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We describe a patient with typical manifestations of 9p monosomy syndrome, including trigonocephaly and sex reversal. Array comparative genomic hybridization (CGH) revealed a 9p terminal deletion of approximately 9 Mb with the breakpoint at 9p23. We compared the deleted segments of 9p associated with reported cases of 9p monosomy syndrome with trigonocephaly. We did not identify a region that was shared by all patients; however, when only pure terminal or interstitial deletions that did not involve material from any other chromosome were compared, we identified a segment from D9S912 to RP11-439I6 of approximately 1 Mb that was deleted in every patient. We propose that this 1-Mb segment might be the critical region for 9p monosomy syndrome with trigonocephaly.

A 68-year-old woman exhibited an increasingly protruding mass on the left heart border on chest X-ray. Transthoracic echocardiography revealed an echo-free mass in the anterior pericardial space. Transesophageal echocardiography revealed blood flow from the proximal left anterior descending coronary into a large coronary artery aneurysm measuring 61 mm × 51 mm in diameter and a quadricuspid aortic valve with a small cusp between the left and right coronary cusps. Coronary angiography demonstrated the presence of a coronary aneurysm connected to the proximal left coronary anterior descending artery. A giant coronary artery aneurysm and pulmonary artery fistulas extending from the left and right coronary arteries were confirmed by surgeons and successfully treated with surgery.

Objective We previously reported the noninferiority of paravertebral block (PVB) to epidural block. In this study, we assessed whether PVB via an intrathoracic approach was also safe for the patients ineligible for epidural block because of, for example, anticoagulation or antiplatelet therapy.

43歳，女性．健診で縦隔腫瘍を疑われ当院受診，胸部CT，血液生化学的諸検査などが施行された．CA19-9の高値（108U/ml），前縦隔に約3×4cmの隔壁を有する嚢胞性病変が認められ，手術が施行された．腫瘍は左前縦隔に存在し，胸腺左葉と連続しており，左上葉，左縦隔胸膜とも強固に癒着していた．左上葉の部分切除と癒着した縦隔胸膜とを一塊にして胸腺摘出術を施行した．病理診断は膵組織を伴う成熟型奇形腫であった．術前上昇していたCA19-9は術後正常化し，腫瘍内産生が示唆された．成熟型奇形腫は診断時に無症状であっても周囲組織と強固な炎症性癒着を起こし，合併切除が必要になることがあり，注意を要すると考えられた．

We report on a boy who had multiple synostosis syndrome 1, an autosomal dominant disorder characterized by progressive symphalangism, multiple joint fusions, conductive deafness, and mild facial dysmorphism. In addition the boy developed delay of puberty, bone age, and closure of the epiphyseal lines of long bones with tall stature. These findings and decreased plasma LH and FSH levels at age 19 years were compatible with hypogonadotropic hypogonadism. G-banded chromosomes showed a balanced translocation t(10;17)(p15.3;q22). Chromosomal FISH analysis, using a series of BAC clones surrounding the translocation breakpoints, detected a 2.2-3.9 Mb deletion at 17q22. The deletion encompassed NOG, a gene responsible for multiple synostosis syndrome 1. It was assumed that a gene for pituitary secretion of gonoadotropic hormones was deleted at the 17q22 segment.

症例は54歳女性．2005年6月会社の健診にて胸部異常陰影を指摘され当院呼吸器内科に紹介となった．胸部CT検査で右中葉胸膜直下に辺縁不整な10mm大の結節影を認められたがPET検査は陰性であったため経過観察されていた．しかし2006年1月のCT検査で肺癌の可能性も否定できず確定診断，治療目的で当科に紹介となり胸腔鏡下手術を施行した．迅速病理検査で血管腫と診断され，病理組織学的検査で毛細血管腫（capillary hemangioma）と最終診断された．術後経過良好で合併症もなく軽快退院となった．肺の孤立性の毛細血管腫についての報告は極めて稀であり文献的考察を加えて報告する．

