Truncus arteriosus with aortic arch interruption: cardiovascular magnetic resonance findings in the unrepaired adult.
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ABSTRACT:
SUBMITTER: Verhaert D
PROVIDER: S-EPMC2846925 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Background:Truncus arteriosus (TA) is a complex congenital heart disease that carries morbidities in the first year of life. Previous authors have reported an operative mortality of 50%. In this report, we aim to report on the survival of patients with TA in our medical center in the recent era. Methods:A retrospective review of all patients diagnosed with TA in Siriraj Hospital, Thailand from August 1995 to March 2018 was performed. Patients with single ventricle, hemiTA were excluded. The characteristics and outcomes of repaired and unrepaired TA patients with a known recent functional status in 2018 were reviewed. Operative mortality risks were analyzed using a multivariate model. Results:A total of 74 patients (median age at referral: 70 days) were included in the cohort. One-third of the patients had associated anomalies including DiGeorge syndrome (13.5%). Anatomical repair was not performed in 22 patients (29.7%). The median age at time of repair for the 52 patients was 133 days (range: 22 days to 16.7 years). Complex TA was 10%. Early mortality occurred in 16 patients (30.8%). Five patients (9.6%) had late deaths at 0.3-1.2 years. Significant mortality risk was weight at time of operation <4 kg (HR 3.05, 95% CI [1.05-8.74], p-value 0.041). Of the 31 operation survivors, 17 required re-intervention within 0.4-11.4 years. Eight patients had reoperation at 8.7 years (range: 2.7-14.6 years) post-repair. Freedom from reoperation was 93%, 70.4%, and 31%, at 5, 10, and 15 years, respectively. All late survivors were in functional class I-II. Of the 22 unrepaired TA patients, 11 patients (50%) died (median age: 13.6 years; range: 14 days-32.8 years). Survival of unrepaired TA patients was 68.2%, 68.2%, and 56.8, at 5, 10, and 15 years of age, respectively. At the end of study, 11 survivors of TA with palliative treatment had a recent mean oxygen saturation value of 84.1 ± 4.8% and a mean weight for height of 81.4 ± 12.7%, which were significantly lower than those of 31 late-survivors who had undergone anatomical repair. Conclusion:Contemporary survival rates of patients with TA following operation in the center has been gradually improved over time. Most of the operative mortality occurs in the early postoperative period. Compared to patients with TA who had palliative treatment, operative survivors have a better functional status even though they carry a risk for re-intervention.
| S-EPMC7227657 | biostudies-literature
Project description:PurposeCardiovascular diseases remain the number one death cause worldwide. Preclinical 4D flow phase contrast magnetic resonance imaging can provide substantial insights in the analysis of aortic pathophysiologies in various animal models. These insights may allow a better understanding of pathophysiologies, therapy monitoring, and can possibly be translated to humans. This study provides a framework to acquire the velocity field within the aortic arch. It analyses important flow values at different locations within the aortic arch. Imaging parameters with high temporal and spatial resolution are provided, that still allow combining this time-consuming method with other necessary imaging-protocols.MethodsA new setup was established where a prospectively gated 4D phase contrast sequence is combined with a highly sensitive cryogenic coil on a preclinical magnetic resonance scanner. The sequence was redesigned to maintain a close to steady state condition of the longitudinal magnetization and hence to overcome steady state artifacts. Imaging parameters were optimized to provide high spatial and temporal resolution. Pathline visualizations were generated from the acquired velocity data in order to display complex flow patterns.ResultsOur setup allows data acquisition with at least two times the rate than that of previous publications based on Cartesian encoding, at an improved image quality. The "steady state" sequence reduces observed artifacts and provides uniform image intensity over the heart cycle. This made possible quantification of blood speed and wall shear stress (WSS) within the aorta and its branches. The highest velocities were observed in the ascending aorta with 137.5 ± 8 cm/s. Peak velocity values in the Brachiocephalic trunk were 57 ± 12 cm/s. Quantification showed that the peak flow occurs around 20 ms post R-wave in the ascending aorta. The highest mean axial wall shear stress was observed in the analysis plane between the left common carotid artery (LCCA) and the left subclavian artery. A stable image quality allows visualizing complex flow patterns by means of streamlines and for the first time, to the best of our knowledge, pathline visualizations from 4D flow MRI in mice.ConclusionThe described setup allows analyzing pathophysiologies in mouse models of cardiovascular diseases in the aorta and its branches with better image quality and higher spatial and temporal resolution than previous Cartesian publications. Pathlines provide an advanced analysis of complex flow patterns in the murine aorta. An imaging protocol is provided that offers the possibility to acquire the aortic arch at sufficiently high resolution in less than one hour. This allows the combination of the flow assessment with other multifunctional imaging protocols.
