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Ventriculo-ventricular interactions are known to exist, though not well quantified. We hypothesised that the ventricular–vascular coupling ratio assessed by cardiovascular MRI would provide insight into this relationship. We also sought to compare MRI-derived ventricular–vascular coupling ratio to echocardiography and patient outcomes.
Methods:
Children with cardiac disease and biventricular physiology were included. Sanz’s and Bullet methods were used to calculate ventricular–vascular coupling ratio by MRI and echocardiography, respectively. Subgroup analysis was performed for right and left heart diseases. Univariate and multivariate regressions were performed to determine associations with outcomes.
Results:
A total of 55 patients (age 14.3 ± 2.5 years) were included. Biventricular ventricular–vascular coupling ratio by MRI correlated with each other (r = 0.41; p = 0.003), with respect to ventricle’s ejection fraction (r = −0.76 to −0.88; p < 0.001) and other ventricle’s ejection fraction (r = −0.42 to −0.47; p < 0.01). However, biventricular ejection fraction had only weak correlation with each other (r = 0.31; p = 0.02). Echo underestimated ventricular–vascular coupling ratio for the left ventricle (p < 0.001) with modest correlation to MRI-derived ventricular–vascular coupling ratio (r = 0.43; p = 0.002). There seems to be a weak correlation between uncoupled right ventricular–vascular coupling ratio with the need for intervention and performance on exercise testing (r = 0.33; p = 0.02).
Conclusion:
MRI-derived biventricular ventricular–vascular coupling ratio provides a better estimate of ventriculo-ventricular interaction in children and adolescents with CHD. These associations are stronger than traditional parameters and applicable to right and left heart conditions.
Three-dimensional visualisation is invaluable for evaluating cardiac anatomy. Patient-specific three-dimensional printed models of the heart are useful but require significant infrastructure. The three-dimensional virtual models, derived from 3D echocardiography, computed tomographic (CT) angiography or cardiac magnetic resonance (CMR), permit excellent visualisation of intracardiac anatomy, but viewing on a two-dimensional screen obscures the third dimension. Various forms of extended reality, such as virtual reality and augmented reality, augment the third dimension but only using expensive equipment. Herein, we report a simple technique of anaglyph stereoscopic visualisation of three-dimensional virtual cardiac models. The feasibility of achieving stereovision on a personal computer, using open-source software, and the need for inexpensive anaglyph glasses for viewing make it extremely cost-effective. Further, the retained depth perception of resulting stereo images in electronic and printed format makes sharing with other members of the team easy and effective.
Bridging bronchi are the rarest of the major airway anomalies reported in the literature. In this brief report, we present a case of a symptomatic adult male patient presenting with a type 2 bridging bronchus associated with left pulmonary artery sling.
Growth differentiation factor-15 is a novel biomarker of increasing importance in cardiovascular diseases. This study aimed to evaluate the relationship between ventricular measurements assessed by cardiac magnetic resonance imaging (MRI) and serum growth differentiation factor-15 levels in children with surgically corrected tetralogy of Fallot.
Materials and method:
Serum growth differentiation factor-15 levels were measured in 40 patients (mean age: 15.2 ± 2.9 years; 52.5% male; 87.5% NYHA I). End-diastolic volume index, end-systolic volume index, and ejection fractions of both ventricles and pulmonary regurgitation fraction were measured on cardiac MRI. The correlation between growth differentiation factor-15 levels and cardiac MRI parameters of the patients was investigated. Also, growth differentiation factor-15 levels of the patients were compared with healthy controls since reference values have not been determined in children.
Results:
The mean growth differentiation factor-15 level was 254.9 ± 6.3 pg/ml in the patient group. There was no correlation between growth differentiation factor-15 levels and cardiac MRI parameters in patients. Also, there was no significant difference in growth differentiation factor-15 levels between the patients and control groups.
Conclusion:
The serum levels of growth differentiation factor-15 were uncorrelated with ventricular size, function, and pulmonary regurgitation fraction assessed by cardiac MRI in children with operated tetralogy of Fallot. Moreover, growth differentiation factor-15 levels were not different in these patients from healthy children.
Conduit stenosis is a major, albeit rare, complication following the Fontan palliation. A single-baffle conduit with polytetrafluoroethylene is widely used for an extracardiac type Fontan palliation. A polyethylene terephthalate conduit (Dacron) is sometimes used for the conduit when more flexibility is required. A Y-shaped conduit is rarely used, but it may reduce the energy loss and achieve better hepatic flow distribution. Data on the long-term patency and complications when using a Y-shaped Dacron conduit is lacking. We report a case of a severely stenotic Y-shaped Dacron conduit in a patient who underwent extracardiac Fontan palliation.
