Treatment of patients with transposition of great arteries and pulmonary vascular obstructive disease

Twenty-two patients with transposition of the great arteries with or without ventricular septal defect and one with double outlet right ventricle, d-malposition, and severe pulmonary vascular obstructive disease were treated surgically. All were cyanosed and had very limited exercise tolerance. Preoperatively, systemic arterial oxygen saturation (SaO2) varied from 45 to 79% (mean 65), haemoglobin was 13 to 23 g/dl (mean 19). Pulmonary arteriolar resistance was 6.4 to 35 units m2 (mean 17). In the patients with a ventricular septal defect the Mustard operation was done without closure of the ventricular septal defect, and in the 3 patients with intact ventricular septum the Mustard operation was combined with creation of a ventricular septal defect. All patients survived the operation and improved. Postoperative SaO2 ranged from 75 to 96% (mean 89) and haemoglobin from 10.6 to 17.8 g/dl (mean 14.0). This improvement was significant (P less than 0.05). Five patients have had a postoperative cardiac catheterisation. The pulmonary arteriolar resistance remains high in all. Postoperative follow-up varies from 4 to 40 months (mean 14 months). So far there have been no late deaths and all patients remain improved.     

Treatment of patients with transposition of great arteries and pulmonary vascular obstructive disease.

Twenty-two patients with transposition of the great arteries with or without ventricular septal defect and one with double outlet right ventricle, d-malposition, and severe pulmonary vascular obstructive disease were treated surgically. All were cyanosed and had very limited exercise tolerance. Preoperatively, systemic arterial oxygen saturation (SaO2) varied from 45 to 79% (mean 65), haemoglobin was 13 to 23 g/dl (mean 19). Pulmonary arteriolar resistance was 6.4 to 35 units m2 (mean 17). In the patients with a ventricular septal defect the Mustard operation was done without closure of the ventricular septal defect, and in the 3 patients with intact ventricular septum the Mustard operation was combined with creation of a ventricular septal defect. All patients survived the operation and improved. Postoperative SaO2 ranged from 75 to 96% (mean 89) and haemoglobin from 10.6 to 17.8 g/dl (mean 14.0). This improvement was significant (P less than 0.05). Five patients have had a postoperative cardiac catheterisation. The pulmonary arteriolar resistance remains high in all. Postoperative follow-up varies from 4 to 40 months (mean 14 months). So far there have been no late deaths and all patients remain improved.     

The palliative Mustard operation for double outlet right ventricle or transposition of the great arteries associated with ventricular septal defect, pulmonary arterial hypertension, and pulmonary vascular obstructive disease. A report of eight patients.

Five patients with double outlet right ventricle, ventricular septal defect, pulmonary arterial hypertension and pulmonary vascular obstructive disease and three patients with complete d-transposition of the great arteries, ventricular septal defect, pulonary arterial hypertension and pulmonary vascular obstructive disease underwent an elective Mustard baffle operation. The ventricular septal defect was not closed. A large patent ductus arteriosus was divided in three patients. Seven of the eight patients are alive five to 32 months after surgery; one patient died 11 months after surgery. Cyanosis, dyspnea on exertion, and exercise limitation improved initially in all and has persisted in the survivors. In pre and postoperative hemodynamic studies in four patients, systemic arterial oxygen saturation and effective pulmonary blood flow increased from mean values of 70% to 90% and 1.7/min/m2 to 3.3 L/mon/m2, respectively. Absolute systemic and pulmonary flows, and pressures and resistances, were not significantly altered. Criteria for selection of patients with transposition of the great arteries of double outlet right ventricle who would benefit from a palliative Mustard procedure (Mustard atrial baffle without closure of the ventricular spetal defect) are: 1) severe symptoms; 2) pulmonary arteiral hypertension (75% systemic) with pulmonary vascular obstructive disease; and 3) pulmonary artieral oxygen saturation greater than systemic (ascending aorta) arterial oxygen saturation by approximately 10%.     

