Individual pulmonary vein size and survival in infants with totally anomalous pulmonary venous connection

AbstractObjectives. We investigated whether mortality in totally anomalous pulmonary venous connection could be predicted from preoperative individual pulmonary vein size.Background. Some infants with this anomaly die with or without surgical repair because of stenosis of individual pulmonary veins.Methods. Individual pulmonary vein, vertical vein and pulmonary venous confluence diameters were retrospectively measured from preoperative echocardiograms in 32 infants with totally anomalous pulmonary venous connection presenting to Children's Hospital, Boston over a 1/2-year period. Data on body surface area, other cardiac anomalies, presence of initial pulmonary venous obstruction and early surgery and outcome were also recorded.Results. Of 32 patients, 6 (18.8%) died before hospital discharge, and 8 (25.0%) died subsequently. Six (75.0%) of the eight patients who died late had individual pulmonary vein stenosis at sites remote from the surgical anastomosis to the left atrium. The remaining 18 patients (56.3%) are alive at a mean follow-up period of 9.7 months. A Cox proportional hazard model revealed that small sum of individual pulmonary vein diameters (p = 0.0004), small confluence size (p = 0.02) and presence of heterotaxy syndrome (p = 0.008) were each significant univariate predictors of survival. Multivariate analysis showed that small pulmonary vein sum was a strong predictor of survival (p = 0.008), independent of the presence of heterotaxy syndrome. An analysis stratified by the presence of heterotaxy syndrome showed that the predictive effect of small pulmonary vein sum on survival was strongest in patients without heterotaxy syndrome.Conclusions. These data show that individual pulmonary vein size at diagnosis is a strong, independent predictor of survival in patients with totally anomalous pulmonary venous connection. In patients with this anomaly and small individual pulmonary veins, the anomaly may not be correctable by surgical creation of an anastomosis between the pulmonary venous confluence and the left atrium

Limitations of fractional shortening as an index of contractility in pediatric patients infected with human immunodeficiency virus

Left ventricular fractional shortening (FS) is dependent on left ventricular preload and afterload, as well as contractility. Contractility may therefore not be accurately described by FS, especially in infants and children infected with human immunodeficiency virus (HIV), who tend to have abnormal left ventricular preload and afterload. We therefore examined the magnitude and clinical impact of the discrepancy between FS and contractility by assessment of 177 echocardiograms from 76 HIV-infected pediatric patients (median age, 1.9 years). The studies included simultaneous measurements of left ventricular FS, contractility, preload, and afterload. The correlation between contractility and FS was modest ( r = 0.70; p < 0.001), and was weaker in children less than 2 years of age ( r = 0.52) than in older children ( r = 0.84). FS incorrectly predicted contractility in 46% of the studies; 43% with depressed FS (1 SD, and for 36% the difference was >2 SD. These differences remained after adjustment of FS for age or body surface area. Afterload was abnormal in 42% and preload in 21% of all echocardiograms. High preload predicted that FS would overestimate contractility ( p = 0.002); high afterload predicted that FS would underestimate contractility ( p <0.001). The discrepancy between FS and contractility was larger among children who were younger, had more advanced HIV disease, or were not sedated during echocardiography. One third of children with congestive symptoms had normal contractility and depressed FS; the discrepancy was primarily due to loading conditions. We conclude that the high incidence of abnormal loading conditions in HIV-infected infants and children limits the usefulness of load-dependent FS for assessing contractility. Measurements of loading conditions and load-independent indexes, which more directly reflect contractility, allow a more accurate determination of myocardial status and may lead to better clinical management. (J P EDIATR 1994;125:563-70

Design and implementation of the North American Pediatric Cardiomyopathy Registry

The Pediatric Cardiomyopathy Registry (PCMR) was established to describe the epidemiologic features and clinical course of selected cardiomyopathies in patients aged 18 years or younger and to promote the development of etiology-specific treatments. Sixty-one private and institutional pediatric cardiomyopathy practices in the United States and Canada were recruited to participate in the PCMR. The registry consists of a prospective, population-based cohort of patients in 2 regions (New England and the Central Southwestern United States) and a retrospective cohort of patients diagnosed between 1991 and 1996. Annual follow-up data are collected on all patients. As of June 1999, the PCMR consisted of 337 prospectively identified and 990 retrospectively identified patients. The PCMR has demonstrated the feasibility of establishing a large database of sociodemographic and clinical information on children with pediatric cardiomyopathy. Through this cooperative effort, the PCMR will obtain precise estimates of the incidence of pediatric cardiomyopathy and a better understanding of the natural history of this disease. (Am Heart J 2000;139:S86-S95.