Angel Cuadrado

Nesiritide in infants and children with congestive heart failure.

Objectives: Nesiritide (synthetic B-type natriuretic peptide) has been shown to be effective in the management of acute decompensated heart failure in adults. The role of nesiritide in pediatric heart failure has not been examined. In the present study, we reviewed our initial experience with nesiritide in children with primary heart failure or low cardiac output after heart surgery. Methods: Nesiritide was administered in an open-label fashion to patients with heart failure who were already receiving inotropic and diuretic therapy. Between July 2003 and August 2004, 30 patients aged 5 days to 16.7 yrs (median age, 4.6 months) received nesiritide therapy. Diagnoses included single-ventricle congenital defect (n = 5), two-ventricle congenital defect (n = 13), heart transplant (n = 5), and dilated cardiomyopathy (n = 7). Sixteen patients were started on nesiritide within 2 wks of corrective or palliative heart surgery. The majority of subjects (n = 24) received an initial bolus dose. Continuous infusion dosage ranged between 0.005 and 0.02 &mgr;g·kg−1·min−1. Nesiritide was discontinued for possible side effects in two patients (arrhythmia and hypotension). Duration of therapy ranged from 1 to 24 days (median, 4 days). Results: Administration of nesiritide was associated with improvement in fluid balance from positive 0.8 ± 1.9 mL·kg−1·hr−1 at baseline to negative 0.3 ± 1.8 mL·kg−1·hr−1 after 24 hrs of therapy (p = .02). There was a nonsignificant trend toward a reduction in right atrial pressure (9.2 ± 3.9 vs. 11.2 ± 4.1, p = .08). Conclusions: Nesiritide is well tolerated in children with heart failure and is associated with improved diuresis. Further prospective studies will be needed to compare nesiritide with other vasoactive agents and examine the cost-efficacy of this therapy.

Low-weight infants are at increased mortality risk after palliative or corrective cardiac surgery.

BACKGROUND Low weight is an established risk factor for mortality after congenital cardiac surgery. Given the advances in the care of neonates and infants after surgery, we sought to examine the effect of low weight on outcomes in the current era. METHODS From 2002 to 2012, 2051 infants aged 90 days or less underwent cardiac surgery including 534 (26.0%) with single-ventricle pathology. Regression models examined the effect of low weight (≤ 2.5 kg; n = 274, 13.4%) on early and late outcomes. RESULTS Overall, the incidence of prematurity, associated chromosomal/extracardiac abnormalities was higher in infants who weighed 2.5 kg or less than in those who weighed more than 2.5 kg; the incidence of single-ventricle pathology was comparable between the 2 groups. In addition, infants who weighed 2.5 kg or less underwent more palliation and had a higher proportion of STAT (Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery) risk category 4 and 5 procedures. Adjusted regression models showed that low weight (≤ 2.5 kg) did not increase unplanned reoperation (odds ratio [OR], 0.90; 95% confidence interval [CI], 0.48-1.67; P = .73) or extracorporeal membrane oxygenation requirement (OR, 1.23; 95% CI, 0.68-2.22; P = .49), however it was associated with significant increase in hospital mortality (OR, 2.15; 95% CI, 1.33-3.50; P = .002). In addition, there was a significant association between low weight and increased duration of postoperative mechanical ventilation and intensive care unit and hospital stays. Adjusted hazard analysis showed that weight equal to or less than 2.5 kg was associated with diminished late survival (hazard ratio, 1.89; 95% CI, 1.39-2.55; P < .001) and that was evident in all patients subgroups (P < .001 for all). CONCLUSIONS In a large single-center series, low weight continues to be associated with increased early mortality risk and resource utilization after palliative and corrective cardiac surgery. The hazard of death in low-weight patients continues beyond the perioperative period for at least 1 year before normalizing. Strategies to improve outcomes for this high-risk population must address perioperative care, outpatient surveillance, and management.

Current results with pediatric heart transplantation

BACKGROUND Cardiac transplantation is an accepted treatment for children with end-stage heart failure or complex or inoperable congenital defects. METHODS Since 1988, 95 transplants have been performed in 89 children aged 4 days to 18 years (median 6.9 years, 42 patients 0-5 years). Fifty-eight (61%) had congenital or acquired heart disease, 31 (33%) had idiopathic cardiomyopathy, and 6 (6%) were retransplants. Fifty-seven of the patients had prior cardiac surgery with a range of one to eight procedures (mean 3.4 procedures/patient). At the time of transplantation, 53 (56%) were United Network for Organ Sharing (UNOS) status I, including 23 children on mechanical ventilation and 4 with mechanical circulatory support. RESULTS Thirty-day survival in this group was 96%. Posttransplant results showed a median time of ventilation of 1 day (mean 3.0+/-5.7 days), median duration of inotropic support of 2 days (mean 2.7+/-2.3 days), median intensive care unit (ICU) stay of 4 days (mean 6.9+/-9.6 days), and median hospitalization of 9 days (mean 14.3+/-13.9 days). Follow-up from 1 month to 10.3 years (mean 3.1 years) has demonstrated a 1-year actuarial survival of 79% and a 5-year actuarial survival of 69%. Rejection, both acute and chronic, accounted for the vast majority of deaths. CONCLUSIONS Pediatric heart transplantation can be accomplished with excellent early survival despite multiple prior cardiac operations and relative severity of illness. Parameters such as postoperative ventilation, inotropic support, ICU stay, and hospitalization can be kept at reasonable levels with acceptable long-term results, although rejection remains a serious problem.

