Congenital quadricuspid aortic valve is a very rare malformation. Most cases have been discovered as an incidental finding at aortic valve surgery or at autopsy. It frequently evolves to aortic regurgitation, which can manifest in adulthood and may require surgical treatment. A 66-year-old man was admitted because of dysprea. Echocardiogram revealed aortic regurgitation, mitral regurgitation, and tricuspid regurgitation. We performed aortic valve replacement, mitral annuloplasty, and tricuspid annuloplasty successfully. The aortic valve showed one large, two intermediate and one smaller cusp, which were classified as typed by Hurwitz's classification. An accessory cusp was situated between the right and left coronary cusps. No coronary abnormality was involved. The postoperative course was uneventful and he is doing well 6 months after operation.

In the presence of Leriche syndrome, the lower extremities are perfused by collateral flow from internal mammary arteries. If an internal mammary artery graft is used in coronary artery surgery, an acute ischemic limb will develop postoperatively. A 52-year-old man was admitted to our department with bilateral claudication. Multidetector row computed tomography with contrast showed total occlusion of the infrarenal abdominal aorta and rich collateral flow to the lower extremities from internal mammary arteries. Cardiac angiography revealed three-vessel disease. Simultaneous coronary artery bypass grafting and an ascending aorto-bifemoral bypass were performed without cardiopulmonary bypass. Postoperative computed tomography angiography showed that grafts to the coronary and bifemoral arteries were patent. This combined procedure is useful for patients with coronary artery disease and aortoiliac occlusive disease. This procedure without cardiopulmonary bypass has not previously been reported.

Some 50% of patients who undergo cardiac surgery will have heparin-induced thrombocytopenia (HIT) antibodies, only 1% will develop typical clinical HIT. Especially delayed-onset HIT is not well-known and extremely rare. A 83-year-old man underwent aortic valve replacement (AVR) with a bioprosthetic valve (Mitroflow 21 mm) and pulmonary vein isolation (PVI). Intravenous unfractionated heparin (8,000 I.U./day) was administered for 5 days after surgery. He had a good recovery and was discharged to home with a platelet count of 100,600/μl on POD 15 on warfarin. On POD 18, he was readmitted to our hospital due to cerebral infarction with hemiparesis. As MRI revealed a multiple left hemispheric infarction, the presence of cardiogenic cerebral infarction was suspected. Continuous intravenous administration of unfractionated heparin (15,000 I.U./day) was started. On POD 24, the platelet count fell to 27,000/μl, and a contrast CT scan revealed a giant thrombus in the aortic arch. He was found to have HIT antibodies, supporting a diagnosis of HIT. After cessation of heparin therapy and administration of vitamin K antagonist, argatroban was administered. He recovered neurologically and was discharged on POD 58. He remains well at 3 years follow up. If a patient has an unexplained and severe thrombocytopenia after cardiovascular surgery, delayed-onset HIT should be taken into consideration.

STUDY DESIGN: Cross sectional study. PURPOSE: The study aimed to analyze mechanisms underlying chronic low back pain (CLBP) using magnetic resonance imaging (MRI) T2 mapping of the intervertebral disc (IVD). OVERVIEW OF LITERATURE: MRI T2 mapping utilizes the T2 values for quantifying moisture content and collagen sequence breakdown. We previously used MRI T2 mapping for quantifying the extent of IVD degeneration (IVDD) and showed a correlation between the degeneration of the posterior annulus fibrosus (AF) and CLBP. METHODS: We enrolled 40 patients with CLBP (17 males, 23 females; mean age, 50.8±1.6 years; range, 22–60 years). IVDs were categorized as the anterior AF, nucleus pulposus (NP), and posterior AF, and T2 value for each disc was measured. T2 values, assessed using the Japanese neuropathic pain (NeP) screening questionnaire, of the NeP and nociceptive pain (NocP) groups were compared. RESULTS: T2 values of the NocP and NeP groups were 64.7±5.6 ms and 58.1±2.3 ms for the anterior AF; 67.0±4.6 ms and 59.6±2.1 ms for NP; and 70.7±4.6 ms and 51.0±1.2 ms for the posterior AF, respectively. T2 values for IVDD were significantly lower in the NeP group than those in the NocP group (p<0.01). CONCLUSIONS: The results indicate a correlation between the degeneration of posterior AF and NeP. MRI T2 mapping may be beneficial for detecting NeP caused by IVDD and can help formulate targeted analgesic therapies.
Asian Continental Ancestry Group ; Collagen ; Female ; Humans ; Intervertebral Disc ; Low Back Pain ; Magnetic Resonance Imaging ; Male ; Mass Screening ; Neuralgia ; Nociceptive Pain

