Echocardiographic analysis of regional left ventricular wall motion in children after the arterial switch operation for complete transposition of the great arteries.

Regional left ventricular wall motion was assessed by two-dimensional echocardiography in 21 patients with complete transposition of the great arteries at a mean of 2.2 years (range 0.3 to 7) after an arterial switch operation. Fourteen patients had undergone a two-stage and seven patients a primary repair. Twenty patients were found to have echocardiographic images adequate for wall motion analysis. The results of these studies were correlated with thallium-201 myocardial perfusion scans. Left ventricular wall motion was assessed by measuring regional area change in parasternal and apical views. After planimetry of an end-systolic and an end-diastolic frame, the ventricle was divided into eight equal segments and the percent area change was calculated. Both a fixed reference and a floating system correcting for translation and rotation were applied. The measurements in the patient group were compared with normal age-matched values previously obtained in 55 normal infants and children. Wall motion abnormalities, found in seven patients, were located at the apex in three, at the inferior septum and anterolateral wall in one and the inferior, anterolateral and lateral walls in one patient each. All had a myocardial perfusion defect in a corresponding anatomic location. One patient with a small reversible perfusion defect at the basilar septum had normal regional wall motion. The sensitivity of detecting impairment of myocardial perfusion was 0.95. Wall motion abnormalities were found only in patients who had undergone a two-stage repair. Echocardiographic wall motion analysis can be used as a screening method to identify patients with suspected myocardial ischemia after the arterial switch operation.

Current results of management in transposition of the great arteries, with special emphasis on patients with associated ventricular septal defect.

Two hundred forty-five patients less than 15 days of age with transposition of the great arteries with or without a ventricular septal defect or pulmonary stenosis were entered into an ongoing 20 institution treatment study between January 1, 1985 and June 1, 1986. Complete follow-up is available on all patients. The ventricular septal defect narrowed in only 1 of 36 patients with combined transposition of the great arteries and ventricular septal defect; pulmonary stenosis developed or worsened in 3 of these 36 patients and in 3 of the 187 patients with simple transposition. Twelve month overall survival among the 245 patients was 80%. No morphologic feature of transposition was a risk factor for death but major associated cardiac and noncardiac anomalies (more common in patients with combined transposition and ventricular septal defect) and low birth weight were risk factors. Neither arterial switch repair (n = 86), atrial switch (Mustard) repair (n = 21) nor atrial switch (Senning) repair (n = 39) was a risk factor for death, but results in all surgical groups were better in the last part of the experience. Death before repair was less frequent late in the study. Possibly, in low birth weight infants, survival was better with the arterial than with the atrial switch repair. These data suggest that survival at 1 year is similar with either the arterial or the atrial switch repair. The early results of repair of combined transposition of the great arteries and ventricular septal defect are as good as those of simple transposition. Special institutional efforts are required to attain good results with the arterial switch repair and to prevent death before repair.

Arrhythmia and mortality after the Mustard procedure: a 30-year single-center experience.

OBJECTIVES: Our purpose was to assess the risk factors for late mortality, loss of sinus rhythm and atrial flutter after the Mustard operation. BACKGROUND: The Mustard operation provides correction of cyanosis with low surgical risk in transposition of the great vessels. However, right ventricular failure, loss of sinus rhythm, atrial flutter and death are frequent long-term complications. METHODS: Records of 534 children who underwent the Mustard operation at a single center since 1962 were reviewed for demographic, anatomic, electrocardiographic and physiologic predictors and outcomes. RESULTS: There were 52 early deaths (9.7%). Survival analysis was undertaken for 478 early survivors with a mean follow-up interval of 11.6 +/- 7.2 years. There were 77 late deaths (16.1%), with sudden death (n = 31) the most frequent cause. Survival estimates were 89% at 5 years and 76% at 20 years of age. Risk factors were an earlier date of operation, operative period arrhythmia and an associated ventricular septal defect. Risk (hazard) of late death declined in the first decade, with further peaks in the second decade. Sinus rhythm was present in 77% at 5 years and 40% at 20 years. Loss of sinus rhythm was associated with previous septectomy, postoperative bradycardia and late atrial flutter. Freedom from atrial flutter was 92% at 5 years and 73% at 20 years of age. Risk factors for atrial flutter were the occurrence of perioperative bradyarrhythmia, reoperation and loss of sinus rhythm during follow-up. Risk of atrial flutter demonstrates a late increase. CONCLUSIONS: Ongoing loss of sinus rhythm and late peaks in the risk of atrial flutter and death necessitate continued follow-up.

