The Prevalence of Scoliosis after Fontan Circulation Surgery Followed-Up to Adolescence.

Introduction: The advancement of surgical techniques and perioperative management for congenital heart disease (CHD) has increased life expectancy. The surgical creation of the Fontan circulation maintains pulmonary blood flow without relying on an effective pump from the abnormal heart, relying on peripheral vascular resistance to maintain effective flow through the lungs. Unfortunately, this delicate mechanism is compromised when scoliosis restricts ventilation, leading to Fontan failure and a poor prognosis for life. This report describes the prevalence of scoliosis with Fontan completion surgery and the role of screening and surgical correction. Methods: Ninety-six consecutive Japanese patients undergoing Fontan completion surgery for CHD between 2000 and 2017 were identified in our institutional records. The inclusion criterion was at least 7 years of follow-up after Fontan completion surgery, while the exclusion criteria were congenital, syndromic, and neuromuscular scoliosis. Radiographic and clinical parameters, including cardio-thoracic ratio (CTR) for cardiomegaly and cyanosis saturation, were compared between with and without scoliosis. Results: There were 23 and 40 patients in the scoliosis and no scoliosis groups, respectively. The mean age at the final follow-up was 18.5 and 16.7 years in the scoliosis and no scoliosis groups, respectively (p=0.02). Mean CTR was 43.7% and 39.4% in the scoliosis and no scoliosis groups (p=0.016), and the mean saturation in room air at the final follow-up was 88.8% and 93.2%, respectively (p=0.036). There were no significant differences to clarify the risk factors with multivariate logistic regression analysis. Conclusions: The prevalence of scoliosis with Fontan completion surgery was 36.5%. Screening for scoliosis is important for children with Fontan circulation surgery as part of their routine follow-up at least until they reach adolescence.Evidence Level: 4.

Segmentation-based cardiomegaly detection based on semi-supervised estimation of cardiothoracic ratio.

The successful integration of neural networks in a clinical setting is still uncommon despite major successes achieved by artificial intelligence in other domains. This is mainly due to the black box characteristic of most optimized models and the undetermined generalization ability of the trained architectures. The current work tackles both issues in the radiology domain by focusing on developing an effective and interpretable cardiomegaly detection architecture based on segmentation models. The architecture consists of two distinct neural networks performing the segmentation of both cardiac and thoracic areas of a radiograph. The respective segmentation outputs are subsequently used to estimate the cardiothoracic ratio, and the corresponding radiograph is classified as a case of cardiomegaly based on a given threshold. Due to the scarcity of pixel-level labeled chest radiographs, both segmentation models are optimized in a semi-supervised manner. This results in a significant reduction in the costs of manual annotation. The resulting segmentation outputs significantly improve the interpretability of the architecture's final classification results. The generalization ability of the architecture is assessed in a cross-domain setting. The assessment shows the effectiveness of the semi-supervised optimization of the segmentation models and the robustness of the ensuing classification architecture.

Spontaneous rupture of the right aortic sinus resulting in a sterile aorto-atrial fistula in a pet rabbit (Oryctolagus cuniculus).

Aortocardiac fistula is a broad term used to describe defects between the aorta and other cardiac chambers that can occur in humans and animals. A 1.5-year-old, 1.7 kg, male castrated Holland lop rabbit (Oryctolagus cuniculus) was presented for a two-week history of a heart murmur with corresponding cardiomegaly on radiographs. Physical examination confirmed a grade-V/VI continuous heart murmur on the right sternal border with a regular rhythm and a gallop sound. Echocardiography revealed an aortic-to-right-atrial fistula causing severe left-sided volume overload. Based on the echocardiographic findings, rupture of the right aortic sinus was suspected. Due to the poor prognosis, euthanasia was elected. On necropsy, a fistula was found connecting the right aortic sinus with the right atrium, without evidence of an inflammatory response nor evidence of an infectious etiology. The sudden onset of a heart murmur supported acquired fistulation from a ruptured aortic sinus (also known as the sinus of Valsalva), though a congenital malformation could not be completely excluded.

Should echocardiogram be undertaken routinely when a child has severe iron deficiency anaemia?

