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Single-stage arterial switch with aortic arch enlargement for transposition complexes with aortic arch obstruction.

BACKGROUND: Patients with transposition complexes and aortic arch obstruction are a surgical challenge with significant mortality. We have adopted an aggressive approach of concurrent aortic arch repair and arterial switch operation with excellent results. METHODS: Since 1989, 12 of 13 patients with aortic arch obstruction and transposition of the great arteries or double-outlet right ventricle with subpulmonary ventricular septal defect have undergone complete single-stage repair. One patient underwent a two-stage repair because of hemodynamic instability. The median age of repair was 27 days and the median weight was 3.5 kg. Surgical technique involved the arterial switch operation and ventricular septal defect closure when present in 12 patients. One patient with severe subaortic stenosis underwent a modified Damus-Kaye-Stansel operation with concomitant aortic arch enlargement. The aortic arch was enlarged in 12 of 13 patients with a pulmonary homograft patch. RESULTS: There have been no early deaths and only one late death at 39 months postoperatively from hepatoblastoma. The mean follow-up is 42 months. There have been no reoperations for recurrent aortic arch obstruction. All survivors are currently well from a cardiac point of view. CONCLUSIONS: Concomitant single-stage repair for transposition complexes with aortic arch obstruction achieves excellent survival and should be the surgical procedure of choice.

Aorta, Thoracic↗

Single ventricle (single- or double-inlet) complicated by subaortic stenosis: surgical options in infancy.

Intracardiac obstruction to the systemic circulation can develop in patients with many forms of congenital heart disease. When transposition of the great arteries accompanies tricuspid atresia, narrowing of the ventricular septal defect (VSD) leads to subaortic stenosis. In a similar fashion, a restrictive bulboventricular foramen compromises systemic arterial outflow in patients who have single left ventricle with subaortic outflow chamber. The same effect can be seen in VSD closure in mitral atresia with normally related great vessels. Although some forms of single ventricle can be treated by ventricular septation, the modified Fontan procedure can be more generally applied to this entity. Pulmonary vascular resistance must be low, however, which excludes the application of the Fontan principle in infants. While pulmonary artery banding may protect the lungs from the development of excessive pulmonary vascular resistance, it may also accelerate the development of subaortic obstruction. We have successfully applied the Norwood operation, as described for hypoplastic left heart syndrome, to palliate subaortic stenosis due to restrictive VSD in 3 infants with single ventricle or atrioventricular valve atresia. Consideration should be given to a primary Norwood procedure in infants with single ventricle in whom subaortic stenosis is likely to develop. Patients who do receive pulmonary artery bands should be followed closely for the development of subaortic stenosis and should undergo an early, physiologically corrective operation if it occurs.

Aortic Stenosis, Subvalvular↗

Two-dimensional echocardiography in complete transposition of the great arteries.

Sixty three patients with complete transposition of the great arteries (d-TGA) were studied by two dimensional echocardiography in order to assess: great artery relationships; ventriculo-arterial connections; presence and nature of the left ventricular outflow obstruction (LVOTO). From the parasternal short axis view at the great arteries level, four different relationships were imaged: the aorta was anterior and to the right in 35 patients, the two vessels were in a direct anterior-posterior position in 15 or side by side in 3 and the aorta was anterior and to the left in 10. Differently from normals, early pulmonary bifurcation of the artery arising from the left ventricle was imaged in 42 patients (84%) from the apical approach and in 36 (72%) from the subcostal longitudinal view. Left ventricular outflow tract obstruction was diagnosed in 29 patients (46%) and two distinct types of obstruction were differentiated: fixed and dynamic. We conclude that information derived from two-dimensional echocardiography is a valuable tool for the medical management, cardiac catheterization and surgical treatment of infants and children with d-TGA.

Adolescent↗

[Technical innovations in total extracardiac cavo-pulmonary connection in Fontan-type operation. An experimental study and first 2 operated clinical cases with excellent immediate results].

INTRODUCTION: Three technical modalities are presented with total extracardiac cava-pulmonary connection, with bidirectional Glenn, as coverage of this type of extracardiac connection for all types of cardiopathies suitable for Fontan type correction. METHODS: Development of an experimental protocol for the perfecting of the three technical variants tested: "Tunnel from inferior cava to right pulmonary artery by means of right atrial wall and vascularized pericardium", "Tunnel from inferior cava to the trunk of the pulmonary artery with or without pulmonary valve with P.T.F.E. hemiconduit" and "Tunnel from inferior cava to the pulmonary graft as a artery with P.T.F.E. graft as a hemiconduit". RESULTS: The first two clinical cases operated on using the third technical variant are presented. Both cases evolved during immediate postoperative period with a very favourable hemodynamic response, with pressures at conduit and P.A. level of 10-12 mmHg, normal cardiac output and sinus rhythm. The later clinical evolution in both cases is very satisfactory. The five cases published by Laschinger with this same technique showed a very favourable evolution during the immediate postoperative period and later. CONCLUSION: The technical facility of its performance, the slight traumatism of this surgery, the important technical advantages with respect to the total cava-pulmonary intraauricular connection and the good initial hemodynamic results make us consider the total extracardiac cava-pulmonary connection as an important contribution in this type of surgery. It is clear that time and greater clinical experience with this type of connection is required for the correct evaluation of all and each one of the numerous theoretical advantages shown and in order to detect possible disadvantages.