症例は55歳，女性．意識消失発作にて，精査が施行された．頭蓋内病変は認められず，24時間ホルター心電図にて2:1高度房室ブロックが認められた．そのため，意識消失はAdams-Stokes発作と診断され，ペースメーカー（Pacemaker:PM）植込み術の予定となった．しかし，金属アレルギーの既往があったため，外来にて金属パッチテストを施行した．PMに使用されている金属にアレルギーは認められなかったが，予防的にPM本体をpolytetrafluoroethylene（PTFE）シート（ゴアテックスシート®）にて被覆し，植込み術を行った．植込み後12カ月が経過したが，PM植込み部のトラブルは生じていない．PMアレルギーのためPM植込み部の皮膚炎を繰り返す症例に対し，PTFEシートを使用した報告はいくつかある．今回われわれは，予防的PTFEシート被覆にてPM植込み部トラブルを回避した，金属アレルギーの1症例を経験したため，報告する．

ObjectivePredicting the risk factors for late aortic events in patients with type B acute aortic dissection without complications may help to determine a therapeutic strategy for this disorder. We investigated whether late aortic events in type B acute aortic dissection can be predicted accurately by an index that expresses the degree of fusiform dilatation of the proximal descending aorta during the acute phase; this index can be calculated as follows: (maximum diameter of the proximal descending aorta)/(diameter of the distal aortic arch + diameter of the descending aorta at the pulmonary artery level).MethodsPatients with type B acute aortic dissection without complications (n = 141) were retrospectively analyzed to determine the predictors of late aortic events; these include aortic dilatation, rupture, refractory pain, organ ischemia, rapid aortic enlargement, and rapid enlargement of ulcer-like projections.ResultsThe fusiform index in patients with late aortic events (0.59) was higher than that in patients without late aortic events (0.53, P < .01). Patients with a higher fusiform index exhibited aortic dilatation earlier than those with a lower fusiform index. By multivariate analysis, we conclude that the predominant independent predictors of late aortic events were a maximum aortic diameter of 40 mm or more, a patent false lumen, and a fusiform index of 0.64 or more (hazard ratios, 3.18, 2.64, and 2.73, respectively). The values of actuarial freedom from aortic events for patients with all 3 predictors at 1, 5, and 10 years were 22%, 17%, and 8%, respectively, whereas the values in those without these predictors were 97%, 94%, and 90%, respectively.ConclusionsThe degree of fusiform dilatation of the proximal descending aorta, a patent false lumen, and a large aortic diameter can be predominant predictors of late aortic events in patients with type B acute aortic dissection. Patients with these predictors should be recommended to undergo early interventions (surgery or stent-graft implantation) or at least be closely followed up during the chronic phase before such events develop.

Context: Natriuretic peptide receptor-B (NPR-B, GC-B in rodents; gene name NPR2) is a guanylyl cyclase-coupled receptor that mediates the effect of C-type natriuretic peptide. Homozygous mutations in human NPR-B cause acromesomelic dysplasia, type Maroteaux (OMIM 602875), an autosomal recessive skeletal dysplasia. NPR-B has an intracellular kinase homology domain (KHD), which has no kinase activity, and its functional significance in vivo is currently unknown.Objective: We examined the functional significance of a novel NPR-B KHD mutation in humans.Patients and Methods: A 28-yr-old Japanese male presented with marked short stature (118.5 cm, −9.3 sd). His limbs showed marked shortening in the middle and distal segments. His parents had relatively short stature with height z-scores of −2.75 and −0.98 (his father and mother, respectively). Direct sequencing of coding region of the NPR2 gene of the family was performed. The mutant receptor activity was investigated by saturation binding assay and cGMP measurement. Additionally, interaction between the mutant and wild type allele was investigated by the titration experiments.Results: We identified a novel missense mutation L658F in KHD of NPR-B in homozygous and heterozygous states in the patient and his parents, respectively. The mutation conferred normal binding affinity for C-type natriuretic peptide but no discernible ligand-induced cGMP production. Furthermore, L658F mutant impaired wild-type NPR-B-mediated cGMP production in a dose-dependent manner, suggesting that short stature found in L658F heterozygote can be caused by its dominant-negative effect.Conclusions: This study provides the first evidence that intact KHD of NPR-B is essential for skeletal development.

