Elena C. Ocampo

North-South twinning in paediatric haemato-oncology : The La Mascota programme, Nicaragua

We describe the La Mascota twinning programme between La Mascota paediatric hospital in Managua, Nicaragua, and hospitals in Monza and Milan, Italy, and Bellinzona, Switzerland. The programme was based on the belief that an attempt to reduce the gap in mortality from cancer in childhood between developed and less developed countries should become an integral part of the care and research activity of a haemato-oncological department of a developed country and not simply an exercise in solidarity. This programme for acute lymphoblastic leukaemia shows that intellectual, organisational, and financial resources can be generated by a twinning programme. What is vital for such programmes is a long-term commitment to a comprehensive and holistic strategy that incorporates supply of drugs, training and supervision of health professionals, and the care of the children and of their parents.

Nutrition algorithms for infants with hypoplastic left heart syndrome; birth through the first interstage period.

Failure to thrive is common in infants with hypoplastic left heart syndrome and its variants and those with poor growth may be at risk for worse surgical and neurodevelopmental outcomes. The etiology of growth failure in this population is multifactorial and complex, but may be impacted by nutritional intervention. There are no consensus guidelines outlining best practices for nutritional monitoring and intervention in this group of infants. The Feeding Work Group of the National Pediatric Cardiology Quality Improvement Collaborative performed a literature review and assessment of best nutrition practices from centers participating in the collaborative in order to provide nutritional recommendations and levels of evidence for those caring for infants with single ventricle physiology.

Incidence and implication of vocal fold paresis following neonatal cardiac surgery.

To study the incidence and implications of vocal fold paresis (VFP) following congenital neonatal cardiac surgery.

Right ventricular infundibulum sparing (RVIS) tetralogy of fallot repair: a review of over 300 patients.

Objective:The natural history of standard (large, transmural right ventriculotomy) repair of tetralogy of Fallot (TOF) is associated with a concerning incidence of right ventricular (RV) failure and reoperation. We believe preserving the infundibulum using a TOF repair method with a mini-(<5 mm) or no ventricular incision optimizes RV function and confers long-term benefit. Over the past 13 years, we have uniformly applied this RV infundibulum sparing (RVIS) strategy. Methods:Using a retrospective cohort study design, 304 TOF patients who underwent the RVIS strategy (July 1995–June 2008) were reviewed. Median weight and age at repair: 8 kg (3–62 kg) and 9 months (2 days–23 years). Seventeen percent (51) of patients required a systemic-to-pulmonary artery shunt. Results:Ninety-nine percent of patients had a mini- 73% (222) or no 26% (79) ventricular incision. Postoperative morbidity included arrhythmias 3% (10), postoperative bleeding 2% (7), temporary renal failure 1% (3), and neurologic injury <1% (2). Thirty-day survival was 99.7%. Overall 1 and 7-year Kaplan-Meier survivals were 97% and 96%. In nonsyndromic children, only 1 patient has died in the RVIS strategy. A total of 3.2% (10) of patients had reoperations. Twenty-one percent (65/304) of patients have been followed for >7 years (median: 8.5 years). None of them have severe dilation, > mild RV outflow obstruction, an arrhythmia, or a pacemaker/AICD. Ninety-five percent of these patients have normal RV function; 3 (4.6%) had mild dysfunction. This cohort has excellent exercise tolerance (MaxVO2 (mean): 41 ± 12 mL/kg/min). Conclusion:The RVIS strategy has allowed morbidity, mortality, and reoperation rates to be minimized. Midterm results suggest that RVIS does appear to preserve RV function. Longer term follow-up will be essential in establishing if the RVIS strategy can change the natural history of repaired TOF.

Treatment of pediatric non-Hodgkin lymphomas in a country with limited resources: results of the first national protocol in Nicaragua.

