Corrected Transposition of the Great Vessels in a 55-Year-Old Woman; Diagnosis by Coronary Angiography
1974; Elsevier BV; Volume: 66; Issue: 2 Linguagem: Inglês
10.1378/chest.66.2.190
ISSN1931-3543
AutoresHoward A. Schwartz, Philip I. Wagner,
Tópico(s)Coronary Artery Anomalies
ResumoThe case of an adult with congenitally corrected transposition of the great vessels without significant intracardiac defects is presented. This case attests to the proposition that the right ventricle can adjust to systemic demands and that patients with his anomaly can reach adulthood. In addition, this entity may be more common than presently assumed, and awareness should lead to an increased frequency of diagnosis. The case of an adult with congenitally corrected transposition of the great vessels without significant intracardiac defects is presented. This case attests to the proposition that the right ventricle can adjust to systemic demands and that patients with his anomaly can reach adulthood. In addition, this entity may be more common than presently assumed, and awareness should lead to an increased frequency of diagnosis. Congenitally corrected transposition of the great vessels (CTGV) implies a heart possessing an abnormal relationship of chambers and vessels, but still maintaining the systemic and pulmonary circuits. It is almost always accompanied by intracardiac defects such as VSD and AV valve insufficiency.1Berry WB Roberts WC Morrow AG et al.Corrected transposition of the aorta and pulmonary trunk.Clinical, hemodynamic and pathologic findings. Am J Med. 1964; 36: 35-53Scopus (24) Google Scholar-6Shem-Tov A Deutsch V Yabini J et al.Corrected transposition of the great arteries: A modified approach to the clinical diagnosis in 30 cases.Am J Cardiol. 1971; 27: 99-113Abstract Full Text PDF PubMed Scopus (21) Google Scholar Assuming the morphologic right ventricle were capable of adapting to systemic demands, cases without other defects could remain asymptomatic throughout life; however, there have been few reports to substantiate this.7Cumming GR Congenital corrected transposition of the great vessels without associated intracardiac anomalies.A clinical, hemodynamic and angiographic study. Am J Cardiol. 1962; 10: 605-614Abstract Full Text PDF PubMed Scopus (18) Google Scholar,8Rotem CE Hultgren HN Corrected transposition of the great vessels without associated defects.Am Heart J. 1965; 70: 305-318Abstract Full Text PDF PubMed Scopus (19) Google Scholar In the English literature only three patients9Benchimol A Tio S Sundararajan V Congenital corrected transposition of the great vessels in a 58-year-old man.Chest. 1971; 59: 634-638Abstract Full Text Full Text PDF PubMed Scopus (10) Google Scholar, 10Lieberson AD Schumacher RR Childress RH et al.Corrected transposition of the great vessels in a 73-year-old man.Circulation. 1969; 39: 96-100Crossref PubMed Scopus (36) Google Scholar, 11Moss AJ Hutler AM Lipchik EO et al.Congenital corrected transposition of the great vessels without cardiac anomalies.Am J Med. 1969; 47: 986-994Abstract Full Text PDF PubMed Scopus (8) Google Scholar with CTGV who have reached adulthood with no cardiac disability have previously been reported. The scarcity of reports of older patients with uncomplicated CTGV may be caused by failure of recognition at routine autopsy or to clinical oversight of the asymptomatic patient.3Gasul BM Arcilla RA Lev M Heart Disease in Children.Diagnosis and Treatment. JB Lippincott Co., Philadelphia1966Google Scholar The following is a report of an adult with CTGV without significant intracardiac anomalies and having no signs of congestive heart failure. A 55-year-old white woman was admitted, who complained of severe precordial chest pain and shortness of breath. She had a previous history of chest pain and had numerous admissions for hypoglycemic attacks of unknown etiology. Her ECGs during these admissions were abnormal showing first degree AV block and QS pattern in the right precordial leads consistent with an old anteroseptal infarct (Fig 1).Physical examination revealed a single second sound at the base and a grade 2/6 pansystolic murmur at the apex. Chest x-ray picture revealed a straightened left heart border (Fig 2). There was no change in the ECG in comparison with previous tracings. In view of the history of chest pain and the abnormal ECG, coronary angiography was