Scimitar Vein Research Articles

Scimitar Syndrome: Experience with 6 Patients

Scimitar syndrome is a rare congenital anomaly characterized by anomalous pulmonary venous drainage to the inferior vena cava, causing a left-to-right shunt. Six patients with scimitar syndrome were diagnosed in our hospital between 2002 and 2008. There were 4 girls and 2 boys; 4 < 5 kg in weight, 2 < 8 kg in weight. Scimitar syndrome was suspected in 5 cases because of dextroversion, and diagnosed by color Doppler echocardiography in all 6 when a scimitar vein was detected entering the inferior vena cava. Computed tomography confirmed the diagnosis in all patients. Two patients had horseshoe lung, 2 had a unilobar right lung, 1 had a hypoplastic right lung, and 1 had a hypoplastic right lower lobe. Three patients had severe pulmonary arterial hypertension, 2 had moderate pulmonary arterial hypertension, and one had normal pulmonary arterial pressure. All patients had lower respiratory tract infections, volume loss of the right lung, a normal or hyperinflated left lung, dextroversion of the heart, and scimitar arteries from the descending aorta. Pneumonectomy was performed in 3 patients, lobectomy in 1, ligation of anomalous vessels in 1, and 1 died before surgery.

Mid-term results of right axillary incision for the repair of a wide range of congenital cardiac defects☆

We evaluated the mid-term results of the right axillary incision used for the repair of various congenital heart defects. All the patients who were operated with this incision between March 2001 and December 2007 were reviewed. There were 123 patients (median age 4.7 {0.4-19.4} years and median weight 16.6 {3.8-62} kg) undergoing atrial septal defect (ASD) closure (62), repair of partial anomalous pulmonary venous connection (PAPVC) (22), correction of partial atrioventricular septal defect (AVSD) (19), and restrictive perimembranous ventricular septal defect (VSD) (20). Additional procedures involved tricuspid valve plasty (10), mitral annuloplasty (3), reduction plasty of the aortic sinus (2), resuspension of the aortic valve cusp (2), sub aortic membrane resection (1), or reimplantation of Scimitar vein (1). The surgical technique involved peripheral (groin) and central (SVC+/-aorta) cannulation for institution of cardiopulmonary bypass. Fibrillatory arrest was used for repair of ASDs and cardioplegic arrest for repairs involving the atrioventricular valves as well as VSDs. The median CPB and aortic clamp times were 72 (35-232) and 0 (0-126) min, respectively. There was no need for conversion to another approach in any patient. Early morbidity included transient paresis of left upper arm (1), stenting of SVC after repair of a sinus venosus defect (1) and revision for bleeding (1). Follow-up echo showed no residual defect in 116 patients and minor residual defects in 7 patients: tiny ASD (2), tiny VSD (1) and mitral regurgitation (4). One patient developed stenosis in the right external iliac artery used for cannulation, necessitating surgical intervention. All the patients are in excellent condition after a median follow-up of 4.1 (0.4-7.1) years. The incision healed well and the thorax and the breast showed no deformity on follow-up. The right axillary incision provides a quality of repair for various congenital defects similar to that obtained by using standard surgical approaches. Because of its deceitful location, and the camouflaging effect of being hidden by the resting arm, it has superior cosmetic appeal compared to conventional incisions. The incision does not interfere with subsequent development of the thorax or the breast (in case of females).

Cardiac MRI and Pulmonary MR Angiography of Sinus Venosus Defect and Partial Anomalous Pulmonary Venous Connection in Cause of Right Undiagnosed Ventricular Enlargement

Patients may be referred for cardiology assessment because of an enlarged right ventricle (RV) with no cause apparent on echocardiography. Cardiac MRI can contribute to the management of these patients by detecting sinus venosus defect or partial anomalous pulmonary venous connection (PAPVC). We sought to show how often sinus venosus defect or PAPVC was detected on MRI in patients with an enlarged RV without a previously established definite diagnosis. First cardiac MRI scans obtained over a 4-year period in adults with an undiagnosed cause of RV enlargement were searched for the MRI diagnosis of sinus venosus defect or PAPVC. Thirty-seven patients (25 female, 12 male) met the study criteria. Nineteen patients had a cardiac MRI diagnosis of sinus venosus defect, with PAPVC being present in 95% of those patients. All PAPVCs associated with sinus venosus defect were from the right side. Eleven of the 19 patients with sinus venosus defect underwent surgery at our institution. Sinus venosus defect was confirmed in all 11 cases. Of the 37 patients, 36 had PAPVC, which was right-sided in 27 patients (75%), left-sided in seven patients (19.4%), and bilateral in two patients (5.6%). Three patients had scimitar veins. The common defects associated with PAPVC were sinus venosus defect in 18 patients (50%) and secundum atrial septal defect in six patients (17%). This article about cardiac MRI in adults with sinus venosus defect and PAPVC shows that cardiac MRI can reliably detect and quantify these lesions when other methods have not provided a complete diagnosis for the cause of right heart enlargement.

