Perioperative course in two cases of tetralogy of fallot with portal venous atresia.

We report here two rare surgical cases of tetralogy of Fallot with portal venous atresia. Both patients, previously diagnosed with tetralogy of Fallot, developed portal hypertension and showed hematemesis and/or melena. One patient needed endoscopic sclerotherapy. Intracardiac repair was performed under cardiopulmonary bypass (CPB) with moderate hypothermia. After surgery, one of the patients developed cardiogenic shock due to cardiac tamponade with accumulated chyle in the pericardial sac in the ninth postoperative day, and showed deteriorated liver function, suggesting that liver might have been vulnerable because of the absence of portal blood.

Studies of magnesium in congenital long QT syndrome.

We studied the role of magnesium (Mg) in congenital long QT syndrome (LQTS). Twenty-two congenital LQTS patients and 30 control subjects were included in this study. We measured serum Mg (SMg) level and Mg retention (MgR) level, and evaluated the role of Mg (a high MgR level reflects Mg deficiency in the body). The influence of intravenous Mg infusion on Mg level was evaluated. Relatively low SMg level and high MgR level (LQTS:control = 53:33%, p < 0.01) were recognized in congenital LQTS patients, but there was an overlap with controls. Mg supplementation did not shorten QT interval and there was no significant correlation between Mg levels and QTc interval. Patients with syncopal history showed a higher MgR level (syncope (+):syncope (-) = 70:46%, p < 0.01) and intravenous Mg infusion improved Mg deficiency. These results suggest that some (not all) congenital LQTS patients are in a Mg-deficient state, which may be associated with syncope, and Mg supplementation may prevent recurrent syncope in these patients. Because there are several subtypes of congenital LQTS, perhaps with genetic testing Mg deficiency may be identified as a significant cofactor in some forms, whereas in other forms it is not relevant.

Characteristics of improved NOVA magnesium ion-selective electrode: changes of ionized magnesium values and reference interval in healthy children.

Following the report of interference between the thiocyanate ion (SCN-) and NOVA's previous ion-selective electrode (ISE) for ionized magnesium (iMg2+), NOVA has developed a new ISE which eliminates the effect of SCN-. Two hundred and sixty healthy children were divided into two groups; those who had presented when using NOVA's previous ISE (group A; n = 160) and those using NOVA's new ISE (group B; n = 100). The mean iMg2+ value and the mean iMg2+ percent fraction (iMg2+/serum Mg) were significantly higher in group B than in group A (0.59 +/- 0.03 vs 0.54 +/- 0.03 mmol/L for iMg2+; p < 0.001 and 64.8 +/- 3.1 vs 58.2 +/- 4.1 per cent for iMg2+ percent fraction; p < 0.001). The mean serum SCN- level was 0.023 +/- 0.008 mmol/L in group A (n = 8) and 0.0.21 +/- 0.007 mmol/L in group B (n = 12), and was not significantly different between the two groups. The suspected change of iMg2+ value interfered by SCN- was 0.037 mmol/L in group A. The difference of iMg2+ percent fraction between two groups was higher at high serum magnesium (SMg) levels. The reference interval of iMg2+ was 0.56-0.62 mmo/L in healthy children with the NOVA's new ISE, and was constant irrespective of growth. The NOVA's previous iMg2+ ISE may be interfered with mainly by SCN-. The newly designed ISE eliminated these effects especially at higher SMg levels.

Malignant fibrosarcoma with features of myxoma.

Left atrial tumor, diagnosed as myxoma at first investigation, was later demonstrated to be a malignant fibrosarcoma with myxoid change. Myxoma should be diagnosed and managed carefully because of its wide range of histopathologic features.

Surgical ligation of anomalous hepatic vein in a case of heterotaxy syndrome with massive intrahepatic shunting after modified fontan operation.

In case of progressive cyanosis after modified Fontan procedure, intrahepatic shunting should be considered. Ligation of the hepatic vein is safe and effective in managing this condition, and it is important to ensure that the pressure difference between superior vena cava and the portal vein system remains minimal after clamping of the hepatic vein before ligation is completed to minimize the possibility of varix due to portal vein hypertension.

Hypoplastic left heart syndrome: duration of survival without surgical intervention.

BACKGROUND: Mortality rate for heart transplantation for patients with hypoplastic left heart syndrome (HLHS) has improved, but there is a considerable wait until a suitable donor is available. Thus it is important to examine the duration of survival and risk factors for early death in patients with HLHS who did not undergo surgical intervention. METHODS AND RESULTS: Twenty-six consecutive patients were studied retrospectively. Duration of survival and the 14 following variables were investigated: date of birth, body weight at birth, cardiothoracic ratio, ascending aorta diameter, interatrial communication size, coarctation of the aorta, tricuspid regurgitation, anatomic subtype (patency) of mitral and aortic valve, arterial blood gas findings (pH, PaO 2, SaO 2, PaCO2, base excess), and ST depression in the electrocardiogram. Twenty patients survived <60 days (group A) and 6 patients survived beyond 60 days (group B). The duration of survival (mean [SD]) was 60 (151) days overall (1 patient is currently alive at 783 days). The long-term survivors (beyond 60 days) increased significantly after 1991 (P <.05). Coarctation of the aorta was a significant risk of early death (<60 days) (P <.05). Interatrial communication size was significantly smaller in group B than in group A (P <.05). The mean pH and base excess were significantly lower in group A than in group B. The other 9 variables showed no significant difference between the 2 groups. CONCLUSIONS: There was a significant correlation of long-term survival with stabilized ductal blood flow without coarctation of the aorta, adequate restriction of interatrial communication without severe hypoxemia, and no metabolic acidosis.

Rapid enlargement of cardiac rhabdomyoma during corticotropin therapy for infantile spasms.

OBJECTIVE: To investigate the influence of corticotropin therapy on cardiac rhabdomyoma. DESIGN: Analysis of data from echocardiography performed on in-patients. PATIENTS: Six patients with rhabdomyoma who were admitted to the authors' medical centre with either convulsion (five cases) or prematurity (one case) between 1985 and 1995. Five had tuberous sclerosis. INTERVENTION: Size of cardiac tumours of each patient was measured by echocardiography, and volume index was calculated as the ratio of the tumour volume to its initial volume. MAIN RESULT: Increase in size of some of the tumours was found during corticotropin therapy on follow-up echocardiography. Maximum volume indexes of tumours in the case of patients (n = 4) who did not receive corticotropin therapy was 1.2 to 3.7, whereas those of patients (n = 2) who received therapy was 9.1 to 12; one of the latter patients died. CONCLUSION: Corticotropin may contribute to the enlargement of cardiac rhabdomyoma. The size of cardiac rhabdomyomas must be carefully followed when patients are treated with corticotropin.

[Superior vena cava-right atrial appendage direct anastomosis for repair of partial anomalous pulmonary venous connection to superior vena cava].

Successful repair was performed for a 7-year-old male with a diagnosis of partial anomalous pulmonary venous connection (PAPVC) to superior vena cava (SVC) and superior sinus venosus atrial septal defect (ASD). The SVC was divided above the orifice of the anomalous pulmonary vein and the cephalad end of the SVC was anastomosed directly to the right atrial appendage. A patch was used to divert pulmonary venous flow from the orifice of the SVC through superior sinus venosus ASD into the left atrium. Postoperative course was uneventful with normal sinus rhythm. There was no evidence of vena caval or pulmonary venous obstruction. At 3-month after surgery, sinus node function was confirmed to be normal by electrophysiological study. This is useful alternative method for repair of PAPVC to high or middle SVC.