William M. Novick

Plastic bronchitis occurring late after the Fontan procedure : Treatment with aerosolized urokinase

Objective To describe the use of aerosolized urokinase in a patient with plastic bronchitis after a Fontan procedure. Design Case report. Setting Pediatric intensive care unit in a university-affiliated children’s hospital. Patients Report of one patient with acute respiratory failure secondary to plastic bronchitis. Interventions Aerosolized urokinase, multiple bronchoscopies, corticosteroids, mucolytics, bronchodilators, and atrial pacing. Measurements and Main Results Airway obstruction secondary to recurring casts improved with the treatments. Histologic analysis of the casts demonstrated less fibrin after treatments with aerosolized urokinase. No adverse events were noted. Conclusions The addition of aerosolized urokinase to this patient’s treatment regimen helped to resolve life-threatening airway obstruction secondary to fibrin casts.

Reducing Mortality and Infections After Congenital Heart Surgery in the Developing World

BACKGROUND: There is little information about congenital heart surgery outcomes in developing countries. The International Quality Improvement Collaborative for Congenital Heart Surgery in Developing World Countries uses a registry and quality improvement strategies with nongovernmental organization reinforcement to reduce mortality. Registry data were used to evaluate impact. METHODS: Twenty-eight sites in 17 developing world countries submitted congenital heart surgery data to a registry, received annual benchmarking reports, and created quality improvement teams. Webinars targeted 3 key drivers: safe perioperative practice, infection reduction, and team-based practice. Registry data were audited annually; only verified data were included in analyses. Risk-adjusted standardized mortality ratios (SMRs) and standardized infection ratios among participating sites were calculated. RESULTS: Twenty-seven sites had verified data in at least 1 year, and 1 site withdrew. Among 15 049 cases of pediatric congenital heart surgery, unadjusted mortality was 6.3% and any major infection was 7.0%. SMRs for the overall International Quality Improvement Collaborative for Congenital Heart Surgery in Developing World Countries were 0.71 (95% confidence interval [CI] 0.62–0.81) in 2011 and 0.76 (95% CI 0.69–0.83) in 2012, compared with 2010 baseline. SMRs among 7 sites participating in all 3 years were 0.85 (95% CI 0.71–1.00) in 2011 and 0.80 (95% CI 0.66–0.96) in 2012; among 14 sites participating in 2011 and 2012, the SMR was 0.80 (95% CI 0.70–0.91) in 2012. Standardized infection ratios were similarly reduced. CONCLUSIONS: Congenital heart surgery risk-adjusted mortality and infections were reduced in developing world programs participating in the collaborative quality improvement project and registry. Similar strategies might allow rapid reduction in global health care disparities.

The fate of homograft conduits in children with congenital heart disease: an angiographic study

The use of homograft conduits in the repair of congenital heart disease is widely accepted. We reviewed the catheterization and angiographic data from 20 patients with homograft conduits. All conduits were to the pulmonary arteries. The age at operation was 4.7 +/- 5.6 years (mean +/- standard deviation) and at follow-up catheterization, 7.8 +/- 6.7 years. At implantation, conduit cross-sectional area and Z value were 219 +/- 96 mm2 and 3.5 +/- 1.8, respectively. At subsequent catheterization, the conduit diameters were measured in two projections at the shaft, annulus, valve opening, and insertion into the pulmonary artery. The transconduit gradient was 47 +/- 26 mm Hg. The cross-sectional areas were 149 +/- 56 mm2 at the shaft, 151 +/- 92 mm2 at the annulus, 108 +/- 116 mm2 at the valve opening, and 127 +/- 84 mm2 at the pulmonary artery insertion. The Z values were -0.9 +/- 2.5, -0.9 +/- 2.8, -3.8 +/- 4.0, and -2.0 +/- 3.4, respectively. The cross-sectional areas and the Z values at the levels of measurement were significantly smaller than the corresponding values at implantation. The change in cross-sectional areas and Z values exceeded what would be expected from growth alone. These data indicate that there is a decrease, with time, in the functional lumen of homograft conduits, and this may have implications for follow-up strategy after implantation.