症例は55歳の男性. 平成17年10月中旬に胸部不快感のため近医受診し, 発作性上室性頻拍と診断され内科的に加療されるも心房細動に移行したため当院循環器内科に紹介となった. EPSにてKENT束を持つ潜在性WPW症候群と診断されたが, 心臓超音波検査で僧帽弁後尖に付着する腫瘍を認められ, 手術目的で当科紹介となった. 準緊急的に腫瘍切除, 僧帽弁形成術および副伝導路切断術を施行した. 病理組織学的検査で乳頭状弾性線維腫と診断された. 術後は洞調律で経過し, EPSにてKENT束の離断を確認した. 現在, 不整脈や腫瘍の再発を認めず, 同時手術は有用であった.

大動脈四尖弁は発生頻度が非常に低く,大動脈弁閉鎖不全に対する手術例の報告も少ない.今回われわれは,経食道および経胸壁心エコーにて術前に大動脈四尖弁による閉鎖不全症と診断し,弁置換術を施行した症例を経験したので報告する.症例は37歳男性で,左胸部違和感を主訴に来院した.経食道および経胸壁心エコーを施行したところ,大動脈四尖弁による閉鎖不全症と診断された.心臓カテーテル検査にて大動脈閉鎖不全(AR) III度と診断され,手術適応として当科紹介となった.術中所見では左右冠尖が同大で,無冠尖と過剰弁は同大であるが他の2つに比してやや小さかった.手術は人工弁にて弁置換術を行い,経過は良好であった.

肩・鎖骨などの整形外科的手術で固定に用いる鋼線の術後の移動・迷入はまれな合併症であると言われているが我々は2例の胸腔内迷入症例を経験したので報告する.症例1は34年前に左胸鎖関節脱臼のため固定術を受けた56歳男性で, 他病で入院した際の胸部X線検査にて縦隔内に存在する金属性異物を指摘された.胸部CTでは前縦隔内大動脈弓部の前方に位置していたため, 胸腔鏡下摘出術を行った.症例2は右鎖骨骨折に対して経皮的整復固定術を受けた71歳女性で, 3ヵ月後に発熱のため撮影した胸部X線写真にて, キルシュナー鋼線 (2本) の移動を認めた.先端がそれぞれ肺実質内と縦隔内の血管の間に達していたため右開胸にて除去した.心血管系の損傷を起こして死亡した症例の報告もあり, 固定用鋼線の移動・迷入が確認された場合には症状の有無にかかわらず早期の摘出術が必要である.

症例は79歳男性.腹部大動脈瘤手術後9年目に間歌性下血を呈し精査中に, Y型人工血管置換部中枢側に約6cmの腹部大動脈瘤の再発を認めた.下血症状は消失し, I型人工血管術を施行した.腹部大動脈と腸管との間に瘻孔はなかったが,術後4日目に再度間欺性の下血症状が出現し,術後8日目には大量下血によるショック症状を呈し緊急開腹手術となった.骨盤腔内の癒着した小腸を剥離すると,Y型人工血管左脚の吻合部から約2cm離れた部位に外腸骨動脈瘤腸管瘻があり,手術にて救命できた.動脈瘤腸管瘻は下血の原因としては稀であるが,術前診断が困難であるだけでなく,手術時期が遅れると致死的となる.腹部大動脈瘤術後9年目に間歌性下血で発症したことで,診断に苦慮したが,早期診断のためには本疾患も念頭に置き精査する必要があると思われる.

症例は58歳女性.検診で胸部X線写真で異常陰影を指摘され当院受診した.胸部CT検査, MRI検査で巨大な心膜嚢腫を疑われた.胸腔鏡下に嚢胞内貯留液を吸引することで容易に巨大嚢腫を切除できた.嚢腫は心膜と連結していたが交通はなく,病理組織学的診断は心膜嚢腫であった.心膜嚢腫は比較的稀な疾患であるが,本症例の如く嚢胞液中の腫瘍マーカー (CA19-9, CA125) が異常高値を示した巨大な心膜嚢腫を胸腔鏡下に切除した症例の報告はない.心膜嚢腫は悪性化の報告もあり,積極的な外科的切除が望ましいと考えているが,胸腔鏡下手術は術中の病理学的検査で確定診断が得られ,嚢腫内容液の吸引することで容易に心膜から剥離切除でき理想的な治療法と考える.

