TMVR after TA-TAVR: a re-redo surgery-case report.

Introduction: Transcatheter mitral valve replacement (TMVR) is a valuable treatment option in patients with severe mitral regurgitation. Prior transapical transcatheter aortic valve replacement (TA-TAVR) may complicate the procedure and is therefore considered a relative contraindication. In this case report, the authors describe the successful TMVR as a tertiary cardiac surgery and transapical redo procedure. Case Summary: An 83-year-old male patient, suffering from dyspnoea and angina, was diagnosed with severe mitral valve regurgitation (MR). He had already undergone cardiac surgery in the form of coronary artery bypass grafting at the age of 64 and TA-TAVR at 79 years. After a failed attempt at mitral valve transcatheter edge-to-edge repair, he opted for TMVR. Pre-TMVR computed tomography simulation was used to analyse possible interactions between the prostheses and to predict the neo-left ventricular outflow tract (neo-LVOT). The operation was carried out without complications. There was no bleeding and the LV function remained unchanged. On MRI, the valves were perfectly aligned without any signs of paravalvular leakage or LVOT obstruction. The patient was discharged seven days postoperatively. At the one-year follow up, there was no need for rehospitalisation and the patient had clinically improved (from NYHA IV to II). Echocardiography demonstrated a mean transvalvular gradient of under 5 mmHg and no residual MR. Conclusion: A redo transapical access for TMVR as a tertiary cardiac operation can be easily performed. Pre-operative CT suggested good alignment of the aortic and mitral valved stent which was confirmed postoperatively.

Pulmonary valve reconstruction for acquired pulmonary regurgitation in patients with treated congenital heart disease.

OBJECTIVES: Pulmonary valve regurgitation is a common problem after relief of right ventricular outflow tract (RVOT) obstruction with a transannular patch. Pulmonary valve replacement with a homograft or xenograft is the routine treatment. Longevity of biological valves and the availability of homografts are limited. Alternatives to restore RVOT competence are evaluated. The goal of this study was to present intermediate-term results for pulmonary valve reconstruction (PVr) in patients with severe regurgitation. METHODS: PVr was performed in 24 patients (August 2006‒July 2018). We analysed perioperative data, pre- and postoperative cardiac magnetic resonance (CMR) imaging studies, freedom from valve replacement and risk factors for pulmonary valve dysfunction. RESULTS: The underlying diagnoses were tetralogy of Fallot (n = 18, 75%), pulmonary stenosis (n = 5, 20.8%) and the double outlet right ventricle post banding procedure (n = 1, 4.2%). The median age was 21.5 (14.8-23.7) years. Main (n = 9, 37.5%) and branch pulmonary artery procedures (n = 6, 25%) and surgery of the RVOT (n = 16, 30.2%) were often part of the reconstruction. The median follow-up after the operation was 8.0 (4.7-9.7) years. Freedom from valve failure was 96% at 2 and 90% at 5 years. The mean longevity of the reconstructive surgery was 9.9 years (95% confidence interval: 8.8-11.1 years). CMR before and 6 months after surgery showed a reduction in the regurgitation fraction [41% (33-55) vs 20% (18-27) P = 0.00] and of the indexed right ventricular end-diastolic volume [156 ml/m2 (149-175) vs 116 ml/m2 (100-143), P = 0.004]. Peak velocity across the pulmonary valve (determined by CMR) half a year after surgery was 2.0, unchanged. CONCLUSIONS: PVr can be achieved with acceptable intermediate-term results and may delay pulmonary valve replacement.

Reference Values for Pediatric Atrial Volumes Assessed by Steady-State Free-Precession Magnetic Resonance Imaging Using Monoplane and Biplane Area-Length Methods.