Do neonates with genetic abnormalities have an increased morbidity and mortality following cardiac surgery

INTRODUCTION Genetic abnormalities occur in approximately 20% of children with congenital heart disease. The purpose of this study was to evaluate the effect of genetic abnormalities on short-term outcomes following neonatal cardiac surgery. METHODS Retrospective review of all neonates (n = 609) undergoing cardiac surgery from January 2003 to December 2006. Genetic abnormalities were identified in 93 neonates (15%). Genetic abnormalities identified were 22q11.2 deletion (23), chromosomal abnormalities including various monosomies, trisomies, deletions, duplications, and inversions (17), dysmorphic undefined syndrome without recognized chromosomal abnormality (27), Down syndrome (9), laterality sequences (9), recognixed syndromes and genetic etiology including Mendelian (i.e. Alagille, CHARGE) (8). RESULTS Neonates with genetic abnormalities had lower birth weights and were older at time of surgery. There was no difference in operative variables, duration of mechanical ventilation or ICU length of stay between the two groups. There was an increase in total hospital length of stay and postoperative complications in the neonates with genetic abnormalities. Importantly, in hospital mortality was not different. CONCLUSION Neonates with genetic abnormalities have a higher risk of postoperative complications and a longer hospital length of stay. However, there is no increase in hospital mortality. This information may aid in patient management decisions and parental counseling. Longer-term studies are needed for understanding the total impact of genetic abnormalities on neonates with congenital heart disease.

Saphenous vein homograft: a superior conduit for the systemic arterial shunt in the Norwood operation

BACKGROUND Excessive pulmonary blood flow increases ventricular volume work in the face of inadequate systemic cardiac output, low diastolic blood pressure, and inadequate coronary perfusion. Using the smallest available 3-mm polytetrafluoroethylene shunts have been successful, although catastrophic shunt thrombosis has occasionally been observed. To avoid thrombosis with a smaller conduit, saphenous vein homografts (SVG) were used to construct the modified Blalock-Taussig (BT) shunts. METHODS From January 1998 to April 1999, 25 patients weighing 3.1 kg (3.0 kg or less, n = 9), at a mean age of 8.9 days, underwent stage I Norwood using an SVG BT shunt. Common heart defects were aortic atresia (n = 8), mitral atresia and double-outlet right ventricle (n = 5), and unbalanced AVC (n = 5). Mean BT shunt size was 3.2 mm, with 12 patients having shunts that were 3 mm or smaller. RESULTS Thirty-day hospital mortality was 8% (2 of 25). No shunt thrombosis was seen, despite banding the BT shunt in 3 patients. One patient had BT revision because of an anatomic issue not directly related to the shunt material. CONCLUSIONS Excellent results may be achieved using SVG BT shunts in the Norwood operation. This conduit seems less likely to thrombose, both acutely and chronically, allowing the use of appropriately smaller-sized shunts in small neonates.

Hemodynamic effects and safety of nesiritide in neonates with heart failure.

Objective: Evaluate effects and safety of nesiritide (Natrecor, Scios Inc) human B-type natriuretic peptide, in neonates with heart failure. Methods: Seventeen neonates, not responding to conventional therapy, treated with nesiritide were retrospectively reviewed. Results: Average age 16 + 8 days; weight 3.2 + 0.6 kg. Fifteen treated with concomitant inotropic therapy; all with diuretics. Twelve received loading dose; followed by continuous infusions of 0.005 mcg/kg/min (2); 0.01 mcg/kg/min (12); 0.02 mcg/kg/min (3). Length of therapy 5 + 4 days. No change in heart rate or blood pressure between baseline, 1 hour or 24 hours of nesiritide infusion. Decrease central venous pressure (CVP) 24 hours after infusion (p = 0.03). Ins-out ratio improved in 29%. No difference in pre and post therapy BUN and creatinine (Cr). 18% had hypotension requiring intervention. Conclusions: Nesiritide use in neonates may improve hemodynamics as demonstrated by reduction in CVP. All patients tolerated bolus dosing, however, transient hypotension occurred in 18% of neonates with continuous infusion.

Risk Adjustment for Congenital Heart Surgery (RACHS): Is It Useful in a Single-center Series of Newborns as a Predictor of Outcome in a High-risk Population?

