Surgical treatment in tetralogy of Fallot diagnosed by echocardiography.

The purpose of this paper is to present the authors' 3-yr experience of echocardiographic examination of patients with the clinical diagnosis of tetralogy of Fallot, and their evaluation for surgical treatment without prior cardiac catheterization. Among the patients with the clinical diagnosis of tetralogy of Fallot 227 had a definite diagnosis made by M-mode, two-dimensional, Doppler and contrast echocardiography. For the diagnosis of tetralogy of Fallot, ventricular septal defect, pulmonary stenosis, and overriding of the aorta were considered to be fundamental. Ventricular septal defect could be seen easily in the subaortic region by two-dimensional echocardiography. However, in some patients whose ventricular septal defect was not seen clearly, peripheral vein contrast echocardiography was performed. The diameters of pulmonary artery, and main branches at a few millimeters distal to their origin were measured. These parameters were correlated with the aortic diameter for evaluation as to whether they were able to accept the total cardiac output. In patients whose left ventricular end-diastolic dimension was small, shunt operation was preferred. In 115 patients the pediatric cardiologist performing the echocardiography thought that cardiac catheterization was necessary. In these cases the reliability of echocardiography in detecting important cardiac abnormalities was evaluated. Detection of ventricular septal defect, presence of pulmonary valve, detection of stenosis on the pulmonary bifurcation and/or main branches revealed a high sensitivity. Two-hundred-and-one patients diagnosed by echocardiography underwent total correction. In all cases except one the preoperative diagnosis was confirmed by surgery.(ABSTRACT TRUNCATED AT 250 WORDS)     

Tetralogy of Fallot with right pulmonary artery origin from the ascending aorta: noninvasive diagnosis and surgical correction in a 7-week-old infant

Anomalous origin of one pulmonary artery from the ascending aorta is a rare anomaly which is almost always associated with hypertension in the contralateral pulmonary artery originating from the right ventricle. We report echocardiographic evaluation of an infant with tetralogy of Fallot and aortic origin of the right pulmonary artery. Since all relevant information regarding anatomy and hemodynamics could be obtained by echocardiography, cardiac catheterization and angiography were unnecessary. Surgical correction including transatrial patch closure of the ventricular septal defect, resection of the infundibular stenosis and direct reimplantation of the right pulmonary artery was performed successfully at the age of 7 weeks. The postoperative course was uneventful and persistent pulmonary hypertension was excluded by echocardiography. Our case shows that complete echocardiographic evaluation is possible in infants with this complex cardiac malformation. We recommend reserving cardiac catheterization for those patients with echocardiographic evidence of significant elevation of pulmonary vascular resistance. In order to avoid irreversible pulmonary vascular changes early surgical correction within the first 3 months of life should be performed in all children with aortic origin of a pulmonary artery including those with associated tetralogy of Fallot.     

Detection of coronary artery abnormalities in tetralogy of Fallot by two-dimensional echocardiography

Patients with tetralogy of Fallot have a 5% to 19% incidence rate of abnormal distribution of coronary arteries. These abnormalities are usually detected by angiography and influence the timing and mortality rate of surgery. This study evaluates two-dimensional echocardiography as a method of assessing coronary artery distribution in tetralogy of Fallot. Forty-five consecutive patients with tetralogy of Fallot, aged 0.1 to 20.5 years (mean 5.7 +/- 4.3), had prospective two-dimensional echocardiographic studies to examine the branching patterns of the coronary arteries and to determine the presence or absence of a branch from the right or left coronary artery that crossed the right ventricular outflow tract. The first two patients had known coronary abnormalities and served as learning models. All other echocardiographic studies were performed without knowledge of angiographic or surgical findings. Twenty-two studies were completed before coronary angiography (group A) and 23 after angiography (group B). All eight patients (18%) with coronary abnormalities were correctly identified by two-dimensional echocardiography (five in group A and three in group B). Three had bilateral anterior descending coronary arteries originating from the left and right coronary arteries, two had the anterior descending artery originating from the right coronary artery, two had a large conal branch from the right coronary artery and one had origin of both left and right coronary arteries from a single left ostium. All abnormal coronary arteries were visualized crossing the right ventricular outflow tract, whereas all 21 small conal branches from the right coronary artery were not seen in the right ventricular outflow tract.(ABSTRACT TRUNCATED AT 250 WORDS)     

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.     

