Mustard operation and creation of ventricular septal defect in two patients with transposition of the great arteries, intact ventricular septum and pulmonary vascular disease.

The condition of patients with transposition of the great arteries, intact ventricular septum and severe pulmonary vascular disease is inoperable with present techniques. In a series of 260 surgically treated patients with transposition of the great arteries and intact ventricular spetum, 5 had severely increased pulmonary vascular resistance, and all 5 died; postmortem examination confirmed the presence of severe pulmonary vascular disease. The concept of the "palliative Mustard" procedure was applied in two children with transposition of the great arteries, intact ventricular spetum and pulmonary vascular disease who underwent the Mustard operation with creation of a ventricular septal defect. The postoperative course was uneventful in both patients. Thirteen and 5 months, respectively, after operation, both are physically active and have respective arterial oxygen saturation levels of 94 and 92 percent.     

Pulmonary blood flow distribution in transposition of the great arteries

Pulmonary blood flow distribution was studied by scintillation scanning of the lungs after the infusion of iodine-131-labeled macroaggregates of human albumin before and after the Mustard operation in 53 patients with transposition of the great arteries. The patients were classified as follows: Group I (24 infants with uncomplicated transposition of the great arteries); Group II (18 patients with transposition and ventricular septal defect); and Group III (11 patients with transposition, ventricular septal defect and pulmonary obstruction).Before operation, 21 patients had a normal distribution of pulmonary blood flow, 10 had preferential flow to the right lung and 2 had preferential flow to the left lung. After operation, 19 had a normal pattern of pulmonary blood flow, 21 had preferential flow to the right lung and 3 had preferential flow to the left lung. The scanning studies have proved helpful in follow-up of patients to rule out recurrence of the shunt, pulmonary or systemic venous obstruction, development of pulmonary hypertension and occlusion of a palliative systemic-pulmonary shunt.     

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.     

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%.     

Mustard operation in the 1st month of life

Since April 1976, nine infants less than 1 month of age underwent a Mustard intraatrial baffle procedure. All had transposition of the great arteries and were persistently symptomatic or hypoxic after the Rashkind balloon atrioseptostomy. Two had an associated large ventricular septal defect patched at the time of physiologic correction. The average age of the infants was 11.6 days (range 36 hours to 28 days). Weight averaged 3.4 kg (range 2.5 to 4.2). In all the Mustard procedure was performed using deep hypothermie circulatory arrest, averaging 71 minutes (range 48 to 88) at a mean core temperature of 13 °C. An average of 2.8 days of postoperative ventilatory assistance was required. One death occurred in a 28 day old male infant with an associated ventricular septal defect who, the morning after operation, had a sudden unresponsive cardiac standstill. The postoperative hospitalization period averaged 15 days (range 10 to 23). Follow-up evaluation has extended from 4 to 63 months (average 31). Six patients were restudied 2 to 21 months postoperatively. One had obstruction of the superior limb of the baffle, which had separated from the right atrial wall permitting a modest right to left shunt. She has had uneventful repair employing a unique modification of Senning's operation. Another child has asymptomatic partial obstruction of the superior limb of the baffle demonstrated angiographically. None has pulmonary venous obstruction and all have prevailing normal sinus rhythm. Significant right ventricular dysfunction has been demonstrated in one. Early correction in these persistently symptomatic or hypoxic neonates with transposition of the great arteries with or without a ventricular septal defect has obviated the need for palliative operations and produced gratifying early and late results.     

Is the function of all cardiac valves after the arterial switch operation influenced by an associated ventricular septal defect?

