Shprintzen-Goldberg syndrome (SGS) is a rare disorder with many characteristics of generalized connective tissue dysplasia. SGS is characterized by Marfanoid habitus with craniosynostosis and mental retardation. Patients with SGS have cardiovascular disorders similar to Marfan syndrome (MFS) and those disorders seem to play an important role in the prognosis of SGS. To our knowledge, only 19 patients with SGS have been reported, and 7 of them had cardiovascular disorders. The major cardiovascular disorders of SGS are aortic root dilatation and mitral valve prolapse. We reported the first case of SGS successfully treated surgically for cardiovascular disorders. Since then, we performed another operation in a patient with SGS. In this paper, we report our surgical results in patients with SGS.

The patient was a 74-year-old woman who had undergone mitral valve replacement with a mechanical valve for rheumatic mitral valve stenosis at age 60. She was scheduled for aortic valve replacement for severe aortic stenosis. However, she had significantly worsening anemia before the operation. Capsule endoscopy showed angiodysplasia with bleeding in her small intestine, which was considered the cause of the anemia. Because of progressive anemia, we tried embolization under angiography. However, there was no evidence of extravasation. Neither melena nor exacerbation of anemia was observed, and she underwent aortic valve replacement. She was discharged on postoperative day 22 without gastrointestinal bleeding. Heyde syndrome is aortic valve stenosis associated with gastrointestinal bleeding induced by von Willebrand disease and angiodysplasia in small intestine. Molecular multimeric analysis of von Willebrand factor and the existence of angiodysplasia with hemorrhage of the digestive tract are important for definitive diagnosis. Capsule endoscopy, which is a general examination, is more useful for diagnosis than molecular multimeric analysis of von Willebrand factor. Aortic valve replacement is the only therapeutic option for Heyde syndrome. It is important to decide the appropriate timing of AVR with cardiopulmonary bypass.

We report a case of type A acute aortic dissection in an elderly woman with immune thrombocytopenia (ITP) who underwent replacement of the ascending aorta and aortic arch and later required aortic root replacement for redissection of the aortic root one month after her initial surgery. She was an 86-year-old woman with severe mitral regurgitation, and surgery was contraindicated because of her age and ITP. In October 2014, the patient presented with back pain. Computed tomography confirmed the diagnosis of her condition as type A acute aortic dissection, and she was immediately transferred to our hospital. Because echocardiography showed severe aortic regurgitation, severe mitral regurgitation, and moderate tricuspid regurgitation, we performed replacement of the ascending aorta and aortic arch, mitral valve repair, and tricuspid annuloplasty. We used Bioglue to fuse the false lumen of the type A acute aortic dissection and used a Teflon felt sandwich for the proximal anastomosis technique. Respiratory support was discontinued 91 h after her first operation ; however, 30 days after surgery, she developed a to-and-fro murmur-a sign of the progression of heart failure. Echocardiography showed aggravation of aortic regurgitation, and computed tomography showed aortic root redissection ; therefore, 39 days after the initial surgery, we performed aortic root replacement. During the operation, we found the entry under the proximal anastomosis with an almost semicircle form at the right coronary cusp to the noncoronary cusp, and the dissection extended close to the right coronary artery ; thus, we performed bypass to the right coronary artery. Pathologic findings did not establish a causal association between the redissection and Bioglue, and we believed the fragility of the tissue and the selection of the surgical procedure to be the cause of redissection. The patient was transferred to another hospital when she was able to walk and eat, which was 121 days after her first operation. The patient required 50 units of platelet transfusion during her first and second operations, but her bleeding was easily controlled during surgery. She needed two procedures of pericardium drainage for pericardiac effusion and cardiac tamponade, which may relate to ITP. The diagnosis of redissection of the aortic root was made 30 days after the patient's first operation, on the basis of exacerbation of the to-and-fro murmur. Here, we emphasize the clinical importance of basic observations over time, such as auscultation, that are liable to be overlooked in the intensive care unit.