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Background Patients with antegrade pulmonary blood flow after a bidirectional cavopulmonary shunt (Glenn) may have better pulmonary artery growth. This study evaluated pulmonary artery growth in patients with and without prior additional pulsatile antegrade flow in a Glenn shunt at midterm follow-up after a Fontan procedure. Methods We reviewed 212 patients who had single-ventricle palliation in a 10-year period;103 (33 in pulsatile group 1 and 70 in nonpulsatile group 2) were selected for analysis. Data on demographics, procedures, perioperative course, and midterm follow-up after the Fontan procedure were compared. Echocardiography data were collected. Pulmonary artery sizes measured at cardiac catheterization and follow-up echocardiograms were used to calculate the Nakata index. Results Perioperative details were comparable in both groups, mean pulmonary artery pressure and systemic oxygen saturations were higher in group 1 compared to group 2. Venovenous collaterals were increased in group 1. There was a significant difference in the pre-Fontan and follow-up Nakata index between groups. There was a significant increase in the Nakata index in group 1 between the pre-Glenn and pre-Fontan assessments as well as the Nakata index between the pre-Fontan and midterm follow-up. There was no significant change in the Nakata index in group 2 between assessments. Conclusions A pulsatile Glenn shunt is associated with better pulmonary artery growth which continues long after the additional pulsatile flow is eliminated. It is possible that the effects of anterograde pulmonary blood flow on pulmonary artery growth in early life continue long after the Fontan completion.

A prenatally diagnosed fetus with hypoplastic left heart syndrome and intact atrial septum was delivered in the cardiac catheterization suite. Using radio frequency energy, a transseptal perforation of the thickened and intact atrial septum was immediately performed following transcatheter cannulation of the right atrium via the umbilical vein. Serial cutting balloon septostomies followed by static balloon septostomies resulted in effective left atrial decompression, atrial mixing, and optimal pulmonary and systemic perfusion. The child is now thriving after both stage I Norwood and bidirectional Glenn procedures.

Over a 12-year period, 40 patients underwent repair of partial anomalous pulmonary veins (PAPV) draining to the superior vena cava (SVC) proximal to the sinus node. Mean age was 6 +/- 2 years. In all patients, the SVC was cannulated superior to the PAPV, which were baffled with pericardium to left atrium. Six patients had associated defects repaired. In 18 patients (group I), an incision was made at the crest of the right atrial appendage (RAA) and extended upward through the sinus node and to the SVC. After rerouting of the PAPV, the SVC was enlarged using the RAA (atriocavoplasty). In 17 patients (group II), rerouting of the PAPV was accomplished through a right atriotomy. Superior vena caval enlargement was not done. Drainage of the PAPV was close to the right atrium in 14 patients (low) and to the azygos vein (high) in 3. In 5 patients (group III), an incision was made on the SVC and RAA sparing the sinus node. After rerouting of the PAPV, the RAA was anastomosed to the SVC (end to side), providing another outlet for SVC flow. There was no early or late death. Two patients (10%) in group I had late sinus bradycardia. Obstruction of the SVC and PAPV developed in 1 patient in group II with high drainage. Intermittent complete heart block developed in 1 patient in group III who also had ventricular septal defect repair. We conclude that atriocavoplasty is effective for rerouting of the PAPV and enlarging the SVC, but may predispose to sinus node disease.(ABSTRACT TRUNCATED AT 250 WORDS)

Thirty-five teenage patients with a history of presyncope or syncope underwent passive head-up tilting to reproduce symptoms of syncope. If tilting alone did not induce syncope, isoproterenol infusion was given to increase heart rate to 150 to 160 beats per minute. In 80% of patients with a history of syncope, identical symptoms could be reproduced during tilting: an abrupt fall in blood pressure combined with profound nodal bradycardia, ranging from 32 to 86 beats per minute. These symptoms were quickly reversed by returning the patient to the supine position. For patients with frequent occurrences of syncope, especially when there was a history of trauma sustained during these episodes, a therapeutic regimen of either β blockers or 9α-fluorocortisol was begun. The mechanism of this common cause of syncope in childhood is neurocardiogenic in response to venous pooling and catecholamine-induced tachycardia. The tilt test is an excellent and cost-effective test for the workup of unexplained syncope in childhood.

We present a rare case of D-transposition of the great arteries (D-TGA) and double aortic arch (DAA). The anatomy was prospectively and preoperatively diagnosed by echocardiography and confirmed by ultra-fast computed tomography. The patient underwent successful arterial switch operation and division of the vascular ring at a single procedure.

Background: Continuous hemofiltration is used in pediatric patients with acute renal fail ure. Many of these patients are treated with catecholamines for hemodynamic instability. The authors studied the pharmacokinetics of dopamine and dobutamine on patients under going continuous venovenous hemofiltration.Methods and Results: Three critically ill pediatric patients with acute renal failure and car diovascular instability treated with hemofiltration and intravenous infusion of dopamine and/or dobutamine were entered into the study. Blood samples were drawn at steady-state levels from the arterial port (inflow) of the hemofilter, from the venous port (outflow) of the hemofilter, and from the ultrafilter. Sixteen (n = 16) pharmacokinetic measurements were made, six (n = 6) for dopamine and 10 (n = 10) for dobutamine. The clearance of dopamine by the hemofilter was 0.078 ± 0.011 mL/kg/min, and for dobutamine it was 0.036 ± 0.008 mL/kg/min. On the average, 0.56% of the dopamine dose and 0.13% of the dobutamine dose was removed by the hemofilter.Conclusions: The pharmacokinetics of dopamine and dobutamine at the dosage range of 5- 25 μg/kg/min are not altered by continuous hemoliltration. Relative to total plasma clear ance. negligible amounts of the drug were removed. The dosage of these catecholamines need not hc adjusted during continuous hemofiltration.

We describe closure of an atrial septal defect in a symptomatic 4.6 kg child with the HELEX Septal Occluder (W.L. Gore and Associates, Flagstaff, AZ) via transhepatic approach. Technical considerations are discussed.

To determine the effect of high school athletics on cardiac structure and function, we performed 2-D directed M-Mode echos on students involved in isometric (IM) and isotonic (IT) sports and compared them to non-athletic controls (C). Measurements were made at rest and immediately after stage 4 treadmill exercise. Twenty students were included in each group. LV mass was greater in both in and IT compared to C (C 111±4, IM 132±3, IT 130±4 g/M2, p . In IT LV diastole volume (LVDV) was increased with normal wall thickness/radius ration (t/r), whereas, in IM, LVDV was normal but, t/r was increased. Stroke index (SI) was increased in IT (C 44±1.7, IM 46±1.6, IT 52.3±2.0 ml/M2, p