The impact of interruption of anomalous systemic arterial supply on scimitar syndrome presenting during infancy

We sought to evaluate the impact of interruption of anomalous systemic arterial supply (ASAS) on clinical course and management outcome of scimitar syndrome (SS) presenting during infancy. No systematic study has been reported so far on this subject, although there are conflicting sporadic reports indicating variable effect. Out of 23 children diagnosed to have SS during the past 25 years, 16 symptomatic infants had ASAS. After interrupting ASAS (coil embolization -14, surgical ligation -2), they were prospectively followed up to define their clinical course and management outcome. All 16 infants had sizable ASAS and 9 had variable scimitar vein (SV) stenosis. Fifteen (94%) had pulmonary hypertension and significant left to right shunt. Post intervention, there was variable reduction of shunt in 14 and pulmonary artery pressure in 15 cases. All showed varying clinical improvement. One died of septicemia shortly afterwards. Definitive surgery was deferred for optimal results in seven children for a mean period of 8 months (range 1 month to 3 years). Remaining eight children did not require definitive surgery. One among them had closure of stenosed partial SV by Amplatzer duct occluder ("physiologic correction"). Two children on short term and six children on long term follow-up (mean 5.2 years; range 3.3-10.3 years) are doing well. Interruption of ASAS helps to avoid or defer definitive surgery for SS during infancy. Therefore, we recommend coil embolization of ASAS as initial palliation, and long term surveillance to assess need for further intervention.

Scimitar Syndrome

Scimitar syndrome is a rare congenital anomaly characterized by total or partial anomalous venous drainage of the right lung to the inferior vena cava. The infantile form presents with congestive heart failure and respiratory distress in early months of life. We report here the case of a 6-month-old male child who presented with second episode of respiratory distress and wheeze. Poor response to bronchodilators led to fibreoptic bronchoscopy and computed tomographic scan that revealed bronchial anomalies with aberrant drainage of right lung into the inferior vena cava by scimitar vein. The case illustrates that one of the rare causes of wheezing in an infant could be scimitar syndrome.

Combined Scimitar Syndrome and Interruption of the Inferior Vena Cava Causing Mega-azygous and Hemiazygous Veins

Computed tomography angiography detected scimitar syndrome with venous drainage to the inferior vena cava caudal to an interruption of the inferior vena cava in a 48-year-old woman. The hepatic veins drained to the supra-hepatic portion of the inferior vena cava cephalic to the interruption, which was a short area of atresia. Azygous and hemiazygous veins were massively enlarged since they drained the right lung, abdomen viscera exclusive of the liver and lower extremities. Inferior vena cava interruption by a short, focal atresia has not previously been described. The inferior vena cava immediately caudal to the interruption is presumed to remain patent because of the scimitar vein flow.

Hemoptysis Complicating Scimitar Syndrome: From Diagnosis to Treatment

We report the case of a 24-year-old patient with known scimitar syndrome presenting with hemoptysis. Multidetector CT angiography showed the scimitar vein draining the right lung to the inferior vena cava associated with right lower lobe bronchopulmonary sequestration. The presence of hemoptysis associated with scimitar syndrome is likely secondary to an anomalous systemic arterial blood supply. We treated this patient successfully with transcatheter occlusion of the anomalous feeding arterial vessels.

Interventional Rerouting of Scimitar Vein to Left Atrium Using an Amplatzer Vascular Plug

Scimitar syndrome is a rare congenital anomaly accounting for about 0.5% of all congenital heart diseases. In its most common form, it is characterized by anomalous drainage of one or all right side pulmonary veins into inferior vena cava or rarely into hepatic, portal, or azygous veins. Other associated anomalies can be hypoplasia or agenesis of right lung, hypoplasia of right pulmonary artery, bronchial anomalies, aberrant blood supply and (or) sequestration of right lung, and variety of intracardiac defects which commonly includes atrial or ventricular septal defects and tetrology of Fallot. The usual treatment of these patients is surgical rerouting of Scimitar vein into left atrium (LA) by baffle formation, lobectomy, embolization or ligation of aberrant blood supply and various combinations of these. Nonsurgical and interventional treatments are rarely possible. We report a rare case where complete rerouting of anomalous venous drainage to LA could be achieved by interventional treatment by a simple deployment of a vascular plug in combination with embolization of aberrant vascular supply to the same area, thus avoiding surgical intervention.