Peters Plus syndrome is a very rare autosomal recessive condition characterized by ocular defects (typically Peters anomaly) and other systemic major/minor anomalies. Mutations in the B3GALTL gene encoding beta 1,3-glucosyltransferase have been found in virtually all patients with typical Peters Plus syndrome. We report on a female patient with unusually severe manifestations of Peters Plus syndrome, including anterior eye staphyloma, cleft lip and palate, and hypoplastic left heart syndrome (HLHS). Analysis of the B3GALTL gene revealed no mutation in the patient. To our knowledge, HLHS has not previously been reported in Peters Plus syndrome so far, and anterior staphyloma, a most severe defect of the anterior eye chamber, is also apparently rare in the syndrome. Our patient might represent a new syndrome of severe Peters Plus syndrome-like phenotype with anterior eye staphyloma and HLHS.

Between August 1992 and February 1998, 43 patients were treated with percutaneous cardiopulmonary support (PCPS) in our institution, and 8 of them subsequently required emergency cardiac surgery. There were 3 males and 5 females with a mean age of 63 years (range, 37 to 81 years). The etiology of shock in these 8 patients was acute myocardial infarction in 3, postinfarction left ventricular (LV) free wall rupture in 1, postinfarction ventricular septal perforation (VSP) in 1, LV free wall rupture and VSP in 1, and fatal arrhythmia due to severe aortic valvular disease in 2. The mean time interval from the onset of cardiogenic shock to the institution of PCPS was 77 min (range, 18 to 183 min). The mean time interval from the institution of PCPS until surgery was 145 min (range, 40 to 603 min). The surgical procedures were coronary artery bypass grafting (CABG) in 3 patients, closure of the LV rupture and/or closure of VSP in 3, and aortic valve replacement in 2. Six patients were weaned from PCPS, and 2 patients were discharged from the hospital (discharge rate, 25%). Although the results of emergency cardiac surgery following PCPS still are not satisfactory, we continue to apply PCPS and perform appropriate surgical procedures to improve the survival rate of the patients who would die without PCPS.

1993年1月より1998年5月までに当院にて経験した胸部大動脈瘤に起因する大動脈気管支肺瘻 (Aortobronchopulmonary fistula, ABPF) 12例を対象とした. 男性7名, 女性5名, 年齢は30～85歳 (平均67.4歳) であった. 非感染性の真性瘤 (4例) によるABPFでは, 手術で肺と癒着している動脈壁との血流を隔離することにより良好な結果を得た. 胸部動脈瘤術後の感染性仮性瘤によるABPF (4例) は, 手術施行, 非施行症例とも全例急性期または遠隔期に再喀血で死亡した. 大動脈解離に合併したもの (1例) は肺との癒着が広範囲であり, 血栓化した偽腔を貫通して複数のABPFが形成されたため術後も喀血が持続し死亡した. 手術非施行となった3症例は全例喀血で死亡した. 感染合併例では確実な感染コントロールが困難で, 予後不良であった. 感染性4症例は無症状にABPFが形成され突然の喀血により死亡したため, 厳密な経過観察が必要である.

