Transposition of the great arteries with posterior aorta, anterior pulmonary artery, subpulmonary conus and fibrous continuity between aortic and atrioventricular valves

A newly recognized type of complete d-transposition of the great arteries is presented. The aortic valve was posterior and inferior to the pulmonary valve in 4 postmortem cases. Deficiency of the subaortic muscular conus permitted tenuous aortic-tricuspid fibrous continuity in 3 of 4 cases and tenuous aortic-mitral fibrous continuity in all by means of a high ventricular septal defect. The crista supraventricularis (parietal band) was entirely above the morphologically left ventricle and was much shorter than normal (average length 3 mm, normal average length 7 mm).

The morphogenesis of these transpositions cannot be explained by the straight truncal septum hypothesis because the truncal septum in these cases was spiral, not straight. These new findings can readily be explained by the conal growth hypothesis, and they indicate the desirability of a literal (accurate) definition of transposition of the great arteries: aorta arising above the morphologically right ventricle and pulmonary artery originating above the morphologically left ventricle. Malpositions of the great arteries include transposition, double-outlet right ventricle, double-outlet left ventricle and anatomically corrected malposition (“transposition”).

Transposition of the great arteries is a relation, not an entity. It is 3 different entities in terms of conal malformations (on the basis of these 4 cases plus a control study of 100 unselected autopsy cases of transposition, accurately defined): (1) subaortic conus with pulmonary-mitral fibrous continuity, 92 percent; (2) subaortic and subpulmonary (bilateral) conus without semilunar-atrioventricular fibrous continuity, 8 per cent; and (3) markedly foreshortened subpulmonary conus with tenuous aortic-atrioventricular fibrous continuity, as in these 4 cases, much less than 1 percent.     

Long-term results of Mustard operation in transposition of the great arteries. Angiographic and nuclear medicine study of ventricular function]

The fate of the right ventricle as systemic ventricle after atrial repair of complete transposition of the great arteries has not been clearly elucidated. In order to assess the long-term results of the Mustard operation in patients with complete transposition of the great arteries we present the clinical data of 23 patients who had been operated in the years 1974 and 1975. Twenty of these patients had simple complete transposition of the great arteries with intact ventricular septum, two had an additional small ventricular septal defect and one an additional left ventricular outflow tract obstruction with a 40 mm Hg systolic pressure gradient. The Mustard operation had been performed at a mean age of 2.2 (1 to 3.7) years. Seventeen of the 23 patients underwent a postoperative hemodynamic study with angiocardiography 1.1 (1 to 1.8) years following surgery. At that time the right ventricular ejection fraction, which had been calculated from biplane angiographic right ventricular volume measurements in twelve patients was 62 (52 to 68) %. However the right ventricle was dilated and the mean enddiastolic volume was 132 (108 to 192) % of normal. In twelve of the 23 patients right ventricular function was reassessed 12.6 (11 to 15.3) years after surgery by Technetium-99m-scintigraphy at rest and in ten of those after exercise with a workload of 2 watt/kg. The mean ejection fraction was 51 (38 to 66) % at rest and 52 (40 to 80) % during exercise. Only three patients had a normal response to exercise, which was defined as an increase of ejection fraction with exercise of more than 5%.(ABSTRACT TRUNCATED AT 250 WORDS)     

Conduction disturbance and pacemaker therapy in patients with corrected transposition of the great arteries.

We observed 4 adult patients with corrected transposition of the great arteries (CTGA) who developed complete atrioventricular block: of 4 patients, 2 received endocardial pacemakers (DDD mode), 1 an epicardial pacemaker (VVI mode), and 1 patient did not have a permanent pacemaker implantation. Endocardial lead fixation in the systemic venous atrium and ventricle is adequate to provide permanent electrode stability in patients with CTGA.     

完全型大动脉转位的形态观察

有关完全性大动脉转位的定义,经复习文献和统一于Van Praagh的意见下,指两个大动脉外在关系有所改变,同时它们必须互相越过室间隔,并与不一致的心室腔相连接。本文研究基于对10例的形态观察,其房室连接均一致和心室动脉连接均不一致。7例属心房正位,2例属心房反位和1例为左侧型心耳并列。主要伴随畸形有:4例膜部间隔完全未发育,1例部分未发育,7例合并室间隔缺损,3例有房室瓣骑跨和1例肺动脉根部闭锁,5例有较重的冠状动脉畸形,其中4例为单个冠状动脉。     

Aortic atresia occurring with complete transposition of great arteries.

