No abstract available.
Scimitar Syndrome*

No abstract available.
Scimitar Syndrome*

No abstract available.
Heterotaxy Syndrome* ; Scimitar Syndrome*

The scimitar syndrome is a rare malfomation that can be defined as a partial or complete right pulmonary venous return into inferior vena cava immediately above or below the siaphragm. This malfomation is often associated with hypoplasia of right lung, anomalous arterial supply of the lower part of the lung, and cardiac dextroversion. We experienced a case of scimitar syndrome in a 15 years old girl and presenting the case with a brief review of the literature.
Adolescent ; Female ; Humans ; Lung ; Scimitar Syndrome* ; Vena Cava, Inferior

The scimitar syndrome, a rare complex anomaly, is defined as an anomalous right pulmonary venous drainage, partial or complete, to the inferior versa cava. The shape of the Turkish curved sword (scimitar) huts provided the name of this syndrome. Additional characteristics of this syndrome such as hypoplasia of the right lung and of the right pulmonary arterial tree, anomalous arterial supply of the right lung from the aorta, dextrocardia and bronchial anomalies are common. Recently we experienced a case of scimitar syndrome (adult form) in a 19-year-old woman patient, so we report the case with a brief review of the literature.
Aorta ; Dextrocardia ; Drainage ; Female ; Humans ; Lung ; Scimitar Syndrome ; Young Adult

The syndromes of left atrial isomerism and right atrial isomerism, called polysplenia and asplenia syndromes, respectively, consist of congenital heart defects with disturbances in normal left right isometry, and the etiology of atrial isomerism remains unclear. Right atrial isomerism is traditionally associated with severe cardiac defects, especially complete atrioventricular septal defect, transposition of great arteries, pulmonary atresia, and total anomalous pulmonary venous return. Recently, we encountered one case of asplenia diagnosed by prenatal ultrasonography. We report a case with brief review of the literatures.
Heart Defects, Congenital ; Heterotaxy Syndrome ; Isomerism ; Pulmonary Atresia ; Scimitar Syndrome ; Transposition of Great Vessels ; Ultrasonography, Prenatal*

PURPOSE: Total anomalous venous return(TAPVR) is associated in more than 60Yo of patients with right isomerism and can significantly complicate the management of single ventricle patients at any stage of management. We studied the results of management and sought to determine factors that may influence survival in patients with TAPVR in right atrial isomerism. METHODS: Between February 1991 and July 1999, 14 patients with TAPVR in right atrial isomerism underwent operations,' we reviewed our experience after performing single ventricle palliation RESULTS: Seven patients were of the obstructive type TAPVR and seven patients were of the non-obstructive type TAPVR. The mean age at operation was 17 months and mean body weight at operation was 7.3kg. Direct surgical repair for the pulmonary vein was performed in seven patients and in the others, TAPVR could be satisfactorily managed by the use of bilateral cavopulrnonary anastomosis(BCPS) to exclude the distal superior vena cava(SVC). At a mean follow-up of 27 months, there were five deaths, arid pulrnonary vein restenosis developed in four patients in the direct surgical repair group. In the other group, there was neither mortality nor morbidity. Also, the presence of pulmonary venous obstruction was associated with high mortality. CONCLUSION: According to our study, TAPVR can be satisfactorily managed by the use of BCPS to exclude the distal SVC in the non-obstructive type. But further evaluation of surgical methods about other types of TAPVR are warranted, because TAPVR not requiring intervention includes any low supracardiac and some mixed types.
Body Weight ; Follow-Up Studies* ; Heterotaxy Syndrome* ; Humans ; Isomerism ; Mortality ; Pulmonary Veins ; Scimitar Syndrome* ; Veins

BACKGROUND: Preoperative identification of systemic and pulmonary venous return is essential for surgical design in situs ambiguus. This study was carried out to evaluate anatomy of systemic and pulmonary venous return and to assess clinical efficacy of magnetic resonance imagine(MR) by comparing with results of cardiac catheterization(Cath) and echocardiography(Echo). MATERIALS AND METHODS: MR performed on 22 patients with cardiac situs ambiguus(right isomerism ; 13, left isomerism ; 9). MR findings were compared with the findings of Cath and Echo for the assessment of diagnostic accuracy of MR in 19 patients. RESULTS: 1) Interruptions of IVC with azygous continuation were found in all patients of left isomerism. But IVC was drained to right of left sided atria in right isomerism. 2) Brlateral SVC were found in 12 of 22 situs ambiguus(left isomersm ; 5, right isomerism ; 8). 3) Total anomalous pulmonary venous returns(TAPVR) were found in 7 of 12 right isomerism. Location of vertical veins were as follows ; prearterial(n=1), retroarterial-prebronchial(n=3), retrobronchial(n=3). In 5 patients of remained 6 right isomerism, pulmonary venous returns(PAPVR) in which right and left pulmonary veins entered to right and left atrium respectively, were found in 5 of 9 left isomerism. 4) Compared with Cath and Echo findings(n=19) in which MR, Cath and Echo were performed simultaneously, TAPVR were found in 6 cases on MR but 2 cases on Cath and Echo. The cases that were not detected by Cath showed severe decrement of pulmonary flow due to hypoplasia of pulmonary artery or obstruction of pulmonary vein. On MR, accurate anatomy of PAPVR were found only in 4 cases. CONCLUSIONS: MR can provide accurate and complete imaging of systemic and pulmonary venous return in sitrs ambiguus. Especially, MR is superior to Cath or Echo in depiction of TAPVR with severe decrement of pulmonary flow of obstruction of pulmonary vein, PAPVR and bilateral SVC.
Heart ; Heart Atria ; Heterotaxy Syndrome* ; Humans ; Isomerism ; Pulmonary Artery ; Pulmonary Veins ; Scimitar Syndrome ; Veins

Total anomalous pulmonary venous return (TAPVR) is a rare and critical congenital vascular anomaly that requires an early operation. However, initial symptoms of TAPVR may be non-specific, and cardiovascular findings may be minimal. The heart may not be enlarged and there is often no cardiac murmur. Without cardiac murmur, these symptoms are similar to those of respiratory distress syndrome in newborns. Therefore, a high degree of suspicion and an early diagnosis of TAPVR are important. This condition generally occurs without a family history and has a low recurrence rate, but several familial cases, including siblings, have been reported worldwide. Additionally, several chromosomal or gene abnormalities associated with TAPVR have been reported. In the case presented here, two brothers with a 6-year age gap were diagnosed with TAPVR. Surgery was performed without cardiac or neurological complications. This is the first report on TAPVR in siblings in Korea.
Early Diagnosis ; Heart ; Heart Murmurs ; Humans ; Infant, Newborn ; Korea ; Recurrence ; Scimitar Syndrome* ; Siblings*

Although laryngomalacia is the most common cause of congenital stridor in infancy, it is a benign, self-limited disease which usually resolves by the age of 18 months with no long- term sequelae. Nevertheless, infants who suffered from recurrent stridor and respiratory distress should be evaluated for other causes of stridor such as cardiopulmonary anomalies. We report 3 cases of infants who had recurrent stridor and respiratory distress from their early infancy. Case 1 had a double aortic arch and a tracheomalacia, case 2 had a hypoplasia of the right lung, and case 3 had a horseshoe lung as well as scimitar syndrome. Physicians should be alert for the possibility of the congenital cardiopulmonary abnormalities in infants with recurrent stridor and/or respiratory distress.
Aorta, Thoracic ; Dyspnea ; Humans ; Infant ; Laryngomalacia ; Lung ; Respiratory Sounds ; Scimitar Syndrome ; Tracheomalacia