法洛四联症根治手术长期疗效观察

法洛四联症是最常见的紫绀型先天性心脏病,其解剖学基础是心室间交通,主动脉骑跨,肺动脉瓣下狭窄,以及由此导致的右心室肥厚。尽管法洛四联症根治术后患者长期生存率很高,但晚期生存患者需要面对严重并发症。进行性肺动脉反流导致的右心衰竭和心律失常是其晚期主要并发症和死亡原因。为此,我们对晚期并发症的发生和治疗的进展进行了综述,强调长期随访的重要性。     

Twenty-to-thirty-seven-year follow-up after repair for Tetralogy of Fallot.

OBJECTIVE: To describe the long-term prognosis after repair of Tetralogy of Fallot with pulmonary stenosis beyond 20 years. METHODS: One hundred and eighty five patients underwent corrective repair of Tetralogy of Fallot at Rigshospitalet in Copenhagen between January 1960 and July 1977. Ninety seven patients had undergone a palliative operation prior to Tetralogy of Fallot repair. All the 125 patients who were discharged from the hospital were traced through the population register and the patients alive July 1997 were contacted by mail and/or telephone and questioned about use of medicine, professional status, family status and ability to perform sport activities. RESULTS: Sixty patients died in hospital and 125 patients, 78 males and 47 females, were discharged alive. Among operative survivors, median age at operation was 12.8 years (range 0.4-41 years). Thirteen patients required a reoperation, the main indication was failed VSD closure. There were 16 late cardiac deaths, out of which seven were sudden and unexpected and three were in immediate relation to reoperations. One hundred and nine patients were alive at follow-up. The mean follow-up time was 25.5 years (range 20-38 years). Sixteen percent used cardiac drugs, 89% were, or had been, working normally (all professions from academics to hard manual labors were represented), 53% (64% of women) had given birth after the repair and 51% performed sport activities regularly. No patients were lost to follow-up. CONCLUSIONS: The vast majority of the patients seemed to live normal lives 20-37 years after Tetralogy of Fallot repair. Late deaths were cardiac in origin, including sudden death from arrhythmias. The number of late reoperation has been low. Considering the natural history of the disease, Fallot repair has proven to be a beneficial procedure even including the very early experience short after introduction of open heart surgery.     

Pitfalls of multi slice computed tomography

Multi slice computed tomography is increasingly being used as a diagnostic modality in congenital heart disease in place of angiography. We report a six year old child with Tetralogy of Fallot in whom multi slice computed tomography (CT) gave incomplete information. The child subsequently needed angiography and a change in the operative plan.     

法乐四联症心肌纤维扫描电镜的观察

为选择法乐四联症手术的适宜年龄，应用扫描电子显微镜对３０例法乐四联症病人的右室流出道心肌纤维进行观察，结果可见多数心肌纤维排列紊乱、增生、肥大；肌纤维表面横纹的宽度粗细不均，有的异常增宽、增高。细胞表面凹凸不平，在肌膜下可见异常大量堆积的线粒体。心肌纤维内的肌原纤维排列方向紊乱，致使横小管不在同一水平。心肌间质明显增生。随着年龄的增长，上述改变愈重。因此法乐四联症病人，在条件允许下，应尽早手术     

Intraoperative hypercyanosis in a patient with pulmonary artery band: case report and review of the literature

A case of intraoperative cyanosis in a patient with a common atrioventricular canal palliated with a pulmonary artery (PA) band is presented. The patient's physiology was consistent with cyanosis due to inadequate pulmonary blood flow, and responded quickly to typical interventions used for a hypercyanotic episode in a patient with unrepaired Tetralogy of Fallot. Differences and similarities in the physiology of PA banding compared with Tetralogy of Fallot are presented, including a rationale for treatment options for hemodynamic decompensation occurring in the setting of anesthesia and surgery.     