Coronary artery imaging with multidetector computed tomography: a call for an evidence-based, multidisciplinary approach

Modern multidetector computed tomography systems are capable of a comprehensive assessment of the cardiovascular system, including noninvasive assessment of coronary anatomy. Multidetector computed tomography is expected to advance the role of noninvasive imaging for coronary artery disease, but clinical experience is still limited. Clinical guidelines are necessary to standardize scanner technology and appropriate clinical applications for coronary computed tomographic angiography. Further evaluation of this evolving technology will benefit from cooperation between different medical specialties, imaging scientists, and manufacturers of multidetector computed tomography systems, supporting multidisciplinary teams focused on the diagnosis and treatment of early and advanced stages of coronary artery disease. This cooperation will provide the necessary education, training, and guidelines for physicians and technologists assuring standard of care for their patients.     

Ductal Stent Implantation in Tetralogy of Fallot with Aortic Arch Abnormality

Stenting of patent ductus arteriosus is an alternative to palliative cardiac surgery in newborns with duct-dependent or decreased pulmonary circulation; however, the use of this technique in patients with an aortic arch abnormality presents a challenge. Tetralogy of Fallot is a congenital heart defect that is frequently associated with anomalies of the aortic arch and its branches. The association is even more common in patients with chromosome 22q11 deletion.We present the case of an 18-day-old male infant who had cyanosis and a heart murmur. After an initial echocardiographic evaluation, the patient was diagnosed with tetralogy of Fallot and right-sided aortic arch. The pulmonary annulus and the main pulmonary artery and its branches were slightly hypoplastic; the ductus arteriosus was small. Conventional and computed tomographic angiograms revealed a double aortic arch and an aberrant left subclavian artery. The right aortic arch branched into the subclavian arteries and continued into the descending aorta, whereas the left aortic arch branched into the common carotid arteries and ended with the patent ductus arteriosus. After evaluation of the ductal anatomy, we implanted a 3.5 × 15-mm coronary stent in the duct. Follow-up injections showed augmented pulmonary flow and an increase in oxygen saturation from 65% to 94%. The patient was also found to have chromosome 22q11 deletion.     

Echocardiographic study of right and left ventricular dimension and left ventricular function in patients with tetralogy of Fallot before and after surgery.

Right and left ventricular dimensions and function were determined by one-dimensional echocardiography in patients with tetralogy of Fallot before and after corrective surgery. Thirty-five children (mean age: 5.9 years) were examined; 5 of them died immediately after operation; 5 had palliative operations only. The remaining 25 had repeat echocardiography 2 to 4 and/or 8 weeks after total correction. Compared with normal values, preoperative left ventricular dimensions were smaller than expected for body surface area (mean = 85.4% +/- 1.9 SEM, range 65 to 105% of normal); 21 values were below the 5th centile. Postoperatively, left ventricular dimensions increased significantly and reached normal values in most cases (mean = 103.2 +/- 2.0% SEM, range 81 to 121%). The main increase took place in the first 4 weeks (P less than 0.001; mean difference 0.7 +/- 0.14 cm). The 5 children who died after operation had smaller left ventricular dimensions than the survivors (P less than 0.01). Left ventricular function was evaluated by measuring mean circumferential fibre shortening, per cent shortening, and ejection fraction; they were normal in most patients and diminished only insignificantly after corrective surgery. Right ventricular dimensions were increased preoperatively but decreased significantly (P less than 0.001) postoperatively. Septal movement was normal in direction and excessive in displacement in most patients before operation; immediately after operation it became flat or showed paradoxical motion. Two months after operation 50 per cent of the children showed a return to normal septal movement. Early appearance of normal septal movement could be related to the presence of significant pulmonary stenosis. It is concluded that a high percentage of patients with tetralogy of Fallot have underdeveloped but normally functioning left ventricles which adapt well to the new postoperative state.     

Anomalous origin of one pulmonary artery from the ascending aorta: a review of echocardiographic, catheter, and morphological features.