Aortic atresia occurring with complete transposition of the great arteries (ventriculoarterial discordance) has not previously been reported. A patient with this condition is described, who is alive and relatively well at the age of 6 years. This survival contrasts conspicuously with that of patients with the far commoner situation of aortic atresia with normally connected great arteries. It is suggested that this difference in longevity is a result of the greater ability of the left ventricle to supply both the systemic and pulmonary circulations.     

Anatomic problems associated with arterial switch procedures for double outlet right ventricle with subpulmonary ventricular septal defect

Two cases of double outlet right ventricle with subpulmonary ventricular septal defect treated by arterial switch operations are reported. The anatomical problems of coronary artery transfer, occult outflow tract obstruction and position of the pulmonary bifurcation are discussed. Cases of double outlet right ventricle and subpulmonary ventricular septal defect with anterior-to-posterior relation of the great arteries are suited to the repair techniques pioneered by Jatene for complete transposition and ventricular septal defect. Cases in which the great arteries are side-by-side pose more difficult problems, partly because of the more complex and varied anatomy of the coronary arteries, and because of the spatial relation of the roots of the great arteries. Although it may be possible to overcome these technical problems, we have reservations about the reproducibility of such a procedure. We believe, however, that cases of this type are best treated without recourse to "inflow" correction. The options are either the arterial switch procedure or a modified Rastelli operation.     

Double outlet right ventricle: Hemodynamic and anatomic correlations

There are 16 possible variations of double outlet right ventricle with regard to interrelations of the great arteries and to location of the ventricular septal defect. In a series of 62 cases, approximately two thirds of patients had the great arteries in a side by side relation, and most (28 of 41) had the ventricular septal defect in a subaortic position. In double outlet right ventricle with malposition of the great arteries, the ventricular septal defect was either subpulmonary or subaortic. Four of the 13 patients with subpulmonary ventricular septal defect had a supracristal defect with side by side relation of the great arteries (Taussig-Bing anomaly), and 9 patients had malposition of the great arteries with an infracristal ventricular septal defect. In all patients with subpulmonary ventricular septal defect, pulmonary arterial oxygen saturation was greater than systemic arterial saturation regardless of the relation of the great arteries.Forty patients had subaortic ventricular septal defect. In 24 of these patients, including 7 with malposition of the great arteries, systemic arterial oxygen saturation was greater than pulmonary arterial saturation. However, in 9 patients (25 percent) the reverse was true, as seen in complete transposition of the great arteries and in Taussig-Bing anomaly. Thus, pulmonary arterial oxygen saturation greater than systemic arterial saturation is not reliable evidence of a Taussig-Bing anomaly. Of the 25 patients with such saturation, only 4 had the Taussig-Bing anomaly.     

Rules for diagnosis of arterioventricular discordances and spatial identification of ventricles. Crossed great arteries and transposition of the great arteries.

Rules are presented for the diagnosis of arterioventricular discordances and the spatial position of the ventricles in these cardiopathies by means of angiocardiography and the position of cardiac catheters. Because these rules are based on previous anatomo-embryological findings, the normal development of the conus and the truncus is briefly analysed. The probable morphogenesis of this group of truncoconal cardiopathies is discussed. The fundamental process required to establish the diagnosis of these cardiopathies is as follows: 1) The truncoconal morphology is identified in the lateral projection. a) The anterior position of the pulmonary artery and its infundibulum with respect to the aorta and its infundibulum is characteristic of crossed great arteries with arterioventricular concordance or discordance. b) The anterior position of the aorta and its infundibulum with respect to the pulmonary artery and its infundibulum is characteristic of transposition of the great arteries with arterioventricular concordance or discordance. 2) Once the truncoconal morphology is identified, the use of the anteroposterior projection allows the establishment of the differential diagnosis between arterioventricular concordances and discordances, and of the spatial location of the ventricles in these entities. a) An anterior pulmonary artery directed from right to left, emerging from an infundibulum placed on the left side (anatomically right ventricle on the left) or an anterior pulmonary artery directed from left to right, arising from an infundibulum located to the right (anatomically right ventricular placed on the right), is the specific image of discordant crossed great arteries. b) An anterior pulmonary artery directed from right to left emerging from an infundibulum placed on the right side (anatomically right ventricle on the right side) or the anterior pulmonary artery directed from left to right arising from a left-sided infundibulum (anatomically right ventricle placed on the left side) is characteristic of concordant crossed great arteries. c) An anterior aorta placed to the right of the pulmonary artery and emerging from a left-sided infundibulum (anatomically right ventricle placed on the left side) or an anterior aorta placed to the left of the pulmonary artery and arising from an infundibulum placed on the right side (anatomically right ventricle placed on the right) is characteristic of discordant transposition of the great arteries. d) An anterior aorta placed to the right of the pulmonary artery emerging from a right-sided infundibulum (anatomically right ventricle placed on the right) or an anterior aorta placed to the left of the pulmonary artery arising from an infundibulum placed on the left (anatomically right ventricle placed on the left) is the specific picture of concordant transposition of the great arteries...     