Between February 1994 and January 1997, 102 of the 146 patients treated by coronary artery bypass grafting (CABG) had undergone magnetic resonance angiography (MRA) of the brain and neck before the operation, and arterial stenosis or occlusion had been detected in 38 (36.9%) of them. Two of these patients had complicating severe calcification of the ascending aorta, and CABG was performed without cardiopulmonary bypass (CPB). Seven patients without stenotic lesions on MRA were also treated by CABG without CPB for other complications. In addition to the 102 patients one patient had been found to have occlusion of the left common carotid artery and poor enhancement of the distal portion, and as a result we switched from CABG to percutaneous transluminal angioplasty (PTCA). We enrolled 93 patients in this study excluding these 10 patients. The patients were distributed into the three groups according to the MRA findings. Group C = no stenotic lesions (58 patients). Group S = stenosis of70% (26 patients), Group SS = stenosis ofor = 70% (9 patients). Enhancement distal to the stenotic or occlusive lesions was good in all patients in group S and SS. We then examined them for the incidence of postoperative neurological complications. There were no significant differences among the three groups in regard to age, male/female ratio, or incidence of hypertension and hyperlipidemia. In Group S, the incidence of diabetes was significantly higher than in the other Groups. The incidence of prior stroke was significantly higher and the number of coronary arteries affected was significantly larger in group SS than the other groups. There were no significant differences among the three groups with regard to intraoperative variables. The lowest mean arterial pressure on CPB was 44.3 +/- 7.4 mmHg, 48.0 +/- 8.8 mmHg, 46.3 +/- 7.8 mmHg in Group C, S, and SS, respectively, In all groups the lowest mean arterial pressure on CPB was below 50 mmHg. There were no significant differences among the three groups with regard to time to awaken and time to extubation. Two patients experienced transient conciousness disturbance after CABG, one in Group C, the other in Group SS, but no new lesions were detected by brain CT. Only one patient, in Group C. suffered a stroke and had a new lesion on brain CT a month after the operation. No strokes occurred in the perioperative period. In nine patients with good enhancement distal to the severe stenotic or occlusive lesion on MRA of the brain and neck the lowest mean arterial pressure on CPB was below 50 mmHg, but there was no postoperative neurological complications due to the low perfusion pressure on CPB. The results of this study suggested that CABG with CPB can be performed safely in patients with good enhancement distal to the stenotic or occlusive lesions on MRA of the brain and neck.

A 37-year-old woman was taken to a hospital because of sudden chest pain. She lapsed into shock, and the ECG indicated acute myocardial infarction. The ECG later showed ventricular fibrillation, and the patient was given cardiac massage while being transported to our hospital, where she was resuscitated with a percutaneous cardiopulmonary support system. Emergency coronary angiography revealed 99% stenosis of the left main coronary artery. PTC A was performed, and the stenotic lesion was released, but dissection and rapid formation of a thrombus were detected in the LAD. Re-PTCA was performed, but the hemodynamics did not improve, and emergency CABG of the LAD, Dl, and LCx was performed. Postoperative max CPK was 18,957 IU/L. Although postoperative MRSA pneumonia developed as a complication, weaning from the respirator was performed 17 days after the operation. The patient was discharged, ambulatory, 74 days after the operation.

The percutaneous cardiopulmonary support system (PCPS) was used in a 64-year-old woman with cardiogenic shock due to sustained ventricular fibrillation (Vf) caused by severe aortic stenosis and regurgitation. The Vf attack was resistant to cardioversion and adrenaline for lack of left ventricular support by PCPS. She was transported to the operation theater with PCPS in situ and emergency aortic valve replacement was performed. Although preoperative cardiac resuscitation time was long (35 minutes), she was discharged from the hospital on foot without any neurological complications on 84th postoperative day. Because PCPS does not decrease left ventricular systolic stress in poorly contracting dilated heart, early surgical treatment is needed in patients with severely damaged heart.

症例は52歳, 男性. 40歳ころより労作時の息切れを生じるようになり, 最近, 呼吸困難, 下腿浮腫が出現するようになった. 精査の結果, 高度の三尖弁閉鎖不全を認め, Carpentier-Edwards ring (34mm) にて三尖弁輪縫縮術を施行した. 術中所見では三尖弁輪の異常拡大を認めたが, 各弁尖は軽度菲薄化しているのみであり, 他の合併心奇形も認めなかったため孤立性三尖弁閉鎖不全と診断した. 術後経過は良好で, 抗凝固療法も必要とせず現在社会復帰している.

われわれは,大動脈弁原発の乳頭状弾性線維腫の1例を経験したので報告する.症例は71歳,女性.畑仕事中に倒れ当院へ緊急搬送され脳梗塞の診断で入院加療されていたが,心エコーにて大動脈弁左冠尖に3×10mm大の疣贅エコーあり,当科紹介となった.全身状態の改善を待ち手術を施行.人工心肺下に手術を行った.左冠尖弁尖に約10mm大の茎を有するイソギンキャク様の腫瘍を認めた.腫瘍切除のみ行い大動脈は温存した.病理組織学的に乳頭状弾性線維腫と診断された.術後は合併症なく順調に経過した.心臓原発の腫瘍は稀であり,その7割は良性腫瘍である.しかし乳頭状弾性線維腫は脳梗塞や心筋梗塞といった重大な合併症をきたしうるため,発見次第早期に摘出をすることが推奨されている.

