Trans-pulmonary closure of an aorto-pulmonary window in a patient of tetralogy of Fallot: a case report

We present a case of tetralogy of Fallot (TOF) accompanied by a type II aorto-pulmonary window with severe pulmonary arterial hypertension in a pediatric patient. A successful repair of tetralogy of Fallot with trans-pulmonary patch closure of aorto-pulmonary window was done.     

A rare case of tetralogy of fallot with congenital tubercular bronchiectasis

A 15% of children with congenital heart disease also have an extracardiac abnormality.¹ Tetralogy of Fallot is occasionally associated with various well known extracardiac malformation. We report a rare extracardiac associatation of congenital bronchiectasis and Tetralogy of Fallot presented in late childhood with breathlessness on exertion and frequent respiratory infections. This late presentation was probably due to less destruction caused by oligemic lung of Tetralogy of Fallot. She was successfully managed by intracardiac repair and left upper lobectomy in a single setting.     

Anesthetic management for transurethral resection of the bladder in a 74-year-old man with uncorrected tetralogy of Fallot

We report a case of general anesthesia for transurethral resection of the bladder in a 74-year-old male patient with uncorrected tetralogy of Fallot. This case illustrates the pertinent pathophysiology of the complex cardiac lesion related to tetralogy of Fallot as well as the feasibility and issues with regard to the anesthetic management.     

Effect of oxygen tension and cardiovascular operations on the myocardial antioxidant enzyme activities in patients with tetralogy of Fallot and aorta-coronary bypass.

Since the chronically cyanotic myocardium appears to be more susceptible to reperfusion injury after cardiac operations than the noncyanotic myocardium, we studied the association between the preoperative arterial oxygen tension and the myocardial superoxide dismutase, catalase, and glutathione peroxidase activities. Fourteen patients with tetralogy of Fallot scheduled for elective operations had baseline arterial blood gas measurements done before operation. During the operation right ventricular biopsy specimens were taken for enzyme analysis immediately before cold blood cardioplegic arrest and 20 minutes after crossclamp removal. The tissue antioxidant enzyme activities of the patients with tetralogy of Fallot were compared with the myocardial results in 15 adults with stable angina pectoris having elective aorta-coronary artery bypass graft operations. Myocardial tissues removed from two patients with hypertrophic obstructive cardiomyopathy who had corrective operations were analyzed for antioxidant activities. There were no changes in myocardial antioxidant enzyme activities during the operation in the patients with tetralogy of Fallot and coronary artery bypass graft. The myocardial superoxide dismutase, catalase, and glutathione peroxidase activities correlated (0.82, 0.68, and 0.89, respectively) significantly (p values were less than 0.01, 0.05, and 0.01, respectively) with the preoperative arterial oxygen tensions in the patients with tetralogy of Fallot. The myocardial glutathione peroxidase activities were at least four times higher in the myocardium of patients with coronary artery bypass graft and hypertrophic obstructive cardiomyopathy than in that of those with tetralogy of Fallot. This study provides putative evidence that the myocardium of patients with tetralogy of Fallot is a risk of oxygen-derived free radical injury during and immediately after corrective cardiovascular operations.     

Pulmonary valve replacement in adults with repaired tetralogy of Fallot

There is a growing population of young adults with tetralogy of Fallot. Although surgical approaches have evolved, many adults with repaired tetralogy of Fallot have been left with residual pulmonary regurgitation. Pulmonary regurgitation is an important contributor to a number of late complications including exercise limitations, right heart failure, arrhythmia, and sudden death. Because bioprosthetic valves are used in this population, clinicians must weigh the beneficial effects of pulmonary valve replacement against the associated risks, including subsequent re-operation. In this review, we will appraise the evidence supporting pulmonary valve replacement in the adult with repaired tetralogy of Fallot, as well as the optimal timing and mode of intervention.     

