Patient-specific three-dimensional aortic arch modeling for automatic measurements: clinical validation in aortic coarctation.

AIM: A validated algorithm for automatic aortic arch measurements in aortic coarctation (CoA) patients could standardize procedures for clinical planning. METHODS: The model-based assessment of the aortic arch anatomy consisted of three steps: first, machine-learning-based algorithms were trained on 212 three-dimensional magnetic resonance (MR) data to automatically allocate the aortic arch position in patients and segment the aortic arch; second, for each CoA patient (N = 33), the min/max aortic arch diameters were measured using the proposed software, manually and automatically, from noncontrast-enhanced three-dimensional steady-state free precession MRI sequence at five selected sites and compared ('internal comparison' referring to the same environment); third, moreover, the same min/max aortic arch diameters were compared, obtaining them independently, manually from common MR management software (MR Viewforum) and automatically from the model (external comparison). The measured sites were: aortic sinus, sino-tubular junction, mid-ascending aorta, transverse arch and thoracoabdominal aorta at the level of the diaphragm. RESULTS: Manual and software-assisted measurements showed a good agreement: the difference between diameter measurements was not statistically significant (at α = 0.05), with only one exception, for both internal and external comparison. A high coefficient of correlation was attained for both maximum and minimum diameters in each site (for internal comparison, R > 0.73 for every site, with P < 2 × 10). Notably, in tricuspid aortic valve patients external comparison showed no statistically significant difference at any measurement sites. CONCLUSION: The automatically derived aortic arch model, starting from three-dimensional MR images, could be a support to take the measurements in CoA patients and to quickly provide a patient-specific model of aortic arch anomalies.

Interventional VSD-Closure with the Nit-Occlud® Lê VSD-Coil in 110 Patients: Early and Midterm Results of the EUREVECO-Registry.

In August 2010, the Nit-Occlud® Lê (EUREVECO) became available for transcatheter coil occlusion of ventricular septal defects (VSDs). Retrospective European Registry for VSD Closure using the Nit-Occlud® Lê-VSD-Coil; analysis of the feasibility, results, safety and follow-up of VSD-closure over a 3-year period in 18 European centers. In 102 of 111 patients (female 66), successful VSD closure was performed (mean age 8.2 years, mean weight 28.82 kg), 81 perimembranous VSDs (48 with aneurysm), 30 muscular VSDs, mean procedure time was 121.1 min, and mean fluoroscopy time was 26.3 min. Short- and midterm term follow-up was possible in 100/102 patients, there was 1 embolization and 1 explantation after 24 months. Immediate complete closure occurred in 49 of 101 patients (48.5%), trivial residual shunt was present in 51 (50.0%), closure rate was 95% after 6 months and 97% after 1 year. Out of the 102 patients, there were 2 severe complications (1.8%) (1 severe hemolysis, 1 embolization) and 8 moderate/transient (=7.2%) including 1 transient AV block. During a mean follow-up period of 31.3 months (range 24-48) and a total follow-up time of 224.75 patient years, no further problems occurred. VSD closure with the Nit-Occlud® Lê VSD coil is feasible and safe with a minimal risk of severe side effects. The long-term effects and safety require further clinical follow-up studies.

Incidence of Respiratory Disease During the First Two Years of Life in Children with Hemodynamically Significant Congenital Heart Disease in Italy: A Retrospective Study.

