We present the case of a 6‐year‐old child who presented with an episode of life threatening hemoptysis. Investigations revealed multiple areas of endobronchial varices and abnormal pleural vessels as well as severe left pulmonary vein stenosis and an atrial septal defect (ASD). After extensive work up and consultation he underwent repair of the left pulmonary vein using a sutureless technique and ASD closure. This resulted in a marked improvement in the appearances of the left lung. The bronchial varices in the right lung remain unchanged. No further hemoptysis has occurred and the child continues to be monitored. Pediatr Pulmonol. 2013; 48:838–840. © 2012 Wiley Periodicals, Inc.

The systemic inflammatory response to cardiac surgery is common, and resultant impairment of multiple organ function is generally mild or subclinical due to physiological reserve within organ systems. Unfortunately, the changing profile of patients referred for surgery suggests that the systemic inflammatory response may prominently influence surgical outcome in the future. Older, co-morbid patients with more limited physiological reserve are being referred for complex lengthy procedures, and paediatric surgery has witnessed a shift to earlier complex primary correction or palliation involving long cardiopulmonary bypass times or a period of suboptimal organ perfusion using circulatory arrest or low flow cardiopulmonary bypass. Unique to cardiac surgery is the predictability of the inflammatory response, but prophylactic therapies have not translated into clinical benefit, which the preconditioning phenomenon may address.

Background: Inappropriate systemic inflammatory endothelial and leucocyte activation is a major pathogenic component of post-operative low cardiac output syndrome (LCOS) and associated pulmonary oedema, low urine output, and generalised oedema, in paediatric cardiac surgery. We hypothesised that acute pre-treatment with a parenteral nutrition component omega-3 fatty acid infusion in-vitro, would prevent the pattern of leucocyte and endothelial activation seen in paediatric cardiac surgery through induction of preconditioning. Methods: Endothelial cells were isolated from saphenous vein of 75 adult cardiac surgery patients, grown in monolayers, pre-treated, and then stimulated with either endotoxin (LPS), TNFalpha, or complement C5a (prime mediators of systemic inflammation with paediatric cardiac surgery). Endothelial production of the neutrophil recruiting cytokines IL-6, IL-8, and endothelial expression of the neutrophil adherence receptors E-Selectin, ICAM-1 were assessed. The effects on the inflammatory pro-coagulant response of the endothelium i.e. loss of surface thrombomodulin and production of tissue factor were also examined. Similar methods were employed using isolated peripheral blood neutrophils. The effects on endothelial nuclear translocation of the acute inflammatory transcription factor NFkB and the heat shock protein (HSP72) response were investigated for mechanistic insight. Results: Four hours pre-treatment with the omega-3 infusion prevented endothelial release of neutrophil recruiting cytokines IL-6 and IL-8, also prevented upregulation of endothelial receptors required for neutrophil adherence E-Selectin and ICAM-1, and maintained surface anticoagulant thrombomodulin levels, in response to stimulation with LPS (Table 1) or TNFalpha.

Objectives: Omega-3 fatty acids exhibit anti-inflammatory, antithrombotic, and antiarrhythmic properties. We investigated the extent and underlying mechanism of protection conferred by a pre-emptive omega-3 infusion in a model of regional cardiac ischemia-reperfusion injury.Methods: New-Zealand White rabbits received either the omega-3 infusion or a control infusion of 0.9% saline ( n = 14 in each group). The large marginal branch of the left coronary artery was occluded for 30 minutes, cardiac function was assessed during 3 hours of reperfusion, and infarct size was measured. Pretreatment-induced alterations in myocardial membrane fatty acid composition and intramyocardial heat shock protein 72 were additionally assessed ( n = 5 in each group). Serum markers of myocardial membrane oxidative stress, malonaldehyde and 8-isoprostane, were also determined. Results are expressed as means +/- standard error of the mean and significance was tested with analysis of variance.Results: Pretreatment increased myocardial membrane omega-3 fatty acid content 5-fold, from 0.94% +/- 0.07% in controls to 5.38% +/- 0.44% in the omega-3 group ( P < .01), and it produced a 225% elevation of levels of heat shock protein 72 ( P = .019) before ischemia-reperfusion. This was associated with a 40% reduction in infarct size ( P < .01). Whereas the reperfusion-induced rise in malonaldehyde levels was higher with omega-3 pretreatment, 10.2 +/- 1.5 mu mol/L versus 6.1 +/- 0.7 mu mol/L in controls ( P = .04), 8- isoprostanes showed a 9-fold reduction, 679 +/- 190 pg/mL in controls vs 74 +/- 45 pg/mL in the omega-3 group ( P= .0077).Conclusions: A pre-emptive omega-3 infusion significantly reduces infarct size through the dual mechanisms of upregulation of heat shock protein 72, a key preconditioning protein, and a dramatic increase in the omega-3 content of myocardial membranes, which appears to facilitate a shift in oxidant ischemia-reperfusion injury. Further study to optimally shorten the pretreatment regimen for this potentially acceptable infusion will now be pursued.

Although cardiopulmonary bypass (CPB) is required in all surgical procedures involving open-heart surgery, the extent to which CPB alters pulmonary vascular regulation has not been systematically investigated. Our objectives were to investigate the acute, subacute, and chronic effects of CPB on the left pulmonary vascular pressure-flow (LP-Q) relationship in conscious dogs. Continuous LP-Q plots were generated in chronically instrumented conscious dogs 2-4 days pre-CPB and again 4 h and 1, 2, 7, and 14 days after 2.5 h of closed-chest hypothermic CPB. In addition, pulmonary vascular reactivity was assessed by examining the dose-response relationship to the thromboxane analogue U-46619 pre- and post-CPB. CPB resulted in an acute (4 h post-CPB) shift in the baseline LP-Q relationship, indicating an increase in pulmonary vascular resistance (P < 0.01). The baseline LP-Q relationship returned to pre-CPB values by 1 day post-CPB. Despite this return of the baseline LP-Q relationship to pre-CPB values, the pulmonary vasoconstrictor response to U-46619 was markedly potentiated 2 days post-CPB compared with the pre-CPB response (P < 0.01). This enhanced pulmonary vasoconstrictor response to U-46619 was still apparent 7 days post-CPB (P < 0.01) but was not evident 14 days post-CPB. These results indicate that CPB results in a pronounced, but transient, increase in pulmonary vascular resistance. Moreover, CPB causes a protracted increase in pulmonary vascular reactivity even when the baseline LP-Q relationship has returned to pre-CPB values.(ABSTRACT TRUNCATED AT 250 WORDS)