Iron deficiency anaemia (IDA) is common in children. Treatment usually consists of oral iron therapy and, if severe, inpatient hospitalisation with blood transfusion. Providers may also undertake an echocardiogram, depending on availability and the severity of anaemia. A male toddler with nutritional IDA, haemoglobin of 1.7 g/dL (the lowest level in the literature) and hypertension had left ventricular hypertrophy (LVH) on the initial echocardiogram. He was managed acutely with judicious blood transfusion, followed by oral iron supplementation and anti-hypertensive medication at discharge. Repeat echocardiogram a month later demonstrated slight improvement of the LVH but the hypertension persisted at follow-up 6 months later. There was complete resolution of the findings a year later. In chronic nutritional IDA, there can be structural cardiac changes which can affect the acute management and requires close follow-up. It is important to use echocardiography in such severe cases.Abbreviations: CHF: congestive heart failure; CM: cardiomyopathy; DCM: dilated cardiomyopathy; ICU: intensive care unit; IDA: iron deficiency anaemia; IVSd: interventricular septum in diastole; LA: left atrium; LV: left ventricle; LVEDD: left ventricular end-diastolic diameter; LVH: left ventricular hypertrophy; LVM: left ventricular mass; LVPWd: left ventricular posterior wall end-diastole; PRBC: packed red blood cells.

Ellis-van Creveld syndrome: a case report.

Ellis-van Creveld syndrome (EVC), also known as chondroectodermal dysplasia, is a rare entity. It most commonly affects the tubular bones leading to dwarfism and a long trunk with ossification defects. Other presentations are wide hands and feet, dysplastic nails, thin hair, and cardiac malformations. An eight-year-old female patient presented to our tertiary care centre with complaints of short stature, abnormal dentition, and fatigue. The child's parents were first-degree relatives. On radiological imaging, it was revealed that the patient had postaxial polydactyly, short stature, and genu valgum deformity along with mild cardiomegaly. All these features were indicative of Ellis-van Creveld syndrome. EVC is a rare clinical syndrome with a distinctive clinical presentation. It requires comprehensive radiological investigations and the management is best done with a multidisciplinary approach.

Massive Pulmonary Arteriovenous Malformation as a Cause of Fetal Heart Failure.

Pulmonary arteriovenous malformations (AVMs) are abnormal connections between the pulmonary arteries and veins that can result in rapid-onset heart failure. We present a case of a fetus with pulmonary AVMs diagnosed at 22 weeks gestation. Fetal echocardiography showed cardiomegaly and dilated pulmonary arteries and veins reflecting the hemodynamic significance of the shunt. Inverted flow through the ductus arteriosus was also present. Fetal autopsy following medical termination of the pregnancy confirmed the morphological findings, including displacement of arteries and veins in proximity to the pleural surface. The genetic study was negative. This report highlights the cardiovascular impact of a rare disorder. Inverted flow through the ductus arteriosus may be another poor prognostic indicator, useful in parental counseling.

Case Report: One heart with two lobes: a rare infantile congenital giant left atrial appendage aneurysm.

Left atrial appendage aneurysm (LAAA) is an extremely rare congenital heart abnormality, with varying degrees of symptoms, ranging from asymptomatic to arrhythmia, thromboembolic event or airway obstruction. Most infantile cases were incidentally found by echocardiography. Contrast-enhanced chest tomography can confirm the diagnosis and inform surgical plan. We describe an asymptomatic young female infant who had a unique extreme cardiomegaly on a chest x-ray and received surgical aneurysmectomy. Her heart was restored to a normal cardiac size after the heart surgery.

Self-Reported Utilization of International Guidelines for Staging Dogs with Myxomatous Mitral Valve Degeneration: A Survey among Veterinary Practitioners.

BACKGROUND: ACVIM developed and published guidelines for staging myxomatous mitral valve degeneration in dogs in 2009. An updated version was published in 2019. The present study aimed to investigate whether these guidelines are actually used by the intended public more than a decade after their first publication. METHODS: An online survey was distributed among Dutch and Belgian veterinarians through online channels and mailing lists. RESULTS: Of the 524 responses, only 363 complete surveys were analyzed. The ACVIM guidelines are used by 60% of the respondents. Veterinarians find it more difficult to differentiate stage B1 from B2 in asymptomatic dogs compared to diagnosing stage C. Three-quarters of the respondents would recommend echocardiography for an incidentally detected new murmur with an intensity of 3 out of 6 in an adult dog. Two-thirds of the respondents find coughing a convincing finding for stage C disease. Close to half of the respondents associate a horizontal, dull percussion line with pulmonary edema. For confirming cardiogenic pulmonary edema, 98% of the respondents used thoracic radiographs. CONCLUSIONS: Veterinary practitioners might not have the expected training and equipment to be able to apply the guidelines in their practices, especially in the differentiation of stage B1 from stage B2.

Multiple vascular anomalies and refractory pericardial effusion in a young patient with Cantu syndrome: a case report and review of the literature.