Repair of conotruncal abnormalities with the use of the valved conduit: improved early and midterm results with the cryopreserved homograft.

Repair of complex cardiac lesions has been facilitated by the availability of valved conduits to reestablish right ventricular to pulmonary artery continuity. From 1977 to June 1991, 148 patients underwent repair with insertion of a conduit. Their mean age was 6.6 years (11 days to 45 years). The diagnosis was transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction in 51, truncus arteriosus in 36, pulmonary atresia with ventricular septal defect in 25, tetralogy of Fallot in 19, double-outlet right ventricle in 10, pulmonary atresia with intact ventricular septum in 6 and atrioventricular canal with pulmonary atresia in 1. A Dacron porcine-valved conduit was used in 37, a homograft conduit in 106 and a nonvalved conduit in 5. There were 13 early deaths overall (8.8%); 8 (22%) of the early deaths occurred in the 37 patients who received a Dacron graft, 4 (3.8%) occurred in the 106 patients who received a homograft and 1 occurred in a patient with a nonvalved Gore-Tex conduit. An additional patient underwent orthotopic heart transplantation in the early postoperative period. In 117 patients operated on from January 1985 to June 1991 the early mortality rate was 2.6% (3 of 117). Among 28 patients receiving a Dacron porcine-valved graft there were two late deaths (7.1%) after a mean follow-up interval of 93 months, and 8 patients required reoperation for conduit obstruction. Among 102 homograft recipients there were two late deaths (1.9%).(ABSTRACT TRUNCATED AT 250 WORDS)     

Surgical options for complex transposition of the great arteries

Between September 1976 and November 1987, 53 patients underwent surgical treatment by the same surgeon for "complex transposition of the great arteries" with ventricular septal defect or severe left ventricular outflow tract obstruction, or both. Six patients with transposition and left ventricular outflow tract obstruction underwent atrial rerouting and direct relief of the left ventricular outflow tract obstruction. Twenty-two patients presented with transposition plus ventricular septal defect; 15 of these patients underwent atrial rerouting and ventricular septal defect closure and 7 underwent an arterial switch procedure. Twenty-five patients presented with transposition plus ventricular septal defect and left ventricular outflow tract obstruction, 23 of whom underwent a Rastelli procedure. There were one early death (mortality rate 1.9%; 90% confidence limits 0-7%) and three late deaths (mortality rate 5.8%) during a mean follow-up period of 42 months (range 2 to 124). These results show that 1) atrial rerouting is an appropriate surgical procedure for transposition of the great arteries with left ventricular outflow tract obstruction; 2) the arterial switch procedure provides excellent early correction of transposition with ventricular septal defect and is currently the preferred procedure for this lesion; and 3) the Rastelli procedure can be performed with a low early mortality rate and excellent long-term results for transposition with ventricular septal defect and left ventricular outflow tract obstruction.     

Congenital absence of pulmonary valve leaflets.

Congenital absence of pulmonary valve leaflets is an uncommon condition usually associated with ventricular septal defect and an obstructive pulmonary valve ring. Twenty-one patients with these malformations are described. Twenty had an associated ventricular septal defect with ventriculoarterial concordance, and one also had transposition of the great arteries, ventricular septal defect, and obstructive pulmonary valve ring. The clinical features, cardiac catheterisation findings, and angiocardiographic results are presented. Twelve patients underwent cardiac surgery. Three patients died, one in the early, and the other two in the late postoperative period. The results, according to the surgical technique employed and postoperative cardiac catheterisation findings, showed that patients in whom the bioprostheses were implanted in the pulmonary position had a better late follow-up.     

Subaortic obstruction after the use of an intracardiac baffle to tunnel the left ventricle to the aorta.