performed.Selective cineangiograms revealed a right-sided artery which immediately divided into two branches. One branch gave off the SA nodal artery and supplied the posterior aspect of the heart. The other branch supplied the anterior surface of the heart and gave rise to right angle vessels resembling septal perforators, but not supplying the septum (Fig 3a). The left-sided coronary artery divided into numerous vessels all supplying the posterior aspect of the heart. No AV nodal artery could be identified. There was no evidence of coronary artery disease.The selective injection of contrast material into the left-sided chamber below the aorta revealed a heavily trabeculated ventricle (Fig 3b). Mild left AV valvular regurgitation could be detected. The relationship of the AV and the aortic valves were abnormal and they were separated by a crista supraventricularis. An outflow tract led to the anteriorly placed aorta. These findings were consistent with a morphologic right ventricle and corrected transposition of the great vessels.FIGURE 2Chest x-ray film from case 1. Note straightened left heart border (arrow).View Large Image Figure ViewerDownload (PPT)FIGURE 3Cineangiograms from case 1. Upper: Right-sided coronary artery injection in the right anterior oblique position. Note right angle vessels (arrow) arising from unusual branch of right-sided coronary. Lower: Systemic ventricular injection in right anterior oblique position. Note the heavily trabeculated ventricle (arrow).View Large Image Figure ViewerDownload (PPT) Various anomalous vessel and chamber relationships have been described which result in CTGV; however, the most common form has situs solitus atria, ventricular inversion (1-bulboventricular loop) and inverted transposition of the great vessels.3Gasul BM Arcilla RA Lev M Heart Disease in Children.Diagnosis and Treatment. JB Lippincott Co., Philadelphia1966Google Scholar,4Lev M Rowlatt VF The pathological anatomy of mixed levocardia: A review of 13 cases of atrial or ventricular inversion with or without corrected transposition.Am J Cardiol. 1961; 8: 216-263Abstract Full Text PDF PubMed Scopus (79) Google Scholar The pathologic anatomy3Gasul BM Arcilla RA Lev M Heart Disease in Children.Diagnosis and Treatment. JB Lippincott Co., Philadelphia1966Google Scholar,4Lev M Rowlatt VF The pathological anatomy of mixed levocardia: A review of 13 cases of atrial or ventricular inversion with or without corrected transposition.Am J Cardiol. 1961; 8: 216-263Abstract Full Text PDF PubMed Scopus (79) Google Scholar of this entity has been well described and will only be summarized. Oxygenated blood from the lungs returns to the normally located left atrium and passes through the left-sided “tricuspid” valve into the left-sided morphologic right ventricle then through an outflow tract and into the aorta which is located anteriorly and to the left of the pulmonary artery. Venous blood returns to the normally located right atrium and then through the rightsided “mitral” valve into the right-sided morphologic left ventricle and into the pulmonary artery. The coronary supply is inverted;4Lev M Rowlatt VF The pathological anatomy of mixed levocardia: A review of 13 cases of atrial or ventricular inversion with or without corrected transposition.Am J Cardiol. 1961; 8: 216-263Abstract Full Text PDF PubMed Scopus (79) Google Scholar,5Schiebler GL Edwards JE Burchell HB et al.Congenital corrected transposition of the great vessels: a study of 33 cases.Pediatrics. 1961; 27: 851-888Google Scholar,12Elliott LP Amplatz K Edwards JE Coronary arterial patterns in transposition complexes: anatomic and angiographic studies.Am J Cardiol. 1966; 17: 362-378Abstract Full Text PDF PubMed Scopus (51) Google Scholar thus, the right-sided coronary artery emerges from a right-sided sinus of Valsalva (which is in reality the embryologic left sinus) and divides into the anterior descending and a circumflex branch. The left-sided coronary artery becomes the posterior descending artery. The noncoronary cusp is anterior (see Fig 4). Classically, auscultation reveals a loud single second sound and flow murmurs.1Berry WB Roberts WC Morrow AG et al.Corrected transposition of the aorta and pulmonary trunk.Clinical, hemodynamic and pathologic findings. Am J Med. 1964; 36: 35-53Scopus (24) Google Scholar, 2Friedberg DZ Nadas AS Clinical profile of patients with congenital corrected transposition of the great arteries: a study of 60 cases.N Engl J Med. 1970; 282: 1053-1059Crossref PubMed Scopus (108) Google Scholar, 3Gasul BM Arcilla RA Lev M Heart Disease in Children.Diagnosis and Treatment. JB Lippincott Co., Philadelphia1966Google Scholar,5Schiebler GL Edwards JE Burchell HB et al.Congenital corrected transposition of the great vessels: a study of 33 cases.Pediatrics. 