先天性僧帽弁狭窄症

A six-year-old neutered male cat was presented for acute respiratory distress. Transthoracic echocardiography identified a left atrial membrane consistent with cor triatriatum sinister (CTS) and turbulent flow across a single fenestration. Additionally, it was suspected that the distal chamber may receive at least one pulmonary vein. Because respiratory signs improved with corticosteroids but not with a short diuretic trial, and because echocardiography suggested that the distal chamber might receive at least one pulmonary vein, cardiac computed tomography angiography was performed to further characterize the pulmonary venous anatomy and to differentiate CTS from pulmonary vein stenosis and other congenital vascular malformations. Cardiac computed tomography angiography confirmed left atrial subdivision with one interchamber orifice (5.33 mm) and demonstrated a pulmonary venous connection pattern in which three pulmonary veins connected to the proximal chamber, whereas the left cranial pulmonary vein connected to the distal chamber, which was continuous with the mitral valve. Adapting the human Lucas classification scheme, the anatomic configuration most closely resembled Lucas type C1a, and no congestive heart failure attributable to CTS was identified.

Left ventricular (LV) longitudinal strain (LS) has prognostic value in adults with valvar aortic stenosis (AS), and impaired left atrial (LA) strain is associated with elevated filling pressures. The role of LA and LV strain in children with congenital AS is unclear. We hypothesized that LA and LV strain would be worse with higher gradients and/or greater ventricular mass. We performed a retrospective cross-sectional study (2015-2025) of children aged 1-18 years with isolated congenital AS and preserved systolic function, stratified by Doppler-derived mean gradient as mild (<20 mmHg), moderate (20-40 mmHg), or severe (>40 mmHg). Patients with critical neonatal AS, more than mild aortic regurgitation, prior intervention, genetic syndromes, or inadequate image quality were excluded. LA reservoir, conduit, and contraction strain and LV LS were measured by two-dimensional speckle-tracking echocardiography. Group comparisons and correlation analyses evaluated associations with aortic valve gradient, LV mass index, and mitral E/A ratio. Eighty-four children were identified (mean age 8.7 ± 4.6 years; 33% female). LA strain components (all p>0.2) and LV LS (p=0.8) did not differ across AS groups. Stratification by LV mass Z score (≤2 vs. >2) showed no differences in LA or LV strain. No strain parameter correlated with aortic valve gradient, LV mass index, or mitral E/A ratio (all p>0.05). In children with congenital AS, preserved systolic function, and no prior intervention, LA and LV strain are preserved across disease severity and ventricular hypertrophy categories, suggesting preserved myocardial function and absence of adverse ventricular remodeling.

INTRODUCTION/OBJECTIVES: Pulmonary stenosis (PS) is a common congenital heart disease in dogs, often associated with poststenotic dilation of the pulmonary trunk or main pulmonary artery (MPA). We hypothesized that vertebrae-based radiographic measurement of the MPA might identify dogs with PS and potentially assess its severity. ANIMALS, MATERIALS, AND METHODS: A retrospective cohort study of 29 dogs with PS, 30 normal dogs, and 30 dogs with myxomatous mitral valve disease was conducted. Vertebral main pulmonary artery size (VMPS) was measured using dorsoventral (vertebral main pulmonary artery size measured using the dorsoventral radiograph [VMPS-DV] type 1, type 2) and right lateral (vertebral main pulmonary artery size measured using the right lateral radiograph [VMPS-LATE]) thoracic radiographs. Group differences were analyzed using the Kruskal-Wallis test. Correlation between VMPS and maximal instantaneous pressure gradient (continuous wave Doppler) was assessed using Spearman's correlation. Diagnostic performance was evaluated using receiver-operator characteristic analysis with Youden's index. RESULTS: Vertebral main pulmonary artery size measured using the dorsoventral radiograph type 1 and VMPS-LATE were significantly greater in the PS group than in the normal group (P<0.001) but not significantly different from the myxomatous mitral valve disease group. After excluding French bulldogs, these indices remained significantly elevated. A VMPS-DV type 1 > 1.06 and a VMPS-LATE >3.6 were identified as optimal cutoffs for distinguishing dogs with PS from normal. No significant correlations between VMPS and pressure gradient were identified. STUDY LIMITATIONS: small sample size limited statistical power. Due to the absence of echocardiographic data, a comparison of MPA size between radiographic and echocardiographic modalities was not possible. CONCLUSIONS: Although VMPS did not reflect PS severity, a VMPS-DV type 1 > 1.06 and a VMPS-LATE >3.60 support the diagnosis of PS in dogs with compatible clinical findings.

