Imaging and surgical management of congenital heart diseases.

Congenital heart disease affects approximately 1% of live births per year. In recent years, there has been a decrease in the morbidity and mortality of these cases due to advances in medical and surgical care. Imaging plays a key role in the management of these children, with chest radiography, echocardiography and chest ultrasound the first diagnostic tools, and cardiac computed tomography, catheterization and magnetic resonance imaging reserved to assess better the anatomy and physiology of the most complex cases. This article is a beginner's guide to the anatomy of the most frequent congenital heart diseases (atrial and ventricular septal defects, abnormal pulmonary venous connections, univentricular heart, tetralogy of Fallot, transposition of the great arteries and coarctation of the aorta), their surgical management, the most common postsurgical complications, deciding which imaging modality is needed, and when and how to image gently.

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.

Primary cardiac fibroma in infants: A case report and review of cases of cardiac fibroma managed through orthotopic heart transplant.

Cardiac fibromas (CF) are the second most common cardiac tumors in children. They can be aggressive tumors despite their benign histopathologic nature, accounting for the highest mortality rate among primary cardiac tumors. CF usually show a progressive growth and spontaneous regression is rare. Therefore, a complete surgical excision is the preferred therapeutic approach when patients become symptomatic or if mass-related life-threatening complications are anticipated, even in asymptomatic patients. However, some cases are not good candidates for surgical excision due to the impossibility of preserving a normal cardiac anatomy or function after the tumor resection. Orthotopic heart transplantation (OHT) can be an exceptional but adequate alternative for some giant unresectable CF in children. In this article, we report our experience with the case of a 7-month-old infant with a giant unresectable cardiac fibroma who was successfully managed through OHT.

Acute Cardiovascular Manifestations in 286 Children With Multisystem Inflammatory Syndrome Associated With COVID-19 Infection in Europe.

BACKGROUND: The aim of the study was to document cardiovascular clinical findings, cardiac imaging, and laboratory markers in children presenting with the novel multisystem inflammatory syndrome associated with coronavirus disease 2019 (COVID-19) infection. METHODS: This real-time internet-based survey has been endorsed by the Association for European Paediatric and Congenital Cardiologists Working Groups for Cardiac Imaging and Cardiovascular Intensive Care. Children 0 to 18 years of age admitted to a hospital between February 1 and June 6, 2020, with a diagnosis of an inflammatory syndrome and acute cardiovascular complications were included. RESULTS: A total of 286 children from 55 centers in 17 European countries were included. The median age was 8.4 years (interquartile range, 3.8-12.4 years) and 67% were boys. The most common cardiovascular complications were shock, cardiac arrhythmias, pericardial effusion, and coronary artery dilatation. Reduced left ventricular ejection fraction was present in over half of the patients, and a vast majority of children had raised cardiac troponin when checked. The biochemical markers of inflammation were raised in most patients on admission: elevated C-reactive protein, serum ferritin, procalcitonin, N-terminal pro B-type natriuretic peptide, interleukin-6 level, and D-dimers. There was a statistically significant correlation between degree of elevation in cardiac and biochemical parameters and the need for intensive care support (P<0.05). Polymerase chain reaction for severe acute respiratory syndrome coronavirus 2 was positive in 33.6%, whereas immunoglobulin M and immunoglobulin G antibodies were positive in 15.7% cases and immunoglobulin G in 43.6% cases, respectively, when checked. One child in the study cohort died. CONCLUSIONS: Cardiac involvement is common in children with multisystem inflammatory syndrome associated with the Covid-19 pandemic. The majority of children have significantly raised levels of N-terminal pro B-type natriuretic peptide, ferritin, D-dimers, and cardiac troponin in addition to high C-reactive protein and procalcitonin levels. In comparison with adults with COVID-19, mortality in children with multisystem inflammatory syndrome associated with COVID-19 is uncommon despite multisystem involvement, very elevated inflammatory markers, and the need for intensive care support.

Living the heart in three dimensions: applications of 3D printing in CHD.

Advances in biomedical engineering have led to three-dimensional (3D)-printed models being used for a broad range of different applications. Teaching medical personnel, communicating with patients and relatives, planning complex heart surgery, or designing new techniques for repair of CHD via cardiac catheterisation are now options available using patient-specific 3D-printed models. The management of CHD can be challenging owing to the wide spectrum of morphological conditions and the differences between patients. Direct visualisation and manipulation of the patients' individual anatomy has opened new horizons in personalised treatment, providing the possibility of performing the whole procedure in vitro beforehand, thus anticipating complications and possible outcomes. In this review, we discuss the workflow to implement 3D printing in clinical practice, the imaging modalities used for anatomical segmentation, the applications of this emerging technique in patients with structural heart disease, and its limitations and future directions.

Apical Muscular Ventricular Septal Defects: Surgical Strategy Using Three-Dimensional Printed Model.