Myocardial fibrosis is associated with adverse events in idiopathic dilated cardiomyopathy. Cardiac MRI with late gadolinium enhancement can detect myocardial fibrosis. We evaluated the conditional survival of children and adolescents based on native T1 mapping (combined proton signal from myocytes and interstitium prior to contrast administration by the measurement of myocardial and blood relaxation time) as a means to assess myocardial fibrosis. This retrospective case–cohort over a 3-year period included all consecutive patients (aged ≤ 21 years) with advanced heart failure from dilated cardiomyopathy (echocardiographic left ventricular ejection fraction ≤ 45% and NYHA class ≥ 2) who underwent cardiac MRI.
Conditional survival (follow-up ≥ 6 months after cardiac MRI) was assessed to include NYHA functional class and time to event (death or heart transplantation). A total of 57 patients (mean age 11.7 ± 6.1 years; 58% male) had a median NYHA Class III (31/57) and median left ventricular ejection fraction 25% (20–38%). Survival data were available in 82% patients (46/57) and the crude mortality rate was 24% (11/46) and one patient (2%) underwent heart transplantation. The median native T1 was elevated at 1351 ms (95% CI 1332, 1394) and it showed no difference between the groups who survived to those who died. Performing a multilevel regression analysis on prognosis failed to predict 6-month conditional survival.
An 11-year-old male was admitted with cough and fever for the last 4 days and also complained of pain in the right lung for some weeks. The boy did not show any other symptoms and his past medical history was unremarkable as well. The radiologist findings showed an aspect that suggested for echinococcosis.
At first, it was realised the heart intervention. About a 2-month period later, the child underwent another cyst removal in lung. He had begun taking albendazole 5 days before the heart intervention. The therapy was continued until the lung intervention and for 12 weeks post-operatively. The patient had an uneventful recovery and after about 4 years.
Improved survival has led to a growing population of adults with congenital heart disease (CHD), followed by numerous reports of late complications. Liver disease is a known complication in some patients, with most studies focusing on Fontan associated liver disease. Whether liver disease also exists in other patients with CHD is not fully investigated. Elevated central venous pressure is considered pivotal in the development of liver disease in Fontan associated liver disease, and other patients with alterations in central venous pressure may also be at risk for developing liver fibrosis. We wanted to see if liver fibrosis is present in patients with tetralogy of Fallot. Many patients with tetralogy of Fallot have severe pulmonary regurgitation, which can lead to elevated central venous pressure. Patients with tetralogy of Fallot may be at risk of developing liver fibrosis.
Materials and methods:
Ten patients (24–56 years) with tetralogy of Fallot and pulmonary regurgitation were investigated for liver fibrosis. All patients were examined with magnetic resonance elastography of liver, hepatobiliary iminodiacetic acid scan, indocyanine green elimination by pulse spectrophotometry, elastography via FibroScan, abdominal ultrasound including liver elastography, and blood samples including liver markers.
Results:
Three out of ten patients had findings indicating possible liver fibrosis. Two of these had a liver biopsy performed, which revealed fibrosis stage 1 and 2, respectively. The same three patients had an estimated elevated central venous pressure in previous echocardiograms.
Conclusions:
Mild liver fibrosis was present in selected patients with tetralogy of Fallot and may be related to elevated central venous pressure.
Superior caval vein stenosis is a known complication following paediatric heart transplantation. Herein, we sought to assess the incidence of superior caval vein stenosis and need for intervention in a single centre paediatric heart transplantation programme. A retrospective review was performed to identify variables associated with superior caval vein stenosis and need for intervention. Patients were identified based on angiographic and echocardiographic signs of superior caval vein stenosis. Of 204 paediatric heart transplantation recipients, 49 (24.0%) had evidence of superior caval vein stenosis with no need for catheter intervention and 12 (5.9%) had superior caval vein stenosis requiring catheter intervention. Overall, patients with superior caval vein stenosis with and without intervention had more cavopulmonary anastomosis (41.7%; 20.4%), pre-transplant superior caval vein procedures (41.7%; 28.6%), and bicaval approach (100.0%; 98.0%), compared to the group with no stenosis (11.9% and p = 0.015, 12.6% and p = 0.004, 73.4% and p < 0.001, respectively). Smaller recipients and donors were more likely to need intervention. Intervention was also seen more frequently in recipients who were younger at diagnosis (4.7 years) compared to non-intervention (13.3 years; p = 0.040). Re-intervention was required in 16.7% patients (n = 2) and was not associated with any complications.
Three-dimensional printing is increasingly utilised for congenital heart defect procedural planning. CT or MR datasets are typically used for printing, but similar datasets can be obtained from three-dimensional rotational angiography. We sought to assess the feasibility and accuracy of printing three-dimensional models of CHD from rotational angiography datasets.
