Surgery for mitral and tricuspid insufficiency associated with secundum atrial septal defect

Pure mitral insufficiency associated with secundum atrial septal defect is not an unusual finding. Thirteen patients with significant mitral insufficiency and an associated secundum defect were operated upon. Two of these patients also had severe tricuspid insufficiency. The mitral valve was repaired in 12 patients and replaced in one. The tricuspid valve was reapired in the two patients with associated tricuspid insufficiency. There were two early deaths due to mediastinitis and one late death due to a cerebral embolus in the only patient who had valve replacement; this patient died 3 years after the operation. It is concluded that mitral and tricuspid valve repair should be performed for patients with significant mitral and tricuspid insufficiency associated with atrial septal defect of the secundum variety. Replacement of the values should be avoided if possible.     

Secundum atrial septal defect associated with mitral valve cleft: Report of a case with chromosomal syndrome of trisomy 3p

A 4-year-old boy was diagnosed as having a chromosomal abnormality with trisomy 3p associated with a secundum atrial septal defect and a cleft of the anterior mitral leaflet. His craniofacial features showed microcephaly, micrognathia, ocular hypertelorism associated with epicanthus, and low-set ears. Moderate mental retardation and hypoplasia of the penis were also noted. A left ventriculogram demonstrated grade 2 mitral regurgitation according to the Sellers' classification with a prolapsing of the mitral leaflets. A cleft of the anterior mitral leaflet was detected at the time of operation. The secundum atrial septal defect was directly closed and the mitral cleft was repaired. This is the first report of trisomy 3p associated with an atrial septal defect and a mitral valve cleft.     

Mitral regurgitation with concomitant atrial septal aneurysm; report of a case

A 64-year-old female was admitted to our hospital because of severe dyspnea. Echocardiography revealed mitral valve regurgitation and atrial septal aneurysm (ASA). After instituting medical treatment for congestive heart failure, euvolemic status was achieved, and the patient underwent; (1) prosthetic patch repair for ASA; (2) mitral valvuloplasty with partial quadrangular resection of the posterior mitral leaflet; and (3) mitral annuloplasty using Physio ring. Pathological examination revealed myxomatous degeneration of the mitral valve, but the resected atrial septum was without any abnormality. ASA can lead to cerebral or pulmonary embolism even in the absence of an atrial septal defect. However, ASA without atrial septal defect is typically asymptomatic and rarely requires surgical correction. By contrast, ASA with concomitant mitral valve prolapse is associated with a high risk of cerebral or pulmonary embolism. Aspirin therapy is indicated for the prevention of thromboembolism in patients with ASA who do not undergo surgical management, and these patients also require careful observation.     

Indications for and limitations of minimally invasive cardiac surgery with the lower ministernotomy approach

The chief benefits of small skin incisions are reduced patient discomfort, accelerated recovery, and cosmetic satisfaction without compromising the quality of surgery. Since April 1997, the lower ministernotomy approach without femoral cannulation has been performed in 43 patients in the authors' institutions. The indications for this approach were initial single valve surgery and secundum-type atrial septal defect. Cases of aortic valve regurgitation that could be repaired, and aortic stenosis that necessitated annular enlargement were excluded. Among patients with mitral valve disease, those with chronic atrial fibrillation were excluded frpm undergoing the Maze procedure and those reguiring chordal reconstruction for anterior leaflet were also excluded. Mitral valve repair for mitral regurgitation was performed in 8 patients, and open mitral commissurotomy in 2. Mitral valve replacement was performed in 3 patients and aortic valve replacement in 13. Closure of an atrial septal defect was carried out in 18 cases. An approximately 10-cm median skin incision was made, and a ministernotomy with a lower semitransverse division (inverted L-shape) was carried out. Cardiopulmonary bypass was initiated with ascending aortic cannulation and right-angled venous cannulae in the superior and inferior vena cava for mitral valve disease. Single venous cannulae from the right atrial appendage was used for aortic valve disease. Surgery was performed with mild hypothermia and intermittent tepid blood cardioplegia with diltiazem. A rigid 30-degree angle scope held by a videoscope holder with a flexible arm was used for mitral valve surgery. There were one hospital death due to perioperative myocardial infarction and pulmonary embolism. There was one reopening for bleeding which resulted in methicillin-resistant Staphylococcus aureus mediastinitis. However, the patients was discharged after rectal muscle flap repair. There was one reoperation for mitral valve repair due to hemolysis. The improvement of surgical instruments and materials will further facilitate this procedure.     

