右心发育不良综合征表现为右心室、三尖瓣和（或）肺动脉瓣发育不良，右心发育不良程度决定远期预后。胎儿肺动脉瓣成形术可改变疾病的自然病史，促进右心继续发育，利于生后双心室循环。本文对心脏介入治疗右心发育不良综合征胎儿的进展进行综述。.

BACKGROUND: Endoscopic lung volume reduction with endobronchial valves has been widely recognized for treating hyperinflation in advanced chronic obstructive pulmonary disease and emphysema patients. The main challenges include the technical complexity of upper lobe implantation and the number of endobronchial valves required. These issues might be addressed by placing larger diameter valves in the lobar bronchus. This study evaluated the feasibility, efficiency, and safety of the new valve PulmValve (model PV-13) in porcine models.
METHODS: Six PV-13 valves were bronchoscopically implanted into the caudal lobe bronchus of six healthy pigs. The procedure time, valve deployment, and removability were recorded. Follow-up examinations included blood tests, chest CT scans, and bronchoscopy at 30 min, 14 days, 28 days, and 84 days post-procedure, with necropsy and pathological evaluations after the final follow-up examination.
RESULTS: The successful in vivo deployment and removal of PV-13 valves was established, with a median procedure time of 6.5 min. The distal lung volume reduction was evident at 30 min post-operation and was persistently monitored on day 84. No migration or malfunction of any PV-13 valves was detected, but a mild angle deviation was found in 3 cases. Coughing was observed in four pigs within the first 7 days and localized granulation tissue was observed in all pigs. No cases of pneumothorax, diffuse pneumonia, or hemoptysis were detected.
CONCLUSIONS: In this study, we report the successful implantation and removal of a new valve PulmValve in a short operation time. Complete lobar atelectasis was induced without device migration, malfunction, or severe complications. Further studies are warranted to evaluate the long-term, sustained effects and potential benefits in human patients.

Transcatheter pulmonary valve replacement (TPVR), also known as percutaneous pulmonary valve implantation, refers to a minimally invasive technique that replaces the pulmonary valve by delivering an artificial pulmonary prosthesis through a catheter into the diseased pulmonary valve under the guidance of X-ray and/or echocardiogram while the heart is still beating not arrested. In recent years, TPVR has achieved remarkable progress in device development, evidence-based medicine proof and clinical experience. To update the knowledge of TPVR in a timely fashion, and according to the latest research and further facilitate the standardized and healthy development of TPVR in Asia, we have updated this consensus statement. After systematical review of the relevant literature with an in-depth analysis of eight main issues, we finally established eight core viewpoints, including indication recommendation, device selection, perioperative evaluation, procedure precautions, and prevention and treatment of complications.

Fallot-type absent pulmonary valve is a rare and complex congenital heart disease. Repair surgery for this condition during the neonatal period has a mortality rate of over 50%. We reported a neonate with Fallot-type absent pulmonary valve and occlusion of the left main bronchus. The patient\'s pulmonary artery had unusual anatomy of a type that has not previously been reported. This case report outlines a successful treatment strategy for patients with complex congenital heart disease and airway occlusion during the neonatal period and the effect of these unusual anatomical conditions on postoperative outcomes.

Percutaneous pulmonary valve implantation (PPVI) with the self-expandable Venus P-valve system is a promising treatment for patients with pulmonary regurgitation (PR) and a native right ventricular outflow tract (RVOT). However, limited data is available regarding its midterm outcomes. This study assessed the midterm clinical and echocardiographic outcomes following Venus P-valve implantation.
From 2013 to 2018, 55 patients with moderate or severe PR after surgical RVOT repair with a transannular or RVOT patch were consecutively enrolled from six hospitals in China. Five-year clinical and echocardiographic outcomes were collected and evaluated. The primary endpoint was a freedom from all-cause mortality and reintervention.
At 5 years, the primary endpoint was met for 96% of patients, corresponding to a freedom from all-cause mortality of 96% (95% confidence interval [CI]: 86%-99%) and freedom from reintervention of 98% (95% CI: 87%-100%). Endocarditis was reported in five patients (four patients within 1 year and one patient at 5 years) following PPVI. Transpulmonary gradient and stent orifice diameter remained stable compared to at discharge (p＞0.05). No paravalvular leak was reported while only 1 patient gradually increased to moderate PR during follow-up. Significant improvement of RV diameter and LVEF (p＜0.001) sustained over the 5-year follow-up, in consistent with remarked improved New York Heart Association(NYHA) functional class (p＜0.001).
The 5-year results of the China VenusP Study demonstrated the midterm benefits of Venus P-valve implantation in the management of patients with severe PR with an enlarged native RVOT by providing sustained symptomatic and hemodynamic improvement.

