Cerebral oxygen saturation and electrical brain activity before, during, and up to 36 hours after arterial switch procedure in neonates without pre-existing brain damage: its relationship to neurodevelopmental outcome.

OBJECTIVE: To monitor the pattern of cerebral oxygen saturation (rSat), by use of NIRS, in term infants before, during and after the arterial switch operation and to evaluate its relation to neurodevelopmental outcome. METHODS: In 20 neonates without pre-existing brain damage hemodynamics and arterial oxygen saturation (AO2-Sat) were monitored simultaneously with rSat and amplitude-integrated EEG (aEEG) from 4 h to 12 h before up to 36 h after cardiopulmonary bypass (CPB) and short duration of cardiac arrest during deep hypothermia (DHCA). The Bayleys developmental scale was performed at 30 months. RESULTS: Before surgery rSat was <50% in 16 patients. During CPB rSat increased to normal values, with a sharp decrease during brief CA (median 6.5 min). Post-CPB rSat showed a transient decrease (30-45%) despite normal PaO2 with sustained normalization after 6-26 h. Recovery time of the rSat seemed longer when pre-operative rSat was below 35%, and for lower minimum nasopharyngeal temperature and longer duration of CPB and of DHCA. Recovery time of the aEEG varied and did not correlate with normalization of rSat. Neurodevelopmental outcome was normal in all but two patients. Patients with lower pre-operative rSat (<35%) tended to have lower DQ (developmental quotient) scores at 30-36 months. (median: mental 102 and motor 101 (range 58-125) compared with mental 100 and motor 110 (range 83-125)) CONCLUSION: Despite prompt normalization of circulation and oxygenation after surgery, recovery of rSat of the brain took 6-26 h, probably because of higher energy demand after CPB. Pre-operative cerebral oxygenation may be underestimated as a possible cause of adverse post-operative outcome.

Comparison of surgical repair with balloon angioplasty for native coarctation in patients from 3 months to 16 years of age.

OBJECTIVE: Surgery and balloon angioplasty (BA) for coarctation of the aorta have shown comparable short-term results, but long-term follow-up remains unclear. Comparison of surgical repair and balloon coarctation for native coarctation of the localised membranous form is performed retrospectively. To allow a valid comparison between both techniques, identical inclusion criteria were applied. METHODS: Results of surgery (group A, 18 patients, age 0.30-14 years, median 0.63 years) and BA (group B, 28 patients, age 0.25-15 years, median 5.8 years) for isolated, native coarctation in children >3 months, performed in a 10-year-period, were compared. Kaplan-Meier analysis was performed in both groups. Mean follow-up ranged from 2.5 to 11 years (mean 7.2+/-2.4 years) in group A and from 1.4 to 10 years (mean 5.4+/-2.8 years) in group B. RESULTS: Immediate success was obtained in all patients following surgery and 27/28 patients (96%) following BA. No statistical difference between surgery and angioplasty with respect to resultant pressure gradient decreases were found. Mortality was not encountered. Hospital stay varied from 6 to 20 days in group A and was 48 h for all patients in group B. Recoarctation occurred in one patient (5.6%) in group A and in two patients (7%) in group B. Log-rank test reveals no statistical difference in freedom from reintervention probabilities between surgery and angioplasty. Aneurysm formation was not encountered. CONCLUSIONS: Both surgical repair and BA for native coarctation yield low reintervention probabilities in comparable patients. Aneurysm formation was not encountered following different treatment types.

Comparative long-term results of surgery versus balloon valvuloplasty for pulmonary valve stenosis in infants and children.