Risk factors for atrial tachyarrhythmias after the Fontan operation.

OBJECTIVES: The purpose of this study was to define the incidence and risk factors for atrial tachyarrhythmias after the Fontan operation. BACKGROUND: Atrial tachyarrhythmias cause morbidity after the Fontan operation. Causative factors may be affected by the type of systemic to pulmonary connection. METHODS: The Fontan operation was performed in 270 consecutive patients between 1982 and 1992. The mean age at operation was 7.0 +/- 4.3 years. Direct atriopulmonary connection was used in 138 patients (51%), total cavopulmonary connection in 94 (35%) and right atrial to right ventricular connection in 38 (14%). RESULTS: Atrial tachyarrhythmias were seen early postoperatively in 55 patients (20%), preoperative atrial tachyarrhythmia being the only risk factor. Follow-up was achieved for 228 early survivors (97%) at a mean interval of 4.4 years. There were 20 late deaths. Late atrial tachyarrhythmias were noted in 29% of patients who received an atriopulmonary connection, 14% of those who received a total cavopulmonary connection and 18% of those who received a right ventricular connection (p < 0.02). Significant risk factors as determined by univariate and multiple logistic regression analysis were atriopulmonary connection type (odds ratio 0.40 for total cavopulmonary relative to atriopulmonary connection [p < 0.05] and 0.37 for right ventricular relative to atriopulmonary connection [p = 0.08]), longer follow-up interval (odds ratio 1.32 for each consecutive year [p < 0.002]) and atrial tachyarrhythmia in the operative period (odds ratio 6.31 [p < 0.0001]). CONCLUSIONS: Early postoperative atrial tachyarrhythmias, length of follow-up and atriopulmonary connection are significant independent risk factors for the presence of late atrial tachyarrhythmias.

An echocardiographic study of the association of ventricular septal defect and right ventricular muscle bundles with a fixed subaortic abnormality.

Since 1983, 36 patients with the combination of right ventricular muscle bundles and a perimembranous ventricular septal defect have been studied in our institution to address the incidence of on associated subaortic abnormality. Of that total 32 (88%) had echocardiographic evidence of such an abnormality (29 had a typical subarotic ridge protruding from the crest of the interventricular septum and the remaining 3 had an echodense area in the same location). Surgical confirmation of the presence or absence of a subaortic abnormality was available in 26. There was correlation between the surgical and echocardiographic findings in all patients. A resting Doppler gradient of greater than or equal to 10 mm Hg was present in only 10. During the study period, 6 patients had Doppler evidence of progression of their gradient. The incidence of subaortic abnormalities in right ventricular muscle bundles and ventricular septal defects appears to be far greater than previously suspected. The exact significance of this finding in the absence of a pressure gradient is still unclear.

Ventriculo-coronary arterial connections in pulmonary atresia with intact ventricular septum, and their influences on ventricular performance and clinical course.

To define the influence of ventriculo-coronary (VC) arterial connections on ventricular performance and late outcome, and associated wall motion abnormalities in patients with pulmonary atresia and intact ventricular septum, 21 patients aged > or = 6 months (mean 4.3 +/- 3.3 years, range 0.5 to 11.8) were reviewed. Ventricular volumes, mass, systolic functional variables and regional wall motion were measured using biplane ventricular cineangiography. Fourteen patients had VC connections, 11 of whom had coronary artery abnormalities. In comparing patients with VC connections and coronary abnormalities (group I) with those without coronary abnormalities (group II), no hemodynamic differences were observed, except the expected increased right-to-left ventricular systolic pressure ratio in group I (1.33 +/- 0.24 vs 0.72 +/- 0.50). Wall motion abnormalities were observed in 8 patients (7 in group I, and only 1 in group II [p < 0.05]). Ejection fraction in patients with abnormal wall motion was significantly lower (50 +/- 6% vs 57 +/- 7%; p < 0.05) and ventricular systolic pressure ratio significantly higher (1.46 +/- 0.16 vs 0.80 +/- 0.45; p < 0.05) than in those with normal wall motion. Five patients died during follow-up, all of whom had wall motion abnormalities. The findings suggest that patients with VC connections and coronary abnormalities have a higher incidence of wall motion abnormalities, which may reflect ongoing ischemia, and are at risk for late death.

