Differences in Quality of Life in Children Across the Spectrum of Congenital Heart Disease.

OBJECTIVE: To create complexity groups based upon a patient's cardiac medical history and to test for group differences in health-related quality of life (HRQOL). METHODS: Patients 8-18 years with congenital heart disease (CHD) and parent-proxies from the Pediatric Cardiac Quality of Life Inventory (PCQLI) Testing Study were included. Outcome variables included PCQLI Total, Disease Impact, and Psychosocial Impact scores. Using a patient's medical history (cardiac, neurologic, psychological, and cognitive diagnosis), latent class analysis (LCA) was used to create CHD complexity groups. Covariates included demographics and burden of illness (number of: school weeks missed, physician visits in the past year, and daily medications). Generalized estimation equations tested for differences in burden of illness and patient and parent-proxy PCQLI scores. RESULTS: Using 1482 CHD patients (60% male; 84% white; age 12.3 ± 3.0 years), latent class analysis (LCA) estimates showed 4 distinct CHD complexity groups (Mild, Moderate 1, Moderate 2, and Severe). Increasing CHD complexity was associated with increased risk of learning disorders, seizures, mental health problems, and history of stroke. Greater CHD complexity was associated with greater burden of illness (P < .01) and lower patient- and parent-reported PCQLI scores (P < .001). CONCLUSIONS: LCA identified 4 congenital heart disease (CHD) complexity groupings. Increasing CHD complexity was associated with higher burden of illness and worse patient- and parent-reported HRQOL.

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.

Intermediate follow-up following intravascular stenting for treatment of coarctation of the aorta.

BACKGROUND: We report a multiinstitutional study on intermediate-term outcome of intravascular stenting for treatment of coarctation of the aorta using integrated arch imaging (IAI) techniques. METHODS AND RESULTS: Medical records of 578 patients from 17 institutions were reviewed. A total of 588 procedures were performed between May 1989 and Aug 2005. About 27% (160/588) procedures were followed up by further IAI of their aorta (MRI/CT/repeat cardiac catheterization) after initial stent procedures. Abnormal imaging studies included: the presence of dissection or aneurysm formation, stent fracture, or the presence of reobstruction within the stent (instent restenosis or significant intimal build-up within the stent). Forty-one abnormal imaging studies were reported in the intermediate follow-up at median 12 months (0.5-92 months). Smaller postintervention of the aorta (CoA) diameter and an increased persistent systolic pressure gradient were associated with encountering abnormal follow-up imaging studies. Aortic wall abnormalities included dissections (n = 5) and aneurysm (n = 13). The risk of encountering aortic wall abnormalities increased with larger percent increase in CoA diameter poststent implant, increasing balloon/coarc ratio, and performing prestent angioplasty. Stent restenosis was observed in 5/6 parts encountering stent fracture and neointimal buildup (n = 16). Small CoA diameter poststent implant and increased poststent residual pressure gradient increased the likelihood of encountering instent restenosis at intermediate follow-up. CONCLUSIONS: Abnormalities were observed at intermediate follow-up following IS placement for treatment of native and recurrent coarctation of the aorta. Not exceeding a balloon:coarctation ratio of 3.5 and avoidance of prestent angioplasty decreased the likelihood of encountering an abnormal follow-up imaging study in patients undergoing intravascular stent placement for the treatment of coarctation of the aorta. We recommend IAI for all patients undergoing IS placement for treatment of CoA.