A ventricular septal defect in transposition of the great arteries is frequently closely related to the cardiac valves. The valvar function after arterial switch operation of patients with transposition of the great arteries and ventricular septal defect or intact ventricular septum was compared. We analysed the function of all cardiac valves in patients who underwent the arterial switch operations pre- and post-operatively, 1 year after the procedure and on follow-up. The study included 92 patients - 64 with transposition of the great arteries/intact ventricular septum and 28 with transposition of the great arteries/ventricular septal defect. The median age at surgery was 5.5 days in transposition of the great arteries/intact ventricular septum (0-73 days) and 7.0 days in transposition of the great arteries/ventricular septal defect (4-41 days). Follow-up was 51.7 months in transposition of the great arteries/intact ventricular septum (3.3-177.3 months) and 55 months in transposition of the great arteries/ventricular septal defect (14.6-164.7 months). Neo-aortic, neo-pulmonary, and mitral valvar function did not differ. Tricuspid regurgitation was more frequent 1 year post-operatively in transposition of the great arteries/ventricular septal defect (n = 4) than in transposition of the great arteries/intact ventricular septum. The prevalence of neo-aortic regurgitation and pulmonary stenosis increased over time, especially in patients with transposition of the great arteries/intact ventricular septum. The presence of a ventricular septal defect in patients undergoing arterial switch operation for transposition of the great arteries only has a minor bearing for the development of valvar dysfunction on the longer follow-up.     

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.     

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.     

Anatomical correction of transposition of great vessels. Considerations on the first 50 patients operated on and analysis of the surgical choice

After having adopted the arterial switch operation as an alternative to the Mustard operation we decided to review our results with this protocol in order to evaluate the validity of our choice. From June 1984 to Oct. 1989 a total of 50 patients underwent an arterial switch operation in our department: 36 (72%) with simple transposition of the great arteries and an essentially intact ventricular septum; 9 (18%) with transposition of the great arteries and ventricular septal defect and 5 (10%) with the Taussing-Bing anomaly. In the group with simple transposition, the age ranged between 3 and 24 days (mean 8.7 +/- 4.3 S.D.) and the weight was between 2.4 and 4.9 kg (mean: 3.46 +/- 0.42 S.D.). In the group with transposition of the great arteries and ventricular septal defect the age ranged between 7 and 690 days (mean: 194 +/- 206 S.) and the weight was between 3.1 and 9.7 kg (mean 6 +/- 2.34 S.D.). In the group with Taussig-Bing anomaly the age ranged between 9 and 216 days (means: 132 +/- 75 S.D.) and the weight was between 3.2 and 5.1 kg (mean: 4.1 +/- 0.75 S.D.). A balloon atrial septostomy was performed in 43 (86%) patients. The Lecompte manoeuvre was adopted in 49 (98%) patients. The total early mortality was 10% (5/50). In the 36 neonates with simple transposition of the great arteries, 4 (11.1%) died. Among the 14 patients with transposition of the great arteries and ventricular septal defect and Taussig-Bing anomaly there was 1 (7.1%) death. There was also 1 (2.2%) late death.(ABSTRACT TRUNCATED AT 250 WORDS)     

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.     

Pulmonary vascular disease in complete transposition of the great arteries: A study of 200 patients

Lung specimens of 200 patients with transposition of the great arteries were examined microscopically for evidence of pulmonary vascular disease. In patients with an intact ventricular septum or a small ventricular septal defect, advanced pulmonary vascular disease was uncommon; only 9 of 107 such patients (8.4 percent) demonstrated greater than grade 2 (Heath-Edwards) pulmonary vascular disease. A persistent large patent ductus arteriosus appeared to promote progressive pulmonary vascular disease in this group since each of the five infants less than 1 year of age with grade 3 or 4 disease had this lesion. In contrast, pulmonary vascular disease was common in patients with a large ventricular septal defect; 37 of 93 patients (40 percent) with this defect had greater than grade 2 pulmonary vascular disease. Among patients more than 1 year of age, 26 of 35 (75 percent) had grade 4 disease. The catheterization data suggest that the calculated pulmonary vascular resistance may underestimate the degree of disease, probably by overestimating the pulmonary blood flow (Fick method). Pulmonic stenosis appeared to protect the lungs from progressive pulmonary vascular disease, and pulmonary arterial banding was protective when performed before age 6 months. Our studies indicate that a persistent large patent ductus arteriosus should be closed as early as possible in view of its association with advanced pulmonary vascular disease in these patients. In infants with a large ventricular septal defect, pulmonary arterial banding or corrective surgery with closure of the defect should be performed between the ages of 4 and 6 months to prevent progressive pulmonary vascular damage.     

