Quadricuspid Aortic Valve with Sinus of Valsalva Rupture

A 22‐year‐old female with no medical history presented to the emergency room with 2 weeks of rapidly worsening dyspnea on exertion, orthopnea, and cough. On cardiac auscultation, she was noted to have to‐and‐fro murmurs and a continuous murmur with signs of right heart failure. Echocardiographic images obtained showed moderate to severe aortic regurgitation, severe tricuspid regurgitation, and a “windsock” originating in the right coronary sinus of Valsalva and terminating in the right atrium. The aortic valve had four leaflets, with the right leaflet function compromised by the ruptured sinus, causing aortic regurgitation. The patient underwent resection of the sinus aneurysm and aortic valve replacement with a bioprosthetic valve. Quadricuspid aortic valves are uncommon and are rarely associated with sinus of Valsalva aneurysm. The prevalence in the general population, clinical progression, and prognosis of this unique congenital abnormality are reviewed.     

Interventional Therapy of Supravalvular Pulmonary Stenosis via a Mechanical Valve in the Pulmonary Position

There is an increasing number of patients with congenital heart disease and pathology of the right ventricular outflow tract in whom a mechanical pulmonary valve replacement is chosen for permanent palliation. Despite corrective surgery, some of these patients may have residual or secondary supravalvular pulmonary stenosis or peripheral pulmonary stenosis, which necessitate interventional therapy after valve replacement. There is a general understanding that interventional therapy via a mechanical valve in pulmonary position may induce mechanical valve dysfunction and should therefore be avoided. We report our experience in three patients with a St. Jude Medical mechanical valve in pulmonary position and supravalvular pulmonary stenosis or a peripheral pulmonary stenosis where we have safely performed standard interventions (i.e., balloon angioplasty and stent implantation) across the mechanical valve without any complications. Our specific technique using a long sheath as safety guard, which holds the mechanical valve open during the procedure but allows the positioning of all mechanical devices and catheters necessary for the procedures, is described. In all patients, the long‐term follow‐up of the valve function is excellent.     

Transcatheter Aortic Valve Implantation Complicated By Acute Structural Valve Failure Requiring Immediate Valve In Valve Implantation

The first percutaneous transcatheter aortic valve implantation (TAVI) was performed in 2002 by Alain Cribier with over 10,000 valve implants since. Despite this, as with all new technologies we remain on a learning curve and continue to encounter new challenges and complications. We report a case of acute structural valve failure treated successfully with a second valve in valve implantation of transcatheter aortic valve in a patient who had severe aortic stenosis (AS) complicated by a severely unfolded aorta.     

Truncal Valve Repair in Neonates Using Pericardial Leaflet Extension

Truncal valve insufficiency is a significant risk factor for post‐operative mortality following repair of truncus arteriosus. The surgical management of dysplastic and insufficient truncal valves remains an operative challenge. We report the cases of two infants with type 2 truncus arteriosus and severely dysplastic and insufficient quadricuspid truncal valves. At primary repair, their truncal valves were successfully repaired using pericardial leaflet extensions. This technique may be used in neonates with truncal valve insufficiency as part of the primary repair of truncus arteriosus.     

青少年心脏瓣膜退行性变

目的：青少年心脏瓣膜退行性变是一种独特的疾病。研究它的发病机制、临床特点和病理变化有重要意义。方法：１９８３年１月至１９９３年１２月，对５３５例心脏瓣膜病患者行心脏瓣膜替换术。结果：２３例１９岁以下患者经手术和病理证实为瓣膜粘液样退行性变，占同期瓣膜替换手术病例的４．２９％。结论：青少年心脏瓣膜退行性变是一组临床特点鲜明、病理改变独特的疾病，其发病机制尚不明确，心脏胶原纤维代谢异常可能是原发缺陷。手术治疗方法仍在探索     

Rare Mycotic Aneurysm of the Mitral Valve without Aortic Valve Involvement

Mycotic aneurysms can be a rare, but serious complication of infectious endocarditis. We report the case of a 20‐year‐old woman who presented with fever and malaise from streptococcal bacteremia and found to have vegetation on the anterior leaflet of the mitral valve. On follow‐up visit, the patient was noted to have a mycotic aneurysm of the anterior mitral valve without aortic involvement. Her clinical course was complicated by mitral valve chordal rupture, severe mitral regurgitation, and dyspnea from severe mitral regurgitation for which she underwent successful surgical repair of the mitral valve.     