1961; 27: 851-888Google Scholar,6Shem-Tov A Deutsch V Yabini J et al.Corrected transposition of the great arteries: A modified approach to the clinical diagnosis in 30 cases.Am J Cardiol. 1971; 27: 99-113Abstract Full Text PDF PubMed Scopus (21) Google Scholar The chest x-ray film often shows straightening of the left heart border.1Berry WB Roberts WC Morrow AG et al.Corrected transposition of the aorta and pulmonary trunk.Clinical, hemodynamic and pathologic findings. Am J Med. 1964; 36: 35-53Scopus (24) Google Scholar, 2Friedberg DZ Nadas AS Clinical profile of patients with congenital corrected transposition of the great arteries: a study of 60 cases.N Engl J Med. 1970; 282: 1053-1059Crossref PubMed Scopus (108) Google Scholar, 3Gasul BM Arcilla RA Lev M Heart Disease in Children.Diagnosis and Treatment. JB Lippincott Co., Philadelphia1966Google Scholar, 4Lev M Rowlatt VF The pathological anatomy of mixed levocardia: A review of 13 cases of atrial or ventricular inversion with or without corrected transposition.Am J Cardiol. 1961; 8: 216-263Abstract Full Text PDF PubMed Scopus (79) Google Scholar, 5Schiebler GL Edwards JE Burchell HB et al.Congenital corrected transposition of the great vessels: a study of 33 cases.Pediatrics. 1961; 27: 851-888Google Scholar, 6Shem-Tov A Deutsch V Yabini J et al.Corrected transposition of the great arteries: A modified approach to the clinical diagnosis in 30 cases.Am J Cardiol. 1971; 27: 99-113Abstract Full Text PDF PubMed Scopus (21) Google Scholar, 7Cumming GR Congenital corrected transposition of the great vessels without associated intracardiac anomalies.A clinical, hemodynamic and angiographic study. Am J Cardiol. 1962; 10: 605-614Abstract Full Text PDF PubMed Scopus (18) Google Scholar, 8Rotem CE Hultgren HN Corrected transposition of the great vessels without associated defects.Am Heart J. 1965; 70: 305-318Abstract Full Text PDF PubMed Scopus (19) Google Scholar,5Schiebler GL Edwards JE Burchell HB et al.Congenital corrected transposition of the great vessels: a study of 33 cases.Pediatrics. 1961; 27: 851-888Google Scholar,6Shem-Tov A Deutsch V Yabini J et al.Corrected transposition of the great arteries: A modified approach to the clinical diagnosis in 30 cases.Am J Cardiol. 1971; 27: 99-113Abstract Full Text PDF PubMed Scopus (21) Google Scholar Studies at autopsy4Lev M Rowlatt VF The pathological anatomy of mixed levocardia: A review of 13 cases of atrial or ventricular inversion with or without corrected transposition.Am J Cardiol. 1961; 8: 216-263Abstract Full Text PDF PubMed Scopus (79) Google Scholar,5Schiebler GL Edwards JE Burchell HB et al.Congenital corrected transposition of the great vessels: a study of 33 cases.Pediatrics. 1961; 27: 851-888Google Scholar,12Elliott LP Amplatz K Edwards JE Coronary arterial patterns in transposition complexes: anatomic and angiographic studies.Am J Cardiol. 1966; 17: 362-378Abstract Full Text PDF PubMed Scopus (51) Google Scholar have shown the coronary arteries to be inverted; however, reports of coronary angiograms in patients with CTGV are regrettably few.9Benchimol A Tio S Sundararajan V Congenital corrected transposition of the great vessels in a 58-year-old man.Chest. 1971; 59: 634-638Abstract Full Text Full Text PDF PubMed Scopus (10) Google Scholar, 10Lieberson AD Schumacher RR Childress RH et al.Corrected transposition of the great vessels in a 73-year-old man.Circulation. 1969; 39: 96-100Crossref PubMed Scopus (36) Google Scholar, 11Moss AJ Hutler AM Lipchik EO et al.Congenital corrected transposition of the great vessels without cardiac anomalies.Am J Med. 1969; 47: 986-994Abstract Full Text PDF PubMed Scopus (8) Google Scholar, 12Elliott LP Amplatz K Edwards JE Coronary arterial patterns in transposition complexes: anatomic and angiographic studies.Am J Cardiol. 1966; 17: 362-378Abstract Full Text PDF PubMed Scopus (51) Google Scholar In the case of a 73-year-old man, Lieberson and Associates10Lieberson AD Schumacher RR Childress RH et al.Corrected transposition of the great vessels in a 73-year-old man.Circulation. 