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• W2124874175 abstract "I read with interest the recent case report by where cardiac catheterization is routinely performed, Kranidis et al. of Holt-Oram syndrome associated its incidence will be comparatively much higher. The with a persistent left superior vena cava [1]. The incidence of associated intracardiac lesion has been authors claimed that their patient was the only case noted to be relatively high, the most frequent being that has been reported so far where the only coexistatrial septal defect [3,4]. Since atrial septal defect is ing cardiovascular anomaly was a persistent left the commonest congenital heart defect in Holt-Oram superior vena cava. syndrome [2–4], the finding of a persistent left Holt-Oram syndrome is an autosomal dominant superior vena cava in the proband is understandable. hereditary disorder characterized by congenital carIn the patient with Holt-Oram syndrome reported diovascular malformations and skeletal anomalies of in 1987 by this author [3], the persistent left superior the upper limb [2]. The association of a persistent left vena cava was associated with an atrial septal defect. superior vena cava with the Holt-Oram syndrome was Persistent left superior vena cava as an isolated first recognized in a 15-year-old female patient by congenital cardiovascular anomaly in Holt-Oram this author in 1963. The case was later reported in syndrome was later reported in 1994 by Basson et al. 1987 [3] along with publication of an illustration in 1 of their 37 patients [5]. showing the typical X-ray appearance of the course of the cardiac catheter advanced up the inferior vena cava and into the persistent left superior vena cava References from the right atrium via the coronary sinus. Persistence of the left superior vena cava, which is due [1] Kranidis A, Filippatos G, Karvounis H. A case of Holt-Oram to failure of the left anterior cardinal vein to become syndrome (heart-hand syndrome). Int J Cardiol 2000;73:95–6. obliterated during fetal life, as an isolated anomaly is [2] Sun Q-B, Zhang K-Z, Cheng TO. Holt-Oram syndrome with high myopia. A hitherto unreported association in a Chinese patient. Am a benign condition and of no hemodynamic conseJ Med 1987;82:326–8. quence. The actual incidence is not known and may [3] Cheng TO. Holt-Oram syndrome in a Puerto Rican family – case vary considerably from one institution to another, reports. Angiology 1987;38:896–902. [4] Zhang K-Z, Sun Q-B, Cheng TO. Holt-Oram syndrome in China: A depending on the case material studied and the collective review of 18 cases. Am Heart J 1986;111:572–7. method of investigation. If based on necropsy find[5] Basson CT, Cowley GS, Solomon SD et al. The clinical and genetic ings in a general hospital, it will be understandably spectrum of the Holt-Oram syndrome (heart-hand syndrome). N low; if based on the findings in a cardiac center Engl J Med 1994;330:885–91." @default.
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• W2124874175 date "2000-10-01" @default.
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• W2124874175 title "Persistent left superior vena cava in Holt-Oram syndrome" @default.
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• W2124874175 doi "https://doi.org/10.1016/s0167-5273(00)00358-2" @default.
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