Pulmonary Regurgitation after Tetralogy of Fallot Repair: Clinical Features,Sequelae, and Timing of Pulmonary Valve Replacement

Pulmonary regurgitation following repair of tetralogy of Fallot is a common postoperative sequela associated with progressive right ventricular enlargement, dysfunction, and is an important determinant of late morbidity and mortality. Although pulmonary regurgitation may be well tolerated for many years following surgery, it can be associated with progressive exercise intolerance, heart failure, tachyarrhythmia, and late sudden death. It also often necessitates re‐intervention. Identifying the appropriate timing of such intervention could be very challenging given the risk of prosthetic valve degeneration and the increased risk of reoperation. Comprehensive informed and regular assessment of the postoperative patient with tetralogy of Fallot, including evaluation of pulmonary regurgitation, right heart structure and function, is crucial to the optimal care of these patients. Pulmonary valve replacement performed in an experienced tertiary referral center is associated with low operative morbidity and mortality and very good long‐term results. Early results of percutaneous pulmonary valve replacement are also promising.     

Outcome of Pulmonary Valve Replacements in Adults after Tetralogy Repair: A Multi‐institutional Study

Objective. The purpose of this study was to assess the outcome of pulmonary valve replacement (PVR) in adults with moderate/severe pulmonary regurgitation after tetralogy repair, with particular emphasis on patient outcome, durability of valve repair, and improvement in symptomatology. Design/Setting/Patients. The project committee of the International Society of Congenital Heart Disease undertook a retrospective multi‐institutional analysis of PVR. Seven centers participated in submitting data on 93 patients >18 years of age who had the operation performed and follow‐up obtained. The average age of PVR was 26± years (median 27 years). Time of follow‐up after replacement was 3 years (range 4 days–28 years). Outcomes/Measures/Results. Kaplan–Meier estimates of durability of PVR showed approximately 50% replacement at 11 years. There were two deaths at 6 and 12 months after valve replacement. Right ventricular (RV) size estimated by echocardiography from pre‐ to postoperative studies decreased in 81% (P < 0.001 testing for equal proportions), but RV systolic function increased in only 36% (P = 0.09). Ability index improved in 59% (P < 0.001) and clinical heart failure status improved in 57% with this problem before PVR. PVR did not improve arrhythmia status in a small group of patients. Conclusions. PVR is associated with low mortality, decrease in RV size and improvement in ability index, and uncertain effects on RV systolic function. Average valve durability was approximately 11 years. Criteria for PVR that will preserve RV function are not clearly identified, and management of these patients remains a difficult enterprise.     

Ebstein's Malformation of the Tricuspid Valve: Short‐term Outcomes of the “Cone Procedure” versus Conventional Surgery

Objectives. We report our analysis of conventional surgery and the cone procedure for Ebstein's malformation (EM) of the tricuspid valve at a single institution. Previous conventional surgery for EM, including use of bioprosthetic valves, has inherent problems especially in pediatrics. The newer cone procedure aims to construct a funnel‐like valve out of native leaflets, obviating problems with artificial valves. Methods. This is a retrospective cohort study to examine short‐term outcomes of both surgeries for EM. Results. Nineteen patients (our initial cohort) had the cone procedure, and 13 had conventional tricuspid valve repair or replacement. No early deaths occurred in either group. Three cone and one conventional repair patients required reoperation. Two of 19 patients in the cone and one of 13 in the conventional group died suddenly >30 days after operation, assumed secondary to dysrhythmias. At discharge, by two‐dimensional echocardiography, the cone group had 85% reduction in tricuspid valve regurgitation (TVR), and the conventional group had 56% reduction, P= .004. This decrease of TVR persisted to a greater extent in the cone group. Discussion. Short‐term results for the cone procedure are similar to conventional surgery. The cone procedure uses autologous tissue; hypothetically, early favorable improvement in reduction of TVR should persist.     

Three‐Dimensional Transesophageal Echocardiography Assessment of a Successful Transcatheter Mitral Valve in Valve Implantation for Degenerated Bioprosthesis

Reoperation for degenerated mitral bioprosthesis is considered a high risk procedure. Transcatheter mitral valve in valve implantation has emerged as an off‐label alternative for patients contra‐indicated to surgery. We report a 46‐year‐old man, with a 29 mm mitral bioprosthesis since 2002, who was admitted for acute heart failure because of a severe intra‐prosthetic regurgitation. His recent medical history revealed a fast growing cavum carcinoma. In view of generally poor prognosis, the heart team decided to perform a transcatheter mitral valve in valve implantation by transapical approach. Live three‐dimensional TEE was used during the implantation for sizing, device positioning, and hemodynamic assessment.     

