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PubMed1985

Congenitally corrected transposition in the adult: detection by radionuclide angiocardiography.

Congenitally corrected transposition (CCT) of the great vessels is an uncommon anomaly usually detected in children because of associated severe cardiac malformations. When these are absent, patients may be hemodynamically normal, but associated cardiac abnormalities are usually present in CCT, leading to symptoms in adulthood. When CCT is suggested in an adult, diagnosis by means of ultrasound may be difficult. Radionuclide angiocardiography (RA) is a simple, noninvasive method by which to diagnose CCT. We found consistent morphologic scintigraphic results in 13 patients with proved CCT, leading to establishment of reliable diagnostic criteria. In all instances of situs solitus the aorta ascends and descends on the left with vertical orientation. In the case of situs inversus, the aorta ascends and descends on the right with vertical orientation. Our criteria are independent of the situs and cardiac position, unlike earlier reports by others. We believe images obtained in the anterior projection are sufficient for the study. The practical application of RA study in patient diagnosis is demonstrated, giving special attention to patients referred because of situs solitus and dextrocardia, in which CCT is known to be present in 50% of cases.

Adolescent↗
PubMed2002

Anatomic variability in coronary arterial distribution with regard to the arterial switch procedure.

BACKGROUND: We investigated the coronary arterial origins and course and the position of the great arteries in hearts with discordant ventriculoarterial connections. At the same time, we sought to evaluate the practicality of alphanumeric classifications in accounting for surgically relevant features of the coronary arteries. METHODS AND RESULTS: We studied 200 postmortem hearts, noting the patterns of coronary arterial branching, the vertical and horizontal location of the arterial orifices within the aortic sinuses, the course of the proximal coronary arteries in relation to the aortic wall, and the relations of the great arteries and their respective commissures. All hearts examined had concordant atrioventricular and discordant ventriculoarterial connections. We found 7 of the 8 predicted patterns for sinusal origin of the 3 major coronary arteries and identified 5 different positions of the arterial trunks relative to each other. A correlation was found between less frequent relationships of the arterial trunks and unusual patterns of coronary arterial branching, as well as with mismatch between the valvar commissures. CONCLUSIONS: The surgically relevant features of the coronary arteries in hearts with discordant ventriculoarterial connections are best described rather than classified. Correlations exist between certain, less frequent relations of the great arteries and unusual patterns of branching of the coronary arteries. The presence of unusual great arterial positions should alert the surgeon to potentially complicated arrangements of the origin and distribution of the coronary arteries.

Aortic Valve↗
PubMed1991

"Anatomically corrected malposition": a rare case with bilateral absence of a complete subarterial muscular infundibulum.

A unique case of anatomically corrected malposition of great arteries with a bilateral absence of a complete subarterial muscular infundibulum is presented. The other unusual feature of the case was absence of any complex associations and interestingly an intact ventricular septum. The presenting lesion was valvar pulmonary stenosis and balloon valvotomy could not be achieved because of abnormal relationship of the great vessels.

Adult↗
PubMed1997

Three-vessel view of the fetal upper mediastinum: an easy means of detecting abnormalities of the ventricular outflow tracts and great arteries during obstetric screening.

The three-vessel view is a transverse view of the fetal upper mediastinum is as simple to obtain as the four-chamber view. It demonstrates the main pulmonary artery, ascending aorta and superior vena cava in cross- or oblique sections. The purposes of this study were to describe the normal anatomy of the three-vessel view and to analyze what anatomical changes would occur in this view when there are lesions of the ventricular outflow tracts and/or great arteries. Sonograms of 29 fetuses with lesions involving the ventricular outflow tracts and/or great arteries were reviewed. Three-vessel views were evaluated in terms of vessel size, number, arrangement and alignment. Twenty-eight of 29 fetuses showed an abnormal three-vessel view that included abnormal vessel size (n = 12), abnormal alignment (n = 8), abnormal arrangement (n = 7) and abnormal vessel number (n = 3). The vessel size was abnormal in obstructive lesions of the right (n = 4) or the left (n = 8) side of the heart. An abnormal alignment was seen in tetralogy of Fallot (n = 6) and double-outlet right ventricle (n = 2) that showed anterior displacement of the aorta. An abnormal arrangement was seen in complete (n = 4) and corrected (n = 1) transposition, double-outlet right ventricle (n = 1) and pulmonary atresia with ventricular septal defect (n = 1). Only two vessels were seen in truncus arteriosus (n = 1). Four vessels were seen in persistent left superior vena cava (n = 2). A fetus with pulmonary atresia and intact ventricular septum showed a normal three-vessel view. In conclusion, most of the lesions involving the ventricular outflow tracts and/or great arteries showed an abnormal three-vessel view.