We report the results of a protocol for the diagnosis and treatment of pediatric non‐Hodgkin lymphomas (NHL) conducted in Nicaragua in the context of an international collaborative program. Fifty‐three children with NHL treated between 1996 and 2003 were retrospectively evaluated. Therapy was designed based on local drug availability and affordability with dose and schedule adaptations for Burkitt and lymphoblastic lymphomas. With a median follow‐up of 3 years, the projected 9‐year overall survival was 63% and event‐free survival 53%. The treatment was efficacious, feasible, and well tolerated in spite of the local socio‐economical conditions. Pediatr Blood Cancer 2008;50:148–152.

Laryngeal ultrasound and vocal fold movement in the pediatric cardiovascular intensive care unit

Vocal fold motion impairment (VFMI) is a known sequela of cardiovascular surgery. The gold standard for the evaluation of vocal fold movement is flexible nasolaryngoscopy (FNL). Although safe, FNL does cause measurable physiologic changes. Noxious stimuli in cardiovascular intensive care unit (CVICU) neonates may cause imbalance between the pulmonary and systemic circulations and potentially circulatory collapse. The goals of this project were to determine the accuracy of laryngeal ultrasound (LUS) compared to FNL to identify VFMI in CVICU neonates and compare their physiologic impact.

Impact of pharmacotherapy on interstage outcomes in single ventricle infants.

OBJECTIVE To characterize the pharmacotherapeutic regimens used in infants with single ventricle heart disease and determine the influence of outpatient medications on interstage weight gain. DESIGN Retrospective review. SETTING Tertiary care pediatric hospital. PATIENTS All patients discharged from our institution with single ventricle heart disease that underwent neonatal first stage surgical palliation between 2002 and 2009 were included. Patients who died prior to second stage palliation or underwent orthotopic heart transplantation were excluded. OUTCOME MEASURES Outpatient medication regimens during the interstage period were reviewed. Medication regimens were compared between surgical eras and between patient groups experiencing different outcomes. A logistic regression model was developed to determine independent factors for an interstage increase in weight-for-age z-score (WAZ) and a linear regression model to determine medications significant for an increase in weight gain per day. RESULTS The study cohort consisted of 161 patients (58% male). Most patients in this cohort had either hypoplastic left heart syndrome (51%) or unbalanced complete atrioventricular canal (29%). Patients were placed on a median of four medications (range 1-9) at discharge from first surgical palliation, with aspirin (79%), furosemide (79%), and angiotensin converting enzyme inhibitors (ACE-I) (73%) most commonly prescribed. A median of six medication doses per day (range 2-18) were prescribed at discharge. Most patients (71%) had a decrease in WAZ during the interstage period. Use of digoxin (P < 0.01) and high-dose furosemide (P = .02) were associated with a decrease in WAZ score during the interstage period. Additionally, the use of ACE-I, ranitidine, proton-pump inhibitors, or promotility agents was not associated with improved somatic growth during the interstage period. CONCLUSIONS Infants with single ventricle heart disease have a high-medication burden during the interstage period. Despite the focused and intensified use of medications to improve feeding tolerance and somatic growth, current pharmacotherapeutic regimens appear to have little effect on interstage weight gain.

Long-term follow-up of vocal fold movement impairment and feeding after neonatal cardiac surgery

OBJECTIVE To determine the long-term prognosis of children with vocal fold mobility impairment (VFMI) after cardiac surgery, with respect to time to normal feeding and incidence of admissions for pneumonia and feeding difficulties. METHODS A retrospective chart review was conducted of all neonates who had otolaryngology exam after cardiac surgery at a tertiary childrens hospital from May 2007 to May 2008. Charts were reviewed for demographics, type of cardiac surgery, vocal fold mobility, diet at time of discharge and at last follow-up, time to full oral feeding, and hospital admissions. RESULTS There were a total of 94 patients included in the study, 17 of whom had VFMI. While significantly more patients with VFMI required modified diet at discharge, 48% compared to 19% of patients with normal vocal fold mobility; there was no statistically significant difference in time to regular diet on long-term follow-up, 0.8 years (VFMI) compared to 0.4 years (normal vocal fold mobility). Of the 25 patients with modified diet or gastrostomy tube at discharge, 52% returned to full feeds within a year. There was no difference in hospitalizations for pneumonia in patients with or without VFMI. However in patients with VFMI, 35% required readmission for feeding difficulty or poor weight gain compared to only 5% in the infants with normal vocal fold mobility. CONCLUSION After neonatal cardiac surgery, there do not appear to be long-term effects of VFMI with regards to readmission for pneumonia. However, there is an increased risk for hospitalization with respect to feeding difficulties in those neonates with VFMI. The overall prognosis for time to oral feeding is good.

