Surgical repair of double-outlet right atrium

The case of a patient with abnormal position of the atrial septum resulting in a right atrium with two atrioventricular valves and a disconnected left atrium is presented. The left superior vena cava drained into the left atrium; the right superior vena cava and the coronary sinus were absent. The surgical technique for repair of this anomaly is described. A possible explanation of the embryopathogenesis of double-outlet right atrium is given. To our knowledge, this is the second case of double-outlet right atrium to be reported in the thoracic surgical literature.     

Surgical management of double-outlet right ventricle associated with atrioventricular discordance

Twenty corrective operations for this unusual syndrome have been performed since 1965. All but two patients also had pulmonary stenosis; one of the exceptions had had pulmonary arterial banding. Two patients had situs inversus of the atria and viscera. In only three of the 20 patients (15 percent) was the apex of the heart positioned normally. Six patients had an entirely intracardiac repair; two early and two late deaths occurred in this group. The other technique, performed in 14 patients, involved insertion of an extracadiac conduit between a ventriculotomy in the morphologically left ventricle and the distal end of the proximally oversewn pulmonary artery; one operative and two late deaths occurred in this group. Heart block occurred in six patients, including two among the eight who had technically satisfactory intraoperative mapping of the His bundle. The intraventricular course of the bundle was not consistently positioned, being anterior to the septal defect in four and posterior in four. No hospital deaths have occurred in the last 13 operations; all except one of the survivors are in New York Heart Association Class I or II. Thus correction currently provides a good early results but leaves the ventricles in an inverted relationship both functionally and anatomically.     

The surgical anatomy of double-outlet right ventricle with concordant atrioventricular connection and noncommitted ventricular septal defect

In describing hearts with double-outlet right ventricle, we have had problems with how best to use the term noncommitted as applied to the ventricular septal defect. We reviewed, therefore, 63 hearts with double-outlet right ventricle in the setting of usual atrial arrangement and a concordant atrioventricular connection. From these, 18 hearts with potentially noncommitted defects were identified and studied in detail. The defect was unequivocally remote from the ventricular outflow tracts in 16 of these hearts, being perimembranous with excavation to open into the inlet of the right ventricle in 12, two of these also having straddling of an atrioventricular valve. One heart had a muscular defect situated in the inlet part of the muscular septum, whereas the defect was the ventricular component of an atrioventricular septal defect in the other three. In the remaining two hearts the defect was anatomically juxtaposed to a subarterial outlet. The pathway to the outflow tract, however, was obstructed by leaflets of a straddling valve. Our study shows, therefore, the need to distinguish between anatomic "commitment" of the defect from the problems in terms of commitment that may confront the surgeon in the operating room. Not only does the distance between the interventricular communication and one of the subarterial outflow tract need to be assessed (the anatomic commitment), but also the presence and nature of any intervening extraneous tissues (the surgical commitment) requires assessment.     

A case of modified Fontan operation for complete atrioventricular canal of right dominance with double-outlet right ventricle, common atrioventricular valve regurgitation

A 6-year-old boy was diagnosed to have complete atrioventricular canal (CAVC) with double-outlet right ventricle (DORV), common atrium and common atrioventricular valve regurgitation. The atrioventricular junction predominantly connected to the right ventricle (right dominance) and the left ventricle was hypoplastic (LVEDV = 64% of normal). The combination of DORV with right dominant CAVC makes more difficult to make intra-ventricular rerouting. A modified Fontan procedure, atrial partition and DeVega's annuloplasty were successfully performed. It is concluded that Fontan procedure might be preferable rather than intra-ventricular rerouting in this case.     

Double-outlet right atrium associated with tetralogy of Fallot and common atrioventricular valve

The successful repair of double-outlet right atrium, tetralogy of Fallot, and common atrioventricular valve is reported. Double-outlet right atrium is reviewed and its pathoembryology and nomenclature are discussed.     