Are we improving after 10 years of humanitarian paediatric cardiac assistance

BACKGROUND Paediatric cardiovascular services are frequently absent or poorly developed in many countries around the world. Our foundation made 83 trips in support of cardiovascular services between April 1993 and March 2003 to help alleviate this problem. In this study, we present an analysis of our results over these period of 10 years. METHODS We performed a review of all available records relating to the trips, including patient databases, audited financial statements, donated product inventory lists, lists of team members, and follow-up data from the host sites concerning the state of the patients treated. RESULTS We made 83 trips to 14 countries, 40 of these being in Central Europe, 5 in Eastern Europe, 10 in Caribbean, and Central America, 18 in South America, 9 in Asia, and 1 in the Middle East. In the first 5 years, we made 23, as opposed to 60 in the second 5 years, this difference being significant (p less than 0.01). The total number of primary operations performed over 10 years was 1,580. The number of procedures performed yearly increased over the two intervals from 97.0 plus or minus 32.7 to 219.0 plus or minus 41.7, p less than 0.002. The probability of survival between the periods increased from 84.6 to 93.3 per cent, and this was also significantly different (p less than 0.001). Overall, the rate of survival for the period of 10 years was 90.5 per cent. Moreover, the value of services donated to support each trip also differed significantly, decreasing from 105,900 dollars plus or minus 14,581 dollars for the first period to 54,617 dollars plus or minus 11,425 dollars for the second period (p less than 0.001). CONCLUSIONS Improving paediatric cardiac services in under-served countries requires significant financial and personnel commitments, but can produce reasonable outcomes.

Double patch closure of ventricular septal defect with increased pulmonary vascular resistance

BACKGROUND Closure of a large ventricular septal defect (VSD) in children with elevated pulmonary vascular resistance is associated with significant morbidity and mortality. Pulmonary hypertensive episodes continue to be a major cause of postoperative morbidity and mortality. We designed a fenestrated flap valve double VSD patch in an effort to decrease the morbidity and mortality associated with the closure of a large VSD with elevated pulmonary vascular resistance. METHODS Eighteen children (mean age, 5.7 years) with a large VSD and elevated pulmonary vascular resistance (mean, 11.4 Wood units) underwent double patch VSD closure using moderately hypothermic cardiopulmonary bypass and cardioplegic arrest. The routine VSD patch was fenestrated (4 to 6 mm) and on the left ventricular side of the patch, a second, smaller patch was attached to the fenestration along its superior margin before closure of the VSD. RESULTS All children survived operation and were weaned from inotropic and ventilator support within 48 hours postoperatively. Postoperative pulmonary artery pressures were significantly lower than preoperative values. One child died 9 months postoperatively. CONCLUSIONS Closure of a large VSD in children with elevated pulmonary vascular resistance can be performed with low morbidity and mortality when a flap valve double VSD patch is used.

Survey of Nongovernmental Organizations Providing Pediatric Cardiovascular Care in Low- and Middle-Income Countries

Background: Nearly 90% of the children with heart disease in low- and middle-income countries (LMICs) cannot access cardiovascular (CV) services. Limitations include inadequate financial, human, and infrastructure resources. Nongovernmental organizations (NGOs) have played crucial roles in providing clinical services and infrastructure supports to LMICs CV programs; however, these outreach efforts are dispersed, inadequate, and lack coordination. Methods: A survey was sent to members of the World Society for Pediatric and Congenital Heart Society and PediHeart. Results: A clearinghouse was created to provide information on NGO structures, geographic reach, and scope of services. The survey identified 80 NGOs supporting CV programs in 92 LMICs. The largest outreach efforts were in South and Central America (42%), followed by Africa (18%), Europe (17%), Asia (17%), and Asia-Western Pacific (6%). Most NGOs (51%) supported two to five outreach missions per year. The majority (87%) of NGOs provided education, diagnostics, and surgical or catheter-based interventions. Working jointly with LMIC partners, 59% of the NGOs performed operations in children and infants; 41% performed nonbypass neonatal operations. Approximately a quarter (26%) reported that partner sites do not perform interventions in between missions. Conclusions: Disparity and inadequacy in pediatric CV services remain an important problem for LMICs. A global consensus and coordinated efforts are needed to guide strategies on the development of regional centers of excellence, a global outcome database, and a CV program registry. Future efforts should be held accountable for impacts such as growth in the number of independent LMIC programs as well as reduction in mortality and patient waiting lists.