右鎖骨下動脈起始異常を伴う偽性大動脈縮窄に合併した遠位弓部大動脈瘤の1手術例を経験した. 症例は22歳女性, 一卵性双生児の姉で, 心室中隔欠損自然閉鎖の既往歴がある. 健康診断の際, 胸部X線写真にて左上縦隔の拡大を指摘され, 精査にて偽性大動脈縮窄に合併した遠位弓部大動脈瘤および右鎖骨下動脈起始異常と診新された. 選択的脳分離体外循環下にて遠位弓部人工血管置換術および左鎖骨下動脈再建術を施行し良好な結果を得た. 偽性大動脈縮窄に合併した動脈瘤は, 壁が脆弱で破裂の危険性が高いため早期手術が必要と思われた. また一卵性双生児の妹には偽性大動脈縮窄を伴っておらず, 同疾患の成因は遺伝的素因でなく大動脈弓の発生異常であるとする説を支持していると思われた.

We report here a patient with features of Down syndrome and tetralogy of Fallot who had a 21q22 duplication. The extent of the duplication was defined using fluorescent hybridization probes that map to the critical region on chromosome 21. Included within the interval was the cell adhesion molecule DSCAM but not the collagen COL6A1. The present case provides further support to the concept that there exists Down syndrome-associated congenital heart disease gene(s) on chromosome 21q22 and that over-expression of DSCAM may contribute to the cardiac defects of Down syndrome.

A computer simulation was carried out to investigate the influence of nonpulsatile left ventricular assistance on hemodynamics. A simulation circuit was constructed to represent the circulatory system. A source of current was added to denote the nonpulsatile blood pump. The left and right ventricles were replaced by variable compliances. Left heart failure was simulated by decreasing the amount of compliance change of the left ventricle. We introduced a pulsatility indicator (PI) to clarify the pulsatility characteristics in the hemodynamics; this PI was defined as the ratio of the pulse pressure (PP) to the mean aortic pressure (AoP). When nonpulsatile bypass flow increased, the mean AoP, tension time index (TTI), and diastolic pressure time index (DPTI) increased, and cardiac output, PP, and PI decreased. When assisted flow increased with the constant total flow rate, the mean AoP and DPTI changed little; the PP, TTI, and PI decreased, and the endocardial viability rate increased. The PI would be helpful in evaluating the effect of pulsatility.

高位腹部大動脈閉塞を伴った2枝梗塞の59歳男性に対して, 左内胸動脈 (LITA), 右胃大網動脈 (RGEA) の動脈グラフトのみを用いた冠状動脈バイパス術と腹部大動脈-大腿動脈バイパス術を同時に施行した. IABPが必要となった際の挿入経路, 体外循環中の下肢灌流, LITAが左下肢動脈への側副血行路, 動脈グラフトのみを使用することを考慮して, 本症例では体外循環に先駆けてYグラフトによる血行再建術を行い, 引き続き冠状動脈バイパス術を行った. 体外循環時間の短縮, 下肢虚血防止に効果があり, 良好な結果を得た.

We report the successful surgical treatment of a rare case of chronic expanding hematoma in the pericardial cavity that developed into a very large mass over a long period. The patient, who had a history of cardiac surgery for a double-outlet right ventricle 14 years previously and for tricuspid regurgitation 8 years ago, noticed a slowly growing mass near the left atrium and ventricle 5 years ago. The mass, which confirmed a diagnosis of chronic expanding hematoma, was resected by left thoracotomy.

Tracheal disruption due to blunt chest trauma is relatively rare but often fetal. Further improvement in life-saving strategy is expected. Here two cases of tracheal disruption due to a traffic accident with different clinical course are reported on. Case I: A 35-year-old woman manifested tension pneumothorax immediately after the injury to develop dyspnea. Emergency operation was carried out within 4 hours after the injury. Tracheoplastic procedure was able to save the patient's life. Case II: A 58-year-old man had a completed atelectasis of the left lung due to encapsulated tracheal disruption three weeks after a traffic accident and was also successfully treated by tracheoplastic procedure. It should be emphasized that early diagnosis and primary repair are important in the treatment of tracheal distruption due to blunt chest trauma.

