Mucolipidosis is an autosomal recessive lysosomal storage disorder that demonstrates a clinical resemblance to mucopolysaccharidosis. Accumulation of glycoproteins throughout the body causes dysfunction of several organs, in particular, valvular heart diseases are an important cause of mortality, however, there is no consensus guideline regarding the indications and optimal timing of the surgical repair because of the unclear and short natural history. Here we present 12- and 15-year-old siblings diagnosed with mucolipidosis who underwent aortic valve replacement. The senior sibling received redo-aortic valve replacement for prosthetic valve dysfunction 11 years after the initial surgery. A few surgical valve replacements in patients with mucopolysaccharidosis have been reported, however, there is no published case of aortic valve replacements in two siblings with mucolipidosis.

A 71-year-old man presented to our hospital with sudden-onset epigastric pain. He reported a history of undergoing the following operations : aortic valve replacement for aortic regurgitation 11 years earlier and graft replacement of the ascending aorta for acute type A aortic dissection, 1 year earlier. His systolic blood pressure was 70 mmHg, and computed tomography revealed a pseudoaneurysm of the distal anastomosis of the ascending aorta with a connection to the right pulmonary artery. Cardiopulmonary bypass was established with cannulation of the right axillary artery and the right femoral vein, and systemic cooling was initiated before sternotomy. We identified an area showing 3 cm dehiscence at the distal aortic anastomosis after hypothermic circulatory arrest and selective cerebral perfusion. The ascending aorta was replaced as hemiarch replacement, and the defect in the right pulmonary artery was closed with bovine pericardium. The patient's postoperative course was uneventful, and he was transferred to a rehabilitation hospital on the 22nd postoperative day.

In acute Stanford type A aortic dissection, except for some thrombosed false-lumen types, graft replacement is a standard treatment. On the other hand, thoracic endovascular aortic repair (TEVAR) might be considered for high-risk patients with retrograde type A aortic dissection when entry is in the descending aorta, although its efficacy in a case of an extensive thrombosed false lumen without obvious entry is unknown. We report a case of successful zone 3 TEVAR using RelayPro NBS for Stanford type A aortic dissection with a localized CT-enhanced false lumen in the proximal descending aorta. An 83-year-old woman was admitted for acute Stanford type A aortic dissection with a thrombosed false lumen of the ascending thoracic aorta. She was initially treated conservatively because of being a high-risk patient for open surgery. One week after hospitalization, the ascending aorta diameter increased and the false lumen in the proximal descending aorta grew sporadically in a CT image. We suspected that the ascending aorta was enlarged due to a partially patent false lumen of the descending thoracic aorta, and performed zone 3 TEVAR using RelayPro NBS to close a possible entry in the proximal descending aorta even though there was no obvious entry. The patient had a good postoperative course and was discharged 15 days after TEVAR. Shrinkage of the false lumen in the ascending aorta was observed in CT images two months after TEVAR.

We present a successful case of redo-tricuspid valve replacement for tricuspid prosthetic valve endocarditis. A 78-year-old man who underwent tricuspid bioprosthetic valve replacement for severe tricuspid regurgitation thirty-two years earlier was referred to our institution with persistent high fever and back pain. The blood culture was positive for Streptococcus oralis, and echocardiography revealed a mobile vegetation attached to the tricuspid prosthetic valve with moderate tricuspid regurgitation. A clinical diagnosis of prosthetic valve endocarditis was established. Redo-tricuspid bioprosthetic valve replacement was performed following antibiotics therapy. The patient was discharged on postoperative day 49 after 6 weeks of additional antibiotic treatment, and had no recurrence of infection for 6 months after redo-surgery.