BACKGROUND: Cantu syndrome is a rare and complex multisystem disorder characterized by hypertrichosis, facial dysmorphism, osteochondroplasia and cardiac abnormalities. With only 150 cases reported worldwide, Cantu syndrome is now gaining wider recognition due to molecular testing and a growing body of literature that further characterizes the syndrome and some of its most important features. Cardiovascular pathology previously described in the literature include cardiomegaly, pericardial effusion, vascular dilation and tortuosity, and other congenital heart defects. However, cardiovascular involvement is highly variable amongst individuals with Cantu syndrome. In some instances, it can be extensive and severe requiring surgical management and long term follow up. CASE PRESENTATION: Herein we report a case of a fourteen-year-old female who presented with worsening pericardial effusion of unknown etiology, and echocardiographic findings of concentric left ventricular hypertrophy, a mildly dilated aortic root and ascending aorta. Her medical history was notable for hemoptysis and an episode of pulmonary hemorrhage secondary to multiple aortopulmonary collaterals that were subsequently embolized in early childhood. She was initially managed with Ibuprofen and Colchicine but continued to worsen, and ultimately required a pericardial window for the management of refractory pericardial effusion. Imaging studies obtained on subsequent visits revealed multiple dilated and tortuous blood vessels in the head, neck, chest, and pelvis. A cardiomyopathy molecular studies panel was sent, and a pathogenic variant was identified in the ABCC9 gene, confirming the molecular diagnosis of autosomal dominant Cantu syndrome. CONCLUSIONS: Vascular anomalies and significant cardiac involvement are often present in Cantu syndrome, however there are currently no established screening recommendations or surveillance protocols in place. The triad of hypertrichosis, facial dysmorphism, and unexplained cardiovascular involvement in any patient should raise suspicion for Cantu syndrome and warrant further investigation. Initial cardiac evaluation and follow up should be indicated in any patient with a clinical and/or molecular diagnosis of Cantu syndrome. Furthermore, whole body imaging should be utilized to evaluate the extent of vascular involvement and dictate long term monitoring and care.

Correlation Between Anemia (Hemoglobin Level) and Cardiomegaly Based On Chest Radiograph and Echocardiography.

Background: Anemia is one of the major global problems that continues to this day. According to World Health Organization (WHO) data, anemia is found in 1.62 billion people or equivalent to 24.8% of the world's population while, the prevalence of anemia in children under 6 years of age in Indonesia reached 38.4%. Anemia, if undiagnosed or untreated for a long period of time can lead to multiorgan failure. The cardiovascular system is the most commonly affected system in chronic anemia. Between one-third and two-thirds of patients with severe anemia have cardiomegaly and this will return to normal within a few weeks after the anemia is well managed. This abnormality can be evaluated on chest radiograph or echocardiography. Objective: This study aims to evaluate the association between anemia and cardiomegaly. Methods: This was a cross-sectional which was carried out in the pediatric ward and pediatric cardiology outpatient clinic in the year 2022. The research subjects were children diagnosed with anemia, then they would undergo chest radiograph and echocardiography to assess whether there is cardiomegaly or not. Results: Eighty one children were included in this study. The majority of them were girl, with a total of 43 people (53.1%). The median age of patients in this study was 7.75 years old. In addition, this study also found that the median of patients' body weight and height were 18.3 kg and 116 cm. In this study, the median hemoglobin level was 10.3 g/dL. For the echocardiography parameters in this study, the median of LA/Ao, EF and FS were 1.24, 65% and 35% respectively.Conclusion There is no association beteween anemia and cardiomegaly based on chest radiograph or echocardiography.

Heart weight must not be measured before dissection during autopsies.

During autopsies, weighing the heart is a standard procedure. In addition to myocardial pathologies, heart size, and ventricular wall thickness, heart weight is a common parameter to describe cardiac pathology and should be recorded as accurately as possible. To date, there exists no standard for recording heart weight at autopsy, although some authors recommend weighing the heart after dissection and removal of blood and blood clots. In the study presented, the hearts of 58 decedents were weighed after being dissected out of the pericardial sac (a), after dissection using the short-axis or inflow-outflow method with manual removal of blood and blood clots (b), and after rinsing and drying (c). Depending on the dissection method, the heart weight was 7.8% lower for the inflow-outflow method and 11.6% lower for the short-axis method after dissection compared to before and correspondingly 2.9% to 5% lower again after rinsing and drying respectively. Accordingly, the heart should be dissected, blood and blood clots removed, rinsed with water, and dried with a surgical towel after dissection, before weighing.

An Efficient, Lightweight, Tiny 2D-CNN Ensemble Model to Detect Cardiomegaly in Heart CT Images.

