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A 50-year-old man with decompensated aortic stenosis displayed significantly reduced ejection fraction, an ascending aortic aneurysm (55 mm in diameter), and bilateral giant bullae, and was evaluated as having extremely high surgical risk. Therefore, as a bridge to definitive treatment, he simultaneously underwent transcatheter aortic valve replacement (TAVR) and upper left lung lobectomy. His heart function recovered 6 months later and he underwent surgical aortic valve replacement (SAVR) and graft replacement of the ascending aorta. TAVR may serve as a bridge procedure before SAVR for aortic stenosis in younger patients with high surgical risk.

The authors describe the case of a 51-year-old woman with an osteonecrosis of her right femoral head after treatment of an atypical subtrochanteric fracture caused by pycnodysostosis. She had this fracture after a low-trauma fall. She was of short stature with typical facial features, short stubby hands, and radiological features including open cranial sutures, obtuse mandible, and generalized skeletal sclerosis. The majority of cases of atypical subtrochanteric fractures are associated with long-term use of bisphosphonates; some occur in bisphosphonate-free patients. We report a rare case of total hip arthroplasty (THA) in a patient with pycnodysostosis who developed an osteonecrosis of the femoral head after treatment of an atypical subtrochanteric femoral fracture. We performed cementless THA in combination with a plate and cables. Cementless THA is a potential intervention in a patient with pycnodysostosis; although the bone quality may have been sclerotic, healing is not a problem in this condition.

We report a 76-year-old female with Takayasu arteritis who was found to have multiple coronary arteriovenous fistulas (CAVFs), a pulmonary-to-systemic ratio of 2:1, and aortic valve insufficiency. Aortic valve replacement and ligation of multiple CAVFs with cardiopulmonary bypass were performed under cardioplegic arrest, thus minimizing coronary steal. doi: 10.1111/jocs.12102 (J Card Surg 2013;28:380–382)

Transcatheter aortic valve replacement (TAVR) has recently been shown to be feasible in patients with severe aortic stenosis who are considered inoperable. We perform TAVR with cardiopulmonary support (CPS) for patients with low left ventricular (LV) function. We report two successful cases of TAVR on CPS in patients with low LV function and describe this technique. (J Card Surg 2012;27:686-688)

We report a successful transcatheter aortic valve replacement (TAVR) using syngo DynaCT (Siemens AG, Forchheim, Germany) in an 86-year-old man who had severe aortic valve stenosis. Syngo DynaCT is a cross-sectional radiological imaging system that facilitates intraoperative imaging via interventional angiographic systems; this navigation system is useful during TAVR, especially in cases of poor calcification at the annulus. (J Card Surg 2012;27:551-553)

Multiple epiphyseal dysplasia (MED) is a common skeletal dysplasia characterized by joint pain and stiffness, delayed and irregular ossification of epiphyses, and early-onset osteoarthritis. Six genes responsible for MED have been identified, including COMP, COL9A1, COL9A2, COL9A3, DSTDT and MATN3.MATN3 encodes matrilin-3, a cartilage-specific extracellular matrix protein. To date, seven different MATN3 mutations have been identified; all are located within the β-sheet regions of the von Willebrand factor type A (vWFA) domain, which is encoded by exon 2. We examined MATN3 mutations in27 Japanese MED patients who were possibly autosomal dominant inheritance and had been excluded for COMP mutations. Ten of them had a positive family history. We examined all eight exons of MATN3 by PCR and direct sequencing from genomic DNA. We have identified four missense mutations in eight unrelated families; two are novel, and two have been characterized previously. Like previously characterized MATN3 mutations, those identified in this study are clustered within exon 2, specifically in and around the 2nd β-sheet region of the vWFA domain (aa. 120-127). Contrary to the previous assumption that the MATN3 mutation in MED is confined to the β-sheet regions, one novel mutation (p.F105S) is located outside the β-sheet region, within an α-helix region. © 2004 Wiley-Liss, Inc.