Pulmonary vascular disease in neonates with transposition of the great arteries and intact ventricular septum.

BACKGROUND--Progressive pulmonary vascular disease in surgically unrepaired transposition of the great arteries with or without ventricular septal defect had been frequently described in the past. Occurrence of progressive pulmonary vascular disease has been reported even after atrial switch procedure done at three months of age. With the advent of neonatal surgical repair, this problem is virtually non-existent. There is a small subgroup of infants with transposition of the great arteries who show pulmonary vascular disease in the neonatal period that can adversely affect the surgical outcome. The clinico-pathological correlation in this group of patients was studied. OBSERVATIONS--Three patients, with transposition of the great arteries and intact ventricular septum, who showed histological evidence of pulmonary vascular disease in the neonatal period or early infancy are described. Two of these patients, continued to have poor systemic oxygenation despite adequate atrial communication. One patient had a close ductus arteriosus within the first two hours of birth while on prostaglandin E1 infusion. CONCLUSIONS--In the absence of left ventricular outflow tract obstruction, a poor response to atrial septostomy suggests pulmonary hypertension and pulmonary vascular disease. Antenatal constriction of the ductus arteriosus may contribute to such changes in pulmonary vasculature.     

Hemodynamic function after the mustard operation for transposition of the great arteries

Pre- and postoperative cardiac catheterization data and cinenangiocardiograms of 82 patients who survived the Mustard operation for transposition of the great arteries (TGA) were reviewed. The postoperative catheterizations were performed 20 days to 10 years after operation (mean 2.5 years). Forty-six patients (56%) had no or insignificant associated cardiac lesions, whereas 36 (44%) had ventricular septal defect, pulmonary stenosis, or both, and required surgical intervention at the time of the Mustard operation. Postoperatively, 11 patients (13%) had significant systemic venous obstruction. Of the 11 patients, 6 required reoperation, and 2 patients had evidence of restenosis or complete obstruction in the superior vena cava after reoperation. In most patients, superior vena caval obstruction was well tolerated even in the presence of high pressure in the superior vena cava. Pulmonary venous obstruction occurred in 5 patients (6 % ), 3 of whom had no clinical symptoms despite severe pulmonary venous obstruction, although all had radiographic evidence of pulmonary venous congestion. The incidence of obstruction was drastically reduced after the Mustard operation was modified to include routine enlargement of the pulmonary venous atrium. Tricuspid regurgitation was uncommon (10%), but did occur in patients who had transatrial closure of a ventricular septal defect. Preoperatively, left ventricular outflow obstruction occurred in 38%. In 12 patients an attempt was made to relieve the obstruction at surgery. The 6 patients who had localized obstruction had a good result, but patients with more diffuse narrowing of left ventricular outflow had little or no relief of obstruction. Mild to moderate left ventricular outflow gradients regressed spontaneously in most patients after the Mustard operation.     

Aneurysm of the membranous ventricular septum in transposition of the great arteries.

In patients with transposition of the great arteries (TGA), both the D- and L- forms, an aneurysm of the membranous ventricular septum (AMS) produces subpulmonic stenosis due to the higher right ventricular pressure which forces the aneurysm to protrude into the left ventricular, i.e., subpulmonic, outflow tract. The clinical signs and symptoms, hemodynamic findings as well as surgical results were analyzed in eight patients with TGA and AMS. The presence of an AMS should be suspected from hemodynamic data consisting of a combination of elevated left ventricular pressure, gradient across the left ventricular outflow tract and presence of a small ventricular septal defect with or without pulmonary artery hypertension. The AMS can be demonstrated by a right ventricular injection in the lateral view. The anomaly needs to be corrected at the time of the Mustard procedure. If uncorrected it may lead to postoperative death or progressive obstruction. In the presence of an aneurysm even small ventricular septal defects should be closed by a patch and the aneurysm should be excised.     

