19 research outputs found
The impact of anatomical subgroups for regional and global function of the right ventricle in hypoplastic left heart syndrome
Assessment of intraatrial lateral tunnel anatomy and venous blood flow in children with hypoplastic left heart syndrome in Fontan circulation
Is the Lecompte technique the last word on transposition of the great arteries repair for all patients? A magnetic resonance imaging study including a spiral technique two decades postoperatively.
ObjectivesTo compare the Lecompte technique and the spiral anastomosis (complete anatomic correction) two decades after arterial switch operation (ASO).MethodsNine patients after primary ASO with Lecompte and 6 selected patients after spiral anastomosis were evaluated 20.8 ± 2.1 years after ASO versus matched controls. Blood flow dynamics and flow profiles (e.g. vorticity, helicity) in the great arteries were quantified from time-resolved 3D magnetic resonance imaging (MRI) phase contrast flow measurements (4D flow MR) in addition to a comprehensive anatomical and functional cardiovascular MRI analysis.ResultsCompared with spiral reconstruction, patients with Lecompte showed more vortex formation, supranatural helical blood flow (relative helicity in aorta: 0.036 vs 0.089; P < 0.01), a reduced indexed cross-sectional area of the left pulmonary artery (155 vs 85 mm²/m²; P < 0.001) and more semilunar valve dysfunctions (n = 5 vs 1). There was no difference in elastic aortic wall properties, ventricular function, myocardial perfusion and myocardial fibrosis between the two groups. Cross-sectional area of the aortic sinus was larger in patients than in controls (669 vs 411 mm²/m²; P < 0.01). In the spiral group, the pulmonary root was rotated after ASO more towards the normal left position (P < 0.01).ConclusionsIn this study, selected patients with spiral anastomoses showed, two decades after ASO, better physiologically adapted blood flow dynamics, and attained a closer to normal anatomical position of their great arteries, as well as less valve dysfunction. Considering the limitations related to the small number of patients and the novel MRI imaging techniques, these data may provoke reconsidering the optimal surgical approaches to transposition of the great arteries repair
Abnormal torsion and helical flow patterns of the neo-aorta in hypoplastic left heart syndrome assessed with 4D-flow MRI.
BackgroundThe Norwood procedure is the first stage of correction for patients with hypoplastic left heart syndrome (HLHS) and may lead to an abnormal neoaortic anatomy. We prospectively studied the neoaorta's fluid dynamics and the abnormal twist of the neoaorta by MRI examinations of HLHS patients in Fontan circulation. This study for the first time investigates the hypothesis that the neoaorta twist is associated with increased helical flow patterns, which may lead to an increased workload for the systemic right ventricle (RV) and ultimately to RV hypertrophy.MethodsA group of forty-two HLHS patients with a median age of 4.9 (2.9-17.0) years, at NYHA I was studied along with a control group of eleven subjects with healthy hearts and a median age of 12.1 (4.0-41.6). All subjects underwent MRI of the thoracic aorta including ECG-gated 2D balanced SSFP cine for an axial slice stack and 4D-flow MRI for a sagittal volume slab covering the thoracic aorta. The twist of the neoaortic arch was quantified by the effective geometric torsion, defined as the product of curvature and geometric torsion. Fluid dynamics and geometry in the neoaorta, including the flow helicity index, were evaluated using an in-house analysis software (MeVisLab-based). Myocardial mass of the systemic ventricle at end-diastole was estimated by planimetry of the short-axis stack.ResultsCompared to the control group, the neoaorta in the HLHS patients shows an increased twist (P=0.04) and higher peak helicity density (P=0.03). The maximum helicity density was correlated with maximum effective torsion of the ascending neoaorta (P<0.001). The degree of maximum twist correlated with the increase in RV myocardial mass (P<0.01).ConclusionsThis study shows that the abnormal twist of the neoaortic arch in HLHS patients is associated with abnormal helical flow patterns, which may contribute to increased RV afterload and may adversely affect the systemic RV by stimulation of myocardial hypertrophy. These findings suggest that further improvements of surgical aortic reconstruction, guided by insights from 4D-flow MRI, could lead to better neoaortic fluid dynamics in patients with HLHS
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4D flow streamline characteristics of the great arteries twenty years after Lecompte and direct spiral arterial switch operation (DSASO) in simple TGA.