Divided right atrium (prominence of the eustachian and thebesian valves)

Division of the morphologically left atrium (cor triatriatum) is a recognized clinical and surgical entity. Division of the right atrium (prominence of the eustachian and thebesian valves) is recognized pathologically, but is rare. It is unusual for this entity to be diagnosed during life. Stimulated by our experience with two patients seen at operation, one with an obstructive spinnaker-like formation and the other with a partitioned right atrium in the setting of pulmonary atresia, we reviewed the specimens in the heart museum of Children's Hospital of Pittsburgh that had prominence of the eustachian and thebesian valves. We identified 14 such hearts, which could be divided into two groups. In the first group, comprising six hearts, the valves were prominent in the form of Chiari networks and were of no functional significance. The valves were more extensive in the other eight hearts and partitioned the right atrium. In two of these, the valves themselves were the impediment to flow through the right side of the heart. In the other six, there was either atresia or severe stenosis along the right-sided pathways so that, after birth, the prominent valves retained their role during fetal life; namely, to deflect inferior caval venous return across the atrial septum to the left atrium. The partitions in these latter hearts would be of functional significance only if it were necessary to perform a Fontan procedure, when they might obstruct flow through an atriopulmonary (or atrioventricular) anastomosis.     

Biventricular repair of complete atrioventricular canal, double-outlet right ventricle and common atrium using a modified double switch technique. A valid alternative to univentricular procedure

An eleven month-old child underwent a successful modified double switch operation for total correction of complete atrioventricular canal, double outlet right ventricle, noncommitted ventricular septal defect, pulmonary stenosis, common atrium and anomalous left superior vena cava to an unroofed coronary sinus. We describe the technique of modified double switch operation, utilizing an atrial switch combined with a Rastelli type reconstruction between the venous ventricle and the pulmonary artery.     

Surgical management of double-outlet right ventricle

From 1977 to 1983, 32 consecutive patients, ranging in age from 15 days to 24 years, underwent operations for double-outlet right ventricle. Twenty patients had a palliative operation either to increase (12 cases) or to reduce (eight cases) pulmonary blood flow: Ten of them have subsequently undergone total repair, and in another six correction was delayed because of possible incremental operative risk factors, such as multiple ventricular septal defects or the need for an extracardiac conduit. Four patients with multiple, complex associated intracardiac anomalies are currently considered to have uncorrectable defects. A total of 22 patients underwent correction either primarily (12) or after palliation (10). Intraventricular tunneling was performed in 16 patients with a subaortic ventricular septal defect and in one with a doubly committed ventricular septal defect. Seven of these had pulmonary stenosis and five had reconstruction of the right ventricular outflow by means of a patch (three) or a conduit (two); among this group, five also had enlargement of the ventricular septal defect. In three patients with a subpulmonary defect and in one with a remote ventricular septal defect, all of them without pulmonary stenosis, total repair was achieved by a Senning, a Mustard, or an arterial switch operation. Finally, the only patient with atrioventricular discordance and pulmonary stenosis had insertion of a left ventricle-pulmonary artery conduit. No operative deaths were observed after palliation, but one patient died of intrapulmonary hemorrhage after total repair (4.5%). Major postoperative complications included detachment of the ventricular septal defect patch in one patient and late progression of pulmonary vascular obstructive disease in another. No late deaths have been recorded. Surgical repair of double-outlet right ventricle can be accomplished with gratifying early and late results, the risk of operative death being below 5%. The outcome in patients with subaortic ventricular septal defect appears particularly favorable, despite the extensive intracardiac procedures required for total correction. An early intervention is recommended to prevent development of pulmonary vascular obstructive disease and to avoid massive cardiac hypertrophy and fibrosis, which may cause late rhythm disturbances and impede the intracardiac repair.     

Atrioventricular septal defect with balanced ventricles and malaligned atrial septum: double-outlet right atrium. Report of two cases

An unusual form of atrioventricular septal defect was found at operation. The anatomy was that of a primum atrial septal defect with deviation of the atrial septum to the left. Thus from the right atrium both right and left atrioventricular valves could be seen. The left-sided valve was tricuspid. Repair was achieved by closure of the septal commissure of the left-sided valve ("cleft"), excision of the lower part of the atrial septum, and insertion of a pericardial patch. The lower part of the patch was sutured between the left and right atrioventricular valves. A specimen with similar anatomy is also described.     

