Cardiac surgical missions: what works, what does not, where we need to go from here.

PURPOSE OF REVIEW: Although overall neonatal and infant mortality continues to decrease around the world, years of life lost to congenital heart disease (CHD) rank among the top 10 leading causes for that age group in Latin America, Central Asia, Africa and the Middle East. Short-term surgical missions (STSMs) continue to be the only resource available in some areas of the world. RECENT FINDINGS: There is a need for better communications between charity organizations and the supported institutions. We observe a raising concern with the limited published information on the results of the STSMs and a call for patient safety, the use of international databases and accountability.Funding and human resources in low-income and middle-income countries (LMIC) are a persistent problem and a shift toward long-term collaboration rather than short-term visits opens the path to sustainability.The cost-effectiveness of STSMs treating congenital heart disease in LMIC seems to be very high whereas the management of adults with CHD and rheumatic valvulopathies appear as a growing concern. SUMMARY: Pediatric cardiac surgery STSMs continue to deserve full support and funding, long-term commitments, accountability and coordinated international funding are needed to achieve global sustainable coverage.

Berry syndrome: a possible genetic link.

Berry syndrome comprises a rare combination of heart defects that includes aortopulmonary window, interrupted aortic arch, intact ventricular septum, and aortic origin of the right pulmonary artery. We report the case of a neonate confirmed to have Berry syndrome by transthoracic echocardiogram and computed tomography (CT). This neonate had the additional finding of an aberrant right subclavian artery arising from the descending aorta. A single-stage repair was successfully performed when the infant was 7 days of age. Genetic testing showed a 102-kb deletion within chromosome band 9p24.2; this deletion has not been previously linked to congenital heart defects. Berry syndrome can be diagnosed accurately by transthoracic echocardiogram and CT. There may be an underlying genetic etiology, and this possibility warrants further investigation.

Pulmonary function and exercise capacity for ELBW survivors in preadolescence: effect of neonatal chronic lung disease.

OBJECTIVE: To assess pulmonary function and exercise capacity of apparently asymptomatic children who were born extremely preterm. STUDY DESIGN: Pulmonary function and treadmill testing were performed on 50 children 9 to 15 years old who had birth weight <801 g (extremely low birth weight [ELBW]) and without apparent neurodevelopmental or pulmonary disabilities, compared with 25 children born at term with normal birth weight (NBW). Medical history and physical activity levels were assessed by parent questionnaire. Group differences were determined by two-sample t test. Secondary analysis was performed to assess significant influence of neonatal chronic lung disease (CLD) on outcome measures. RESULTS: Twenty percent of ELBW subjects but no subjects with NBW had clinically abnormal pulmonary function (>2 SD from norms, P=.026). All significant pulmonary function differences except peak expiratory flow rate percentages were accounted for by ELBW subjects who had CLD. Oxygen consumption measurements were significantly lower for subjects with ELBW (30.3+/-6.9 and 38.5+/-5.2 mL/kg/min, P=.000), independent of CLD status. CONCLUSION: Pulmonary function abnormalities consistent with obstructive lung disease were more frequently detected in ELBW children who had neonatal CLD. Low oxygen consumption measurements suggest a lower level of fitness for ELBW children compared with children with NBW.