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Aortic stenosis (AS) and pulmonic stenosis (PS) are two of the most common canine congenital heart diseases (CHD), with a high relative risk for Newfoundland dogs to develop inherited subvalvular AS. For this reason, a cardiovascular screening program has been set up by the French Newfoundland kennel club in order to manage mattings and reduce AS prevalence. The records of untreated and non-anesthetized adult Newfoundland dogs screened between 2010 and 2023 were retrospectively reviewed. All dogs underwent physical examination and standard transthoracic echocardiography with concomitant ECG tracing. All examinations were reviewed by one single board-certified specialist in cardiology. A total of 921 dogs were screened during the study period (female:male sex ratio = 1.94, median age [IQR] = 1.9 years [1.6-2.7], body weight = 55.0 kg [50-60]). For most dogs (90.6% for AS and 91% PS), a single examination was required to obtain a definitive cardiac status, although most operators (122/133 = 91.7%) were non-specialist general practitioners. Out of the 921 screened dogs, 913/921 (99.1%) and 919/921 (99.8%) were respectively free of AS and PS, with no AS and PS detection during the last 3 years of the program. The inbreeding coefficient, which was assessed from the pedigree analysis of all screened dogs except one, was not significantly different between dogs with either AS (0.59%; P = 0.86) or PS (0.39%; P = 0.72) and those without any arterial stenosis (0.39%). This 14-year cardiovascular screening program has experienced a strong involvement of veterinarians, breeders, and owners throughout France. Unlike reports from other European and North American countries, this program suggests the low and decreasing prevalence of both AS and PS in the Newfoundland breed in France.

Transluminal balloon valvuloplasty is an alternative to surgical valvotomy for congenital pulmonary valve stenosis. The aim of this study was to evaluate the long term results (to 13.5 years) of balloon pulmonary valvuloplasty. From June 1998 to January 2012, percutaneous balloon pulmonary valvuloplasty for congenital pulmonary valve stenosis was performed in 98 patients (50 males, 48 females, with a median age of 6.75 years) underwent balloon valvuloplasty of pulmonary valve stenosis. Follow-up was performed based on the Doppler echocardiographic data and clinical findings. Forty three of ninety eight patients were 10 years of age or older. The mean peak to peak pressure gradient across pulmonary valve before and immediately after balloon pulmonary valvuloplasty (BPV) was 88.7±36.4 mmHg and 21.8±15.9 mmHg (P<0.001) respectively. Doppler pressure gradient across pulmonary valve before BPV, at 3 month (short term), at 1 year (intermediate term) and long-term follow-up were 93.2±41.3 mmHg, 18.7±15.8 mmHg (P<0.001), 15.8±13.1 mmHg (P<0.001) and 13.6±7.4 mmHg (P<0.017) respectively. Mild pulmonary regurgitation (PR) was observed in 55 (57%) patients immediately after BPV and 30 (31%) patients at late follow up. Rupture of the right ventricular outflow tract was the major complication in two patients with fatal event. Short, intermediate and long-term results of BPV for typical valvular pulmonary stenosis are excellent. Therefore, it can be considered as treatment of choice for patients with typical valvular pulmonary stenosis.

Outcome data for neonates with critical pulmonary stenosis (PS) is limited. We aimed to review the outcomes after balloon pulmonary valvuloplasty (BPV) for neonates with critical PS at our institution. All neonates with critical PS who underwent BPV from 1990 to 2017 were included. A total of 44 neonates underwent BPV for critical PS. Nonright ventricular dependent coronary artery fistulas was seen in 6/44 (13.6%) patients. Tricuspid valve z-scores were −1.9 (interquartile range [IQR] −3.04, −0.48) in those with coronary artery fistulas as compared with −0.27 (IQR −0.5, 0.8) in those without (p = 0.03). Fifteen of forty-four subjects (34.1%) patients underwent reintervention with 10 patients (22.7%) requiring an alternate source of pulmonary blood flow (3 patients subsequently underwent right ventricular overhaul, 2 underwent Glenn operations, and 1 underwent repeat BPV). Five patients underwent reintervention for right ventricular outflow tract obstruction. Pulmonary valve annulus z-score was significantly smaller in those who needed reintervention −2.4 (IQR −2.9 to −0.95) versus −0.59 (IQR −1.3, −0.15); p = 0.02. At a median follow-up of 8.2 (IQR 3.4 to 13.1) years, moderate or severe pulmonary regurgitation was seen in 22/42 (53.7 %) patients with biventricular circulation, 3 requiring pulmonary valve repair/replacement. In conclusion, coronary artery fistulas occur in a significant number of patients with critical pulmonary stenosis, occurring more frequently in patients with small tricuspid valves. Reintervention is required for 1/3 of patients. Patients with small pulmonary valve annuli are more likely to undergo reintervention for right ventricular outflow tract obstruction. Significant pulmonary regurgitation is common and may require eventual pulmonary valve replacement.

