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PubMed2001

Late results of the Rastelli operation for transposition of the great arteries.

The Rastelli operation, first performed in 1968, was developed for repair of transposition of the great arteries with associated ventricular septal defect and severe pulmonary stenosis. This operation includes placement of an intracardiac baffle to direct left ventricular blood to the aorta and an extracardiac valved conduit to establish continuity between the right ventricle and the pulmonary arteries. Over the last 3 decades, the Rastelli operation has been performed with a progressive decline in early mortality, and it remains the preferred repair for transposition, ventricular septal defect, and severe fixed valvular or subvalvular pulmonary stenosis. This chapter examines the late results of our 33-year experience with the Rastelli operation and describes our operative technique.

Abnormalities, Multiple↗
PubMed1985

Anatomically corrected malposition of the great arteries: diagnostic difficulties and surgical repair of associated lesions.

Four patients, aged 5 to 14 years, had repair of abnormalities associated with anatomically "corrected malposition," a condition associated with mitral/aortic discontinuity with atrioventricular and ventriculoarterial concordance, and with the aorta lying anterior and to the left of the pulmonary artery. All 4 patients had ventricular septal defect; in addition, 1 patient had coarctation, 2 patients had pulmonary infundibular stenosis, 1 patient had acquired pulmonary valve atresia, 1 patient had acquired fixed subaortic stenosis and 2 patients had left juxtaposition of the atrial appendages. The position of the great arteries suggest corrected transposition, but the true diagnosis is made from finding atrioventricular and ventriculoarterial concordance with wide mitral/aortic separation on the cross-sectional echocardiogram. The electrocardiogram shows normal ventricular activation. Three of the 4 patients had anticlockwise rotation of the heart, which displaced the apex. This made echocardiography difficult and caused a problem in visualizing the right-sided valved conduit and closing the VSD in conventional ways. One patient died at reoperation for an obstructed conduit. Mitral regurgitation developed after resection of subaortic stenosis in 1 patient. No arrhythmias occurred in the 3 surviving patients, followed for 1 to 5 years, but the risk of subaortic stenosis remains.

Adolescent↗
PubMed1999

The use of 'pericardial hoods' for maintaining exact coronary artery geometry in the arterial switch operation with complex coronary anatomy.

OBJECTIVE: Complex coronary artery anatomy is the major risk factor for the arterial switch operation. Of the many approaches described the 'trap door' technique for coronary reimplantation is most flexible and allows safer transfer in complex arterial configurations. However, we have occasionally been concerned regarding torsion of the vessels with this approach. We therefore explored the role of trap-door augmentation with pericardial hoods to maintain exact coronary geometry during coronary transfer. METHODS: Between February 1992 and December 1997, 80 patients underwent an arterial switch procedure at our institution. Sixty-seven patients underwent direct coronary reimplantation. In ten, coronary/great vessel anatomy was considered unfavourable and the trap-door approach was adopted primarily. In two an augmented trap-door was performed as the primary procedure and in the last patient Aubert's approach was used. In five patients during rewarming, ischaemic changes were noted on the electrocardiogram and/or regional wall motion abnormalities on transoesophageal echocardiography. This prompted revision of the appropriate coronary anastomosis. In three it was considered the anastomosis was kinked due to angulation of the button; in two the coronary was overstretched. In four, revision of the anastomosis was by pericardial hood augmentation. RESULTS: In all patients there was normalization of the electrocardiogram and immediate improvement in cardiac function documented by transoesophageal echocardiography. No early or late death occurred in the pericardial hood group nor were there any readmissions for any reason. CONCLUSIONS: Pericardial augmentation of trap-door aortic anastomoses allows for the maintenance of exact coronary artery geometry during the arterial switch procedure and minimizes the risk of myocardial ischaemia. We believe it broadens the application of the arterial switch procedure to even the most complex coronary anatomy and is a useful adjunct to the other techniques of coronary transfer.

Adolescent↗
PubMed2005

Direct microvascular repair of an infant's transected coronary artery: case report.