一岁以内婴儿法洛四联症纠治术

目的 总结婴儿法洛四联症(TOF)的纠治经验，以提高婴儿TOF纠治术的成功率。方法 1999年3月一2001年11月我科收治43例小于1岁TOF患者，术前均经二维超声心动图或心导管造影检查确诊，均在体外循环下行纠治手术，其中5例行急诊手术。结果 手术近期死亡1例(2．3％)，30例患者随访1—18个月，恢复良好，紫组消失，生长发育明显加快。结论 小于1岁婴儿行TOF纠治术是安全可靠的，对反复缺氧发作的TOF患者，强调早期手术；对缺氧发作呈持续状态的患者要考虑行急诊手术。改善手术及体外循环方法，加强术后监护是提高婴儿TOF纠治术成功的关键。     

A case of tetralogy of Fallot with pulmonary aneurysm

A case of pulmonary artery aneurysm associated with tetralogy of Fallot is presented. A 28-year-old man was admitted for evaluation of cyanosis and cardiac murmur. Preoperative angiography revealed tetralogy of Fallot and an aneurysm of the main pulmonary artery aneurysm. Operation consisted of pulmonary artery aneurysmectomy and total correction of the tetralogy of Fallot. Microscopic examination of the resected aneurysm showed the thickening of intima and fragmented elastic fibers of media. A brief review of the literature emphasizes the necessity of the surgical intervention.     

Surgical Treatment of Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery Associated with Tetralogy of Fallot

An 8-year-old boy with anomalous origin of the left coronary artery from the pulmonary artery associated with tetralogy of Fallot, which was definitely diagnosed preoperatively, was operated on with success. Direct implantation of the left coronary artery into the aorta following division of the left coronary artery from the pulmonary artery and, concomitantly, total repair for tetralogy of Fallot using an external valved conduit were performed. Postoperative cineangiogram revealed a hemodynamically well-repaired intracardiac condition and anterograde filling of the left coronary artery, compared with retrograde left coronary flow from intercoronary collateral vessels preoperatively.To the best of our knowledge, there is not a previously published report of anomalous origin of the left coronary artery from the pulmonary artery associated with tetralogy of Fallot that was treated surgically with success.     

Aortic root and ascending aortic aneurysm in an adult with a repaired tetralogy of fallot

Surgical repair of the tetralogy of Fallot is one of the most successful operations in the treatment of congenital heart diseases. We report the case of a 65-year-old man who had an aortic valve replacement at the time of complete repair of the tetralogy of Fallot at the age of forty-three. He subsequently had progressive aortic root and ascending aorta dilation to 9 cm. The aortic root and ascending aorta replacement was done using a composite valve-graft and was performed along with other procedures. Thus, meticulous follow-up of aortic root and ascending aorta after corrective surgery for tetralogy of Fallot is recommended following initial curative surgery.     

Successful repair of tetralogy of Fallot combined with aortic valve replacement in a 65-year-old woman]

Survival to seventh decade with tetralogy of Fallot is uncommon. Herein we report a successful repair of tetralogy of Fallot combined with aortic valve replacement for concomitant aortic valve regurgitation in a 65-year-old woman. She had been in severe congestive heart failure and respiratory failure, and also showed liver and renal dysfunction preoperatively. Postoperative course has been good but ventricular arrhythmia of Lown grade II persisted.     

Repair of tetralogy of Fallot with obstruction of right pulmonary artery; report of a case

Patients with tetralogy of Fallot showing unilateral obstruction of a pulmonary artery, especially the right pulmonary artery, are a high-risk group for pulmonary hypertension after repair. This case of tetralogy of Fallot with the obstruction of the right pulmonary artery received a Blalock-Taussig shunt at 7 months old, and the occluded right pulmonary artery caused empyema after surgery. At 2 years old, a cardiac catheter study showed a pulmonary artery index of 193.6 mm2/m2, so we undertook intracardiac repair. After the repair, she showed a relativery favorable clinical course. Systolic pulmonary artery pressure and right ventricular pressure were about 30 and 50 mmHg, respectively. We considered that tetralogy of Fallot with obstruction of right pulmonary artery could be repaired, as long as the pulmonary artery index was within the limits of indication and the left ventricle was well-developed.     