We have operated upon 18 cases of true arch aneurysm, including 2 cases of rupture, in a recent 6 years and 3 months. Seventeen aneurysms were arteriosclerotic and one was syphilitic. Operative adjuncts during aortic cross clamping were left heart bypass with a centrifugal pump (LHB, 4 cases), retrograde cerebral perfusion (RCP, 5 cases) and selective cerebral perfusion (SCP, 9 cases). LHB was applied to localized aneurysm apart from the left subclabina artery. It was safely conducted during operation, but cerebral embolism happened in 2 cases by aortic cross clamping, leading to one hospital death. RCP was carried for emergency cases by rupture or inpending rupture. Recent three cases was operated on by left thoracotomy under RCP. SCP was conducted in 9 cases of large aneurysm, including 6 cases of total arch replacement. There was two operative deaths, but minimum complications in the survivors. Operative adjunct was selected by condition of true arch aneurysm, but we have thought that SCP was safely applied among these adjuncts.

Our ventricular assist device uses a valveless volumetric pump operating on the Maillard-Wankel rotary principle. It is driven by an electric motor and provides a semi pulsatile flow. At each cycle, blood is actively aspirated into the device, and overpumping results in collapse at the pump inlet. To prevent overpumping, it is necessary to ensure that pump intake does not exceed venous return. Poor long-term reliability rules out the use of current implantable pressure sensors for this purpose. To resolve this problem, we have developed a method of control based on monitoring of the intensity of electric current consumed by the motor. The method consists of real time monitoring of current intensity at the beginning of each pump cycle. A sudden change in intensity indicates underfilling, and motor speed is reduced to prevent collapse. The current consumed by the motor also depends on the afterload, but the form of the signal remains the same when afterload changes. After demonstrating the feasibility of this technique in a simulator, we are now testing it in animals. We were able to detect and prevent collapse due to overpumping by the cardiac assist device. This system also enables us to know the maximum possible assistance and to thus adapt assistance to the user.

A new membrane oxygenator was devised and assessed for performance in animal experiments. A silicone fiber membrane oxygenator, heat exchanger, and arterial blood reservoir are the components. The oxygenator is an outside flow type with minimum intrapulmonary resistance and, consequently, gas transfer is by gravity venous drainage. This device was used to conduct normothermic cardiopulmonary bypass (CPB) in 10 sheep over a period of 8 hrs (high flow group: 6 sheep, mean flow, 4.7 + 0.5 L/min; low flow group: 4 sheep, mean flow, 1 L/min). Performance was evaluated with gas transfer capacity, hematologic examination during CPB, and scanning electromicroscopic (SEM) appearance of the membrane after CPB. Blood gas was maintained with high PO2 and acceptable PCO2 levels throughout CPB. Plasma free hemoglobin was significantly low and fibrinogen was also maintained within physiologic levels. Scanning electron microscopy showed no thrombus formation on the membranes of either group. These results indicate this oxygenator to be quite adequate for gas exchange and prevention of blood cell trauma; it is also applicable to pulsatile or separating CPB because of the arterial blood reservoir.

We report a case of a 45-year-old man with thymic carcinoids in multiple endocrine neoplasm-type 1. Extended total thymectomy was performed through a median sternotomy, and the mediastinal regional lymph nodes and fibroadipose tissue were dissected. Multiple endocrine neoplasm-type 1 related thymic carcinoids are rare and have a poor prognosis. The efficacy of radiotherapy and chemotherapy for prolonging survival is limited, and an aggressive surgical approach with complete excision of the tumor with resection of the peripheral tissues and dissection of the mediastinal lymph nodes seems to be the best available treatment today. We have found that this practice of thymectomy at an early stage in combination with genetic and effective radiological treatment can be effective for multiple endocrine neoplasm-type 1 patients.

