Associated coronary and cardiac anomalies in the tetralogy of Fallot. An angiographic study

Numerous studies have pointed out the frequent association of tetralogy of Fallot (TF) with other cardiovascular defects and coronary tree anomalies. We found cardiac defects in 181 (68%) out of 265 patients with TF investigated by catheterization and selective coronary angiography. These anomalies were isolated in 88 cases (49%) and associated with others in 93 patients. In the case of an isolated anomaly associated with TF, the coronary tree was involved in 37.5% and the cardiovascular system in the remaining 62.5%; in the case of two anomalies, the coronary system was involved in 66% of the patients and the cardiovascular apparatus in 34%; in the case of three or more anomalies, the coronary arteries were involved in 71% and the cardiovascular system in 29%. Anomalies in the course and/or distribution of coronary arteries were present in 96 patients (36%): 10 had a single coronary ostium, 13 a left anterior descending artery arising from the right coronary artery, one a circumflex artery arising from the right coronary artery. Small fistulas between coronary arteries and the pulmonary artery were found in 20 cases; anastomoses between coronary and bronchial arteries or right atrium in 42. In 39 patients we observed a large conus artery or large anterior ventricular branches crossing the right ventricle. A right aortic arch was found in 56 patients (21%), a stenosis of the trunk and/or the peripheral pulmonary artery in 35 (13%) and pulmonary artery atresia in five. Four patients showed a complete atrioventricular canal, three an atrial septal defect (primum type) with cleft of the mitral valve, 61 (23%) an atrial septal defect (ostium secundum). Eleven patients had anomalies of the systemic venous return, 26 (10%) a patent ductus arteriosus. Four patients had valvular abnormalities. In our series, a large proportion of cardiac defects associated with TF consists of anomalies of coronary arteries. Our data confirm the usefulness of performing preoperatively routine coronary angiography in patients with complex congenital heart disease.     

成人法洛四联症根治术135例临床分析

【】：目的 总结成人法洛四联症(TOF)的外科手术治疗经验。 方法 回顾性分析2006年1月~2016年8月我院135例成人法洛四联症患者手术治疗的临床资料。所有患者在低温体外循环下行根治术,其中91例采用跨瓣环补片加宽右心室流出道及肺动脉,17例仅行右心室流出道扩大补片,27例疏通流出道后直接缝合右室切口。 结果 住院期间死亡率2.2%(3/135),死亡原因均为低心排血量综合征。其余并发症包括室缺残余漏2例,一过性Ⅲ度房室传导阻滞4例未安装永久起博器,心包积液2例,胸腔积液5例,肺不张4例,肺部感染2例,肾衰竭2例,行血液滤过治疗后其中1例恢复1例死亡。随访128例,平均随访16.6(3~55)月,心功能Ⅰ级96例(75%),心功能Ⅱ级32例(25%)。结论 彻底矫正畸形、加强心肌保护、避免发生低心排血量综合征、加强术后管理,成人法洛四联症外科手术可取得良好的疗效。     

Facial and immunological anomalies associated with tetralogy of Fallot

Dysmorphic facial features were present in 9 of 31 children with tetralogy of Fallot anatomy (29%). These anomalies included hypertelorism, low-set ears, small mouth, short philtrum, and micrognathia. Ten children had pulmonary atresia, 13 (42%) had a right aortic arch, and 13 had extracardiac congenital anomalies. There were 16 children in the series (52%) who had hospital admissions for important or recurrent infections, and 18 who had immune deficiency: low levels of T lymphocytes were found in 9, low levels of complement in 8, and low immunoglobulins in 3. Embryologically, the cardiac outflow tracts, the aortic arch, the face, and the thymus develop at the same time, and all receive migrating cells from the neural crest. Teratogenic factors possibly produce this constellation of anomalies, which is in the spectrum of the Di George syndrome (third and fourth pharyngeal pouch syndrome). It is of importance for the management of such children, that their immune deficiency be recognized and treated appropriately.     

Detection of coronary artery anomalies in tetralogy of Fallot using a specific angiographic protocol

Patients with tetralogy of Fallot were studied with a prespecified angiographic protocol aimed to delineate the types and incidence of coronary artery anomalies and to look for any correlation with other abnormalities detected by simultaneous detailed angiography. The incidence of coronary anomalies was found to be 7.0%; no association was found with these anomalies with any of the other abnormalities detected.     

Distribution and anomalies of coronary arteries in tetralogy of Fallot (author's transl)]

Anomalies in the course and distribution of coronary arteries may complicate the surgical correction of tetralogy of Fallot (TF). With the aim of preventing possible serious, and even lethal, cardiac events caused by the injury of aberrant vessels, 119 patients with TF were subjected to selective coronary angiography. Apart from demonstrating anastomoses between the coronary and bronchial arteries in 31 cases, small fistulas between the coronary arteries and pulmonary artery (in 14 cases) and hypoplasia of the entire coronary tree in 1 case, this investigation revealed anomalies in the origin of coronary branches in 11 cases. In some cases the first septal branch was absent and the diagonal branches were poorly developed. An injury to the coronary system, following the closure of the interventricular septal defect, may possibly explain the onset of a "left anterior hemiblock" after total correction of TF. Selective coronary angiography in small children presents no riskes provided it is correctly performed. In our cases no complications arose.     

End-on aortogram: improved identification of important coronary artery anomalies in tetralogy of Fallot.