Coronary artery obstruction due to membranous ridge of the right sinus valsalva associated with Tetralogy of Fallot: syncope mimics anoxic spell

We report an infant with Tetralogy of Fallot who had suffered from repetitive attacks of syncope without obvious cyanosis. Careful observation by means of echocardiography and angiography revealed that the attacks resulted from acute coronary artery obstruction due to membranous ridge covering the sinus Valsalva. Surgical resection of the abnormal ridge and repair of Tetralogy of Fallot successfully improved the patient's symptoms. Syncope in children should be extensively investigated to exclude obstruction of the coronary arteries.     

Tetralogy of Fallot: T-shaped infundibulotomy for pulmonary valve-sparing procedure

This new and easily reproducible pulmonary valve-sparing technique for the correction of Tetralogy of Fallot is based on a conservative management of the native pulmonary valve to preserve its growth potential. From July 2015 to December 2019, 67 children presenting with a Tetralogy of Fallot were operated consecutively in a single centre using this technique in all cases. A T-shaped infundibulotomy is used to release the anterior pulmonary annulus from any muscular attachment. After myocardial resection and ventricular septal defect closure, an extensive commissurotomy is achieved. Finally, the right ventricular outflow tract remodelling is completed by a shield-shaped bovine patch with an oversized square superior edge, attached directly on the pulmonary valve annulus, with an effect of systolic traction. Sixty patients (89.5%) had a Tetralogy of Fallot repair with preservation of the pulmonary valve. To date, with a median follow-up of 38.2 [14–64] months, no patient has needed a surgical or interventional procedure for pulmonary valve stenosis or regurgitation, with low residual gradients. This procedure could provide a significant increase in native pulmonary valve preservation. Long-term studies are needed to assess pulmonary valve growth and the consequent reduction in surgical or interventional reoperations.     

Off-pump pulmonary valve implantation

Abstract   In congenital heart surgery, especially after Tetralogy of Fallot (TOF), problems from the right ventricular outflow tract may lead to serious complications as morbidity and mortality. To resolve these problems by reoperating using cardiopulmonary bypass (CPB) with valves or conduits is affecting surgical morbidity and mortality rates. The ability of a newly developed Shelhigh Pulmonic Valved Injectable No-React-Treated Conduit (NR 4000-PA MIS Shelhigh Inc., Union, NJ, USA) to be implanted into a beating heart provides great advantages for both patient and surgeon. Early results of the first application in our clinic are presented in this article.     

Failure of neuraxial anaesthesia in a patient with Velocardiofacial syndrome

Velocardiofacial or 22q11 deletion syndrome is a genetic condition caused by deletion 22q11, the deletion of a small segment of the long arm of chromosome 22. To our knowledge this is the first case report of a woman with Velocardiofacial syndrome presenting in late pregnancy for caesarean delivery. She had undergone a Tetralogy of Fallot repair as an infant and had residual pulmonary regurgitation. In addition examination revealed micrognathia and scoliosis. Neuraxial anaesthesia was unsuccessful and subsequent conversion to general anaesthesia was necessary despite concerns regarding her facial abnormalities, pulmonary regurgitation and mild intellectual impairment.     

Brain abscess associated with patent foramen ovale

Background

Brain abscesses can develop with Tetralogy of Fallot and pulmonary anterior venous fistula with large right-to-left shunt. However, some patients exhibit cryptogenic brain abscess (CBA) in the absence of any such congenital disease or other infections. Patent foramen ovale (PFO) is a very common disease that exhibits right-to-left shunt. This study reports the potential for concern between CBA and PFO.

Methods

We enrolled patients with CBA in our hospital between January 2003 and January 2013. Patients underwent transesophageal echocardiography (TEE) with contrast medium to investigate the presence of PFO.

Results

Seven patients were included. Four were females, and the mean age was 67.7?±?9.2 years. In all patients, TEE failed to reveal any new findings, however, six patients had PFO, and another patient had pulmonary arteriovenous shunt. Four patients had odontopathy.