Isolated and Complex Scimitar Vein Anomalies and Their Differentiation from the Meandering Right Pulmonary Vein

PurposeFour pediatric patients with isolated (the adult form) and complex (the infantile form) scimitar vein anomalies were reviewed and compared with patients with meandering right pulmonary veins.Materials and MethodsFrom January 1990 to December 2006, 4 female patients, aged 2 days to 3.5 years, with isolated and complex scimitar vein anomalies were retrospectively studied. The clinical features, chest radiographs, echocardiography, magnetic resonance imaging, magnetic resonance angiography, computer tomography, bronchography, cardiac catheterization with angiography, surgery, and autopsy were reviewed to substantiate the diagnosis of isolated and complex scimitar vein anomalies.ResultsClinical manifestations were lung infections with radiographic scimitar signs in 4 patients (3 right; 1 left), respiratory distress, congestive heart failure, pulmonary hypertension and/or cyanosis in 3 patients, dextrocardia, heterotaxy, and/or right atrial isomerism in 2 patients, as well as dextroversion, right bronchial isomerism, bronchial stenosis, and/or sepsis in 1 patient. Two patients with right atrial isomerism expired.ConclusionThe clinical discrimination between scimitar vein anomaly with and without cardiovascular and bronchopulmonary malformations is crucial for the outcomes vary.

Transcatheter occlusion of the isolated scimitar vein anomaly camouflaged under dual pulmonary venous drainage of the right lung by the Amplatzer Ductal Occluder

A 3.5-year-old girl, who presented recurrent pneumonia without discernible cyanosis or desaturation due to isolated scimitar vein anomaly camouflaged by dual pulmonary venous drainage of the right lung, underwent anterograde transvenous occlusion for this abnormal vessel successfully by the Amplatzer Ductal Occluder at 4 years old.

Scimitar syndrome versus meandering pulmonary vein: evaluation with three-dimensional computed tomography

We report two cases of abnormal configuration of the pulmonary venous system of the right lung evaluated with the use of three-dimensional spiral computed tomography (3D-CT). In the first case, an unusual form of scimitar syndrome was detected, consisting of two scimitar veins uniting into a common trunk immediately before their confluence into the inferior vena cava (IVC). The second case concerns a meandering pulmonary vein that followed a circuitous course through the right lung and came in close proximity to the IVC, before draining the entire right lung to the left atrium. In both cases, 3D-CT enabled a non-invasive, detailed anatomic evaluation of the right pulmonary vascular bed.

Scimitar Syndrome

A 56-year-old woman who underwent secundum atrial septal defect closure at the age of 25 years was referred to the Mayo Clinic for exertional dyspnea and symptomatic atrial fibrillation. On examination, she was acyanotic, with an irregularly irregular heart rate of 86 beats per minute and a blood pressure of 140/80 mm Hg. She had a fixed S2 split with a normal apical impulse. During the course of her work-up, transthoracic echocardiography demonstrated anomalous pulmonary venous return of the right upper and lower pulmonary veins to the inferior vena cava just above the diaphragm (Scimitar syndrome). The …

Usefulness of Magnetic Resonance Angiography for Diagnosis of Scimitar Syndrome in Early Infancy

This study evaluated the feasibility and accuracy of the magnetic resonance angiography (MRA) evaluation of scimitar syndrome in early infancy in 4 subjects. MRA imaging was successful, with excellent imaging quality to evaluate the main and lobar pulmonary artery branches, lobar pulmonary veins, scimitar vein, and systemic-pulmonary collateral arteries in all subjects. There was good agreement between findings with MRA, x-ray angiography (n = 3), surgical inspection (n = 3), and autopsy (n = 1).

Isolated Left-sided Scimitar Vein Connecting All Left Pulmonary Veins to the Right Inferior Vena Cava

When the common pulmonary vein fails to develop, the embryonic connections of the pulmonary veins to one or more of the systemic veins almost always persist. Anomalous pulmonary venous connections to the inferior vena cava (IVC) are typically characterized by hypoplasia of the involved pulmonary veins and pulmonary artery, as well as abnormal parenchyma of the involved lung. Such cases have been described as "scimitar syndrome." We report the case of a young female patient in whom all the left pulmonary veins converged into a common vessel that drained into the IVC but who had a normal left pulmonary artery and left lung. Surgical intervention was successful, and our patient is still alive.