Adolescent↗

Concordant ventriculoarterial connections with parallel arterial trunks in the setting of isomerism of the right atrial appendages.

We describe an autopsied specimen, from an infant who died in 1967, in which concordant ventriculoarterial connections coexisted with parallel arterial trunks, usually described as "anatomically corrected malposition." Based on their shape, the extent of the pectinate muscles, and the absence of a coronary sinus, we diagnosed isomeric right atrial appendages. Despite this, the arrangement of the remaining thoracic and abdominal organs, as well as the patterns of venous drainage, was almost completely mirror imaged.

Abnormalities, Multiple↗

Internal mammary artery bypass after the arterial switch operation.

Myocardial ischemia may ensure after coronary artery translocation during the arterial switch operation. We report the successful use of a right internal mammary artery to right coronary artery bypass graft in an infant with angiographic documentation of persistent graft patency 6 months postoperatively.

Aorta↗

Influence of coronary anatomy and reimplantation on the long-term outcome of the arterial switch.

BACKGROUND: Abnormal coronary artery anatomy is reported to have a significant influence on the outcome of the arterial switch operation. This study examines the impact of coronary anatomy and the occurrence of late coronary obstruction on left ventricular (LV) function and long-term outcome. METHODS: Coronary artery anatomy, of 170 patients after arterial switch operation (1977-1999), was determined based on operative reports and pre-operative aortograms. Current status was evaluated using ECGs, echocardiograms, scintigraphy, and post-operative coronary angiograms. RESULTS: In 133/170 patients, coronary artery anatomy consisted of an anterior descending (LAD) and circumflex artery (Cx) from the left sinus and the right coronary artery (RCA) from the right or posterior sinus. The left coronary had an intramural initial course in two of these patients. Fifteen patients had the LAD from the left and Cx and RCA from the right sinus; eight had LAD and RCA from one sinus and Cx from the other; four had single ostium; and three had three separate ostia. Four patients had complex patterns and four patients had a supra commissural coronary. To date, follow-up angiography was performed in 59 patients. Surgical coronary sequellae were found in five patients. Two patients had an occluded left ostium. Initially, they were asymptomatic but showed polymorphic ventricular extrasystoles on ECG and moderate LV dysfunction with large irreversible perfusion defects on scintigraphy. Both patients developed ventricular fibrillation at the age of 14 years. One patient did not survive. The other patient required implantation of a defibrillator. One patient has an occluded RCA, one patient has stenosis of the right ostium and one patient has multiple tortuous collaterals without obstruction of a major branch. In the latter three patients, coronary sequellae were not suspected on ECG, echo, or scintigraphy and were only found on follow-up angiography. Retrograde collateral flow was noted in all three occluded coronaries. LV dysfunction, with normal coronaries, was noted in three patients. All, of these patients, had peri-operative ischaemia suggesting failure of myocardial protection. Two are now asymptomatic with mild LV dysfunction. One patient continues to have severe myocardial dysfunction and secondary aortic insufficiency. A Ross-like procedure was performed placing the original aortic valve in the neo-aortic root. Coronary artery anatomy did not influence early survival or late coronary sequellae. CONCLUSION: Abnormal coronary anatomy was not a determinant of outcome in our study. Surgical coronary obstruction is independent of original anatomy. It can be almost silent and is potentially fatal. Follow-up angiography must be considered in all patients after the arterial switch operation.

Adolescent↗

Double outlet right ventricle, non-committed ventricular septal defect and pulmonic stenosis. Anatomical and surgical considerations.

A case of double outlet right ventricle with pulmonary stenosis, a non-committed muscular ventricular septal defect occupying the sinus and trabecular portions of the ventricular septum, an additional small subtricuspid ventricular septal defect, situs solitus, atrioventricular concordance and aortic d-position is described. Atrioventricular valvular canal type malformations, splenic, systemic or pulmonary venous anomalies, all frequently seen in double outlet right ventricle with uncommitted VSD, were not present.

Blood Vessel Prosthesis↗

Arterial switch operation without coronary translocation: mid-term results.

We innovated a technique of arterial switch operation without coronary translocation in 1995, which avoids problems related to coronary artery translocation with good mid-term results. It is a better alternative for surgeons who are not well versed with coronary translocation of conventional arterial switch operation and with difficult coronary anatomy. This report deals with the mid-term results of our new technique.

Abnormalities, Multiple↗