Detection rates of congenital heart disease in Guatemala.

OBJECTIVES: In developing countries, congenital heart disease is often unrecognised, leading to serious morbidity and mortality. Guatemala is one of the few developing countries where expert paediatric cardiac treatment is available and affordable, and therefore early detection could significantly improve outcome. We assessed regional congenital heart disease detection rates in Guatemala, and determined whether they correlated with the regional human development index. METHODS: We retrospectively reviewed all new cardiac referrals made in 2006 to the Unidad de Cirugia Cardiovascular Pediatrica, the only paediatric cardiac centre in Guatemala. We calculated regional detection rates by comparing the number of congenital heart disease referrals with the expected incidence using the National Ministry of Health birth data. We then compared the regional detection rates with the human development index data published in the United Nations 2006 Development Program Report using Spearman's rank correlation. RESULTS: An estimated 3935 infants with cardiac defects were born in Guatemala in 2006, an expected 1380 (35%) of whom had severe forms. Overall, only 533 children (14%) with cardiac defects were referred. Of these, 62% had simple shunt lesions, 13% had cyanotic lesions, and 10% had left-sided obstructive lesions. Only 11.5% of referred patients were neonates. Regional detection rates, ranged 3.2-34%, correlated with the regional human development index (r = 0.75, p < 0.0001). CONCLUSIONS: Current detection of congenital heart disease in Guatemala is low and correlates with the regional human development index. Those detected are older and have less severe forms, suggesting a high mortality rate among Guatemalan neonates with complex cardiac defects.

Usefulness of intra-operative epicardial three-dimensional echocardiography to guide aortic valve repair in children.

The aim of this study was to determine the additional important information obtained on prebypass epicardial 3-dimensional imaging (E-3D) compared with transesophageal 2-dimensional echocardiography (TEE-2D) in young patients who undergoing aortic valve repair. From January 2004 to May 2007, all patients who underwent reconstructive surgery of the native aortic valve and intraoperative TEE-2D and E-3D were retrospectively reviewed. Thirteen structural anatomic variables of the aortic valve for TEE-2D and E-3D were evaluated, scored, and compared (by a blinded observer) with intraoperative surgical findings. Nineteen patients underwent valve repair. The median age at surgery was 10 years (range 1 day to 24 years). The primary aortic valve disease was regurgitation (n = 19), and 2 patients had additional valvar stenosis. TEE-2D and E-3D were able to detect 82% (n = 204) and 91% (n = 225), respectively, of the intraoperative findings (n = 247) (p = 0.006). Individual evaluation scores were higher for E-3D (median 12, interquartile range 11 to 13) than for TEE-2D (median 11, interquartile range 10 to 12) (p = 0.01) compared with surgical findings (score 13). Differences in detection sensitivity occurred for commissural fusion (n = 7), leaflet perforation or deficiency (n = 5), and leaflet prolapse (n = 2). TEE-2D was more likely to have false-negative findings than E-3D (36 vs 16 findings, p = 0.001). In conclusion, intraoperative E-3D provides additional important information over TEE-2D for aortic valve repair in young patients. Such 3-dimensional echocardiographic imaging has become an important intraoperative modality for valve repair at the investigators' institution.

Ventricular function deteriorates with recurrent coarctation in hypoplastic left heart syndrome.

BACKGROUND: Recurrent coarctation (re-CoA) after stage I palliation in hypoplastic left heart syndrome (HLHS) is deleterious. We studied whether re-CoA had an effect on ventricular systolic function. METHODS: Retrospectively reviewed were HLHS patients surviving stage I Norwood palliation (stage I) and cavopulmonary shunt (CPS) between January 2004 and February 2007. Echocardiographic right ventricular fractional area change (RV-FAC) was used to evaluate ventricular systolic function after stage I, before CPS, and before Fontan procedures. Cardiac catheterization and magnetic resonance imaging data before CPS were reviewed to assess re-CoA, using a coarctation index (CI = isthmus diameter/descending aortic diameter). RESULTS: Fifty-one patients were included, and 21 had a CI of less than 0.75 (mean, 0.82 +/- 0.19; 21). Twelve patients required arch balloon dilation between CPS and Fontan. The change of RV-FAC for all patients between stage I and CPS was -2.2% +/- 9.6%. Pearson correlation coefficient demonstrated a significant correlation between lower CI values and lower RV-FAC at the pre-CPS echocardiogram (r = .35, p = 0.03); and lower CI values and greater decrease in RV-FAC between stage I and pre-CPS evaluation (r = 0.40, p = 0.018). At follow-up pre-Fontan, RV-FAC for patients who underwent balloon dilation for re-CoA recovered to a level that was inferior but not significantly different from that of patients who did not need balloon dilation. CONCLUSIONS: Recurrent aortic arch obstruction after stage I for HLHS is associated with worse RV systolic function at the time of stage II operation. Timely intervention on the re-CoA results in recovery of RV function.

Measurement of technical performance in congenital heart surgery: a pilot study.

BACKGROUND: Although adequacy of repair after congenital heart surgery is a crucial determinant of clinical outcome, there is no current method of assessment. We sought to develop a process to measure the adequacy of repair for a diverse group of congenital heart procedures. METHODS: Selected surgical procedures, consisting of repair of ventricular septal defect (VSD), tetralogy of Fallot (TOF), complete common atrioventricular canal (CAVC), and arterial switch operation, were divided into component subprocedures, each of which was assessed separately. Three outcome categories of "optimal," "adequate," and "inadequate" were defined by consensus according to postprocedure echocardiographic assessment. Outcome categories for conduction disturbance were also created. All patients undergoing one of the four procedures in 2004 were identified, and each subprocedure was assessed. Other clinical data were obtained from medical records. Repairs were scored as "optimal" if all attempted subprocedures and conduction were optimal, and "inadequate" if any was inadequate. RESULTS: A total of 138 procedures were included. VSD repair was done in 46 patients (33%), TOF repair in 33 (24%), arterial switch operation in 36 (26%), and CAVC repair in 23 (17%). Optimal technical score was found in 28 (20%), adequate in 106 (77%), and inadequate in 4 (3%) (2 VSD, 1 TOF, 1 CAVC). Median length of stay was 8 days, and no patients died. CONCLUSIONS: Despite procedural diversity and complexity, technical adequacy of repair can be assessed for congenital heart surgery.