Six patients with anomalous origin of one pulmonary artery from the ascending aorta were reviewed. Four had anomalous origin of the right pulmonary artery and two had anomalous origin of the left pulmonary artery from the ascending aorta. Two of these six patients had tetralogy of Fallot. Two patients died in the first month of life. No changes in the pulmonary vasculature were seen at necropsy. Corrective surgery was attempted in two patients with associated tetralogy of Fallot when they were two years old but both died. At necropsy there was severe pulmonary vascular disease in the lung supplied by the anomalous pulmonary artery but no pulmonary vascular hypertensive changes in the lung supplied by the pulmonary artery from the right ventricle. Two recent patients underwent successful anastomosis of the anomalous pulmonary artery to the main pulmonary artery at three months and one month and three weeks of age respectively. Intraoperative lung biopsy in the latter patient showed early changes in both lungs. Both echocardiography and cardiac catheterisation were used in the diagnoses. Systemic or suprasystemic pressures were found in the pulmonary artery arising from the right ventricle as well as the anomalous pulmonary artery in the three patients without tetralogy of Fallot. Anomalous origin of a pulmonary artery from the ascending aorta is a distinct entity and differs from other aorto-pulmonary arterial connections. Early surgical intervention is recommended in all patients (including those patients with associated tetralogy of Fallot) because of the risk of rapid development of irreversible pulmonary vascular disease.     

Echocardiographic study of right and left ventricular dimension and left ventricular function in patients with tetralogy of Fallot before and after surgery.

Right and left ventricular dimensions and function were determined by one-dimensional echocardiography in patients with tetralogy of Fallot before and after corrective surgery. Thirty-five children (mean age: 5.9 years) were examined; 5 of them died immediately after operation; 5 had palliative operations only. The remaining 25 had repeat echocardiography 2 to 4 and/or 8 weeks after total correction. Compared with normal values, preoperative left ventricular dimensions were smaller than expected for body surface area (mean = 85.4% +/- 1.9 SEM, range 65 to 105% of normal); 21 values were below the 5th centile. Postoperatively, left ventricular dimensions increased significantly and reached normal values in most cases (mean = 103.2 +/- 2.0% SEM, range 81 to 121%). The main increase took place in the first 4 weeks (P less than 0.001; mean difference 0.7 +/- 0.14 cm). The 5 children who died after operation had smaller left ventricular dimensions than the survivors (P less than 0.01). Left ventricular function was evaluated by measuring mean circumferential fibre shortening, per cent shortening, and ejection fraction; they were normal in most patients and diminished only insignificantly after corrective surgery. Right ventricular dimensions were increased preoperatively but decreased significantly (P less than 0.001) postoperatively. Septal movement was normal in direction and excessive in displacement in most patients before operation; immediately after operation it became flat or showed paradoxical motion. Two months after operation 50 per cent of the children showed a return to normal septal movement. Early appearance of normal septal movement could be related to the presence of significant pulmonary stenosis. It is concluded that a high percentage of patients with tetralogy of Fallot have underdeveloped but normally functioning left ventricles which adapt well to the new postoperative state.     

Anomalous origin of one pulmonary artery from the ascending aorta: a review of echocardiographic, catheter, and morphological features

Six patients with anomalous origin of one pulmonary artery from the ascending aorta were reviewed. Four had anomalous origin of the right pulmonary artery and two had anomalous origin of the left pulmonary artery from the ascending aorta. Two of these six patients had tetralogy of Fallot. Two patients died in the first month of life. No changes in the pulmonary vasculature were seen at necropsy. Corrective surgery was attempted in two patients with associated tetralogy of Fallot when they were two years old but both died. At necropsy there was severe pulmonary vascular disease in the lung supplied by the anomalous pulmonary artery but no pulmonary vascular hypertensive changes in the lung supplied by the pulmonary artery from the right ventricle. Two recent patients underwent successful anastomosis of the anomalous pulmonary artery to the main pulmonary artery at three months and one month and three weeks of age respectively. Intraoperative lung biopsy in the latter patient showed early changes in both lungs. Both echocardiography and cardiac catheterisation were used in the diagnoses. Systemic or suprasystemic pressures were found in the pulmonary artery arising from the right ventricle as well as the anomalous pulmonary artery in the three patients without tetralogy of Fallot. Anomalous origin of a pulmonary artery from the ascending aorta is a distinct entity and differs from other aorto-pulmonary arterial connections. Early surgical intervention is recommended in all patients (including those patients with associated tetralogy of Fallot) because of the risk of rapid development of irreversible pulmonary vascular disease.     

Pulmonary arterial anatomy in tetralogy of Fallot

We have searched for any abnormalities of the pulmonary arteries visualized by angiography in 200 consecutive patients with tetralogy of Fallot. All the patients were studied by conventional right ventricular angiography in the right and left anterior oblique views. Thirty six patients (18%) showed a total of 61 pulmonary artery stenoses, 32 being severe (52%), 17 moderate (28%) and 12 mild (20%). Bifurcational stenoses were the most common (31), followed by stenosis of the main pulmonary trunk, the right or the left pulmonary arteries (22), combined central and peripheral stenosis (6), and isolated peripheral pulmonary artery stenosis (2). Nine patients showed segmental stenosis involving both the right and left pulmonary arteries and three of them had atresia of the left pulmonary artery. The detailed preoperative evaluation of obstruction within the pulmonary arterial pathways is essential in tetralogy of Fallot to ensure normalisation of the right ventricular pressure following total correction.     

