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The prenatal diagnosis of, and short-term outcome for, patients with congenitally corrected transposition

Published online by Cambridge University Press:  21 January 2005

Enrico Chiappa ,
Angelo Micheletti ,
Andrea Sciarrone ,
Gianni Botta  and
Piero Abbruzzese
Enrico Chiappa
Affiliation:
Division of Pediatric Cardiology, Azienda Ospedaliera Materno-Infantile O.I.R.M.-S. Anna, Turin, Italy
Angelo Micheletti
Affiliation:
Division of Pediatric Cardiology, Azienda Ospedaliera Materno-Infantile O.I.R.M.-S. Anna, Turin, Italy
Andrea Sciarrone
Affiliation:
Obstetric Ultrasound and Prenatal Diagnosis Center, Azienda Ospedaliera Materno-Infantile O.I.R.M.-S. Anna, Turin, Italy
Gianni Botta
Affiliation:
Department of Pathology, Azienda Ospedaliera Materno-Infantile O.I.R.M.-S. Anna, Turin, Italy
Piero Abbruzzese
Affiliation:
Division of Pediatric Cardiac Surgery, Azienda Ospedaliera Materno-Infantile O.I.R.M.-S. Anna, Turin, Italy
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Abstract

Congenitally corrected transposition is a rare congenital anomaly, with only a few cases diagnosed and reported prenatally even in the largest fetal series. To determine the morphologic features and outcome for the lesion as recognized during fetal life, we reviewed the fetal and postnatal echocardiograms and medical records of 11 consecutive cases of congenitally corrected transposition. These were identified among 230 (4.7%) consecutive cases of structural cardiac disease referred to our fetal cardiology unit over a period of 4 years. The mean gestational age at diagnosis was 24.7 weeks. Reasons for referral were suspected complete transposition, abnormal position of the heart, and bradyarrhythmias. Associated cardiac lesions included an abnormal cardiac position in 6 cases, ventricular septal defect in 8, obstruction of the subpulmonary outflow tract in 6, tricuspid valvar displacement in 5, and complete atrioventricular block in 2. Only 3 of the cases had mild tricuspid regurgitation prior to birth. Termination was chosen in 4 cases with severe obstruction to pulmonary flow. Of the remaining cases, 2 patients died at 3 and 12 months after birth, respectively. Both developed significant tricuspid regurgitation associated with unexpected major arrhythmias. The remaining 5 patients are alive and relatively well at a mean follow-up of 25.4 months. An epicardial pacemaker was inserted in 1 because of complete atrioventricular block. We conclude that prenatal counseling must be guarded following the diagnosis of congenitally corrected transposition, even in fetuses with an apparently favorable state at initial examination. Some of these cases may undergo major and unexpected changes, particularly with regard to cardiac rhythm and tricuspid valvar function, with concomitant significant changes in prognosis.

Keywords

Discordant atrioventricular connectionsdouble discordancefetal echocardiography
Type
Original Article
Information
Cardiology in the Young , Volume 14 , Issue 3 , June 2004 , pp. 265 - 276
Copyright
© 2004 Cambridge University Press