To evaluate the utility of a three-dimensional printed model (3D-model) for surgical decision planning in a complex case of multiple ventricular septal defect (VSD) (so-called Swiss-cheese septum). A 3 month-old-girl with diagnosis of multiple apical VSDs in the trabecular septum, perimembranous VSD, and atrial septal defect that resulted in congestive heart failure was referred for surgical intervention. Due to inconclusive understanding of the total number, openings and locations of the apical VSDs, there were concerns regarding the best surgical approach. Using computed tomography images, a 3D-model was fabricated by fused deposition modeling of polyurethane filament. The 3D model helped identifying the total number of VSD, their origins and exits as well as the minimum number of septal bands to resect, the optimal patch size and morphology to completely close all VSDs without reducing significantly the RV cavity. The procedure was successfully replicated in the patient by right atrial approach, resulting in good RV cavity size, function and no residual VSDs evaluated by transesophageal echocardiography. Direct evaluation of the 3D printed replica anatomy and surgical simulation may help optimizing surgical approach, patch size and location and therefore it might reduce complications and residual defects.

Hypertension in Coarctation of the Aorta: Challenges in Diagnosis in Children.

Evidence indicates that patients with coarctation of the aorta (COA) suffer from increased cardiovascular morbidity and mortality in later life despite successful repair of COA in childhood. Systolic arterial hypertension is common, presenting in up to one-third of patients, and is regarded as the main driver of premature cardiovascular events in this group of patients. In this review, we discuss the prevalence and pathophysiology of hypertension in children following successful COA repair with no residual arch obstruction. The challenges in accurate blood pressure assessment at this early phase are considered and non-invasive measures of central blood pressure are discussed. Although the pathways for investigations in adults are well defined, we highlight the need to address the issues of cardiovascular surveillance in children and describe techniques which can provide complementary information for cardiovascular assessment in this group of patients such that timely treatment can occur.

Three-dimensional printed models for surgical planning of complex congenital heart defects: an international multicentre study.

OBJECTIVES: To evaluate the impact of 3D printed models (3D models) on surgical planning in complex congenital heart disease (CHD). METHODS: A prospective case-crossover study involving 10 international centres and 40 patients with complex CHD (median age 3 years, range 1 month-34 years) was conducted. Magnetic resonance imaging and computed tomography were used to acquire and segment the 3D cardiovascular anatomy. Models were fabricated by fused deposition modelling of polyurethane filament, and dimensions were compared with medical images. Decisions after the evaluation of routine clinical images were compared with those after inspection of the 3D model and intraoperative findings. Subjective satisfaction questionnaire was provided. RESULTS: 3D models accurately replicate anatomy with a mean bias of -0.27 ± 0.73 mm. Ninety-six percent of the surgeons agree or strongly agree that 3D models provided better understanding of CHD morphology and improved surgical planning. 3D models changed the surgical decision in 19 of the 40 cases. Consideration of a 3D model refined the planned biventricular repair, achieving an improved surgical correction in 8 cases. In 4 cases initially considered for conservative management or univentricular palliation, inspection of the 3D model enabled successful biventricular repair. CONCLUSIONS: 3D models are accurate replicas of the cardiovascular anatomy and improve the understanding of complex CHD. 3D models did not change the surgical decision in most of the cases (21 of 40 cases, 52.5% cases). However, in 19 of the 40 selected complex cases, 3D model helped redefining the surgical approach.

Follow-up of a simple method for aortic valve reconstruction with fixed pericardium in children.

In 2013, we published a simple method for aortic valve reconstruction in children using fixed pericardium in this journal. This was not expected to be a durable solution but a temporizing measure in the absence of other solutions, in order to buy time for growth that would allow subsequent prosthetic valve insertion. As such, this was implemented in 3 patients with excellent immediate results, as reported in our original publication. We are now writing to provide longer term information. In 2 cases, a newborn and a 12-year-old boy, the valve calcified heavily at 4 years' follow-up. By then, the valvar annulus had grown sufficiently to accept a prosthetic valve in both cases. In these 2 cases, the material used for valve reconstruction was autologous pericardium fixed in 0.6% gluteraldehyde. In the third patient, a 3-year-old girl, the valve failed by dehiscence of the suture line 6 months following surgery. In this case, fixed bovine pericardium had been used, which is more rigid than autologous pericardium, thus possibly explaining the dehiscence. However, the pericardial patch was not calcified. Thus, the described method lived up to our expectation of buying time for growth for later insertion of a prosthetic valve in 2 of the 3 cases.

Magnetic resonance imaging planning in children with complex congenital heart disease - A new approach.

