Surgical approaches to the blalock shunt: does the approach matter?

OBJECTIVE: The Blalock-Taussig (BT) shunt is an excellent palliative procedure for cyanotic congenital heart defects. We reviewed two techniques of performing the BT shunt, median sternotomy and thoracotomy, in relation to morbidity and mortality. METHODS: Forty-five modified BT shunts in 41 patients, mean age 93 days (1-1045 days), were performed between January 2002 and October 2004. Twenty-four (53.3%) shunts in 21 (51.2%) patients were performed through thoracotomy and 21 (46.7%) shunts in 20 (48.8%) patients through median sternotomy. One surgeon preferred thoracotomy and the other sternotomy approach irrespective of age/weight or elective/emergency. Thirty-eight (84.4%) cases underwent elective operation and 7 (15.6%) cases were operated as emergencies. In both groups the most frequent diagnosis was complex Tetralogy of Fallot. RESULTS: Postoperative oxygen saturation was same in both groups and there were no significant complications in either group. Patients undergoing BT shunt via median sternotomy approach had longer duration of ventilation (mean 183 h vs. 53 h, P<0.001) and inotropic requirements (33.3% vs. 4.2%, P<0.05) leading to longer intensive care unit stay (mean 9.14 days vs. 3.3 days, P<0.05) and hospital stay (mean 14.59 days vs. 5 days P<0.005). CONCLUSIONS: Median sternotomy approach to performing BT shunt seems to carry a higher morbidity than thoracotomy. We recommend a large case series study and longer follow up.

Pulmonary atresia with intact ventricular septum associated with aortic coarctation.

Pulmonary atresia with intact septum is itself a rare congenital abnormality, albeit known to be associated with other cardiac and non-cardiac anomalies. The combination of right- and left-sided obstructive lesions, however, is extremely rare. We describe a patient having pulmonary atresia with intact septum associated with aortic coarctation, which to the best of our knowledge has been previously described on but one occasion.

Diagnosis and management of individuals with Fetal Valproate Spectrum Disorder; a consensus statement from the European Reference Network for Congenital Malformations and Intellectual Disability.

BACKGROUND: A pattern of major and minor congenital anomalies, facial dysmorphic features, and neurodevelopmental difficulties, including cognitive and social impairments has been reported in some children exposed to sodium valproate (VPA) during pregnancy. Recognition of the increased risks of in utero exposure to VPA for congenital malformations, and for the neurodevelopmental effects in particular, has taken many years but these are now acknowledged following the publication of the outcomes of several prospective studies and registries. As with other teratogens, exposure to VPA can have variable effects, ranging from a characteristic pattern of major malformations and significant intellectual disability to the other end of the continuum, characterised by facial dysmorphism which is often difficult to discern and a more moderate effect on neurodevelopment and general health. It has become clear that some individuals with FVSD have complex needs requiring multidisciplinary care but information regarding management is currently lacking in the medical literature. METHODS: An expert group was convened by ERN-ITHACA, the European Reference Network for Congenital Malformations and Intellectual Disability comprised of professionals involved in the care of individuals with FVSD and with patient representation. Review of published and unpublished literature concerning management of FVSD was undertaken and the level of evidence from these sources graded. Management recommendations were made based on strength of evidence and consensus expert opinion, in the setting of an expert consensus meeting. These were then refined using an iterative process and wider consultation. RESULTS: Whilst there was strong evidence regarding the increase in risk for major congenital malformations and neurodevelopmental difficulties there was a lack of high level evidence in other areas and in particular in terms of optimal clinical management.. The expert consensus approach facilitated the formulation of management recommendations, based on literature evidence and best practice. The outcome of the review and group discussions leads us to propose the term Fetal Valproate Spectrum Disorder (FVSD) as we feel this better encompasses the broad range of effects seen following VPA exposure in utero. CONCLUSION: The expert consensus approach can be used to define the best available clinical guidance for the diagnosis and management of rare disorders such as FVSD. FVSD can have medical, developmental and neuropsychological impacts with life-long consequences and affected individuals benefit from the input of a number of different health professionals.

Expert consensus document: Clinical and molecular diagnosis, screening and management of Beckwith-Wiedemann syndrome: an international consensus statement.