Postoperative hemodynamic studies in five patients document subaortic obstruction after surgical repair utilizing an intracardiac baffle to establish continuity between the left ventricle and the aorta. Four of the patients had a Rastelli procedure for D-transposition of the great arteries with a ventricular septal defect and pulmonary stenosis; one patient had repair of double outlet right ventricle with a ventricular septal defect and pulmonary stenosis. The left ventricular outflow was shown to be a long narrow tunnel by angiography in four of five patients and by echocardiography in one patient. Resting aortic peak systolic pressure gradient ranged from 10 to 42 mm Hg (mean 24). The obstruction was localized to the proximal end of the left ventricule to aorta tunnel (i.e., at the site of ventricular septal defect) in five patients. One patient with a gradient of 42 mm Hg has angina and decreased exercise tolerance. Subaortic obstruction is a newly described sequelae after the Rastelli procedure for transposition or repair of double outlet right ventricle. The obstruction may be hemodynamically significant and should be searched for at postoperative cardiac catheterization.     

Doppler echocardiographic analysis of the transmitral flow velocity profile in patients with ventricular septal defects

Using continuous-wave Doppler echocardiography, we evaluated the mitral flow velocity pattern in 30 ventricular septal defect patients, 11 of whom had severe pulmonary vascular obstructive disease (Group I); 10 of whom had severe pulmonary hypertension without pulmonary vascular obstructive disease (Group II); and 9 of whom had no pulmonary hypertension and hemodynamically unimportant left-to-right shunts (Group III). In addition, 25 healthy subjects (Group IV) were studied for comparative purposes. The peak velocity of early left ventricular filling (E) was significantly lower in Group I than in all the other groups (p < 0.01). The peak velocity of late left ventricular filling (A) was significantly higher (p < 0.01) in Group I than in Group III, or than in normal individuals (Group IV) (p < 0.01). The ratio A/E was the most prominent difference between Group I patients and the other groups, with Group I having a significantly higher ratio (p < 0.01), which was 1 or greater in 9 of 11 patients. In contrast, none of the remaining ventricular septal defect patients or normal subjects had an A/E ratio of 1 or greater. Group II had increased mitral flow velocities, while Group III had normal mitral flow velocity profiles. A positive correlation between the magnitude of the left-to-right shunt and early mitral flow velocity peak (r = 0.86) and late peak (r = 0.81) was found, regardless of the degree of pulmonary hypertension. These results indicate that significant alterations of the mitral flow velocity pattern, which mimic the abnormalities associated with impaired left ventricular diastolic function (A/E ratio of 1 or greater), occur in ventricular septal defect patients who have severe pulmonary vascular obstructive disease. The transmitral velocity profiles in the ventricular septal defect patients without severe pulmonary vascular obstructive disease were similar to those of the normal patients, although the values relative to the degree of left-to-right shunting were higher in the ventricular septal defect patients.     

Late death after arterial switch operation for transposition of the great arteries.

Fifty-nine patients survived for more than 1 month after an arterial switch operation (ASO). Diagnoses in these patients included transposition of the great arteries in 27, transposition of the great arteries with ventricular septal defect in 28, and double-outlet right ventricle in four. There were six late deaths (10%) during the follow-up period, and all of them occurred suddenly and unexpectedly. Four of the six late deaths were in patients who had undergone ASO in the neonatal period. Late deaths occurred from 40 days to 10 months after the operation. Autopsies were performed in all six patients. The cause of these late deaths was acute myocardial infarction. Five patients died of subendocardial infarction resulting from stenosis of the left main coronary artery. On pathologic examination, a fibrocellular intimal thickening was noted at the proximal region of the right and left coronary arteries, which resulted in 80% stenosis on average.     

Midterm results after the arterial switch operation for transposition of the great arteries with intact ventricular septum: clinical, hemodynamic, echocardiographic, and electrophysiologic data