Pulmonic valve annular enlargement with valve repair in tetralogy of Fallot

We present an operative technique of pulmonic valve annular enlargement with concomitant valve repair using two pericardial patches to reduce pulmonary regurgitation after complete repair of tetralogy of Fallot. We have used this technique in 18 patients with tetralogy of Fallot with excellent results.     

Pulmonary origin of left anterior descending coronary artery in tetralogy of Fallot

We report the case of a 4-year-old girl with an anomalous origin of the left anterior descending coronary artery from the pulmonary artery in association with tetralogy of Fallot. Creation of an intrapulmonary tunnel from a newly created aortopulmonary window to the coronary ostium in the pulmonary trunk and total repair of tetralogy of Fallot were successfully carried out. The advantage of intrapulmonary tunnel technique (Takeuchi method) for this very rare combination is stressed.     

Two cases of tetralogy of Fallot with absent pulmonary valve repaired by modified Kreutzer’s technique

Two severely symptomatic infants of tetralogy of Fallot with absent pulmonary valve successfully repaired by a modified Kreutzer’s technique are described. In addition to the standard repair of tetralogy of Fallot, our surgical technique consists of extensive pulmonary reduction arterioplasty, which allows tracheobronchial decompression. Both patients tolerated surgery and showed significant relief of airway compression and clinical symptoms, although they required prolonged mechanical ventilatory support. The modified Kreutzer’s technique is relatively simple and could be sufficiently efficacious even for symptomatic infants of tetralogy of Fallot with absent pulmonary valve.     

Tetralogy of Fallot Associated with Complete Atrioventricular Canal

A 6-year-old girl had the clinical appearance of tetralogy of Fallot but was found at cardiac catheterization to have tetralogy of Fallot associated with complete atrioventricular canal. She underwent repair, and at recatheterization two years later, the hemodynamic result was excellent. Proper angiography is necessary to diagnose this combination, and correct preoperative diagnosis is necessary to plan a rational operative approach.     

One-stage correction of tetralogy of Fallot associated with anomalous origin of the left pulmonary artery from the aorta and aortopulmonary window

We report a rare case of tetralogy of Fallot associated with anomalous origin of the left pulmonary artery from the aorta and aortopulmonary window. Successful one-stage total correction was performed under cardiopulmonary bypass, including implantation of the left pulmonary artery, repair of aortopulmonary window, and correction of tetralogy of Fallot. The patient experienced an uneventful postoperative course and was asymptomatic at one-year follow-up.     

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     

Pseudoaneurysm of homograft placed in right ventricular outflow tract

Pseudoaneurysm of the right ventricular outflow tract after homograft placement is an infrequent complication after intracardiac repair for tetralogy of Fallot. We report two cases of pseudoaneurysm of right ventricular outflow tract after homograft placement for surgical repair of tetralogy of Fallot with pulmonary atresia.     

Thrombosis of the left main pulmonary artery in an infant with tetralogy of Fallot. Treatment by thrombectomy and simultaneous Potts-Smith anastomosis

Thrombosis in the small pulmonary arteries is a common finding in patients dying of severe tetralogy of Fallot. The development of these thrombi are thought to be a result of slow blood flow and polycythemia. A case is described wherein thrombosis of the left main pulmonary artery developed in a six month old infant with tetralogy of Fallot associated with severe hypoxic spells. A successful pulmonary artery thrombectomy was achieved and a Potts-Smith anastomosis performed to the same arteriotomy.     

Recent results in the surgical treatment of Fallot's tetralogy.

Tetralogy of Fallot consists essentially of two abnormalities: a large, unrestrictive ventricular septal defect and severe obstruction to the outflow of the right ventricle. A clear understanding of the principles described in this article should result in a mortality of under 5% after complete correction of tetralogy of Fallot.