The congenitally cleft posterior mitral valve leaflet. An anticedent to mitral regurgitation

Five patients with isolated congenitally cleft posterior mitral valve leaflets and 1 patient with an associated ostium secundum atrial septal defect are described. The valvular lesion is notable for the constant insertion of normal chordae tendineae into the cleft's free edge and the presence of an additional papillary muscle from which these chordae originate. The clinical presentation varies greatly, and there is no recognized clinical sign that has allowed preoperative diagnosis. The cleft predisposes the valve to significant malfunction in the presence of acquired lesions, thereby necessitating surgical therapy. The choice of procedure to restore mitral competence depends on the degree of valvular and annular disorganization and the associated defects, but the lesion lends itself to successful conservative surgical procedures. Four of the 5 patients with the isolated valve lesion have been successfully treated surgically.     

Secundum Atrial Septal Defect and Cleft Mitral Valve

The association of a secundum atrial septal defect and mitral insufficiency is not uncommon. Five patients with this combination of lesions are presented and the pathological anatomy of the mitral valve is discussed. All 5 patients demonstrated a similar cleft mitral valve; 2 had cleft valves when only mitral valve prolapse was suspected preoperatively. The surgical implication of these lesions is discussed.     

Extended vertical transatrial septal approach to the mitral valve

Optimal mitral valve operation requires adequate exposure without impairment of atrial physiology, namely sinus node and atrioventricular node function. We used an extended vertical transseptal atrial approach in 34 consecutive patients. The extended vertical transseptal approach combines two semicircular atrial incisions circumscribing the tricuspid and mitral annuli anteriorly and superiorly, allowing exposure of the mitral valve by deflecting the ventricular side using stay sutures. The right atrium is opened anteriorly along the atrioventricular sulcus. The atrial septum is incised vertically through the fossa ovalis. The right atriotomy is extended superiorly in the right coronary fossa between the right atrial appendage and the atrioventricular sulcus to meet the septal incision. The two joint incisions are extended onto the left atrial roof transversely. At this point, the two semicircular incisions are performed and joined, and mitral valve operation is performed. There were 18 women and 16 men. Five patients had ischemic mitral valve regurgitation, 18 had mitral valve prolapse, and 11 had rheumatic heart disease. The mitral valve was replaced in 17 patients and repaired in 17. There were no perioperative complications associated with the atriotomies, ie, no bleeding, no atrioventricular nodal dysfunction, and no sinus node dysfunction. The extended vertical transatrial septal approach provides good mitral valve exposure without inherent complications.     

Septum primum defect repair.

Two technical maneuvers in septum primum defect repair have resulted in improved mitral valve function and security against heart block. One maneuver, critical to restoring mitral competence, is accurate reconstruction of the leading edge of the mitral valve. A second maneuver, suturing the septal defect patch at its inferior aspect to the mitral valve near the annulus instead of to the inferior septal defect margin or to the tricuspid annulus, eliminates the hazard of atrioventricular node or bundle injury.     

Intraoperative Assessment of the Mitral Valve Following Reconstructive Procedures

A technique facilitating intraoperative assessment of the degree of mitral insufficiency during and after mitral reconstructive procedures has been developed. A multiholed left ventricular vent catheter is advanced across the aortic valve, thereby creating aortic insufficiency, filling the left ventricle with blood at aortic perfusion pressure, and approximating the leaflets of the mitral valve in the closed position. If present, mitral insufficiency can be estimated by the size of the regurgitant jet.In addition to assessing valve function following open mitral valvotomy, the method has also been helpful in managing leaks around the prosthetic valve, in assessing the closure of cleft mitral valve leaflets associated with ostium primum atrial septal defects, in confirming the completeness of closure, and in detecting obscure ventricular septal defects.     

Nonobstructing Accessory Mitral Valve Tissue and Ventricular Septal Defect

A 4-month-old boy with ventricular septal defect was found to have accessory mitral valve tissue attached to the anterior leaflet of the mitral valve. Operation was successfully performed to excise the accessory mitral tissue in the left ventricular outflow tract and close the ventricular septal defect. Most previously reported cases with accessory mitral valve tissue were associated with left ventricular outflow tract obstruction. This boy had no pressure gradient across the left ventricular outflow tract. The indications for prophylactic excision of nonobstructing accessory mitral valve tissue in a patient with other forms of congenital cardiac disease are discussed.     

Translocation of the atrial septum

Translocation of the atrial septum to the left of the mitral valve is a congenital anomaly: the author has not found a similar case previously reported in the English literature. The curious anomaly described here was associated with deformed mitral and tricuspid valves, a postero-inferior atrial septal defect, a persistent left superior vena cava, a hypoplastic aorta, and a short innominate artery. The patient lived to a remarkable age.     