心脏瓣膜病巨大左心室病人的瓣膜置换

目的：探讨瓣膜病巨大左心室病人的临床特点，及影响手术疗效的主要因素，提高瓣膜置换术后的疗效。方法：共47例瓣膜病巨大左心室病人行瓣膜置换术，其中主动脉与二尖瓣双瓣置换35例，二尖瓣置换5例，主动脉置换7例，同时行三尖瓣成形42例，左房折叠4例。结果：术后早期并发症14例(349／6)，死亡2例(4．259／6)，影响瓣膜置换手术早期疗效的主要因素是严重左室扩大，严重左室收缩功能下降，射血分数(EF)＜0．40，左室短轴缩短率(FS)＜0．25和严重低心输出量综合征，和围术期室颤。结论：掌握合适手术时机，注意心肌保护措施，最大限度地保留心内结构是巨大左心室病人瓣膜置换手术成功的重要因素。     

Tricuspid Valve Replacement through a Left Atriotomy and Transseptal Approach in a Congenitally Malformed Heart

More and more children with congenital heart disease are surviving into adulthood. These patients are forcing adult congenital cardiac surgeons to develop innovative approaches to correct their complex anatomy and physiology. This report describes a patient with a congenitally malformed heart necessitating a novel approach to access the tricuspid valve—a left atriotomy and transseptal incision. Three‐dimensional preoperative imaging allowed for successful surgical planning.     

风湿性心脏病合并冠心病的同期外科治疗

目的 探讨风湿性心脏病合并冠心病的同期外科治疗,提高手术效果.方法 回顾性分析9例患者同期施行冠状动脉旁路移植术和心脏瓣膜手术的临床资料及远期随访资料,其中二尖瓣置换6例,二尖瓣成形2例,主动脉置换1例.合并冠状动脉单支病变中7例用左乳内动脉做血管桥,二支病变中1例用左乳内动脉加大隐静脉做血管桥,1例用左乳内动脉加左桡动脉做血管桥.结果 术后平均呼吸机辅助时间19 h,平均重症监护室监护时间2.6天,出院前行超声检查心功能,射血分数上升0%～20%.发生呼吸功能不全3例,严重心律失常3例,出血再次开胸1例,经治疗均好转.心绞痛不同程度消失,无围手术期死亡,远期随访心功能明显改善.结论 积极、正确的围手术期处理, 改善心功能,尽量缩短主动脉阻断时间,术中心肌保护良好,是提高瓣膜病合并冠心病患者手术成功率、降低死亡率、减少并发症的关键.     

Isolated Cleft Mitral Valve with Posterior and Anterior Clefts: a Rare Cause of Congenital Valve Regurgitation

We report a case of isolated cleft mitral valve with two clefts in the posterior and one in the anterior leaflet. Our case adds to the few reports of posterior and multiple mitral valve clefts and to our knowledge is the first using real‐time transoesophageal three‐dimensional echocardiography (3DE) for assessment of isolated cleft mitral valve. (Echocardiography 2010;27:E50‐E52)     

降低人造瓣膜置换术后抗凝相关并发症的策略

机械瓣膜血栓形成、栓塞及抗凝相关出血是瓣膜置换术后最常见、最严重的并发症,严重影响病人生存及生活质量。近年来,通过合理抗凝监测,降低抗凝强度,改进人造瓣膜设计、工艺以及建立个体化用药方案等综合措施,抗凝相关并发症有了明显降低。然而,最理想的方法是研究发明一种勿需抗凝或仅需短期抗凝的人造瓣膜,因此,组织工程瓣膜和基因工程瓣膜的研究发明与应用,将可能从根本上改变人造瓣膜置换术后抗凝治疗的现状。     

心内直视手术后三尖瓣置换临床研究

目的 :探讨心内直视手术后三尖瓣发生严重病变的原因及二次手术时三尖瓣置换的方法。　　方法 :在全麻、体外循环下完成 8例心内直视术后因严重三尖瓣病变所致三尖瓣置换术。对 7例术后存活者进行随访。　　结果 :手术死亡 1例。 7例术后存活者随访中 (0 5～ 5年 )无死亡及瓣膜相关并发症发生。　　结论 :三尖瓣置换是心内直视手术后严重三尖瓣病变有效的治疗方法     

An Incidental Finding of Mitral Valve Network

A 63‐year‐old male presented with a 6‐month history of worsening exertional dyspnea and was found to have three‐vessel coronary artery disease. Transesophageal echocardiography revealed a filamentous structure attached to the anterior mitral valve leaflet, which was confirmed during surgery as filamentous network. To our knowledge, this is the first report to describe such a network attached to the mitral valve. (Echocardiography 2010;27:E87‐E89)     