Congenital heart diseases (CHDs) constitute the most prevalent congenital pathology, and they are a consequence of structural and functional abnormalities during fetal development. The etiology of CHD involves the interaction of genetic and environmental factors. Fetal cardiac surgery aims at preventing natural pathways of CHD in utero, mitigating progression to more complex abnormalities. The goal of this review was to demonstrate the benefits and risks of fetal interventions in the two most prevalent CHDs, pulmonary stenosis and pulmonary atresia with an intact ventricular septum, but also critical aortic stenosis and hypoplastic left heart syndrome. Original and relevant articles were selected by meta-aggregation to perform a qualitative analysis of fetal cardiac interventions for pulmonary stenosis and critical aortic stenosis. The Joanna Briggs Institute's Qualitative Assessment and Review Instrument (or JBI-QARI) was used for data quality appraisal. Of 61 potential articles, 13 were selected, and nine were finally included. Discussion: The present review demonstrated that fetal cardiac surgery increases right ventricular growth and hemodynamic flow in pulmonary stenosis, whereas in critical aortic stenosis it enables growth of the left ventricle and increases left ventricular pressure. However, it has a high complication rate, along with considerable morbidity and mortality. The benefits of fetal cardiac surgery for pulmonary stenosis and critical aortic stenosis are well-described in the literature; however, there is a significant risk of complications which can be reduced by the surgeon's technical expertise and well-structured hospital facilities.

Background Fetal deformity is a disease caused by abnormal chromosome structure, which may be influenced by genetic factors as well as the maternal and external environment. Magnetic resonance imaging (MRI) may be used to effectively diagnose fetus deformities. However it has been reported that gene analysis is a more accurate diagnostic method. The aim of the present study was to investigate the effectiveness of MRI in combination with gene analysis for the diagnosis of fetal congenital heart disease, a form of fetus deformity. Methods MRI, array comparative genome hybridization analysis and fluorescence in situ hybridization were used to analyze the effectiveness of the two methods in a total of 78 pregnant women with suspected fetal congenital heart disease. Results Our findings demonstrated that the combination of MRI and gene analysis resulted in significantly improved diagnostic accuracy, sensitivity and specificity for fetal congenital heart disease compared with either method alone. MRI combined with gene analysis confirmed 42 fetuses with pulmonary stenosis, 24 with aortic stenosis and 12 healthy fetuses, which was significantly improved compared with MRI or gene analysis alone. It was also observed that gene analysis was a more efficient method of diagnosis compared with MRI; however, the combination of the two methods was the most effective. Conclusion In conclusion, the results of the present study suggest that MRI combined with gene analysis may be a more effective diagnostic method for fetal congenital heart disease compared with the current protocol.

Described herein is an 84-year-old woman, the oldest reported, with severe pulmonic stenosis who underwent a highly successful pulmonic valvotomy at age 77 and highly unsuccessfully attempted percutaneous pulmonic valve implantation at age 84. During the 84 years she developed nearly all clinical and morphologic consequences of pulmonic stenosis, including heavy calcification of the pulmonic valve, heavy calcification of the tricuspid valve annulus, severe right ventricular wall thickening without ventricular cavity dilation, aneurysm of the pulmonary truck, multiple focal ventricular wall scars without narrowing of the epicardial coronary arteries, wall thickening and luminal narrowing of the intramural coronary arteries, and extremely low 12-lead QRS electrocardiographic voltage.

Pulmonary stenosis (PS) is a congenital heart disease characterized by a dynamic or fixed anatomic obstruction of blood flow from the right ventricle to the pulmonary arterial vasculature. In the present study, extracellular vesicle long RNAs (EVLRs) from pregnant females who had healthy infants or PS infants were analyzed by RNA sequencing, and their diagnostic potential for PS during pregnancy was evaluated. A method for the selection of genes that could be considered as informative for the prediction PS based on extracellular vesicles (EVs) from pregnant females using long-read RNA sequencing was developed. Blood samples were collected from females carrying fetuses with PS and females carrying unaffected fetuses (n=6 in each group). Physical characterization of EVs was performed using nanoparticle tracking analysis, transmission electron microscopy and western blotting. EVLRs from plasma were profiled by RNA sequencing and mRNA co-expression modules were constructed by weighted gene co-expression network analysis (WGCNA). Gene Ontology (GO) enrichment analysis was used to predict the function of the genes in each module. Hub genes were filtered out based on WGCNA and visualized using Cytoscape. EVLRs consisted of mRNAs, microRNAs and long non-coding RNA. Overall, 26 modules were identified containing 16,394 genes. All modules were independent of each other. One particular module, referred to as the blue module, was markedly different between the two groups. A total of 735 hub genes in the blue module were identified, of which 33 were visualized, demonstrating the connection between these hub genes. GO enrichment analysis demonstrated that the analyzed hub genes were enriched in 'glucose transport', 'ATP-dependent chromatin remodeling', 'histone deacetylation', 'histone H3-K4 methylation', 'DNA methylation', 'apoptotic signaling pathway' and 'glucocorticoid receptor signaling pathway'. The hub genes identified in this module may provide a genetic framework for prenatal PS diagnosis. Furthermore, functional analysis of these associated genes may provide a theoretical basis for further research on PS pathogenesis.