先天性心脏病术中心脏瓣膜的保护和矫治

目的 总结先天性心脏病术后因瓣膜功能不全再手术的经验。方法 回顾分析先天性心脏病术后再行瓣膜手术13例患者的临床资料,其中室间隔缺损修补术后8例,部分心内膜垫缺损修补术后3例,法洛四联症和房间隔缺损修补术后各1例。第1次手术时即存在二尖瓣轻～中度关闭不全6例,主动脉瓣关闭不全1例;新出现瓣膜功能异常6例,其中2例因补片漏致三尖瓣关闭不全,2例因前叶腱索断裂致三尖瓣关闭不全,1例因残留右心室流出道狭窄继发三尖瓣关闭不全,1例因伤及主动脉瓣并发二尖瓣和三尖瓣关闭不全。13例中,行二尖瓣置换6例,三尖瓣置换2例,主动脉瓣置换1例,行主动脉瓣置换并二尖瓣、三尖瓣成形1例,三尖瓣成形3例。同时修补残余漏,疏通右心室流出道。结果 术后发生低心排综合征3例。2例术后早期分别死于脑气栓和呼吸循环衰竭。11例术后痊愈出院,随访1～8年,心功能良好。结论 先天性心脏病矫治术中应注意心脏瓣膜的保护,合并的瓣膜功能异常应积极修补,及时地再手术可取得良好效果。     

Results of surgery after failed mitral percutaneous dilatation

OBJECTIVES: Percutaneous balloon mitral valve commissurotomy (BMC) is an alternative to surgical commissurotomy. Complications following BMC includes mitral regurgitation, iatrogenic atrial septal defect, residual mitral stenosis, and pericardial hemorrhage. This study analyzes the outcomes of surgery following failed BMC for mitral stenosis. METHODS: In a series of 298 patients treated with BMC, 53 patients (17.7%) had a complication that necessitated a surgical treatment. Twenty-eight patients needed an immediate surgery before the discharge (group I) and 25 patients were operated on an elective basis (group II). RESULTS: In group I, 27 patients have been operated and one died before the operation. In 21 patients an acute mitral regurgitation occurred, 3 patients had a residual mitral stenosis, and 3 had a left atrial perforation. The operation consisted of 26 mitral valve replacements, 20 concomitant reparations of iatrogenic atrial septal defect, and one open mitral valve commissurotomy. Operative mortality was 3.7% (1 out of 27). In group II, 25 patients have been operated at a mean 18 +/- 14 months after BMC. In the 25 patients the operation was indicated for significant mitral regurgitation (2 + and more). The operation consisted of 25 mitral valve replacements, 9 concomitant reparations of iatrogenic atrial septal defect, 3 patients had also coronary artery bypasses. The operative mortality was 8% (2 out of 25). The echocardiographic score was similar for both groups, it was 8.4 +/- 2.0 in group I and 8.0 +/- 1.5 in group II (P = NS). Despite these complications following failed BMC, surgery appears a safe procedure with an acceptable mortality.     

Diagnosis and management of the double orifice mitral valve: three case reports

Double orifice mitral valve (DOMV) is an uncommon congenital heart defect. The isolated occurrence of this anomaly is very rare and, more often, is associated with another congenital malformation, dominated by atrioventricular canal defects (AVCD). Mitral insufficiency and/or stenosis may complicate this malformation. Treatment may be summarized as abstention, surgical valve repair, or valve replacement. In here, we report three cases with DOMV. The first patient was an 18-month-old boy who was operated for severe mitral valve stenosis and mild insufficiency (in 1980s), the second was a 47-year-old female, who was operated recently for mitral valve replacement (MVR) under cardiopulmonary bypass due to severe mitral valve insufficiency. This patient had been operated due to the secundum-type atrial septal defect in our institution previously. The last one, a 3-year-old boy, with DOMV and three papillary muscles, was on clinical follow-up because he had no symptoms.     

Absence of posterior mitral leaflet with secundum atrial septal defect

A rare case of a 54-year-old woman with absence of congenital posterior mitral leaflet, moderate mitral insufficiency, and large secundum-type atrial septal defect is reported. Two-dimensional color Doppler and transesophageal echocardiography revealed complete absence of the posterior mitral leaflet, a thick muscular formation replacing the posterior leaflet, a 3.3-cm secundum type atrial septal defect, and severe pulmonary hypertension. This report describes the rare case of congenital absence of posterior mitral leaflet associated with secundum type large atrial septal defect in a middle-age woman.     

Left atrial myxoma

8 patients with atrial myxoma were reviewed. They ranged from 5 years to 55 years. Recently echocardiography has been the most helpful diagnostic procedure. Hemodynamically they simulated mitral valve disease with severe pulmonary hypertension. Tumour was demonstrated by angiocardiography. One of these tumours presented into the right atrium through an associated atrial septal defect. All of them underwent open heart surgery and removal of the tumour. One died as a result of septicemia. Others have had excellent hemodynamic improvement.     

