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.     

Treatment options for transposition of the great arteries with ventricular septal defect complicated by pulmonary vascular obstructive disease

The arterial switch operation is the procedure of choice for patients with isolated transposition of the great arteries or those with associated atrial and/or ventricular septal defects. After the development of pulmonary arterial hypertension, the surgical options for patients with a late presentation include either retraining the left ventricle by pulmonary artery banding followed by an arterial switch operation or palliative atrial or arterial switch, with or without medical management of pulmonary hypertension. We present a case with D-transposition of the great arteries with ventricular septal defects and irreversible pulmonary arterial hypertension who improved after a palliative atrial switch operation.     

Results of intraventricular baffle procedure for ventricular septal defect and double outlet right ventricle or d-transposition of the great arteries

Thirty patients with ventricular septal defect and d-transposition of the great arteries or double outlet right ventricle underwent placement of an intraventricular baffle to achieve physiologic correction. Eighteen of these patients also required an external conduit to establish continuity between the right ventricle and the pulmonary artery. There was an overall 22 percent early mortality rate, although the rate was 63 percent in patients who weighed less than 10 kg. There was an additional 20 percent late mortality rate, primarily related to conduction disturbances and pulmonary vascular obstructive disease, problems that have not occurred in patients operated on since 1974. Of the survivors, all are asymptomatic and as a group have achieved a significant weight gain. Many have residual hemodynamic abnormalities that required cardiac catheterization for precise assessment. Four patients have been reoperated on, with no additional mortality.     

Anatomic problems associated with arterial switch procedures for double outlet right ventricle with subpulmonary ventricular septal defect

Two cases of double outlet right ventricle with subpulmonary ventricular septal defect treated by arterial switch operations are reported. The anatomical problems of coronary artery transfer, occult outflow tract obstruction and position of the pulmonary bifurcation are discussed. Cases of double outlet right ventricle and subpulmonary ventricular septal defect with anterior-to-posterior relation of the great arteries are suited to the repair techniques pioneered by Jatene for complete transposition and ventricular septal defect. Cases in which the great arteries are side-by-side pose more difficult problems, partly because of the more complex and varied anatomy of the coronary arteries, and because of the spatial relation of the roots of the great arteries. Although it may be possible to overcome these technical problems, we have reservations about the reproducibility of such a procedure. We believe, however, that cases of this type are best treated without recourse to "inflow" correction. The options are either the arterial switch procedure or a modified Rastelli operation.     

Repair of double outlet right ventricle with doubly-committed ventricular septal defect

OBJECTIVE: To investigate our surgical results of intraventricular rerouting in patients having double outlet right ventricle with doubly-committed ventricular septal defect. METHODS: We undertook repair in 8 patients with this particular feature. Of these, 2 patients had pulmonary stenosis, and another had interruption of the aortic arch. The subarterial defect was unequivocally related to both the aortic and the-pulmonary orifices in all, albeit slightly deviated towards the aortic orifice in one, and towards the pulmonary orifice in another. Intraventricular rerouting was carried out via incisions to the right atrium and the pulmonary trunk. To ensure reconstruction of an unobstructed pulmonary pathway, a limited right ventriculotomy was made in 5. RESULTS: All patients survived the procedure, and are currently doing well, with follow-up of 25 to 194 months, with a mean of 117+/-68 months. Catheterization carried out 16+/-6 months after repair demonstrated excellent ventricular parameters. Mean pulmonary arterial pressure was 16+/-7 mmHg, being higher than 20 mmHg in 2 patients. No significant obstruction was found between the right ventricle and the pulmonary arteries. A pressure gradient across the left ventricular outflow tract became significant in one patient in whom a small outlet septum was present, and a heart-shaped baffle had been used for intraventricular rerouting. Reoperation was eventually needed in this patient for treatment of the obstruction, which proved to be progressive. CONCLUSION: Precise recognition of the morphologic features is of paramount importance when choosing the optimal options for biventricular repair in patients with double outlet right ventricle and doubly-committed interventricular communication.     

Late right ventricular failure after Mustard operation for transposition of the great arteries

The clinical course and postmortem findings of an unusual case of complete transposition of the great arteries, in which the Mustard operation was not performed until the patient was 12 years of age, are documented with serial catheterizations showing adequate repair. The clinical course of the patient was uneventful for 8 years after surgery, when right (systemic) ventricular failure occurred, resulting in the patient's death at 20 years of age. Late cardiac failure, the cause of death, is not a commonly described problem after successful surgical repair of transposition of the great arteries.     