Pulmonary valve (PV) monocusp reconstruction in transannular patch (TAP) right ventricular outflow tract (RVOT) repair for Tetralogy of Fallot has variable clinical outcomes across different surgical approaches. The study purpose was to systematically evaluate how monocusp leaflet design parameters affect valve function in-vitro.
A 3D-printed, disease-specific RVOT model was tested under three infant physiological conditions. Monocusps were sewn into models with the native main pulmonary artery (MPA) forming backwalls that constituted 40% and 50% of the reconstructed circumference for z-score zero PV annulus and MPA diameters (native PV z-score - 3.52 and - 2.99 for BSA 0.32m2). Various leaflet free edge lengths (FEL) (relative to backwall), positions (relative to PV STJ), and scallop depths were investigated across both models. Pressure gradient, regurgitation, and coaptation were analyzed with descriptive statistics and regression models.
Increasing FEL beyond 100% of the MPA backwall decreased gradient but mildly increased regurgitation to a peak of 25%. Positioning the free edge 2 mm past the STJ mildly increased gradient for each FEL without significantly changing regurgitation compared to STJ placement. Scalloping leaflets trivially affected performance. Pre-folding leaflets improved mobility and slightly reduced gradient.
Balancing gradient, regurgitation, and oversizing for growth, a set of leaflet designs have been selected for pre-clinical evaluation. Designs with leaflet widths 140-160% in the 40% backwall model (110-120% in the 50% backwall), positioned at or 2 mm past the STJ, demonstrated the best results. The next stage of ex-vivo testing will additionally consider native RVOT distensibility, native leaflet interactions, and TAP characteristics.

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BACKGROUND: Due to the wide variety of morphology, size, and dynamics, selecting an optimal valve size and location poses great difficulty in percutaneous pulmonary valve implantation (PPVI). This study aimed to report our experience with in vitro bench testing using patient-specific three-dimensional (3D)-printed models for planning PPVI with the Venus P-valve.
METHODS: Patient-specific 3D soft models were generated using PolyJet printing with a compliant synthetic material in 15 patients scheduled to undergo PPVI between July 2018 and July 2020 in Central China Fuwai Hospital of Zhengzhou University.
RESULTS: 3D model bench testing altered treatment strategy in all patients (100%). One patient was referred for surgery because testing revealed that even the largest Venus P-valve would not anchor properly. In the remaining 14 patients, valve size and/or implantation location was altered to avoid valve migration and/or compression coronary artery. In four patients, it was decided to change the point anchoring because of inverted cone-shaped right ventricular outflow tract (RVOT) ( n = 2) or risk of compression coronary artery ( n = 2). Concerning sizing, we found that an oversize of 2-5 mm suffices. Anchoring of the valve was dictated by the flaring of the in- and outflow portion in the pulmonary artery. PPVI was successful in all 14 patients (absence of valve migration, no coronary compression, and none-to-mild residual pulmonary regurgitation [PR]). The diameter of the Venus P-valve in the 3D simulation group was significantly smaller than that of the conventional planning group (36 [2] vs. 32 [4], Z = -3.77, P <0.001).
CONCLUSIONS: In vitro testing indicated no need to oversize the Venus P-valve to the degree recommended by the balloon-sizing technique, as 2-5 mm sufficed.

Objectives: To evaluate the feasibility and preliminary clinical results of transcatheter pulmonary valve replacement (TPVR) with the domestically-produced balloon-expandable Prizvalve system. Methods: This is a prospective single-center observational study. Patients with postoperative right ventricular outflow tract (RVOT) dysfunction, who were admitted to West China Hospital of Sichuan University from September 2021 to March 2023 and deemed anatomically suitable for TPVR with balloon-expandable valve, were included. Clinical, imaging, procedural and follow-up data were analyzed. The immediate procedural results were evaluated by clinical implant success rate, which is defined as successful valve implantation with echocardiography-assessed pulmonary regurgitation目的： 初步探讨使用国产球囊扩张式瓣膜Prizvalve进行经导管肺动脉瓣置换（TPVR）的可行性及临床效果。 方法： 本研究为单中心前瞻性单组观察性研究，连续纳入2021年9月至2023年3月于四川大学华西医院就诊、经评估适合通过球囊扩张式瓣膜进行TPVR治疗的外科术后右心室流出道功能障碍患者，均使用Prizvalve进行TPVR治疗。记录患者的临床、影像和手术资料、手术效果及随访结果。即刻效果主要通过临床植入成功率评价，定义为瓣膜顺利植入且术后超声心动图评估的肺动脉瓣反流程度在中度以下、肺动脉瓣峰值压差<40 mmHg（1 mmHg=0.133 kPa）。 结果： 共纳入5例患者，其中4例男性，年龄14~37岁。初始诊断包括法洛四联症（2例）、永存动脉干（1例）、肺动脉闭锁（1例）和主动脉瓣下狭窄（1例，既往行Ross手术）；4例通过同种或人工管道行右心室流出道重建，1例通过跨瓣补片技术重建右心室流出道。干预指征包括右心室流出道-肺动脉狭窄合并反流（3例）、单纯狭窄（1例）及单纯反流（1例）。4例存在右心室流出道-肺动脉狭窄的患者术前平均右心室流出道峰值流速为3.5 m/s，平均右心室流出道峰值压差为50.0 mmHg。除1例患者既往因主肺动脉严重狭窄已植入覆膜Cheatham-Platinum（CP）支架外，其余患者在术中均先预植入覆膜CP支架再进行瓣膜植入。5例患者均临床植入成功，围术期无严重并发症出现。术后超声心动图测量平均右心室流出道-肺动脉峰值流速为2.3 m/s，平均右心室流出道-肺动脉峰值压差为21.2 mmHg。术后患者症状均明显改善，所有患者均已完成3个月随访，其中4例已完成6个月随访，随访期内无感染性心内膜炎发生，最近一次随访时评估纽约心脏病协会（NYHA）心功能均为1级。 结论： 对于解剖条件合适的外科术后右心室流出道-肺动脉功能障碍患者，通过国产球囊扩张式短瓣膜Prizvalve进行TPVR是可行的治疗方案，安全性、有效性和长期耐久性数据值得进一步研究。.

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