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Preoperative diagnosis of coronary artery anatomy in dextrotransposition of the great arteries.

BACKGROUND: Preoperative diagnosis of the coronary artery pattern in dextrotransposition of the great arteries is important because deviation from the usual pattern may influence the surgical strategy. METHODS: For assessment of the value of angiography and echocardiography in this setting, we analyzed the preoperative echocardiographic and angiographic findings in 74 patients with dextrotransposition of the great arteries in whom an arterial switch operation was performed. RESULTS: Two-dimensional echocardiography established a diagnosis of the coronary artery pattern in 40 of the 74 patients; that diagnosis was correct in 38-30 of 32 (94%) with the usual coronary artery pattern and 8 (100%) with an atypical pattern. Angiography established a diagnosis of the coronary artery pattern in 52 patients; that diagnosis was correct in 50-41 of 42 (98%) with the usual coronary artery pattern and 9 of 10 (90%) with an atypical pattern. The accuracy of echocardiography in diagnosing the coronary artery pattern in dextrotransposition of the great arteries in this series was 95% and that of angiography was 96%. CONCLUSION: Both echocardiography and angiography can establish the diagnosis of the coronary artery anatomy in most patients. Echocardiography is routinely performed in infants with dextrotransposition of the great arteries and is proving to be increasingly sensitive for diagnosing the coronary artery anatomy; the role of angiography may evolve to be supplementary, especially in cases in which the coronary artery anatomy is not clearly demonstrated by echocardiography.

Child, Preschool↗

Anomalies associated with the parachute mitral valve: a pathologic analysis of 52 cases.

Fifty-two specimens of heart with parachute mitral valve were reviewed. An associated anomaly was present in all but two patients. In these two patients parachute mitral valve was an isolated anomaly. The associated anomalies were divided into those which form part of the developmental complex described by Shone and associates and other anomalies designated as major anomalies. Normally related great vessels were present in 35 cases, double outlet right ventricle in eight cases, complete transposition in four cases, corrected transposition and single ventricle in two cases each and straddling tricuspid valve in one case. The most common associated anomaly was an interventricular communication occurring in 37 cases. Less common anomalies were an atrial septal defect (18 cases), patent ductus arteriosus (21 cases), complete atrioventricular canal (five cases), right-sided obstructive anomalies (13 cases) and left-sided obstructive anomalies other than those as part of the Shone syndrome (11 cases).

Double Outlet Right Ventricle↗

Coronary artery anatomy in corrected transposition of the great arteries.

Congenitally corrected transposition of the great arteries is an unusual cardiac malformation with discordant atrioventricular and ventriculoarterial alignments. Because knowledge of the coronary artery anatomy is a prerequisite for successful repair of this cardiac anomaly, selective coronary arteriography was performed in 13 children (4 male and 9 female; age range 18 months to 16 years) and 1 adult (aged 59 years) with congenitally corrected transposition of the great arteries and associated intracardiac defects. The typical coronary distribution of corrected transposition (that is, coronary artery-ventricular concordance) was found in 11 patients. In one patient, a single coronary ostium was observed; the right sinus of Valsalva gave rise to a short common branch that divided into three arteries: a left circumflex artery going to the right, a well developed left anterior descending artery running into the anterior interventricular groove and a third vessel that continued on the normal course of the right coronary artery directed posteriorly. In one patient, the left circumflex artery was particularly small. In another patient, with severe hypoplasia of the left anterior descending coronary artery, the anterior ventricular wall of the heart was supplied by three small branches that ended a short distance from their origins. The adult patient had a large anterior ventricular branch arising from the morphologic left coronary ventricular as well as a large acute marginal branch, with a wide distribution, from the morphologic right coronary artery. Presurgical coronary angiographic documentation is helpful because, in congenitally corrected transposition as well as in complex congenital heart disease, coronary anomalies (in origin, course and distribution) are occasionally present and knowledge of their presence can help determine the most appropriate surgical approach.

Child↗

Pulmonary artery remodeling in transposition of the great arteries: relevance for neoaortic root dilatation.

