暂无摘要。

BACKGROUND: The repair of certain types of complex congenital cardiac defects may require a right ventricle-pulmonary artery (RV-PA) conduit. Using the Ozaki Aortic valve neocuspidization (AVNeo)technique, a valved RV-PA conduit was constructed with an Ozaki valve inside a Dacron graft. This study aims to evaluate the short-term outcome of the Ozaki valved RV-PA conduit.
METHODS: A total of 22 patients received the Ozaki valved RV-PA conduit from November 2019 until December 2023. The median age was 12 years (interquartile range [IQR], 5.5-21), median body weight was 35 kg (IQR, 15.8-48.5). The conduit was used in 16 patients (72.7%) under 18 years of age. Indications for conduit placement included: anatomic repair of corrected transposition of the great arteries, ventricular septal defect/pulmonary stenosis, conduit replacement, pulmonary atresia with associated anomalies, pulmonary artery aneurysm with dysplastic pulmonary valve, tetralogy of Fallot with coronary artery crossing the right ventricular outflow tract, bioprosthetic pulmonary valve regurgitation, and rheumatic heart disease. Native pericardium was used for the Ozaki valve in 12 patients and bovine pericardium for 10 patients. Conduit sizes ranged from 18 mm to 30 mm.
RESULTS: The median intensive care unit stay was 4 (IQR, 2-6) days and the median hospital stay was 9 (IQR, 5.5-13.5) days. There were two perioperative mortalities (9.1%) both unrelated to the conduit. The median follow-up was 12.3 (IQR, 4.43-21.2) months. There was no infective endocarditis of the conduit. The median peak gradient across the conduit was 22 mm Hg (range 0-44 mm), and all were competent with trivial regurgitation on follow up.
CONCLUSIONS: Creation of an Ozaki valved conduit is an attractive option due to low cost, reproducibility, and excellent hemodynamics. Longer-term studies are needed to confirm the durability.

Heart valve tissue engineering holds the potential to transform the surgical management of congenital heart defects affecting the pediatric pulmonary valve (PV) by offering a viable valve replacement. While aiming to recapitulate the native valve, the minimum requirement for tissue engineered heart valves (TEHVs) has historically been adequate mechanical function at implantation. However, long-term in situ functionality of TEHVs remains elusive, suggesting that a closer approximation of the native valve is required. The realization of biomimetic engineered pediatric PV is impeded by insufficient characterization of healthy pediatric tissue. In this study, we comprehensively characterized the planar biaxial tensile behaviour, extracellular matrix (ECM) composition and organization, and valvular interstitial cell (VIC) phenotypes of PVs from piglets to provide benchmarks for TEHVs. The piglet PV possessed an anisotropic and non-linear tension-strain profile from which material constants for a predictive constitutive model were derived. The ECM of the piglet PV possessed a trilayer organization populated by collagen, glycosaminoglycans, and elastin. Biochemical quantification of ECM content normalized to wet weight and DNA content of PV tissue revealed homogeneous distribution across sampled regions of the leaflet. Finally, VICs in the piglet PV were primarily quiescent vimentin-expressing fibroblasts, with a small proportion of activated α-smooth muscle actin-expressing myofibroblasts. Overall, piglet PV properties were consistent with those reported anecdotally for pediatric human PVs and distinct from those of adult porcine and human PVs, supporting the utility of the properties determined here to inform the design of tissue engineered pediatric PVs. STATEMENT OF SIGNIFICANCE: Heart valve tissue engineering has the potential to transform treatment for children born with defective pulmonary valves by providing living replacement tissue that can grow with the child. The design of tissue engineered heart valves is best informed by native valve properties, but native pediatric pulmonary valves have not been fully described to date. Here, we provide comprehensive characterization of the planar biaxial tensile behaviour, extracellular matrix composition and organization, and valvular interstitial cell phenotypes of pulmonary valves from piglets as a model for the native human pediatric valve. Together, these findings provide standards that inform engineered heart valve design towards generation of biomimetic pediatric pulmonary valves.

A young female patient was referred by her general practitioner to the ear, nose and throat outpatient clinic with a 5-year history of progressive hoarseness and loss of voice. A chest radiograph was reported as showing marked dilatation of the left pulmonary artery without any other features to suggest underlying pulmonary arterial hypertension. A provisional diagnosis of left recurrent laryngeal nerve palsy secondary to its compression between the left pulmonary artery and the undersurface of the aortic arch-Ortner\'s or cardiovocal syndrome-was suggested and confirmed on further investigation including CT, microlaryngoscopy and echocardiography.