Surgical repair of congenitally corrected transposition of the great arteries: Results and follow-up

Eighteen patients with congenitally corrected transposition of the great arteries had open heart repair for intracardiac associated defects. Fourteen patients (78%) are alive during the follow-up period (mean 4.5 years). Seventeen (94%) of the 18 patients had ventricular septal defect closure, and 12 (66%) insertion of a pulmonary artery conduit. Surgical repair of the tricuspid valve was required in 6 patients (33%) during the first operation and in 3 additional patients during a second operation (total 50%). When hemodynamic overload or cardiac compromise was detected after surgery it was directly related to identifiable residual defects such as atrioventricular valvular insufficiency, residual ventricular septal defect, or pulmonary conduit stenosis. Repeat open heart operation for residual defects was common during the follow-up period (8 of 18 patients, 44%). No patient showed primary systemic or pulmonary ventricular dysfunction during the follow-up period. None of the last 11 patients developed complete heart block. Postoperative intraventricular conduction defects were common and are presumably caused by surgical injury of the bundle branches.Our observations suggest that surgical repair of congenitally corrected transposition of the great arteries can be currently achieved with acceptable risk. Improved knowledge of the precise location of the specialized conduction system resulted in a marked decrease in the Incidence of atrioventricular (A-V) block in patients with congenitally corrected transposition of the great arteries undergoing intracardiac repair. In the absence of postoperative residual defects it can be expected that longevity and quality of life will improve considerably, but many of these patients may require a repeat operation.     

Preliminary experience with GORE-TEX grafting for right ventricle-pulmonary artery conduits

A consecutive series of 12 patients between the ages of 6 1/2 and 37 years underwent implantation of venous ventricle-pulmonary artery conduits. GORE-TEX material without prosthetic valves was used. Four patients had L-transposition of the great vessels with ventricular septal defect (VSD) and pulmonary stenosis; four had tetralogy of Fallot with pulmonary atresia; and one had double outlet right ventricle, pulmonary stenosis, and a complete form of A-V canal. Two patients had D-transposition of the great arteries, VSD, and pulmonary stenosis; and one patient had L-transposition of the great arteries and isolated pulmonary stenosis. All patients had low pulmonary resistance and pressures. The technique for implanting this noncrimped type of prosthesis is described in detail. Follow-up ranged from 2 months to 5 1/2 years. GORE-TEX offers a good choice of material for the construction of this type of conduit to prevent pseudointima formation and obstruction, which is often observed in woven Dacron grafts.     

An unusual case of congenitally corrected transposition of the great arteries associated with noncompaction-like remodeling of the morphological right ventricle

Congenitally corrected transposition of the great arteries is a rare disease characterized by atrio-ventricular and ventriculo-arterial discordance (double discordance) and is associated with other cardiac anomalies in the majority of cases. The important associated anomalies include ventricular septal defect, abnormalities of left atrioventricular valve, subpulmonary stenosis, and conduction abnormalities. However, the noncompaction-like remodeling of the subaortic, morphologic right ventricle is not a commonly reported association. We, report a case of congenitally corrected transposition of the great arteries in a 40-year-old male patient, who had noncompaction-like remodeling of the morphologic right ventricle with severe ventricular dysfunction. He also had, left-sided Ebstein's anomaly, severe left atrioventricular valve regurgitation and a 2:1 atrioventricular conduction block.     