OBJECTIVE: Transposition of the great arteries is currently treated by performing the arterial switch operation. Dilatation of the neoaortic root is a late complication with unknown cause. Samples of patients with untreated transposition of the great arteries and patients with normally related great arteries were compared to investigate a possible role for vascular remodeling in the dilatation process. METHODS: Aortic and pulmonary artery vessel wall and sinus samples were taken from 20 untreated human heart specimens with transposition of the great arteries and 9 age-matched, normal, postmortem human heart specimens, divided into 2 groups according to age. Routine histology and immunohistochemical staining for smooth muscle cell differentiation markers alpha-smooth muscle actin, SM22, and calponin were performed. RESULTS: This study revealed structural differences between the normal aorta and pulmonary artery in the early group, which became more pronounced in the late group. In the early stage in transposition of the great arteries, no marked differences were seen between the aorta and pulmonary artery. With increasing age, however, there was a pronounced down-regulation of all smooth muscle cell markers in the pulmonary artery. CONCLUSIONS: There is a structural difference between the normal neonatal aorta and pulmonary artery. The great arteries in transposition of the great arteries differ from each other and from normal vessels, indicating a structural vascular difference in transposition of the great arteries. In the pulmonary artery and sinus of untreated transposition of the great arteries, there is a dedifferentiation of smooth muscle cells with increasing age that we could not correlate to altered flow. This structural abnormality might provide an explanation for the neoaortic root dilatation that has been reported as a late complication of the arterial switch operation.

Aorta↗

Echocardiographic diagnosis of transposition of the great arteries in a neonatal foal.

The clinical and echocardiographic findings in a 19-h-old female foal with a ventricular septal defect, patent foramen ovale, patent ductus arteriosus, and complete transpositon of the great vessels are described. Clinical signs were suggestive of complex congenital heart disease and echocardiography of the foal allowed definitive diagnosis prior to postmortem.

Abnormalities, Multiple↗

New modification of the Damus-Kaye-Stansel operation.

We report a successful modification of the Damus-Kaye-Stansel operation for transposition of the great arteries when the coronary arteries are unsuitable for transfer. The procedure includes creation of a neoaorta with end-to-end anastomosis of the proximal pulmonary artery to the distal ascending aorta and creation of an aortopulmonary window between the proximal great arteries. A valved conduit is interposed between the right ventricle and the distal pulmonary artery.

Aorta↗

Systemic atrioventricular valve regurgitation in corrected transpositon of the great vessels. Angiographic differentiation of operable and nonoperable valve deformities.

Abnormalities of the systemic atrioventricular (A-V) valve are frequently present in patients with corrected transposition. Systemic A-V valve regurgitation is usually present and may be amenable to operative correction with valve replacement if the regurgitation is caused by normally positioned but deformed valve leaflets or a dilated valve ring, or both. Systemic A-V valve regurgitation secondary to the more common Ebstein-type malformation of the inverted tricuspid valve is an inoperable conditon at present. Displacement of the valve leaflets below the level of the valve ring may be seen in the angiocardiograms of patients with this finding and is the differentiating feature between the two lesions. Ten cases of systemic A-V valve regurgitation in patients with corrected transposition are reviewed to illustrate these features.

Adolescent↗

Surgical management of single ostium anomalous coronary artery from pulmonary artery.

A 6-week-old infant presenting in heart failure was suspected on echocardiography of having anomalous origin of the whole coronary circulation from the pulmonary artery arising from a single ostium. This was confirmed at operation, and the child underwent successful correction. The management of cardiopulmonary bypass and myocardial protection in this abnormality is discussed.

Aorta, Thoracic↗

Modification of the arterial switch procedure in the presence of a rare coronary arterial pattern.

Numerous techniques have been described for translocation of the coronary arteries in the setting of the arterial switch procedure. In the case we describe, the close alignment to the aortic and the pulmonary wall of the main stem supplying the left anterior descending and circumflex arteries, and dual origin of this vessel and the right coronary artery from sinus #2, required an individual approach. After taking down the main stem feeding the left anterior descending and circumflex arteries in the usual manner, we opted for a connection maintaining the artery in its existing site, and punched a hole into the neo-aorta just in front of the coronary orifice. We then used a piece of glutaraldehyde-preserved pericardium as a hood, anastomosing the right coronary artery to the new aorta by means of the usual trap door technique.

Cardiac Surgical Procedures↗

Ventricular pacing from the coronary sinus of a patient with a Fontan circulation.

Permanent pacing of the left ventricle was achieved by transvenous implantation of a steroid-eluting electrode into the coronary sinus in a patient who had undergone a Fontan operation. This approach to endocardial pacemaker implantation is potentially of considerable value in patients who do not have transvenous access to the right ventricle.

Adult↗