OBJECTIVE: Managing pediatric mitral valve (MV) conditions remains demanding. This study characterizes contemporary practice patterns and outcomes in pediatric MV surgery across the European Congenital Heart Surgeons Association Congenital Database (ECHSA-CD). METHODS: We conducted a retrospective, multi-institutional analysis using ECHSA-CD. The cohort included 6,736 patients <18 years who underwent MV surgery (01/01/2003-12/31/2024). Exclusion criteria were atrioventricular septal defects, functionally univentricular physiology, and non-systemic mitral valves. RESULTS: MV-Dysplasia was the primary etiology (5,916/6,736=87.8%), and regurgitation was the predominant hemodynamic mechanism (4,284/5,548=77.2%). MV repair was the most common surgical strategy (5,513/6,736=81.8%), and, while MV repair predominated across all age groups, the frequency of mitral valve replacement (MVR) increased with advancing age. Overall, in-hospital mortality rate was 245/6,736=3.6%, with a significant reduction observed in the most recent decade (130/2,814=4.6% versus 115/3,922=2.9%; p<0.001). In-hospital mortality was significantly elevated among neonates (OR:14.8; 95%CI:9.86-22.2; p<0.001), patients with MV stenosis (OR:2.50; 95%CI=1.77-3.54; p<0.001), and those receiving MVR (OR:2.35; 95%CI:1.78-3.10; p<0.001), and intraoperative conversion from MV repair-to-MVR (OR:3.62; 95%CI:1.71-7.65; p<0.001). Receiver operating characteristic analysis identified critical weight thresholds for mortality at 5.8kg for the overall cohort (AUC=0.73; 95%CI:0.70-0.77; p<0.001) and 12.6kg in MVR (AUC=0.74; 95%CI:0.67-0.80; p<0.001). CONCLUSIONS: Pediatric MV surgery outcomes have significantly improved over time. However, the procedure remains a high-risk intervention specifically for infants, where low body weight is one of the primary drivers of adverse outcomes.

BackgroundPediatric mechanical mitral valve replacement (mMVR) is a high-risk procedure associated with long-term complications and lifelong anticoagulation. This study aimed to evaluate long-term outcomes of mMVR in patients under 18 years of age and to identify risk factors for survival and reoperation.MethodsWe retrospectively analyzed 118 patients under 18 years of age who underwent mMVR at a single center from 1965 to 2024. Demographics, operative data, and outcomes were reviewed. Survival was assessed using Kaplan-Meier analysis, and competing risk models evaluated reoperation. Cox regression identified risk factors for mortality and redo mMVR.ResultsMedian age and weight at initial mMVR were 6.3 years and 14.0 kg, respectively. Early mortality was 12% (14/118), with 24 late deaths over a median follow-up of 15.6 years. Overall survival at 5, 10, 20, and 30 years was 70.5%, 65.0%, 61.5%, and 61.5%, respectively. Multivariate analysis identified non-bileaflet valve use (HR = 2.46) and surgery year (HR = 1.03) as independent predictors of mortality. Thirty three patients underwent 36 redo mMVR, mainly for structural valve deterioration or patient-prosthesis mismatch, with a median interval of 10.1 years. Age <10 years at initial mMVR was an independent predictor of redo mMVR. Young patients receiving adequately sized prostheses demonstrated excellent long-term freedom from redo mMVR.ConclusionsLong-term survival after pediatric mMVR is satisfactory. Valve type and surgery year significantly impact mortality, while younger age at surgery increases the risk of redo mMVR. These findings underscore the importance of timing and prosthesis selection to improve long-term outcomes in pediatric mMVR patients.