Scimitar syndrome

A 66-year-old man presented with a non-resolving right lower lobe infiltrate. The chest radiograph showed prominent central pulmonary arteries (PA) with normal tapering into the periphery and a typical scimitar-shaped shadow in the right lung (figure A, arrows). On auscultation we heard a soft systolic ejection murmur over the pulmonic area and a soft mid-diastolic flow murmur at lower sternum, suggesting a left-to-right shunt. Echocardiography showed a dilated right ventricle (RV) with normal kinetics and a systolic pressure gradient between the right ventricle and atrium of 25 mm Hg. We excluded an atrial septal defect. The left ventricle and all heart valves were normal. Contrast-enhanced electrocardiogram-triggered ultrafast multi-slice CT showed a dilated pulmonary artery (trunk diameter 36 mm), dilated RV and the scimitar vein (SV) at the level of its confluence with the inferior vena cava (IVC) (figure B). In the coronal plane (figure C), the anomalous SV could be visualised in its entire course to the IVC; the picture also shows the dilated PA. The three-dimensional image reconstructed from the CT (figure D) showed that nearly all venous return from the right lung was directed via the anomalous SV to the IVC just above the diaphragm. The left pulmonary veins drained normally into the left atrium (LA). At catheter-isation, a left-to-right shunt ratio of 48% due to the anomalous pulmonary venous drainage through the SV was confirmed. Corrective surgery (redirecting the scimitar vein into the left atrium) was not done in our patient because he had no symptoms, no coexistent cardiovascular anomalies or pulmonary hypertension, and a low shunt ratio.The scimitar syndrome is a congenital anomalous connection of the pulmonary vein with the inferior vena cava. On the chest radiograph, the vein produces a vascular shadow to the right of the heart that descends toward the diaphragm, resembling a scimitar, which is a short curved Turkish sword. In the adult, it is most often a benign anomaly usually discovered as an incidental finding. The scimitar syndrome should be considered in the presence of an atypical right paracardial shadow on the posteroanterior chest radiograph; contrast-enhanced CT with 3-dimensional reconstruction establishes the diagnosis. A 66-year-old man presented with a non-resolving right lower lobe infiltrate. The chest radiograph showed prominent central pulmonary arteries (PA) with normal tapering into the periphery and a typical scimitar-shaped shadow in the right lung (figure A, arrows). On auscultation we heard a soft systolic ejection murmur over the pulmonic area and a soft mid-diastolic flow murmur at lower sternum, suggesting a left-to-right shunt. Echocardiography showed a dilated right ventricle (RV) with normal kinetics and a systolic pressure gradient between the right ventricle and atrium of 25 mm Hg. We excluded an atrial septal defect. The left ventricle and all heart valves were normal. Contrast-enhanced electrocardiogram-triggered ultrafast multi-slice CT showed a dilated pulmonary artery (trunk diameter 36 mm), dilated RV and the scimitar vein (SV) at the level of its confluence with the inferior vena cava (IVC) (figure B). In the coronal plane (figure C), the anomalous SV could be visualised in its entire course to the IVC; the picture also shows the dilated PA. The three-dimensional image reconstructed from the CT (figure D) showed that nearly all venous return from the right lung was directed via the anomalous SV to the IVC just above the diaphragm. The left pulmonary veins drained normally into the left atrium (LA). At catheter-isation, a left-to-right shunt ratio of 48% due to the anomalous pulmonary venous drainage through the SV was confirmed. Corrective surgery (redirecting the scimitar vein into the left atrium) was not done in our patient because he had no symptoms, no coexistent cardiovascular anomalies or pulmonary hypertension, and a low shunt ratio. The scimitar syndrome is a congenital anomalous connection of the pulmonary vein with the inferior vena cava. On the chest radiograph, the vein produces a vascular shadow to the right of the heart that descends toward the diaphragm, resembling a scimitar, which is a short curved Turkish sword. In the adult, it is most often a benign anomaly usually discovered as an incidental finding. The scimitar syndrome should be considered in the presence of an atypical right paracardial shadow on the posteroanterior chest radiograph; contrast-enhanced CT with 3-dimensional reconstruction establishes the diagnosis.