"缘对缘"瓣膜成形术在先天性心脏病合并重度三尖瓣关闭不全中的应用

目的 总结运用"缘对缘"成形技术治疗先天性心脏病病人的重度三尖瓣关闭不全的效果.方法 2001年4月至2010年3月,对14例先大性心脏病合并重度三尖瓣关闭不全病人采用常规三尖瓣瓣环成形和"缘对缘"技术行三尖瓣成形.年龄7～62岁,平均(31.2±16.1)岁.先大性心脏畸形包括继发孔房间隔缺损6例,房室管畸形5例,继发孔房间隔缺损合并二尖瓣关闭不全2例,三房心1例.结果 14例出院时均无不适,无住院死亡及术后并发症.术后超声心动图检查示三尖瓣关闭不全无或微量11例,轻度3例.随访3～97个月,平均(51.6±26.8)个月.随访时超声心动图检查示均无三尖瓣狭窄,三尖瓣关闭不全无或微量5例,轻度8例,中度1例.结论 "缘对缘"成形技术纠治先天性心脏病合并重度三尖瓣关闭不全简单、有效.     

成人先天性心脏病合并冠心病的外科治疗

目的探讨年龄>40岁的先天性心脏病合并冠心病手术治疗的安全性和有效性。方法 2002年2月～2009年5月,26例先天性心脏病(房间隔缺损18例,室间隔缺损4例,房室管畸形3例,三心房1例)合并冠心病(冠状动脉造影显示:单支病变10例,双支病变9例,三支病变7例)接受心脏畸形矫治联合冠状动脉搭桥手术,22例体外循环下行心脏畸形矫治和冠状动脉搭桥术,4例食管超声引导下行房间隔缺损术中伞堵(intraoperative device closure,IODC)及非体外循环冠状动脉旁路移植术(off-pump coronary artery bypass,OPCAB)。冠状动脉旁路移植共完成46处远端吻合,同期行二尖瓣置换术2例,二尖瓣成形术3例,三尖瓣成形术5例,房颤射频消融术2例。结果 1例房室管畸形因肺部感染和多器官衰竭死亡,其余25例康复出院,无手术并发症。术后随访17～105个月,(57.6±24.7)月:1例术后18个月再发心绞痛,未接受再次医疗干预;术后患者心功能和肺动脉高压情况改善;4例杂交手术随访中未发现残余分流、血栓和封堵装置移位等并发症。结论外科治疗成人先天性心脏病合并冠心病效果良好。IODC联合OPCAB治疗房间隔缺损安全、有效。     

Complicated unroofed coronary sinus syndrome

A young boy planned for the surgical closure of atrial septal defect (ASD) and mitral valve regurgitation (MR) was found peroperatively as having a complete unroofed coronary sinus (URCS). Intracardiac re-routing of left superior vena cava (LSVC) and mitral valve replacement (MVR) were performed concomitantly with success.     

Living donor liver transplantation in children with congenital heart disease

BACKGROUND: The occurrence of congenital heart disease (CHD) with congenital biliary disease is uncommon. Our aim is to present our experience in living donor liver transplantation (LDLT) as treatment for end-stage liver disease (ESLD) in children with CHD. METHODS: A review of transplant records from June 1994 to December 2004 was performed. Twenty-three LDLT (13 males, 10 females) recipients were diagnosed to have both CHD and ESLD. RESULTS: CHD diagnoses were made preoperatively using transthoracic two-dimensional color flow Doppler echocardiography. The mean age was 22.3 months. There were 20 (87%) biliary atresia, two (9%) neonatal hepatitis, and one (4%) glycogen storage disease patients. Isolated CHD associated with ESLD included atrial septal defect (11, 48%), pulmonary stenosis (including 2 Alagille syndrome; 4, 17%), patent foramen ovale (4, 17%), ventricular septal defect (1, 4%), and mitral valve prolapse (1, 4%). Complex CHD included atrial septal defect + patent ductus arteriosus + patent foramen ovale (1, 4%), and atrial septal defect + pulmonary stenosis (1, 4%). The median Child's and Pediatric End-stage Liver Disease scores were 9, and 17, respectively. In all, 70% presented with varying degrees of pulmonary congestion pretransplant. There were no perioperative cardiac complications. Posttransplant, the patent foramen ovale in four recipients and atrial septal defect in four recipients closed spontaneously; and two recipients with pulmonary stenosis had their stenoses resolved spontaneously. The overall rejection rate was 17%. There was no mortality. The overall recipient and graft survivals at 1 and 5 years were both 100%. CONCLUSION: LDLT is a safe procedure in a select group of ESLD patients with CHD.