Venovenous malformation: a common finding after Kawashima operation.

OBJECTIVES: It has been reported that systemic venovenous malformation (VVM) can develop in patients with interrupted inferior vena cava (IVC) and univentricular type of congenital heart disease who undergo superior vena cava to pulmonary artery connection (Kawashima operation). These malformations can lead to profound systemic desaturation postoperatively. However, there have been few reports that characterise the prevalence, anatomic details and clinical correlations of these systemic VVM arising after Kawashima operation. In this study, we describe our experience with VVM after Kawashima operation, and discuss issues regarding their evaluation and postoperative management. METHODS: Eight patients with median age 19 months (range: 5-238) who underwent Kawashima operation were subjected to postoperative angiography, prospectively. Sites of VVM origin and entry, as well as their course, were documented. The presence of pulmonary arteriovenous malformations (AVMs) was also documented. RESULTS: At median follow-up of 31 months (range: 16-72 months), a total of 14 VVM were found in different supra- and infra-diaphragmatic sites in six patients (75%); four of them had concomitant pulmonary AVM while the remaining two patients had only pulmonary AVM. CONCLUSIONS: Our findings suggest that systemic VVM can occur frequently after Kawashima operation and can produce significant desaturation postoperatively, and hence we support hepatic incorporation. Performing detailed angiographic studies of the supra- and infra-diaphragmatic systemic veins in routine assessment of patients before Kawashima operation is, probably, warranted.

Hemolytic anemia: an unusual presentation of cor triatriatum sinistrum.

Cor triatriatum is a congenital malformation of the heart which is usually presented with heart failure. We have dealt with an unusual presentation of a two-month-old child with cor triatriatum presented with hemolytic anemia.

Propranolol: a new indication for an old drug in preventing postoperative junctional ectopic tachycardia after surgical repair of tetralogy of Fallot.

Junctional ectopic tachycardia (JET) is a major cause of postoperative morbidity after complete repair of tetralogy of Fallot (TOF). Propranolol is a known medication used in patients with TOF to prevent and control hypercyanotic spells. Despite this, there is little information regarding the relation between preoperative use of propranolol and the incidence of postoperative JET. The aim of this study was to examine the effect of preoperative use of propranolol on the incidence of postoperative JET after full surgical repair of TOF. A retrospective analysis of 109 patients in whom 57 patients received preoperative propranolol (propranolol group) was compared with 52 patients who did not receive propranolol preoperatively (control group). The incidence of postoperative JET was significantly higher in the control group (38%) than the propranolol group (21%) P=0.042. The propranolol group had significantly less mechanical ventilation time, less ICU stay and less total hospital stay than the control group (P<0.05). Our findings suggest that the preoperative use of propranolol may decrease the incidence of JET after full surgical repair of TOF. A prospective randomized study may help to elucidate the exact relationship between the preoperative use of propranolol and the incidence of postoperative JET.

Effect of modified ultrafiltration on pulmonary function after cardiopulmonary bypass.

BACKGROUND: Pulmonary dysfunction is one of the most common manifestations of inflammatory response after cardiopulmonary bypass (CPB). OBJECTIVE: This prospective randomized study was conducted to evaluate the effect of a modified ultrafiltration (MUF) technique on pulmonary function after CPB in children. METHODS: Forty patients weighing from 5 to 10 kg with congenital heart disease who required CPB for primary biventricular operative repair were prospectively randomized into two groups. The control group received conventional ultrafiltration (CUF) during CPB, and the study group received CUF and MUF. Pulmonary compliance (static and dynamic) and gas exchange capacity of the lung expressed as oxygen index, respiratory index, ventilation index, and alveolar-arterial oxygen pressure difference were measured after intubation (baseline), at the termination of CPB, at the end of MUF, on admission to the ICU, and 6 h postoperatively. RESULTS: There was no significant difference in lung compliance and gas exchange between the two groups before CPB. CPB produced a significant decrease in static and dynamic lung compliance in both groups. In the control group, static and dynamic lung compliance decreased from 1.0 +/- 0.3 to 0.90 +/- 0.3 mL/cm/kg and 0.87 +/- 0.2 to 0.71 +/- 0.1 mL/cm/kg (+/- SE) [p = 0.0002 and p = 0.002, respectively]. In the study group, static and dynamic lung compliance decreased from 1.0 +/- 0.2 to 0.89 +/- 0.03 mL/cm/kg and 0.94 +/- 0.2 to 0.77 +/- 0.1 mL/cm/kg (p = 0.002 and p = 0.002, respectively). There was no significant difference in the decrease in static (p = 0.9) or dynamic lung compliance (p = 0.3) between the two groups. MUF produced a significant immediate improvement in both static lung compliance (0.89 +/- 0.2 to 0.98 +/- 0.2 mL/cm/kg, p = 0.03) and dynamic lung compliance (0.77 +/- 0.1 to 0.93 +/- 0.2 mL/cm/kg, p = 0.007). The same was observed regarding the gas exchange capacity. CPB produced a significant decrease in lung gas exchange capacity, and MUF produced a significant immediate improvement in lung gas exchange capacity. The effect of MUF on lung compliance and gas exchange capacity was not sustained after admission to the ICU nor 6 h later postoperatively. There was no significant difference in the time of extubation between the two groups (12 +/- 3 h and 13 +/- 2 h, p = 0.4), the length of ICU stay, or the total hospital stay postoperatively. CONCLUSIONS: The use of MUF after CPB can produce an immediate improvement in lung compliance and gas exchange capacity, which may effectively minimize pulmonary dysfunction postbiventricular repair of congenital heart disease. However, these improvements are not sustained for the first 6 h postoperatively and do not reduce the duration of postoperative intubation, ICU stay, or total hospital stay.