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Associated defects were found in 1\/3 of patients with normal great arteries, but only in 1\/10 of patients with transposition of great arteries (TGA). The extracardiac defects were especially rare in the groups \u201cTGA with intact ventricular septum\u201d and \u201cTGA with ventricular septal defect.\u201d Patients with multiple associated defects outnumbered patients with isolated associated defects in the ratio 2.5:1. The associated defects were heterogeneous: 46 patients had chromosome abnormalities, 16 had different Mendelian syndromes, and 36 had associations (DiGeorge sequence and VACTERL association were the most frequent). A new syndrome of multiple congenital abnormalities including tetralogy of Fallot, and rare cases of chromosomal and Mendelian syndromes (distal trisomy 1q, tetrasomy 8p, Holzgreve syndrome) are described briefly. Sufficient variability of a spectrum of conotruncal defects in the patients with the same chromosomal or Mendelian syndromes suggests that at least in some cases different conotruncal defects are stages of the same morphologic spectrum. The analysis of conotruncal defects in sibs of patients with Mendelian syndromes may provide new data about the links between different definitive forms of CVM. \u00a9 1995 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajmg.1320590116","type":"journal-article","created":{"date-parts":[[2005,6,11]],"date-time":"2005-06-11T18:09:09Z","timestamp":1118513349000},"page":"76-84","source":"Crossref","is-referenced-by-count":18,"title":["Non\u2010cardiac malformations in individuals with outflow tract defects of the heart: The Baltimore\u2010Washington infant study (1981\u20131989)"],"prefix":"10.1002","volume":"59","author":[{"given":"I. 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