Molecular cloning and developmental expression of human cardiac troponin T.

We have isolated a full-size cDNA coding for cardiac troponin T (cTnT) from a human adult heart library, using a slow skeletal TnT probe. This cDNA detected a 1.2 kb mRNA in fetal and post-natal human heart, the amount of which increased during ontogenic development. Interestingly, a similar transcript was coexpressed in fetal skeletal muscle, together with the 0.9 kb slow skeletal muscle mRNA, and its expression was down-regulated during further development.

Cor triatriatum and total anomalous pulmonary venous connection: a rare, surgically correctable anomaly.

Two cases of a very uncommon congenital association (cor triatriatum and total anomalous pulmonary venous connection) are presented. This association should be suspected in any infant with signs of pulmonary venous obstruction.

Intrauterine versus postnatal repair of created pulmonary artery stenosis in the lamb. Morphologic comparison.

Experimental lamb models were used for intrauterine creation of pulmonary artery stenosis and later intrauterine repair or postnatal repair. Intrauterine creation of pulmonary artery stenosis was performed in 23 fetal lambs at 90 +/- 1 days of gestation. Eight lambs underwent intrauterine repair of pulmonary artery stenosis at 135 +/- 1 days of gestation and were studied 110 +/- 13 days after repair. Seven lambs underwent postnatal repair at 57 +/- 9 days after birth and were studied 162 +/- 32 days after repair. Eight fetal lambs with unrepaired pulmonary artery stenosis were studied 89 +/- 18 days after birth. All study lambs were compared with normal control lambs. The systolic right ventricular pressure was significantly higher after unrepaired stenosis (78.6 +/- 6.8 mm Hg) than in other lambs, but there was no statistically significant difference after intrauterine repair (23.3 +/- 2.9 mm Hg), postnatal repair (25.9 +/- 3.4 mm Hg), and normal lambs (21.6 +/- 1.1 mm Hg). The systolic pulmonary artery pressure was also not statistically different in these three groups. The weight measurements were age-adjusted for comparison of postnatal and intrauterine repair with normal lambs. The adjusted heart weights were similar in the three groups. The comparison of the adjusted heart weight/adjusted body weight ratio (10(-3) showed a significantly higher ratio in postnatal repair (7.4 +/- 0.1) than in intrauterine repair (6.1 +/- 0.1). The adjusted right ventricular weight/adjusted left ventricular weight ratio was significantly higher in the postnatal repair group (0.71 +/- 0.01) than in both the intrauterine repair group (0.59 +/- 0.01) and normal lambs (0.59 +/- 0.01). The transverse myocyte diameter was not statistically different in all groups of animals and there were no ultrastructural changes even when the pulmonary stenosis was unrepaired. We conclude that intrauterine repair was more satisfactory than postnatal repair in terms of age-adjusted heart weight results, but we did not find any advantages of intrauterine repair in terms of histologic and ultrastructural changes.

Intrauterine creation and repair of pulmonary artery stenosis in the fetal lamb. Weight and ultrastructural changes of the ventricles.

Fetal lamb experimental models were employed for intrauterine creation and repair of pulmonary artery stenosis. The study group was composed of 51 fetal lambs including 29 models of pulmonary artery stenosis and 22 control lambs. Gestational age was 89 days at creation of pulmonary artery stenosis. Fourteen fetal lambs (Group A) were studied after creation of the stenosis at 131 days of gestation and compared to normal age-matched control lambs. The systolic right ventricular pressure was significantly higher after creation of pulmonary artery stenosis (76.6 +/- 17.8 versus 50.3 +/- 23.5 mm Hg), but the systolic pulmonary artery pressure was unchanged. The mean right ventricular weight and the mean right ventricular/left ventricular weight ratio were significantly greater after pulmonary artery stenosis than in normal control animals. The transverse myocyte diameter was not modified by pulmonary artery stenosis, but on electron microscopic study the myocytes appeared mature. Ten lambs (Group B) underwent intrauterine repair of pulmonary artery stenosis at 131 days of gestation without cardiopulmonary bypass. The pulmonary artery was clamped and patched. Immediately after repair the right ventricular pressure fell significantly from 85.8 +/- 18.9 to 62.2 +/- 14.6 mm Hg. At birth, 7 +/- 6 days after repair, Group B was compared to Group C (unrepaired pulmonary artery stenosis, five fetuses) and to normal control lambs. The mean right ventricular weight and the mean right ventricular/left ventricular weight ratio were not statistically different in Group B and in the control group. There were no ultrastructural changes after intrauterine repair. We conclude that intrauterine creation of pulmonary artery stenosis causes right ventricular hypertrophy with more mature myocytes. Intrauterine repair of pulmonary artery stenosis is feasible without cardiopulmonary bypass and rapidly abolishes the preponderance of right ventricular weight over left ventricular weight.