OBJECTIVES: To compare a standard sequential 2D Planning Method (2D-PM) with a 3D offline Planning Method (3D-PM) based on 3D contrast-enhanced magnetic resonance angiography (CE-MRA) in children with congenital heart disease (CHD). DESIGN: In 14 children with complex CHD (mean: 2.6 years, range: 3 months to 7.6 years), axial and coronal cuts were obtained with single slice spin echo sequences to get the final double oblique longitudinal cut of the targeted anatomical structure (2D-PM, n = 31). On a separate workstation, similar maximal intensity projection (MIP) images were generated offline from a 3D CE-MRA. MIP images were localizers for repeated targeted imaging using the previous spin echo sequence (3D-PM). Finally, image coverage, spatial orientation and acquisition time were compared for 2D-PM and 3D-PM. MAIN OUTCOME MEASURES: 2D-PM and 3D-PM images were similar: both perfectly covered the selected anatomic regions and no spatial differences were found (p>0.05). The mean time for creation of the final imaging plane was 241 ± 31 s (2D-PM) compared to 71 ± 18 s (3D-PM) (p<0.05). CONCLUSIONS: 3D-PM shows similar results compared to 2D-PM, but allows faster and offline planning thereby reducing the scan time significantly. As newly developed high-resolution 3D datasets can also be used further improvement of this technology is expected.

Three-dimensional Printed Cardiac Models: Applications in the Field of Medical Education, Cardiovascular Surgery, and Structural Heart Interventions.

In recent years, three-dimensional (3D) printed models have been incorporated into cardiology because of their potential usefulness in enhancing understanding of congenital heart disease, surgical planning, and simulation of structural percutaneous interventions. This review provides an introduction to 3D printing technology and identifies the elements needed to construct a 3D model: the types of imaging modalities that can be used, their minimum quality requirements, and the kinds of 3D printers available. The review also assesses the usefulness of 3D printed models in medical education, specialist physician training, and patient communication. We also review the most recent applications of 3D models in surgical planning and simulation of percutaneous structural heart interventions. Finally, the current limitations of 3D printing and its future directions are discussed to explore potential new applications in this exciting medical field.

NT-proBNP as Marker of Ventricular Dilatation and Pulmonary Regurgitation After Surgical Correction of Tetralogy of Fallot: A MRI Validation Study.

The goal of this study is to evaluate whether NT-proBNP plasma levels may help as a screening biomarker for monitoring right ventricular dilatation, pulmonary regurgitation and the onset of heart failure in patients with repaired Tetralogy of Fallot. Our single-centre observational prospective study involved 43 patients (15.1 years, SD = 8) with corrected Tetralogy of Fallot. Data collection included: clinical parameters (electrocardiogram, chest X-ray, NYHA scale, time since last surgery), biochemistry (NT-proBNP levels) and MRI values (ventricular volumetry, pulmonary flow assessment). Mean time since last surgery was 13.5 years (SD = 7.8). There was a statistically significant correlation between the NT-proBNP levels (187.4 pg/ml, SD = 154.9) and right ventricular dilatation for both the right ventricular end-diastolic volume (124.9 ml/m2, SD = 31.2) (Pearson = 0.19, p < 0.01) and end-systolic volume (56.1 ml/m2, SD = 18.8) (Pearson = 0.21, p < 0.01) and also with the pulmonary regurgitation fraction (36.5%, SD = 16, Pearson = 0.12, p < 0.01). No significant correlation was found between NT-proBNP and right ventricular ejection fraction (54.6%, SD = 10.6, Pearson = -0.07), left ventricular ejection fraction (59.9%, SD = 7.1, Pearson = -0.18) or any clinical parameters. The receiver operating curve analysis evidenced that a NT-proBNP cut-off value above 133.2 pg/ml predicted the presence of dilated right ventricular end-diastolic and end-systolic volumes over centile 95 (sensitivity 82 and 83% and specificity 93 and 79%, respectively). In conclusion, in patients with surgically corrected Tetralogy of Fallot, NT-proBNP levels correlate with right ventricular dilatation and the degree of pulmonary regurgitation. Ambulatory determination of NT-proBNP might be an easy, readily available and cost-effective alternative for MRI follow-up evaluation of these patients.

New technologies applied to surgical processes: Virtual Reality and rapid prototyping.

AYRA is software of virtual reality for training, planning and optimizing surgical procedures. AYRA was developed under a research, development and innovation project financed by the Andalusian Ministry of Health, called VirSSPA. Nowadays AYRA has been successfully used in more than 1160 real cases and after proving its efficiency it has been introduced in the clinical practice at the Virgen del Rocío University Hospital . Furthermore, AYRA allows generating physical 3D biomodels using rapid prototyping technology. They are used for surgical planning support, intraoperative reference or defect reconstruction. In this paper, some of these tools and some real cases are presented.

Cardiovascular magnetic resonance catheterization derived pulmonary vascular resistance and medium-term outcomes in congenital heart disease.

