Abstract
Congenitally corrected transposition of the great arteries (CCTGA) is a rare and complex congenital anomaly characterized by atrial-ventricular (AV) discordance and ventricular-arterial discordance. Ventricular noncompaction (VNC) is a rare unclassified cardiomyopathy due to the arrest in intrauterine endomyocardial morphogenesis and it is characterized by numerous prominent trabeculations and intratrabecular recesses. We reported the case of a 47-year old female patient. When she was 35-year old an “isolated” CCTGA was diagnosed because of a heart murmur. Since then she attended periodically echocardiograms. She showed us 2 of them where right ventricle apical trabeculation was reported, without any others details. We performed a periodic evaluation in a patient still active, with a 6-month history of mild dyspnea occurring during exertion, no episodes of chest discomfort or palpitation. The ECG showed ectopic atrial rhythm, 83 bpm, normal QRS duration, QS complex in V1–V2 leads. The echocardiogram demonstrated: CCTGA, moderate enlargement and dysfunction of the right systemic ventricle, moderate to severe systemic AV valve regurgitation, severe thinning and dyskinesia of the basal segment of the septum, apical and mid-segments prominent and numerous trabeculations with deep intertrabecular recesses, better showed by Color Doppler, in continuity with the ventricular cavity. This case presents some distinctive features: (1) the association between two rare congenital anomalies; (2) Striking right VNC, involving the apex and mid-segments, rarely described in literature; right VNC has been proposed according to the presence of 3 over 4 criteria proposed by Jenni et al. (Heart 86:666–671, 2001); (3) Severe thinning and dyskinesia of the basal segment of the septum, probably related to coronary artery abnormalities frequently described in CCTGA patients.
References
Kawakubo M, Funabashi N, Uehara M, Ueda M, Hasegawa H, Sekine T, Ichimoto E, Yasukawa K, Takahashi O, Yamamoto Y, Komuro I (2007) Appearance of noncompaction-like remodeling of the anatomical right ventricle in a middle-aged subject with modified transposition of the great arteries who did not undergo surgery. Int J Cardiol 122(2):161–163. doi:10.1016/j.ijcard.2006.11.034
Ulusoy RE, Kucukarslan N, Kirilmaz A, Demiralp E (2006) Noncompaction of ventricular myocardium involving both ventricles. Eur J Echocardiogr 7(6):457–460. doi:10.1016/j.euje.2005.07.011
Alehan D, Dogan OF (2005) Right ventricular noncompaction in a neonate with complex congenital heart disease. Cardiol Young 15(4):434–436. doi:10.1017/S1047951105000910
Jenni R, Oechslin E, Schneider J et al (2001) Echocardiographic and pathoanatomical characteristics of isolated left ventricular noncompaction: a step towards classification as a distinct cardiomyopathy. Heart 86:666–671. doi:10.1136/heart.86.6.666
Hornung TS, Bernard EJ, Jaeggi ET, Howman-Giles RB, Celermajer DS, Hawker RE (1998) Myocardial perfusion defects and associated systemic ventricular dysfunction in congenitally corrected transposition of the great arteries. Heart 80:322–326
Ismat FA, Baldwin HS, Karl TR, Weinberg PM (2002) Coronary anatomy in congenitally corrected transposition of the great arteries. Int J Cardiol 86:207–216. doi:10.1016/S0167-5273(02)00325-X
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Patrignani, A., D’Aroma, A. & Cicogna, S. Unusual association between “congenitally corrected transposition of the great arteries” and “noncompaction” of the right systemic ventricle. Int J Cardiovasc Imaging 25, 551–553 (2009). https://doi.org/10.1007/s10554-009-9469-7
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DOI: https://doi.org/10.1007/s10554-009-9469-7