BACKGROUND: Measurement of atrial volumes by MRI is becoming increasingly important in pediatric cardiac disorders. However, MRI normal values for atrial volumes in children are lacking. PURPOSE: To establish pediatric reference values for atrial volumes. STUDY TYPE: Retrospective. SUBJECTS: A total of 155 healthy children from two large institutions (103 male, age 13.9 ± 2.8 years, range 4-18 years). FIELD STRENGTH/SEQUENCE: A 1.5 T; balanced steady-state free precession (bSSFP) sequence. ASSESSMENT: The monoplane and biplane area-length methods were used to measure minimal and maximal left and right atrial volumes (LAmin , LAmax , RAmin , and RAmax ) from four-chamber (4ch) and two-chamber (2ch) MR cine images. Centile charts and tables for atrial volumes were created. STATISTICAL TESTS: Descriptive statistics, lambda-mu-sigma (LMS)-method of Cole and Green, univariable and multivariable linear regression models. A P value < 0.05 was considered to be statistically significant. RESULTS: In the multivariable linear model, body surface area was significantly associated with all atrial volumes and sex was significantly associated with RA volumes, LA volumes measured in the 2ch-view as well as biplane LAmax. Average atrial volumes measured: monoplane 4ch: LAmin 13.1 ± 4.8 mL/m2 , LAmax 33.4 ± 8.8 mL/m2 , RAmin 18.5 ± 6.8 mL/m2 , RAmax 33.2 ± 9.6 mL/m2 ; monoplane 2ch: LAmin 12.7 ± 4.9 mL/m2 , LAmax 30.5 ± 9.5 mL/m2 ; biplane: LAmin 12.3 ± 4.5 mL/m2 , LAmax 30.9 ± 8.7 mL/m2 . DATA CONCLUSION: Pediatric MRI reference values for atrial volumes have been provided. TECHNICAL EFFICACY: 2 EVIDENCE LEVEL: 4.

Radiation safety for cardiovascular computed tomography imaging in paediatric cardiology: a joint expert consensus document of the EACVI, ESCR, AEPC, and ESPR.

Children with congenital and acquired heart disease may be exposed to relatively high lifetime cumulative doses of ionizing radiation from necessary medical invasive and non-invasive imaging procedures. Although these imaging procedures are all essential to the care of these complex paediatric population and have contributed to meaningfully improved outcomes in these patients, exposure to ionizing radiation is associated with potential risks, including an increased lifetime attributable risk of cancer. The goal of this manuscript is to provide a comprehensive review of radiation dose management and cardiac computed tomography performance in the paediatric population with congenital and acquired heart disease, to encourage informed imaging to achieve indication-appropriate study quality at the lowest achievable dose.

Surveillance of Fontan Associated Liver Disease in Childhood and Adolescence.

Fontan associated liver disease (FALD) has been recognized as a potentially serious sequela of the Fontan circulation. Prevalence of FALD among different age groups and risk factors for advanced changes were assessed. FALD screening included abdominal ultrasound and laboratory tests. A "liver disease score (LDS)" incorporating items from ultrasound and blood testing was calculated to grade FALD severity (5 items each, maximum score 10 points). 240 patients (male: n = 139, female: n = 101, systemic right ventricle: n = 160) underwent FALD screening 10 (IQR 7-15) years after Fontan surgery. Ultrasound was abnormal in 184 (76.6%) patients (surface nodularity / blunted liver edge: n = 133, 55.4%; heterogeneous parenchyma: n = 93, 38.8%; splenomegaly: n = 68, 28.3%; ascites: n = 23, 9.6%). At least one abnormal laboratory test was detected in 218 (90.8%) patients. Gamma-glutamyl-transpeptidase was elevated in the majority of patients (n = 206, 85.8%). Median LDS was 3 (2-4). Scores ≥5 were observed in 32 (13.3%) patients. Longer follow-up (15 (11-20) vs 9 (6-14) years, P <0.001), higher central venous (13 (11-15) vs 10 (9-12) mmHg, P <0.001) and end-diastolic pressure (8 (5-10) vs 6 (5-7) mmHg, P = 0.001), impaired ventricular function and absence of sinus rhythm were associated with LDS ≥5. Longer follow-up (OR 1.2 (1.1-1.3), P <0.001) and higher central venous pressure (OR 1.6 (1.3-2.1), p < 0.001) were the only independent predictors of advanced FALD. Abdominal ultrasound and laboratory abnormalities suggestive of FALD are common during routine follow-up already in childhood and adolescence irrespective of ventricular morphology. More advanced findings are associated with longer follow-up and higher central venous pressure.

Myocardial Perfusion in Hypoplastic Left Heart Syndrome.

BACKGROUND: The 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. METHODS: One 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). RESULTS: Hyperemic 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). CONCLUSIONS: The 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.

Serial right ventricular assessment in patients with hypoplastic left heart syndrome: a multiparametric cardiovascular magnetic resonance study.

