| The rare association, in a left-sided heart with hypoplastic left heart syndrome, of right aortic arch, bilateral patent arterial ducts and origin of the left subclavian artery from the left pulmonary artery are described. cardiac catheterization was performed because of the abnormal anatomy of the arch noted at echocardiographic examination. This abnormality is of surgical importance when planning the Norwood operation. ( info) |
2/145. Familial association of congenital left heart abnormalities and sustained fetal arrhythmia. hypoplastic left heart syndrome (HLHS) is the most common cause of death from heart disease in the first week of life. There are reports about familial concordance by presumed morphogenetic mechanisms of abnormal embryonic blood flow with phenotypes of varying severity. The risk of having a child with a left heart lesion after a previously affected child may be as high as 5% to 12%. We present case reports from four families in which sustained fetal arrhythmia (three ectopic atrial tachycardias and one severe bradycardia due to excessive ectopic atrial beats) was demonstrated. Within these four families a close relative of the mother (a previous child, a brother, or a nephew) had severe left heart abnormality (three with HLHS and one with severe aortic valve stenosis). The association of sustained fetal arrhythmia of ectopic atrial origin and severe left heart abnormalities could be expected to occur by chance in a very low percentage of cases. We conclude that sustained fetal atrial ectopic arrhythmia is a congenital abnormality and should be considered as a risk factor for inherited congenital heart abnormalities. ( info) |
3/145. Norwood procedure without circulatory arrest. In the Norwood procedure for hypoplastic left heart yndrome, the distal descending thoracic aorta was cannulated just superior to the diaphragm through median sternotomy. In combination with cerebral perfusion through the graft anastomosed to the innominate artery, which was used as a systemic-to-pulmonary shunt later, this technique enabled us to completely avoid circulatory arrest and deep hypothermia throughout the operation. ( info) |
4/145. Atrioventricular canal defect and hypoplastic left heart syndrome as discordant congenital heart defects in twins. We report on a twin pair presenting with atrioventricular canal defect (AVCD) with right ventricular dominance in one twin, and classic hypoplastic left heart syndrome (HLHS) in the other. According to the developmental-mechanistic approach, AVCDs belong to the group of extracellular matrix abnormalities, whereas classic HLHS is included among flow lesions. Twin pairs with congenital heart defect (CHD) generally have concordant defects by mechanistic group. The occurrence of AVCD and classic HLHS in twins or siblings has never been reported. Interestingly, hypoplasia of the left ventricle is the anatomic characteristic which unifies the discordant CHDs observed in our twins. The occurrence of CHD in both members of the twin pair implies a strong influence of genetic factors. At present, the genetic basis determining the different cardiac phenotypes observed in our twins is unknown. The report of these peculiar associations may be useful to stimulate further studies and shed light on the etiology of CHDs. ( info) |
| 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. ( info) |
6/145. Pulmonary failure after Norwood procedure: indication for extracorporeal membrane oxygenation? A case report. Today some authors consider univentricular repair a contraindication for postoperative cardiac extracorporeal membrane oxygenation (ECMO). The question is whether or not ECMO is indicated as pulmonary support in case of an overwhelming pulmonary infection during the postoperative course after a Norwood procedure. During the prolonged weaning period after a Norwood procedure using a 4 mm aortopulmonary shunt, proven respiratory syncytial virus (RSV) bronchiolitis occurred at the time of expected weaning from artificial ventilation. Venovenous ECMO was able to improve oxygenation, but when pulmonary opacification failed to resolve, ECMO was terminated after 12 days. ( info) |
7/145. FISH studies in 45 patients with rubinstein-taybi syndrome: deletions associated with polysplenia, hypoplastic left heart and death in infancy. rubinstein-taybi syndrome (RTS) is a dominant Mendelian disorder characterised by mental retardation, a typical facies, broad thumbs and short stature. Previous reports indicated that 4-25% of RTS patients have a submicroscopic 16p13.3 deletion of the CBP gene. Using FISH and cosmid probes RT100, RT191 and RT203 we studied 45 RTS patients from germany, the czech republic, austria and turkey and found four deletions (8.9%, pooled data including other studies: 11%). All deletions were interstitial; three spanned the CBP gene (RT100-RT203) and one was smaller (RT100 only). Previous studies reported no phenotype-genotype correlation between RTS patients with or without a deletion. Our findings suggest a more severe phenotype. The mean age at presentation was 0.96 years in patients with a deletion as against 11.12 years in those without. patients A and B with a deletion died in infancy which is rare in RTS and was not observed among the other patients. patients A and D had accessory spleens, Patient A with hypoplastic left heart, abnormal pulmonary lobulation and renal agenesis. This is the second report of hypoplastic left heart and the first report of polysplenia with RTS. The signs suggest a developmental field defect (disturbance of laterality) either as a newly recognised pattern of RTS, or alternatively a novel contiguous gene syndrome. ( info) |
8/145. Transcatheter management of innominate vein thrombosis prior to bidirectional cavopulmonary anastomosis. Innominate vein thrombosis was diagnosed in an 8-months-old infant after stage I surgery for hypoplastic left heart syndrome. cardiac catheterization identified venous collaterals bypassing the innominate vein via the azygous vein. Coil occlusion of the distal azygous vein allowed a subsequent cavopulmonary anastomosis directing collateral flow into the pulmonary bed. Cathet. Cardiovasc. Intervent. 49:61-63, 2000. ( info) |
| The presence of a restrictive atrial septal defect in hypoplastic left heart syndrome represents a surgical emergency and may negatively affect survival after operation. A neonate with such a disease association, requiring septectomy upon birth developed intractable respiratory failure due to congenital pulmonary lymphangiectasis. The therapeutic implications of this rare pathologic condition are discussed. ( info) |
10/145. Repair of aortic atresia and hypoplastic left heart syndrome without using graft material. We present a modification of the Norwood stage 1 operation, where the neo-aorta was reconstructed without using graft material. After extensive mobilization of the descending aorta the ductus arteriosus was excised and an end-to-end anastomosis was created between the main pulmonary artery and the distal ductal-aortic junction. The proximal ascending aorta was anastomosed side-to-side to the neo-aorta. Finally either a direct side-to-side anastomosis was created or a 3.5-mm Gore-Tex graft was implanted between the innominate artery and the right pulmonary artery. The first three patients had a favourable outcome: echocardiography showed good ventricular function and acceptable saturation (85%) at the follow up. ( info) |