Congenitally corrected transposition with atrioventricular septal defect

We describe 4 cases of congenitally corrected transposition associated with atrioventricular septal defect, diagnosed by echocardiography and angiocardiography. Two had usual atrial arrangement and two had mirror imaged atrial arrangement . All cases were associated with subpulmonary valvar stenosis. All patients presented with cyanosis and were in sinus rhythm. Atrioventricular septal defect with common atrioventricular junction was easily diagnosed on the basis of a common atrioventricular valve permitting interatrial and interventricular communications. All patients had balanced right and left ventricles. The echocardiographic recognition of the ventricles was based on the presence of the moderator band within the morphologically right ventricle, the characteristics of the apical septal trabeculations, and the shape of the ventricles. Angiocardiographic recognition of the ventricles was achieved on the basis of right and left ventriculography. In one case with usual atrial arrangement, we recorded two His bundle potentials, one anteriorly and another posteriorly. Atrial stimulation revealed blocked atrioventricular conduction at the level of the posterior bundle, and normal atrioventricular conduction through the anterior bundle. In both cases with atrial mirror-imagery, only a posterior His bundle potential was found, with normal atrioventricular conduction revealed by atrial stimulation The clinical course with this combination depends on the other lesions present in addition to the common atrioventricular valve. Our electrophysiological studies show that the conduction system in presence of a common atrioventricular valve is as expected for congenitally corrected transposition with two atrioventricular valves.     

Three-dimensional reconstruction of abnormal ventriculoarterial relationship by electron beam CT

PURPOSE: Assessment of the relationships of the ventricles and great vessels is critical in evaluating cardiac anomalies before surgery. We investigated the usefulness of 3D CT in their demonstration. METHOD: From July 1995 through June 1996, 20 patients with abnormal ventriculoarterial relationships were analyzed by electron beam CT. A 3D gradient shading surface rendering was done on a workstation. We focused on the morphologies of the atria, ventricles, and great vessels and particularly their spatial relationships on the 3D images. RESULTS: There were two cases of isolated ventricular inversion, three of anatomically corrected malposition, seven of congenitally corrected transposition, and eight of complete transposition. Anatomic presentations of 3D surface images in all were superior to cineangiography and echocardiography presentations and were consistent with intraoperative findings. CONCLUSION: 3D CT images provide useful information that is not available by sectional or projective imaging. Independent segmental analysis of the anomalies by this method provides a complement for surgeons to understand the spatial relationships of the vessels and chambers of the heart, which may be of value in preoperative planning.     

Subpulmonary obstruction in congenitally corrected transposition of the great arteries due to ventricular membranous septal aneurysms

The clinical, hemodynamic, and angiographic observations, as well as the surgical approach used for repair in three patients with congenitally corrected transposition of the great arteries and ventricular membranous septal aneurysms, are presented. In two of the three patients the membranous septal aneurysm caused subpulmonary obstruction, with 94 and 125 mm Hg systolic gradients. In each patient the aneurysm was demonstrated by angiocardiography, which also showed differences in size and shape with cardiac systole and diastole. Review of the previously described reports indicates that patients with congenitally corrected transposition often display various forms of pulmonary outflow obstruction and when a ventricular membranous septal aneurysm exists, a significant subpulmonary obstruction is present in most patients. The unique anatomic relationship between the pulmonary artery and a ventricular membranous septal aneurysm in patients with transposition of the great arteries with and without atrioventricular discordance explains why subpulmonary obstruction sometimes develops.     

Ventricular tachycardia: a life-threatening arrhythmia in a patient with congenitally corrected transposition of the great arteries

Ventricular tachycardia (VT) is an uncommon finding in patients with congenitally corrected transposition of the great arteries (CCTGA). Cardiac death in patients with CCTGA has been attributed to complete heart block, systemic ventricular dysfunction, or severe AV valve regurgitation with heart failure. We describe the case of a patient who presented with palpitations and near-syncope that was associated with clinical episodes of VT. Programmed ventricular stimulation revealed easily inducible sustained VT that immediately degenerated to ventricular fibrillation and subsequently required therapy with an implantable cardioverter defibrillator.     