Transposition of the great arteries (TGA) is caused by discordance between the great arteries and the ventricles. If left untreated, this anomaly has a disastrous perspective. More recent surgical approach for correction includes the Lecompte technique in which the pulmonary bifurcation is transposed anterior to the aorta, which may be less physiologic. Although the early results are excellent, there is potential for future problems involving the great arteries and semilunar valves1. These potential problems necessitate the development of other improved surgical techniques2. Here we report an MRI 4D flow study related to a case of simple TGA whose primary surgical correction -Â direct spiral arterial switch operation (DSASO) -Â was performed twenty years ago in an attempt to restore physiologic arrangement among the great arteries and semilunar valves
Right ventricular outflow tract reconstruction with the Labcor® stentless valved pulmonary conduit
OBJECTIVES
The right ventricular outflow tract reconstruction is a common necessity in congenital cardiac surgery. As homograft availability is limited, alternatives need to be evaluated. The Labcor® conduit consists of a porcine tricomposite valve assembled inside a bovine pericardium tube. This study presents intermediate-term results for its utilization for right ventricular outflow tract reconstruction.
METHODS
Labcor conduits were implanted in 53 patients (February 2009-July 2016). We analysed perioperative data, freedom from conduit failure and risk factors for conduit dysfunction.
RESULTS
The most common diagnosis was Tetralogy of Fallot (n = 20, 37.7%). The median age at surgery was 10.0 [interquartile range (IQR) 4.9-14.3] years. Pulmonary artery plasty (n = 37, 69.8%) and augmentation of the right ventricular outflow tract (n = 16, 30.2%) were often part of the procedure. The median conduit size was 21 (range 11-25) mm. There was no in-hospital death. The median follow-up after surgery was 4.6 (IQR 3.4-5.6) years. Fourteen patients (27.5%) developed conduit failure with stenosis being the main cause. Freedom from conduit failure was 98.0% at 2 and 80.5% at 5 years. The median longevity of the conduit was 7.4 years (95% confidence interval 5.1-9.8 years). Younger age and smaller conduit size were related to conduit failure.
CONCLUSIONS
Utilization of the Labcor conduit revealed acceptable intermediate-term results. The conduit appeared to be functioning sufficiently well within the first 5 years in the majority of patients. The higher rate of failure concerning smaller conduits might be associated with somatic outgrowth; however, conduit degeneration as common and long-term outcome still needs to be evaluated
Individual voxel-based subtype prediction can differentiate progressive supranuclear palsy from idiopathic parkinson syndrome and healthy controls
Exercise capacity of a contemporary cohort of children with hypoplastic left heart syndrome after staged palliation☆☆☆
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Myocardial Perfusion in Hypoplastic Left Heart Syndrome
BackgroundThe status of the systemic right ventricular coronary microcirculation in hypoplastic left heart syndrome (HLHS) is largely unknown. It is presumed that the systemic right ventricle's coronary microcirculation exhibits unique pathophysiological characteristics of HLHS in Fontan circulation. The present study sought to quantify myocardial blood flow by cardiac magnetic resonance imaging and evaluate the determinants of microvascular coronary dysfunction and myocardial ischemia in HLHS.MethodsOne hundred nineteen HLHS patients (median age, 4.80 years) and 34 healthy volunteers (median age, 5.50 years) underwent follow-up cardiac magnetic resonance imaging ≈1.8 years after total cavopulmonary connection. Right ventricle volumes and function, myocardial perfusion, diffuse fibrosis, and late gadolinium enhancement were assessed in 4 anatomic HLHS subtypes. Myocardial blood flow (MBF) was quantified at rest and during adenosine-induced hyperemia. Coronary conductance was estimated from MBF at rest and catheter-based measurements of mean aortic pressure (n=99).ResultsHyperemic MBF in the systemic ventricle was lower in HLHS compared with controls (1.89±0.57 versus 2.70±0.84 mL/g per min; P<0.001), while MBF at rest normalized by the rate-pressure product, was similar (1.25±0.36 versus 1.19±0.33; P=0.446). Independent risk factors for a reduced hyperemic MBF were an HLHS subtype with mitral stenosis and aortic atresia (P=0.017), late gadolinium enhancement (P=0.042), right ventricular diastolic dysfunction (P=0.005), and increasing age at total cavopulmonary connection (P=0.022). The coronary conductance correlated negatively with systemic blood oxygen saturation (r, -0.29; P=0.02). The frequency of late gadolinium enhancement increased with age at total cavopulmonary connection (P=0.014).ConclusionsThe coronary microcirculation of the systemic ventricle in young HLHS patients shows significant differences compared with controls. These hypothesis-generating findings on HLHS-specific risk factors for microvascular dysfunction suggest a potential benefit from early relief of frank cyanosis by total cavopulmonary connection