High origin of the right coronary artery with partial anomalous pulmonary venous connection to the left superior caval vein in tetralogy of Fallot

The prevalence of anomalous origin of coronary artery in tetralogy of Fallot has been reported to be around 4% to 6%. The association of high take-off of the right coronary artery from the distal part of the ascending aorta in tetralogy of Fallot in the presence of a partial anomalous pulmonary venous connection (PAPVC) to the left superior caval vein draining into the left atrium is not known to the best of our knowledge. We herein describe such a case when the anomalous right coronary artery and the PAPVC were detected incidentally during intracardiac repair; signifying the importance of a thorough assessment of the anatomy before surgery.     

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.     

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.     

Repair of tetralogy of Fallot associated with anomalous origin of right pulmonary artery and accessory left anterior descending coronary artery

We present an unusual combination of lesions in an 8-month-old child diagnosed with tetralogy of Fallot, anomalous origin of right pulmonary artery, and anomalous coronary artery crossing the pulmonary annulus. The association of anomalous origin of branch pulmonary artery from aorta and tetralogy of Fallot (TOF) is extremely rare with an incidence of 0.4%. The incidence of anomalous coronary artery in TOF is 10.3%. However, a combination of all three lesions poses challenges to surgical repair and has not been previously reported.     

Tetralogy of Fallot with anomalous origin of left anterior descending coronary artery. Surgical options

Anomalous origin of the left anterior descending coronary artery from the right coronary artery can interfere with the location of the usual ventriculotomy during repair of tetralogy of Fallot. The purpose of this study was to compare the results of two operative techniques: (1) a "tailored" right ventricular incision and outflow patch reconstruction and (2) placement of a conduit from the right ventricle to the main pulmonary artery. We reviewed the records of 416 patients who had complete repair of tetralogy of Fallot at the Mayo Clinic from 1973 through 1984. Twenty (5%) (median age 6.5 years) had anomalous origin of the left anterior descending coronary artery from the right coronary artery. Twelve of these patients had right ventricular outflow patch reconstruction, and eight had placement of a conduit from the right ventricle to the pulmonary artery. Three deaths occurred, all during hospitalization, two in the patch reconstruction group and one in the conduit group. The average reduction in right ventricular pressure postoperatively was slightly but not significantly greater for the conduit group. These data indicate that correction of tetralogy of Fallot with anomalous origin of the left anterior descending coronary artery can be done with either patch reconstruction or conduit placement. Selection of the more appropriate procedure depends on the exact location and degree of tortuosity of the anomalous artery and the level and severity of right ventricular outflow obstruction.     

An alternative procedure for correction of anomalous origin of left coronary artery from the pulmonary artery

Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital cardiac malformation that is commonly associated with mitral insufficiency. Direct implantation of the anomalous origin of the left coronary artery from the pulmonary artery into the aorta is ideal, but it may not be fit for some patients whose anomalous left coronary arteries arise remotely from the ascending aorta. To solve the same technical problem in a boy with anomalous origin of the left coronary artery from the pulmonary artery in combination with moderate mitral insufficiency, we successfully elongated the anomalous coronary artery by creating a tube-shape graft using part of the pulmonary arterial wall. Simultaneous mitral annuloplasty was performed after a dual-coronary repair.     

Tetralogy of Fallot associated with anomalous origin of the left coronary artery from the pulmonary artery, pulmonary artery hypoplasia and atrial septal defect. A case report

A 7-year-old girl had tetralogy of Fallot with anomalous origin of the left coronary artery from the pulmonary artery, pulmonary artery hypoplasia and atrial septal defect. The case is presented and diagnostic and surgical implications are discussed.     

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.     

Primary repair minimizing the use of conduits in neonates and infants with tetralogy or double-outlet right ventricle and anomalous coronary arteries

OBJECTIVE: The purpose of this study was to review our results with an approach of early primary repair for tetralogy of Fallot or double-outlet right ventricle with anomalous coronary arteries, using several techniques to minimize the use of a conduit. METHODS: Twenty consecutive neonates and infants with anomalous coronary arteries crossing an obstructed right ventricular outflow tract underwent primary repair. Median age was 5.5 months and mean weight 6.22 kg. The anomalous coronary arteries included the left anterior descending from the right coronary artery (n = 10), the right coronary artery from the left anterior descending (n = 1), the left anterior descending from the right sinus (n = 1), and a significant conal branch from the right coronary artery (n = 7) or left anterior descending (n = 1). Two neonates had pulmonary atresia. The right ventricular outflow tract was reconstructed without a conduit in 18 patients, including those with pulmonary atresia. Surgical techniques included main pulmonary artery translocation in 4 patients, transannular repair under a mobilized left anterior descending coronary artery in 2 patients, and displaced ventriculotomy with subcoronary suture lines in 8 patients. In 4 patients the right ventricular outflow tract was repaired via the ventriculotomy and/or pulmonary arteriotomy. A homograft was used as the sole right ventricle-pulmonary artery connection in 1 patient and in another a homograft was added to a hypoplastic native pathway. RESULTS: There have been no early or late deaths. The right ventricular/left ventricular pressure ratio within 48 hours of the operation was 0.47 +/- 0.10. There were 2 reoperations at 8 and 11 years after the operation, during a mean follow-up of 5.2 years (1-11.3 years). CONCLUSIONS: Primary repair of tetralogy of Fallot or double-outlet right ventricle with anomalous coronary arteries can be done in neonates and infants with excellent results. Alternative surgical techniques for right ventricular outflow tract reconstruction, such as main pulmonary artery translocation, can avoid the use of a conduit in most patients.     