The double switch procedure for anatomical repair of congenitally corrected transposition of the great arteries in infants and children

AIMS: To assess outcomes of anatomical repair (double switch procedure) in infants and children with congenitally corrected transposition of the great arteries. METHODS AND RESULTS: Between September 1993 and August 1996, 17 patients with congenitally corrected transposition underwent surgery at UCSF. Anatomical repair was performed in 11 of these patients, at ages ranging from 4.8 months to 7.8 years (median 3.2 years). The remaining six patients did not undergo anatomical repair due to unfavourable anatomy (n = 2), prior conduit repair (n = 2), biventricular dysfunction (n = 1), and isolated complete atrioventricular block (n = 1). The 11 patients who underwent anatomical repair make up the study group for the present report. All 11 patients had a malalignment ventricular septal defect, while pulmonary outflow tract obstruction was present in nine patients and significant tricuspid valve pathology or dysfunction was present in five. Anatomical repair was achieved with a Senning (n = 7) or a Mustard (n = 4) procedure combined with an arterial switch operation plus ventricular septal defect closure (n = 4), or a Rastelli procedure with left ventricle to aortic baffle and right ventricle to pulmonary artery conduit (n = 7). There was one early death and no patients developed surgical complete atrioventricular block. At a median follow-up of 22 months, there were no late deaths. Two patients required a total of three late reoperations, and all patients were asymptomatic on no cardiac medication. Follow-up echocardiography revealed normal biventricular function in all patients. CONCLUSIONS: Anatomical repair of corrected transposition can be achieved with low rates of early mortality and surgical heart block, and favourable mid-term results. Long-term follow-up will be necessary to determine if the double switch approach improves the natural history of corrected transposition when compared to less aggressive surgical approaches that leave the right ventricle in the systemic circulation.     

Congenitally corrected transposition of the great arteries: morphologic study of 32 cases

The detailed anatomy of the heart is described in 32 autopsy cases of congenitally corrected transposition of the great arteries.Tis condition is defined as the combination of atrioventricular (A-V) discordance and transposition of the great arteries. Examples of primitive (single) ventricle with "inverted" (that is, left-sided in situs solitus) outlet chamber are excluded. Six hearts with A-V discordance and pulmonary atresia are described in an appendix. In 29 cases of corrected transposition the heart was in situs solitus; in 3 it was in situs inversus totalis. Only 5 of these 32 hearts had no potential for intracardiac shunting. Anomalies of the tricuspid valve (91 percent of cases), ventricular septal defect (78 percent) and pulmonary outflow tract obstruction (44 percent) occurred with sufficient frequency to be considered part of the basic malformation and are described in detail. The precise anatomy and disposition of the A-V valve tension apparatus, the coronary arteries and the conducting tissues are described with special reference to possible surgical approaches for repair of the anomalies. In two hearts with situs solitus the aortic valve was right-sided with respect to the pulmonary valve. This finding is important for both diagnosis and nomenclature.     

Senning plus arterial switch operation for discordant (congenitally corrected) transposition

BACKGROUND: Congenitally corrected transposition of the great arteries is a complex cardiac lesion, usually associated with ventricular septal defect, left ventricular outflow tract obstruction, and tricuspid valve abnormalities. A subset of patients without left ventricular outflow tract obstruction have undergone Senning plus arterial switch operation in an attempt to place the left ventricle in the systemic circuit and the right ventricle in the pulmonary circuit. METHODS: Fourteen patients have had the operation performed since July 1989. Age and weight medians were 12 months (range, 0.5 to 120 months) and 8.2 kg (range, 3.2 to 34 kg). All but 1 patient had a left ventricular to right ventricular pressure ratio greater than 0.7, due to a large ventricular septal defect (with or without a previous pulmonary artery band), severe congestive heart failure caused by right ventricular dysfunction and tricuspid insufficiency, or a pulmonary artery band for left ventricular retraining. At least 10 patients had strong contraindications to "classic" repair, including right ventricular hypoplasia (n = 2), moderate to severe right ventricular dysfunction (n = 5), or moderate to severe tricuspid insufficiency (n = 9). RESULTS: There was one hospital death, occurring in a neonate (7%; 95% confidence interval = 0% to 34%). Actuarial survival beyond 10 months is 81% (95% confidence interval = 42% to 95%), currently with 389 patient-months of total follow-up time. The median grade of tricuspid insufficiency fell from 3/4 preoperatively to 1/4 postoperatively (p = 0.003). Right ventricular function is normal in 11/12 current survivors, all but 1 of whom are in New York Heart Association class I or II. CONCLUSIONS: Senning plus arterial switch operation is a good option for selected patients with congenitally corrected transposition of the great arteries with a similar or lower early risk (as compared with classic repairs). Some of the long-term problems associated with congenitally corrected transposition of the great arteries may be avoided with this strategy.     

