Background: Three dimensional echo is a relatively new technique which may offer a rapid alternative for the examination of the right heart. However its role in patients with non-standard ventricular size or anatomy is unclear. This study compared volumetric measurements of the right ventricle in 25 patients with adult congenital heart disease using both cardiovascular magnetic resonance (CMR) and three dimensional echocardiography.
Methods: Patients were grouped by diagnosis into those expected to have normal or near-normal RV size (patients with repaired coarctation of the aorta) and patients expected to have moderate or worse RV enlargement (patients with repaired tetralogy of Fallot or transposition of the great arteries). Right ventricular end diastolic volume, end systolic volume and ejection fraction were compared using both methods with CMR regarded as the reference standard
Results: Bland-Altman analysis of the 25 patients demonstrated that for both RV EDV and RV ESV, there was a significant and systematic under-estimation of volume by 3D echo compared to CMR. This bias led to a mean underestimation of RV EDV by -34% (95%CI: -91% to + 23%). The degree of underestimation was more marked for RV ESV with a bias of -42% (95%CI: -117% to + 32%). There was also a tendency to overestimate RV EF by 3D echo with a bias of approximately 13% (95% CI -52% to +27%).
Conclusions: Statistically significant and clinically meaningful differences in volumetric measurements were observed between the two techniques. Three dimensional echocardiography does not appear ready for routine clinical use in RV assessment in congenital heart disease patients with more than mild RV dilatation at the current time.
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http://dx.doi.org/10.1186/1532-429X-13-78 | DOI Listing |
J Echocardiogr
December 2024
Division of Cardiovascular Surgery, Nagano Children's Hospital, Nagano, Japan.
Background: Perimembranous ventricular septal defect (VSD) can be classified as having trabecular, inlet, or outlet extension. The surgical approach used in patch closure depends on the which valve of the tricuspid valve to suture around and the avoidance of the specialized conducting system. This retrospective study evaluated the usefulness of the "En face view" method for classifying perimembranous VSD.
View Article and Find Full Text PDFWe present a clinical observation of an 18-year-old female patient with congenital bronchiectasis combined with congenital cystic degeneration of the upper lobes of both lungs, Williams-Campbell syndrome, long-COVID, severe course. The patient was treated in infectious disease department (three times), with subsequent transfer to pulmonology department of Kursk Regional Multi-Purpose Clinical Hospital from 31.01.
View Article and Find Full Text PDFEur J Hum Genet
December 2024
National EDS Service, London North West University Healthcare NHS Trust, London, UK.
Vascular Ehlers-Danlos syndrome (vEDS) is a rare inherited connective tissue disorder predominantly caused by pathogenic COL3A1 variants. Characteristic arterial and intestinal fragility and generalised severe tissue friability can lead to clinical events from childhood. We highlight a paucity of literature regarding children diagnosed with vEDS, possibly explained by a restraint in predictive testing, and present data on 63 individuals (23 index cases) with a clinical and genetic diagnosis of vEDS in childhood (<18 years) to address this.
View Article and Find Full Text PDFAnn Thorac Surg
December 2024
Department of Cardiac Surgery, Vanderbilt University Medical Center, Nashville Tennessee.
Background: Predicted heart mass ratio (PHMr) has become the standard donor-recipient size matching method in heart transplantation. While utilization of small PHMr hearts is associated with increased one-year mortality, the underlying mechanisms and time horizon of mortality remain uncertain.
Methods: A single institution analysis of isolated heart transplant recipients (01/2019-7/2022) was performed (N=334).
Metabolites
December 2024
Department of Family and Community Medicine and Medical Education, College of Medicine, Taibah University, Madinah 42353, Saudi Arabia.
Background: Congenital heart diseases are among the most common birth defects, significantly impacting infant health. Recent evidence suggests that exposure to endocrine-disrupting chemicals may contribute to the incidence of congenital heart diseases. This study systematically reviews and analyzes the association between maternal endocrine-disrupting chemicals exposure and congenital heart diseases.
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