In this study, we report the phenotype and genotype of fourteen unrelated individuals with heterozygous LoF and/or de novo heterozygous variants in EIF3B, as well as four unrelated individuals with de novo heterozygous LoF variants in EIF3A. These individuals present with a variable phenotype that includes CHD, craniofacial dysmorphisms, and mild neurodevelopmental abnormalities. Here, EIF3A is linked to coronary artery disorder.