Outcome of children with Pentalogy of Cantrell following cardiac surgery.

Although single individual reports have documented outcomes in children with pentalogy of are few data available for postoperative outcome of this cohort of patients after cardiac surgery. The aim of this study was to retrospectively review the clinical details of patients with pentalogy of Cantrell managed at two centers. Two cardiac surgical institutions retrospectively studied all patients with pentalogy of Cantrell and significant congenital heart disease who underwent surgical intervention, excluding PDA ligation, between 1992 and 2004. Seven children with pentalogy of Cantrell underwent surgical intervention at a median age of 60 days (range, 1-11 months). Three patients had tetralogy of Fallot, two double outlet right ventricle, one patient had tricuspid atresia, and one patient a perimembranous ventricular septal defect. The mean duration of postoperative ventilation was 112.8 days (range, 4-335 days) but three patients required ventilation for more than 100 days. Patients who had a preoperative diaphragmatic plication required a longer duration of ventilation (mean = 186.5 days [range, 100-273 days] compared with mean = 132 days [range, 4-335 days]). Four patients survived, with three patients weaned from ventilation. Three patients had withdrawal of care following failure to wean from ventilation, following multisystem organ failure, and at the request of their parents. In conclusion, the postoperative care of children with pentalogy of Cantrell after cardiac surgery is often complicated by prolonged need for ventilatory support and multiple postoperative complications. Earlier surgical intervention does not necessarily reduce morbidity and mortality. These data may help in the counseling of parents prior to surgical intervention.

Changes in cerebral saturation profile in response to mechanical ventilation alterations in infants with bidirectional superior cavopulmonary connection.

OBJECTIVES: To document cerebral saturation in response to alterations in mechanical ventilation in infants with bidirectional superior cavopulmonary connection. DESIGN: Prospective study. SETTING: Pediatric cardiovascular intensive care unit. PATIENTS: Children with functional single ventricle who have undergone bidirectional superior cavopulmonary connection. INTERVENTIONS: We measured cerebral oxygenation using near-infrared spectroscopy in response to three ventilator interventions (change 50% from baseline): a) hyperventilation with increased tidal volume; b) hyperventilation with increased respiratory rate; and c) hypoventilation by decreased respiratory rate. Physiologic variables documented were cerebral oxygenation index (rSO2i), arterial pH, and PCO2. MEASUREMENTS AND MAIN RESULTS: Ten patients (eight males and two females) underwent placement of bidirectional superior cavopulmonary connection. There were no mortalities. The mean age was months 8.6 (+/-2.1) months. Hyperventilation (tidal volume increase) caused an increase in pH from 7.35 to 7.42 (p = .001), a decreased PCO2 from a baseline 45.9 to 33.9 mm Hg. (p = .002), a decrease in rSO2i from 64.4 to 52.0 (p < .001), and a decreased Po2 from 52.8 to 46.9 mm Hg (p = .008). Hyperventilation (respiratory rate increase) caused increased pH from 7.35 to 7.39 (p = .002), decreased PCO2 from a baseline 41 to 37 mm Hg. (p = .021), decreased rSO2i from 65.9 to 56.7 (p = .007), and decreased PO2 from 54.9 to 48.9 mm Hg (p = .006). Hypoventilation (respiratory rate decrease) did not change pH did not change from baseline 7.35. The PCO2 increased from 40.8 to 42, and the rSO2i increased from 64.0 to 68.6 (p = .004). CONCLUSIONS: Hyperventilation can potentially cause a decrease in cerebral oxygenation and should be avoided in children with bidirectional superior cavopulmonary connection. Normoventilation and mild respiratory acidosis, however, preserve cerebral oxygenation in these patients.

A noninvasive estimation of mixed venous oxygen saturation using near-infrared spectroscopy by cerebral oximetry in pediatric cardiac surgery patients.

BACKGROUND: Near-infrared spectroscopy (NIRS) is a noninvasive optical monitor of regional cerebral oxygen saturation (rSO2). The aim of this study was to validate the use of NIRS by cerebral oximetry in estimating invasively measured mixed venous oxygen saturation (SvO2) in pediatric postoperative cardiac surgery patients. METHODS: Twenty patients were enrolled following cardiac surgery with intraoperative placement of a pulmonary artery (PA) or superior vena cava (SVC) catheter. Five patients underwent complete biventricular repair--complete atrioventricular canal (n=3) and other (n=2). Fifteen patients with functional single ventricle underwent palliative procedures--bidirectional Glenn (n=11) and Fontan (n=4). Cerebral rSO2 was monitored via NIRS (INVOS 5100) during cardiac surgery and 6 h postoperatively. SvO2 was measured from blood samples obtained via an indwelling PA or SVC catheter and simultaneously correlated with rSO2 by NIRS at five time periods: in the operating room after weaning from cardiopulmonary bypass, after sternal closure, and in the CICU at 2, 4, and 6 h after admission. RESULTS: Each patient had five measurements (total=100 comparisons). SvO2 obtained via an indwelling PA or SVC catheter for all patients correlated with rSO2 obtained via NIRS: Pearson's correlation coefficient of 0.67 (P<0.0001) and linear regression of r2=0.45 (P<0.0001). Separate linear regression of the complete biventricular repairs demonstrated an r=0.71, r2=0.50 (P<0.0001). Bland-Altman analysis showed a bias of +3.3% with a precision of 16.6% for rSO2 as a predictor of SvO2 for all patients. Cerebral rSO2 was a more accurate predictor of SvO2 in the biventricular repair patients (bias -0.3, precision 11.8%), compared with the bidirectional Glenn and Fontan patients. CONCLUSIONS: Regional cerebral oximetry via NIRS correlates with SvO2 obtained via invasive monitoring. However, the wide limits of agreement suggest that it may not be possible to predict absolute values of SvO2 for any given patient based solely on the noninvasive measurement of rSO2. Near-infrared spectroscopy, using the INVOS 5100 cerebral oximeter, could potentially be used to indicate trends in SVO2, but more studies needs to be performed under varying clinical conditions.