Transposition of the great arteries, ventricular septal defect, and pulmonary outflow tract obstruction. Rastelli or Lecompte procedure?

During a 10-year period (1980 to 1990), 62 patients underwent complete repair for transposition of the great arteries, ventricular septal defect, and pulmonary outflow tract obstruction. Twenty-two patients (35%) (mean age 8.1 +/- 7.2 years) underwent the Rastelli operation: The ventricular septal defect was enlarged anteriorly in eight patients, and right ventricular-pulmonary artery continuity was established with an extracardiac valved (9/22) or nonvalved (13/22) conduit. Forty patients (65%) (mean age 3.3 +/- 3.2 years) underwent the Lecompte modifications: The conal septum was extensively excised when present (30/40), anterior translocation of the pulmonary bifurcation was performed in 32 patients, and right ventricular-pulmonary artery continuity was established by direct anastomosis without a prosthetic conduit. There were seven early deaths (11%; 70% confidence limits, 7% to 17%): two after the Rastelli procedure (9%; 70% confidence limits, 3% to 20%) and five after the Lecompte operation (12.5%; 70% confidence limits, 7% to 20%). Four patients were lost to follow-up, yielding a 93% complete follow-up (mean follow-up 55 months). There were two late deaths (one in each group). Actuarial probability of survival (+/- standard error) at 5 years was 83% +/- 9% after the Rastelli operation and 84% +/- 6% after the Lecompte procedure. All long-term survivors (except one in the Rastelli group) were in functional class I. Five patients in the Rastelli group underwent late reoperation for obstruction of the extracardiac conduit (28%; 70% confidence limits, 16% to 42%). Three late reoperations (10%; 70% confidence limits, 4% to 19%) were required after the Lecompte operation (one for residual ventricular septal defect and two for residual pulmonary outflow tract obstruction). At most recent examination, residual pulmonary outflow tract obstruction was present in seven patients of the Rastelli group (39%; 70% confidence limits, 26% to 53%) and in six patients of the Lecompte group (19%; 70% confidence limits, 12% to 29%). The combined likelihood of reoperation for pulmonary outflow tract obstruction and residual pulmonary outflow tract obstruction was significantly higher in the Rastelli group (67% versus 26%; p = 0.005). Both procedures provide satisfactory early and late results. The Lecompte operation allows complete repair in infancy, is feasible in patients with anatomic contraindications to the Rastelli operation, and may reduce the need for reoperation and the prevalence of residual pulmonary outflow tract obstruction.

Modified technique for correction of tricuspid atresia.

This study presents a new technique for correction of tricuspid atresia. All 13 patients operated upon had the same form of the anomaly, with the great arteries normally related. The principle is to conserve the pulmonary valve and anulus of the patient in its anatomic position and to exclude the hypoplastic right ventricle. Because there is no need for closing the ventricular septal defect, the risk of heart block and residual left-to-right shunts is avoided. Deaths are related to questionable indications for two patients and to a bilateral pneumothorax for the third one. The long-term follow-up (5 years for the first patient) shows good results for the 10 survivors. Six of them are in Class 1 of the New York Heart Association and four are in Class II.