BACKGROUND: We compared the long-term results of surgical valvotomy (S) versus balloon valvuloplasty (BV) for pulmonary valve stenosis in infants and children. METHODS: Results after surgical pulmonary valvotomy (with concomitant ASD/VSD closure) (n = 62, age 2.9 +/- 3.5 years) and balloon valvuloplasty (n = 108, age 3.6 +/- 3.9 years) were analyzed. Transvalvular mean pressure gradient decrease, freedom from reintervention for restenosis, pulmonary valve insufficiency, and tricuspid valve insufficiency were considered. RESULTS: Mean pressure gradient decreased significantly more in the surgical group (from 64.8 +/- 30.8 mm Hg to 12.8 +/- 9.8 mm Hg at a mean follow-up of 9.8 years) than after BV (decreasing from 66.2 +/- 21.4 mm Hg to 21.5 +/- 15.9 mm Hg after a mean of 5.4 years; p < 0.001). Moderate pulmonary valve insufficiency occurred in 44% after surgery, and in 11% after BV (p < 0.001). Tricuspid valve insufficiency occurred in 2% after surgery, and in 5% after BV. Restenosis occurred in 3 surgical patients (5.6%), 2 patients required reoperation, and 1 patient required a balloon valvotomy. Restenosis developed in 13 BV patients (14.1%): 6 patients were redilated and 7 patients required surgery. Surgical valvotomy led to significantly less reinterventions than balloon valvuloplasty (p < 0.04). CONCLUSIONS: Surgical relief of pulmonary valve stenosis produces lower long-term gradients and results in longer freedom from reintervention. Balloon valvuloplasty may remain, despite these results, the preferred therapy for isolated pulmonary valve stenosis, because it is less invasive, less expensive, and requires a shorter hospital stay. Surgery should remain the exclusive form of therapy in the presence of concomitant intracardiac defects, which need to be addressed.

Comparison of polytetrafluoroethylene patch aortoplasty and end-to-end anastomosis for coarctation of the aorta.

OBJECTIVES: Although aneurysm formation and recoarctation after Dacron patch aortoplasty have been reported on extensively, less is known about these outcomes after polytetrafluoroethylene patch repair, which was compared with resection and end-to-end anastomosis in this study. METHODS: Two hundred sixty-two children had surgical repair of coarctation of the aorta by means of either resection and end-to-end anastomosis (n = 137; mean age, 1.85 +/- 3.1 years) or polytetrafluoroethylene patch aortoplasty (n = 118; mean age, 1.09 +/- 1.9 years) during a 28-year period. Coarctation was isolated in 109 (41.6%), associated with ventricular septal defect in 77 (29.4%), and associated with complex intracardiac anomalies in 76 (29.0%) patients. Follow-up ranged from 2 days to 29.3 years (median, 11.9 years). Seven patients were lost to follow-up. Kaplan-Meier survival curves were estimated, and multivariable Cox regression analysis was performed for several outcome variables. RESULTS: Mortality was 8.2% and was associated with intracardiac pathology in all cases. Recoarctation occurred in 53 patients, 23 after resection and anastomosis and 30 after patch repair, not differing statistically (P =.4, log-rank test). Aneurysm formation occurred in 8 patients after patch repair that included ridge resection in 7 of the 8 patients. Late hypertension occurred in less patients (n = 3) after resection and anastomosis than after patch repair (n = 8) (P <.03). Arch hypoplasia (P <.01) and age less than 1 month (P <.001) were found to be independent risk factors for recoarctation. CONCLUSIONS: Polytetrafluoroethylene patch repair including coarctation ridge resection was found to be a risk factor for aneurysm formation and late hypertension. Arch hypoplasia and young age must be considered to predispose to recoarctation.

Prenatal diagnosis of the fetus with hypoplastic left heart syndrome management and outcome.

OBJECTIVES: To review our 13-year experience with prenatally detected hypoplastic left heart syndrome (HLHS) of which management remains controversial. MATERIAL AND METHODS: Retrospective study of the management and outcome in all cases of HLHS diagnosed prenatally in a tertiary referral center for pediatric cardiology and cardiac surgery between January 1988 and July 2001. RESULTS: The diagnosis of HLHS was made in 32 fetuses. One mother had two pregnancies associated with HLHS. In 16 cases parents opted for termination of pregnancy and in five for compassionate care. Four fetuses died in utero, and seven patients received a palliative reconstructive Norwood procedure. In seven fetuses, associated anomalies were detected: three chromosomal and structural and four only structural. In six fetuses, other associated intracardiac anomalies were detected. Of seven infants operated, six had no associated anomalies and only one is alive at an age of 17 months. CONCLUSION: The low percentage of intention to treat among patients in our center (34%) is in accordance with the percentage found in another study from the UK (36.2%), but differs significantly from reported series across the Atlantic (67%). Prenatal diagnosis of the HLHS provides opportunities not only for getting patients in optimal preoperative condition when surgery is offered, but also for in-depth counseling of the parents on this severe malformation. A minority of parents faced with the difficult decision of possible termination of pregnancy, compassionate care or the Norwood strategy, choose surgical treatment which might be based on socioreligious differences and the interpretation of the long-term quality of life.

Comparable systemic ventricular function in healthy adults and patients with unoperated congenitally corrected transposition using MRI dobutamine stress testing.

