (II) and MWCE Tornado coils. Complete closure of the collateral was achieved.     

BMPR2 mutations in pulmonary arterial hypertension with congenital heart disease.

The aim of the present study was to determine if patients with both pulmonary arterial hypertension (PAH), due to pulmonary vascular obstructive disease, and congenital heart defects (CHD), have mutations in the gene encoding bone morphogenetic protein receptor (BMPR)-2. The BMPR2 gene was screened in two cohorts: 40 adults and 66 children with PAH/CHD. CHDs were patent ductus arteriosus, atrial and ventricular septal defects, partial anomalous pulmonary venous return, transposition of the great arteries, atrioventicular canal, and rare lesions with systemic-to-pulmonary shunts. Six novel missense BMPR2 mutations were found in three out of four adults with complete type C atrioventricular canals and in three children. One child had an atrial septal defect and patent ductus arteriosus; one had an atrial septal defect, patent ductus arteriosus and partial anomalous pulmonary venous return; and one had an aortopulmonary window and a ventricular septal defect. Bone morphogenetic protein receptor 2 mutations were found in 6% of a mixed cohort of adults and children with pulmonary arterial hypertension/congenital heart defects. The current findings compliment recent reports in mouse models implicating members of the bone morphogenetic protein/transforming growth factor-beta pathway inducing cardiac anomalies analogous to human atrioventricular canals, septal defects and conotruncal congenital heart defects. The small number of patients studied and the ascertainment bias inherent in selecting for pulmonary arterial hypertension require further investigation.     

Atresia of the common pulmonary vein

A newborn girl with atresia of the common pulmonary vein, presented immediately after birth with severe cyanosis and acidosis. The diagnosis of totally obstructed total pulmonary venous return was made by cross-sectional echocardiography. Subsequent cardiac catheterization failed to demonstrate the site of pulmonary venous return. Necropsy showed the pulmonary veins to be connected bilaterally to an atretic common pulmonary vein. There was no obvious alternative pathway for pulmonary venous return.