Aortic coarctation with hypoplastic aortic arch. Results of extended end-to-end aortic arch anastomosis.

Between 1980 and 1986, 80 infants (less than or equal to 3 months old) with symptomatic aortic coarctation and associated severe tubular hypoplasia of the transverse aortic arch underwent surgical treatment. Extended end-to-end aortic arch anastomosis was used in an attempt to correct both the isthmic stenosis and the hypoplasia of the transverse arch. After complete excision of the coarctation tissue, a long incision was made in the inferior aspect of the aortic arch, which was then anastomosed to the obliquely trimmed distal aorta. Pure coarctation was present in 17 patients (group I); 24 infants had an additional ventricular septal defect (group II), and 39 patients had associated complex heart disease (group III). The overall early mortality rate was 26% (confidence limits 21% to 32%) (18% in group I, 17% in group II, and 36% in group III). The early risk declined with time and was 18% (confidence limits 12% to 26%) for the last 2 years (seven deaths in 39 patients). Follow-up was 100% for a mean of 19 months. Actuarial survival rate at 3 years was 82% for group I, 78% for group II, and 32% for group III. Recurrent coarctation (gradient greater than or equal to 20 mm Hg) occurred in six operative survivors (10%, confidence limits 6% to 16%) and necessitated reoperation in three. Freedom from recoarctation at 4 years was 88%. Because extended end-to-end aortic arch anastomosis provides adequate correction of the aortic obstruction and entails a low risk of restenosis, it is our procedure of choice in infants with coarctation and severe hypoplasia of the aortic arch.

Anatomic repair of anomalies of ventriculoarterial connection associated with ventricular septal defect. II. Clinical results in 50 patients with pulmonary outflow tract obstruction.

From November 1980 to November 1985, 50 patients underwent anatomic repair of anomalies of ventriculoarterial connection associated with ventricular septal defect and pulmonary outflow tract obstruction. The technique used was one that we have previously described, which we call REV. The principles of this technique are resection of the infundibular septum, construction of a tunnel connecting the left ventricle to the aorta, and direct anastomosis, without a prosthetic conduit, of the pulmonary arterial trunk with the right ventricle. The tunnel is situated beneath the aortic valve and occupies very little space in the right ventricular cavity. Age at operation ranged from 4 months to 13 years (mean 3.5 years). Twenty-six patients had a classic type of transposition of the great arteries; all other patients had various types of anomalies of ventriculoarterial connection in which it was impossible, after the intraventricular connection of the left ventricle to the aorta, to use the natural pulmonary orifice for the pulmonary outflow tract reconstruction. There were nine hospital deaths (18%) and one late death. Twenty-six of 29 patients whose follow-up time exceeded 1 year had an excellent clinical result. No stenosis of the aortic outflow tract was found. Four patients had significant pressure gradients on the pulmonary outflow tract. Our present experience with REV suggests that this technique allows anatomic repair in a wide variety of anomalies of ventriculoarterial connection associated with ventricular septal defect and pulmonary outflow tract obstruction, even in infants, with an acceptable rate of mortality and morbidity.

Primary repair of tetralogy of Fallot in infancy.

