Rapid cardiopulmonary support for children with complex congenital heart disease.

BACKGROUND: Extracorporeal membrane oxygenation has limitations in children with congenital heart disease (prolonged setup times, increased postoperative blood loss, and difficulty during transport). We developed a miniaturized cardiopulmonary support circuit to address these limitations. PATIENTS AND METHODS: The cardiopulmonary support system includes a preassembled, completely heparin-coated circuit, a BP-50 Bio-Medicus centrifugal pump, a Minimax plus membrane oxygenator, a Bio-Medicus flow probe, and a Bio-trend hematocrit/oxygen saturation monitor. Short tubing length permits a 250-mL bloodless prime in less than 5 minutes. From 1995 to 1997, 23 children with congenital heart disease were supported with this technique. RESULTS: Overall survival to discharge was 48% (11 of 23 patients). Survival to discharge was 80% (4 of 5) in the preoperative support group, 20% (1 of 5) in the postoperative failure to wean from cardiopulmonary bypass group, 44% (4 of 9) in the group placed on support postoperatively after transfer to the intensive care unit, and 50% (2 of 4 patients) in the nonoperative group. Neonatal cardiopulmonary support survival to discharge was 46% (6 of 13 patients). CONCLUSIONS: This pediatric cardiopulmonary support system is safe and effective. Advantages over conventional extracorporeal membrane oxygenation include rapid setup time, decreased postoperative blood loss, and simplified transport.

Outcome of cardiopulmonary resuscitation in a pediatric cardiac intensive care unit.

OBJECTIVE: To determine the eventual outcome of children with heart disease who had cardiopulmonary resuscitation (CPR) in a specialized pediatric cardiac intensive care unit (CICU), and to define the influence of any prearrest variables on the outcome. DESIGN: A retrospective review of patients' medical records. SETTING: A pediatric CICU of a tertiary pediatric teaching hospital. PATIENTS AND METHODS: Patients were all children who presented with cardiopulmonary arrest and who were administered CPR in the pediatric CICU between June 1995 and June 1997. Prearrest variables such as age, diagnosis, prior cardiac surgery, and inotropic support with epinephrine, as well as cause of arrest, were evaluated. MEASUREMENTS AND MAIN RESULTS: Thirty-two patients, ranging in age from 1 day to 21 yrs (median, 1 month), satisfied criteria for inclusion in the study group. These 32 patients had a total of 38 episodes of cardiopulmonary arrest. Twenty-five of these patients (78%) had cardiac surgery before arrest. Inotropic support with continuous infusion of epinephrine was being administered at the time of arrest in 18 of 38 (47%) arrests. These prearrest variables did not influence outcome of CPR. Of the 38 episodes of CPR, 24 episodes (63%) were successful, with 20 episodes resulting in return of spontaneous circulation and four patients being successfully placed on mechanical cardiopulmonary support. Fourteen children, including all four patients who were rescued with mechanical cardiopulmonary support, survived to discharge. At 6-month follow-up, 11 patients were still alive, with three having neurologic impairment. CONCLUSIONS: After cardiopulmonary resuscitation in this pediatric CICU, the rate of success was 63% and the rate of survival was 42%. Prior cardiac surgery and use of epinephrine before arrest did not influence the outcome of CPR. The availability of effective mechanical cardiopulmonary support can improve the outcome of CPR.

Video-assisted cardioscopy for left ventricular thrombectomy in a child.

Left ventricular thrombus is a complication of myocardial infarction, ventricular aneurysm, cardiomyopathy, and myocarditis. Left ventriculotomy has been the standard approach for removal of left ventricular thrombus. This approach has produced an unacceptable incidence of ventricular dysfunction, arrhythmias, and aneurysm formation. We describe a case of left ventricular thrombus with systemic embolization in a patient with myocarditis. Video-assisted cardioscopy allowed visualization and removal of the thrombus via an aortotomy, thereby avoiding a left ventriculotomy.

Recurrent congenital left atrial aneurysm in a newborn.

Recurrent congenital left atrial aneurysm presenting in a newborn as cardiac tamponade is described. Such a presentation has not been previously reported.

Emergent stent placement for acute Blalock-Taussig shunt obstruction after stage 1 Norwood surgery.

A neonate underwent a stage 1 Norwood surgery for hypoplastic left heart syndrome and subsequently developed profound cyanosis and hemodynamic instability. Catheterization revealed an occluded modified Blalock-Taussig shunt. Angioplasty and stent implantation resulted in immediate angiographic and clinical improvement, which has persisted at 5-month follow-up. This therapy may provide lifesaving treatment in selected patients.

Extracardiac Fontan operation without cardiopulmonary bypass.

Choreoathetosis developed after bilateral bidirectional cavopulmonary anastomosis in a 17-month-old boy with univentricular heart. To avoid exacerbating this neurologic problem, the Fontan operation was later completed without cardiopulmonary bypass. The left cavopulmonary anastomosis maintained pulmonary blood flow. A tube graft was anastomosed to the junction of the right cavopulmonary anastomosis. A femoral vein-to-right atrial shunt was established, the inferior vena cava divided, the cardiac end oversewn, and the noncardiac end anastomosed to the tube graft.

Significance of the Doppler-derived gradient across a residual aortic coarctation.