OBJECTIVES: The aim of this study was to assess single right ventricular (RV) function in a large cohort of hypoplastic left heart syndrome (HLHS) patients after the completion of total cavopulmonary connection by analysing serial cardiovascular magnetic resonance (CMR) studies. METHODS: CMR studies from 95 HLHS patients were analysed. RV end-diastolic and end-systolic volumes (RVEDV, RVESV), ejection fraction (RVEF) and long-axis strain (LAS) were measured from cine images. RESULTS: All 95 patients had at least 2 CMR scans and 35 patients had 3 CMR scans. The median age (first quartile-third quartile) at the 3 examinations was 4.2 (3.3-6.1), 9.4 (6.1-11.4) and 14.6 (11.8-16.8) years. RV indexed volumes (RVEDVi and RVESVi) increased from first to the second and from the first and second examination to the third examination in patients with >10 years of age (P < 0.05). There was a slight decrease in RVEF and LAS throughout the examinations, but this was not statistically significant. Correlations were found between RVEF and LAS (r = -0.23; P < 0.01). Both RVEF and LAS correlated with RVEDVi and RVESVi (r = -0.17 to 0.43; P < 0.05). CONCLUSIONS: Serial assessment of CMR studies in HLHS patients after total cavopulmonary connection completion demonstrate an increase in indexed RV volumes in older HLHS patients but only mild reduction in RVEF and LAS. The correlation of indexed RV volumes with RVEF and LAS together with the significant increase in RV volumes over time suggests that indexed RV volumes might be superior to RV functional markers to monitor the RV in HLHS patients.

MRI-based comprehensive analysis of vascular anatomy and hemodynamics.

BACKGROUND: Standardized methods for mapping the complex blood flow in vessels are essential for processing the large data volume acquired from 4D Flow MRI. We present a method for systematic and efficient analysis of anatomy and flow in large human blood vessels. To attain the best outcomes in cardiac surgery, vascular modifications that lead to secondary flow patterns such as vortices should be avoided. In this work, attention was paid to the undesired cancelation of vortices with opposite directions of rotation, known as Dean flow patterns, using hemodynamic parameters such as circulation and helicity density. METHODS: Our approach is based on the multiplanar reconstruction (MPR) of a multi-dimensional feature-space along the blood vessel's centerline. Hemodynamic parameters and anatomic information were determined in-plane from the reconstructed feature-space and from the blood vessel's centerline. A modified calculation of circulation and helicity density and novel parameters for quantifying Dean flow were developed. To test the model performance, we applied our methods to three test cases. RESULTS: Comprehensive information on position, magnitude and interrelation of vascular anatomy and hemodynamics were extracted from 4D Flow MRI datasets. The results show that the Dean flow patterns can be efficiently assessed using the novel parameters. CONCLUSIONS: Our approach to comprehensively and simultaneously quantify multiple parameters of vascular anatomy and hemodynamics from 4D Flow MRI provides new insights to map complex hemodynamic conditions.

Role of cardiovascular magnetic resonance in an adolescent with a giant intrapericardial mass.

A 14 -year-old boy presented with chest pain and breathlessness. Echocardiography showed a large pericardial effusion with cardiac tamponade features and suspicion of cardiac mass. Cardiovascular magnetic resonance demonstrated a large, well-defined pericardial mass, suggesting atypical large coronary fistula with pericardial haematoma or primary cardiac/pericardial tumour such as angiosarcoma. Histology confirmed a mixed-type vascular malformation. Sirolimus therapy was initiated.

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.

Aortic elasticity after aortic coarctation relief: comparison of surgical and interventional therapy by cardiovascular magnetic resonance imaging.

BACKGROUND: Patients after aortic coarctation (CoA) repair show impaired aortic bioelasticity and altered left ventricular (LV) mechanics, predisposing diastolic dysfunction. Our purpose was to assess aortic bioelasticity and LV properties in CoA patients who underwent endovascular stenting or surgery using cardiovascular magnetic resonance (CMR) imaging. METHODS: Fifty CoA patients (20.5 ± 9.5 years) were examined by 3-Tesla CMR. Eighteen patients had previous stent implantation and 32 had surgical repair. We performed volumetric analysis of both ventricles (LV, RV) and left atrium (LA) to measure biventricular volumes, ejection fractions, left atrial (LA) volumes, and functional parameters (LAEFPassive, LAEFContractile, LAEFReservoir). Aortic distensibility and pulse wave velocity (PWV) were assessed. Native T1 mapping was applied to examine LV tissue properties. In twelve patients post-contrast T1 mapping was performed. RESULTS: LV, RV and LA parameters did not differ between the surgical and stent group. There was also no significant difference for aortic distensibility, PWV and T1 relaxation times. Aortic root distensibility correlated negatively with age, BMI, BSA and weight (p < 0.001). Native T1 values correlated negatively with age, weight, BSA and BMI (p < 0.001). Lower post-contrast T1 values were associated with lower aortic arch distensibility and higher aortic arch PWV (p < 0.001). CONCLUSIONS: CoA patients after surgery or stent implantation did not show significant difference of aortic elasticity. Thus, presumably other factors like intrinsic aortic abnormalities might have a greater impact on aortic elasticity than the approach of repair. Interestingly, our data suggest that native T1 values are influenced by demographic characteristics.