Although the short-term results of atrial level repair of transposition of the great arteries (TGA) are satisfactory, longer follow-up has disclosed a significant incidence of systemic right ventricular dysfunction and rhythm disturbances. The arterial switch operation (ASO) may represent a major improvement by restoring the left ventricle as the systemic ventricle and avoiding extensive atrial surgery. We have prospectively evaluated 49 consecutive survivors of ASO for TGA with intact ventricular septum (IVS) by clinical examination, echocardiography, cardiac catheterization, ambulatory electrocardiographic monitoring, and invasive electrophysiologic studies. The mean length of follow-up has been 29 +/- 14 (SD) months after surgery. All children are currently asymptomatic and on no medications. Severe supravalvular pulmonary stenosis (greater than 60 mm Hg) was present in five children, all of whom have undergone reoperation. No patient has severe supravalvular aortic obstruction. Mild degrees of supravalvular pulmonary or aortic obstruction have not progressed. Seven children (14%) have trivial or mild aortic regurgitation. Two children have proximal occlusion of the left anterior descending coronary artery with adequate retrograde collateral perfusion. One child had an electrocardiographic pattern of inferior myocardial infarction without evidence of ventricular dysfunction. Systemic (left) ventricular function is normal as measured by end-diastolic pressure (mean 7 +/- 6 mm Hg), ejection fraction (mean 68 +/- 6%), end-diastolic volume (mean 101 +/- 22% of predicted normal), and cardiac index (mean 4.7 +/- 1.3 liters/min/m2). Only one patient has sinus node dysfunction. There have been no late deaths. These early results are encouraging. We conclude that the arterial switch operation is currently the procedure of choice for neonates with TGA and IVS.     

Transposition of the great arteries: the arterial switch operation

Primary arterial switch operation in neonates for repair of transposition of the great arteries with an intact ventricular septum or with a ventricular septal defect has become the operation of choice in our institution. By now, the surgical risk has been decreased to approximately 2 per cent. Early in our experience, postoperative supravalvar pulmonary stenosis was the only late hemodynamic complication of significance. Postoperative left ventricular function, including echocardiographic indices of left ventricular function, including echocardiographic indices of left ventricular systolic and diastolic function, have been within normal range in all patients. Almost all patients are in sinus rhythm as late as 6 years of the operation. It is our contention that primary arterial switch operation in infants with transposition of the great arteries results in improved left ventricular performance; the potential hazard of late coronary insufficiency and of aortic valve dysfunction requires continued monitoring of these patients.     

BMPR2 mutations in pulmonary arterial hypertension with congenital heart disease.

The aim of the present study was to determine if patients with both pulmonary arterial hypertension (PAH), due to pulmonary vascular obstructive disease, and congenital heart defects (CHD), have mutations in the gene encoding bone morphogenetic protein receptor (BMPR)-2. The BMPR2 gene was screened in two cohorts: 40 adults and 66 children with PAH/CHD. CHDs were patent ductus arteriosus, atrial and ventricular septal defects, partial anomalous pulmonary venous return, transposition of the great arteries, atrioventicular canal, and rare lesions with systemic-to-pulmonary shunts. Six novel missense BMPR2 mutations were found in three out of four adults with complete type C atrioventricular canals and in three children. One child had an atrial septal defect and patent ductus arteriosus; one had an atrial septal defect, patent ductus arteriosus and partial anomalous pulmonary venous return; and one had an aortopulmonary window and a ventricular septal defect. Bone morphogenetic protein receptor 2 mutations were found in 6% of a mixed cohort of adults and children with pulmonary arterial hypertension/congenital heart defects. The current findings compliment recent reports in mouse models implicating members of the bone morphogenetic protein/transforming growth factor-beta pathway inducing cardiac anomalies analogous to human atrioventricular canals, septal defects and conotruncal congenital heart defects. The small number of patients studied and the ascertainment bias inherent in selecting for pulmonary arterial hypertension require further investigation.     