Methods:
Retrospective review of CHD catheterisations using rotational angiography was performed, and patient and procedural details were collected. Imaging data from rotational angiography were segmented, cleaned, and printed with polylactic acid on a Dremel® 3D Idea Builder (Dremel, Mount Prospect, IL, USA). Printing time and materials’ costs were captured. CT scans of printed models were compared objectively to the original virtual models. Two independent, non-interventional paediatric cardiologists provided subjective ratings of the quality and accuracy of the printed models.
Results:
Rotational angiography data from 15 catheterisations on vascular structures were printed. Median print time was 3.83 hours, and material costs were $2.84. The CT scans of the printed models highly matched with the original digital models (root mean square for Hausdorff distance 0.013 ± 0.003 mesh units). Independent reviewers correctly described 80 and 87% of the models (p = 0.334) and reported high quality and accuracy (5 versus 5, p = NS; κ = 0.615).
Conclusion:
Imaging data from rotational angiography can be converted into accurate three-dimensional-printed models of CHD. The cost of printing the models was negligible, but the print time was prohibitive for real-time use. As the speed of three-dimensional printing technology increases, novel future applications may allow for printing patient-specific devices based on rotational angiography datasets.
Disadvantages of intravenous therapeutic unfractionated heparin, the first-line anti-coagulant agent in children with complex congenital heart disease, include unpredictable pharmacokinetics requiring frequent phlebotomies and the need for continuous intravenous access.
Objective:
To compare efficacy and safety of low-molecular-weight heparin administered by a subcutaneous indwelling catheter with intravenous unfractionated heparin.
Materials and methods:
Clinical data from 31 inpatients prospectively enrolled to receive subcutaneous low-molecular-weight heparin were compared with those from a historical group of 44 inpatients receiving intravenous unfractionated heparin. Investigation of parents’ satisfaction by telephone survey.
Results:
The percentage of anti-factor Xa levels outside therapeutic range was lower in the subcutaneous low-molecular-weight heparin group compared with the percentage of activated partial thromboplastin times outside therapeutic range in the intravenous unfractionated heparin group (40% versus 90%, p < 0.001). Neither group had a major complication. Transient local reactions occurred in 19% of patients of the subcutaneous low-molecular-weight heparin group. The number of needle punctures and that of placement of indwelling catheters were significantly lower in the subcutaneous low-molecular-weight heparin compared with the intravenous unfractionated heparin group (p < 0.001). In total, 84.2% of parents in the subcutaneous low-molecular-weight heparin group reported a positive experience when asked about comparison with prior intravenous unfractionated heparin treatment.
Conclusion:
Subcutaneous low-molecular-weight heparin offers a safe anti-coagulation regimen for children with complex congenital heart disease providing more efficient therapeutic anti-coagulation and a reduction in needle punctures, thus causing less pain and anxiety in this children.
Post-procedural right ventricular pseudoaneurysm is a rare but life-threatening complication of interventional catheterisation. We describe a 3-day-old newborn who underwent transcatheter intervention for pulmonary atresia with a complication of right ventricular pseudoaneurysms, and transcatheter embolisation of the pseudoaneurysms was performed at 3-week-old. It is the first described case that receives transcatheter closure of right ventricular pseudoaneurysms in a newborn with a favourable outcome.
Right pulmonary artery to left atrial fistula is classified based on the right pulmonary artery branching, individual right pulmonary venous drainage, and presence of an aneurysmal segment. A rare association with scimitar syndrome and right lung devoid of blood supply from right pulmonary artery is described in this report. The anatomical and management differences between the different types are highlighted.
A fistula between the pulmonary artery and the left atrium is a rare entity and its diagnosis is uncommon in the neonatal period. There are more reported surgical treatments in the literature than with a transcatheter closure. We report the case of a prenatal diagnosis of a large fistula between the right pulmonary artery and the left atrium with successful transcatheter closure with an Amplatzer duct occluder II 6/4 mm.
Coronary artery aneurysms in children were observed as a rare complication associated with coronavirus disease 2019 (COVID-19). This case report describes the severe end of the spectrum of the new multisystem inflammatory syndrome in a 12-year-old child with coronary aneurysms, myocardial dysfunction, and shock, managed successfully with extracorporeal membrane oxygenation support and immunomodulation therapy. This report also highlights the additional benefits of cardiac CT in the diagnosis and follow-up of coronary aneurysms.
To evaluate whether Kawasaki disease predisposes to premature atherosclerosis and to assess status of coronary artery abnormalities at least 10 years after diagnosis.