BACKGROUND: Failure of the systemic right ventricle (RV) often complicates adult survival in unoperated or physiologically repaired congenitally corrected transposition of the great arteries (CCTGA). Healthy controls schematically represent an optimal outcome of anatomic repair, which is increasingly performed to treat CCTGA. Magnetic resonance imaging dobutamine stress testing measures cardiac reserve, and sets to compare the left ventricle of controls with the systemic RV of unoperated and physiologically repaired patients with CCTGA. METHODS: Baseline and stress magnetic resonance imaging (maximum dobutamine dose, 15 microg/kg/min) assessed systemic RV function in 13 minimally or asymptomatic adult patients with CCTGA (unoperated, n = 7; physiologically repaired, n = 6). The left ventricles of 11 healthy age-matched adults served as controls. RESULTS: Baseline and stress end-diastolic volumes similar between the systemic RV of unoperated patients and the left ventricle of controls, as well as base end-systolic volumes. Stress ejection fraction was lower in unoperated and physiologically repaired patients (70 +/- 6% and 60 +/- 5%, respectively, vs healthy controls (84 +/- 8%). However, comparable with healthy controls, both subsets of CCTGA patients responded appropriately to dobutamine stress, as illustrated by similar RV stroke volume, heart rate, mean blood pressure, and cardiac index. CONCLUSIONS: Compared with the left ventricles of healthy controls, both patient groups had larger systemic RV volumes, diminished ejection fraction, but an appropriate response to dobutamine stress. Values of unoperated patients are closer to normal than physiologically repaired patients. Magnetic resonance imaging dobutamine may help to define the subgroups of CCTGA patients with favorable anatomy, whereby asymptomatic adult survival could be anticipated without the need for an operation.

Right ventricular dysfunction and pulmonary valve replacement after correction of tetralogy of Fallot.

BACKGROUND: Correction of tetralogy of Fallot often leads to pulmonary regurgitation, sometimes warranting pulmonary valve replacement, for which indications and timing to achieve optimal results are not yet clear. This retrospective study describes follow-up and reinterventions in our tetralogy of Fallot population. METHODS: Review of all consecutive patients operated on for tetralogy of Fallot between 1977 and 2000 was conducted. Included are date and type of repair, Doppler echocardiography (two-dimensional echocardiography), electrocardiographs, reoperations, and physical condition. RESULTS: Total repair was performed in 171 patients at a mean age 1.9 +/- 2.5 years, follow-up time counted 9.6 +/- 7.0 years. Right ventriculotomy was used in 92%, and transatrial ventricular septal defect closure was used in 8%; 74% received a transannular outflow patch. Twenty-year survival was 91%. Last follow-up electrocardiographs showed right bundle branch block in 67% and serious arrhythmias in 11%. Two-dimensional echocardiography demonstrated severe pulmonary insufficiency and dilated right ventricle in 31% and 38%, respectively, increasing with postrepair age (p < 0.001). Poor clinical condition (New York Heart Association class II+) and echocardiographic proof of right atrial dilatation (p = 0.012) and arrhythmias (p = 0.03) were significantly associated. Furthermore, the influence of residual hemodynamic lesions, such as a remaining ventricular septal defect or pulmonary stenosis, or right ventricular dilatation was important (p = 0.04). Reintervention was necessary in 32 patients (19%; 10-year freedom, 83%), including angioplasty for residual stenosis and pulmonary valve replacement. At a mean age of 9.2 years after correction, 14 patients received a homograft, and 2 patients received a heterograft. In 7 patients the right ventricle returned to normal dimensions and symptoms disappeared. The incidence of right ventricular dilatation was considerably higher (p = 0.020) in patients with a transannular patch; the transatrial approach showed the opposite (p = 0.03), and patients presented with lower QRS duration (p = 0.007), although no difference could be found between survival after both surgical techniques. Effects of early timing (correction < 6 months) on right ventricular dysfunction could not be established. CONCLUSIONS: Severe right ventricular dilatation and pulmonary regurgitation secondary to outflow tract repair in tetralogy of Fallot are frequently occurring sequelae developing slowly over time. Indications for pulmonary valve replacement remain controversial because echocardiographic findings or arrhythmias are not always accompanied by deterioration of clinical condition. However, right atrial dilatation and additional hemodynamic lesions demand increased vigilance. Transatrial repair is associated with a favorable outcome.