Long-term results of the "palliative" Mustard operation

A long-term follow-up study after 41 palliative Mustard operations for transposition of the great arteries and pulmonary vascular obstructive disease is presented. The operations were performed from 1973 to 1980. Mean pulmonary arteriolar resistance was 13.96 +/- 6.7 Um2. A ventricular septal defect was not closed in 34 patients; in 7 it was created at operation. There were three hospital and two late deaths. Survivors were followed up for 3 to 10 years (mean 76.7 +/- 22.8 months). Before operation 4 children were in New York Heart Association functional class IV and 33 were in class III. Mean hemoglobin concentration was 19.43 +/- 3.14 g/dl and arterial oxygen saturation was 63.44 +/- 11.29%. After operation 18 patients were in functional class I, 17 in class II and only 1 in class III. Hemoglobin level decreased to 14.19 +/- 2.3 g/dl and arterial oxygen saturation improved to 89.12 +/- 7.25%. Cardiac catheterization in 21 survivors confirmed no change in pulmonary artery pressure and resistance. Effective pulmonary blood flow improved from 1.39 +/- 0.39 before to 2.6 +/- 0.78 liters/min per m2 after operation. The palliative Mustard operation carries a low risk and provides excellent symptomatic improvement up to 10 years in patients with transposition of the great arteries and pulmonary vascular disease.     

Long-term outcome of patients operated for large ventricular septal defects with increased pulmonary vascular resistance

BACKGROUND: There is a paucity of data regarding the long-term outcome of patients operated for ventricular septal defect with severe pulmonary arterial hypertension and elevated pulmonary vascular resistance. METHODS AND RESULTS: We evaluated the long-term follow-up results of a selected cohort of patients with nonrestrictive ventricular septal defect and elevated pulmonary vascular resistance (>6 Wood units). Thirty-eight patients, median age 7.5 years (range 6 months-27 years), with nonrestrictive ventricular septal defect with severe pulmonary hypertension were operated between 1985 and 1996 at our institute. Preoperative pulmonary vascular resistance, ratio of pulmonary blood flow to systemic blood flow, and ratio of pulmonary vascular resistance to systemic vascular resistance were 7.63+/-1.8 Wood units, 1.9+/-0.48, and 0.41+/-0.12, respectively. The majority (68.4%) had perimembranous ventricular septal defect. Thirty patients (79%) had a good outcome and were asymptomatic at a mean follow-up of 8.7 years, with significant reduction in pulmonary artery pressures. Eight patients (21%) had a poor outcome, which included 5 immediate postoperative deaths, 1 late death and 2 surviving patients with persistent severe pulmonary arterial hypertension. There was no significant difference regarding hemodynamic parameters at baseline between those who had a good outcome and those who did not. Eleven patients with a preoperative pulmonary blood flow to systemic blood flow ratio of <2:1. who had a good outcome following surgery, underwent repeat catheterization at follow-up. There was a significant reduction in their mean pulmonary vascular resistance (8.03+/-1.4 v. 4.16+/-1.6 Wood units, p=0.001) and pulmonary vascular resistance to systemic vascular resistance ratio (0.41+/-0.12 v. 0.19+/-0.06, p=0.05). CONCLUSIONS: The late results of surgery on this selected group of patients with nonrestrictive ventricular septal defect with high pulmonary vascular resistance are encouraging. Operative correction of the ventricular septal defect should be actively considered in all children presenting with nonrestrictive ventricular septal defect with a significant left-to-right shunt, despite moderately elevated pulmonary vascular resistance. Even among older patients with ventricular septal defect and moderately elevated pulmonary vascular resistance, there is a specific group that does well after operation.     

Immediate and mid-term results of complete repair of truncus arteriosus during the first year of life

Twenty five infants with truncus arteriosus underwent complete surgical correction in the first year of life between January 1984 and June 1990 at Marie Lannelongue Hospital. All had cardiac failure and pulmonary hypertension. Another severe cardiac malformation was present in 6 cases. Complete repair was carried out under cardiopulmonary bypass with moderate hypothermia. After closing the ventricular septal defect the continuity of the right ventricle and pulmonary artery was reestablished by a valved Dacron conduit with a bioprosthesis (13 patients), by an autologous pericardial conduit with the same type of prosthesis (5 patients), by a valveless conduit (1 patient) or by direct insertion of the pulmonary artery (6 patients). Eight children (32%) died shortly after surgery. Seventy one per cent of children operated in the first month of life died compared with only 17% of those operated after one month of life (p less than 0.05). The seventeen survivors have been followed up for an average of 21 +/- 22 months. Three secondary deaths were observed at 33 days, 2 and 10 months after surgery: the first child died of left ventricular failure and pulmonary vascular disease related to the complexity of the associated cardiac malformations; the other 2 deaths were unexpected. The one and three year survival rate is 54%. Pulmonary stenosis with a systolic pressure gradient of more than 30 mmHg was found in 7 patients of whom 6 had valved Dacron conduits (p less than 0.01). One child was successfully operated 60 months after the total correction and another child is on the waiting list for reoperation 69 months after the total correction.(ABSTRACT TRUNCATED AT 250 WORDS)     