Ventricular septal defect and subaortic stenosis: an analysis of 41 patients.

Forty-one patients with subaortic stenosis (SAS) and ventricular septal defect (VSD) were identified from the cardiac records of the Hospital for Sick Children, Toronto, Ontario. The diagnosis of an associated SAS was made clinically in only 1 patient, who had findings of left ventricular (LV) hypertrophy with strain on the electrocardiogram. There was a delay of 3.1 years between initial presentation and detection of SAS. The SAS was not diagnosed at initial catheterization in 17 patients and was confirmed at subsequent catheter studies in 8 patients, surgery in 5 and autopsy in 4. Associated defects included coarctation of the aorta in 12 patients, mitral valve abnormalities in 4, and right-sided obstructions, including anomalous right ventricular muscle bundles in 6 patients, tetralogy in 4 and pulmonic stenosis in 1 patient. The mean gradient across the LV outflow tract was 25 mm Hg. Nineteen patients had serial catheters without intervening surgery, and the outflow gradient increased from a mean of 9 to 36 mm Hg. The mechanism of SAS consisted of fibrous diaphragm and fibromuscular obstruction in 31 cases, muscular narrowing in 4, protruding tricuspid valve leaflet in 2, hypertrophic cardiomyopathy in 2, anterolateral twist in 1 patient and redundant tissue tag in 1. Thirty-eight patients had a perimembranous VSD, 19 of whom had an associated so-called aneurysm of the membranous septum; 2 had an infundibular VSD and 1 patient had a central muscular defect. Although the SAS was located below the VSD in 30 cases, the associated heart failure and reduced cardiac output can mask the presence or severity of associated SAS.(ABSTRACT TRUNCATED AT 250 WORDS)

Calcification in porcine xenograft valves in children.

Calcification developed in the degenerating collagen of the cusps of three porcine xenograft heart valves implanted in children for less than 4 years. The morphologic features and effects of this calcification are presented. Calcification of porcine xenografts seems to occur more frequently and at an earlier stage after insertion in children than in adults. Host factors, possibly related to calcium homeostasis, may promote calcification; hence, these valves may not be appropriate for use in children.

Echocardiographic recognition of atrioventricular valve stenosis associated with endocardial cushion defect: pathologic and surgical correlates.

The association of either mitral or tricuspid stenosis with endocardial cushion defects has been well recognized pathologically but is infrequenctly diagnosed clinically. M mode echocardiographic features such as markedly disproportionate ventricular size, abnormal mitral or tricuspid diastolic echoes or failure to define adequately an atrioventricular (A-V) valve are strongly suggestive of associated stenosis of that valve. In this study mitral stenosis in association with endocardial cushion defect was correctly diagnosed in four of five patients. In the fifth patient (the first seen) the condition was easily diagnosed retrospectively. Tricuspid stenosis was similarly diagnosed prospectively in three of four patients. Significant stenosis was not overlooked or misdiagnosed in any patient with an endocardial cushion defect. Significant stenosis of an A-V valve in association with endocardial cushion defect carries a high mortality. It is important to recognize the combined lesion clinically because infants with it are not suitable for total surgical correction. Palliative surgical procedures with aggressive medical therapy probably offer the infant the best chance of surviving until ventricular growth may render corrective surgery feasible.

Survival after systemic to pulmonary arterial shunts in infants less than 30 days old with obstructive lesions of the right heart chambers.