Children affected by hemodynamically significant congenital heart disease (HSCHD) experience severe respiratory complications that can increase the frequency of hospitalizations. The aim of the SINERGY study was to describe the incidence of respiratory diseases and to collect information on active and passive immunoprophylaxis in the first 2 years of life. In this retrospective, multicenter, and epidemiologic study, children with HSCHD were enrolled across 11 Italian sites. Children born between December 31, 2007, and December 31, 2012, were observed during their first 2 years of life. Data were collected through hospital database searches and parent interviews. Four hundred twenty children were enrolled: 51.7 % were female, 79.5 % were born full-term (≥37 weeks), and 77.6 % weighed >2500 g at birth. The most frequent heart defects were ventricular septal defect (23.1 %) and coarctation of the aorta (14.3 %). The incidence of respiratory diseases was 63.1 %. Frequent respiratory diseases not requiring hospitalization were upper respiratory tract infections (76.4 %), acute bronchitis (43.3 %), and influenza (22.1 %), while those requiring hospitalization were bronchitis and bronchiolitis (8.3 % each one). While active immunoprophylaxis was applied with wide compliance (diphtheria/pertussis/tetanus, 99.5 %; Haemophilus influenzae type b, 72.5 %; pneumococcus, 79.9 %; meningococcus, 77.4 %), only 54 % of children received respiratory syncytial virus (RSV) passive prophylaxis (palivizumab). Of the 35 hospitalizations due to bronchiolitis, 27 (77.1 %) did not receive prophylaxis against RSV, compared with 8 (22.9 %) who received prophylaxis (P < 0.0001). Children with HSCHD are at major risk of respiratory diseases. Passive immunoprophylaxis can help to prevent hospitalizations for bronchiolitis.

MRI as a tool for non-invasive vascular profiling: a pilot study in patients with aortic coarctation.

AIM: While the overall concept of aortic coarctation has changed from one of simple obstruction to one that includes significant vascular dysfunction, this has not yet been translated into the diagnostic and treatment process. To close this gap, we sought to demonstrate the usefulness of an additional non-invasive vascular profile. METHOD: During a pilot study in eight coarctation patients, aortic area compliance, aortic distensibility, time phase shift and blood flow (distribution) were calculated from cine-MRI and 2D-/4D-velocity-encoded MRI sequences. RESULTS: Compared to healthy individuals, a significantly lower aortic compliance and reduced flow to the descending aorta were found in patients with coarctation. DISCUSSION: These differences underline the potential usefulness of a combined vascular profile in coarctation patients. CONCLUSION: It was successfully shown that functional vascular profiling of the aorta is feasible to be acquired non-invasively in a clinical setting and can provide additional diagnostic information. These can be the key input parameters for computational fluid dynamics-modeling.

Endomyocardial biopsy safety and clinical yield in pediatric myocarditis: An Italian perspective.

OBJECTIVES: The objective of this investigation is to evaluate the safety, the impact of endomyocardial biopsy (EMB) results in myocarditis management and the incidence of different etiologies of myocarditis in a pediatric population. BACKGROUND: Although EMB is an established diagnostic tool to evaluate suspected myocarditis, there is lack of clear diagnostic and management guidelines for myocarditis in pediatric patients, particularly in infants. METHODS: We performed a retrospective database review and subsequent outcomes analysis from five Italian pediatric cardiology centers to identify patients aged 0-18 years who underwent EMB for suspected myocarditis or inflammatory cardiomyopathy (ICMP) between 2009 and 2011. RESULTS: EMB was performed in 41 children, of which 16 were male. The population ranged between 16 days of age to 17 years (mean age at EMB = 5.2 ± 4.9 years). The overall incidence of EMB-related complications was 15.5% (31.2% in infants, and 6.8% in children > 1 year of age; P = 0.079) while the incidence of EMB-driven treatment changes was 29.2%. Histological examination together with PCR on heart biopsy specimens allowed an etiological diagnosis in 26/41 patients (63%). Among the 15 patients (36.5%) with diagnosis of dilated cardiomyopathy (DCM) 11 had idiopathic DCM. Finally, we found an overall incidence of death/cardiac transplantation of 24%. CONCLUSIONS: In a pediatric population with suspected myocarditis/ICMP, EMB was useful in confirming the diagnosis only in 41% of cases but showed an overall diagnostic power of 63%. As complications of EBM are not negligible, particularly in infants, the risk/benefit ratio should be taken into account in each patient.

Coronary artery ectasia in Noonan syndrome: Report of an individual with SOS1 mutation and literature review.