Angiocardiographic diagnosis of ventricular septal defect localization in double outlet right ventricle

Children with double outlet right ventricle, aged 3 months to 23.5 years, were examined to assess the possibility of angiocardiographic diagnosis of defect localization. It was found angiocardiography allows satisfactory localization of the defect in question. To be able localize the defect, it is critical to determine the interaction between the defect and the conus septum, and the origin of large arteries. To establish the diagnosis of ventricular septal defect, right and left ventriculography in standard and axial projections must be performed.     

Double outlet left ventricle with subpulmonary ventricular septal defect and pulmonary hypertension

A two-month old male infant with the rare occurrence of double outlet left ventricle, subpulmonary ventricular septal defect and pulmonary hypertension is presented. The infant was managed temporarily with banding of the pulmonary trunk, with a favorable result, and is scheduled for definitive intraventricular repair.     

Radionuclide measurement of right ventricular function in atrial septal defect, ventricular septal defect and complete transposition of the great arteries

Right ventricular (RV) function was assessed in 80 patients with congenital heart disease by first-pass and gated equilibrium radionuclide angiography. In 30 patients with a ventricular septal defect (VSD) the mean RV ejection fraction (+/- standard deviation) was 64 +/- 7%. In 30 patients with a secundum atrial septal defect it was 61 +/- 9% and in 20 patients with surgically corrected complete transposition of the great arteries it was 49 +/- 13%. These values are in close agreement with values established with cineangiography for similar groups of patients. The mean ejection fraction in the group with transposition of the great arteries was significantly less than in the group with VSD (p less than 0.001). Phase analysis of the equilibrium studies showed that there was delayed RV contraction in many patients in the absence of conduction abnormalities. This delay was significantly greater in patients with atrial septal defect than in those with VSD (p less than 0.05). There was a strong correlation between size of left-to-right shunt and phase delay in patients with VSD (r = -0.72). Thus, first-pass gated radionuclide angiography provides a valid measurement of RV ejection fraction, and delayed RV contraction on phase analysis may be a sensitive index of early RV dysfunction.     

Spontaneous closure of ventricular septal defect in a case of double outlet right ventricle.

A 5 year old child, previously diagnosed as having tetralogy of Fallot, was admitted to hospital in severe congestive heart failure. The electrocardiogram showed left anterior hemiblock and incomplete right bundle-branch block, neither of which was previously present. The child died in intractable congestive heart failure and the necropsy showed a double outlet right ventricle with complete spontaneous closure of the subaortic ventricular septal defect by fibrous tissue. The possible mechanism involved in the production of this unusual complication of double outlet right ventricle is discussed, together with an explanation for the electrocardiographic changes.     

Left ventricle to pulmonary artery conduit in treatment of transposition of great arteries, restrictive ventricular septal defect, and acquired pulmonary atresia.

Progressive cyanosis after banding of the pulmonary artery in infancy occurred in a child with transposition of the great arteries and a ventricular septal defect, and a Blalock-Taussig shunt operation had to be performed. At the time of correction a segment of pulmonary artery between the left ventricle and the band was found to be completely occluded so that continuity between the left ventricle and the pulmonary artery could not be restored. A Rastelli type of operation was not feasible as the ventricular septal defect was sited low in the muscular septum. Therefore, in addition to Mustard's operation, a Dacron conduit was inserted from the left ventricle to the main pulmonary artery to relieve the obstruction. Postoperative cardiac catheterization with angiocardiography indicated a satisfactory haemodynamic result. The patient remains well 11 months after the operation. This operation, a left ventricle to pulmonary artery conduit, may be used as an alternative procedure in patients with transposition of the great arteries, intact interventricular septum, and obstruction to the left ventricular outflow, if the obstruction cannot be adequately relieved.     

Successful extensive enlargement of a non-committed ventricular septal defect in double outlet right ventricle

We performed an arterial switch operation in a patient with double outlet right ventricle with non-committed ventricular septal defect, and abnormal insertion of the tension apparatus of the tricuspid valve which produced moderate tricuspid regurgitation. This required extensive enlargement of the ventricular septal defect between the attachments of the cords of the tricuspid valve so as to create the interventricular rerouting that made possible the arterial switch operation. Postoperatively, we produced a straight, unobstructed, left ventricular outflow tract, improved the extent of tricuspid regurgitation, and achieved low right atrial pressures. Enlargement of the interventricular communication can set the scene for biventricular repair in this particular subset of patients with both arterial trunks arising from the morphologically right ventricle.     