Ninety-seven infants less than 30 days old with obstructive lesions of the right heart chambers underwent palliative surgery from 1950 through 1972. The diagnosis was made at cardiac catheterization and confirmed at operation or subsequent autopsy. Because of unavailability of complete preoperative studies and sufficient blood gas data, 36 patients presenting between 1950 and 1965 (Group I) were analyzed for surgical risk only. In 61 patients who underwent palliative procedures between 1966 and 1972 (Group II) a more detailed analysis was done. In this group, 31 had a Potts shunt, 29 a Waterston shunt and 1 a Blalock-Taussig shunt. The most frequently encountered malformation was severe tetralogy of Fallot (30 percent) with or without pulmonary atresia, followed by pulmonary atresia with intact ventricular septum (25 percent). The overall surgical mortality rate in patients seen after 1965 (Group II) was 34 percent compared with the 78 percent mortality rate in patients seen earlier (Group I). The surgical mortality in infants operated on during the 1st week of life was double that of those operated on in the 2nd through 4th weeks. Survivors were compared with nonsurvivors for timing of surgery, age at presentation and clinical profile. Only two significant differences were found. Preoperative continuous murmurs were more common in those who survived operation (13 of 40 patients) than in those who died (2 of 21). Arterial pH during cardiac catheterization was the best predictor of subsequent survival, nonsurvivors having significantly greater metabolic acidosis. Palliation of these severe lesions still carries a high mortality rate to which must be added the risk of subsequent repair. Our data suggest that primary repair is to be preferred if it can be undertaken with a risk approximating that of palliative procedures.

Congenital unilateral pulmonary vein stenosis complicating transposition of the great arteries.

Four patients with transposition of the great arteries and unilateral pulmonary vein (PV) stenosis, all left-sided, were studied. Two patients had an intact ventricular septum (1 with a patent ductus arteriosus), 1 patient had a ventricular septal defect and 1 had a ventricular septal defect with pulmonary stenosis. Clinical signs, such as recurrent pneumonia, unilateral pulmonary edema and pleural effusion, were present preoperatively in 2 patients. Diagnosis was made at cardiac catheterization by cineangiography in 2 patients and at Mustard operation when the PV orifices were inspected in the other 2. PV dilatation was attempted in 3 patients, 1 before correction (age 6 months), 1 during and after it (ages 1 and 3 years, respectively) and 1 during corrective surgery (age 15 months). In the fourth patient only the intracardiac baffle was enlarged near the left PV orifices. In the first patient, at Mustard operation (age 18 months), only a fibrotic PV without an orifice was found; this patient died after surgery. The mean follow-up in the 3 survivors was 3.2 years (range 2 months to 7.6 years). All have severe residual PV obstruction documented by technetium-99m lung perfusion scans that show decreased flow to the left lung (0 to 16% total counts; normal 45%); 2 have unilateral pulmonary edema and 1 has pulmonary artery pressure at systemic level. It is believed that in patients with transposition of the great arteries, left-sided unilateral PV stenosis is a congenital anomaly that becomes progressive as a result of postnatal preferential flow to the right lung.

Coarctation of the aorta or subaortic stenosis with atrioventricular septal defect.

Thirty patients are reported with atrioventricular (AV) septal defect and either coarctation of the aorta (C of A) or subaortic stenosis (SAS) or both. All patients had normal left ventricles as assessed by angiography (21 of 30 patients) or necropsy (9 of 30). Three groups were recognized. Groups I and II included 19 patients with AV septal defect (12 complete, 7 partial) and C of A with or without SAS, 11 patients with AV septal defect (5 complete, 6 partial) and SAS. In Group I, preductal C of A was diagnosed in 16 of 19 patients. Concomitant angiographic evidence of SAS was present in 2 cases, the mechanism being exaggerated anterior displacement of the left AV valve. In Group III, at the time of diagnosis left ventricular-aortic peak systolic pressure gradients of greater than 20 mm Hg were present in 9 patients, 2 of whom had gradients greater than 50 mm Hg. Angiographic diagnoses were: discrete fibrous diaphragm in 4, fibromuscular obstruction in 5, dynamic tunnel in 1, and chordae from left AV valve to LV outflow tract in 1. Thus, SAS in AV septal defect is most often due to a discrete anatomic lesion. Hemodynamic data show that SAS can be progressive, both before and after the surgical management of the AV septal defect.

Analysis of survival in patients with pulmonic valve atresia and ventricular septal defect.

