Delivering pediatric cardiac care in sub-Saharan Africa: a model for the developing countries.

PURPOSE OF REVIEW: It is projected that by 2050, around 40% of all births, and about 40% of all children, will be in Africa, up from about 10% in 1950. Consequently, this trend will cause an increase in noncommunicable diseases in children, such as congenital and rheumatic heart diseases. The current state of pediatric cardiac care in sub-Saharan Africa is dire with some countries without cardiac surgical services at all. The purpose of this review is to highlight those components needed to build a sustainable model for a pediatric cardiac care center in sub-Saharan Africa. RECENT FINDINGS: Review of the literature reveals that capacity-building for pediatric cardiac care in sub-Saharan Africa can be a challenging entity. Several factors must come into play to lay the foundation for a successful cardiac program. Key among them are early diagnosis of heart disease, human resources, financing cardiac care, and political commitment. SUMMARY: The burgeoning pediatric population in sub-Saharan African lends itself to an increase in the incidence of pediatric heart disease. The need for sustainable, patient-centered cardiac centers is pressing. Establishing such pediatric cardiac care models will require the essential components of early diagnosis, increasing human resources, financing cardiac care, and political commitment. VIDEO ABSTRACT: http://links.lww.com/HCO/A59.

Celiac artery flow pattern in infants with single right ventricle following the Norwood procedure with a modified Blalock-Taussig or right ventricle to pulmonary artery shunt.

A potential advantage of the right ventricle to pulmonary artery versus modified Blalock-Taussig shunt in patients undergoing the Norwood procedure is limitation of diastolic runoff from the systemic to pulmonary circulation. We evaluated mesenteric flow patterns and gastrointestinal outcomes following the Norwood procedure associated with either shunt type. Patients randomized to a right ventricle to pulmonary artery versus modified Blalock-Taussig shunt in the Pediatric Heart Network Single Ventricle Reconstruction Trial at centers participating in this ancillary study were eligible for inclusion; those with active necrotizing enterocolitis, sepsis, or end-organ dysfunction were excluded. Celiac artery flow characteristics and gastrointestinal outcomes were collected at discharge. Forty-four patients (five centers) were included. Median age at surgery was 5 days [interquartile range (IQR) = 4-8 days]. Median celiac artery resistive index (an indicator of resistance to perfusion) was higher in the modified Blalock-Taussig shunt group (n = 19) versus the right ventricle to pulmonary artery shunt group (n = 25) [1.00 (IQR = 0.84-1.14) vs. 0.82 (IQR = 0.74-1.00), p = 0.02]. There was no difference in interstage weight gain, necrotizing enterocolitis, or feeding intolerance episodes between the groups. The celiac artery resistive index was higher in patients with the modified Blalock-Taussig shunt versus the right ventricle to pulmonary artery shunt but was not associated with measured gastrointestinal outcomes.

Group B streptococcal meningitis: cerebrospinal fluid parameters in the era of intrapartum antibiotic prophylaxis.

OBJECTIVE: Describe cerebrospinal fluid parameters in infants with culture-proven Group B streptococcal meningitis in the era of intrapartum antibiotic prophylaxis. STUDY DESIGN: Cohort study of the first lumbar puncture from 13,495 infants cared for at 150 neonatal intensive care units. We compared cerebrospinal fluid parameters [white blood cell count, red blood cell count, glucose, and protein], demographics, and outcomes between infants with and without Group B streptococcal meningitis. RESULTS: We identified 46 infants with Group B streptococcal meningitis. The median cerebrospinal fluid white blood cell count was 271 cells/mm(3) for infants with Group B streptococcal meningitis and 6 cells/mm(3) for infants without meningitis (p=0.0001). Of the infants with Group B streptococcal meningitis, 9/46 (20%) had negative blood cultures. Meningitis complicated 22/145 (15%) of episodes of early-onset Group B streptococcal sepsis and 13/23 (57%) of episodes of late-onset Group B streptococcal sepsis. CONCLUSIONS: Group B streptococcal meningitis occurs in the presence of negative blood cultures. In hospitalized infants who undergo a lumbar puncture, Group B streptococcal sepsis is frequently complicated by GBS meningitis.

Electrocardiographic response to enzyme replacement therapy for Pompe disease.

PURPOSE: Electrocardiogram (ECG) abnormalities are universal in infantile Pompe disease or glycogen storage disease type II, a fatal genetic muscle disorder caused by deficiency of acid alpha-glucosidase (GAA). Hallmarks of this disease include a shortened PR interval, an increased QT dispersion (QTd), and large left ventricular (LV) voltages. We evaluated the effect of recombinant human GAA (rhGAA) enzyme replacement therapy (ERT) on these ECG parameters in patients with infantile-onset Pompe disease. METHODS: A total of 134 ECGs were evaluated from 19 patients (5 females and 14 males) with a median age of 5.5 months at the time of enrollment in open-label clinical trials exploring the safety and efficacy of ERT at a single center from 1999 to 2004. rhGAA was purified from genetically engineered Chinese hamster ovary cells overproducing GAA and infused intravenously at doses ranging from 10 mg/kg per week to 20 to 40 mg/kg every 2 weeks in patients with infantile-onset Pompe disease. The PR interval, QTd (longest to shortest QT), and LV voltage (SV1 + RV6) were blindly determined by two independent observers. RESULTS: The median follow-up period was 6 months (range 2-30 months). The PR interval lengthened from 83 (42-110) ms to 107 (95-130) ms (P < .001), and the QTd decreased from 83 (40-125) ms to 53 (20-80) ms (P = .003). There were significant decreases in LV voltage (67 [17-83] mV vs. 48 [18-77] mV, P = .03), which correlated with decrease in LV mass on two-dimensional echocardiogram. There was no evident change in the QTc interval (429 [390-480] ms vs. 413 [370-450] ms, P = not significant). CONCLUSION: rhGAA ERT for infantile Pompe disease results in an increase in PR interval and a decrease in both the QTd and the LV voltage. These results suggest that these ECG parameters may be useful markers of the severity of cardiac disease and the response to ERT treatment in patients with infantile Pompe disease.

Ambulatory electrocardiogram analysis in infants treated with recombinant human acid alpha-glucosidase enzyme replacement therapy for Pompe disease.

PURPOSE: Infantile Pompe disease is caused by deficiency of lysosomal acid alpha-glucosidase. Trials with recombinant human acid alpha-glucosidase enzyme replacement therapy (ERT) show a decrease in left ventricular mass and improved function. We evaluated 24-hour ambulatory electrocardiograms (ECGs) at baseline and during ERT in patients with infantile Pompe disease. METHODS: Thirty-two ambulatory ECGs were evaluated for 12 patients with infantile Pompe disease from 2003 to 2005. Patients had a median age of 7.4 months (2.9-37.8 months) at initiation of ERT. Ambulatory ECGs were obtained at determined intervals and analyzed. RESULTS: Significant ectopy was present in 2 of 12 patients. Patient 1 had 211 and 229 premature ventricular contractions (0.2% of heart beats) at baseline and at 11.5 weeks of ERT, respectively. Patient 2 had 10,445 premature ventricular contractions (6.7% of heart beats) at 11 weeks of therapy. CONCLUSION: Infantile Pompe disease may have preexisting ectopy; it may also develop during the course of ERT. Therefore, routinely monitoring patients using 24-hour ambulatory ECGs is useful. Periods of highest risk may be early in the course of ERT when there is a substantial decrease in left ventricular mass and an initial decrease in ejection fraction.