A new approach to correction of tetralogy of Fallot with absent pulmonary valve

A new technique for primary repair of tetralogy of Fallot with absent pulmonary valve syndrome is suggested. In addition to the standard intracardiac portion of tetralogy of Fallot repair, this approach includes translocation of the pulmonary artery anterior to the aorta, shortening of the ascending aorta, and shortening and plication of the pulmonary artery. This new technique should eliminate compression of the tracheobronchial tree caused by impingement by the pulmonary artery.     

Coronary artery anomalies in patients with tetralogy of Fallot.

A left anterior descending coronary artery arising from the right coronary artery can be easily injured during performance of a right ventriculotomy for correction of tetralogy of Fallot. This occurred in 2 of the 23 patients in this series, and both patients died from myocardial failure in the early period after operation. Of 19 patients who presented a combination of tetralogy of Fallot and unusual coronary artery distribution, injury to the abnormal coronary artery was avoided by a transverse right ventriculotomy either alone or combined with an upper vertical incision in 17 patients. In 2 patients a Dacron tube graft was inserted between the right ventricular outflow tract and the pulmonary artery. In 2 patients a right ventriculotomy was avoided by closing the ventricular septal defect through a transaortic approach. All 21 patients survived. Before a vertical or longitudinal incision in the right ventricular outflow tract is performed, the coronary artery distribution should always be checked and confirmation made of the normal origin of the left anterior descending branch from the left coronary artery to the left of the pulmonary artery.     

"Spiral-cuff" technique for repair of anomalous left coronary artery from the pulmonary artery

We describe a new technique for coronary transfer in the repair of anomalous origin of the left coronary artery from the pulmonary artery. The left coronary artery is elongated with the native pulmonary artery wall to form a spiral-shaped coronary cuff to construct an unstretched new left coronary artery system from the ascending aorta. A postoperative angiographic scan showed good flow of the left coronary artery without any kinking or narrowing. This technique is considered useful when an anomalous coronary artery arises a long way from the ascending aorta.     

The need for follow-up after surgical correction of anomalous left coronary artery arising from the pulmonary artery

Four patients are described with an anomalous origin of the left coronary artery from the pulmonary artery. Three were treated by ligation of the abnormal left coronary artery at its anomalous origin; one of them died during surgery. One patient was treated with reimplantation of the left coronary artery into the aorta. Of the survivors 2 are in normal health, but 1 developed aortic valve incompetence after reimplantation of the left coronary artery into the aorta and 1 had persistent left ventricular wall motion abnormalities and developed mitral valve prolapse. The third survivor has impaired health with diffuse left ventricular wall motion abnormality. Careful follow-up after surgery for an anomalous left coronary artery arising from the pulmonary artery is necessary for secondary prevention of problems due to incomplete recovery of myocardium or problems as a consequence of surgical therapy.     

Anterior translocaton of pulmonary artery without aortic transection in repair of tetralogy of Fallot with absent pulmonary valve.

We developed a technique of translocation of the pulmonary artery anterior to the ascending aorta without transection of the aorta in the repair of tetralogy of Fallot with absent pulmonary valve. Our technique includes detachment of the main pulmonary artery (MPA) from the pulmonary annulus, vertical division of the MPA, anterior translocation of the pulmonary artery with patch augmentation between the vertically divided MPA.     

Internal Mammary–Anomalous Left Anterior Descending Coronary Artery Graft in 16-Month-Old Infant with Tetralogy of Fallot: 30-Month Follow-up

Congenital anomalies of the coronary arteries can occur in conjunction with tetralogy of Fallot. We describe a case of unsuspected anomalous origin of the left anterior descending coronary artery arising from the right coronary artery that was discovered at the time of operation in a 16-month-old infant with tetralogy of Fallot. Successful direct vascular repair of the intracardiac anomalies was performed, including left internal mammary–coronary artery anastomosis. The patient, whom we believe to be the youngest to undergo this technique, underwent cardiac catheterization 21 months after operation. The successful results were confirmed at that time.     

The dimensions of the right ventricular outflow tract and pulmonary arteries in tetralogy of Fallot and pulmonary stenosis.