Noonan syndrome (NS) is the second most frequent hereditary syndrome with cardiac involvement. Pulmonary valve stenosis and hypertrophic cardiomyopathy are the most prevalent cardiovascular abnormalities. We report on a 14-year-old girl with NS due to SOS1 mutation with pulmonary stenosis and idiopathic coronary ectasia. To the best of our knowledge, this is the first report describing coronary ectasia in a patient with NS secondary to a SOS1 mutation. We include a literature review of this rare association.

Left Ventricular Mass Indexing in Infants, Children, and Adolescents: A Simplified Approach for the Identification of Left Ventricular Hypertrophy in Clinical Practice.

OBJECTIVE: To determine a simplified method to identify presence of left ventricular hypertrophy (LVH) in pediatric populations because the relationship between heart growth and body growth in children has made indexing difficult for younger ages. STUDY DESIGN: Healthy children (n = 400; 52% boys, 0-18 years of age) from 2 different European hospitals were studied to derive a simplified formula. Left ventricular mass (LVM) was calculated according to the Devereux formula. The derived approach to index LVM was tested on a validation cohort of 130 healthy children from a different hospital center. RESULTS: There was a strong nonlinear correlation between height and LVM. LVM was best related to height to a power of 2.16 with a correction factor of 0.09. Analysis of residuals for LVM/[(height(2.16)) + 0.09] showed an homoscedastic distribution in both sexes throughout the entire height range. A partition value of 45 g/m(2.16) was defined as the upper normal limit for LVM index. As opposed to formula suggested by current guidelines (ie, LVM/height(2.7)) when applying the proposed approach in the validation cohort of 130 healthy participants, no false positives for LVH were found (0% vs 8%; P < .01). CONCLUSIONS: Our data support the possibility to have a single partition (ie, 45 g/m(2.16)) value across the whole pediatric age range to identify LVH, without the time-consuming need of computing specific percentiles for height and sex.

Optical coherence tomography for characterization of cardiac allograft vasculopathy in late survivors of pediatric heart transplantation.

BACKGROUND: Optical coherence tomography (OCT) has been shown to reliably detect cardiac allograft vasculopathy (CAV). In recent studies performed in adult heart transplant (HTx) recipients, OCT revealed the presence of vulnerable plaques and complicated coronary artery lesions, thus challenging the current concept that CAV disease is a diffuse concentric and fibrosing vasculopathy. The aim of our study was to characterize CAV by OCT in a young population of HTx recipients. METHODS: We prospectively enrolled 21 young HTx recipients (mean age 27 years, range 22 to 38 years) to undergo OCT of the left anterior descending coronary artery (LAD) in addition to annual CAV screening by coronary angiography and virtual histology intravascular ultrasound (VH-IVUS). Quantitative OCT analysis was performed at the site of maximal intimal thickness (MIT) for each LAD segment. RESULTS: Patients were 27 years old with a mean time from cardiac transplantation of 14.7 ± 6.8 years. All patients exhibited intimal hyperplasia with an abnormal (>1) intima-to-media ratio. The median (interquartile range) MIT values by OCT were 0.37 (0.22 to 0.54) mm, 0.46 (0.29 to 0.54) mm and 0.34 (0.25 to 0.49) mm in the distal, middle and proximal LAD segments, respectively. Qualitative OCT analysis rarely showed features of vulnerable plaque or complicated lesions. Consistently, at VH-IVUS, the prevalent component at the site of MIT per vessel assessed by OCT was fibrous tissue. CONCLUSIONS: Unlike recent evidence in adult HTx recipients, OCT findings of vulnerable plaque and complicated coronary lesions were found to be rare among late survivors of pediatric HTx.

Advanced Parameters of Cardiac Mechanics in Children with CKD: The 4C Study.