Reliability of intraoperative transesophageal echocardiography during Tetralogy of Fallot repair

There is limited information available concerning the accuracy of intraoperative transesophageal echocardiography (TEE) in predicting the extent of residual abnormalities after recovery from surgical repair of tetralogy of Fallot. Therefore, we investigated differences between the results of final postbypass TEE and those of postrecovery (mean, 6 days after surgery) transthoracic echocardiography in a total of 28 consecutive pediatric patients who underwent repair of tetralogy of Fallot with biplane or multiplane TEE. Both postbypass and postrecovery echocardiographic examinations included measurements of the right ventricle (RV)-main pulmonary artery (PA) and the main PA-branch PA peak instantaneous gradients, the degree of pulmonary valvar insufficiency, and color Doppler interrogation of the ventricular septum for residual defects. The RV-main PA gradient did not change significantly: 15 +/- 13 vs 18 +/- 14 mmHg (postbypass versus postrecovery, mean +/- SD). None of the patients had a decrease of > or = 10 mmHg; and only one patient had an increase of > or = 15 mmHg. There also was no change in the degree of pulmonary insufficiency (3.0 +/- 1.2 versus 3.1 +/- 1.1, using a scale of 0 to 4). Only one of the seven very small (< or = 2 mm) residual ventricular septal defects was not discovered during postbypass TEE. However, postrecovery transthoracic echocardiography detected significant branch PA stenosis (peak gradient, > or = 15 mmHg) in five patients (18%) that was not detected during postbypass TEE (P < 0.03). Of the branch PA stenoses that were not detected during TEE, four were left and one was right. Conclusions: Postbypass TEE after tetralogy of Fallot repair reliably predicts residual postrecovery hemodynamic abnormalities, except for branch PA stenosis.     

Early results and follow-up of balloon angioplasty for branch pulmonary artery stenoses

Two hundred eighteen balloon angioplasty procedures were performed in 135 patients with branch pulmonary artery stenoses from June 1984 to February 1989. Arteries were dilated in patients with tetralogy of Fallot (n = 49), tetralogy of Fallot/pulmonary atresia (n = 64), isolated peripheral pulmonary artery stenoses (n = 58) and "other" lesions (the majority had truncus arteriosus or single ventricle and surgically induced pulmonary artery stenoses (n = 47). Mean age at dilation was 6.6 +/- 6.3 years (range 1 month to 38.5 years). The mean diameter of the lesion increased from 3.8 +/- 1.7 to 5.5 +/- 2.1 mm with dilation (p = 0.001). The overall success rate was 58% (127 of 218 dilations), assessed by the following criteria: an increase greater than or equal to 50% of predilation diameter, an increase greater than 20% in flow to the affected lung or a decrease greater than 20% in systolic right ventricular to aortic pressure ratio. Success did not correlate with patient age. Mean balloon to artery ratio was higher in successful (4.2) than in failed (3.0) angioplasty procedures (p = 0.0001). There were four early deaths: two of the patients had pulmonary artery rupture with angioplasty performed less than 1 month after pulmonary artery surgery. An aneurysm occurred in 11 arteries and transient pulmonary edema in four patients. At angiography performed a mean of 10 months (range 1 to 54) after dilation, the mean diameter of 57 arteries was unchanged (5.5 versus 5.4 mm). However, 5 of 32 initially successfully dilated vessels had returned to predilation size as a result of restenosis.(ABSTRACT TRUNCATED AT 250 WORDS)     