Anatomical correction for complete transposition and double outlet right ventricle: intermediate assessment of functional results

Thirty three patients were followed up after anatomical correction of transposition of the great arteries or double outlet right ventricle and subpulmonary ventricular septal defect (Taussig-Bing anomaly). There were no late deaths and clinical progress was excellent. Cardiac catheterisation was performed in 17 patients two weeks to 44 months after operation. There were six patients with simple transposition, six with complete transposition and large ventricular septal defect, and five with the Taussig-Bing anomaly. Pressure gradients across the right ventricular outflow tract ranged from 5 to 72 mm Hg, being greater than 40 mm Hg in five patients. No patient was shown to have important valvar regurgitation and in 15 patients the coronary anastomoses were widely patent. Left ventricular function was assessed from digitised ventriculograms. Ventricular volume, shape, and ejection fraction were all normal for the group although patients with complex transposition showed a significantly lower mean (SD) ejection fraction than those with simple transposition (62(9) vs 77(9]. Analysis of regional wall motion showed a totally normal pattern in four patients; however, in seven patients a characteristic abnormality of anterior hypokinesis with delayed onset of inward wall motion was seen. Anatomical correction of transposition of the great arteries and the Taussig-Bing anomaly can be performed with satisfactory anatomical and functional results. The implications of the left ventricular wall motion abnormalities is unknown.     

Anatomical correction for complete transposition and double outlet right ventricle: intermediate assessment of functional results.

Thirty three patients were followed up after anatomical correction of transposition of the great arteries or double outlet right ventricle and subpulmonary ventricular septal defect (Taussig-Bing anomaly). There were no late deaths and clinical progress was excellent. Cardiac catheterisation was performed in 17 patients two weeks to 44 months after operation. There were six patients with simple transposition, six with complete transposition and large ventricular septal defect, and five with the Taussig-Bing anomaly. Pressure gradients across the right ventricular outflow tract ranged from 5 to 72 mm Hg, being greater than 40 mm Hg in five patients. No patient was shown to have important valvar regurgitation and in 15 patients the coronary anastomoses were widely patent. Left ventricular function was assessed from digitised ventriculograms. Ventricular volume, shape, and ejection fraction were all normal for the group although patients with complex transposition showed a significantly lower mean (SD) ejection fraction than those with simple transposition (62(9) vs 77(9]. Analysis of regional wall motion showed a totally normal pattern in four patients; however, in seven patients a characteristic abnormality of anterior hypokinesis with delayed onset of inward wall motion was seen. Anatomical correction of transposition of the great arteries and the Taussig-Bing anomaly can be performed with satisfactory anatomical and functional results. The implications of the left ventricular wall motion abnormalities is unknown.     

Rastelli procedure for transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction. Early and late results in 41 patients (1971 to 1978).

Forty-one children with transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction underwent a Rastelli operation between 1971 and 1978. A homograft valve preserved in an antibiotic solution and extended with A dacron tube was the conduit of choice. Alternatively, conduits with porcine heterografts or valves constructed from calf pericardium were used. They were positioned to the left of the aorta whenever possible. The intraventricular tunnel from the left ventricle to the aorta was constructed from Dacron velour. There were four early and seven late deaths. The last 13 consecutive patients have survived. Early deaths were related to unfavourable anatomy, conduit compression, and sepsis. Residual ventricular septal defects and postoperative infection were the main factors contributing to the late deaths.     