The Total Inotrope Exposure Score: an extension of the Vasoactive Inotrope Score as a predictor of adverse outcomes after paediatric cardiac surgery

OBJECTIVE The aim of the present study was to explore and compare the association between a new vasoactive score - the Total Inotrope Exposure Score - and outcome and the established Vasoactive Inotrope Score in children undergoing cardiac surgery with cardiopulmonary bypass DESIGN: The present study was a single-centre, retrospective study. SETTING The study was carried out at a 21-bed cardiovascular ICU in a Tertiary Childrens Hospital between September, 2010 and May, 2011 METHODS: The Total Inotrope Exposure Score is a new vasoactive score that brings together cumulative vasoactive drug exposure and incorporates dose adjustments over time. The performance of these scores - average, maximum Vasoactive Inotrope Score at 24 and 48 hours, and Total Inotrope Exposure Score - to predict primary clinical outcomes - either death, cardiopulmonary resuscitation, or extra-corporeal membrane oxygenation before hospital discharge - and secondary outcomes - length of invasive mechanical ventilation, length of ICU stay, and hospital stay - was calculated. Main results The study cohort included 167 children under 18 years of age, with 37 (22.2%) neonates and 65 (41.3%) infants aged between 1 month and 1 year. The Total Inotrope Exposure Score best predicted the primary outcome (six of 167 cases) with an unadjusted odds ratio for a poor outcome of 42 (4.8, 369.6). Although the area under curve was higher than other scores, this difference did not reach statistical significance. The Total Inotrope Exposure Score best predicted prolonged invasive mechanical ventilation, length of ICU stay, and hospital stay as compared with the other scores. CONCLUSION The Total Inotrope Exposure Score appears to have a good association with poor postoperative outcomes and warrants prospective validation across larger numbers of patients across institutions.

Serum cortisol and early postoperative outcome after stage-1 palliation for hypoplastic left heart syndrome.

Objectives: The postoperative cortisol profile and its association with early outcomes are poorly understood in neonates undergoing surgery for complex congenital heart disease. We investigated the postoperative profile of cortisol and its relationship with the clinical course in a cohort of newborns after stage-1 palliation for hypoplastic left heart syndrome. Design: Prospective observational study. Setting: Pediatric cardiovascular ICU at a tertiary children’s hospital. Subjects: Twenty-three neonates after stage-1 palliation for hypoplastic left heart syndrome between 2009 and 2011. Interventions: None. Measurements and Main Results: Three serial measurements of total serum cortisol after surgery. The first measurement was taken immediately after surgery and the second and third—on the first and second postoperative mornings. The median weight of the infants was 3.0 kg (2.7–3.4 kg), and the age at surgery was 7 days (6–9 d). The median (25th–75th percentile) cortisol levels at admission, day 1, and day 2 were 96.2 &mgr;g/dL (51.1–112 &mgr;g/dL), 17.3 &mgr;g/dL (9.7–25.1 &mgr;g/dL), and 10 &mgr;g/dL (6.5–17 &mgr;g/dL), respectively (p < 0.0001 between admission and day 1). Higher cortisol was associated with greater morbidity, including the need for preoperative ventilation, increased total duration of ventilation, duration of inotropic support, and hospital length of stay. Conclusions: Cortisol levels fell significantly over the first 24 hours after stage-1 palliation for hypoplastic left heart syndrome. A higher postoperative cortisol was associated with increased postoperative morbidity, which warrants further investigation.