BACKGROUND AND OBJECTIVES: Newer parameters of cardiac mechanics provide additional insights on cardiac dysfunction in adult patients with CKD. The aim of this study was to identify prevalence of subclinical abnormalities in cardiac function through the analysis of novel indices of cardiac mechanics in a large population of children with CKD. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Between 2009 and 2011, the prospective observational Cardiovascular Comorbidity in Children with CKD Study enrolled patients with CKD ages 6-17 years old with eGFR=10-45 ml/min per 1.73 m(2) in 14 European countries. Cardiac morphology and function were assessed through echocardiography. The analysis presented encompasses global radial, longitudinal, and circumferential strains as well as time to peak analysis. Data were compared with 61 healthy children with comparable age and sex. RESULTS: Data on 272 patients with CKD with complete echocardiographic assessment are reported (age =12.8±3.5 years old; 65% boys). Patients with CKD showed mildly higher office BP values and higher prevalence of left ventricular hypertrophy, but no differences were observed among groups in left ventricular ejection fraction. Strain analysis showed significantly lower global radial strain (29.6%±13.3% versus 35.5%±8.9%) and circumferential strain components (-21.8%±4.8% versus -28.2%±5.0%; both P<0.05) in patients with CKD without significant differences observed in longitudinal strain (-15.9%±3.4% versus -16.2%±3.7%). Lower values of global radial strain were associated with lower circumferential endocardial-to-epicardial gradient (r=0.51; P<0.01). This association remained significant after adjusting for BP, eGFR, and presence of left ventricular hypertrophy. Eventually, patients with CKD also showed higher delay in time to peak cardiac contraction (58±28 versus 37±18 milliseconds; P<0.05). CONCLUSIONS: A significant proportion of children with CKD show impaired systolic mechanics. Impaired systolic function is characterized by lower radial strain, transmural circumferential gradient, and mild cardiac dyssynchrony. This study suggests that analysis of cardiac strain is feasible in a large multicenter study in children with CKD and provides additional information on cardiac pathophysiology of this high-risk population.

Noninvasive hemodynamic assessment, treatment outcome prediction and follow-up of aortic coarctation from MR imaging.

PURPOSE: Coarctation of the aorta (CoA) is a congenital heart disease characterized by an abnormal narrowing of the proximal descending aorta. Severity of this pathology is quantified by the blood pressure drop (△P) across the stenotic coarctation lesion. In order to evaluate the physiological significance of the preoperative coarctation and to assess the postoperative results, the hemodynamic analysis is routinely performed by measuring the △P across the coarctation site via invasive cardiac catheterization. The focus of this work is to present an alternative, noninvasive measurement of blood pressure drop △P through the introduction of a fast, image-based workflow for personalized computational modeling of the CoA hemodynamics. METHODS: The authors propose an end-to-end system comprised of shape and computational models, their personalization setup using MR imaging, and a fast, noninvasive method based on computational fluid dynamics (CFD) to estimate the pre- and postoperative hemodynamics for coarctation patients. A virtual treatment method is investigated to assess the predictive power of our approach. RESULTS: Automatic thoracic aorta segmentation was applied on a population of 212 3D MR volumes, with mean symmetric point-to-mesh error of 3.00 ± 1.58 mm and average computation time of 8 s. Through quantitative evaluation of 6 CoA patients, good agreement between computed blood pressure drop and catheter measurements is shown: average differences are 2.38 ± 0.82 mm Hg (pre-), 1.10 ± 0.63 mm Hg (postoperative), and 4.99 ± 3.00 mm Hg (virtual stenting), respectively. CONCLUSIONS: The complete workflow is realized in a fast, mostly-automated system that is integrable in the clinical setting. To the best of our knowledge, this is the first time that three different settings (preoperative--severity assessment, poststenting--follow-up, and virtual stenting--treatment outcome prediction) of CoA are investigated on multiple subjects. We believe that in future-given wider clinical validation-our noninvasive in-silico method could replace invasive pressure catheterization for CoA.

Imaging modalities in children with vascular ring and pulmonary artery sling.