Cardiac transplantation in New Zealand: eight years experience

Between December 1987 and December 1995, 62 patients underwent orthotopic cardiac transplantation at Green Lane Hospital. Their cardiac dysfunction resulted from dilated cardiomyopathy (32), coronary artery disease (21), rheumatic heart disease (7), congenitally corrected transposition of the great arteries (1) and hypertrophic cardiomyopathy (1). Before transplantation all patients were in New York Heart Association (NYHA) functional class III or IV. Eight patients (13%) died in the perioperative period and a further seven (11%) died 1 to 4 years after transplantation. Actuarial 1 and 3 year survival was 87% and 80% respectively. Forty-five of forty-seven surviving patients were in NYHA functional class I at the time of analysis. The results confirm that cardiac transplantation has a limited but valuable role in the treatment of end stage myocardial dysfunction.     

Complete transposition of great vessels: clinical aspects, diagnosis, echocardiography

The paper reports the study of 12 patients admitted to hospital between January 1992 and October 1994. The patients were almost all neonates. Clinical, electrocardiographic and radiological data led to the suspected diagnosis of complete transposition of the great vessels. 2-D echocardiography consistently showed the parallel disposition of great vessels, the anterior position of the aorta compared to the pulmonary artery, the origin of the pulmonary artery from the left ventricle and the aorta from the right ventricle, all of which are the anatomopathological characteristics of this congenital cardiopathy, together with associated malformations, thus enabling a certain diagnosis to be made. The authors underline the value of echocardiography in the diagnosis of complete transposition of the great vessels.     

Therapeutic assessment of adult patients with isolated corrected transposition of the great arteries

Corrected transposition of the great arteries is a rare congenital heart disease, affecting 1% of children with cardiac malformation. Patients with transposition of the great arteries and without associated cardiovascular anomalies are very infrequent and may remain undiagnosed until adult life, because they usually are asymptomatic until the fourth or fifth decades. At this time, most symptoms occur in close connection with deterioration in systemic (right) ventricle performance and with an increase in left atrial pressure. In this report, we describe two new adult cases of isolated, corrected transposition of the great arteries, offering several considerations on their clinical profile and therapeutic assessment.     

Co-twin study of the effect of environment and dietary elements on acquisition of Helicobacter pylori infection

We report a rare case of corrected transposition of great arteries and mitral atresia associated with double aoric arch. The patient showed polypnea and cyanosis soon after birth. She underwent balloon atrioseptostomy at the age of 18 days and pulmonary artery banding (PAB) at 24 days. But she showed severe persistent stridor after PAB. Esophagography and left counter-current aortography revealed double aortic arch. At the age of 49 days, division of the left lesser arch and ligation of patent ductus arteriosus were performed through left lateral thoracotomy. Stridor disappeared completely following operation. She underwent total cavopulmonary connection using a 18 mm Gore-Tex graft as intraatrial conduit concomitant with pacemaker implantation at the age of 8 years. Postoperative course was uneventful. To our knowledge, this is the first successful case report of surgical correction of corrected transposition of great arteries and mitral atresia associated with double aoric arch.     

An elderly case of mixed levocardia with situs inversus including inverted atria and complete transposition of the great vessels--an autopsied case

We reported on the autopsy of a 35-year-old man who had mixed levocardia with situs inversus including atrial inversion, transposition of the great vessels, and other complex cardiac malformations. Levocardia was discussed.     

A study of methods for surgical interruption of the His bundle

The relationship of the atrioventricular (AV) node and His bundle to surrounding structures is described from the viewpoint of the surgeon. A series of experiments were carried out for interruption of atrial to ventricular conduction that included electrocauterization of the His bundle, incision just above the tricuspid annulus to attempt to divide the His bundle, and finally excision of the right atrial wall and the insertion of the atrial septum into the right fibrous trigone to remove all connections from the AV node and atrium to the His bundle. The last procedure was the most satisfactory method, as judged by the certainty of accomplishing both His bundle interruption and interruption at its highest possible point.     

Surgery for congenital heart disease in the adult

The present experiments were conducted on isolated dog hearts to demonstrate that conduction disturbances can be induced in the bundle branches by transection of about 50 per cent of the cross-sectional area of the His bundle on the right or left side. The His bundle, the posterior and anterior divisions of left bundle, and the right bundle were exposed by careful dissection, and microelectrode techniques were used to record action potentials from the three bundle branches. Pacing stimuli were applied to the nonbranching portion of His bundle proximal and then distal to the site of transection to study the effect of such lesions on impulse conduction to the bundle branches. It was demonstrated that conduction to the bundle branches was not affected by such lesions in the His bundle at pacing rates slower than 100 per minute; however, conduction disturbances were rate-dependent and manifested at faster pacing rates. In nine out of all 16 experiments, partial or complete block occurred in all three bundle branches regardless of the side of the lesion. In the remaining seven experiments, they were observed in the bundle branch on the same side as the lesion. It was assumed that conduction disturbances of the bilateral bundle branches resulted from decremental conduction in the uncut portion of His at the level of lesion, and those of the ipsilateral branch from the functional failure of transverse crossover connections between the longitudinal His bundle fibers. The results indicate that localized lesions in the nonbranching portion of His bundle can indeed produce the pattern of bundle branch block under certain conditions.     