1969; 39: 96-100Crossref PubMed Scopus (36) Google Scholar reported mirror image coronary arteries at angiography; however, selective angiograms were apparently not performed. At autopsy, the arteries were in mirror image with the noncoronary cusp being anterior. Moss and co-workers,11Moss AJ Hutler AM Lipchik EO et al.Congenital corrected transposition of the great vessels without cardiac anomalies.Am J Med. 1969; 47: 986-994Abstract Full Text PDF PubMed Scopus (8) Google Scholar using selective cineangiograms, described a large left-sided artery resembling the morphologic right coronary. The right-sided artery was described as resembling the morphologic left, ending as the circumflex and anterior descending, although the authors could not identify the latter with certainty. Neither the SA nor the AV nodal artery could be identified. Alternatively, the angiograms in the case described by Benchimol and colleagues,9Benchimol A Tio S Sundararajan V Congenital corrected transposition of the great vessels in a 58-year-old man.Chest. 1971; 59: 634-638Abstract Full Text Full Text PDF PubMed Scopus (10) Google Scholar were interpreted as showing a normal pattern. The angiographic coronary artery pattern from the present case is compatible with inverted coronary arteries. The explanation of the discrepancies between the angiographic and pathologic findings are unclear, but it is apparently possible to find a variable angiographic representation in these patients even though they embryologically have inverted coronarles. Knowledge of the origin of the AV nodal artery and the septal supply in CTGV would be helpful in the angiographic identification of the embryologic right and left coronary arteries; however, it is not presently available. If the abnormal (posterolateral) coronary cusp positions could be determined it would be helpful, but this is difficult to detect with certainty. AV block was present in this patient and is often found in CTGV, although the pathogenesis is not clear.1Berry WB Roberts WC Morrow AG et al.Corrected transposition of the aorta and pulmonary trunk.Clinical, hemodynamic and pathologic findings. Am J Med. 1964; 36: 35-53Scopus (24) Google Scholar, 2Friedberg DZ Nadas AS Clinical profile of patients with congenital corrected transposition of the great arteries: a study of 60 cases.N Engl J Med. 1970; 282: 1053-1059Crossref PubMed Scopus (108) Google Scholar, 3Gasul BM Arcilla RA Lev M Heart Disease in Children.Diagnosis and Treatment. JB Lippincott Co., Philadelphia1966Google Scholar,5Schiebler GL Edwards JE Burchell HB et al.Congenital corrected transposition of the great vessels: a study of 33 cases.Pediatrics. 1961; 27: 851-888Google Scholar,6Shem-Tov A Deutsch V Yabini J et al.Corrected transposition of the great arteries: A modified approach to the clinical diagnosis in 30 cases.Am J Cardiol. 1971; 27: 99-113Abstract Full Text PDF PubMed Scopus (21) Google Scholar,8Rotem CE Hultgren HN Corrected transposition of the great vessels without associated defects.Am Heart J. 1965; 70: 305-318Abstract Full Text PDF PubMed Scopus (19) Google Scholar,13Fernandez F Laurichesse S Scebat L et al.Electrocardiogram in corrected transposition of the great vessels of the bulbo-ventricular inversion type.Am Heart J. 1970; 32: 165-171Crossref Scopus (5) Google Scholar,14Ruttenberg HD Elliott LP Anderson RC et al.Congenital corrected transposition of the great vessels: Correlation of electrocardiograms and vectorcardiograms with associated cardiac malformations and hemodynamic states.Am J Cardiol. 1966; 17: 339-354Abstract Full Text PDF PubMed Scopus (18) Google Scholar Progression into complete heart block may add to the morbidity and mortality of this lesion.3Gasul BM Arcilla RA Lev M Heart Disease in Children.Diagnosis and Treatment. JB Lippincott Co., Philadelphia1966Google Scholar The histopathology of one case of CTGV with AV block revealed normal AV node; however, it was not connected to the AV bundle.15Lev M Fielding RT Zaeske D Mixed levocardia with ventricular inversion (corrected transposition) with complete atrioventricular block: A histopathologic study of the conduction system.Am J Cardiol. 1963; 12: 875-883Abstract Full Text PDF PubMed Scopus (24) Google Scholar The QRS morphology is related to the inverted conduction system16Lev M Licata RH May RC Conduction system in mixed levocardia with ventricular inversion (corrected transposition).Circulation. 