Ventricular development and function in complete transposition: angiocardiographic evaluation

We studied 50 left ventricular cineangiograms and 41 right ventricular cineangiograms of 40 patients with usual atrial arrangement (situs solitus) together with concordant atrioventricular and discordant ventriculo-arterial connexions (complete transposition), catheterized between 1 day and 12 months of age. Our purpose was to evaluate ventricular development and function. The patients were subdivided on the basis of associated lesions into groups with intact ventricular septum; with ventricular septal defect; with ventricular septal defect together with pulmonary stenosis and with pulmonary stenosis in isolation. Each group was further separated according to age into those patients below and above 60 days. Ventricular volumes, ejection fraction and the ratio between systolic pressure and end-systolic volume were evaluated for both ventricles. The left ventricular mass, stress, and the ratio of stress to end-systolic volume were also calculated. A volume overload leads to increased left ventricular volume even at birth. With an intact ventricular septum, the left ventricle in patients with complete transposition is normal at birth and also during the first weeks of life. Myocardial mass, however, does not increase proportionately with increase in volume as the patient grows and it remains inadequate by the age of one year. The left ventricular mass is also inadequate in patients with associated anomalies when the left ventricular pressure is less than 60 mm Hg. Moreover, the left ventricle in presence of an intact ventricular septum presents a decrease in contractility during the first year of life despite the finding of a satisfactory arterial oxygen saturation. The right ventricle has a normal volume at birth which increases during the first year due to a greater diastolic filling following atrial septostomy. We noted a progressive decrease in ejection fraction, however, which is related to various factors including a reduction in contractility.     

Transposition of the great arteries: anatomic types of left ventricular outflow tract obstruction (author's transl)]

A postmortem investigation has been carried out in 48 heart specimens with transposition of the great arteries (TGA) to evaluate incidence and type of left ventricular outflow tract obstruction. The frequence of pulmonary stenosis in our series (23%) is similar to those reported from previous studies. Cases have been divided in two groups according to presence or absence of ventricular septal defect. In cases with intact ventricular septum the obstruction was determined by asymmetric hypertrophy of the septum (1 observation) and by abnormal attachement of clefted anterior mitral leaflet to the interventricular septum (2 observations). In the specimens with ventricular septal defect, 4 presented a stenosis due to malalignment of the infundibular septum, associated with valvular stenosis in 1 case and with valvular stenosis and fibrous subvalvular ring in 2. Another case presented a stenotic pulmonary valve and a parachute mitral valve. The remaining 3 showed a cleft of the anterior leaflet of the mitral valve. The high incidence of infundibular malalignment obstruction is underlined together with its association with A-P or L-position of the aorta. This peculiar relation of the great arteries could be angiocardiographically employed as indicative for the presence of this type of left outflow tract obstruction in TGA.     

Transposition of the great arteries

Many patients with ventriculoarterial discordance have survived to adulthood. Those with complete transposition of the great arteries have often had an atrial switch procedure (Mustard or Senning operation) performed, which leaves the morphological right ventricle (RV) supporting the systemic circulation. RV failure and tricuspid regurgitation are common. Some patients may ultimately require cardiac transplantation. Sinus node dysfunction is increasingly common with longer follow-up, and some patients need pacemaker implantation. Atrial arrhythmias are frequent, and atrial flutter may be a marker for sudden death. Patients with an arterial switch procedure are also surviving to adulthood. Long-term problems include coronary stenoses, distortion of the pulmonary arteries, dilatation of the neoaortic root, and aortic regurgitation. Patients with congenitally corrected transposition have both atrioventricular and ventriculoarterial discordance and therefore also have a morphological RV and delicate tricuspid valve in the systemic circulation. Associated defects, such as abnormalities of the tricuspid valve, ventricular septal defect, and pulmonary stenosis, occur in the majority of patients. Heart block occurs with increasing age. Atrial arrhythmias occur frequently, and their occurrence should prompt a search for a hemodynamic problem. Progressive tricuspid regurgitation occurs with age and is associated with deterioration of RV function. Surgical treatment should be considered at the earliest sign of RV dilatation or dysfunction. All patients should be seen periodically in a center where expertise in the clinical evaluation, imaging, and hemodynamic assessment of adult congenital heart disease is available.