Pulmonary hypertension in infants with congenital heart defects: are leukotrienes involved?

The circulating levels of leukotriene E4 in infants with congenital heart defects, increased pulmonary blood flow and pulmonary arterial hypertension, were determined and compared with infants with decreased pulmonary blood flow (Tetralogy of Fallot). There was no correlation (r=0.38) between the pulmonary arterial pressure (56 ± 4 mmHg) and the leukotriene E4 levels (1.37 ± 0.67 ng/ml blood) measured in peripheral blood samples from the hypertensive group prior to surgery. There was considerable variation in the detectable leukotriene E4 levels in blood samples from different patients. The levels detected in the blood samples between the two groups of patients was similar. These data suggest that neither the surgical repair during cardiopulmonary bypass nor the pulmonary hypertension appeared to modify the leukotriene E4 blood levels in the small number of patients studied

732-1 An Institutional Experience with Second and Third Stage Palliative Procedures for Hypoplastic Left Heart Syndrome: The Impact of the Bidirectional Cavopulmonary Shunt

We reviewed 71 consecutive pts who underwent stage II and III operations following stage I palliation for hypoplastic left heart syndrome (HLHS) at our institution since 1983. 6 surgeons participated in the care of these pts. Follow-up is 97% complete. We examined 17 potential risk factors for mortality, including preoperative anatomic and physiologic factors, and procedural features of the stage II operation. Multivariate analysis revealed that the only significant risk factor for stage II mortality was the performance of a non-fenestrated completion Fontan procedure (p<0.001). There were 9 hospital deaths (69%) in the 13 pts undergoing the Fontan procedure at stage II. In contrast, 49 pts underwent bidirectional cavopulmonary shunting (47) or hemi-Fontan procedure(2) as an intermediate step to the Fontan procedure with 4 (8%) early deaths. The first bidirectional cavopulmonary shunt was performed in this population in 1988. Median age at this stage II procedure was 8.4 months. Surgical augmentation of the pulmonary arteries was performed in 18 (37%) pts at the time of stage II surgery and was not associated with increased operative risk. Also, HLHS anatomic subtype was not a risk factor for stage II mortality or pre-stage III attrition. There have been 2 (4%) intermediate deaths prior to the performance of a stage III procedure, which at our institution is the fenestrated Fontan procedure. This has been performed in 25 pts at a median age of 30 months with 1 early death and no mortality at a median follow-up of 22 months. There are presently 34 HLHS pts who have modified Fontan anatomy following a course of surgical palliation performed entirely at this institution. Follow-up in this group ranges up to 92 months, with a median of 28 months. 33 of these patients are NYHA class 1 or 2.2 pts have required pacemaker implantation, but there have otherwise been no surgical reinterventions in pts who have completed palliation. We conclude that the incorporation of the bidirectional cavopulmonary shunt into a course of surgical palliation for HLHS has dramatically reduced mortality in this challenging group of pts, allowing them to undergo the modified fenestrated Fontan procedure with low operative mortality and good intermediate outcome

The fate of children with microdeletion 22q11.2 syndrome and congenital heart defect: clinical course and cardiac outcome

BACKGROUND: This study aimed to evaluate the cardiac outcome for children with microdeletion 22q11.2 and congenital heart defect (CHD). METHODS: A total of 49 consecutive children with 22q11.2 and CHD were retrospectively identified. The CHD consisted of tetralogy of Fallot and variances (n = 22), interrupted aortic arch (n = 10), ventricular septal defect (n = 8), truncus arteriosus (n = 6), and double aortic arch (n = 1). Extracardiac anomalies were present in 46 of 47 children. RESULTS: The median follow-up time was 8.5 years (range, 3 months to 23.5 years). Cardiac surgical repair was performed for 35 children, whereas 5 had palliative surgery, and 9 never underwent cardiac surgery. The median age at repair was 7.5 months (range, 2 days to 5 years). The mean hospital stay was 35 days (range, 7-204 days), and the intensive care unit stay was 15 days (range, 3-194 days). Significant postoperative complications occurred for 26 children (74%), and surgery for extracardiac malformations was required for 21 patients (43%). The overall mortality rate was 22% (11/49), with 1-year survival for 86% and 5-year survival for 80% of the patients. A total of 27 cardiac reinterventions were performed for 16 patients (46%) including 15 reoperations and 12 interventional catheterizations. Residual cardiac findings were present in 25 patients (71%) at the end of the follow-up period. CONCLUSIONS: Children with microdeletion 22q11.2 and CHD are at high risk for mortality and morbidity, as determined by both the severity of the cardiac lesions and the extracardiac anomalies associated with the microdeletion

Strategies to prevent intraoperative lung injury during cardiopulmonary bypass

During open heart surgery the influence of a series of factors such as cardiopulmonary bypass (CPB), hypothermia, operation and anaesthesia, as well as medication and transfusion can cause a diffuse trauma in the lungs. This injury leads mostly to a postoperative interstitial pulmonary oedema and abnormal gas exchange. Substantial improvements in all of the above mentioned factors may lead to a better lung function postoperatively. By avoiding CPB, reducing its time, or by minimizing the extracorporeal surface area with the use of miniaturized circuits of CPB, beneficial effects on lung function are reported. In addition, replacement of circuit surface with biocompatible surfaces like heparin-coated, and material-independent sources of blood activation, a better postoperative lung function is observed. Meticulous myocardial protection by using hypothermia and cardioplegia methods during ischemia and reperfusion remain one of the cornerstones of postoperative lung function. The partial restoration of pulmonary artery perfusion during CPB possibly contributes to prevent pulmonary ischemia and lung dysfunction. Using medication such as corticosteroids and aprotinin, which protect the lungs during CPB, and leukocyte depletion filters for operations expected to exceed 90 minutes in CPB-time appear to be protective against the toxic impact of CPB in the lungs. The newer methods of ultrafiltration used to scavenge pro-inflammatory factors seem to be protective for the lung function. In a similar way, reducing the use of cardiotomy suction device, as well as the contact-time between free blood and pericardium, it is expected that the postoperative lung function will be improved