1963; 28: 232-237Crossref PubMed Scopus (45) Google Scholar and the associated defects.6Shem-Tov A Deutsch V Yabini J et al.Corrected transposition of the great arteries: A modified approach to the clinical diagnosis in 30 cases.Am J Cardiol. 1971; 27: 99-113Abstract Full Text PDF PubMed Scopus (21) Google Scholar,13Fernandez F Laurichesse S Scebat L et al.Electrocardiogram in corrected transposition of the great vessels of the bulbo-ventricular inversion type.Am Heart J. 1970; 32: 165-171Crossref Scopus (5) Google Scholar,14Ruttenberg HD Elliott LP Anderson RC et al.Congenital corrected transposition of the great vessels: Correlation of electrocardiograms and vectorcardiograms with associated cardiac malformations and hemodynamic states.Am J Cardiol. 1966; 17: 339-354Abstract Full Text PDF PubMed Scopus (18) Google Scholar The ECG pattern in CTGV without additional defects has not been consistent, although reversal of septal Q-waves is often described.6Shem-Tov A Deutsch V Yabini J et al.Corrected transposition of the great arteries: A modified approach to the clinical diagnosis in 30 cases.Am J Cardiol. 1971; 27: 99-113Abstract Full Text PDF PubMed Scopus (21) Google Scholar,8Rotem CE Hultgren HN Corrected transposition of the great vessels without associated defects.Am Heart J. 1965; 70: 305-318Abstract Full Text PDF PubMed Scopus (19) Google Scholar,14Ruttenberg HD Elliott LP Anderson RC et al.Congenital corrected transposition of the great vessels: Correlation of electrocardiograms and vectorcardiograms with associated cardiac malformations and hemodynamic states.Am J Cardiol. 1966; 17: 339-354Abstract Full Text PDF PubMed Scopus (18) Google Scholar The ECG from this patient revealed a QS pattern in the anterior precordial leads, whereas septal Q waves were present in V4-V6. Electrocardiographic data from the other reported adult cases are presented in Table 1.Table 1Reported Cases of Adults with Congenitally Corrected Transposition of Great Vessels (Arranged According to Age at Diagnosis)AuthorAgeReason for Cardiac Work-upPresenting SymptomsChest X-ray FilmECGMoss et al11Moss AJ Hutler AM Lipchik EO et al.Congenital corrected transposition of the great vessels without cardiac anomalies.Am J Med. 1969; 47: 986-994Abstract Full Text PDF PubMed Scopus (8) Google Scholar 196954—Palpitations and syncopeHeart rotated to right1) PE=0.262) Accentuated R waves in right precordial leads3) Lack of septal Q waves in 1, AVL, V5-V6Present case55Rule out coronary artery diseaseShortness of breath and chest painStraightened left heart border1) PR =0.242) QS pattern in V1-V33) Septal Q waves V4-V6Benchimol et al9Benchimol A Tio S Sundararajan V Congenital corrected transposition of the great vessels in a 58-year-old man.Chest. 1971; 59: 634-638Abstract Full Text Full Text PDF PubMed Scopus (10) Google Scholar 197058—Dizziness on exertion—1) PR = 0.22) QS in V13) Large R in V24) No Q waves in V2-V6Lieberson et al10Lieberson AD Schumacher RR Childress RH et al.Corrected transposition of the great vessels in a 73-year-old man.Circulation. 1969; 39: 96-100Crossref PubMed Scopus (36) Google Scholar 196973—Shortness of breath due to chronic obstructive lung diseaseAbscence of pulmonary segment of cardiac silhouette1)No AV block2) QS in V13) Normal R wave progression in V2-V6 with no Q wavesDiagnosis was established in all cases by angiography, and confirmed by autopsy in Lieberson's case. These cases, with the exception of the patient presented by Moss, had angiographic evidence of mild tricuspid insufficiency. Open table in a new tab Diagnosis was established in all cases by angiography, and confirmed by autopsy in Lieberson's case. These cases, with the exception of the patient presented by Moss, had angiographic evidence of mild tricuspid insufficiency. In conclusion, this case is further evidence of the fact that patients with CTGV may reach adulthood. The presence of this anomaly is probably more common than is currently thought, but awareness of the possibility of CTGV without associated defects in asymptomatic patients with abnormal ECGs (especially anterior infarct pattern and AV block) and typical physical signs should lead to an increased frequency of diagnosis.
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