PURPOSE: Our aim is to compare new non-invasive imaging modalities in the evaluation of vascular ring (VR) and pulmonary artery sling (PAS) and to understand the role of bronchoscopy in comparison with them in assessing tracheobronchial tree. METHODS: We have retrospectively analyzed the data from 41 patients with a VR or a PAS diagnosed at Bambino Gesù Children's Hospital of Rome, between 2008 and 2012. Age, gender, presenting symptoms, clinical history, comorbidities, imaging modalities used for diagnosis (cardiac magnetic resonance [CMR], computed tomography [CT], tracheobronchoscopy [TB]) and surgical treatment were recorded. RESULTS: The vascular anatomy was completely defined in all patients, whether evaluated by CMR or CT, with a diagnostic accuracy of 100% based on surgical observation. All CT exams were performed without sedation with a mean dose-length product (DLP32 ) of 29 ± 9 and an effective dose of 1.56 ± 0.6 mSv, range 0.5-2.5 mSv. CMR required general anesthesia in all patients but involved no exposure to ionizing radiation. CT performed better than CMR in assessing tracheal stenosis when compared to TB. It detected complete tracheal cartilage rings in 2/3 patients with PAS, besides tracheomalacia and/or bronchomalacia in 54% of patients. CONCLUSIONS: Both cross-sectional imaging modalities (CT and CMR) can reliably and accurately diagnose these congenital vascular anomalies. While CT involves exposure to ionizing radiation, it avoids the risks related to anesthesia needed for CMR, and provides a more accurate assessment of tracheobronchial anatomy. TB remains a fundamental tool in tracheomalacia diagnosis in VR symptomatic patients and PAS.

Identification of TBX5 mutations in a series of 94 patients with Tetralogy of Fallot.

Tetralogy of Fallot (TOF) (OMIM #187500) is the most frequent conotruncal congenital heart defect (CHD) with a range of intra- and extracardiac phenotypes. TBX5 is a transcription factor with well-defined roles in heart and forelimb development, and mutations in TBX5 are associated with Holt-Oram syndrome (HOS) (OMIM#142900). Here we report on the screening of 94 TOF patients for mutations in TBX5, NKX2.5 and GATA4 genes. We identified two heterozygous mutations in TBX5. One mutation was detected in a Moroccan patient with TOF, a large ostium secundum atrial septal defect and complete atrioventricular block, and features of HOS including bilateral triphalangeal thumbs and fifth finger clinodactyly. This patient carried a previously described de novo, stop codon mutation (p.R279X) located in exon 8 causing a premature truncated protein. In a second patient from Italy with TOF, ostium secundum atrial septal defect and progressive arrhythmic changes on ECG, we identified a maternally inherited novel mutation in exon 9, which caused a substitution of a serine with a leucine at amino acid position 372 (p.S372L, c.1115C>T). The mother's clinical evaluation demonstrated frequent ventricular extrasystoles and an atrial septal aneurysm. Physical examination and radiographs of the hands showed no apparent skeletal defects in either child or mother. Molecular evaluation of the p.S372L mutation demonstrated a gain-of-function phenotype. We also review the literature on the co-occurrence of TOF and HOS, highlighting its relevance. This is the first systematic screening for TBX5 mutations in TOF patients which detected mutations in two of 94 (2.1%) patients.

"Baby Heart Project": the Italian project for accreditation and quality management in pediatric cardiology and cardiac surgery.

Optimization of the relationship between the supply and the demand for medical services should ideally be taken into consideration for the planning within each national Health System. Although government national health organizations embrace this policy specifically, the contribution of expert committees (under the scientific societies' guarantee in any specific medical field) should be advocated for their capability to collect and analyze the data reported by the various national institutions. In addition, these committees have the competence to analyze the need for the resources necessary to the operation of these centers. The field of pediatric cardiology and cardiac surgery may represent a model of clinical governance of particular interest with regard to programming and to a definition of the quality standards that may be extended to highly specialized institutions and ideally to the entire Health System. The "Baby Heart Project," which represents a model of governance and clinical quality in the field of pediatric cardiology and cardiac surgery, was born from the spontaneous aggregation of a committee of experts, supported by duly appointed Italian Scientific Societies and guided by a national agency for accreditation. The ultimate aim is to standardize both procedures and results for future planning within the national Health System.

Holt-Oram syndrome with intermediate atrioventricular canal defect, and aortic coarctation: functional characterization of a de novo TBX5 mutation.

