No abstract available.
Heterotaxy Syndrome*

No abstract available.
Heterotaxy Syndrome*

No abstract available.
Heterotaxy Syndrome*

No abstract available.
Heterotaxy Syndrome*

No abstract available.
Heterotaxy Syndrome* ; Scimitar Syndrome*

Heterotaxy is defined as an abnormality where the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body. This broad term includes patients with a wide variety of very complex cardiac lesions. Patients with heterotaxy can be stratified into the subsets of asplenia syndrome and polysplenia syndrome, or the subsets of heterotaxy with isomerism of the right atrial appendages and heterotaxy with isomerism of the left atrial appendages. Treatment of patients with isomerism is determined by the nature and severity of the associated cardiac and extracardiac lesions. Most cardiac operations for patients with isomerism are palliative in nature, since normal anatomy is rarely achieved and mortality rates remain high for patients with heterotaxy syndrome. Patients with left isomerism in general have less severe cardiac malformations than those with right isomerism and, hence, more chance of biventricular repair. For almost all patients with right isomerism, and for many with left isomerism, biventricular repair will not be feasible, and all palliative protocols are then staging procedures prior to a Fontan-type repair. Recent advances in medical management, and improvements in surgical techniques have resulted in improved survival for these patients, and the surgical outcomes are comparable to those with Fontan circulation irrespective of the presence or absence of heterotaxy.
Atrial Appendage ; Axis, Cervical Vertebra ; Heterotaxy Syndrome ; Humans ; Isomerism

Atrial Fibrillation/surgery* ; Catheter Ablation ; Heterotaxy Syndrome/surgery* ; Humans

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

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

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*