Spontaneous closure of ventricular septal defect in a case of double outlet right ventricle

A 5 year old child, previously diagnosed as having tetralogy of Fallot, was admitted to hospital in severe congestive heart failure. The electrocardiogram showed left anterior hemiblock and incomplete right bundle-branch block, neither of which was previously present. The child died in intractable congestive heart failure and the necropsy showed a double outlet right ventricle with complete spontaneous closure of the subaortic ventricular septal defect by fibrous tissue. The possible mechanism involved in the production of this unusual complication of double outlet right ventricle is discussed, together with an explanation for the electrocardiographic changes.     

Double outlet right ventricle with l-malposition and uncommitted ventricular septal defect.

A case is described which exhibited Double Outlet Right Ventricle (DORV) with l-malposition of the aorta in association with a ventricular septal defect of the noncommitted, atrioventricular canal variety. When the ventriculoarterial relationship of DORV is encountered, it is more usual to find the aorta to the right of the pulmonary artery. Review of the literature shows that examples of DORV and l-malposition have been described with subaortic, subpulmonary and doubly committed defects, but we are unaware of any previous example reported with a noncommitted defect. These findings indicate that DORV with l-malposition should be anticipated in association with the same accompanying defects as those described in DORV with d-malposition, and emphasize the necessity for adopting a segmental approach to nomenclature. They also have morphogenetic significance, suggesting that DORV with l-malposition is closely linked with the anomalies for anatomically corrected malposition and concordant (complete) l-transposition. The present case exhibited an electrocardiographic superior axis, which is probably related to the presence of the atrioventricular canal deformity. The coexistence of hypoplasia of the aortic isthmus is speculatively related to the presence of conal and valvar factors, which can be considered to have reduced aortic blood flow.     

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.     

Impact of pregnancy on the systemic right ventricle after a Mustard operation for transposition of the great arteries

OBJECTIVES: We sought to determine the impact of pregnancy on the systemic right ventricle (RV) after a Mustard operation for transposition of the great arteries. BACKGROUND: Dysfunction of the RV remains a continuing problem after a Mustard operation. Concerns exist about the potentially deleterious effects of pregnancy on this ventricle. METHODS: The records of 16 women who completed 28 pregnancies were reviewed for clinical status, echocardiographic evaluation of RV dimensions, RV function, and tricuspid regurgitation (TR) before, during, and after pregnancy. RESULTS: Women were in New York Heart Association functional class I (n = 21) and II (n = 7) before pregnancy. The functional class deteriorated in six women, with no return to the pre-pregnancy level after delivery in two. Data on RV dimensions were available in 18 pregnancies, on RV function in 21, and on TR in 20. Before pregnancy, RV dilation was absent (n = 4), mild/moderate (n = 12), or severe (n = 2) and progressed in five women (31%), with no recovery in all patients at the last follow-up. Right ventricular systolic dysfunction was absent (n = 16), mild/moderate (n = 4), or severe (n = 1) before pregnancy and progressed in four women (25%), with no recovery in three cases. Tricuspid regurgitation was absent (n = 8), mild (n = 9), or moderate (n = 3) before pregnancy and deteriorated in eight women (50%), with no recovery in three patients. CONCLUSIONS: Pregnancy after a Mustard operation is clinically well tolerated but carries a risk of RV dysfunction, which is sometimes irreversible.     

Late banding operation in children with ventricular septal defect and pulmonary arterial hypertension.

Since 1960 31 children, 2-10 years old, with large ventricular septal defects and pulmonary arterial hypertension, underwent pulmonary artery banding in Munich. 22 of these children were recatheterized 3-5 years after the operation. Only 8 of these children were shown to have a definite decrease in pressure distal to the band, and in only 2 of these children a reduction of pulmonary vascular resistance to values at the upper limit of normal could be shown. In the remaining children either little or no alteration and rarely even a definite increase of pulmonary vascular resistance was found. There was only a poor correlation between pressure changes and resistance changes. During preoperative catheterization oxygen breathing was shown to produce a more than 50% reduction in the pulmonary arterial pressure in 8 cases. Of these, only 5 showed a decrease of pulmonary vascular resistance after pulmonary artery banding. This decrease, however, was relatively small.     

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.