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Linda Cripe

Publications and source records attributed to Linda Cripe.

4 recordsLinked to original sources

Bicuspid aortic valve is heritable.

OBJECTIVES: Previous studies have established familial clustering of bicuspid aortic valve (BAV), presumably indicating genetic inheritance. Our objective was to statistically test whether the segregation pattern of BAV is consistent with genetic inheritance and to obtain an estimate of the size of the genetic effect (heritability). BACKGROUND: Bicuspid aortic valve occurs in 1% of the population, making it the most common cardiovascular malformation (CVM). Bicuspid aortic valve is frequently an antecedent to aortic valve stenosis or insufficiency and is often associated with other CVMs, including aortic root dilation. The genetic and developmental significance of these findings remains obscure. METHODS: In 50 probands with BAV, we obtained a three-generation family history and echocardiograms on first-degree relatives. Heritability (h2) of BAV and BAV and/or other CVMs were estimated using maximum-likelihood-based variance decomposition extended to dichotomous traits implemented in the computer package Sequential Oligogenic Linkage Analysis Routines (SOLAR, San Antonio, Texas). RESULTS: A total of 309 probands and relatives participated. Bicuspid aortic valve was identified in 74 individuals (prevalence = 24%). A total of 97 individuals had BAV and/or other CVM (prevalence = 31%), including aortic coarctation, ventricular or atrial septal defect, abnormal mitral valve, aortic root dilation, or hypoplastic left heart syndrome. The heritability (h2) of BAV and BAV and/or other CVMs were 89% and 75%, respectively. CONCLUSIONS: The high heritability of BAV suggests that in this study population BAV determination is almost entirely genetic. The heritability of BAV plus other cardiovascular anomalies suggests that valve malformation can be primary to defective valvulogenesis or secondary to other elements of cardiogenesis.

Adolescent↗

Risk of recoarctation should not be a deciding factor in the timing of coarctation repair.

To determine whether early coarctation repair is a significant risk for recoarctation in the modern era, 120 patients, including 87 infants, who underwent isolated coarctation repair at a single institution, were reviewed. At a mean follow-up of 44.4 months, there have were no late reoperations, and 2 patients required balloon aortoplasty. The overall incidence of late reintervention was 1.7%, with only 2.4% (2 of 83) in those <1 year old.

Aortic Coarctation↗

Usefulness of the mitral valve Z score in predicting the need to close moderate- to large-sized atrial septal defects in infants with aortic coarctation.

Left ventricular anatomic substrates predisposing the development of congestive heart failure in infants with atrial left-to-right shunting after aortic coarctation repair were characterized. Mitral valve, but not aortic or left ventricular, hypoplasia correlated with the development of heart failure and the need for atrial defect closure shortly after coarctation repair.

Aortic Coarctation↗

Conversion to digital technology improves efficiency in the pediatric echocardiography laboratory.

BACKGROUND: Although the videotape method (VTM) is commonly used to record and intrepret ecocardiographic images, many pediatric echocardiographers are considering acquiring to, interpreting from, and storing their images to digital disk using the single-beat digital acquisition method (SBM). The paucity of image redundancy using SBM should translate into improved laboratory efficiency compared with VTM, but hard data are lacking. The purpose of this study was to test the hypothesis that the time to acquire images to videotape using VTM and to disk using SBM would be the same for normal hearts and corresponding congenital heart diseases, but interpretation times would be shorter using SBM. METHODS: We measured the times to acquire and interpret 403 echocardiograms using standard VTM from Children's Hospital in Cincinnati, Ohio, and 352 echocardiograms acquired using SBM from Children's Hospital in San Diego, Calif. Diagnostic categories at each site included: (1) normal, (2) simple shunt or isolated valve disease, and (3) multiple-lesion disease. RESULTS: As a group, SBM echocardiograms included more hemodynamic measurements and took more time to acquire (P <.037), but less time to read (P <.001) than corresponding images acquired using VTM. Using SBM, it took more time to acquire normals and isolated valve or shunt lesions, whereas the average time to acquire multiple-lesion disease was the same using both VTM and SBM. With SBM, in contrast, interpretation times were significantly less for all corresponding diagnoses. CONCLUSION: SBM studies took longer to acquire because more hemodynamic measurements were acquired, but they were read in less time than corresponding VTM studies even though all videotapes were replayed in search fast-forward mode. Pediatric echocardiographers can increase their laboratory efficiency by converting from VTM to SBM.

Analog-Digital Conversion↗