彩色多普勒超声心动图诊断完全性大动脉转位

目的 探讨彩色多普勒超声早期诊断完全性大动脉转位的价值。方法 采用彩色超声检查完全性大动脉转位80例,按顺序分段法进行。结果 80例均显示主动脉在前,发自右心室,肺动脉在后,发自左心室,合并畸形包括VSD、ASD、PDA、PS等。其中2例手术病例及7例尸解病例与超声诊断相符。结论 超声心动图能准确诊断大动脉转位及伴随畸形,并提供部分血流动力学资料。     

Diagnosis of ventriculo-arterial discordance (transposition of the great arteries) by contrast echocardiography.

The constant anatomical relation of the aortic arch and the pulmonary artery bifurcation permits echocardiographic differentiation of the two great arteries when viewed from the suprasternal notch. When this technique was employed with contrast echocardiography in 12 infants, 6 of whom had ventriculo-arterial discordance (transposition of the great arteries), it was possible to establish with certainty in all but 1 case which great artery was most directly connected to the systemic venous return.     

Congenitally corrected transposition of the great arteries: An update

Opinion statement  Congenitally corrected transposition of the great arteries is a rare condition in which systemic venous blood returns to normally positioned atria. However, the atria are connected to the opposite ventricle, right atrium to left ventricle, left atrium to right ventricle; so-called atrioventricular disordance. In addition, the ventricles are inverted (right to left change in position) and are connected to the opposite great artery, left ventricle to pulmonary artery, right ventricle to aorta; thus, forming ventricular-arterial discordance. The aorta is anterior and to the left of the pulmonary artery, L-transposed. Atrioventricular discordance plus ventricular-arterial discordance results in normal blood flow (ie, congenitally corrected). The right ventricle with the tricuspid valve is the systemic ventricle. Common associated conditions are ventricular septal defects (VSDs), pulmonary stenosis, and congenital heart block. Major issues related to management revolve around the status of the systemic right ventricle, which can develop dysfunction with increasing age and tricuspid regurgitation, which can increase in severity with age and contribute to ventricular dysfunction. One emerging treatment is the double switch operation. In patients with no pulmonary obstruction, it is possible to switch the systemic and pulmonary venous return using an atrial baffle procedure followed by an arterial switch procedure. This results in the anatomical left ventricle now functioning as the systemic ventricle. In those patients with associated pulmonary obstruction and a VSD, another type of double switch can be performed in which the left ventricle is tunneled through the VSD to the aorta, the right ventricle is connected to the pulmonary artery with a homograft or other conduit, and the atrial baffle procedure is performed. The most difficult challenge is choosing the patient who is a candidate for the double-switch operation and the timing of that operation, or the timing of a more classical operation for associated defects.     

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.     

Coronary anatomy in congenitally corrected transposition of the great arteries

BACKGROUND: The advent of double switch procedures for the treatment of transposition of the great arteries with L-looped ventricles, e.g. typical congenitally corrected transposition of the great arteries, has made delineation of the coronary artery anatomy in these hearts important. Previous studies have suggested a consistently inverted coronary arterial pattern. METHODS: A morphologic study was conducted of the coronary arterial anatomy of all heart specimens in our registry of approximately 2600 hearts with segmental anatomy [S,L,L] (situs solitus of the viscera and atria, ventricular L-loop, and levoposition of the aorta), with anatomical features to permit such repair. RESULTS: Twenty specimens collected between 1965 and 1993 ranged in age from 1 day to 23 years. Transposition of the great arteries or double outlet right ventricle was seen in 12 (60%). One had a single coronary artery that arose from the right anterior sinus and trifurcated. Another had the anterior descending artery arise from the right ventricular coronary artery while the circumflex arose alone, directly above the intercoronary commissure. Eccentric ostia were seen in another four, with one left ventricular coronary artery originating directly above the intercoronary commissure. Right ventricular aorta with pulmonary atresia was in eight of the total (40%). Three had eccentric ostia: one with both arteries from the left posterior sinus, and one with the left ventricular coronary over the intercoronary commissure. None of these specimens had additional coronary anomalies that would further complicate surgery. CONCLUSION: Nearly half of the specimens (45%) had coronary artery abnormalities that could have complicated, but not necessarily precluded, anatomic surgical repair.     

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.     

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)     

Congenitally corrected transposition of the great arteries treated by partial systemic ventriculectomy.

Congenitally corrected transposition of the great arteries (CCTGA) is a rare congenital heart disease characterized by atrioventricular (AV) and ventriculo-arterial discordance;(1) that is, the left ventricle supports the pulmonary circulation and the right ventricle supports the systemic circulation. The most common cardiac anomalies in CCTGA include ventricular septal defect, pulmonary outflow tract obstruction and abnormalities of the systemic AV valve. (1) The dysfunction of the systemic ventricle occurs with increasing frequency in older patients with CCTGA, independent of their commonly associated structural defects.(2-4) We report a patient with CCTGA undergone the partial ventriculectomy for his systemic ventricular dysfunction.     

Congenitally corrected transposition of the great arteries: Current treatment options

Opinion statement Congenitally corrected transposition of the great arteries is a relatively rare cardiac malformation characterized by atrioventricular and ventriculoarterial discordance. This double discordance results in a physiologically corrected circulation with the morphologic right ventricle (RV) serving as the systemic pump. Associated anomalies are present in approximately 98% of cases and include most commonly ventricular septal defect, pulmonary stenosis, and anomalies of the systemic atrioventricular valve (SAVV). Conduction abnormalities are common as well, predisposing these patients to the development of complete heart block. The management of these patients is primarily determined by the presence and severity of the associated anomalies. Conventional repair, which leaves the morphologic RV as the systemic ventricle, has resulted in high incidence of tricuspid regurgitation (TR) and progressive dysfunction of the RV. In the majority of patients, congestive heart failure secondary to RV dysfunction occurs by the fifth or sixth decade. The cause and effect relationship between TR and RV dysfunction remains to be determined. The advent of the Double Switch operation, which restores the morphologic left ventricle (LV) as the systemic ventricle, has yielded favorable outcomes at early follow-up. This procedure should be applied in young patients to achieve optimal results. Retraining the LV by pulmonary artery banding is associated with high morbidity and mortality when attempted after infancy or early childhood. Long-term follow-up is needed to evaluate the potential superiority of this procedure and the incidence of rhythm, baffle, and conduit complications for this management option.     

Right ventricular dysfunction in congenitally corrected transposition of the great arteries

Although right ventricular (RV) dysfunction is an important complication in subjects with congenitally corrected transposition of the great arteries, its pathogenesis is poorly understood. We assessed the role of RV myocardial perfusion and found perfusion defects at rest in all 20 patients, involving 4.6 +/- 2.3 of a total of 12 segments; the extent of the resting perfusion defects correlated inversely with the RV ejection fraction.