25 years after the first arterial switch procedure: mid-term results

BACKGROUND: 25 years after the first successful arterial switch operation (ASO) for complete transposition of the great arteries (TGA), it is possible to evaluate the mid-term results after this procedure. PATIENTS AND METHODS: From March 1986 through December 1993, 188 patients with TGA underwent ASO (primary in 181 patients and secondary after previous banding of the pulmonary artery in 7 patients). Of the 181 patients with primary ASO, 152 had simple TGA or TGA with a small ventricular septal defect, 90 patients had TGA with a large ventricular septal defect, and 10 had a Taussig-Bing complex. Unselected subgroups of the patients were specifically examined with respect to general health, cognitive and motor development, exercise tolerance and cardiological status. Follow-up time was over 5 years except for the cardiac catheterizations, which were performed one year after ASO. RESULTS: Early mortality was 6.4% in the total group and 5.5% in the 181 patients with primary ASO. 5 patients (2.7%) died late and 5 patients needed reoperation during the follow-up period. Cumulative survival for the whole group was 91% at 5 and 10 years after ASO. 96% of the patients were unlimited in their physical activity, and 99% received no medication. Intelligence was not different from a normal population, but motor and neurological impairment was more frequent (23% and 9%, respectively). The Bruce treadmill test was normal in 47 of 50 patients, but provoked signs of myocardial ischemia or salvos of ventricular tachycardia in 3. ECG and Holter ECG showed normal sinus rhythm in 94.5%, important ventricular ectopy in 2.8% and ischemic changes in 1.4%. Right heart catheterization in 67 patients revealed flattening of the pulmonary trunk and frequently mild or rarely moderate pulmonary artery branch stenosis. Left heart catheterization in 71 patients demonstrated significant enlargement of the aortic annulus and root as well as frequent mild aortic insufficiency. One child had unexpected coronary artery occlusion,and 2 showed a hypoplastic left anterior descending coronary artery. In all patients except one, left ventricular ejection fraction was normal, but 6 patients had regional wall motion abnormalities. CONCLUSION: These results and those of other groups are encouraging, but prospective studies over a much longer follow-up time are necessary before definite conclusions can be drawn.     

Surgical experience with congenital heart disease in Down's syndrome

Children with Down's syndrome and congenital heart defects have multiple problems. The role of cardiac surgery in the management of these patients was investigated by reviewing the clinical data, hospital course and follow-up of 21 patients (9 males and 12 females, age range 1 month to 14 years) with Down's syndrome and congenital heart defects operated in our institute. Twelve (57%) of these were infants and nine (43%), older children. Five were in congestive cardiac failure, four were hypothyroid. The heart lesions ranked in incidence as follows: atrioventricular septal defect 7 (33.3%), tetralogy of Fallot 3 (14.3%), tetralogy of Fallot & atrioventricular septal defect both 2 (9.5%), double outlet right ventricle with pulmonary stenosis 1 (4.8%), patent ductus arteriosus 2 (9.5%), patent ductus arteriosus plus coarctation 1 (4.8%), ventricular septal defect 2 (9.5%), atrial septal defect plus ventricular septal defect 1 (4.8%), atrial septal defect plus patent ductus arteriosus plus right pulmonary artery stenosis 1 (4.8%) and transposition of great arteries with multiple ventricular septal defect 1 (4.8%). Four (19%) patients had palliative procedures while the rest (81%) underwent primary repair. All survived the operation. The post-operative period was complicated in 6 (28.5%), with respiratory infections in 3, pulmonary hypertensive crisis in 2 and complete heart block in 1. The early mortality was 0, while there were 2 (9.5%) late deaths. The number of hospitalisations was markedly reduced according to the parents. Follow-up showed near normal pulmonary artery pressure in 50 percent children with large shunts and a good developmental spurt was seen in 60 percent. From a purely surgical viewpoint, the prognosis for children with Down's syndrome and congenital heart disease is good.