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References

Fontana RS, Edwards JE. Frequency of occurrence of various anatomic types of malformation among patients with congenital cardiac disease. In: Congenital Cardiac Disease: A Review of 357 Cases Studied Pathologically. WB Saunders, Philadelphia, 1962, pp 28–65.
Losekoot TG, Anderson RH, Becker AE, Danielson GK, Soto B. Congenitally Corrected Transposition. Churchill Livingstone, New York, 1983, pp 15–16.
Allwork SP, Bentall HH, Becker AE, et al. Congenitally corrected transposition of the great arteries. Morphologic study of 32 cases. Am J Cardiol 1976; 38: 910–923.Google Scholar
Freedom RM, Dyck JD. Congenitally corrected transposition of the great arteries. In: Moss AJ, Adam FJ (eds). Heart Disease in Infants, Children, Adolescents. Lippincott Williams & Wilkins, Philadelphia, 2001, pp 1085–1101.
Zahn EM, Smallhorn JF, Freedom RM. Congenitally corrected transposition of the great arteries with hypoplasia of the morphologically left ventricle in the setting of situs inversus. Int J Cardiol 1992; 36: 9–12.Google Scholar
Celermajer DS, Cullen S, Deanfield JE, Sullivan ID. Congenitally corrected transposition and Ebstein’s anomaly of the systemic atrioventricular valve: association with aortic arch obstruction. J Am Coll Cardiol 1991; 18: 1056–1058.Google Scholar
Presbitero P, Somerville J, Rabaioli F, Stone S, Conte MR. Corrected transposition of the great arteries without associated defects in adult patients: clinical profile and follow up. Br Heart J 1995; 74: 57–59.Google Scholar
Hutha JC, Danielson GK, Ritter DG, Ilstrup DM. Survival in atrioventricular discordance. Pediatr Cardiol 1985; 6: 57.Google Scholar
Lundstrom U, Bull C, Wise RKH, Somerville J. The natural and “unnatural” history of congenitally corrected transposition. Am J Cardiol 1990; 65: 1222–1229.Google Scholar
Connelly MS, Liu PP, Williams WG, Webb GD, Robertson P, McLaughlin PR. Congenitally corrected transposition of the great arteries in the adult: functional status and complication. J Am Coll Cardiol 1996; 27: 1238–1243.Google Scholar
Prieto LR, Allan JH, Secic M, Rosenbaun MS, Gersony WM. Progressive tricuspid valve disease in patients with congenitally corrected transposition of the great arteries. Circulation 1998; 98: 997–1005.Google Scholar
Voskuil M, Hazekamp MG, Kroft LJM, et al. Postsurgical course of patients with congenitally corrected transposition of the great arteries. Am J Cardiol 1999; 83: 558–562.Google Scholar
Graham TP Jr, Bernard YD, Mellen BG, et al. Long term outcome in congenitally corrected transposition of the great arteries. A multi-institutional study. J Am Coll Cardiol 2000; 36: 255–261.Google Scholar
Rutledge JM, Nihill MR, Fraser CD, O’Brian Smith E, McMahon CJ, Bezold LI. Outcome of 121 patients with congenitally corrected transposition of the great arteries. Pediatr Cardiol 2002; 23: 137–145.Google Scholar
Beauchesne LM, Warnes CA, Connoly HM, Ammash NM, Tajik AJ, Danielson GK. Outcome of the unoperated adult who presents with congenitally corrected transposition of the great arteries. J Am Coll Cardiol 2002; 40: 285–290.Google Scholar
Allan LD, Sharland GK, Milburn A, et al. Prospective diagnosis of 1006 consecutive cases of congenital heart disease in the fetus. J Am Coll Cardiol 1994; 23: 1452–1458.Google Scholar
Allan LD. Atrioventricular discordance. In: Allan LD, Hornberger LK, Sharland GK (eds). Textbook of Fetal Cardiology. Greenwich Medical Media, London, 2000, pp 183–189.
Fesslová V, Nava S, Villa L, and The Fetal Cardiology Study Group of the Italian Society of Pediatric Cardiology. Evolution and long term outcome in cases with fetal diagnosis of congenital heart disease: Italian Multicenter Study. Heart 1999; 82: 594–599.Google Scholar
Paladini D, Rustico M, Todros T, et al. Conotruncal anomalies in prenatal life. Ultrasound Obstet Gynecol 1996; 8: 241–246.Google Scholar
Cordes TM, O’Leary PW, Seward JB, Hagler DJ. Distinguishing right from left: a standardized technique for fetal echocardiography. J Am Soc Echo 1994; 7: 47.Google Scholar
Sharland GK, Allan LD. Normal fetal cardiac measurements derived by cross-sectional echocardiography. Ultrasound Obstet Gynecol 1992; 2: 175–181.Google Scholar
Schmidt KG, Ulmer HE, Silverman NH, Kleinman CS, Copel JA. Perinatal outcome of fetal complete atrioventricular block: a multicenter experience. J Am Coll Cardiol 1991; 17: 1360–1366.Google Scholar
Honey M. The diagnosis of corrected transposition of the great vessels. Br Heart J 1963; 25: 313.Google Scholar
Friedberg DZ, Nadas AS. Clinical profile of patients with congenital corrected transposition of the great arteries. New Engl J Med 1970; 282: 1053.Google Scholar
Daliento L, Corrado D, Buja G, John N, Nava A, Thiene G. Rhythm and conduction disturbances in isolated, congenitally corrected transposition of the great arteries. Am J Cardiol 1986; 58: 314–318.Google Scholar
Bahrati S, Rosen K, Steinfeld L, Miller RA, Lev M. The anatomic substrate for preexcitation in corrected transposition. Circulation 1980; 62: 831–842.Google Scholar
Acar P, Sidi D, Bonnet D, Aggoun Y, Bonhoffer P, Kachaner J. Maintaining tricuspid valve competence in double discordance: a challenge for the paediatric cardiologist. Heart 1998; 80: 479–483.Google Scholar