Few data exist which address the significance of the Doppler gradient across a residual narrowing in older children who have had a coarctation repaired. Therefore, we evaluated 11 patients with repaired aortic coarctation with and without residual obstruction by Doppler echocardiography. The Doppler-derived transcoarctation pressure gradient correlated poorly with catheter-measured peak-to-peak and catheter maximal instantaneous gradients when only the maximal velocity across the repair was utilized in the simplified Bernoulli equation, [r = 0.73, standard of error of the estimate (SEE) = 5.0 mmHg and r = 0.56, SEE = 7.6 mmHg, respectively]. However, when the precoarctation velocity was included in the simplified Bernoulli equation, the correlation between Doppler-derived and catheter-measured gradients became excellent. The maximal Doppler gradient correlated well with catheter peak-to-peak gradient (r = 0.95, SEE = 2.2 mmHg) and catheter maximal instantaneous gradient (r = 0.94, SEE = 3.2 mmHg). However, the maximal Doppler gradient slightly overestimated the catheter peak-to-peak gradient and underestimated the catheter maximal instantaneous gradient. The Doppler mean gradient showed excellent correlation with the catheter mean gradient (r = 0.97, SEE = 0.85 mmHg). Precoarctation velocities were generally twofold greater than published normals due to a spatial acceleration phenomenon. All subjects had residual hypoplasia of the transverse aorta such that its transverse diameter was 29% less than, and its cross-sectional area was 50% less than, the ascending and descending aorta.(ABSTRACT TRUNCATED AT 250 WORDS)

Doppler estimation of pressure gradient in pulmonary stenosis: maximal instantaneous vs peak-to-peak, vs mean catheter gradient.

We undertook a study to identify the hemodynamic significance of a Doppler-derived gradient across a stenotic pulmonary valve. Furthermore, we attempted to define the optimal plane for velocity data acquisition. A total of 17 children with valvar pulmonary stenosis were evaluated using Doppler echocardiography. Flow-velocity profiles were obtained from both the parasternal and subxiphoid windows. Ten of 17 patients were studied before and after balloon valvotomy. Therefore, 27 different transvalvar gradients were assessed by Doppler and these data were compared with the catheter-derived maximal instantaneous, peak-to-peak, and mean pressure gradients. The maximal Doppler gradient correlated well with the catheter-derived peak-to-peak pressure gradient (r = 0.95) and catheter maximal instantaneous pressure gradient (r = 0.95). Although these correlation coefficients were similar, the Doppler maximal gradient consistently overestimated the peak-to-peak catheter gradient by as much as 25%-40%. Such an overestimation was not observed when we compared the maximal Doppler gradient with the catheter-derived maximal instantaneous gradient. Moreover, the regression line of the latter comparison closely approximated the line of identity. The correlation coefficient between Doppler mean and catheter mean gradients was only 0.91. Doppler velocities were best derived when multiple transducer positions were employed to interrogate pulmonary artery velocity.

Diagnosis and treatment of intrapericardial teratoma.

Intrapericardial teratoma is a rare mediastinal tumor that originates from aberrant clusters of multipotential cells from three germinal layers. Previous reports have used the combination of cardiac angiography, CT scan, and echocardiogram to establish the diagnosis prior to surgery. We report a case of intrapericardial teratoma diagnosed noninvasively and removed surgically within the first three days of life. Furthermore, we compare the diagnostic accuracy of echocardiography and computerized tomography (CT), and discuss the superiority of noninvasive evaluation in the management of these critically ill infants.

Prostaglandins and echocardiography in the assessment of patent ductus arteriosus.

Prostaglandin (PG) levels and M-mode echocardiography were used to evaluate the severity of patent ductus arteriosus (PDA) in 19 premature infants. Mean 6-keto-PGF1 alpha levels in infants with more severe left-to-right shunting were significantly higher than those in infants with a moderate level of shunting (1335 +/- 763 vs. 504 +/- 348 pg/ml, respectively). Furthermore, there was a significant correlation between this elevation and a decrease in the left ventricular systolic time interval, suggesting that both reflect the severity of ductal shunting. Although other echocardiographic measurements of cardiovascular function generally showed some tendency to vary with 6-keto-PGF1 alpha levels, none was as closely correlated with the extent of PG elevation. Levels of PGE2 also seemed to vary with PDA severity; however, this correlation was not as significant.

Prostaglandin levels: predictors of indomethacin responsiveness.

Pretreatment plasma dilator prostaglandin levels were measured in 16 premature infants with patent ductus arteriosus in an attempt to correlate abnormally elevated levels with clinical responsiveness to indomethacin therapy. Nine of the 16 infants responded well to indomethacin, with complete disappearance of their murmurs by 48 h. Eight of these nine infants had elevated baseline 6 keto PGF1 alpha levels (greater than 500 pg/ml). In contrast, seven of the 16 infants did not respond to indomethacin, and six of these had 6 keto PGF1 alpha within the normal range (less than 500 pg/ml). PGE2 levels varied in the same general direction, but lacked the specificity and sensitivity of the 6 keto PGF1 alpha levels. Thus, 6 keto PGF1 alpha levels seem to correlate with, and may eventually be helpful in predicting, clinical indomethacin responsiveness in the premature neonate with patency of the ductus arteriosus.