Conclusion

In this study, all CBA patients exhibited right-to-left shunt. CBA might be caused by paradoxical embolization of a bacterial mass via PFO. Thus, more patients with CBA need to undergo TEE to detect PFO.     

Surgical correction of tetralogy of Fallot in a 61-year-old patient

Tetralogy of Fallot (TOF) is a congenital heart disease that is usually diagnosed and treated during infancy. Only 3% of such patients reach the age of 40 without surgery. We describe a 61-year-old woman with uncorrected TOF that was successfully treated by radical surgery. The patient presented with exertional dyspnea, insomnia, and malaise. Echocardiography and cardiac catheterization indicated a dilated, severely hypertrophic right ventricle, ventricular septal defect, an overriding aorta, and infundibular stenosis in the right ventricular outflow tract. All symptoms disappeared after full surgical correction, which remains the preferred treatment for adult TOF because it confers long-term survival and an improved quality of life.     

合并单侧肺动脉缺如先天性心脏病的外科治疗

9例合并单侧肺动脉缺如的先天性心脏病,其中7例为法乐四联症合并单侧肺动脉缺如,系重症先天畸形。手术均采用带瓣的补片疏通流出道。围术期处理以纠治低心输出量为重点。     

Pulmonary valve replacement with a mechanical prosthesis. Promising results of 28 procedures in patients with congenital heart disease.

OBJECTIVE: Pulmonary valve replacement is performed increasingly late after correction of Tetralogy of Fallot. Most reports deal with pulmonary allografts as the valvar substitute of choice, although late deterioration and reoperation(s) are the rule. Mechanical valves are scarcely reported and if so only because of complications. Although life-long anticoagulation therapy is indicated for mechanical prostheses, the chance of subsequent re-operations can be expected to be low. We report the results of 28 mechanical valve replacements in the pulmonary position. METHODS: A mechanical valve was implanted in 27 of 79 patients indicated for pulmonary valve replacement. Tetralogy of Fallot was the most common basic lesion. The results and follow-up were reviewed retrospectively, where death and re-operation were primary end points. Routine outpatient follow-up, including trans-thoracic echocardiography, was performed. RESULTS: Twenty-eight pulmonary valve implantations were done in 27 patients. Thirty-day hospital mortality was 1/28 (3.6%), because of a cerebro-vascular accident. One patient died late (2.8 years postoperatively). Median age was 33 years and the median interval between primary repair and insertion of the prosthesis was 26 years. Freedom from re-operation at 1 year was 100%. One valve had to be replaced 14 years after implantation because of malfunction due to ingrowth of endomyocardial fibrosis. No thrombo-embolic events were observed. CONCLUSIONS: Our series do not confirm the bad reputation of mechanical valvar prostheses in the pulmonary position. On the contrary they perform well and result in a much lower re-operation rate than can be expected and in fact is reported after allograft usage. No thrombo-embolic complications were noted. In our experience pulmonary mechanical valve prostheses do well.     

Postoperative prone position management of tetralogy of fallot with absent pulmonary valve syndrome

Tetralogy of Fallot with absent pulmonary valve syndrome is commonly associated with respiratory failure both before and after surgery. This report describes our experience using prone positioning with bilateral pillows to avoid compression of the anterior chest wall after surgery. In the case here, the patient’s respiratory distress was improved by this positioning. Prone position and avoiding anterior chest compression has an effect on severe respiratory distress of tetralogy of Fallot with absent pulmonary valve syndrome.     