法乐四联症术后血浆内皮素-1浓度与肺动脉压的变化规律和意义

目的探讨法乐四联症(TOF)手术后血浆内皮素1(ET1)浓度与肺动脉压的变化规律和意义。方法将1999年8月至2003年2月间收治的36例确诊为TOF的患者根据手术中右心室流出道的加宽方式不同分为瓣膜组(16例)和非瓣膜组(20例),同期选择30例无症状室间隔缺损患者作为对照组,分别于手术前、手术后1周、1个月、3个月、6个月、12个月抽取肘静脉血,检测血浆ET1浓度,同时行超声心动图监测肺动脉压力。结果手术后1周血浆ET1浓度各组明显高于手术前,有显著性差异(P<0.05);瓣膜组、非瓣膜组较对照组术后1周、1个月、3个月、6个月、12个月均升高,有显著性差异(P<0.05);瓣膜组较非瓣膜组术后上述各时段明显低,有显著性差异(P<0.05)。肺动脉瓣反流轻、中、重度各组之间手术后各时段之间有显著性差异(P<0.05)。瓣膜组、非瓣膜组血浆ET1浓度术后上述各时段均高于对照组,有显著性差异(P<0.05)。肺动脉压的变化情况与血浆ET1的变化趋势基本一致。结论TOF根治术后发生相对性或绝对性肺动脉高压,影响术后心功能特别是右心功能的恢复。     

成人法洛四联症60例外科治疗体会

目的:探讨成人法洛四联症(TOF)外科手术治疗。方法:成人TOF外科治疗60例,全组均有右心室流出道狭窄,15例肺动脉瓣狭窄,其中4例重度狭窄行成型术不满意或无法成型,而行跨环补片,2例因冠状动脉畸形而行(20～22)号牛颈静脉带瓣管道。结果:本组术后死亡5例,病死率8.3%。死于严重低心排出量综合征2例,无法停机2例,灌注肺1例。结论:成人TOF手术效果确切,患者术后生活质量明显改善。     

Doppler echocardiographic assessment of hemodynamic parameters on patients with tetralogy of Fallot

Continuous Doppler echocardiography was used for the study of hemodynamic parameters on various stages of treatment of 61 children (mean age 12.4 months) with tetralogy of Fallot. Twenty five patients were studied before surgery during spells of cyanosis and hyperpnea. Thirty six patients were treated with systemic - pulmonary arterial anastomosis and in 25 patients total correction was carried out. Doppler echocardiographic study was repeated in 2 weeks and 1 year after surgery. Attack of cyanosis and hyperpnea was associated with reduction of pulmonary blood flow and increase of venoaortic shunting. Comparison of hemodynamic parameters before and after creation of aorta-to-pulmonary artery anastomosis allowed to establish Doppler echocardiographic criteria of assessment of the anastomosis functional state. Repetitive Doppler echocardiographic studies showed that hemodynamic results of total correction depended on the type of repair of right ventricular outflow tract. Severity of pulmonary artery valve insufficiency also depended on the type and extent of surgical intervention.     

A rare case of anomalous origin of the left anterior descending artery from the pulmonary artery

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart disease, with an incidence of <1 in 300 000 live births. We describe a rare case of a 23‐month‐old infant admitted for cough and fever. Echocardiography showed a branch of the left coronary artery originating from the pulmonary artery. The coronary computed tomographic angiography revealed anomalous origin of the left anterior descending (LAD) artery from the pulmonary artery with the left circumflex artery and right coronary artery arising normally from the aorta. The infant successfully underwent surgical reimplantation of the LAD to the ascending aorta. Our case emphasizes that echocardiography and computed tomographic angiography are valuable imaging modalities for making an accurate diagnosis and determining the precise surgical plan.     

Two-dimensional echocardiographic detection of left pulmonary artery aneurysm following Potts' anastomosis

The two-dimensional echocardiographic detection of left pulmonary artery aneurysm following a Pott's anastomosis in a patient with tetralogy of Fallot is described. The diagnosis was confirmed at angiography and surgery.     

Pulmonary arteriography in congenital heart disease

Pulmonary outflow tract obstruction may occur at subvalve, valve, or pulmonary artery level. Selective ventriculography will usually define the intracardiac obstruction (whether valve, subvalve, or both) and may define pulmonary arterial abnormalities. Increasing experience in the surgical management of patients with tetralogy of Fallot and other conotruncal abnormalities characterized by significant pulmonary outflow tract obstruction suggests that preoperative recognition of pulmonary arterial stenosis is essential to the successful operative management of these patients (1-4).     

Anomalous origin of the right coronary artery from the pulmonary artery associated with tetralogy of Fallot: description of the pre-surgical diagnosis and surgical repair

Anomalous origin of the right coronary artery from the pulmonary artery is a rare congenital defect. We describe the case of an infant with anomalous origin of the right coronary artery from the pulmonary artery in association with tetralogy of Fallot. This patient had a pre-operative echocardiographic diagnosis, which was confirmed by angiography, and later underwent a successful surgical repair.