From June 1983 to April 1988, 100 consecutive infants with symptomatic tetralogy of Fallot (without pulmonary atresia) were operated on. Ages ranged from 0.5 to 12 months (mean 7.3 +/- 3.7). Twenty patients were 0.5 to 3 months, 21 were 3 to 6 months, and 59 were 6 to 12 months of age. Mean weight was 6.5 kg +/- 1.7. Seventy patients received a transannular patch. The hospital mortality rate was 3% and there were no late deaths. Cumulative follow-up was 180 patient-years. Causes of death included hypoplastic pulmonary arteries (4 and 5 months old) and right ventricular failure (4 months old). The most important factors influencing right ventricular outflow tract reconstruction were neither weight (p = 0.90) nor age (p = 0.05) but rather were the ratio between weight and pulmonary arterial outflow tract diameter (p = 0.0005) and the ratio between body surface area and pulmonary arterial outflow tract diameter (p less than 0.0001). The last 48 patients were operated on with no deaths. During this period, operative management differed essentially in myocardial protection with blood cardioplegia. The predicted 30-day survivorship after repair was 90% to 99% (95% confidence limits). No ventricular arrhythmias have been detected after repair (mean follow-up 22.2 months). Mean right ventricular/left ventricular end-diastolic dimension ratio was (0.53 +/- 0.10 with M-mode echocardiography. These early results encourage us to proceed with primary repair of infants with symptomatic tetralogy of Fallot thanks to improved surgical management and enhanced myocardial protection.

Anomalous left coronary artery from the pulmonary artery in infants. Which operation? When?

The surgical management of anomalous left coronary artery from the pulmonary artery in infants and small children remains controversial, because the ideal surgical procedure and the optimal time for operation are yet to be determined. From 1977 to 1985, 22 patients less than 4 years of age (mean age 18.2 months) underwent direct aortic reimplantation of the anomalous left coronary artery. There were five operative deaths (23%, confidence limits 13%-36%). The determinant risk factor of early mortality was the severity of preoperative left ventricular dysfunction (p = 0.05), not age at operation (p = 0.64) or preoperative clinical status (p = 0.36). There were not late deaths (mean follow-up 38 months). All survivors but one were symptom free. The reimplanted anomalous left coronary artery was patent in each reevaluated case (9/17). Left ventricular function improved significantly in all survivors. Moderate to severe preoperative mitral incompetence lessened in all patients but one, without mitral valve repair. When technically feasible, direct aortic reimplantation of the anomalous left coronary artery is an attractive procedure because it offers a high rate of patency and avoids the potential drawbacks of procedures involving autogenous venous or arterial tissue. Optimal intraoperative myocardial preservation and institution of temporary left ventricular assistance at the end of the operation may decrease the operative risk. Left ventricular function nearly always recovers after successful revascularization, and resection of left ventricular myocardium is rarely indicated, if ever. Mitral incompetence almost always lessens, and the mitral valve should not be repaired at initial operation; however, residual mitral incompetence may necessitate reoperation in a few cases. In infants with moderate left ventricular damage (usually asymptomatic with medical therapy), surgical treatment should be delayed until 18 to 24 months of age so that it can be performed with a low operative risk. Infants with severely impaired left ventricular function and persistent congestive heart failure should probably undergo operation as soon as the diagnosis has been made.

Late results after left-sided cardiac valve replacement in children.

Selection of types of cardiac valve substitutes for children remains controversial. Between 1976 and 1984, 166 children, 15 years of age or younger, underwent aortic (N = 53) or mitral valve replacement (N = 90) or both (N = 23). Biological prostheses were used in 84 patients and mechanical prostheses in 71; both a mitral bioprosthesis and an aortic mechanical valve were used in 11 patients. The overall early mortality was 9%. Mean follow-up intervals were 4.1 years for the bioprosthesis group, 3.3 years for the mechanical valve group, and 3.5 years for the group receiving both. The 7 year survival rates (+/- standard error) were 63% +/- 6% in the bioprosthesis group and 70% +/- 7% in the mechanical valve group (p = NS). After aortic valve replacement the 7 year survival rates were 66% +/- 14% (bioprosthesis group) and 77% +/- 9% (mechanical valve group) (p = NS); after mitral valve replacement the rates were 65% +/- 7% (bioprosthesis group) and 54% +/- 17% (mechanical valve group) (p = NS). The incidence of thromboembolic events was 0.6% +/- 0.4% per patient-year in the bioprosthesis group (none after aortic valve replacement, 0.8% +/- 0.6% per patient-year after mitral valve replacement) and 1.4% +/- 0.8% per patient-year in the mechanical valve group (0.7% +/- 0.7% per patient-year after aortic valve replacement, 4.0% +/- 2.8% per patient-year after mitral valve replacement) (p = NS). The linearized rates of reoperation were 10.4% +/- 1.8% per patient-year (bioprosthesis group) and 2.3% +/- 1.0% per patient-year (mechanical valve group) (p less than 0.001). The 7 year probability rates of freedom from all valve-related complications were 43% +/- 6% in the bioprosthesis group and 86% +/- 4% in the mechanical valve group (p less than 0.001). In the aortic position, a mechanical adult-sized prosthesis can always be implanted, and satisfactory long-term results can be anticipated. In the systemic atrioventricular position, the results are less than satisfactory with either type of prosthesis; every effort should be made to preserve the natural valve of the child.

