Vascular Ehlers-Danlos Syndrome (EDS) causes fragility of the blood vessels, skin, and hollow organs, and the most common clinical feature is vascular rupture. We report a surgical case of a spontaneous ruptured dissection of the left common iliac artery (CIA) in a patient with vascular EDS. A 27-year-old man with documented vascular EDS with a mutation of the collagen type III alpha-1 [COL3A1] gene, presented with acute left lower abdominal pain. The enhanced computed tomography (CT) scan revealed a ruptured dissection of the left CIA with extravasation, and left iliopsoas hematoma. The enhanced CT performed 1 month previously showed no arterial aneurysm. The left CIA was anastomosed end-to-end using a 7 mm heparin-bonded polytetrafluorethylene (PTFE) graft (Fusion, MAQUET Cardiovascular LLC, USA). A PTFE graft was anastmosed end-to-side to the left external iliac artery (EIA). The distal side of the CIA was closed. The postoperative enhanced CT showed aneurysmal formation in the right inferior gluteal artery. The follow-up CT showed enlargement of the left EIA (from 7.5 to 12 mm in a diameter), and aneurysm formation in the proper hepatic artery. Therefore, a close follow-up based on noninvasive imagings is mandatory to detect life-threatening vascular complecations in patients with vascular EDS.

We report a case of re-repair mitral valve replacement (MVR) for paravalvular leaks that were unsuitable for catheter treatment. Three years ago, a 67-year-old woman, who had undergone MVR for rheumatic mitral valve replacement at the age of 33 years and undergone re-MVR at the age of 47 years, was admitted with hemolytic anemia. We performed paravalvular leak (PVL) repair directly with 5-0 polypropylene sutures because of calcifications in the annulus. Three years after the operation, she presented with dyspnea on exertion, and transthoracic echocardiography revealed several paravalvular leaks. We consulted with cardiologists in our and other institutions, and these leaks were determined to be unsuitable for catheter treatment. We removed the artificial valve, and found the calcifications and residual cuffs from the first or second artificial valves. These residual cuffs were removed with Cusa® and Harmonic Synergy®. We performed re-repair MVR without reconstruction of the annulus. She was discharged on postoperative day 39 with no complications and did not experience any recurrence of PVL for 2 years. Residual cuffs from the artificial valve may cause PVLs, and Cusa® and Harmonic Synergy® are useful for removing residual cuffs and calcifications.

A 43-year-old pregnant woman was transferred to our hospital at 30 weeks' gestation for evaluation of chest and back pain. The patient was diagnosed with Stanford type A acute aortic dissection and had a family history of aortic dissection, with physical findings characteristic of hereditary connective tissue disease; however, she did not undergo comprehensive evaluation. The patient underwent cesarean delivery followed by total hysterectomy to prevent uncontrollable obstetric bleeding. She subsequently underwent total arch replacement and the Bentall procedure. The patient's postoperative course was unremarkable, and neither the mother nor the newborn showed any complications. Genetic testing revealed Loeys-dietz syndrome in the mother.