Beckwith-Wiedemann syndrome (BWS), a human genomic imprinting disorder, is characterized by phenotypic variability that might include overgrowth, macroglossia, abdominal wall defects, neonatal hypoglycaemia, lateralized overgrowth and predisposition to embryonal tumours. Delineation of the molecular defects within the imprinted 11p15.5 region can predict familial recurrence risks and the risk (and type) of embryonal tumour. Despite recent advances in knowledge, there is marked heterogeneity in clinical diagnostic criteria and care. As detailed in this Consensus Statement, an international consensus group agreed upon 72 recommendations for the clinical and molecular diagnosis and management of BWS, including comprehensive protocols for the molecular investigation, care and treatment of patients from the prenatal period to adulthood. The consensus recommendations apply to patients with Beckwith-Wiedemann spectrum (BWSp), covering classical BWS without a molecular diagnosis and BWS-related phenotypes with an 11p15.5 molecular anomaly. Although the consensus group recommends a tumour surveillance programme targeted by molecular subgroups, surveillance might differ according to the local health-care system (for example, in the United States), and the results of targeted and universal surveillance should be evaluated prospectively. International collaboration, including a prospective audit of the results of implementing these consensus recommendations, is required to expand the evidence base for the design of optimum care pathways.

FloWatch versus conventional pulmonary artery banding.

OBJECTIVE: We sought to compare the efficacy of conventional pulmonary artery banding with that of FloWatch pulmonary artery banding. METHODS: Forty consecutive infants underwent conventional pulmonary artery banding (n = 20; mean age, 1.8 +/- 1.5 months; mean weight, 3.7 +/- 0.7 kg) or FloWatch pulmonary artery banding (n = 20; mean age, 2.6 +/- 1.3 months; mean weight, 4.1 +/- 0.8 kg). Indications were preparation for biventricular repair in 16 of 20 infants, univentricular repair in 2 of 20 infants, and left ventricular retraining in 2 of 20 infants in the conventional pulmonary artery banding group versus 13 of 20, 5 of 20, and 2 of 20 infants, respectively, in the FloWatch pulmonary artery banding group. Twelve of 20 infants required preoperative mechanical ventilation in the conventional pulmonary artery banding group (mean duration, 3.3 +/- 4.3 days) versus preoperative mechanical ventilation required by 14 of 20 in the FloWatch pulmonary artery banding group (mean duration, 17.5 +/- 19.0 days; P < .005). RESULTS: There were 3 early and 2 late deaths after conventional pulmonary artery banding (mean follow-up, 10.8 +/- 9.6 months; range, 1-33 months) versus no early and 2 late deaths after FloWatch pulmonary artery banding (mean follow-up, 13.4 +/- 10.4 months; range, 1-38 months). Postoperative mechanical ventilation and intensive care unit and hospital stays were significantly longer after conventional pulmonary artery banding than those after FloWatch pulmonary artery banding, respectively (10.4 +/- 11.2 vs 3.0 +/- 3.1 days [P < .01], 20.3 +/- 19.9 vs 5.3 +/- 4.6 days [P < .005], and 45.6 +/- 41.6 vs 15.4 +/- 6.4 days [P < .005]). Reoperation to adjust the band was required in 7 (35%) of 20 infants after conventional pulmonary artery banding, whereas no reoperations were required after FloWatch pulmonary artery banding (P < .005). Average cost of stay in the intensive care unit and hospital was, respectively, $45,000 and $45,300 cheaper with FloWatch pulmonary artery banding than average cost with conventional pulmonary artery banding, largely surpassing the cost of the device ($10,000). CONCLUSIONS: FloWatch pulmonary artery banding appears superior to conventional pulmonary artery banding because (1) reoperations are not required; (2) postoperative management is simplified and postoperative mechanical ventilation, stay in the intensive care unit, and stay in the hospital are significantly reduced; and (3) the reduction in costs of postoperative mechanical ventilation, stay in the intensive care unit, and stay in the hospital significantly outweigh the cost of the device.

Aortopulmonary collateral arteries in a child with trisomy 21.

We describe an infant born prematurely at 30 weeks gestation with Down's syndrome who became dependent on oxygen at 3 weeks of age after an uneventful initial neonatal period. There had been no evidence of bronchopulmonary dysplasia. An isolated aortopulmonary collateral artery of moderate size was mistakenly diagnosed as persistent patency of the arterial duct on echocardiography, and subsequently successfully occluded using two coils with an excellent clinical result. To the best of our knowledge, this is the first description of a congenital aortopulmonary collateral artery in a symptomatic infant with Down's syndrome and no evidence of bronchopulmonary dysplasia. We discuss the possible etiologies of these collateral arteries. It is important to include aortopulmonary collateral arteries in the differential echocardiographic diagnosis of an arterial duct.