Holt-Oram syndrome (HOS) is a rare autosomal dominant disorder characterized by upper limb defects and congenital heart defects (CHD), which are often simple septal and conduction defects, less frequently complex CHDs. We report on a 9 year-old boy with clinical and radiologic features of HOS consisting of bilateral asymmetric hypoplastic thumbs, generalized brachydactyly, limited supination due to radioulnar synostosis, and sloping shoulders, and intermediate atrioventricular canal defect (AVCD) with aortic coarctation. A de novo, previously described mutation, (Arg279ter) was identified in the TBX5 gene. Molecular characterization of this mutation was carried out due to the atypical CHD. In order to investigate whether the mutated transcript of TBX5 was able to escape the post-transcriptional surveillance mechanism and to produce a truncated TBX5 protein, we analyzed the TBX5 transcript, and protein pattern in HOS, and WT cardiac tissues. Our results demonstrate that the mutant TBX5 transcript is cleared by the cellular mechanism of surveillance. This data provides some support for the hypothesis that a dominant negative mutation, which strongly impairs the WT allele, might be too hazardous to be maintained. The literature suggests that HOS is relatively common among syndromes associated with AVCD.

Coronary plaque composition assessed by intravascular ultrasound virtual histology: association with long-term clinical outcomes after heart transplantation in young adult recipients.

OBJECTIVES: To assess coronary plaque composition by virtual histology intravascular ultrasound (VH-IVUS) analysis in young adult recipients and to correlate these findings with time from heart transplant (HTx) and long-term outcomes. BACKGROUND: Rapid progression of coronary allograft vasculopathy after heart transplantation is a powerful predictor of mortality and clinical events at long-term. METHODS: Forty consecutive young adult recipients transplanted during childhood undergoing VH-IVUS during coronary surveillance have been prospectively included in this study. According to the time interval from HTx to VH-IVUS assessment, our cohort was divided into two groups (group A: ≤5 years, n = 13; group B: >5 years, n = 27). RESULTS: Group B showed an higher percentage of necrotic core and dense calcium (12 ± 2 vs. 5 ± 1%, P = 0.04; 8.2 vs. 2.1%, P = 0.03; respectively). An "inflammatory plaque" (necrotic core and dense calcium ≥30%) was detected in 34.8% of patients in group B and in none among group A patients (P = 0.03). Patients in group B had a number of adverse clinical events significantly higher than group A patients (53.8 vs. 14.3%; HR 4.45; 95% CI 1.62-12.16; P = 0.029) at long-term follow-up (4.2 years). The multivariate regression analysis showed that age (HR 1.5; 95% CI 1.1-2.0; P = 0.007), time from HTx (HR 1.8; 95% CI 1.6-4.8; P = 0.02), and inflammatory plaque (HR 2.4; 95% CI 1.1-5.3; P = 0.03) were independent predictors of adverse clinical events. CONCLUSIONS: This study supports the hypothesis that time-dependent differences in plaque composition, as assessed by VH-IVUS, occur after HTx in young adult recipients, probably determining an increased risk of long-term clinical events.

[Informed consent in pediatric cardiology and cardiac surgery]. / Il consenso informato in Cardiologia e Cardiochirurgia Pediatrica.

BACKGROUND: A multidisciplinary study group was established to review and approve the informed consent froms in Pediatric Cardiology and Cardiac Surgery. METHODS: The work was carried out in several stages, starting with an analysis of what was already in use in several Italian Centers. Subsequently, shared forms for pediatric cardiac surgery and interventional catheterization procedures were developed, pointing to clarity of information, prediction of therapeutic options, quantification for verbal categories of risk associated with cardiac surgery procedures, and provision of information also to young patients. RESULTS: Two versions of informed consent for pediatric cardiac surgery and pediatric interventional catheterization procedures were developed. CONCLUSION: The work oerformed by the multidisciplinary study group, under the supervision of the Italian Society of Pediatric Cardiology and the Section of Surgery for Congenital Heart Disease of the Italian Society for Cardiac Surgery, resulted in a clear and evolutionary summary of the relationship between available therapeutic options and the patient needs to understand and share the healthcare pathway in terms of risk and perspectives.

Percutaneous treatment of abdominal coarctation in children using a covered stent.