Cardiomegaly is a significant global health concern, especially in developing nations. Although advanced clinical care is available for newly diagnosed patients, many in resource-limited regions face late diagnoses and consequent increased mortality. This challenge is accentuated by a scarcity of radiography equipment and radiologists. Hence, we propose the development of a computer-aided diagnostic (CAD) system, specifically a lightweight, tiny 2D-CNN ensemble model, to facilitate early detection and, potentially, reduce mortality rates. Deep learning, with its subset of convolutional neural networks (CNN), has shown potential in visual applications, especially in medical image diagnosis. However, traditional deep CNNs often face compatibility issues with object-oriented human factor technology. Our proposed model aims to bridge this gap. Using CT scan images sourced from the Mendeley data center, our tiny 2D-CNN ensemble learning model achieved an accuracy of 96.32%, offering a promising tool for efficient and accurate cardiomegaly detection.

Spectrum and Outcome of Prenatally Diagnosed Fetal Primary Cardiomyopathies-A Twenty-Year Overview.

OBJECTIVE: to assess the course and outcome of fetuses affected by primary cardiomyopathy (CM). METHODS: Retrospective study of 21 cases with prenatal diagnosis of a primary CM in one tertiary center over a period of 20 years. Charts were reviewed for echocardiographic findings, pregnancy outcome, and postnatal course. The utility of prenatal evaluation was discussed. RESULTS: The mean gestational age (GA) at diagnosis was 26.7 (±5.1) weeks. A total of 33.3% (7/21) had associated anomalies. Genetic etiology was confirmed in 50.0% (10/20, with one case lost to follow up). The overall survival rate of the entire study population was 40% (8/20) including termination of pregnancy in 20% (4/20) and an intrauterine mortality rate of 5% (1/20). Of the initial survivors (n = 15), a neonatal and early infant mortality rate of 46.7% (7/15) was calculated. Prenatal isolated right ventricular involvement was the only identified significant parameter for survival (p = 0.035). Four phenotypical groups were identified: 42.9% (9/21) hypertrophic (HCM), 38.1% (8/21) dilated (DCM), 14.3% (3/21) isolated noncompaction (NCCM), and 4.8% (1/21) restrictive CM (RCM). Fetuses assigned to isolated NCCM revealed a 100% survival rate. CONCLUSION: Prenatal detection is feasible but needs to a introduce classification method for better consulting and management practices. A poor outcome is still observed in many cases, but an increase in examiners' awareness may influence optimal multispecialized care.

Hybrid Classical-Quantum Transfer Learning for Cardiomegaly Detection in Chest X-rays.

Cardiovascular diseases are among the major health problems that are likely to benefit from promising developments in quantum machine learning for medical imaging. The chest X-ray (CXR), a widely used modality, can reveal cardiomegaly, even when performed primarily for a non-cardiological indication. Based on pre-trained DenseNet-121, we designed hybrid classical-quantum (CQ) transfer learning models to detect cardiomegaly in CXRs. Using Qiskit and PennyLane, we integrated a parameterized quantum circuit into a classic network implemented in PyTorch. We mined the CheXpert public repository to create a balanced dataset with 2436 posteroanterior CXRs from different patients distributed between cardiomegaly and the control. Using k-fold cross-validation, the CQ models were trained using a state vector simulator. The normalized global effective dimension allowed us to compare the trainability in the CQ models run on Qiskit. For prediction, ROC AUC scores up to 0.93 and accuracies up to 0.87 were achieved for several CQ models, rivaling the classical-classical (CC) model used as a reference. A trustworthy Grad-CAM++ heatmap with a hot zone covering the heart was visualized more often with the QC option than that with the CC option (94% vs. 61%, p < 0.001), which may boost the rate of acceptance by health professionals.

Parallels between oncogene-driven cardiac hyperplasia and heart regeneration in zebrafish.

The human heart is poorly regenerative and cardiac tumors are extremely rare. Whether the adult zebrafish myocardium is responsive to oncogene overexpression and how this condition affects its intrinsic regenerative capacity remains unknown. Here, we have established a strategy of inducible and reversible expression of HRASG12V in zebrafish cardiomyocytes. This approach stimulated a hyperplastic cardiac enlargement within 16 days. The phenotype was suppressed by rapamycin-mediated inhibition of TOR signaling. As TOR signaling is also required for heart restoration after cryoinjury, we compared transcriptomes of hyperplastic and regenerating ventricles. Both conditions were associated with upregulation of cardiomyocyte dedifferentiation and proliferation factors, as well as with similar microenvironmental responses, such as deposition of nonfibrillar Collagen XII and recruitment of immune cells. Among the differentially expressed genes, many proteasome and cell-cycle regulators were upregulated only in oncogene-expressing hearts. Preconditioning of the heart with short-term oncogene expression accelerated cardiac regeneration after cryoinjury, revealing a beneficial synergism between both programs. Identification of the molecular bases underlying the interplay between detrimental hyperplasia and advantageous regeneration provides new insights into cardiac plasticity in adult zebrafish.