OBJECTIVE Risk adjustment for congenital heart surgery (RACHS) was developed to compare outcome data for pediatric patients undergoing cardiac surgery. RACHS stratifies anatomic diversity into 6 categories based on age, type of surgery performed, and similar in-hospital mortality. The purpose of this retrospective review was to evaluate the use of RACHS in a single-center series as a predictor of outcome in a high-risk newborn population. METHODS In 2003, 793 pediatric cardiac surgical operations (584 open; 209 closed) were performed at our institution. Mortality was 2.1%. Of the 793 operations, 114 were in newborns less than 15 days of age. These 114 newborns were stratified according to RACHS. Two patients could not be stratified and were excluded from analysis. Preoperative, operative, and postoperative variables were compared between the RACHS stratified newborns. RESULTS Unexpectedly, newborns in RACHS category 4 had lower birth weights (3.0 +/- 0.5 kg vs. 3.5 +/- 0.5 kg; P < .05) and a trend toward increased postoperative inotropic score (19 +/- 7 vs. 16 +/- 4), increased postoperative lactic acid (72 +/- 48 vs. 63 +/- 25), increased length of mechanical ventilation (23 +/- 72 days vs. 8 +/- 6 days), increased length of stay (34 +/- 72 days vs. 31 +/- 17 days), and increased mortality (16% vs. 11%) compared with newborns in RACHS category 6. CONCLUSION Limitations of risk assessment using RACHS in a single-center series of high-risk newborns include the lack of consideration of confounding variables. Further risk adjustments that include such confounding variables are warranted.

Short-Term Outcomes in Premature Neonates Adhering to the Philosophy of Supportive Care Allowing for Weight Gain and Organ Maturation Prior to Cardiac Surgery

Background: Prematurity is a recognized risk factor for morbidity and mortality following cardiac surgery. The purpose of this study was to examine short-term outcomes following cardiac surgery in premature neonates adhering to our institutional philosophy of supportive care allowing for weight gain and organ maturation. Methods: Retrospective review of all neonates undergoing cardiac surgery from January 2002 to May 2008. A total of 810 neonates (<30 days of age) were identified. Prematurity defined as less than 36 weeks of gestation. Neonates undergoing ductus arteriosus ligation alone were excluded. In all, 63 neonates comprised the premature group. Term group comprised 244 randomly selected term neonates in a 1:4 ratio. Outcome variables were compared between the 2 groups. Results: Median gestation 34 weeks, range 24 to 35 weeks. Defects: 2 ventricle, normal arch (41% premature vs 44% term; P = .7), 2 ventricle, abnormal arch (24% vs 22%; P = .8), single ventricle, normal arch (21% vs 15%; P = .2), single ventricle, abnormal arch (14% vs 19%; P = .4). Premature neonates were older and smaller at surgery. Cardiopulmonary bypass procedures were performed less frequently in premature neonates (49% vs 69%; P = .004). Length of mechanical ventilation at our institution (6 days [0.5-54) vs 4 days [0.5-49); P = .06); postoperative hospital stay at our institution (17 days [1-161) vs 15 days [0-153); P = .06); and mortality (16% vs 11%; P = .2) was not different between the 2 groups. Conclusion: Early outcome seems independent of weight, prematurity, cardiopulmonary bypass, and type of first intervention. Importantly, there was no statistical difference in mortality between the 2 groups, regardless of how they were treated. Further long-term follow-up is needed in this patient population.

Does preoperative mechanical ventilation affect outcomes in neonates undergoing cardiac surgery

OBJECTIVE To review, in retrospective fashion, the effect of preoperative mechanical ventilation on neonatal outcomes after cardiac surgery. METHODS We studied 114 newborns less than 15 days old admitted to the cardiac intensive care unit for cardiac surgery. Of the newborns, 71 (62%) were mechanically ventilated at the referring hospital before transport to our institution. Of the 71 ventilated patients, 14 were extubated and breathing spontaneously before cardiac surgery. We compared variable haemodynamics and outcomes between the 57 patients mechanically ventilated at time of cardiac surgery, and the 57 patients breathing spontaneously at this time. RESULTS Newborns mechanically ventilated before cardiac surgery had increased preoperative haemodynamic compromise, increased postoperative sepsis (p equal to 0.02) and mortality (p equal to 0.005) compared with those breathing spontaneously before cardiac surgery. CONCLUSION Newborns requiring preoperative mechanical ventilation had greater risk of postoperative morbidity and mortality. Heightened vigilance is warranted in this population of patients at high risk.

Idiopathic infantile arterial calcification: a case report of successful extracorporeal membrane oxygenation support

Idiopathic infantile arterial calcification is a rare cause of infantile ischemic cardiac failure with extremely poor prognosis. We present the first case report of successful extracorporeal membrane oxygenation support and outcome in a child with idiopathic infantile arterial calcification (MAC). This 6-week-old infant presented with cardiogenic shock and circulatory collapse. The patient underwent extracorporeal cardiopulmonary resuscitation, allowing stabilization, diagnosis, and treatment with etidronate, followed by successful discharge to home.