Corrected Transposition of the Great Arteries Involving Situs Inversus {I,D, D} and Mild Pulmonary Stenosis: Conduction System Identified During Preoperative Investigations for a Double-Switch Operation

The atrioventricular (AV) conduction system in corrected transposition of the great arteries (cTGA) involving situs inversus remains unclear. A case of cTGA involving situs inversus, mild pulmonary stenosis, and ventricular septal defect (VSD) is reported. An electrophysiologic study using the CARTO system and angiography was performed to identify the AV conduction system before a double-switch operation with VSD enlargement. The His bundle was located at the anteroseptum of the left-sided RA. It descended along the posteroinferior margin of the VSD with cTGA involving situs inversus and a normal-sized pulmonary trunk. No AV block occurred after surgery. The combination of the CARTO system and angiography could be useful for identifying the conduction system.     

Corrected Transposition of the Great Arteries with Isolated Aortic Coarctation: In Utero Echocardiographic Diagnosis

Physiologically corrected transposition of the great arteries (cTGA), defined by discordant atrioventricular and ventriculoarterial connections, is an uncommon congenital cardiac malformation. It rarely exists without associated cardiac anomalies, the most common of which are ventricular septal defect, pulmonary outflow obstruction, tricuspid valve (systemic) deformity, and rhythm disturbances. Conversely, hypoplasia of the systemic ventricle and systemic inflow or outflow obstructions have seldom been reported, although their recognition may significantly influence surgical repair and the patient's prognosis. We report a case of cTGA with complete heart block, moderate hypoplasia of the systemic ventricle, and severe aortic coarctation that was echocardiographically diagnosed in utero at 30 weeks' gestation because of fetal growth retardation and persistent fetal bradycardia. After delivery the patient underwent epimyocardial pacemaker implantation and aortic coarctation repair at 2 weeks of age. Unfortunately, the patient died on the seventh postoperative day because of systemic ventricular hypertrophy. Although it is well known that fetal echocardiography may reliably diagnose uncommon congenital cardiac malformations, to the best of our knowledge, this paper represents the first reported case of antenatal diagnosis of this complex anomaly.     

Reduction of Atrioventricular Valve Regurgitation by Atrioventricular Sequential Pacing for an Adult with Corrected Transposition of the Great Arteries

The case of a 64-year-old woman who underwent corrected transposition of the great arteries with significant systemic atrioventricular valve regurgitation and complete atrioventricular block is described. The patient underwent implantation of a DDD-type pacemaker and experienced relief of symptoms after discharge. Follow-up evaluation 30 months after the pacemaker implantation demonstrated a decrease in the cardiothoracic ratio on chest X-ray, the b-type natriuretic peptide level, and regurgitation at the systemic atrioventricular valve.     

Unusual Longevity Without Surgical Intervention in Complete Transposition of the Great Arteries

A case of unusual longevity to the age of 58 years is reported for a female patient with complete transposition of the great arteries. The association with a wide atrial septal defect with intact interventricular septum may have contributed to the long survival without surgery. Factors determining intercirculatory mixing and systemic oxygen saturation may be the high pulmonary flow, the location of the anatomic communication, sufficient hemoglobin concentration to allow an adequate level of systemic resistance and recirculated systemic flow, and the belated development of pulmonary vascular disease.     

Vascular Ring Due to Right Aortic Arch with Mirror-Image Branching and Left Ligamentum Arteriosus: Complete Preoperative Diagnosis by Magnetic Resonance Imaging

Magnetic resonance imaging (MRI) has been increasingly employed in the evaluation of aortic arch abnormalities. We present a rare form of vascular ring, that of a right aortic arch with mirror-image branching and left ligamentum arteriosus, which was clearly defined preoperatively by MRI. The patient subsequently underwent successful surgical division of the vascular ring followed by improvement in respiratory symptoms.     

Exercise Studies in Patients with Transposition of the Great Arteries After Atrial Repair Operations (Mustard/Senning): A Review

Exercise evaluation studies of patients after atrial repair surgery for transposition of the great arteries, as in tetralogy of Fallot, represent only a small fraction of the 3970 Medline references (1966 to mid-1997) concerning this congenital heart lesion. We have abstracted data from 27 studies from 20 institutions reporting on measurements during exercise on work capacity, heart rate response, respiratory gas exchange, or radionuclide/radiographic systemic ventricular ejection fraction measurements in addition to resting pulmonary function measurements. These studies provide almost uniform general conclusions that even after 20 or more years of follow-up (1) most patients ``report' that they are asymptomatic in performing usual levels of physical activities; (2) significant abnormalities are present, often in more than half of the patients studied, in one or more of the exercise measurements when compared to control subjects; and (3) the diminished exercise performance is related to a diminished cardiac output, results from diminished stroke volume but is also related to a blunted heart rate response.     

Transposition of the Great Arteries, Pulmonary Atresia, and Multiple Ventricular Septal Defects Associated with Multiple Cardiac Rhabdomyomas in a Case of Tuberous Sclerosis

Multiple cardiac rhabdomyomas are frequently associated with tuberous sclerosis. However, very few cardiac malformations have been described with tuberous sclerosis. We report an unusual case of tuberous sclerosis with cerebral and renal lesions associated with dextrotransposition of the great arteries, pulmonary atresia, patent ductus arteriosus, multiple ventricular septal defects, and parachute mitral valve deformity. After reviewing other cases of congenital heart diseases associated with tuberous sclerosis, emphasis is made on the potential influence of multiple rhabdomyomas developing very early during fetal life on final cardiac structures.     