This study reviews the clinical course of 104 consecutive patients with pulmonic valve atresia and ventricular septal (VSD) defect who were diagnosed in the first year of life and followed for a mean period of 4.95 years (range 2 days to 13.75 years). Specific attention was paid to the nature of the pulmonary blood supply and to its influence on patient outcome. Confluent pulmonary arteries supplied by a single ductus arteriosus were present in 72 patients (69%, group I), whereas 32 patients (31%, group II) had a pulmonary blood supply that was partially or exclusively dependent on systemic collateral arteries. An estimate of the probability of survival for 10 years was 69% in the entire cohort, with no different between patients in group I and group II. Definitive surgical repair was performed in 33 of 72 group I patients (46%), compared with 5 of 32 group II patients (16%). Arborization and distribution abnormalities of the pulmonary arteries as well as intrapulmonary stenoses that were exclusively present in patients with systemic collateral arteries (p less than 0.00001) accounted for the significantly lower probability of undergoing corrective surgery in group II patients.

Course of tricuspid atresia in the Fontan era.

Tricuspid atresia is an uncommon form of congenital heart disease and long-term survival was rare before the Fontan era. It was thought that the long-term survival of patients with tricuspid atresia would be improved by the introduction of the Fontan procedure and its subsequent modifications. This study reviews the clinical course of 84 patients with tricuspid atresia identified in the first year of life in the Fontan era. Prior palliative operations, their results and their ultimate application for the Fontan procedure were considered. Eleven patients died before surgical intervention and 5 did not undergo catheterization or echocardiographic confirmation before death. Five children underwent the Fontan procedure without prior palliation and 1 child does not require palliation at the present time. Sixty-seven patients (80%) had surgical procedures before evaluation for the suitability of a Fontan operation. Thirty-four patients had a second surgical palliation and 9 patients had a third palliation. The surgical mortalities for the first, second and third palliative surgery were 17.9, 17.6 and 0%, respectively. Thirty-two patients (38%) underwent the Fontan procedure and 2 deaths occurred (6%). An estimate of the probability of surviving for 1 year was 64% (95% confidence limits 54 to 74%) and that of 8 years was 55% (95% confidence limits 44 to 66%).

Pulmonary vein wedge angiography: indications, results, and surgical correlates in 25 patients.

Pulmonary vein wedge angiography was applied to 25 patients ranging in age from 4 months to 16 years. The indications for this technique include (1) determination of the presence or absence of central (intrapericardial or mediastinal) pulmonary arteries in patients with pulmonary atresia where standard anterograde injections of contrast medium do not demonstrate these, (2) determination of the patency and caliber of a nonvisualized pulmonary artery when there is distal "occlusion" by a systemic-to-pulmonary artery anastomosis, (3) determination of the patency and caliber of a nonvisualized pulmonary artery when previous pulmonary arterial banding has led to acquired pulmonary artery atresia, and (4) determination of the presence of a mediastinal pulmonary artery in patients with so-called isolated congenital unilateral absence of a pulmonary artery. Surgical correlates to the interpretation of pulmonary vein wedge angiograms are provided in 18 patients. The present data suggest that pulmonary vein wedge angiography is a safe technique that provides considerable information about (1) the caliber of the parenchymal pulmonary arteries, (2) their sizes at the hilum of the lung, and (3) the presence or absence of a mediastinal confluence of pulmonary arteries. This technique may also complement those anatomic findings derived from anterograde injection of contrast material in patients with pulmonary atresia of a congenital or acquired nature.

Tricuspid atresia: results of treatment in 160 children.

Experience in managing 160 patients with tricuspid atresia is reviewed. The majority of these children require operative treatment in the 1st year of life. Clinical management of this rather complex anomaly is simplified by classifying each child according to total pulmonary blood flow. An overall plan of management for patients with decreased pulmonary flow is suggested. This consists of a Potts shunt as an initial procedure, limiting its anastomotic growth so that pulmonary flow becomes inadequate at age 10 to 12 years. A Glenn anastomosis is then constructed as the second procedue. The Fontan procedure should be considered carefully as an alternative to the Glenn anastomosis in these older children. Children with increased pulmonary blood flow and transposition of the great arteries are likely to require pulmonary arterial banding in infancy. Otherwise, patients in this smaller group are managed with the same overall plan. Results of long-term palliation have been good.

