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At least 19 recordsLinked to original sources
PubMed1987

A case with pseudo-scimitar syndrome: "scimitar sign" with normal pulmonary venous drainage.

The case reported here showed a radiological appearance of hypoplasia of the right lung, dextroposition of the heart, and a curved vascular shadow in the right lower lung field known as a scimitar sign. However, a computed tomography of the chest showed this abnormal vascular shadow draining into the left atrium (pseudo-scimitar sign). Therefore, in patients with a radiological appearance of the scimitar syndrome, computed tomography of the chest should be indicated to rule out the pseudo-scimitar sign.

Cardiac Catheterization↗
PubMed1996

Antenatal sonographic findings associated with scimitar syndrome.

Scimitar syndrome is a rare congenital anomaly that consists of secondary dextroposition in situs solitus, due to right pulmonary hypoplasia. The prenatal sonographic findings of this abnormality have not been previously described. We describe early sonographic findings in a fetus that included isolated cardiac dextroposition with normal abdominal situs, hydramnios and mild narrowing of the right pulmonary artery. Postnatally, the infant developed heart failure, and a right hypoplastic lung was found. Catheterization revealed collateral supply to the right lung arising from the descending aorta and anomalous drainage of right pulmonary veins to the inferior vena cava. The diagnosis of scimitar syndrome was therefore established. A coil embolization of arterial collaterals to the right lung was performed. Scimitar syndrome should be considered in a fetus with the sonographic findings of a right shift of the mediastinal structures, an intact diaphragm and narrow right pulmonary artery. This may allow early neonatal stabilization and treatment.

Adult↗
PubMed2006

Scimitar syndrome.

Scimitar syndrome is a rare congenital anomaly consisting in part of right pulmonary venous return to the inferior vena cava. There is a clear bimodal presentation of this syndrome with either an infantile manifestation or a pediatric/adult form. The infantile variant is marked by a higher incidence and severity of associated defects, heart failure, pulmonary hypertension, and significant mortality. The patient with the pediatric/adult form is less severely affected and may be asymptomatic on diagnosis. In this article, we review the historical aspects, presentation, and pathophysiology of Scimitar syndrome and discuss available treatment strategies. We emphasize the safe and effective approach developed at Indiana University that obviates both the need for an intra-atrial baffle or use of cardiopulmonary bypass. The results with our alternative approach to Scimitar syndrome are summarized and they compare favorably with other published reports.

Adult↗
PubMed2003

A novel operative approach to scimitar syndrome.

Scimitar syndrome is a rare congenital cardiac anomaly defined by an anomalous right pulmonary vein draining the right lung to the inferior vena cava. We describe a unique operative approach performed on 2 patients with infantile scimitar syndrome.

Cardiopulmonary Bypass↗
PubMedDate not supplied

Embolization of the systemic arterial supply via a detachable silicon balloon in a child with scimitar syndrome.

Scimitar syndrome is a rare congenital disorder. It is characterized by partial or total abnormal venous drainage of the right lung into the inferior vena cava, which is often associated with anomalous systemic arterial supply to the right lung, congenital cardiac anomalies, hypoplasia of the right lung and bronchial anomalies. Symptoms depend on the degree of the shunt and severity of the associated anomalies, which determine the treatment. We present a 6-year-old boy who was diagnosed as having the adult form of scimitar syndrome during evaluation for recurrent pulmonary infections, and underwent embolization with a detachable silicon balloon of the anomalous systemic arterial supply from the abdominal aorta to the right lower lung lobe. Successful elective surgery was performed 6 months later, in which right pulmonary veins were directed to the left atrium using a Gore-Tex patch by creating an intra-atrial tunnel. The patient has been symptom-free period during 6 months of follow-up, which supports the idea that recurrent pulmonary infections can be eliminated by embolization of the anomalous arterial supply.

Aorta, Abdominal↗
PubMed1994

[A case of partial anomalous pulmonary venous return to inferior vena cava and left atrium (Scimitar syndrome)].

Scimitar syndrome is relatively uncommon syndrome. A 14-year-old female referred to our hospital for abnormal shadow of chest fluoroscopic examination. She had no symptom except cough due to bronchial asthma. Chest X-ray film showed Scimitar sign. Cardiac catheterization revealed 30% left to right shunt without atrial septal defect. Pulmonary angiography showed anomalous pulmonary vein was drained to inferior vena cava and left atrium. Surgical correction was attempted monitoring the pulmonary arterial pressure. Anomalous pulmonary vein and systemic arteries were ligated and divided at the level of diaphragm. Postoperative course was smooth. We have found 4 similar operated cases previously reported in the world.