Surgically created double-orifice left atrioventricular valve: a valve-sparing repair in selected atrioventricular septal defects.

OBJECTIVES: Some features of the left atrioventricular valve (large mural leaflet, dystrophic tissue) represent a challenge for repair of atrioventricular septal defects without postoperative regurgitation. A retrospective study was conducted to evaluate the results of surgically creating a double-orifice left atrioventricular valve in such circumstances. Clinical results were analyzed according to valvular and subvalvular left atrioventricular valve measurements in pathologic specimens with atrioventricular septal defects. METHODS: Among 157 patients operated on for atrioventricular septal defect since October 1989, 10 patients underwent primary repair (n = 8) or reoperation (n = 2) by this procedure. Median age at repair was 3.3 years (0.1-33 years). Anatomic types were complete (n = 3), intermediate (n = 5), and partial (n = 2). Preoperative moderate to severe left atrioventricular valve regurgitation was present in 6 patients. After the repair (two-patch technique in complete atrioventricular septal defect, cleft closed in each case), these 10 patients were found to have moderate to severe residual regurgitation not amenable to repair by annuloplasty. The top edge of the mural leaflet was anchored to the facing free edge of the cleft. RESULTS: No hospital death or morbidity was observed. Left atrioventricular valve regurgitation was absent or trivial (8 patients) and mild (2 patients). Color-coded echocardiography did not show significant left atrioventricular valve stenosis. The mean diastolic pressure gradient across the left atrioventricular valve was 3.2 +/- 1.1 mm Hg (1.4-4.5 mm Hg). At a median follow-up of 72 months (6-91 months), there was 1 late death, unrelated to left atrioventricular valve malfunction, due to pulmonary vascular obstructive disease. Left atrioventricular valve regurgitation did not increase over time, except in 1 patient in whom regurgitation recently progressed from mild to moderate. At rest, the mean diastolic pressure gradient across the left atrioventricular valve was 3.8 +/- 2.9 mm Hg (1.5-11.2 mm Hg). One child had an early moderate stenosis without pulmonary hypertension. Studies on pathologic specimens (n = 34) indicated that long chordal lengths and large mural leaflet size are essential independent anatomic features to assess its feasibility. CONCLUSIONS: Surgical creation of a double-orifice left atrioventricular valve is an effective additional procedure for repair of atypical cases of atrioventricular septal defect. The operation may decrease the need for reoperation or left atrioventricular valve replacement.

Long-term follow-up after prosthetic replacement of the superior vena cava combined with resection of mediastinal-pulmonary malignant tumors.

