Unguarded tricuspid orifice is a rare anomaly of the tricuspid valve characterized by complete absence of tricuspid valve tissue and chordae on a proportion of the annulus. Management strategies vary widely. We report a case of successful repair of an unguarded tricuspid orifice with a simplified technique. A 35-year-old male presented with severe tricuspid regurgitation and right ventricular volume overload. Intraoperative inspection of the valve revealed an unguarded tricuspid orifice. For repair, sliding plasty of anterior and posterior leaflets were performed, followed by ring annuloplasty and commissuroplasty. Postoperative, echocardiogram showed minimal residual tricuspid regurgitation and significantly improved right ventricular dimensions. This case highlights the possibility of successful repair of an unguarded tricuspid orifice. If feasible, repair can be a good choice.

Surgical repair of ventricular septal defects (VSDs) with straddling atrioventricular (AV) valve chordae is challenging due to the risk of disrupting valve integrity. We report the successful use of a dual-patch technique in a 5-month-old girl (6.1 kg) with Down syndrome, presenting with a large inlet VSD, secundum atrial septal defect (ASD), and straddling chordae involving both AV valves. Ventricular septal defects closure was performed via right atriotomy using 2 glutaraldehyde-treated autologous pericardial patches placed on the superior and inferior septal margins, encasing the chordae without division. Mitral and tricuspid valve clefts were repaired, and the ASD was closed primarily. Postoperative echocardiography showed no residual VSD and only mild AV valve regurgitation. This approach preserved valvular geometry and avoided conduction disturbance. The dual-patch technique offers a physiologic and conservative solution when conventional VSD repair is precluded by straddling chordae. It avoids chordal translocation or reimplantation, maintaining the native architecture and function of the AV valves. Tricuspid regurgitation is a comparatively common anomaly. Although mild tricuspid regurgitation is commonly present, hemodynamically significant tricuspid regurgitation can lead to right ventricular dysfunction and cause substantial morbidity and mortality. Structural modifications of any or all of the tricuspid valve apparatus may cause the development of tricuspid regurgitation. Anatomy The right atrioventricular valve apparatus, or tricuspid valve complex (see Image. Valves of the Heart), consists of the following 4 components: Valve leaflets: Anterior, posterior, and septal: There may be as few as two and as many as 6 leaflets. The anterior leaflet is typically the largest, and the septal leaflet is the smallest. . Fibrous tricuspid valve annulus. Supporting chordae tendineae. Papillary muscles: from 2 to 9. : Just before the onset of the right ventricular systole, the papillary muscle contracts to increase tension in the chordae tendineae so that the 3-valve cusps coapt, preventing regurgitation across the tricuspid valve. The conduction system and the fibroelastic cardiac skeleton's supporting structure coordinate the tricuspid valve's actions. The tricuspid valve is located between the right atrium and the right ventricle and has a valve area of 4 to 6 cm2. The pathophysiological variants of the tricuspid valve include the following: Ebstein anomaly. Tricuspid atresia. Congenital tricuspid stenosis. Congenital cleft of the anterior leaflet.