The surgical anatomy of hearts with no direct communication between the right atrium and the ventricular mass--so-called tricuspid atresia

Seventy-six hearts were studied having no direct communication between the right atrium and the ventricular mass. The different cardiac chamber combinations producing so-called "tricuspid atresia" were considered. The nature of the atrioventricular valve atresia, the morphology of the ventricular mass, the size and position of the interventricular communication, the ventriculoarterial connection, and the presence of subarterial outflow tract obstruction were all analyzed. The majority of cases were of the "classical" type, i.e., absent right atrioventricular connection with the left atrium connected to the left ventricle. In another group there was absence of the right atrioventricular connection but the left atrium drained into the morphologically right ventricle, which was left-sided. In a third group both atrial chambers connected with the ventricular mass but some structure, an imperforate valve or muscular partition, completely blocked the flow pathway through the right side of the heart. All the hearts described are candidates for the Fontan procedure with either an atrioventricular or an atriopulmonary conduit. The surgical options would not be affected by the observed variability at the atrioventricular junction but would be dictated by the feasibility of incorporating the subpulmonary ventricle within the pulmonary circulation.     

Complete atrioventricular canal associated with tetralogy of Fallot or double-outlet right ventricle and right ventricular outflow tract obstruction: a report of successful surgical treatment

Six patients, 4 of whom had complete atrioventricular (AV) canal and tetralogy of Fallot (TOF) and 2 of whom had double-outlet right ventricle with subaortic ventricular septal defect (VSD) and right ventricular outflow tract obstruction, were treated. Two of the patients with complete AV canal and TOF had a shunt procedure only. The other 4 patients underwent complete repair. All 6 patients survived the operation. Complete repair was performed through the right atrium using the two-patch technique. The size and shape of the VSD patch is important. Residual VSD after repair was common. Two patients have undergone successful reoperation for this problem. Another patient has a small residual VSD.     

Surgical treatment of acyanotic double-outlet right ventricle

Two cases of double-outlet right ventricle in which surgical correction was successful are described. The diagnosis of this lesion should always be suspected in apparently large ventricular septal defects with pulmonary hypertension at systemic level, and further investigation by double catheterization or by angiography should be carried out. The catheterization data do not give accurate measurements of the pulmonary and systemic flows and resistances and are of no help in forecasting the results of surgical treatment.     

Surgical outcome of double-outlet right ventricle with subpulmonary VSD

BACKGROUND: Optimal management of double-outlet right ventricle with subpulmonary ventricular septal defect remains controversial. We reviewed our 7-year experience with patients who had this anatomic configuration. METHODS: Between January 1992 and January 1999, 20 patients underwent an arterial switch operation (ASO group), and 12 underwent a bidirectional Glenn procedure followed by a modified Fontan in 10 (Glenn/Fontan). Mean follow-up was 23 +/- 18 months. RESULTS: An initial palliative operation was done in 19 patients (9 in the ASO group, 10 in the Glenn/Fontan group). There were no deaths in the Glenn/Fontan group. Four patients in the ASO group died within 33 days postoperatively. Two of them had a single coronary artery, 1 had a straddling mitral valve, 1 had a hypoplastic aortic arch, and 1 had multiple ventricular septal defects. Three patients had reoperation for subaortic stenosis (n = 2) or pulmonary stenosis (n = 1) after the ASO. Four patients (3 in the ASO group, 1 in the Glenn/Fontan) required a pacemaker for postoperative complete atrioventricular block. Actuarial survival at 5 years for the entire group was 87% (70% confidence interval, 81% to 93%). CONCLUSIONS: The ASO remains our preferred treatment for infants with double-outlet right ventricle and subpulmonary ventricular septal defect. However, associated anatomic defects are important risk factors.     

Blunt rupture of both atrioventricular valves

Younger patients are surviving extensive damage to the heart and supporting structures, often associated with multiple systems injuries. A 23-year-old patient who sustained blunt trauma to the chest resulting in a pericardial laceration, injury to tricuspid and mitral valves, myocardial contusion, and paresis of the left phrenic nerve is reported. Porcine bioprosthetic valve replacement of both atrioventricular valves was necessary. Prompt diagnosis and aggressive intervention of such injuries can lead to successful repair of complex cardiac trauma.