Treatment of absent pulmonary valve syndrome with homograft

We report the successful treatment of absent pulmonary valve syndrome in an infant. The treatment consisted of intracardiac repair of pulmonary regurgitation with a homograft valve, which allowed for early extubation and survival.     

Stentless bioprosthetic aortic valve replacement after a homograft root replacement: Toronto SPV implantation after a homograft root

Stentless bioprosthetic valves for the aortic position offer excellent hemodynamic characteristics, making them an attractive choice ahead of other valve prostheses. We present a unique case in which a patient underwent aortic valve replacement with a stentless porcine valve and mitral valve repair for severe aortic and mitral regurgitation 1 year after a homograft root replacement for acute aortic endocarditis. The rationale for our approach is outlined in the context of current surgical trends.     

Surgical approach to severely symptomatic newborn infants with tetralogy of Fallot and absent pulmonary valve

The surgical management of severely symptomatic newborn infants with tetralogy of Fallot and absent pulmonary valve has been controversial, and the results of a variety of operative approaches have not been satisfactory. We report on a technique for the treatment of these patients, which consists of (1) ligation of the main pulmonary artery to eliminate pulmonary regurgitation, excessive right ventricular stroke output, and secondary pulmonary artery dilation and airway obstruction and (2) insertion of a subclavian-pulmonary artery polytetrafluoroethylene shunt to provide pulmonary blood flow. The procedure was used in four neonates. Two patients operated on at 2 and 3 days of age are doing well 15 and 19 months postoperatively. The other two, operated on at 3 and 4 weeks of age after unsuccessful prolonged medical treatment and positive-pressure ventilation, failed to show long-term improvement and died of sepsis and respiratory failure 3 and 5 months after operation. This experience, though limited, suggests that early surgical intervention to control pulmonary regurgitation prevents progressive pulmonary artery dilatation and secondary bronchial compression, decreases the need for prolonged preoperative and postoperative ventilation, and improves the outcome of these critically ill neonates.     

Mitral valve replacement with stored inverted pulmonary homograft valve

The fate is described of 11 patients who had a stored pulmonary valve homograft mounted on a frame or in a Dacron jacket used for mitral valve replacement. Mitral regurgitation due to holes in the attenuated cusps occurred in nine by one year, requiring re-operation or causing death. Only one patient remains well with moderate mitral regurgitation after two years.     

Tricuspid valve replacement using an unstented pulmonary homograft

In consideration of the limitations of mechanical and stent-mounted tissue valves, a technique was developed for tricuspid valve replacement using an inverted pulmonary homograft. It was used successfully in a 4-year-old patient operated on for pulmonary atresia with intact ventricular septum and in a 3 1/2-year-old girl with Ebstein's anomaly.     

A modified technique for pulmonary valve replacement with a homograft

We present a modified technique for replacement of the pulmonary valve with a pulmonary homograft. The principles of this technique are similar to the "scalloped subcoronary technique" for aortic valve replacement with a homograft. The advantages of using this technique in the pulmonary position are briefly discussed.     

肺动脉瓣缺如综合征外科治疗

目的 回顾性研究肺动脉瓣缺如综合征围手术期处理及手术操作技术,探讨减轻术后呼吸系统并发症的综合措施.方法 2005年1月至2012年1月,11例肺动脉瓣缺如综合征患儿接受体外循环下畸形矫治手术,其中男7例,女4例;年龄1～5岁;体质量10.2 ～ 17.5 kg,平均(12.3±3.4)kg.手术选择右心室流出道切口,应用经戊二醛处理的自体心包补片修补室间隔缺损;修剪主肺动脉及左、右肺动脉前壁冗余组织至双侧肺门,缝合重塑肺动脉至正常内径;应用0.1 mm Gore-Tex心包膜在右心室流出道切口处制备人工肺动脉单瓣后,应用自体心包行跨瓣环补片,扩大右心室流出道及肺动脉瓣环.对于支气管压迫严重的患儿,采用Lecompte技术将肺动脉移至升主动脉前方.术中及术后早期行支气管纤维镜检查,判断气管压迫的位置、程度以及是否存在气管发育不良,并充分灌洗气道,将其中积聚的分泌物吸出.5例术中及术后应用纤维支气管镜探查支气管并清理气道分泌物,5例采用Lecompte技术以减轻肺动脉对于左、右支气管的压迫,4例应用深低温停循环技术.术后随访3～ 87个月,中位数为39个月,分别于出院前、术后3个月和之后每年1次常规心脏超声随访检查.结果 全组无死亡.随访未见心脏残余畸形.2例患儿术后1年内反复出现肺部感染,之后改善;行Lecompte调转的患儿支气管无肺动脉压迫性狭窄.所有患儿无气道压迫症状.结论 对于肺动脉瓣缺如综合征,理想的肺动脉重建、减轻肺动脉对同侧支气管的压迫及应用纤维支气管镜辅助检查及治疗是获得理想疗效的基本保障措施.但是由于分支肺动脉的瘤样病变可能会对伴行细支气管造成压迫,因此患儿术后仍可能面临较长时间的内科治疗,其远期疗效尚待进一步研究.     