Transcatheter Device Closure of a Perimembranous Ventricular Septal Defect in Congenitally Corrected Transposition of the Great Arteries

The majority of patients with congenitally corrected transposition of the great arteries, also known as transposition of the great arteries {S,L,L} have ventricular septal defects (VSD), most commonly perimembranous VSD (pmVSD). Transcatheter device closure of pmVSD in these patients has not been widely described. We present a case of device closure of pmVSD in L-TGA with an Amplatzer Duct Occluder II (ADOII) device using a deployment starting in the subpulmonary left ventricle. The case demonstrates some of the technical advantages of the ADOII device for VSD closure, specifically its low profile, symmetric shape, and soft material. These characteristics are advantageous in closing conventional pmVSD but are especially useful in patients with challenging anatomic substrates.     

Catheterization of the artery originating from the left ventricle by means of flow-directed catheter. A new technic]

The described heart catheterization technique with the Swan-Ganz-flow-directed catheter allows the catheterization of the aorta or, in transposition of the great arteries, the pulmonary artery, from the left ventricle via a venous entrance form the saphenous vein if an interatrial connection exists (patent foramen ovale, atrial septal defect). With the help of a stiff steel guide wire rail it is possible in the apex of the left ventricle to bend the catheter sharply toward the outflow tract. This technique was used on 51 children. Every catheterization attempt was successful . It is possible by avoiding the retrograde arterial catheter technique carry out to the complete examination of the left heart and the great artery via a venous entrance.     

Congenitally corrected transposition of the great arteries and participation in competitive sport

Congenitally corrected transposition of the great arteries is a rare anomaly, where the systemic circulation is supported by the morphological right ventricle. We present a 43 year-old asymptomatic male, a former competitive short-distance runner, with recently diagnosed congenitally corrected transposition of the great arteries. To our knowledge this is the first report of such a case.     

Rules for diagnosis of arterioventricular discordances and spatial identification of ventricles. Crossed great arteries and transposition of the great arteries.

Rules are presented for the diagnosis of arterioventricular discordances and the spatial position of the ventricles in these cardiopathies by means of angiocardiography and the position of cardiac catheters. Because these rules are based on previous anatomo-embryological findings, the normal development of the conus and the truncus is briefly analysed. The probable morphogenesis of this group of truncoconal cardiopathies is discussed. The fundamental process required to establish the diagnosis of these cardiopathies is as follows: 1) The truncoconal morphology is identified in the lateral projection. a) The anterior position of the pulmonary artery and its infundibulum with respect to the aorta and its infundibulum is characteristic of crossed great arteries with arterioventricular concordance or discordance. b) The anterior position of the aorta and its infundibulum with respect to the pulmonary artery and its infundibulum is characteristic of transposition of the great arteries with arterioventricular concordance or discordance. 2) Once the truncoconal morphology is identified, the use of the anteroposterior projection allows the establishment of the differential diagnosis between arterioventricular concordances and discordances, and of the spatial location of the ventricles in these entities. a) An anterior pulmonary artery directed from right to left, emerging from an infundibulum placed on the left side (anatomically right ventricle on the left) or an anterior pulmonary artery directed from left to right, arising from an infundibulum located to the right (anatomically right ventricular placed on the right), is the specific image of discordant crossed great arteries. b) An anterior pulmonary artery directed from right to left emerging from an infundibulum placed on the right side (anatomically right ventricle on the right side) or the anterior pulmonary artery directed from left to right arising from a left-sided infundibulum (anatomically right ventricle placed on the left side) is characteristic of concordant crossed great arteries. c) An anterior aorta placed to the right of the pulmonary artery and emerging from a left-sided infundibulum (anatomically right ventricle placed on the left side) or an anterior aorta placed to the left of the pulmonary artery and arising from an infundibulum placed on the right side (anatomically right ventricle placed on the right) is characteristic of discordant transposition of the great arteries. d) An anterior aorta placed to the right of the pulmonary artery emerging from a right-sided infundibulum (anatomically right ventricle placed on the right) or an anterior aorta placed to the left of the pulmonary artery arising from an infundibulum placed on the left (anatomically right ventricle placed on the left) is the specific picture of concordant transposition of the great arteries...     