Aortic Valve Stenosis↗
PubMed2003

Development of the coronary arteries in a murine model of transposition of great arteries.

Transposition of great arteries in humans is associated with a wide spectrum of coronary artery patterns. However, no information is available about how this pattern diversity develops. We have studied the development of the coronary arteries in mouse embryos with a targeted mutation of perlecan, a mutation that leads to ventriculo-arterial discordance and complete transposition in about 70% of the embryos. The perlecan-deficient embryos bearing complete transposition showed a coronary artery pattern consisting of right and left coronary arteries arising from the morphologically dorsal and ventral sinuses of Valsalva, respectively. The left coronary artery gives rise to a large septal artery and runs along the ventral margin of the pulmonary root. In the earliest embryos where transposition could be confirmed (12.5 d post coitum), a dense subepicardial vascular plexus is located in this ventral margin. In wild-type mice, however, capillaries are very scarce on the ventral surface of the pulmonary root and the left coronary artery runs dorsally to this root. We suggest that the establishment of the diverse coronary artery patterns is determined by the anatomical arrangement and the capillary density of the peritruncal vascular plexus, a plexus that spreads from the atrio-ventricular groove and grows around the aortic or pulmonary roots depending on the degree of the short-axis aortopulmonary rotation. This simple model, based on very few assumptions, might explain all the observed variation of the coronary artery patterns in humans with transposition, as well as our observations on the perlecan-deficient and the normal mice.

Animals↗
PubMed2002

Coronary artery pattern and outcome of arterial switch operation for transposition of the great arteries: a meta-analysis.

BACKGROUND: Prior studies of coronary pattern and outcome after arterial switch operation (ASO) for transposition of the great arteries (TGA) have been hindered by limited statistical power. This meta-analysis assesses the effect of coronary anatomy on post-ASO mortality, both overall and adjusted for time. METHODS AND RESULTS: A literature search revealed 9 independent series that reported post-ASO mortality by coronary pattern in a total of 1942 patients. Odds ratios comparing all-cause mortality in patients with usual versus variant coronary patterns were calculated and combined by use of an empirical Bayesian model. Single coronary patterns, both of which loop around the great vessels, were associated with significant mortality (OR 2.9, 95% CI 1.3 to 6.8), whereas looping patterns that arose from 2 separate ostia were not (OR 1.2, 95% CI 0.8 to 1.9). This latter group includes patients with the most common variant, circumflex from right coronary artery. Patients with an intramural coronary artery had the greatest mortality (OR 6.5, 95% CI 2.9 to 14.2). Overall, patients with any variant coronary pattern had nearly twice the mortality seen in those with the usual pattern (OR 1.7, 95% CI 1.3 to 2.4). Single ostium patterns and intramural coronary arteries remained associated with significant added mortality after adjustment for time-trend effects. CONCLUSIONS: Over the past 2 decades, patients with common coronary variants have undergone ASO without added mortality compared with those with the usual coronary pattern. Those with intramural or single coronary arteries have significant added mortality that has persisted over time.

Coronary Vessel Anomalies↗
PubMed1982

[Malpositions of the heart and the great vessels by CT (author's transl)].

The CT-findings of the most important kinds of malpositions of the heart are described. The differentiation between the venous and arterial heart cavities is possible noninvasively using a rapid bolus injection of contrast medium. If there is a malposition of the heart, uncertain findings of the heart configuration in the plain chest film can be explained by CT. Anomalies of the great vessels are detectable.

Dextrocardia↗
PubMed2005

Arterial switch operation for transposition of the great arteries with coronary arteries from a single aortic sinus.