The contraindication to curative excision of mediastinal and pulmonary cancers because of invasion of the superior vena cava is now challenged by the existence of vascular prostheses that are suitable for venous replacement. Between 1979 and 1990 22 patients underwent resection of lung cancer (n = 6) or malignant mediastinal tumors (n = 16) involving the superior vena cava. Resection was done with concomitant venous reconstruction, and polytetrafluorethylene grafts were used. All bronchogenic carcinomas necessitated right pneumonectomy, whereas the excision of mediastinal tumors had to include pulmonary resections in nine patients (five lobectomies and four sublobar resections) and the right phrenic nerve in 12 patients. Venous reconstruction was performed by interposition of a large polytetrafluoroethylene graft between the proximal and cardiac ends of the superior vena cava (n = 8), or between one (n = 10) or both brachiocephalic veins (n = 4) and the right atrium. One patient died postoperatively (4.5%), and another had mediastinitis that was successfully treated by omentopexy. Chemotherapy was administered preoperatively to five patients and postoperatively to seven patients; radiotherapy was administered to two and 10 patients, respectively. The overall actuarial survival rate is 48% at 5 years, with 11 patients presently alive. The survival rate of patients with mediastinal tumors is 60% at 5 years. Among the patients with lung cancer, two with N1 disease are alive at 16 and 51 months, and one died at 38 months; the two patients with N2 disease died at 6 and 8 months. Only one graft occlusion occurred in the postoperative period; another occurred 14 months after operation and was precipitated by insertion of a central venous catheter. The patency of all remaining grafts was demonstrated after an average time of 23 (1 to 98) months. On the basis of these results, polytetrafluoroethylene graft replacement of the superior vena cava should be part of the planning and execution of radical excision with curative intent of mediastinal and right pulmonary malignant tumors that are not present with other contraindications, such as pleural or distant metastasis and severe systemic disease.

Tricuspid atresia. Results of treatment in 115 children.

We present our experience in the management of tricuspid atresia in 115 children. The anatomic data are categorized as follows: type I, 83.5%, type II, 16.5%. Type IB is the most frequent, representing 63.5% of all the cases. Each patient was operated upon one to four times. The age at first operation ranged from 10 days to 20 years. The first operation was a shunt in 94 children, a Fontan operation in four, and banding of the pulmonary artery in 17. Hospital mortality for the first operation was 12.2%, significantly higher in children under 6 months and in those having Waterston shunts. Potts and Blalock-Taussig operations give low long-term mortality; although few (six) have been done, Potts shunts also seem to give good long-term palliation in this series. The Glenn anastomosis is a good operation when performed after a systemic-pulmonary arterial shunt. The Fontan operation was performed in 24 children (hospital mortality 16.6%). There have been no late deaths after the third month postoperatively. Mean follow-up for this operation is only 2 years, but 88% of the survivors lead a normal life, two thirds of them receiving no treatment. There has been one reoperation for stenosis of a Dacron conduit with a good result. Late arrhythmias are well tolerated. In conclusion, the Fontan procedure is a good operation, but palliative procedures still allow good long-term survival.

Reconstruction of the pulmonary outflow tract without prosthetic conduit.

New techniques of correction of complex congenital anomalies, avoiding the use of prosthetic conduits, are presented. In transposition of the great arteries (TGA) with ventricular septal defect (VSD) and pulmonary stenosis, the technique comprised the resection of infundibular septum, the suturing of an intraventricular baffle directing blood from the left ventricle to the aorta, and the reconstruction of the pulmonary outflow tract by direct implantation of the posterior rim of the pulmonary arterial trunk on the right ventricle, completed by an anterior patch. In most cases, the pulmonary bifurcation was translated anterior to the ascending aorta. This technique was feasible even in infants and in patients with a small VSD. Thirteen patients, from 3 months to 8 years of age, were treated by this technique, with four deaths, one residual VSD (reoperated), and eight good results (4 to 16 months). A similar repair was used in three cases of double-outlet right ventricle (DORV) with subpulmonic VSD and pulmonary stenosis or pulmonary artery banding, with two operative deaths and one good result. The same technique of pulmonary outflow tract reconstruction was used in four cases of truncus arteriosus. Two deaths were attributed to severe pulmonary regurgitation, a complication which should be prevented in future cases by a reliable method of inserting a valve in the pulmonary outflow tract. In pulmonary atresia with VSD and absent pulmonary trunk, the continuity between the right ventricle and the pulmonary branches was established via an arterial tube resected from the ascending aorta. This technique was successfully used in one child with extremely small pulmonary branches. These preliminary results led us to conclude that many complex congenital cardiac anomalies can be effectively treated without a prosthetic conduit.

