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.

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.