Introduction Percutaneous balloon valvuloplasty is the primary treatment for critical pulmonary valve stenosis in neonates. Thus far, a few studies have reported long-term results of this technique in neonatal critical pulmonary valve stenosis. We carried out a retrospective study of all consecutive newborns with critical pulmonary valve stenosis subjected to percutaneous balloon valvuloplasty at a single centre, between 1994 and 2014, to assess its immediate and long-term safety and efficacy. A total of 24 neonates presented with critical pulmonary valve stenosis. The mean diameter of the pulmonary annulus was 7 mm (±1.19); 33.3% had a dysplastic pulmonary valve, and 92% were started on prostaglandin E1 treatment. Percutaneous balloon valvuloplasty was performed at a mean age of 4.0±4.3 days using, on average, a balloon-to-pulmonary annulus ratio of 1.18 mm (with a range from 0.9 to 1.43). Immediate success was achieved in 22/24 patients (92%) with a reduction in the pulmonary transvalvular peak gradient (p<0.05) and in the right ventricle/systemic pressure ratio (p<0.05). There was one death (4%) 6 days after the procedure, and 29.2% of them had transient rhythm complications. For a mean follow-up time of 8.4 years, the re-intervention rate was 42.9%. In total, 14 re-interventions were performed in nine neonates, including surgery in six. Freedom from re-intervention was 50% at 8 years and 43% at 10 and 15 years. This series, to the best of our knowledge, has had the longest follow-up of neonates with critical pulmonary valve stenosis. Percutaneous balloon valvuloplasty is a safe and effective treatment, and in our study 75% of the patients were exclusively treated using this technique.

Residual right ventricular outflow obstruction during Tetralogy of Fallot repair necessitates peri-operative revision often requiring trans-annular patch with its negative sequels. Bidirectional Glenn shunt in this setting reduces trans-pulmonary gradient to avoid revision. Bidirectional Glenn shunt was added during Tetralogy repair in patients with significant residual obstruction. A total of 53 patients between January, 2011 and June, 2018 were included. Final follow-up was conducted in July, 2018. Mean age at operation was 5.63±3.1 years. Right to left ventricular pressure ratio reduced significantly (0.91±0.09 versus 0.68±0.05; p<0.001) after bidirectional Glenn, avoiding revision in all cases. Glenn pressures at ICU admission decreased significantly by the time of ICU discharge (16.7±3.02 versus 13.5±2.19; p<0.001). Pleural drainage ≥ 7 days was seen in 14 (26.4%) patients. No side effects related to bidirectional Glenn-like facial swelling or veno-venous collaterals were noted. Mortality was 3.7%. Discharge echocardiography showed a mean trans-pulmonary gradient of 32.11±5.62 mmHg that decreased significantly to 25.64±5 (p<0.001) at the time of follow-up. Pulmonary insufficiency was none to mild in 45 (88.2%) and moderate in 6 (11.8%). Mean follow-up was 36.12±25.15 months (range 0.5-90). There was no interim intervention or death. At follow-up, all the patients were in NYHA functional class 1 with no increase in severity of pulmonary insufficiency. Supplementary bidirectional Glenn shunt significantly reduced residual right ventricular outflow obstruction during Tetralogy of Fallot repair avoiding revision with satisfactory early and mid-term results.

The Melody® transcatheter pulmonary valve system was developed for placement within right ventricle-to-pulmonary artery conduits in patients with CHD for treatment of stenosis or regurgitation, providing an alternative to open-heart surgery. Abnormal systemic venous connections altering the catheter course to the right ventricle-to-pulmonary artery conduit may present a challenge to Melody® valve implantation. We present two such cases, in which the Melody® valve was successfully implanted in teenage patients with congenitally corrected transposition of the great arteries after Senning atrial switch operation. Despite the abnormal catheter course, the right ventricle-to-pulmonary artery was approachable via the right femoral vein allowing for deployment of the Melody® valve in the appropriate position. This suggests that systemic vein-to-left atrium baffles are not prohibitive of Melody® valve implantation. This is an important implication considering the substantial population of ageing patients with CHD who have undergone atrial switch. Melody® valve implantation can be considered as a viable option for treatment of these patients if they develop right ventricle-to-pulmonary artery conduit failure.