The authors report the direct, microvascular repair of a right coronary artery transected during reoperation fOr complications of arterial switch operation (ASO) in a 3 month-old child. This is the first documented use of direct microsurgical anastamosis in the repair of an infant's transected coronary artery. Deviation from standard coronary bypass graft repair was permitted by vessel characteristics, as well as close collaboration between plastic surgery and cardiac surgery services. Patency of repair was confirmed intraoperatively with Doppler ultrasound and through postoperative echocardiograms documenting stable right ventricular function. This case highlights the benefit of a multidisciplinary approach to an emergent clinical problem using microsurgical techniques.

Cardiac Surgical Procedures↗
PubMed1991

[Successful surgical treatment of a case of corrected transposition in situs inversus with associated anomalies].

A 33-year-old female with corrected transposition (situs inversus) with ventricular septal defect (VSD), atrial septal defect (ASD), and pulmonary stenosis (valvular and subvalvular) was operated successfully. Closure of ASD and infracristal VSD was performed through right atrial approach. In order to get complete exposure of VSD, a part of anterior leaflet of mitral valve was detached temporally. An external conduit was interposed between the morphologic left ventricle and the main pulmonary artery to relieve pulmonary stenosis. Postoperative course was uneventful except a transient complete heart block for a day. Several technical aspects of the radical operation were discussed including VSD closure method to avoid damaging the atrioventricular conducting tissues and reconstructive procedure of morphological left ventricular outflow.

Abnormalities, Multiple↗
PubMed1985

Magnetic resonance imaging of rerouted pulmonary blood flow.

Correction of structural defects of the heart associated with cyanotic heart disease often requires rerouting of systemic venous return into the pulmonary arteries and pulmonary venous return into the ventricles. Preliminary experience suggests that magnetic resonance imaging, through its superior depiction of the resultant complex anatomy, provides important supplemental information to echocardiography. Together, these noninvasive techniques may lessen the present need for repeat catheterization in children with surgically corrected cyanotic heart disease.

Blood Vessel Prosthesis↗
PubMed2004

Selective timing for the arterial switch operation.

BACKGROUND: To determine outcomes for the arterial switch operation individualized according to the underlying anatomy and clinical status. METHODS: A retrospective review of a consecutive series of infants less than 90 days of age who underwent the arterial switch operation at a single institution. RESULTS: From July 1993-April 2001, 117 infants underwent an arterial switch operation before 90 days of age. Seventy-five patients (64%) had transposition of the great arteries with intact ventricular septum with the aim of operation before 14 days of age; however, 8 of these patients had delayed presentation (range 15-46 days). Thirty-five patients (30%) had transposition with a ventricular septal defect (30 patients) or double outlet right ventricle (5 patients) and normal arch anatomy and were repaired within the first 90 days of life depending on the severity of heart failure at a median of 12 days of age (range 3-83 days). Seven patients (6%) had associated aortic coarctation (5 patients) or interrupted aortic arch (2 patients). One patient died during hospitalization (0.85% hospital mortality) and one patient died from noncardiac causes during a median follow-up of 35 months (1.7% total mortality). Four patients required intervention during follow-up (3.4%) for new aortic coarctation (2 patients), supravalvar pulmonic stenosis (1 patient), or right hemi-diaphragm paralysis (1 patient). CONCLUSIONS: Individualized timing for the arterial switch operation within the first ninety days of life produces excellent survival rates for all types of transposition physiology with the expectation of a satisfactory course during follow-up.

Coronary Vessel Anomalies↗
PubMed1976

The pulmonary vascular bed in patients with complete transposition of the great arteries.

Histological material obtained at autopsy from 35 patients over three months of age with complete transposition of the great arteries (TGA) was examined. Two of six patients less than a year of age with an intact ventricular septum and closed ductus arteriosus were found to have pulmonary vascular changes of at least grade 3 severity; in addition, two of seven patients with a large ventricular septal defect in this age group showed changes of similar severity. The reported low incidence of marked pulmonary vascular changes in patients with only an interatrial communication dying during the first year of life would appear to be due in part to the high proportion of cases less than three months old in whom there was not sufficient time for such changes to develop. Although the incidence is not as high as that found beyond a year of age, it is sufficiently high to influence the management of infants beyond three months of age. Hemodynamic studies were performed following atrial baffle repair in 34 patients. All but one of the 16 patients operated upon prior to two years of age had a pulmonary arteriolar resistance (Rpa) less than 3 units M2 at the time of review, including three with a large communication at ventricular or great vessel level who underwent surgery before two months of age. Repair at an older age did not preclude a low Rpa on follow-up, but in 11 of the 18 patients who were two or more years of age at the time of repair it was greater than 3 units M2. None of these latter patients had a large ventricular septal defect or patent ductus arteriosus. Comparison of postoperative hemodynamic data with autopsy studies would suggest that advanced pulmonary vascular disease is associated with earlier death among patients with complete transposition of the great arteries.