Adolescent↗
PubMed2003

A case series of five infants with scimitar syndrome.

Scimitar syndrome is a rare association of congenital cardiopulmonary anomalies consisting of a partial anomalous pulmonary venous connection of the right lung to the inferior vena cava, right lung hypoplasia, dextroposition of the heart, and anomalous systemic arterial supply to the right lung. To date, this syndrome has not been reported or discussed in the nursing literature. In this article, 5 patients are presented to illustrate the variable presentation of this syndrome, ranging from infants who are asymptomatic to those with heart failure and severe pulmonary hypertension. The clinical signs and symptoms, diagnostic dilemmas, and medical and surgical management are discussed. The triad of respiratory distress, right lung hypoplasia, and dextroposition of the heart should alert the clinician to the possibility of this syndrome.

Biomarkers↗
PubMed2005

Scimitar syndrome.

Scimitar syndrome, a rare anomaly of partial pulmonary venous drainage into the hepatic portion of the inferior vena cava, detected in a ten-day-old neonate who presented clinically with respiratory distress and diagnosed on ultrasound of the thorax is being reported here.

Fatal Outcome↗
PubMed2004

Scimitar vein draining to the left atrium and a historical review of the scimitar syndrome.

Scimitar vein draining to the left atrium is the rarest of the pulmonary venolobar anomalies with less than a dozen reported cases. A case is reported of a patient whose plain film radiographic findings showed a typical scimitar vein but whose echocardiography showed normal pulmonary venous drainage. Cardiac catheterization confirmed drainage of the scimitar vein to the left atrium and systemic arterial supply which was embolized. The case described is used to review the history of "scimitar syndrome" with reflections on the significant contributions of Halasz and colleagues, who in 1956 helped define the anatomy, and Neill and colleagues, who in 1960 described the familial occurrence and clinical spectrum of the condition.

Cardiac Catheterization↗
PubMed2005

Scimitar syndrome.

Scimitar syndrome is a relatively uncommon constellation of cardio-pulmonary anomalies, its typical feature being partial anomalous pulmonary venous connection. It can present in the neonatal period as well as later in life. We present the case of a girl diagnosed in the newborn period, along with a brief review of literature.

Fatal Outcome↗
PubMed2004

Bilateral scimitar syndrome.

Scimitar syndrome is a rare congenital anomaly that affects classically the right lung and the heart. We present a rare variant that involves both lungs in association with totally anomalous pulmonary venous connection, horseshoe lung, and right pulmonary cyst.

Cysts↗
PubMed1999

Coil occlusion of aortopulmonary collateral arteries in an infant with scimitar syndrome.

Scimitar syndrome in infancy is a rare condition, presenting with severe congestive heart failure and pulmonary hypertension. The presence of large systemic-pulmonary collateral arteries may play a role in the cause of heart failure and pulmonary hypertension. A 4-month-old infant underwent coil occlusion of large anomalous systemic arteries supplying the right lower pulmonary lobe. Symptoms of severe congestive heart failure and pulmonary hypertension improved dramatically with coil occlusion, and surgical correction was performed 3 months later without any complications. Coil occlusion of anomalous systemic arteries can improve symptoms of heart failure and pulmonary hypertension in infants and may bring about a good surgical result for this disease.

Collateral Circulation↗
PubMedDate not supplied

Echocardiographic clues and accuracy in the diagnosis of scimitar syndrome.

Scimitar syndrome was diagnosed in 27 patients seen between July 1974 and May 1993. All available echocardiograms taken before surgery or death were reviewed. Age at presentation ranged from 1 day to 14 years. Dextrocardia or mesocardia was noted in 70%, atrial septal defect in 70%, and increased right ventricular dimension in 70% of the patients. The ratio of the proximal and distal diameters of the right/left pulmonary arteries were reduced 0.68 +/- 0.17 and 0.66 +/- 0.17, respectively. "Blunting" of the right side of the left atrium was seen in all patients with total anomalous right pulmonary venous drainage and none with partial drainage. Anomalous right pulmonary venous drainage was characterized in 91% of echocardiograms with color flow mapping versus 14% without color flow mapping (p < 0.0002). Aortopulmonary collateral arteries were detected in all four cases in which color flow mapping was performed, but not detected otherwise.

Adolescent↗