Novel use of extracellular matrix graft for creation of pulmonary valved conduit.

We report the use of a commercially available graft material to create a trileaflet pulmonary valved conduit, which was implanted in a 12-year-old girl with mixed aortic valve disease as part of a Ross procedure. The patient presented with severe aortic insufficiency and left ventricular dilatation following balloon valvuloplasty. The patient lives in Ecuador, where most commonly used replacements for the pulmonary valve are either unavailable or unaffordable. Our technique involves the use of porcine small intestinal submucosal extracellular matrix (SIS-ECM), commercially available as CorMatrix ECM (CorMatrix Cardiovascular, Inc, Tallahassee, Florida) to create a trileaflet-valved conduit.

Impact of Ultrafiltration on Blood Use for Atrial Septal Defect Closure in Infants and Children

BACKGROUND Infants and children undergoing open cardiac operations have a high incidence of blood product transfusion. Ultrafiltration has been shown to reverse hemodilution and improve myocardial function and hemodynamics after cardiopulmonary bypass (CPB). METHODS The effect of ultrafiltration on the amount of blood transfusion and hospital charge in 39 consecutive patients who underwent elective atrial septal defect repair was examined. Patients in group I (n=26) had a conventional cardiopulmonary circuit prime with blood, whereas 13 patients had bloodless prime (group II). Ultrafiltration was used immediately after weaning from CPB in group II. The patients in group I received blood products after discontinuation of CPB to achieve a hematocrit of 30%. The amount of blood product used, hematocrit immediately after CPB and on arrival in intensive care unit, postoperative hemodynamics and saturations, total operating room charge, blood charge, hospital stay, and hospital charge were compared. RESULTS Mean body weight (15.8 kg in group I versus 17.5 kg in group II) and preoperative hematocrit values (35.6% in group I versus 34.2% in group II) were similar. Mean hematocrit immediately after CPB was 22% and 14% in group I and II, respectively (p < 0.0001). The mean hematocrit upon arrival to the intensive care unit was 34% in group I and 22% in group II (p < 0.0001). The amount of blood product transfusion was 32 mL/kg in group I and 3 mL/kg in group II patients (p < 0.0001). The patients in group II had significantly less blood bank charges; however, operating room charges and total hospital charges were similar between the two groups. CONCLUSIONS Elective atrial septal defect repair was performed with no blood product transfusion without increased morbidity or hospital stay. Ultrafiltration can be used to reverse hemodilution resulting from a bloodless CPB prime without an increase in hospital charge.

Pediatric Cardiovascular Surgery in South America: Current Status and Regional Differences

Very little information is available about the epidemiology of congenital heart disease in developing parts of the world, including South America. This article describes the incidence of congenital cardiac disease, the different treatment rates among countries, and future solutions for achieving improved coverage for the children with cardiac diseases in South America. An incidence of congenital cardiac disease of 8 per 1000 live births appears to be a fair approximation for the population of the world and also the population in South America. Nevertheless, a wide variation exists in the observed incidence of congenital cardiac disease in South American countries, which can be partly explained by inequalities in the access to diagnosis, differences in the diagnostic criteria, and true regional variations. It is estimated that 58,718 children are born yearly with congenital heart disease in South America. Brazil, Colombia, and Argentina have the highest number, followed by Peru, Venezuela, Chile, Ecuador, Bolivia, Paraguay, Uruguay, and Guyana. It is also estimated that in South America, 24,081 children per year with a new diagnosis of congenital cardiac disease do not receive any treatment. This paper provides strategies for improving the access to and quality of pediatric cardiac surgery in South America.

Dr William Novick (Founder and Medical Director of International Children’s Heart Foundation), speaks to associate editor Dr Tom R. Karl

WILLIAM NOVICK’S NAME IS SYNONYMOUS WITH humanitarian initiatives in paediatric cardiac surgery. As the founder and director of the International Children’s Heart Foundation he has reached out to children and families in some 28 countries over the past three decades. International Children’s Heart Foundation now has over 7300 surgical procedures in the organisational database, and 16 former host units are operating autonomously or with only minimal technical assistance. I spoke with Bill on 5 September, 2014, and had a unique opportunity to hear the viewpoint of the driving force behind one of the most successful teams working in this realm.