| S-EPMC5678712 | biostudies-literature
Project description:Here, we report a fetus with a rare aortic arch anomaly with left aortic arch and right ductus arteriosus, which has not been reported so far. In this condition, the aorta extends to the left of the trachea as in normal, while the ductus arteriosus extends to the right of the trachea and joins the descending aorta posterior to the trachea, with a cross-ribbon sign.
| S-EPMC5385781 | biostudies-literature
Project description:We present a 31-year-old female with repaired tetralogy of Fallot (TOF) and right-sided aortic arch (RAA) with left-sided patent ductus arteriosus (PDA) originating from the left brachiocephalic artery. This is a rare finding but most common site for a PDA in TOF and a RAA. To the best of our knowledge, this is the first demonstration of this rare finding on MRI in the literature.
| S-EPMC4248572 | biostudies-literature
Project description:BackgroundInterrupted aortic arch (IAA) is a rare but severe congenital abnormality often associated with bicuspid aortic valve (BAV). Complex re-interventions are often needed despite surgical advances, but the impact of aortic hemodynamics in repaired patients is unknown.ObjectiveInvestigate effect of IAA repairs on aortic hemodynamics, wall shear stress and flow derangements via 4-D flow MRI.Materials and methodsWe retrospectively analyzed age- and gender-matched cohorts (IAA [n=6], BAV alone [n=6], controls [n=6]) undergoing cardiac MRI including 4-D flow. Aortic dimensions were measured from standard MR angiography. We quantified peak systolic velocities, regurgitant fractions and wall shear stress in the ascending aorta (AAo), transverse arch and descending aorta (DAo) from 4-D flow, and we graded helix/vortex flow patterns from 3-D blood flow visualization.ResultsChildren and young adults with IAA had a wide range of arch dimensions, peak systolic velocities, regurgitant fractions and flow grades. Peak transverse arch systolic velocities were higher in patients with IAA versus controls (P=0.02). Flow derangements in the AAo were found in patients with IAA (median grade=2, 5/6 patients, P=0.04) and BAV (median grade=3, 5/6 patients, P=0.03) versus controls. Flow derangements in the DAo were only seen in patients with IAA (median grade=1, 5/6 patients, P=0.04), and 5/6 people with IAA had helical flow in head and neck vessels. Wall shear stress was increased in people with IAA along the superior transverse arch and proximal DAo versus controls (P=0.02).ConclusionComplex congenital aortic arch repairs can change aortic hemodynamics. Associated cardiac defects can further alter findings. Studies are warranted to investigate clinical implications in larger cohorts.
| S-EPMC6943192 | biostudies-literature
Project description:Cardiovascular magnetic resonance (CMR) has an emerging role in aortic valve disease evaluation, becoming an all-in-one technique. CMR evaluation of the anatomy and flow through the aortic valve has a higher reproducibility than echocardiography. Its unique ability of in vivo myocardial tissue characterization, significantly improves the risk stratification and management of patients. In addition, CMR is equivalent to cardiac CT angiography for trans-aortic valvular implantation and surgical aortic valve replacement planning; on the other hand, its role in the evaluation of ventricular function improving and post-treatment complications is undisputed. This review encompasses the existing literature regarding the role of CMR in aortic valve disease, exploring all the aspects of the disease, from diagnosis to prognosis.