Mediastinitis after pediatric cardiac surgery: a 15-year experience at a single institution.

BACKGROUND: The spectrum of sternal wound infections after cardiac surgery ranges from superficial infections to a deep sternal infection known as mediastinitis. Mediastinitis is a rare but clinically relevant source of postoperative morbidity and mortality in adult and pediatric patients after cardiac surgery. METHODS: We retrospectively identified all patients diagnosed with mediastinitis after cardiac surgery from January 1987 to December 2002 (17 patients/7,616 surgeries = 0.2%). Demographic data, cardiac diagnosis, cardiac surgery, hospital length of stay, associated medical diagnosis, and surgical treatment for mediastinitis were collected. RESULTS: Fifteen pediatric patients (age < 18 years) were diagnosed with mediastinitis (mean age at diagnosis 37.5 months, range 21 days to 17 years. The median postoperative day of diagnosis was 14 days (6 to 50 days). The most common organism was Staphylococcus species (n = 9). Six patients had an associated bacteremia. The median hospital length of stay for all patients was 42.5 days (range 16 to 163 days). The hospital mortality was 1 of 15 (6%). Each patient was treated with intravenous antibiotics; sternal debridement; and rectus abdominus flap reconstruction (n = 7), pectoralis muscle flap reconstruction (n = 3), omentum reconstruction (n = 1), or primary sternal closure (n = 4). Three patients have undergone redo-sternotomy with orthotopic heart transplantation, bidirectional cavopulmonary anastomosis, and replacement of a right ventricle to pulmonary artery homograft. CONCLUSIONS: Timely diagnosis, aggressive sternal debridement, and liberal use of rotational muscle flaps can potentially minimize the morbidity and mortality in pediatric postoperative cardiac patients. Subsequent redo-sternotomy has not been problematic.

Frequency of recurrence among infants with supraventricular tachycardia and comparison of recurrence rates among those with and without preexcitation and among those with and without response to digoxin and/or propranolol therapy.

Approximately 60% of children with supraventricular tachycardia (SVT) develop their initial episode by 1 year of age. Despite resolution in most of these patients by 1 year, approximately 30% of the SVT will recur. We performed a retrospective review of all patients <1 year of age with SVT between January 1984 and December 2000. Recurrence was defined as documented SVT at >1 year of age. Patients were divided into: (1) a first line (FL) group (controlled with digoxin and/or propranolol) and (2) a second line (SL) group (requiring additional antiarrhythmics). The groups were divided based on the presence of preexcitation. The FL group included 116 patients, 20 of whom (17%) had Wolff-Parkinson-White (WPW) syndrome. The SL group included 34 patients, 21 of whom (62%) had WPW (p <0.001). Recurrence of SVT occurred in 32 patients (28%) in the FL group and in 23 patients (68%) in the SL group (p <0.001). SVT recurred in 36 of 41 patients (88%) with WPW compared with 19 of 109 patients (17%) without WPW syndrome (p <0.001). Logistic regression analysis demonstrated that the presence of WPW syndrome was associated with a 29-fold higher odds of SVT recurrence (p <0.001), and that patients with WPW syndrome were more likely to require additional antiarrhythmic therapy (p <0.001). Thus, patients with WPW syndrome who had SVT at <1 year of age have 29-fold higher odds of recurrence at >1 year of age versus those patients with preexcitation. These patients are also more likely to require additional antiarrhythmic therapy to control SVT. Furthermore, children with WPW syndrome who are refractory to treatment with digoxin and/or propranolol are at increased risk of SVT recurrence.

Meandering right pulmonary vein to the left atrium and inferior vena cava: the first case with associated anomalies.

We report a case of a healthy, asymptomatic 6-year-old boy in whom an anomalous right pulmonary vein was noted to drain into both the inferior vena cava and left atrium in association with findings consistent with scimitar syndrome. The anomalous pulmonary vein took a very circuitous route through the lungs before draining into the left atrium, a condition previously termed "meandering pulmonary vein." To aid in the diagnosis, cardiovascular magnetic resonance imaging and magnetic resonance angiography were used to delineate this complex course and the connection of the anomalous pulmonary vein. To our knowledge, this is the 1st reported case of a meandering pulmonary vein with dual drainage to the inferior vena cava and left atrium in association with other anomalies.