Characteristics of Cardiovascular Magnetic Resonance Imaging and Outcomes in Adults with Repaired Truncus Arteriosus

Truncus arteriosus (TA) is a rare form of complex cyanotic congenital heart disease (CHD) (accounting for ∼2-4% of CHD lesions) where the pulmonary arteries (PAs), aorta and coronary arteries arise from a single great vessel.1,2 Complete surgical repair typically occurs in early infancy and involves PA reconstruction, insertion of a valved conduit or homograft between the right ventricular outflow tra ct (RVOT) and PAs, ventricular septal defect closure and, in some instances, repair or replacement of the native truncal valve.
Source: The American Journal of Cardiology - Category: Cardiology Authors: Source Type: research

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Higher odds seen for specific lesions, including atrial, ventricular septal defects
Source: The Doctors Lounge - Psychiatry - Category: Psychiatry Tags: Cardiology, Family Medicine, Neurology, Pediatrics, Psychiatry, Journal, Source Type: news
This study is used to determine whether the VSD can be closed during surgery. A collateral flow study circuit is also set up for first-time operations to measure the residual collateral flow after all of the MAPCAs have been harvested. Patients who require midline unifocalization will invariably require very lengthy periods of support on cardiopulmonary bypass. We have adapted our perfusion circuitry to prepare for the demands on the bypass circuit to meet the requirements of this patient population. Our institution has developed a systematic approach for the conduct of perfusion to best serve our patients presenting with ...
Source: Journal of Extra-Corporeal Technology - Category: Cardiovascular & Thoracic Surgery Tags: J Extra Corpor Technol Source Type: research
Abstract CITED2 is a cardiac transcription factor that plays a critical role in cardiac development. Gene mutations in CITED2 lead to a series of cardiac malformations and congenital heart defects (CHD). Congenital heart disease generally refers to defects in the heart's structure or function and often seen in many forms such as ventricular septal defects (VSDs), atrial septal defects (ASDs), and tetralogy of Fallot (TOF). However, the mechanisms involved in these mutations are poorly understood. The aim of the present study was to evaluate the mutations of the CITED2 gene in pediatric patients with congenital hea...
Source: Applied Biochemistry and Biotechnology - Category: Biochemistry Authors: Tags: Appl Biochem Biotechnol Source Type: research
ille Iolascon Mario Capasso In the past years, genome wide association studies (GWAS) have provided evidence that inter-individual susceptibility to diverse pathological conditions can reveal a common genetic architecture. Through the analysis of congenital heart disease (CHD) and neuroblastoma (NB) GWAS data, we aimed to dissect the genetic susceptibility shared between these conditions, which are known to arise from neural crest cell (NCC) migration or development abnormalities, via identification and functional characterization of common regions of association. Two loci (2q35 and 3q25.32) harbor single nucleotide ...
Source: Genes - Category: Genetics & Stem Cells Authors: Tags: Article Source Type: research
Double-outlet right ventricle is a complex congenital heart disease that encompasses various common and rare subtypes. Surgical management of these patients needs to be individualized owing to extremely variable morphology and hemodynamics. Imaging plays a crucial role in determination and characterization of outflow tract morphology. The assessment of ventricular septal defect routability with identification of associated anomalies has therapeutic implications in these patients. Multislice computed tomography with advanced 3-dimensional postprocessing techniques and dose-reduction strategies is invaluable in defining the ...
Source: Journal of Thoracic Imaging - Category: Radiology Tags: Web Exclusive Content: Pictorial Essays Source Type: research
ConclusionsDue to the relatively high prevalence of abnormal stress MPI, tailored protocols with a stress-first MPI as well as the use of 2-day protocols and advanced imaging technologies including CZT SPECT, novel image reconstruction software, and PET MPI could substantially reduce radiation dose in complex CHD.
Source: Journal of Nuclear Cardiology - Category: Nuclear Medicine Source Type: research
Ventricular septal defect (VSD) is a fatal congenital heart disease showing severe consequence in affected infants, to which early diagnosis plays an important role, particularly through genetic variants. Existing panel-based approaches of variants mining suffer from shortage of large panels, costly of sequencing and missing of rare variants. Although a trio-based method alleviates these limitations to some extent, it is agnostic to novel mutations and computational intensive. Considering these limitations, we are studying a novel variants mining algorithm from trio-based sequencing data and apply it on a VSD trio to ident...
Source: Frontiers in Genetics - Category: Genetics & Stem Cells Source Type: research
ConclusionBased on our preliminary experience, 4D Flow has become a complementary imaging modality accessible in current practice and open to all patients, infant, child or adult, without restriction of age, weight, size or pathology. The quality of this examination was satisfactory and seems to require an estimated learning curve of 3 months according to our experience.
Source: Archives of Cardiovascular Diseases Supplements - Category: Cardiology Source Type: research
Neurologic impairment is frequently observed in children with congenital heart disease. Impairment in cerebrovascular carbon dioxide reactivity (CO2R) is related with poor neurologic outcomes. The present study examined CO2R measured with transcranial Doppler (TCD) and near-infrared spectroscopy (NIRS) in children with ventricular septal defect undergoing cardiac surgery.
Source: Journal of Cardiothoracic and Vascular Anesthesia - Category: Anesthesiology Authors: Tags: Original Article Source Type: research
CONCLUSIONS: We replicated the copy number variations at the disease-candidate genes of PRKAB2 and PPM1K with tetralogy of Fallot in a Chinese Han population, and in patients with ventricular septal defect mutations in these two genes were not found. These results indicate the same molecular population genetics exist in these two genes with different ethnicity. This shows that these two genes are possibly specific pf tetralogy of Fallot candidates.RESUMO OBJETIVO: Este estudo teve como objetivo avaliar a associa ção genética do número de cópias em dois genes (PRKAB2 e PPM1K) localizados e...
Source: Revista da Associacao Medica Brasileira - Category: General Medicine Source Type: research
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