Migración de stent implantado en la arteria pulmonar en un paciente con tetralogía de Fallot

Tetralogy of Fallot is a congenital heart disease that accounts for 11-13% of the congenital cardiomyopathies. Stenosis and hyperplasia of the pulmonary arteries occur in a high proportion of them as this disease causes a native stenosis of the pulmonary branch, which can be surgically repaired with a stent. The use of balloon expandable intravascular stents is an alternative technique to further surgery in patients with congenital heart diseases. However, despite the gradual increase in their use, the limited number of procedures, combined with the wide anatomical variability and different characteristics of these patients, even in expert hands, stent implants are associated with a not inconsiderable incidence of complications. These are not always obvious and often require performing surgery in patients who have already had multiple interventions, thus increasing the complexity and the risk.     

Andreas Vesalius (1514-1567)

ABSTRACT

Vivien Thomas (1910–1985) was an African-American scientist, pioneer, and renowned educator. Thomas's contributions to cardiovascular surgery were unequivocal, and yet it was only after his death that he gained more widespread recognition. Thomas's more notable work involves aiding in the discovery of the cause of traumatic shock, designing and guiding the first operation to treat Tetralogy of Fallot, carrying out the first atrial septectomy, and helping develop the electrical defibrillator.

Thomas struggled amidst the adversities of racism and the Great Depression (1929–1941), armed merely with a high school degree. Nevertheless, his genius and determination eventually led him to receive an honorary doctorate from John Hopkins University. His story inspired the creation of the award winning PBS documentary in 2003 Partners of the Heart and also the 2004 Emmy Award-winning HBO film Something the Lord Made. This article will aim to provide an overview to the more notable events in Thomas's amazing tale, with a particular focus on his work on the Tetralogy of Fallot.     

心外带瓣管道在复杂先心病外科治疗中的应用

目的 总结心外带瓣管道在复杂先天性心脏病外科治疗中的应用经验。方法 1979年9月至2001年12月，采用心外带瓣管道对131例复杂性先天性心脏病病人行外科治疗。其中同种带瓣管道22例，异种(猪肺动脉瓣)带瓣管道73例及抗钙化猪心包瓣管道36例。结果全组手术死亡率17．5％。近10年手术65例，死亡率10．7％。近5年手术44例，死亡率4．5％。结论 液氮保存的同种带主动脉瓣或肺动脉瓣管道其取材困难，而抗钙化猪心包瓣管道可以弥补上述不足，还可制成各种型号管道备用。在使用时，按心脏病理解剖确定管道置入方法，掌握管道尺寸，可保持管道完好的几何形状，获得更好的血流动力学效果及持久性。     

Surgical treatment of tetralogy of Fallot with unilateral absence of a pulmonary artery

Tetralogy of Fallot with unilateral absence of a pulmonary artery imposes more marked symptomatology and a much shorter life-span in comparison with the usual tetralogy of Fallot. The operative mortality with total correction has been 50%, and the survivors require prolonged hospitalization for control of severe right heart failure.     

不同年龄的法乐氏四联症病人心肌细胞核染色体倍性分布特征

对６０例不同年龄法乐氏四联症病人的心肌及３０例正常心肌的细胞核染色体倍性分布进行研究，结果发现：心肌细胞核染色体的二倍体随年龄增长而显著减少，四倍体和八倍体显著增多，且四联症病人与正常的心肌细胞核染色体倍性分布有显著性差异，６岁时已与正常成人相似。本研究结果提示为了防止法乐氏四联症病人心肌的不可逆病理损害，尽早实施心内修复术是必要的。     

Complete atrioventricular canal and tetralogy of Fallot with pulmonary atresia

Our patient was diagnosed with complete atrioventricular canal and Tetralogy of Fallot with pulmonary atresia at the age of 1 month. Then he underwent right and left Blalock-Taussig shunts at the ages of 2 months and 5 years, respectively. His cyanosis had increased at 20 years of age. Cardiac catheterization showed occlusion of the left Blalock-Taussig shunt and absence of the left pulmonary artery. Lung perfusion scintigram showed late phase perfusion in the left lung. Chest computed tomographic scan demonstrated the left pulmonary artery. We describe the operative technique of total correction.