Chiropractic manipulation

A unique case of aortic atresia and tricuspid atresia associated with transposition of the great vessels is described in a newborn infant. The clinical, echocardiographic, and heart catheterization data are presented. The embryological and surgical implications are discussed.     

Truncus arteriosus communis. Unusual case associated with transposition

A child with truncus arteriosus communis, characterized by the posterior origin of an individualized pulmonary trunk is presented. This relationship between the great arteries is unusual in truncus arteriosus communis and the spatial orientation resembles that seen in transposition of the great vessels. A brief discussion is proposed about a proper terminology in this type of complex anomaly.     

Extragenital donovanosis of the foot

Kartagener's syndrome (KS) usually includes mirror-image dextrocardia. The incidence of congenital heart disease in KS is comparable with that in the general population. This paper reports on a case of Kartagener's syndrome associated with dextrocardia, corrected transposition of the great arteries (I,D,D), ventricular septal defect, and valvar pulmonary stenosis in an 8-year-old girl.     

Clinical diagnosis of enamel defects: pitfalls and practical guidelines

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.     

Expression of collagens and decorin during aortic arch artery development: implications for matrix pattern formation

The elastic matrix of the large arteries shows a high level of spatial order. However, the mechanisms by which such order is established and maintained are largely unknown. The embryonic development of the avian heart and great vessels provides an appropriate model to investigate these mechanisms. In control embryos, an elastic matrix with a high level of spatial order develops in the nascent great vessels. But after the normal vascular smooth muscle (VSM) progenitor cells in the great vessels are experimentally replaced by other VSM progenitor cells, the elastic extracellular matrix is congenitally disordered. The present study used this model to test the hypothesis that the proteoglycan decorin was involved in the establishment and maintenance of the normal three-dimensional spatial order of the vascular elastic matrix. The temporospatial expression of decorin was analysed during development of normal vessels and in experimental vessels with surrogate VSM. The results showed the following: (1) the expression of decorin was related in time and space to the establishment of large helical collagen type III fibers that are characteristic of the normal elastic extracellular matrix; (2) in the experimental extracellular matrix there were few helical fibers of collagen type III, but those that were present remained positive for decorin; and (3) in both control and experimental vessels, decorin associated with neither fibers of collagen type I nor fibers of collagen type III in any conformation other than the large helical fibers. These data indicate a previously unrecognized relationship between decorin and the spatial order of the physiologically significant helical fibers of collagen type III.     

Bilateral total hip arthroplasty comparing hydroxyapatite coating to porous-coated fixation

Intraventricular together with atrial-axis and nodoventricular discontinuity, in which various parts of the conduction system are replaced by fibrous or fatty tissue, constitute the three major pathological categories of isolated congenitally complete heart block. Intraventricular discontinuity is distinctly rare, with only two previous cases reported in the literature, one of which was associated with a familial history of heart block. The cardiac conduction systems of two cases of isolated congenitally complete heart block were serially sectioned and analyzed histopathologically. The findings were correlated with the clinical features, in particular, the family histories and maternal serum anti-Ro antibodies. Both cases, a 9-day-old neonate and an 8-year-old schoolgirl, showed a combination of nodoventricular and intraventricular discontinuity, with absence of the atrioventricular penetrating bundle, the entire right, and the proximal portion of the left bundle branch. The branching bundle was absent in the first case and replaced by fatty tissue in the second. In contrast to the commoner atrial-axis discontinuity in which the atrioventricular node itself is usually replaced by fibrous or fatty tissue with variable involvement distally, the sinus node, and in particular, the atrioventricular node were normal in both of our cases. There was no family history in either case, whereas tests for the maternal serum anti-Ro antibody were positive in the first but negative in the second case. Intraventricular discontinuity as a cause of isolated congenitally complete heart block is very rare. In our cases, it co-existed with nodoventricular discontinuity. It can be sporadic, familial, or associated with positive maternal serum anti-Ro antibodies.