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 | 2010 Mar
REPOSITORIES: biostudies-literature
Publications
Truncus arteriosus with aortic arch interruption: cardiovascular magnetic resonance findings in the unrepaired adult.
Verhaert David D Arruda Janine J Thavendiranathan Paaladinesh P Cook Stephen C SC Raman Subha V SV
Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance 20100322
Truncus arteriosus (TA) is a rare congenital condition defined as a single arterial vessel arising from the heart that gives origin to the systemic, pulmonary and coronary circulations. We discuss the unique case of a 28 year-old female patient with unrepaired TA and interruption of the aortic arch who underwent cardiovascular magnetic resonance (CMR). ...[more]
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Project description:BackgroundTruncus 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.MethodsA 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.ResultsA 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.ConclusionContemporary 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.
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Project description:The previously unreported case of a child with an exceedingly rare amalgamation of complex defects, including truncus arteriosus (TA), double aortic arch (DAA), tracheoesophageal fistula, and choanal atresia is presented. First, on day-of-life (DOL) 2, with a joint effort involving Pediatric Cardiac Surgery, General Surgery, and Otolaryngology, division of tracheoesophageal fistula and repair of esophageal atresia, along with choanal atresia repair, was carried out. Via a right thoracotomy, the tracheoesophageal fistula, located medial to the azygous vein, was skeletonized and ligated. The proximal esophagus was then mobilized up to the thoracic inlet as it coursed through the vascular ring. This enabled esophageal anastomosis with preservation of both aortas. Next, on DOL 11, the child underwent TA repair. Following a standard midline sternotomy and cooling to moderate hypothermia, the left aortic arch was divided and oversewn. The aorta was then transected anteriorly, and the main pulmonary artery (MPA) exiting the posterior aorta was harvested as a single button. The aortic defect from the pulmonary artery button was repaired with autologous pericardium. Next, through a right ventriculotomy, the previously seen conoventricular septal defect was identified and closed. Finally, a 10-mm pulmonary homograft was anastomosed to the pulmonary artery bifurcation to complete the repair. The patient was discharged on DOL 78 and was noted to be doing well at 1-year follow-up. This case validates the feasibility of fistula repair complicated by DAA through a right thoracotomy, the durability of staged, complete repair of TA and DAA, and the advantages of a holistic, team-based approach that optimizes timing of all repairs based upon a careful consideration of the exponential, rather than additive, effects of multi-organ disease on post-cardiac surgery outcomes in neonates.
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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.
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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