A 72-year-old female received surgical aortic valve replacement for severe aortic stenosis in our hospital. During surgery, black pigmentation was observed in the aortic valve, aorta intima and mitral valve anterior leaflet collocated with calcification. We suspected Alkaptonuria (AKU) as a possible diagnosis for those surgical findings, past medical history and physical findings. A urine test for organic acids showed homogentisic, confirming the diagnosis of AKU. AKU is very rare genetic metabolic abnormality that occurs in about 1 in 25,000 to 100,000 people. AKU involves deficiency in the gene coding for HGA-1,2-dioxygenase, which metabolizes homogentisic acid to maleylacetoacetic acid in the tyrosine metabolic pathway. HGA accumulates in the body, causing black pigmentation in places including the aorta intima and mitral valve.

We report a case of surgery for an infectious left subclavian artery aneurysm in a patient with metal allergy. The patient was a 41-year-old man allergic to iron, silver, manganese, and chromium. He had received a Nitinol stent in the left subclavian artery at a previous hospital. One stent had fallen out during implantation, and was put away in the terminal aorta. Ten days after the left subclavian implantation, the patient developed left shoulder pain and fever, which continued for 2 weeks. Contrastenhanced CT scan revealed a pseudoaneurysm of the left subclavian artery and abdominal aortitis. The patient underwent left subclavian artery aneurysmectomy, aorto-left subclavian artery bypass using the great saphenous vein, and removal of the stents in the left subclavian artery and abdominal aorta. The surgery was performed through a median sternotomy with cardiopulmonary support. A contrast-enhanced CT scan taken on the 12th postoperative day revealed a pseudoaneurysm of the abdominal aorta, and the patient underwent abdominal aortic artery replacement surgery on the 14th postoperative day. The patient was discharged from the hospital on the 27th day after the first surgery. The treatment of an aneurysm should be selected according to the patient’s background as well as anatomical factors.

Objective: DNA methyltransferase 1 (DNMT1) is crucial to maintaining methylation during DNA replication and DNA repair. DNMT1 mutations have been identified in two neurological syndromes, including hereditary sensory and autonomic neuropathy type IE (HSAN IE) with dementia and hearing loss and autosomal dominant cerebellar ataxia, deafness and narcolepsy. It is likely that DNMT1 mutations lead to various symptoms of the central and peripheral nervous system. The aim of this study was to examine the clinical characteristics, especially the initial symptoms, in the cases of DNMT1 mutations. Methods: We investigated the clinical manifestation and examination findings of four cases of HSAN IE from one family with the DNMT1 mutation c.1531Y>C (p.Try511His). Results: All four cases exhibited sensory neuropathy, cerebellar ataxia, and hearing loss, all of which were demonstrated by the audiograms. The initial symptoms of the four cases included hearing loss (n=1), gait disturbance (n=1), and depressive mood (n=2). Depressive symptoms are reported in some cases with HSAN IE, however, there are currently no published reports that describe them as primary symptoms. The CSF orexin level was measured in three cases, revealing normal values in two cases and intermediate values in one case, in which the patient exhibited rapid eye movement (REM) sleep behavior disorder. Conclusion: Our findings suggest that in cases with HSAN IE or the DNMT1 mutation, psychiatric symptoms should be taken into account as one of the initial manifestations of the disease.