Cardiac Output↗
PubMed1975

[The correlation between pulmonary hypertension and histological findings of the pulmonary vessels in congenital heart disease (author's transl)].

In nearly 100 infants the hämodynamic and histological findings of pulmonary hypertension in congenital heart diseases are correlated when a banding-procedure was done. There were: 45 ventricle septal defects, 10 atrio-ventricular canals, 13 transpositions of the great arteries, 16 patent ductus arteriosus and 7 miscellaneous. A hämodynamic hypertension was more frequent than the histological hypertension; but there was a difference in the different heart diseases: The patients with patent ductus arteriosus and hypertension show only in a few cases histological alterations; in infants with VSD the correlation was much more better. Whether a banding procedure of the pulmonary artery is able to provide the progress of pulmonary sclerosis may be discussed after a second excision of lung-tissue when the correction of the heart disease is done.

Age Factors↗
PubMed1985

Surgical treatment of subpulmonary obstruction in transposition of the great arteries by means of a left ventricular-pulmonary arterial conduit. Late results and further considerations.

Between January, 1976, and June, 1983, 16 patients with transposition of the great arteries and fixed subpulmonary obstruction underwent repair by means of a combined Mustard procedure and placement of a conduit between the left ventricle and main pulmonary artery. Their mean age and weight were 5.3 years and 19 kg. Ten patients had an intact ventricular septum and six had a ventricular septal defect, which because of its size or location precluded a Rastelli repair. A fibromuscular tunnel was the most common type of subpulmonary obstruction (10/16, 62%). There were three early deaths and one late death. Early cardiac catheterization (mean interval from operation, 45 days), performed in 10 of 13 survivors, showed a significant decrease in the mean left ventricular-pulmonary arterial gradient from 66 to 8.5 mm Hg. Late cardiac catheterization (mean interval from operation, 4 years) showed continuing relief of the subpulmonary obstruction with a mean increase in peak systolic transconduit pressure gradient of 6.4 mm Hg. This procedure is an option to direct relief in the management of severe fixed subpulmonary obstruction in patients with transposition and intact ventricular septum or in the presence of a ventricular septal defect when a Rastelli repair is precluded.

Blood Pressure↗
PubMed1986

The coronary arterioles in newborns, infants and children. A morphometric study of normal hearts and hearts with aortic atresia and complete transposition.

The state of development of the intramyocardial coronary arterioles in normal hearts and hearts with congenital malformations is unknown, although these vessels play an important role in regulating myocardial blood flow. To remedy the situation, we studied 64 normal hearts (ranging in age from 23 weeks gestational age to 75 years), 13 hearts with complete transposition (5 with intact ventricular septum and 8 with a defect) and 11 hearts with classical aortic atresia. The medial thickness of arterioles with an external diameter from 20 to 100 microns was measured. The number of arterioles was calculated per mm2; a distinction was made between vessels between 20 and 100 microns and those smaller than 20 microns. The results show that the arteriolar vascular bed is not yet full grown at term. Within the first year of life the medial smooth muscle mass increases, most likely due to growth and peripheral extension onto the smaller branches. In the hearts of patients with aortic atresia who lived for a week or less, the arteriolar media appeared to lag behind that in normal hearts. In those patients who survived into the second post-natal week, however, the arteriolar bed was almost as anticipated for normal. The normal values obtained in the latter group could be due to intrinsic differences, rather than expressing actual and rapid growth.

Age Factors↗
PubMed2002

Aortic coarctation, vascular ring, and right aortic arch with aberrant subclavian artery.

Right aortic arch, in all situations, is relatively rare. In association with coarctation and vascular compression, it is extremely rare. We present a patient with a right aortic arch and an aberrant left subclavian artery, in addition to coarctation. This was dealt with through a left thoracotomy by dividing the ligamentum arteriosum and placing a Dacron graft from the ascending aorta to the descending aorta.

Aorta, Thoracic↗