Studies were undertaken of the cineangiograms in 196 consecutive patients entering two institutions with tetralogy of Fallot and pulmonary stenosis, none of whom had previously undergone a surgical procedure. The median age of the patients at the time of the study was 5.9 months. The diameters of the right ventricular infundibulum, pulmonary trunk, and the entirety of the right and left pulmonary arteries were measured (in millimeters), corrected for magnification, and expressed in standard deviation units (Z-values). The median values of the cineangiographically determined diameters of the right ventricular infundibulum and pulmonary trunk were smaller than those of 95% of normal individuals. The median values throughout the right and left pulmonary arteries were within the range of normal. Those of the distal branches of both the right and left pulmonary arteries were similar to the mean values in normal individuals. However, great variability of the dimensions between individuals, and along the pathway in individuals, characterized patients with tetralogy of Fallot. Diffuse narrowing of the pathways both proximally and distally was uncommon. The relation between the diameters of the pulmonary "anulus" and of the distal pulmonary trunk and origin of the left pulmonary artery explained the difficulty of extending an enlarging patch into a wide area distally in some patients.     

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.     

Right atrial approach for surgical correction of tetralogy of Fallot

Total correction of tetralogy of Fallot was performed without a ventriculotomy in 39 patients aged 8 months to 39 years (mean age, 9.1 years) between May 1984 and July 1988. A transatrial approach was used to resect the obstructed infundibulum and to close the ventricular septal defect. In 14 patients, the pulmonary annulus was not enlarged (group 1). Twenty-five patients required a transannular patch (group 2), placed by extending the pulmonary artery incision 1 cm into the right ventricular infundibulum. Eleven patients had repair of pulmonary artery branch stenosis, and associated intracardiac anomalies were simultaneously corrected in 10 patients. After repair, the right ventricular to left ventricular systolic pressure ratios ranged from 0.36 to 0.59 (mean ratio, 0.45) in group 1 and 0.33 to 0.70 (mean ratio, 0.51) in group 2. There were no hospital or late deaths in group 1. Two patients in group 2 with a small left ventricle died shortly after operation. The 37 survivors were followed for 2 to 51 months. Postoperative catheterization in 7 patients detected no residual ventricular septal defects, mild pulmonary regurgitation in 2 patients (group 2), and right ventricular to left ventricular pressure ratios ranging from 0.25 to 0.42 (mean ratio, 0.34). Only 1 patient with a previous total repair by ventriculotomy is symptomatic and requires antiarrhythmic agents and diuretics. The other 36 patients are asymptomatic. In conclusion, tetralogy of Fallot can be safely repaired at any age without a ventriculotomy. The results indicate a minimal incidence of postoperative arrhythmias and pulmonary regurgitation, as well as improved right ventricular function.     

法洛四联症伴冠状动脉畸形的外科治疗

目的：总结法洛四联症伴冠状动脉畸形手术治疗经验,探讨冠状动脉畸形在法洛四联症纠治手术中的意义。方法：自1994年1月至1999年12月,手术治疗法洛四联症伴冠状动脉畸形15例,年龄43d-8岁,其中右冠状动脉起源于左冠状动脉或左前降支6例,单支左冠状动脉和左前降支起源于右冠状动脉各3例。双前降支,单支右冠状动脉和右冠状动脉肺动脉瘘各1例。一期根治手术13例,姑息手术2例。结果：姑息手术无死亡,根治手术早期死亡1例;无冠状动脉手术意外损伤;随访结果满意。结论：根据肺血管条件和畸形冠状动脉走行特点,选择适当的手术方式和右室流出道重建方法可提高手术效果;冠状动脉畸形不再是法洛四联症纠治手术中的风险因素。     

肺动脉及分支量化分析在法洛四联症根治术中的意义

目的：对法洛四联症患者的肺动脉（PA）,左肺动脉（LPA）和右肺动脉（RPA）分支进行量化分析,探讨其临床意义。方法：术前测量236例法洛四联症患者PA及其分支直径,计算PA与主动脉（AO）直径比值（PA／AO＿,PA与体表面积（BSA）比值（PA／BSA）,PA与正常肺动脉面积（NPA）比值（PA／NPA）,（LPA＋RPA）／AO,（LPA＋＿RPA）／PA,（LPA＋RPA）／BSA等,测量术后右心室与左心室收缩压比值（PRV／LV）,分析存活者与死亡者这些指标判别意义。结果（LPA＋RPA）／AO＜0．5时,手术危险性显著增加,PA／BSA大于等于2．0时,（LPA＋RPA）／BSA大于等于2．4及PA／NPA大于等于0．6时,其手术安全性是显著增加,是否需跨肺动脉瓣环补片主要与PA／SBA,PVA／BSA,PA／NPA有关;术后PRV／LV比值与PA及其分支发育情况无关,而主要受术中右心室流出道和PA疏通情况的影响。结论：PA及其分支发育情况虽然对手术结果有影响,但更重要的是手术过程对右心室流出道及肺动脉狭窄的纠正情况／