Objective: To evaluate the intermediate and long-term outcomes and technical aspects of transcatheter closure (TCC) of coronary cameral fistulas (CCF) in pediatric patients. Methods: This was a case-control study. All pediatric patients with CCF who underwent TCC between January 2005 and December 2019 were retrospectively reviewed. Data was collected from medical records, including demographic characteristics, procedural details, intraoperative and postoperative serious adverse events, follow-up results and prognosis. Patients with serious adverse events and without serious adverse events were compared regarding their clinical features and CCF characteristics. Comparisons between groups were performed with independent sample t test, chi-square test or Fisher exact test. Results: A total of 66 CCF patients (34 boys, 32 girls, 3.9 (1.9, 6.2) years old, 15 (11, 20) kg) underwent attempted TCC. All of the CCF were all medium or large fistulas including 55 proximal fistulas (83%) and 11 distal fistulas (17%). The CCF originated more frequently from the right coronary artery (38 cases (58%)), followed by the left coronary artery (28 cases (42%)). The incidence of coronary artery aneurysms (CAA) was 61% (40/66).Procedural treatment was achieved in 64 patients and procedural success was achieved in 59 patients (92%). Six (9%) serious adverse events occurred in 5 patients during the perioperative period. Acute complications included procedure-related death in one patient and acute myocardial infarction in one patient. Periprocedural complications occurred in 3 patients at one day postoperatively including acute myocardial infarction (2 cases), occluder detachment (1 case), and tricuspid chordae tendinae rupture (1 case). Clinical follow-up data were available in 58 of the 62 patients who underwent initial successful TCC with a follow-up period of 9.3 (6.5, 13.4) years. Ten adverse events occurred in 9 patients including 5 complications consisted of aortic valve perforation (1 case), coronary thrombosis (1 case), progressive aneurysmal dilation after reintervention (1 case), and new-onset tricuspid valve prolapse with significant regurgitation (2 cases) and large residual shunts due to fistula recanalization (5 cases). Therefore, the incidence of intermediate and long-term adverse events was 17% (10/58). During the periprocedural and follow-up period, 16 adverse events occurred in 13 patients, whereas no adverse events occurred in 51 patients. Patients with seriovs adverse events presented with larger proportion of large CCF (11/13 vs. 39% (20/51), P=0.005), giant CAA (10/13 vs.14% (7/51), P=0.030), and higher mean pulmonary artery pressure ((20±9) vs.(16±6) mmHg, 1 mmHg=0.133 kPa, t=2.02, P=0.048) compared to patients without serious adverse events. Conclusions: TCC in CCF children appears to be effective with favorable intermediate and long-term outcomes. Strict indication of TCC is mandatory. 目的： 总结经导管介入治疗儿童先天性冠状动脉-心腔瘘（CCF）的经验及中远期随访结果。 方法： 病例对照研究。选择2005年1月至2019年12月在广东省人民医院接受经导管介入治疗的66例先天性CCF患儿作为研究对象，收集并分析其临床基线资料、手术效果、术中及术后严重不良事件、随访结果及预后等资料。根据是否发生严重不良事件将患儿进行分组，比较严重不良事件组和无严重不良事件组一般情况及CCF特征的差异。组间比较采用独立样本t检验、χ2检验或Fisher确切概率法。 结果： 66例先天性CCF的患儿中男34例、女32例，年龄3.9（1.9，6.2）岁，体重15（11，20）kg。66例CCF均为中型或大型瘘管，其中近端型瘘管55例（83%），远端型瘘管11例（17%）；瘘管起源于右冠状动脉38例（58%），起源于左冠状动脉28例（42%）；合并冠状动脉瘤（CAA）40例（61%）。66例患儿中64例患儿完成封堵治疗，5例患儿发生6例次围手术期严重不良事件。围手术期手术成功率为92%（59/64），围手术期不良事件发生率为9%（6/64），其中2例患儿术中发生了2例次严重不良事件，包括手术相关死亡1例，急性心肌梗死1例；3例患儿术后随访1 d发生了4例次严重不良事件，包括急性心肌梗死2例、三尖瓣腱索断裂1例、封堵器脱落1例。共58例患儿完成中远期随访，随访时间9.3（6.5，13.4）年。9例患儿发生10例次严重不良事件，中远期严重不良事件发生率为17%（10/58），其中5例次并发症（1例次新发CAA、1例次主动脉瓣穿孔、1例次冠状动脉血栓形成、2例次新发三尖瓣脱垂伴重度反流）以及5例次大量残余分流（瘘管再通）。围手术期及随访期间，13例患儿发生16例次严重不良事件，51例患儿未发生严重不良事件。发生严重不良事件组患儿的大型瘘管［11/13比39%（20/51），P=0.005］、巨大CAA［10/13比14%（7/51），P=0.030］所占比例均明显高于无不良事件组患儿，肺动脉平均压明显高于无不良事件组患儿［（20±9）比（16±6）mmHg，1 mmHg=0.133 kPa，t=2.02，P=0.048］。 结论： 经导管介入治疗儿童先天性CCF即刻疗效确切，中远期有效性和安全性尚可，但需严格把握CCF介入治疗适应证。.

We report on a 6-month-old infant (6 Kg/ 64 cm) with perimembranous ventricular septal defect (absent sub-aortic rim, 10 mm left ventricular entry, and 4 and 6 mm right ventricular exists) and successful retrograde closure using an 8x6 mm KONAR-MF™ VSD occluder (Lifetech, China). Immediate and 48 hours post-procedure ultrasounds showed an accurately positioned device and two jets of mild-to-moderate residual shunts. At the 2-week follow-up, the device did not change position and the shunt was stable and intra-prosthetic. The scheduled 3-month follow-up was skipped for familial reasons. The patient came back without alarming symptoms for the regular 6-month follow-up, and the device was found embolised to the left pulmonary artery. The device was retrieved surgically, and the defect was patch-closed with excellent outcomes. There was a pseudoaneurysm involving the tricuspid valve chordae and the device was endothelialized partially on one edge suggesting that embolization occurred somewhere between 3 months and 6 months post-operative. Defects with compromised anatomies should be closed surgically to avoid suboptimal results, especially in small infants.

Tricuspid regurgitation (TR) in children may be secondary to congenital anomalies of the tricuspid valve complex which is composed by annulus, leaflets, commissures, chordae tendineae, and papillary muscles. The most common congenital cause is Ebstein's anomaly; however, there are less frequent causes such as abnormal number of tricuspid leaflets, tricuspid cleft, leaflet prolapse, double orifice tricuspid valve, and congenital tricuspid valve dysplasia. Identifying the precise cause is important to plan an appropriate repair surgery. In this article, the case of a 4-year-old patient with a tetracuspid valve with significant tricuspid regurgitation is presented and the morphological analysis was made by two-dimensional (2D) and three-dimensional (3D) transthoracic echocardiography. The morphological differences between a tetracuspid valve and a cleft of the anterior leaflet tricuspid valve are exposed. 3D echocardiographic evaluation of the tricuspid valve allowed a better understanding of the tricuspid valve anatomy, which includes evaluation of the tricuspid annulus, leaflets, commissures, and subvalvular apparatus. Recognizing the accurate cause of isolated tricuspid regurgitation allows better planning of the surgical technique.