OBJECTIVE--To identify coronary artery anomalies in patients with tetralogy of Fallot with an aortogram taken with steep caudal and left oblique angulation ("end-on" aortogram). DESIGN--Prospective evaluation of end-on aortogram in the preoperative angiographic assessment of consecutive patients with tetralogy of Fallot. SETTING--Regional paediatric cardiology centre. PATIENTS--34 patients, aged 3 months to 12 years (median age 9 months). METHODS--An aortogram was performed with steep caudal (38 degrees-45 degrees) and left oblique (0 degrees-30 degrees) angulation under general anaesthetic as part of routine preoperative angiographic assessment. RESULTS--The origins and courses of the coronary arteries were visualised in all patients and important coronary artery anomalies were identified in four patients: single left coronary artery; single right coronary artery (two patients); separate high origin of left anterior descending. These anomalous coronary vessels crossed the right ventricular outflow tract. CONCLUSIONS--It is important to identify preoperatively coronary arteries that may interfere with right ventricular outflow tract reconstruction. An aortogram with steep caudal and left oblique angulation is useful in identifying anomalous coronary arteries and more importantly it defines the relation of these vessels to the right ventricular outflow tract.     

Evaluation of coronary artery anatomy in patients with tetralogy of Fallot by two-dimensional echocardiography

A major coronary artery crossing the right ventricular outflow tract in patients with tetralogy of Fallot interferes with a transannular patch, and preoperative detection of this artery is important. We evaluated the ability of two-dimensional echocardiography to define noninvasively the coronary artery anatomy in 37 consecutive patients (age range, 1 day to 18 years; mean age, 40.9 months). The origin and distribution of the right anterior descending and circumflex coronary arteries, as well as any anteriorly coursing vessel, were examined from parasternal views. Complete studies were obtained in 29 (78%) of the 37 patients. Coronary artery anatomy was determined to be normal by echocardiography in 20 (69%) of the 29 patients. An anterior vessel across the right ventricular outflow tract was detected in the remaining nine patients. Six patients had an anterior descending artery from the left main coronary artery (paired anterior descending arteries in three patients, a right anterior descending artery from the left main coronary artery in two patients, and a right coronary-to-pulmonary artery fistula in one patient). Three patients had no anterior descending artery from the left main coronary artery (anterior descending artery from the right main coronary artery in two patients, and anterior descending and circumflex arteries from the right main coronary artery in one patient). Angiography, surgery, or autopsy confirmed the diagnoses in all but the final patient in whom the anterior descending artery arose from the right main coronary artery as observed at surgery, but the circumflex artery was not seen. Accurate evaluation of coronary artery anatomy is possible by echocardiography in the majority of patients with tetralogy of Fallot. Noninvasive identification of a major coronary artery coursing anteriorly can influence the timing of cardiac catheterization and surgery and the need for angiography.     

Right coronary artery translocation in tetralogy of Fallot

We describe a simple and cost-effective technique to repair anomalous origin of the right coronary artery from the left coronary artery in tetralogy of Fallot. The proximal right coronary artery is re-implanted into the aorta after it is mobilized and transected. This technique avoids the use of conduits in infants or adults with tetralogy of Fallot and anomalous right coronary arteries, and maintains the growth potential of the translocated native coronary artery.     

Re-operation for tetralogy of Fallot with single right coronary artery

A 7-year-old girl with tetralogy of Fallot in association with a single right coronary artery, was successfully re-operated on for right ventricular outflow tract obstruction. To identify the course of the abnormal coronary arteries during the re-operation, a probe was directly inserted into the coronary arteries after aortotomy. Ventriculotomy was successfully performed under the guide of the probe, without damaging the coronary arteries. Double outflow technique was applied for the relief of the right ventricular outflow tract obstruction.     

Transvenous approach to coronary angiography in infants with tetralogy of Fallot

A new technique is offered for selective imaging of the coronary arterial system from a transvenous approach in patients with tetralogy of Fallot as an alternative to other traditional approaches. This technique should identify important coronary anomalies while avoiding femoral arterial cannulation and compromise.     

Exercise capacity of patients with post-operative tetralogy of Fallot

Twenty-one patients with tetralogy of Fallot following intracardiac repair underwent a treadmill test for evaluation of their exercise capacity. They were divided into two groups according to their post-operative hemodynamic status, the good result group and the poor result group. The maximum oxygen consumption during exercise was significantly decreased in the poor result group, but that of the good result group showed no significant difference from the control group. However, the maximum heart rate in both the good and the poor result groups was significantly lower than that of the control group. Thus, the exercise tolerance in post-operative tetralogy patients was found to be within the normal range as long as their hemodynamic operative result was satisfactory, but the limited capability of increasing heart rate with exercise could present a potential problem in the long-term prognosis.     

法洛四联征患者血小板功能状态的研究

目的 :观察法洛四联征 ( TOF)患者血小板功能状态。方法 :采用放射免疫分析法测定 2 6例 TOF患者血浆及尿液中血栓素 A2 ( TXA2 )的代谢产物尿液中血栓素 B2 ( TXB2 )和前列环素( FGI2 )的代谢产物 6-酮 -前列腺素 1α( 6- keto- PGF1α)的含量 ,以及血浆α-颗粒膜蛋白 ( GMP- 14 0 )的含量 ,并与 14例年龄相匹配的正常健康者作对照。结果 :TOF组患者血浆及尿液中 TXB2 的水平显著高于对照组 ( P<0 .0 1) ;TXB2 与 6- keto- PGF1α的比值明显大于对照组 ( P<0 .0 1) ;TOF组血浆GMP- 14 0水平亦显著高于对照组 ( P <0 .0 1)。结论 :TOF患者体内 TXA2 与 PGI2 代谢失衡 ,GMP- 14 0释放增加 ,血小板呈激活状态 ,有利于血小板的粘附、聚集 ,参与血栓性疾病的发生。