Valve remodeling is a process involving extracellular matrix organization and elongation of valve leaflets. Here, through single-cell RNA sequencing of human fetal valves, we identified an elastin-producing valve interstitial cell (VIC) subtype (apolipoprotein E (APOE)+, elastin-VICs) spatially located underneath valve endothelial cells (VECs) sensing unidirectional flow. APOE knockdown in fetal VICs resulted in profound elastogenesis defects. In valves with pulmonary stenosis (PS), we observed elastin fragmentation and decreased expression of APOE along with other genes regulating elastogenesis. Cell-cell interaction analysis revealed that jagged 1 (JAG1) from unidirectional VECs activates elastogenesis in elastin-VICs through NOTCH2. Similar observations were made in VICs cocultured with VECs under unidirectional flow. Notably, a drastic reduction of JAG1-NOTCH2 was also observed in PS valves. Lastly, we found that APOE controls JAG1-induced NOTCH activation and elastogenesis in VICs through the extracellular signal-regulated kinase pathway. Our study suggests important roles of both APOE and NOTCH in regulating elastogenesis during human valve remodeling.

BACKGROUND: Isolated pulmonary valve endocarditis (IPE) accounts for less than 2% of all infective endocarditis patients. It is commonly associated with several predisposing factors, including intravenous drug use (IVDU) and congenital heart disease. The most common causative pathogens of IPE are Staphylococcus aureus and Streptococcus viridans. We report a Down\'s syndrome patient with IPE and with no standard risk factors caused by the rare pathogen Acinetobacter spp. This led to respiratory failure and systemic infection due to septic pulmonary emboli. Early elective surgery was decided upon as the patient was no longer responding to medical therapy, and his clinical condition was worsening over time.
METHODS: A 15-year-old male with Down syndrome and no underlying heart defect presented with a 3-month history of episodic fever, nausea, vomiting, and diarrhea. Transthoracic echocardiography (TTE) revealed large vegetation on the pulmonary valve leaflet, another mobile mass at the pulmonary artery bifurcation, and severe pulmonary regurgitation. Serial blood cultures isolated Acinetobacter spp. Despite initial antibiotic therapy, the patient continued to have sepsis, unresolved vegetations, and developed life-threatening complications and respiratory distress, which convinced us to perform a pulmonary valve replacement surgery with a homograft. After surgery, the patient recovered and was discharged on the ninth postoperative day (POD).
CONCLUSIONS: This report highlights IPE\'s diagnostic and therapeutic challenges, alongside the importance of a comprehensive cardiopulmonary workup in patients with unexplained fever, sepsis, and pulmonary symptoms, even without typical risk factors. Based on the patient\'s aggravating condition despite medical treatment, early surgical intervention and pulmonary valve replacement were deemed crucial. However, there still needs to be a definitive guideline on when and how surgery should be performed in patients with complicated IPE, especially in pediatric patients.

暂无摘要。

Objectives: It is well-known that right ventricle-to-pulmonary artery homograft conduit durability is worse for smaller conduits and smaller/younger patients. However, there is limited literature on age and conduit-size specific outcomes, or on the role of conduit oversizing. Methods: Patients diagnosed with tetralogy of Fallot and major aortopulmonary collateral arteries undergoing right ventricular outflow tract (RVOT) reconstruction with a valved aortic homograft conduit from November 2001 through March 2023, at our institution were included. Conduits were grouped and evaluated by diameter, diameter Z-score, and patient age at implant. The primary time-related outcome was freedom from RVOT reintervention. Factors associated with freedom from time-related outcomes were assessed with univariable Cox regression analysis. Results: A total of 863 RVOT conduits were implanted in 722 patients. On multivariable analysis, younger age, male sex, Alagille syndrome, smaller diameter of the conduit, and smaller Z-score were associated with shorter freedom from reintervention. Among patients with smaller diameter conduits, larger Z-scores were associated with longer freedom from conduit reintervention (P < .001). Transcatheter interventions were commonly used to extend conduit lifespan across ages and conduit sizes. Conclusions: Larger conduit diameter, older age, and higher conduit Z-score were associated with longer freedom from reintervention in patients undergoing RVOT reconstruction in this cohort. Oversizing of conduits, even beyond a Z-score of 4, is generally appropriate.

This consensus document for the performance of cardiovascular computed tomography (CCT) to guide intervention in the right ventricular outflow tract (RVOT) in patients with congenital disease (CHD) was developed collaboratively by pediatric and adult interventionalists, surgeons and cardiac imagers with expertise specific to this patient subset. The document summarizes definitions of RVOT dysfunction as assessed by multi-modality imaging techniques and reviews existing consensus statements and guideline documents pertaining to indications for intervention. In the context of this background information, recommendations for CCT scan acquisition and a standardized approach for reporting prior to surgical or transcatheter pulmonary valve replacement are proposed and presented. It is the first Imaging for Intervention collaboration for CHD patients and encompasses imaging and reporting recommendations prior to both surgical and percutaneous pulmonary valve replacement.

暂无摘要。