心脏瓣膜病伴发冠心病的流行病学研究

目的探讨心脏瓣膜病(HVD)患者术前行冠脉造影检查的指征。方法回顾性分析我院40岁以上因心脏瓣膜病拟行瓣膜手术的868例患者术前冠脉造影检查资料。结果男性伴发冠心病高于女性,发病的年龄较女性低,在选择瓣膜病患者进行冠脉造影检查时,应充分考虑是否并发有冠心病发病危险因素,不同部位瓣膜病变的冠心病发生率相仿。结论50岁以上尤其是男性心脏瓣膜病患者易合并冠心病,或冠心病引起瓣膜病变,尤其是左房室瓣关闭不全;故50岁以上瓣膜手术的患者术前应常规行冠脉造影;40～50岁心脏瓣膜病患者虽不需常规行冠脉造影,但如具备多个冠心病危险因素(如高血压、糖尿病、严重肥胖、吸烟、家属病史等),或者术前有明显冠心病症状,条件允许也建议行冠脉造影排除冠心病。     

Heart Valve Disease Predict Mortality in Hemodialysis Patients: A Single Center Experience

Our aim is to investigate the clinical and laboratory findings affecting the mortality of the patients in 3 years follow‐up who underwent hemodialysis at our center. In this retrospective, observational cohort study, 432 patients who underwent hemodialysis at our center for at least 5 months were included. The first recorded data and subsequent clinical findings of patients who died and survived were compared. Two hundred and ninety patients survived, 142 patients died. The mean age of the patients who died was higher (63.4 ± 12.3 years, vs. 52 ± 16.1 years, P = 0.0001), 60.5% of them had coronary artery disease (P = 0.0001), 93.7% of them had a heart valve disease. Duration of hemodialysis (survived 57 [21–260] months; died 44 [5–183] months, P = 0.000) was lower in patients who died. Serum potassium level before dialysis (5.1 ± 0.6; 4.9 ± 0.7 mEq/L, P = 0.030), parathyroid hormone (435 [4–3054]; 304 [1–3145] pg/mL, P = 0.0001), albumin (3.9 ± 0.4; 3.8 ± 0.4 mg/dL, P = 0.0001) and Kt/V (1.48 ± 0.3; 1.40 ± 0.3, P = 0.019) levels were lower, C‐reactive protein (5[1–208]; 8.7[2–256] mg/L, P = 0.000) levels were higher in patients who died. Logistic regression analysis showed age (OR = 1.1), coronary artery disease (OR = 1.7) and more than one heart valve disease (OR = 2.4) are independent risk factors for mortality. Potassium level before dialysis (OR = 0.60), parathyroid hormone (OR = 0.99), and higher Kt/V (OR = 0.28) were found to be an advantage for survival. Age, coronary artery disease and especially pathology in more than one heart valve are risk factors for mortality. Heart valve problems might develop because of malnutrition and inflammation caused by the chronic renal failure.     

Perimount® Bovine Pericardial Valve to Restore Pulmonary Valve Competence Late after Right Ventricular Outflow Tract Repair

Objective. No ideal option exists for restoring pulmonary valve competence late after repair of the congenitally abnormal right ventricular outflow tract (RVOT). This has driven a continued search for new alternatives. Texas Children’s Hospital has recently used the Carpentier‐Edwards Perimount RSR Pericardial Aortic Prosthesis (Edwards Lifesciences, Irvine, Calif, USA) for this indication and reports the initial experience. Design. Retrospective chart review. Setting. Academically affiliated tertiary‐care pediatric hospital. Patients. Twenty‐six patients who underwent pulmonary valve replacement with the Perimount^® valve late after RVOT reconstruction between June 2002 and November 2005. Interventions. No prospective interventions. Outcomes Measures. Hospital morbidity and mortality. Valve function assessed by follow‐up visits and echocardiograms. Results. Mean age and weight of the patients were 20.3 ± 9.8 years (range 7.0–45.1 years) and 56.2 ± 18.1 kg (range 35.8–109 kg). Twenty‐two patients (85%) had severe pulmonary insufficiency (PI), 23 (89%) had symptomatic right heart failure, and 14 (54%) had moderate to severe right ventricular dysfunction. Average prosthetic valve size was 23 mm (range 19–27 mm). Twenty‐one (88%) patients were extubated within 24 hours. There was no hospital mortality. Median length of stay for all patients from day of surgery was 6 days (range 3–56 days). Median length of last echocardiography follow‐up was 12.4 months (range 0.1–37.6 months). At that time, 16 of the 26 (62%) patients had improved right ventricular function, no patient demonstrated significant RVOT obstruction, and 24 patients (92%) have no PI or mild PI. Freedom from death, reintervention, or reoperation on the pulmonary valve is 100% at 2.5 years. Conclusion. Initial results with the Perimount^® bovine pericardial tissue prosthesis for pulmonary valve replacement are encouraging. Further follow‐up is required to define long‐term function and durability.