| S-EPMC6732913 | biostudies-literature
Project description:BackgroundImage-domain motion correction of black-blood contrast T2-weighted fetal cardiovascular magnetic resonance imaging (CMR) using slice-to-volume registration (SVR) provides high-resolution three-dimensional (3D) images of the fetal heart providing excellent 3D visualisation of vascular anomalies [1]. However, 3D segmentation of these datasets, important for both clinical reporting and the application of advanced analysis techniques is currently a time-consuming process requiring manual input with potential for inter-user variability.MethodsIn this work, we present novel 3D fetal CMR population-averaged atlases of normal and abnormal fetal cardiovascular anatomy. The atlases are created using motion-corrected 3D reconstructed volumes of 86 third trimester fetuses (gestational age range 29-34 weeks) including: 28 healthy controls, 20 cases with postnatally confirmed neonatal coarctation of the aorta (CoA) and 38 vascular rings (21 right aortic arch (RAA), 17 double aortic arch (DAA)). We used only high image quality datasets with isolated anomalies and without any other deviations in the cardiovascular anatomy.In addition, we implemented and evaluated atlas-guided registration and deep learning (UNETR) methods for automated 3D multi-label segmentation of fetal cardiac vessels. We used images from CoA, RAA and DAA cohorts including: 42 cases for training (14 from each cohort), 3 for validation and 6 for testing. In addition, the potential limitations of the network were investigated on unseen datasets including 3 early gestational age (22 weeks) and 3 low SNR cases.ResultsWe created four atlases representing the average anatomy of the normal fetal heart, postnatally confirmed neonatal CoA, RAA and DAA. Visual inspection was undertaken to verify expected anatomy per subgroup. The results of the multi-label cardiac vessel UNETR segmentation showed 100[Formula: see text] per-vessel detection rate for both normal and abnormal aortic arch anatomy.ConclusionsThis work introduces the first set of 3D black-blood T2-weighted CMR atlases of normal and abnormal fetal cardiovascular anatomy including detailed segmentation of the major cardiovascular structures. Additionally, we demonstrated the general feasibility of using deep learning for multi-label vessel segmentation of 3D fetal CMR images.
| S-EPMC9753334 | biostudies-literature
Project description:Autosomal dominantly inherited PRKAG2 cardiac syndrome is due to a unique defect of the cardiac cell metabolism and has a distinctive histopathology with excess intracellular glycogen, and prognosis different from sarcomeric hypertrophic cardiomyopathy. We aimed to define the distinct characteristics of PRKAG2 using cardiovascular magnetic resonance (CMR).CMR (1.5 T) and genetic testing were performed in two families harboring PRKAG2 mutations. On CMR, segmental analysis of left ventricular (LV) hypertrophy (LVH), function, native T1 mapping, and late gadolinium enhancement (LGE) were performed.Six individuals (median age 23 years, range 16-48; two females) had a PRKAG2 mutation: five with an R302Q mutation (family 1), and one with a novel H344P mutation (family 2). Three of six mutation carriers had LV mass above age and gender limits (203 g/m2, 157 g/m2 and 68 g/m2) and others (with R302Q mutation) normal LV masses. All mutation carriers had LVH in at least one segment, with the median maximal wall thickness of 13 mm (range 11-37 mm). Two R302Q mutation carriers with markedly increased LV mass (203 g/m2 and 157 g/m2) showed a diffuse pattern of hypertrophy but predominantly in the interventricular septum, while other mutation carriers exhibited a non-symmetric mid-infero-lateral pattern of hypertrophy. In family 1, the mutation negative male had a mean T1 value of 963 ms, three males with the R302Q mutation, LVH and no LGE a mean value of 918?±?11 ms, and the oldest male with the R302Q mutation, extensive hypertrophy and LGE a mean value of 973 ms. Of six mutations carriers, two with advanced disease had LGE with 11 and 22 % enhancement of total LV volume.PRKAG2 cardiac syndrome may present with eccentric distribution of LVH, involving focal mid-infero-lateral pattern in the early disease stage, and more diffuse pattern but focusing on interventricular septum in advanced cases. In patients at earlier stages of disease, without LGE, T1 values may be reduced, while in the advanced disease stage T1 mapping may result in higher values caused by fibrosis. CMR is a valuable tool in detecting diffuse and focal myocardial abnormalities in PRKAG2 cardiomyopathy.
| S-EPMC4619453 | biostudies-literature
Project description:Danon disease is a rare X-linked dominant lysosomal glycogen storage disease that can lead to severe ventricular hypertrophy and heart failure. We report a case of Danon disease with cardiac involvement evaluated with cardiovascular magnetic resonance, including late gadolinium enhancement and perfusion studies.
| S-EPMC2683831 | biostudies-literature
Project description:We report a rare case of a quinticuspid aortic valve associated with regurgitation and dilation of the ascending aorta, which was diagnosed and post-surgically followed up by cardiovascular magnetic resonance and dual source computed tomography.
| S-EPMC2734562 | biostudies-literature