Surgical management of scimitar syndrome: An alternative approach

Objective: The scimitar syndrome is a congenital anomaly that consists in part of total or partial anomalous venous drainage of the right lung to the inferior vena cava. Surgical approaches to the scimitar syndrome have varied according to the anatomic and pathologic features presented in each case. The aim of this study was to present an alternative approach to the surgical correction of scimitar syndrome. Methods: Nine patients with the scimitar syndrome were operated on between 1990 and 2000. They comprised 1 male and 8 female patients (mean age 11.5 ± 17.6 years). All patients had symptoms, with recurrent pneumonia or respiratory tract infections and pulmonary/systemic flow ratios greater than 1.5:1.0. None of the patients had pulmonary hypertension or an atrial septal defect. All patients underwent repair of the anomalous scimitar vein by direct reimplantation into the left atrium without cardiopulmonary bypass. Two patients underwent concomitant resection of a right lower lobe sequestration. Follow-up was complete in all cases. Results: There were no operative or late deaths, and no patients have required reoperation. At the time of follow-up (mean 55 ± 46 months), echocardiography demonstrated a patent anastomosis in all patients without any evidence of restenosis. Conclusion: This clinical experience indicates that an alternative surgical approach to scimitar syndrome is direct anastomosis of the scimitar vein to the posterior aspect of the left atrium using a right thoracotomy without cardiopulmonary bypass. This procedure is safe and effective and obviates the need for long intra-atrial baffles and the use of the extracorporeal circuit.J Thorac Cardiovasc Surg 2003;125:238-45

Scimitar vein anomaly with multiple cardiac malformations, craniofacial, and central nervous system abnormalities in a brother and sister: Familial scimitar anomaly or new syndrome?

The scimitar vein "syndrome" is an anomaly of lobar aplasia or hypoplasia and total or partial anomalous venous drainage of one lung. We report a brother and sister born to nonconsanguineous Italian parents with a fatal infantile form of scimitar vein anomaly associated to multiple cardiac anomalies. The infants had major craniofacial anomalies. In addition, the boy had iris coloboma and enlarged cisterna magna; both sibs showed poor brain myelination at neuroimaging confirmed by histopathology in the girl. The cardiovascular system in the family members was fully investigated and all results were completely normal. The association of the above craniofacial anomalies has been occasionally mentioned in syndromes with anomalous venous return. The familial occurrence of isolated total anomalous pulmonary venous return has been documented in sibs, first cousins, and through consecutive generations. Familial pulmonary hypoplasia, as an isolated finding, has been observed in siblings and twins. To the best of our knowledge, even though five familial cases of scimitar "syndrome" have been described thus far, the constellation of anomalies shown by these two sibs has not been reported previously. It appears that scimitar vein "syndrome" is not a "syndrome" per se: it is most likely an anomaly of heterogeneous etiology. This family may represent its own novel syndrome of multiple congenital anomalies of which scimitar vein is a component.

Obstructed hemianomalous pulmonary venous drainage: is intervention necessary?

Obstructed infradiaphragmatic hemianomalous pulmonary venous drainage is associated with high mortality and morbidity, and treatment is best tailored to the individual patient. Resolution of an obstructed scimitar vein through collateralization has rarely been reported. We report two such cases and review the literature.

Diagnostic Aid of Transesophageal Echocardiography in an Adult Case of Scimitar Syndrome: Confirmation of the Findings at Surgery and Review of the Literature

The diagnosis of scimitar syndrome is straightforward when the scimitar vein is visible on the chest radiograph; however, a diagnosis is not definitive when this vein is obscured by dextroversion, and therefore other investigative modalities are needed. These methods include pulmonary angiography, computed tomographic scan, and magnetic resonance imaging, which have known limitations, and transthoracic echocardiography (TTE) and transesophageal echocardiography (TEE), which are particularly useful because they are noninvasive, versatile, and accurate. TEE is superior to TTE in the diagnosis of this syndrome and other forms of partial anomalous pulmonary venous return. TEE should compliment TTE whenever the latter is of poor quality or the information is incomplete. Moreover, TEE is vital in both the perioperative evaluation of the repair and the confirmation of the patency of the pulmonary venous to left atrial anastomosis. The advantages of TEE are demonstrated by this case report, and its accuracy is confirmed at surgery. (J Am Soc Echocardiogr 1998;11:387-92.)

Anomalous Origin of Left Circumflex Artery in a Scimitar Syndrome

This report concerns a twenty-seven-year-old woman with scimitar syndrome of abnormal origin of the left circumflex artery from the pulmonary artery. Operation was performed to redirect the abnormal venous return to the left atrium and abnormal origin of the left circumflex artery to the aorta. Unfortunately, pulmonary hypertension with hemoptysis occurred nineteen months after the operation. A second angiogram showed total occlusion of the graft to the left circumflex artery and impaired left ventricle function, rather than thrombosis of the anastomosis to the scimitar vein, as the cause of pulmonary hypertension.