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Epstein-Barr virus (EBV) immortalized lymphoblastoid cell lines (LCLs) are widely used for banking. This bioresource could be leveraged for creating human iPSC lines to model diseases including CHD. We generated an LCL-derived iPSC line (NCHi001-A) from a patient with congenital aortic valve stenosis. NCHi001-A was EBV and transgenes free, exhibited stem cell-like morphology, expressed pluripotency markers, has a normal karyotype, and could be differentiated into cells of three germ layers in vitro. Relationship inference via a microarray-based analysis showed NCHi001-A is identical to the parental cell line. NCHi001-A can be used for disease modeling, drug discovery, and cell therapy development.

Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect characterized by underdeveloped structures on the left side of the heart, including hypoplasia of the left ventricle and stenosis or atresia of the aortic and mitral valves. Here, we generated an iPSC line from the peripheral blood mononuclear cells of a male patient with HLHS through Sendai virus-mediated transfection of 4 Yamanaka factors. This iPSC line exhibited normal morphology, expressed pluripotency markers, had a normal karyotype, and could differentiate into cells of three germ layers. This iPSC line can be used for studying cellular and developmental etiologies of HLHS.

Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare congenital heart defect defined by membranous or muscular atresia of the right ventricular outflow tract where patients display varying degrees of hypoplasia of the right ventricle. This condition results in cyanosis due to an inability of blood to flow from the right ventricle to the pulmonary arteries, thus requiring immediate surgical intervention after birth. An iPSC line was generated from peripheral blood mononuclear cells of a 11-year-old male patient diagnosed with PA-IVS through Sendai virus-mediated reprogramming. This disease-specific iPSC line was characterized by immunocytochemistry, STR analysis, karyotype analysis, and mycoplasma testing.