Material and methods:
A prospective study was carried out on 21 patients who were diagnosed with Kawasaki disease at least 10 years back and are on regular follow-up. The study was conducted on 128 Slice Dual Source computed tomography scanner with electrocardiography-triggered radiation optimised protocols for assessment of coronary artery abnormalities and calcifications.
Results:
Study cohort had 21 subjects – 15 males and 6 females (age range: 11–23 years; mean: 15.76 + 3.72 years). Mean age at time of diagnosis was 3.21 + 2.48 years. Mean time interval from diagnosis of Kawasaki disease to computed tomography coronary angiography was 12.59 + 2.89 years. Four children had evidence of coronary artery abnormalities on transthoracic echocardiography at time of diagnosis. Of these, two had persistent abnormalities on computed tomography coronary angiography. One subject (4.76%) had coronary calcification that was localised to abnormal coronary artery segment. Four coronary artery abnormalities (one saccular; three fusiform aneurysms) were noted in two subjects.
Conclusion:
Prevalence of coronary artery calcification is low and, if present, is localised to abnormal segments. This calcification is likely dystrophic rather than atherosclerotic. It appears that coronary artery abnormalities can persist for several years after acute episode of Kawasaki disease. Periodic follow-up by computed tomography coronary angiography is now a feasible non-invasive imaging modality for long term surveillance of patients with Kawasaki disease who had coronary artery abnormalities at time of diagnosis.
Anomalous coronary arteries from the pulmonary artery are uncommon causes of heart failure in the adult population. This case demonstrates the unusual presentation in a patient with anomalous right coronary artery from the pulmonary artery and discusses the complex pathophysiology of this lesion and the role of guideline-directed medical therapy in the management of these patients.
Haematopoietic stem cell transplantation is an important and effective treatment strategy for many malignancies, marrow failure syndromes, and immunodeficiencies in children, adolescents, and young adults. Despite advances in supportive care, patients undergoing transplant are at increased risk to develop cardiovascular co-morbidities.
Methods:
This study was performed as a feasibility study of a rapid cardiac MRI protocol to substitute for echocardiography in the assessment of left ventricular size and function, pericardial effusion, and right ventricular hypertension.
Results:
A total of 13 patients were enrolled for the study (age 17.5 ± 7.7 years, 77% male, 77% white). Mean study time was 13.2 ± 5.6 minutes for MRI and 18.8 ± 5.7 minutes for echocardiogram (p = 0.064). Correlation between left ventricular ejection fraction by MRI and echocardiogram was good (ICC 0.76; 95% CI 0.47, 0.92). None of the patients had documented right ventricular hypertension. Patients were given a survey regarding their experiences, with the majority both perceiving that the echocardiogram took longer (7/13) and indicating they would prefer the MRI if given a choice (10/13).
Conclusion:
A rapid cardiac MRI protocol was shown feasible to substitute for echocardiogram in the assessment of key factors prior to or in follow-up after haematopoietic stem cell transplantation.
The present study aims to develop and test the effectiveness of a “HeartBEAT” Adolescent Transition Psychoeducation Program (ATPP) in facilitating the well-being of adolescents who have been diagnosed with congenital heart defects (CHD).
Methods:
The HeartBEAT ATPP was developed after reviewing existing transition interventions and determining adolescents’ needs. It was based upon two theories, namely the Shared Management Model and Antonovsky’s Salutogenic Model and included five aspects: (i) knowledge on illness and treatments, (ii) self-management and transition skills, (iii) purpose of life, (iv) social support, and (v) emotional regulation skills. Mixed-methods pilot testing was then conducted. Paired t-test was used to explore the effectiveness and qualitative interviews were also conducted.
Results/Conclusions:
Fourteen patients aged 16–19 enrolled in this study, but only eight patients successfully complete the programme. Results showed that a paired t-test was conducted to determine its effectiveness. Results showed that the programme had significantly increased self-management knowledge (t (7) = −6.328, p < 0.05) and affectionate support (t (7) = −1.029, p < 0.05). Qualitative findings also showed that all patients were able to share emotional regulation and identify their purpose of life after the programme. However, there was no significant increase in their self-management skills. In summary, the “HeartBEAT” ATPP has demonstrated efficacy in enhancing self-management knowledge, affectionate support, identifying the patients’ life plan, and enhancing the emotional regulation skills of the adolescents with CHD.
Mammalian target of rapamycin inhibitors was found recently to be an effective treatment for manifestations of Tuberous sclerosis complex, including cardiac rhabdomyomas. Most cases with Cardiac rhabdomyoma treated with mammalian target of rapamycin inhibitors to date were diagnosed with Tuberous sclerosis. We report a case of cardiac rhabdomyoma and severe right ventricular outflow obstruction in a baby with negative genetics for Tuberous sclerosis that responded rapidly to Sirolimus.