Coarctation of the abdominal aorta is extremely rare. It generally involves a long segment of the descending aorta and causes uncontrolled and unexplainable hypertension in children. The therapeutic choice is very challenging because acute and chronic complications are reported for both the surgical and the percutaneous approaches. The two reported cases of abdominal coarctation were treated primarily and successfully through the use of covered stents. Three covered stents were implanted in two children. No complication occurred with either procedure. At this writing, an 18-month follow-up assessment has found the patients in good health with no restenosis at the coarctation site. Covered stent implantation in children with abdominal coarctation is a feasible, safe, and effective procedure. It provides adequate relief of symptoms and reduces the risk of aneurysm formation. To avoid covering important side branches with polytetrafluoroethylene, this type of procedure must be preceded by precise study of the aorta and its branches.

Change in cardiac geometry and function in CKD children during strict BP control: a randomized study.

BACKGROUND AND OBJECTIVES: Left ventricular hypertrophy (LVH) and abnormal systolic function are present in a high proportion of children with CKD. This study evaluated changes in left ventricular (LV) geometry and systolic function in children with mild to moderate CKD as an ancillary project of the Effect of Strict Blood Pressure Control and ACE Inhibition on Progression of Chronic Renal Failure in Pediatric Patients trial. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Echocardiograms and ambulatory BP monitoring were performed at baseline and at 1- or 2-year follow-up in 84 patients with CKD and 24-hour mean BP above the 50th percentile and/or receiving fixed high-dose angiotensin converting enzyme inhibition and randomized to conventional or intensified BP control. RESULTS: LVH prevalence decreased from 38% to 25% (P<0.05). Changes in LV mass index (LVMI) were restricted to patients with LVH at baseline (-7.9 g/m(2.7); P<0.02). Changes in LVMI were independent of randomization, reduction in BP, hemoglobin, and estimated GFR. A significant increase in midwall fractional shortening was observed in the total cohort (P<0.05), and was greater in the intensified group compared with the conventional BP control group (12%±1.9% versus 8%±1.5%; P=0.05). In multivariate analysis, improvement in myocardial function was associated with reduction in BP (r=-0.4; P<0.05), independently of LVMI reduction. CONCLUSIONS: In children with CKD, angiotensin converting enzyme inhibition with improved BP control, LVH regression, and improved systolic function was observed within 12 months. Lowering BP to the low-normal range led to a slightly more marked improvement in myocardial function but not in LVMI.

Computational modelling of the right ventricle in repaired tetralogy of Fallot: can it provide insight into patient treatment?

AIMS: Pulmonary regurgitation (PR) causes progressive right ventricle (RV) dilatation and dysfunction in repaired tetralogy of Fallot (rToF). Declining RV function is often insidious and the timing of pulmonary valve replacement remains under debate. Quantifying the pathophysiology of adverse RV remodelling due to worsening PR may help in defining the best timing for pulmonary valve replacement. Our aim was to identify whether complex three-dimensional (3D) deformations of RV shape, as assessed with computer modelling, could constitute an anatomical biomarker that correlated with clinical parameters in rToF patients. METHODS AND RESULTS: We selected 38 rToF patients (aged 10-30 years) who had complete data sets and had not undergone PVR from a population of 314 consecutive patients recruited in a collaborative study of four hospitals. All patients underwent cardiovascular magnetic resonance (CMR) imaging: PR and RV end-diastolic volumes were measured. An unbiased shape analysis framework was used with principal component analysis and linear regression to correlate shape with indexed PR volume. Regurgitation severity was significantly associated with RV dilatation (P = 0.01) and associated with bulging of the outflow tract (P = 0.07) and a dilatation of the apex (P = 0.08). CONCLUSION: In this study, we related RV shape at end-diastole to clinical metrics of PR in rToF patients. By considering the entire 3D shape, we identified a link between PR and RV dilatation, outflow tract bulging, and apical dilatation. Our study constitutes a first attempt to correlate 3D RV shape with clinical metrics in rToF, opening new ways to better quantify 3D RV change in rToF.