case_images: PMC10720308_01
case_images: A-C: Pulmonary artery CTA reveals the absence of the left lung structure, with no display of the left pulmonary artery and its branches. The right lung structure and pulmonary artery show no filling defect, and the branches of each lobe segment artery are well displayed with compensatory dilatation. D-F: Aortic CTA shows localized narrowing at the beginning of the descending aorta and branches are formed at the distal ends of the subclavian artery, intercostal arteries, intrathoracic arteries, and both iliac arteries. G-I: Aortic angiography assesses the degree of narrowing, locates the position of the balloon-expandable covered stent, and evaluates the post-release effects.
case_images: fcvm-10-1239624-g001.jpg
case_images: PMC10720308_01_fcvm-10-1239624-g001.jpg
case_images: F1
case_images: Computed tomographic thoracic imaging was performed and revealed left pulmonary agenesis (Figures 1A-C) along with a severe isthmic coarctation of the aorta just distal to the dilated left subclavian artery. There was abundant collateral circulation bypassing the coarctation in the suprascapular, internal thoracic, and superficial abdominal arteries (Figures 1D-F). 24 h ambulatory blood pressure monitoring showed non-dipper hypertension. Subsequently, a 45 mm covered 8-Zig CP-stent (NuMED, USA) was mounted on a 13 x 40 mm balloon and implanted in the initial segment of the descending aorta (Figures 1G-I).
case_images: Full exon gene sequencing first generation sequencing family verification chart verification site: FBN2, c.6583C > T, chromosome position: chr5:127624873. (A) Proband: heterozygous mutation at chr5:127624873; (B) Mother: wild-type at chr5:127624873; (C) Son: heterozygous mutation at chr5:127624873.
case_images: fcvm-10-1239624-g002.jpg
case_images: PMC10720308_01_fcvm-10-1239624-g002.jpg
case_images: F2