Complicated coarctation repair: The importance of three-dimensional cross-sectional imaging in late postoperative assessment.

Coarctation of the aorta (CoA) represents 5%-8% of congenital heart disease patients and is one of the most common causes of neonatal surgical intervention. These patients require close lifelong follow-up due to frequent long-term complications. Although transthoracic echocardiography is the first-line technique for its diagnosis and follow-up, cross-sectional imaging with cardiovascular magnetic resonance (CMR) gives excellent anatomical and functional information, especially in complex CoA. We present the case of a 17-year-old patient who underwent complicated neonatal CoA repair and demonstrate how CMR and thorough operative records helped to define the exact anatomy of repair many years after surgery. Furthermore, we conclude that keeping surgical drawings in the patient records can be of great importance, especially in complicated cases.

Myocardial Architecture, Mechanics, and Fibrosis in Congenital Heart Disease.

Congenital heart disease (CHD) is the most common category of birth defect, affecting 1% of the population and requiring cardiovascular surgery in the first months of life in many patients. Due to advances in congenital cardiovascular surgery and patient management, most children with CHD now survive into adulthood. However, residual and postoperative defects are common resulting in abnormal hemodynamics, which may interact further with scar formation related to surgical procedures. Cardiovascular magnetic resonance (CMR) has become an important diagnostic imaging modality in the long-term management of CHD patients. It is the gold standard technique to assess ventricular volumes and systolic function. Besides this, advanced CMR techniques allow the acquisition of more detailed information about myocardial architecture, ventricular mechanics, and fibrosis. The left ventricle (LV) and right ventricle have unique myocardial architecture that underpins their mechanics; however, this becomes disorganized under conditions of volume and pressure overload. CMR diffusion tensor imaging is able to interrogate non-invasively the principal alignments of microstructures in the left ventricular wall. Myocardial tissue tagging (displacement encoding using stimulated echoes) and feature tracking are CMR techniques that can be used to examine the deformation and strain of the myocardium in CHD, whereas 3D feature tracking can assess the twisting motion of the LV chamber. Late gadolinium enhancement imaging and more recently T1 mapping can help in detecting fibrotic myocardial changes and evolve our understanding of the pathophysiology of CHD patients. This review not only gives an overview about available or emerging CMR techniques for assessing myocardial mechanics and fibrosis but it also describes their clinical value and how they can be used to detect abnormalities in myocardial architecture and mechanics in CHD patients.

Fatal severe coronary artery stenosis in Williams syndrome: decision making using late gadolinium enhancement cardiovascular MRI.

Williams syndrome is a well-recognised congenital disorder characterised by cardiovascular, connective tissue, and central nervous system abnormalities. Coronary artery abnormalities are seen in patients with supravalvar aortic stenosis, but end-stage ischaemic heart disease is rare. We report a case of end-stage ischaemic heart disease due to severe coronary arterial stenosis, highlighting how cardiovascular MRI contributed to the management.

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.

OBJECTIVES: To compare the Lecompte technique and the spiral anastomosis (complete anatomic correction) two decades after arterial switch operation (ASO). METHODS: Nine 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. RESULTS: Compared 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). CONCLUSIONS: In 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.

Juxtaposition of the atrial appendages: A nidus for thrombus in atriopulmonary Fontan?

Juxtaposition of atrial appendages is a rare cardiac congenital anomaly, usually associated with other cardiac malformations. Until now, it has not been linked to any significant clinical implications. We report cardiovascular magnetic resonance (CMR) findings of two adult patients who underwent atriopulmonary Fontan operation in the setting of left juxtaposition of the atrial appendages. The patients were in sinus rhythm at the time of the CMR study. Both patients had episodes of sustained atrial tachyarrhythmia requiring electrical cardioversion and were anticoagulated with warfarin with target INR 2-3. CMR images showed a thrombus located in the enlarged and juxtaposed right appendage in both patients. Blood flow frequently appears slow or sluggish in the dilated right atrium following atriopulmonary Fontan surgery. In addition, cine CMR suggested that blood flow reaches very low velocities in the massively dilated juxtaposed right atrial appendage cul-de-sac, thus potentially creating a substrate for clot formation. These findings propose that juxtaposed atrial appendages in atriopulmonary Fontan is an additional risk factor for clot formation, specifically in the dilated right atrial appendage on the left side juxtaposed with the left atrial appendage and that prophylactic anticoagulation is highly justified in these patients.