Surgical treatment of absent pulmonary valve syndrome

The absent pulmonary valve syndrome includes agenesis of the pulmonary valve, annular stenosis, and pulmonary insufficiency. The pulmonary arteries are aneurysmal and usually compress the tracheobronchial tree. These features are associated with a ventricular septal defect and right ventricular hypertrophy. Nineteen children aged 5 days to 11 years were treated between 1976 and 1983. Nine were younger than 1 year. Intractable respiratory symptoms and heart failure were the main features in the infant group; five required preoperative assisted ventilation. Older children had decreased exercise tolerance and repeated respiratory infections. Several surgical techniques were used in this series. The best results were achieved when the size of the main, right, and left pulmonary arteries was reduced by extensive anterior resection; the ventricular septal defect was closed; and an aortic homograft was interposed between the right ventricle and the pulmonary artery. Four infants and four children treated in this way survived the operation. Five of the nine operated infants died (56%); all older children survived the operation. At follow-up, six children were asymptomatic and one was in New York Heart Association Class II. All four infants were progressing satisfactorily although all had some residual pulmonary symptoms. Closure of the ventricular septal defect, reduction of the size of the pulmonary arteries, and insertion of an aortic homograft was the optimal treatment in our series.     

肺动脉瓣缺如综合征的外科治疗

目的总结肺动脉瓣缺如综合征的外科治疗经验。方法1985年1月至2003年9月，纠治肺动脉瓣缺如综合征18例中男13例，女5例。伴法洛四联症17例，伴室间隔缺损1例。全组均在中低温体外循环下行纠治手术，即切除、折叠整形扩张的肺动脉；解除右心室流出道梗阻同时关闭室间隔缺损。7例肺动脉瓣处置单瓣。结果术后死亡1例，死亡率5．6％。术后并发低心排血量综合征3例，Ⅲ度房室传导阻滞和心包积液各1例，经治疗均痊愈。术后随访0．5—5．0年，轻度残余右室流出道梗阻1例、轻度肺动脉瓣反流7例、中度肺动脉瓣反流3例、重度肺动脉瓣反流伴充血性右心衰1例。结论婴幼儿型肺动脉瓣缺如需立即手术，在解除右室流出道梗阻和肺动脉做切除、折叠整形时，须在肺动脉瓣区置入单瓣或带瓣管道。儿童型肺动脉瓣缺如选择性根治，处理原则同普通伴发畸形。     

Tricuspid valve replacement with a mitral homograft in children with rheumatic tricuspid valvulopathy

OBJECTIVE: Tricuspid valve replacement in children is associated with a nonnegligible complication rate because of specific disadvantages of mechanical or biologic prostheses. The objective of this study was to examine the midterm clinical outcomes of tricuspid valve replacement with a mitral homograft in 8 children with unreparable rheumatic tricuspid valve involvement. METHODS: Between 1993 and 2003, tricuspid valve replacement with a mitral homograft was performed in 8 patients (2 male and 6 female patients; mean age, 14.2 years) with rheumatic tricuspid valve disease. All patients were in New York Heart Association class III or IV. In all patients with rheumatic valve disease, conservative operations had previously been performed on the tricuspid valve during concomitant left-sided surgical intervention. Mean follow-up was 56 +/- 12 months and was complete. RESULTS: There were no operative or late deaths. All patients were alive at the most recent follow-up contact and were in New York Heart Association functional class I or II. None of the patients required homograft-related reoperation. At the most recent echocardiograhic examination, 6 patients had trivial residual tricuspid regurgitation, and 2 had mild tricuspid regurgitation. None of the patients had maximal transvalvular tricuspid gradients greater than 2 mm Hg during their yearly follow-up visits. CONCLUSION: On the basis of our midterm results, tricuspid valve replacement with a mitral homograft in children seems to be a valuable alternative surgical option.     

Mitral valve replacement for mitral stenosis secondary to Hunter's syndrome

Hunter's syndrome is a rare, X-linked recessive, mucopolysaccharidosis. Survival into adulthood is uncommon. Mitral valve disease, predominantly regurgitation, has been reported in these patients. We have found no reports of mitral valve replacement for mitral stenosis secondary to Hunter's syndrome in the English literature. We report that mitral valve replacement for this pathology is a viable treatment option in an adult patient; however, specific precautions must be considered.     

Successful aortic valve replacement for Heyde syndrome with confirmed hematologic recovery

Aortic valve stenosis can be complicated by recurrent gastrointestinal bleeding, particularly that due to angiodysplasia, also called Heyde syndrome. Recently, acquired type 2A von Willebrand disease, which is characterized by the loss of the large multimer of von Willebrand factor by the shear stress of aortic valve stenosis, was reported to be associated with this hemorrhagic syndrome. A 78-year-old woman, with severe aortic stenosis, presented with advanced anemia due to recurrent gastrointestinal bleeding and was diagnosed Heyde syndrome. By perioperative supplementation of von Willebrand factor and factor VIII, aortic valve replacement was safely performed without gastrointestinal bleeding. After the operation, the multimer of von Willebrand factor was normalized and thereafter no gastrointestinal bleeding occurred. This case reports the successful aortic valve replacement for Heyde syndrome, with confirmed hematologic recovery.