BACKGROUND: Selection of patients with congenital heart disease for surgical septation in biventricular repair or surgical palliation in functionally single ventricles requires low pulmonary vascular resistance (PVR). Where there is uncertainty, PVR can be assessed using hybrid cardiovascular magnetic resonance (CMR) and fluoroscopic (X-Ray) guided cardiac catheterizations (XMR). CMR/XMR catheterization is a validated technique for accurate assessment of pulmonary vascular resistance. However, data concerning its application in clinical practice is lacking. METHODS: PVR assessments were performed in 167 studies in 149 congenital heart disease patients by CMR/XMR catheterization. Data was collated on patient demographics, procedural data, complications and outcomes. Institutional ethics approval was obtained. RESULTS: Median age was 3.6 years (6 days-67 years) and weight 13.8 kg (2.3-122 kg). One hundred and eight studies were in biventricular circulations and 59 in functionally single ventricles. Median radiation dose was 0.72 mSv. A baseline Qp:Qs ≤2.75 in biventricular circulations with left-to-right shunts predicted a PVR ≥6 WU x m(2) with 100% sensitivity and 48% specificity. Median follow up until death or last review was 4.2 years (4 days-11 years). Eighty-four patients had a surgical or catheter intervention based on CMR/XMR catheterization findings at a median of 94 days after the study. This included successful biventricular repair at resting PVR values ≤6 WU x m(2) and Fontan completion at ≤4 WU x m(2). CONCLUSION: PVR measured by CMR/XMR catheterization allows accurate stratification for intervention in patients with congenital heart disease in both, biventricular and univentricular circulations.

Three-dimensional patient-specific cardiac model for surgical planning in Nikaidoh procedure.

PURPOSE: To explore the use of three-dimensional patient-specific cardiovascular models using rapid prototyping techniques (fused deposition modelling) to improve surgical planning in patients with complex congenital heart disease. DESCRIPTION: Rapid prototyping techniques are used to print accurate three-dimensional replicas of patients' cardiovascular anatomy based on magnetic resonance images using computer-aided design systems. Models are printed using a translucent polylactic acid polymer. EVALUATION: As a proof of concept, a model of the heart of a 1.5-year-old boy with transposition of the great arteries, ventricular septal defect and pulmonary stenosis was constructed to help planning the surgical correction. The cardiac model allowed the surgeon to evaluate the location and dimensions of the ventricular septal defect as well as its relationship with the aorta and pulmonary artery. CONCLUSIONS: Cardiovascular models constructed by rapid prototyping techniques are extremely helpful for planning corrective surgery in patients with complex congenital malformations. Therefore they may potentially reduce operative time and morbi-mortality.

Cardiovascular magnetic resonance imaging in congenital heart disease as an alternative to diagnostic invasive cardiac catheterization: a single center experience.

OBJECTIVE: The study aims to assess whether the increasing use of cardiovascular magnetic resonance imaging in place of diagnostic cardiac catheterization in the management of pediatric patients with congenital heart disease has had an impact on pediatric cardiac care. DESIGN: Retrospective analysis of data was used. SETTING: The study was performed at the Evelina Children's Hospital Cardiology Department. PATIENTS. : Elective diagnostic cardiac catheterization or magnetic resonance imaging (MRI) from 2005-2010 are included (n = 896). OUTCOME MEASURES: Indication, length of stay, and incidence of complications were recorded. In cases used to plan surgery, 30-day survival following the procedure was recorded. Surgical outcomes were compared between the two groups. Surgical outcomes planned using MRI were compared with national outcomes from Congenital Cardiac Audit Database. RESULTS: For catheterizations (50 patients, [31 male, median age 3 years, interquartile range 1 to 12]), median hospital stay was 1 day (interquartile range 0 to 3), and complications occurred in 11 (22%). Median hospital stay for MRI (846 patients [517 male, median age 3 years, interquartile range 0 to 9]) was significantly shorter: 0 days (interquartile range 0 to 1, P <.001), with fewer complications (16 [1.9%], P <.0001). Twenty-four catheter and 283 MRI patients underwent surgery within 18 months. One catheter patient (2.0%) and four MRI patients (1.4%) died within 30 days (P =.48). CONCLUSION: Replacing catheterization with cardiovascular magnetic resonance imaging has resulted in reduced rates of complication and shorter hospital stays without a significant impact on surgical outcome.

Hemiazygos vein "steal hypoxic-syndrome" after hemi-Fontan operation: comprehensive four-dimensional flow magnetic resonance visualisation.

A 2-year-old boy with hypoplastic left heart syndrome and previous hemi-Fontan palliation surgery was referred for cardiovascular magnetic resonance evaluation because of progressive cyanosis. This case report illustrates the advantages of non-invasive four-dimensional magnetic resonance imaging for comprehensive identification and quantification of venovenous collaterals in patients with palliated hemi-Fontan staged surgery.