Anatomical correction for complete transposition and double outlet right ventricle: intermediate assessment of functional results

Thirty three patients were followed up after anatomical correction of transposition of the great arteries or double outlet right ventricle and subpulmonary ventricular septal defect (Taussig-Bing anomaly). There were no late deaths and clinical progress was excellent. Cardiac catheterisation was performed in 17 patients two weeks to 44 months after operation. There were six patients with simple transposition, six with complete transposition and large ventricular septal defect, and five with the Taussig-Bing anomaly. Pressure gradients across the right ventricular outflow tract ranged from 5 to 72 mm Hg, being greater than 40 mm Hg in five patients. No patient was shown to have important valvar regurgitation and in 15 patients the coronary anastomoses were widely patent. Left ventricular function was assessed from digitised ventriculograms. Ventricular volume, shape, and ejection fraction were all normal for the group although patients with complex transposition showed a significantly lower mean (SD) ejection fraction than those with simple transposition (62(9) vs 77(9]. Analysis of regional wall motion showed a totally normal pattern in four patients; however, in seven patients a characteristic abnormality of anterior hypokinesis with delayed onset of inward wall motion was seen. Anatomical correction of transposition of the great arteries and the Taussig-Bing anomaly can be performed with satisfactory anatomical and functional results. The implications of the left ventricular wall motion abnormalities is unknown.     

Anatomical correction for complete transposition and double outlet right ventricle: intermediate assessment of functional results.

Thirty three patients were followed up after anatomical correction of transposition of the great arteries or double outlet right ventricle and subpulmonary ventricular septal defect (Taussig-Bing anomaly). There were no late deaths and clinical progress was excellent. Cardiac catheterisation was performed in 17 patients two weeks to 44 months after operation. There were six patients with simple transposition, six with complete transposition and large ventricular septal defect, and five with the Taussig-Bing anomaly. Pressure gradients across the right ventricular outflow tract ranged from 5 to 72 mm Hg, being greater than 40 mm Hg in five patients. No patient was shown to have important valvar regurgitation and in 15 patients the coronary anastomoses were widely patent. Left ventricular function was assessed from digitised ventriculograms. Ventricular volume, shape, and ejection fraction were all normal for the group although patients with complex transposition showed a significantly lower mean (SD) ejection fraction than those with simple transposition (62(9) vs 77(9]. Analysis of regional wall motion showed a totally normal pattern in four patients; however, in seven patients a characteristic abnormality of anterior hypokinesis with delayed onset of inward wall motion was seen. Anatomical correction of transposition of the great arteries and the Taussig-Bing anomaly can be performed with satisfactory anatomical and functional results. The implications of the left ventricular wall motion abnormalities is unknown.     

Corrected transposition of the great vessels in the adult. Presentation of a case simulating ischemic cardiopathy

We report the clinical and laboratory findings in a 58 years old woman with corrected transposition of the great arteries (CTGA), who that presented typical angina pectoris. The diagnosis of ischemic heart disease was supported by the history of a previous myocardial infarction. Other findings were a systolic murmur of mild mitral regurgitation, left bundle branch block and enlarged left ventricle on the chest X-ray. Cardiac catheterization showed a corrected transposition of the great arteries (L-malposition with situs solitus); left and right coronary arteries were free of luminal stenosis. We suggest therefore that anginal chest pain may be due to myocardial ischemia induced by discrepancy between myocardial oxygen consumption and coronary blood flow. This complication may occur in patients with corrected transposition of great arteries surviving in adulthood.     

Selective coronary arteriography in congenitally corrected transposition of the great arteries.

