Beyond the OPDSDs, FLNA variants have also been shown to cause XCVD, PVNH1 and PVNH4, childhood interstitial lung disease (ChILD), structural cardiac and aortic anomalies, thoracic aortic aneurysms (TAA), chronic intestinal pseudo-obstruction (CIPO), and congenital short bowel syndrome (CSBS) (Fig. 5a) [57, 59–71]. The gene discussed is FLNA; the disease is congenital short bowel syndrome.