Diagnostic and Interventional Trends in Tetralogy of Fallot and Transposition of the Great Arteries in a Population-Based Study

The goal of this study was to analyze diagnostic and interventional trends in tetralogy of Fallot and transposition of the great arteries in Malta for individuals born during 1920–1994 and calculate birth prevalences. The design was population based, in the setting of a regional hospital providing exclusive diagnostic and follow-up services for the entire population of Malta. Data collection and analysis was retrospective and included comparison with earlier epidemiological studies. Patients included were all Maltese live births diagnosed as having tetralogy of Fallot (TF) and transposition of the great arteries (TGA) born by the end of 1994. There were 109 cases of TF and 30 cases of TGA (n= 30). A significant decline in age at diagnosis and age at surgery was found for both lesions (p < 0.0001), which was associated with a significant decline in perioperative mortality (p= 0.005). Underascertainment was present prior to 1980, however, more cases were diagnosed due to improvement of postmortem services in the early 1980s followed by improvement of clinical services with eventually exclusive premortem diagnosis. The birth prevalence of TGA was 0.31/1000 live births, well within the range described in previous studies, unlike tetralogy of Fallot, which has been found in excess in this population in an earlier study. TF and TGA, the two lesions which comprise the majority of cyanotic congenital heart disease presenting in infancy, have been diagnosed and have undergone intervention at progressively earlier ages over the period under study. This decline was associated with a declining perioperative mortality.     

Premature ``Closing' of the Foramen Ovale in Transposition of the Great Arteries with Intact Ventricular Septum: Rare Cause of Sudden Neonatal Death

We report a male neonate who developed severe cyanosis and bradycardia at birth unresponsive to resuscitation. At autopsy he was found to have premature ``closure' of the foramen ovale together with transposition of the great arteries and an intact ventricular septum. Reviewing the literature, we found only one case report describing a similar neonate with this lethal combination of cardiac malformations.     

Atrial Natriuretic Peptide and Atrial Pressures in Newborns with Transposition of the Great Arteries

ABSTRACT. The relation of atrial pressure to atrial plasma concentration of atrial natriuretic peptide (ANP) was studied in four newborns undergoing catheterization because of transposition of the great arteries. In three patients, mean left atrial pressure clearly exceeded right atrial pressure (12±7 vs. 2±3 mmHg; mean ±SD). In one patient mean atrial pressures were identical (4 mmHg). In all patients the ANP concentration in the left atrium exceeded that of the right (833±464 vs. 415±366 pg/ml; mean ±SD). There was a significant ( r =0.97; p < 0.01) correlation between pressure and ANP concentration in the left atria. In the right atria, no linear correlation existed between pressure and ANP concentration. ANP concentration in the left atrium decreased after a lowering of the left atrial pressure by atrial septostomy. In these patients, the left atrium seems to be the main source of circulating ANP.     

Left-Side Pulmonary Vein Obstruction After Arterial Switch Operation in Infants with D-Transposition of the Great Arteries

We describe two cases of left-side pulmonary vein obstruction observed after the arterial switch operation (Jatene) for D-transposition of the great arteries. This appears to be related to left-sided pulmonary vein obstruction occurring coincidently with D-transposition of the great arteries, rather than a consequence of arterial switch operation.     

Pulmonary hypertension in patients with complete transposition of the great arteries: Midterm results after surgery

Postoperative results of surgical repair of complete transposition of the great arteries (TGA) with pulmonary hypertension (PH) in 19 patients in whom mean pulmonary arterial pressure was >50 mmHg or the pulmonary/systemic arterial pressure ratio was >0.8 were examined. TGA with intact ventricular septum was diagnosed in 10 patients and TGA with ventricular septal defect in 9. At the time of corrective surgery (arterial switch 10, atrial switch 9), patients ranged in age from 7 months to 14 years (mean 2.4 years). Seventeen patients (89%) survived with New York Heart Association functional class I. Seven patients in whom calculated pulmonary vascular resistance was 10–20 U · m² after surgery survived with subsequent regression of PH. Residual PH was diagnosed in 36% of survivors. Two patients in whom pulmonary vascular resistance was 26 and 36 U · m², respectively, died after surgery. In patients with TGA, severe PH was not necessarily fatal and was potentially reversible after successful surgery.     

Assessment of Progressive Changes in Exercise Performance in Patients with a Systemic Right Ventricle Following the Atrial Switch Repair

There is a high prevalence of right ventricular dysfunction and reduced exercise performance in survivors of atrial switch repair for transposition of the great arteries. However, it is not known whether the impairment in exercise performance is progressive. We performed paired comparison of exercise performance in 28 patients who underwent two serial incremental exercise tests at an interval of 5.0 ± 1.4 years between the two tests (age 11.5 ± 3.7 years at first test, 16.4 ± 3.6 years at second test). There was no change in the chronotropic response between the two tests. However, there was a reduction in both the peak VO₂ (32.5 ± 8.3 vs 29.6 ± 5.7 ml/kg/min, p= 0.05) and anerobic threshold (22.1 ± 5.1 vs 18.3 ± 4.2 ml/kg/min, p < 0.01) with time. Furthermore, there was a decline in the O₂ pulse (oxygen uptake/beat) at anaerobic threshold (% predicted value 95 ± 23% vs 82 ± 23%, p= .02), O₂ pulse at a heart rate of 140 (% predicted value 100 ± 30% vs 85 ± 19%, p= 0.02), and the maximum O₂ pulse (z value −0.27 ± 1.31 vs −1.27 ± 1.16, p < 0.01) when compared to growth-related normal values. We conclude that there is a progressive reduction in aerobic response to exercise in patients with a systemic right ventricle. The maintenance of chronotropic response suggests that the stroke volume response of the systemic right ventricle during exercise does not increase commensurate with somatic growth.