Three cases of congenitally corrected transposition of the great arteries in adults who underwent selective coronary arteriography are presented. The morphologic features of the epicardial coronary anatomy are distinctive and are identifiable angiographically as morphologically right and left coronary arteries that are specifically concordant with the morphologically right and left ventricles. This relation is constant in the presented cases, in previously published coronary arteriograms of congenitally corrected transposition of the great arteries and in a review of the anatomic studies of congenitally corrected transposition of the great arteries that identify the coronary arterial pattern. Thus the angiographic characteristics of the epicardial coronary arterial pattern permit identification of the morphologic features of the underlying ventricle regardless of other spatial relations.     

Comparison of ventricular function after Senning and Jatene procedures for complete transposition of the great arteries

Postoperative right (RV) and left ventricular (LV) volume characteristics in patients with complete transposition of the great arteries were studied to compare ventricular function after Senning and Jatene procedures and to analyze RV dimensional change during systole in patients after the Senning procedures. RV end-diastolic volume (EDV) was 181 +/- 74% of normal (mean +/- standard deviation) and RV ejection fraction (EF) was 0.48 +/- 0.09 in 15 patients who underwent the Senning procedure. In 9 patients who underwent the Jatene procedure, LVEDV was 152 +/- 27% of normal and LVEF was 0.61 +/- 0.09. One patient with aortic regurgitation, 1 with aortic regurgitation and residual ventricular septal defect, and 1 with aortic regurgitation and generalized LV wall hypokinesia of unknown cause had large LVEDVs. Pulmonary ventricular EDV and EF were within normal ranges except in the patients with persistent pulmonary hypertension, who had large EDVs and low EFs regardless of the anatomic type of ventricle, either the left or right. The study of RV dimensional change in the Senning group showed a reduced systolic shortening of the anteroposterior diameter compared with the preoperative transposition of the great arteries and normal. This reduced shortening may be related to postoperative adhesion of the RV free wall to the anterior chest wall and fixation of the atrium secondary to the intraatrial repair. In conclusion, systemic ventricular function after intraatrial repair for complete transposition of the great arteries is depressed by unavoidable residua and sequelae: persistent RV hypertension, anatomy of the right ventricle and, possibly, postoperative adhesions.(ABSTRACT TRUNCATED AT 250 WORDS)     

Hang-out time of pulmonary valve in d-transposition of great arteries.

Recently it has been shown that in patients with transposition of the great arteries the isometric relaxation time of the left ventricle could be negative in the presence of normal pulmonary artery pressure. In order to find an explanation for this apparently paradoxical situation, it was decided to evaluate the importance of the delay of closure of the pulmonary valve in 15 patients with transposition of the great arteries. This delay is called the hang-out time. The hang-out time was found to correlate inversely with the isometric relaxation time as well as with the pulmonary artery systolic (r = -0 . 70) and mean pressures. A weaker correlation was found between the isometric relaxation time and the time of mitral valve opening. These results show that in transposition of the great arteries with normal pulmonary artery pressure, the pulmonary valve has a prolonged hang-out time, to the extent that it frequently closes after the opening of the mitral valve, explaining the negative isometric relaxation time found in these cases. This finding may help in the non-invasive assessment of the pulmonary vascular resistance of patients with transposition of the great arteries.     

Appearance of noncompaction-like remodeling of the anatomical right ventricle in a middle-aged subject with modified transposition of the great arteries who did not undergo surgery

We describe noncompaction-like remodeling of the anatomical right ventricle (ARV) in a middle-aged subject with modified transposition of the great arteries (TGA). A 54 year-old male had been diagnosed with modified TGA at age 40, but no surgery was performed. Enhanced multislice CT revealed the ascending aorta coursing left of the anterior pulmonary trunk. Furthermore, the myocardium of the ARV appeared thickened, but contrast material could be observed in the ARV myocardium, which resembled noncompaction of the left ventricle (LV). We speculated trabecular development of the ARV, such as a Chiari network, and with TGA, the ARV provided systemic circulation through the aorta. The ARV wall may have thickened due to systemic pressure load, resulting in reduction of wall motion of the ARV on transthoracic echocardiogram. Recent advances in multislice CT imaging have revolutionized the exploration of RV anatomy, especially for depicting the three-dimensional appearance of noncompaction-like remodeling of the ARV in modified TGA in addition to transposition of the great arteries.