BACKGROUND: The reimplantation of the coronary arteries from a single aortic sinus (single sinus coronary artery) in an arterial switch operation remains a technically challenging procedure. The technique of coronary transfer in this situation should be individualized depending on coronary ostial anatomy. We reviewed our techniques of coronary reimplantation with early and midterm results. METHODS: Among 103 patients who underwent arterial switch operations from March 1994 to June 2004, 16 (15.5%) had single sinus coronary artery (median age, 9 days; mean body weight, 3.5 kg). Fourteen patients (14/16, 87.5%) had coronary arteries from right facing sinus (sinus 2). Of these 14 patients, 11 had separate ostia including intramural course of the left coronary artery (n = 9) and 3 had single ostium. Two patients (2/16, 12.5%) had coronary arteries from left facing sinus (sinus 1) with single ostium. Aortic arch obstruction was associated in 5 patients. RESULTS: All 5 single sinus coronary arteries with single ostium were reimplanted with the trap-door technique. Of the 11 patients with separate ostia, 8 underwent coronary transfer with the aortocoronary flap technique and 3 with the double-button technique. Two of the 3 patients who underwent the double-button technique required left coronary artery bypass using left subclavian artery free graft as the salvaging procedure. There was one early death (1/16, 6.3%), which occurred during our earlier experience, in a patient who had arch anomaly and intramural left coronary artery. There was no late death. All but one patient had good ventricular function. CONCLUSIONS: All single sinus coronary artery with single ostium can be transferred with the trap-door technique with excellent results. The aortocoronary flap technique in those with separate ostia with or without intramural left coronary artery may be a good option. However, reimplantation of the intramural left coronary artery using separate coronary buttons should be performed with great care.

Anastomosis, Surgical↗
PubMed1991

A case of an intramural coronary artery associated with transposition of the great arteries.

One case of an intramural coronary artery in association with transposition of the great arteries is presented. A 16-month-old infant was admitted for the second-stage operation of complete transposition after a subclavian patch plasty for the aortic isthmus stenosis, division of the persisting arterial duct, and pulmonary artery banding had been performed in the first-stage operation soon after birth. The primarily planned arterial switch operation was, however, not performed because of an unusual coronary artery morphology and pattern--a separated origin of both coronary arteries from the posterior, "facing" sinus of Valsalva and an intramural course of the left anterior descending artery. This very rare case of coronary artery variation is presented from the surgical point of view.

Abnormalities, Multiple↗
PubMed1987

Diagnosis of coronary artery anatomy by two-dimensional echocardiography in patients with transposition of the great arteries.

With the increasing popularity of the Jatene procedure for the treatment of common or D-transposition of the great arteries (D-TGA), the preoperative definition of coronary artery anatomy in D-TGA has assumed great importance. Consequently, the reliability of two-dimensional echocardiography for determining the coronary artery anatomy was studied in 32 infants with D-TGA. Surgical observation of the coronary anatomy was used to assess the accuracy of the echocardiographic diagnosis. The coronary arteries were visualized in 29 of 32 patients (90%), predominantly with the use of parasternal and apical views. In the three remaining patients visualization of the coronary arteries was inadequate to allow determination of their anatomy. The coronary artery anatomy was correctly predicted in 25 of the 29 patients in whom the coronary arteries were visualized. The anatomic patterns included usual coronary anatomy for D-TGA (n = 16), left circumflex coronary from the right coronary artery (n = 6), single right coronary artery (n = 1), single left coronary artery (n = 1), and inverted origin of the coronary arteries (n = 1). The errors in the remaining four patients were (1) false-negative diagnosis of origin of the left circumflex coronary from the right coronary artery (n = 1); (2) false-positive diagnosis of origin of the left circumflex coronary from the right coronary artery (n = 1), and (3) diagnosis of origin of the left circumflex coronary from the right coronary artery when the correct diagnosis was single right coronary artery (n = 2).(ABSTRACT TRUNCATED AT 250 WORDS)

Coronary Vessels↗
PubMed1988

Early results for anatomic correction of transposition of the great arteries and for double-outlet right ventricle with subpulmonary ventricular septal defect.

Fifty patients, 21 with transposition of the great arteries plus ventricular septal defect, four with double-outlet right ventricle plus ventricular septal defect, and 25 with transposition of the great arteries plus intact ventricular septum, have undergone anatomic correction. There were four hospital deaths, all in the patients with ventricular septal defects (overall mortality rate 8%). The Lecompte maneuver was used in all patients, and in the last 25 patients the coronary arteries were transposed to medially hinged trapdoor flaps created in the neoaorta. In 10 patients (20%) intraoperative adjustments were necessary to the great vessels and coronary arteries to achieve unobstructed coronary artery flow. There have been no late deaths. Postoperative cardiac catheterization in 19 patients has revealed stenosis on the new main pulmonary artery suture line to be the main early problem. Anatomic correction of transposition (with or without ventricular septal defect) and double-outlet right ventricle septal defect would seem to be a good operative alternative to the Mustard or Senning operation, with the advantage of incorporating the left ventricle into the systemic circulation.

Aorta↗