Prenatal screening for congenital heart disease: a moving proposal.
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Biomedical subjects
Publications and source records attributed to Mark S Sklansky.
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OBJECTIVE: We sought to identify in utero predictors of postnatal outcomes in fetal patients with pulmonary atresia with intact ventricular septum (PAIVS) or critical pulmonary stenosis. BACKGROUND: Although PAIVS or critical pulmonary stenosis can be diagnosed in utero by echocardiography, our ability to predict outcomes is limited. METHODS: Fetal echocardiograms from 28 patients with PAIVS/critical pulmonary stenosis were retrospectively reviewed. Tricuspid valve (TV) annulus, right and left ventricular internal dimensions, and degree of tricuspid regurgitation were recorded. To establish normal fetal values, echocardiograms from healthy patients were analyzed in an identical fashion. RESULTS: Both a fetal TV z score of -4 or less beyond 23 weeks of gestation and a fetal TV annulus of 5 mm or less beyond 30 weeks of gestation were predictive of poor postnatal outcomes. In addition, right:left ventricular length or width less than 0.5 and/or the absence of tricuspid regurgitation were predictive of poor outcome. CONCLUSIONS: TV annulus size, right:left ventricular ratios, and presence of tricuspid regurgitation on fetal echocardiograms may aid in guiding prenatal counseling regarding postnatal outcome in PAIVS.
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Sano and colleagues recently described a modification of first stage palliation for hypoplastic left heart syndrome utilizing a right ventricle to pulmonary artery conduit. Preliminary results are favorable, but experience with this technique is limited. We report a case of sudden death due to obstruction of the proximal conduit by fibrointimal hyperplasia. This case of lethal conduit obstruction presented 3 months after initial palliation. Early cardiac catheterization and second stage palliation may be necessary to minimize the risk of such adverse events after the Sano modification.
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OBJECTIVE: Random fetal motion, rapid fetal heart rates, and cumbersome processing algorithms have limited reconstructive approaches to 3-dimensional fetal cardiac imaging. Given the recent development of real-time, instantaneous volume-rendered sonographic displays of volume data, we sought to apply this technology to fetal cardiac imaging. METHODS: We obtained 1 to 6 volume data sets on each of 30 fetal hearts referred for formal fetal echocardiography. Each volume data set was acquired over 2 to 8 seconds and stored on the system's hard drive. Rendered images were subsequently processed to optimize translucency, smoothing, and orientation and cropped to reveal "surgeon's eye views" of clinically relevant anatomic structures. Qualitative comparison was made with conventional fetal echocardiography for each subject. RESULTS: Volume-rendered displays identified all major abnormalities but failed to identify small ventricular septal defects in 2 patients. Important planes and views not visualized during the actual scans were generated with minimal processing of rendered image displays. Volume-rendered displays tended to have slightly inferior image quality compared with conventional 2-dimensional images. CONCLUSIONS: Real-time 3-dimensional echocardiography with instantaneous volume-rendered displays of the fetal heart represents a new approach to fetal cardiac imaging with tremendous clinical potential.
Balloon angioplasty and stent placement for pulmonary arterial stenoses in children are well-established therapies. In contrast, management of isolated peripheral pulmonary arterial stenoses in adults remains relatively unexplored. Four women (ages 18-63 years) with multiple discrete intralobar pulmonary arterial stenoses were treated with balloon angioplasty. Initially, 4-5 stenoses were dilated in each patient. The mean minimum diameter of the stenoses increased from 1.3 to 3.1 mm (P < 0.001), and the mean ratio of right ventricular to aortic systolic pressure decreased from 0.92 to 0.62 (P < 0.05). Each patient had marked symptomatic improvement. However, three patients developed recurrence of symptoms 4-24 months after angioplasty, and two had angiographic evidence of restenosis at previously dilated sites. These restenoses were treated with repeat angioplasty or stent implantation (three stents in each patient). One of these two patients developed near-occlusive restenosis of the stents and had successful bilateral lung transplantation. The other patient had a third catheterization with successful implantation of three additional stents. The third patient with recurrent symptoms died 2 years later, without further intervention. Transcutaneous catheter therapy for multiple intralobar pulmonary arterial stenoses in adults is highly successful acutely, but restenosis is common within several months. For some patients, balloon angioplasty and stent implantation may provide definitive management, while for others these procedures may serve as a bridge to lung transplantation.
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.