Anatomic correction of transposition of the great arteries.

A new technique of anatomic correction of transposition of the great arteries with ventricular septal defect is presented. The procedure described, which avoids the use of a prosthetic conduit in the reconstruction of the pulmonary outflow tract, has been used in nine consecutive patients. Six remain alive and well 4 to 24 months postoperatively. Details and illustrations of the technique utilized are presented. Avoiding the use of a prosthetic conduit in the procedure re-established the basic appeal of arterial correction of transposition of the great arteries with ventricular septal defect.

Adult heart transplantation: adverse role of chronic alcoholism in donors on early graft function.

Because of the increasing shortage of heart donors, selection criteria have been gradually extended. The purpose of this study was to determine the donor-related factors implied in early graft dysfunction and to define new selection criteria. The 70 consecutive adult patients who underwent heart transplantation in our institution between January 1988 and February 1992 were retrospectively studied. Mean donor age was 38 +/- 11 years (10 donors were more than 50 years of age; two donors were more than 60 years of age). Mean ischemic time was 130 +/- 39 minutes. An important proportion of donors (20%) had a history of chronic alcoholism. Thirteen patients experienced immediate graft dysfunction; five of them died within the first operative month. The different parameters studied, which were found to have no significant influence on the early graft function, were the age of the donor, the duration of inotropic support and the dose administered, a relative hemodynamic instability, resuscitation maneuvers, chest trauma, and weight mismatch between donor and recipient. Ischemic time was significantly longer in patients who died of cardiac dysfunction (p < 0.05). Chronic alcoholism in the donor was a very detrimental factor: 54% of patients who had early graft dysfunction versus only 12% of patients who had immediate normal graft function had received a graft from an alcoholic donor (p = 0.003). Excluding such alcoholic donors or reserving them for critically-ill recipients, with an increased risk of early graft dysfunction would be preferable.

Surgical repair of tetralogy of Fallot with complete atrioventricular canal.

Total repair of tetralogy of Fallot and complete atrioventricular canal remains a surgical challenge; however, good results can be obtained if the lesions are repaired properly at operation. Our technique involves a combined right atrial and right ventricular approach with closure of the ventricular septal defect through the ventriculotomy, double-patch closure of the septal defects without dividing the bridging atrioventricular leaflets, and functional repair of the newly constructed mitral valve.

Surgical management of diffuse subaortic stenosis: an integrated approach.

An integrated approach to the surgical management of diffuse subaortic stenosis has been designed to provide adequate relief of left ventricular outflow tract obstruction whatever the anatomical features encountered at operation. This approach was used in 22 patients with tunnel subaortic stenosis (19 patients) or diffuse hypertrophic obstructive cardiomyopathy (3 patients). The obstructive tissue was resected through an aortoseptal approach. In 18 patients, associated hypoplasia of the aortic orifice necessitated aortic valve replacement using the Konno procedure; in 4 patients with a normal-sized aortic orifice, the native aortic valve was preserved. There were two early deaths and one late death (all after a Konno operation). Long-term adequate relief of left ventricular outflow tract obstruction was achieved in all survivors. Operation for diffuse subaortic stenosis should be performed with two main goals: (1) to obtain complete relief of the left ventricular outflow tract obstruction by the appropriate procedure and (2) to preserve the native aortic valve whenever possible, particularly in young patients.

Ventricular septal defect creation for relief of tunnel subvalvular aortic stenosis.

In infants, the management of severe tunnel subvalvular aortic stenosis associated with hypoplastic aortic annulus remains a major surgical challenge. We report a case of such lesion treated by rerouting the systemic blood flow through a created subpulmonary ventricular septal defect toward the right ventricular outflow tract and the pulmonary valve. This technique provided complete relief of the left ventricular outflow tract obstruction without the implantation of a systemic prosthetic valve.