Complete transposition of the great arteries and coarctation of the aorta.

Thirty-two patients with complete transposition of the great arteries (TGA) and coarctation of the aorta (C of A) were seen at The Hospital for Sick Children, Toronto, Canada, between 1963 and 1983. Three patients had only mild C of A and have not required coarctectomy (Group I); 29 had a severe form of C of A (Group II). Two patients in Group I and 21 in Group II had a ventricular septal defect. Subaortic obstruction was present in 5 patients in Group II. The mechanisms included anterior deviation of the infundibular ventricular septum, anomalous right ventricular muscle bundles, and abnormal ventriculoinfundibular fold. Five patients in Group II had a hypoplastic right ventricle. Coarctectomy was performed in 25 patients, and 5 died (20% mortality rate). Sixteen patients had repair for TGA (13 Mustard, 2 Jatene , 1 Rastelli), and 2 died (12% mortality rate). Life-table analysis shows that only 68% of the patients with TGA and C of A survived the first month of life. The 5-year survival in this group was 57%. In the same period, 94% of patients with uncomplicated TGA survived the first month of life and the 5-year survival rate was 89%.

Bilateral ductus arteriosus (or remnant): an analysis of 27 patients.

Bilateral ductus arteriosus (DA) was clinically recognized in 27 patients studied angiographically from 1963 through May 1983. Distal bilateral DA origin of non-confluent pulmonary arteries was identified in 15 patients, ectopic or distal ductal origin of 1 pulmonary artery in 9 patients (5 without evidence of intracardiac disease) and isolation of the left subclavian artery in 3 (all 3 of whom had a right aortic arch). Other conditions reported to be associated with bilateral DA include interruption of the aortic arch with isolation of a subclavian artery, aortic atresia with interruption of the aortic arch in which bilateral DA supports the entire systemic circulation, bilateral DA complicating forms of congenitally malformed hearts other than those just stated, and, rarely, bilateral DA in isolation. Understanding the symmetric or paired nature of the primitive aortic arch system in the developing human heart facilitates recognition of the patterns of fourth and sixth arch anomalies seen with bilateral DA.

Interrupted aortic arch and ventricular septal defect. Direct repair through a median sternotomy incision in a 13-day-old infant.

A type B interrupted aortic arch was successfully repaired in a 13-day-old infant during profound hypothermia and circulatory arrest. Through a median sternotomy incision it was possible to resect a patent ductus arteriosus and mobilize the descending thoracic aorta for anastomosis to the side of the ascending aorta. At the same time a large ventricular septal defect (VSD) and a small atrial septal defect were closed through the right atrium. Cardiac catheterization 5 months after operation showed a small persistent VSD with a pulmonary to systemic blood flow ratio of 1.1/1. The systolic gradient between the ascending and descending aorta was 20 mm. Hg.

Pulmonary artery banding.

Two hundred nine children with congenital heart defects characterized by excessive pulmonary blood flow underwent pulmonary artery banding at The Hospital for Sick Children from January, 1972, through December, 1982. The indications for banding, rather than complete repair, varied with the type of cardiac defect as well as with the year of presentation to our hospital. A simplified method of estimating required band circumference has been developed for infants with ventricular septal defect, with or without major intracardiac mixing disorders. Infants with simple defects without intracardiac bidirectional mixing disorders receive a band at a circumference of 20 mm + 1 mm for each kilogram of body weight, whereas infants with bidirectional mixing disorders receive a band at a circumference of 24 mm + 1 mm for each kilogram of body weight. The overall operative mortality varies with the underlying cardiac defect and with associated medical conditions but is relatively low in the less-complicated cases. The use of a formula to predict a starting band circumference, with loosening only as required by cyanosis or bradycardia, allows predictable control of congestive symptoms and pulmonary hypertension in the majority of infants. The cumbersome measurement of pulmonary artery pressure and the unpredictable changes in pressure during anesthesia are avoided. Pulmonary artery banding remains an effective means of achieving satisfactory palliation in infants with congenital heart disease and excessive pulmonary blood flow.