We report a relatively rare surgical treatment for two cases of inflammatory pseudotumors of the lung. In case 1, a 52-year-old male with a history of left chest pain was admitted to our hospital for an abnormal nodule with an irregular margin that was detected in the left upper lung field. The nodule, measuring 15 mm in diameter, was larger than the one observed six months earlier, which had been removed by a thoracoscopic resection. In case 2, a 64-year-old female with a history of chronic cough and hemoptysis was admitted to our hospital, and an abnormal nodule with pleural indentation was detected in the lower left lung field. The nodule, measuring 8 mm in diameter, was also removed by a thoracoscopic resection. In both cases, the histologic examination enabled us to diagnose the lesion as an inflammatory pseudotumor. In general, it is very difficult to differentiate inflammatory pseudotumors from malignant tumors of the lung. The best treatment for inflammatory pseudotumors is usually early and complete surgical resection, since it can lead to improved survival. Therefore, we consider thoracoscopy-aided surgery to be less invasive and more useful than other surgical methods in the diagnosis and treatment of inflammatory pseudotumor of the lung.

Peroneal artery aneurysms in Behçet's disease have not been described to date. We present such a patient who was treated successfully using transcatheter embolization.

Deletion of TWIST on 7p21 leads to Saethre-Chotzen syndrome, whereas deletion of the HOXA cluster on 7p15.2 leads to hand-foot-genital syndrome. We report here a patient with 46,XY,del(7)(p15.2p21) who had craniosynostosis, maxillary hypoplasia, prominent ear crus, rectoperineal fistula, and hypoplastic fifth fingers. Using fluorescence in situ hybridization, we demonstrated the deletion to encompass the TWIST locus and the HOXA cluster. We suggest that many, if not all, of the features of this patient could be accounted for by combined haploinsufficiency of the TWIST and HOXA cluster. Hence, the patient's phenotype may define a new contiguous gene syndrome on 7p.

We performed surgical treatment for abnormal echoes in the left ventricular outflow tract caused by ruptured mitral chordae tendineae. An asymptomatic 68-year-old man had a chordal rupture exhibiting a tumor-like lesion in the left ventricular outflow tract on echocardiography. Considering a high level of mobility of the tumor as well as its texture, the risk of embolization was found to be significant. Therefore, it was decided on the surgical resection of the tumor-like lesion under extracorporeal circulation. We report a highly rare case of rupture in redundant mitral chordae tendineae without mitral regurgitation, revealed by uncommon echoes in the left ventricular outflow tract during systole.

Association of the pink-eye-dilution gene (P) with hypopigmentation is seen in patients who have oculocutaneous albinism type 2 (OCA2) and Prader-Willi syndrome (PWS) or Angelman syndrome (AS). However, it remains unknown whether duplication or amplification of the P gene causes hyperpigmentation. We previously reported a woman who had hyperpigmentation with a duplication of the proximal part of 15q, including the P gene. Here, we describe an additional patient with mosaicism of inv dup(15) and clinical manifestations of severe psychmoter retardation, epilepsy, and pigmentary dysplasia showing mottled and linear patterns of hyperpigmentation. His karyotype was 47,XY,+idic(15)(pter-->q14::q14-->pter)[38]/46,XY[12] de novo. Chromosomal fluorescence in situ hybridization (FISH) showed six copies of the P gene. Therefore, his cutaneous mosaicism might be caused by the presence of both normal and hyperpigmented skin due to multicopies of the P gene.

A pump circuit was assembled and examined for use as an implantable artificial heart. The circuit consisted of a gear pump and 4 artificial heart valves. Mitral and pulmonary arterial valves were placed at the inflow port of the pump, and aortic and tricuspid valves were placed at the outflow port. The mitral and the tricuspid valves were connected to each reservoir at 10 mm Hg, and the aortic and the pulmonary arterial valves were connected to the head tanks at 80 and 20 mm Hg, respectively. The pump discharged pulsatile flows into both systemic and pulmonary arteries alternately by switching the direction of rotation periodically. Because the rated discharge was 1.7 L/min for the gear pump used, the measured flow rate was 0.8-0.75 L/min at a heart rate of 60–110 bpm.

A 19-year-old woman with a history of drug addiction suffered from sepsis and heart failure. Blood culture was positive for Streptococcus viridans. An operation was indicated because the echocardiography showed massive vegetation on the anterior leaflet of the tricuspid valve and severe regurgitation even though the endocarditis was healed with drug therapy. At operation all of the anterior leaflet of the tricuspid valve was resected with the vegetation. Using the technique of cusp commissuroplasty, the disrupted commissure was reconstructed by approximating the septal and posterior cusps at the level of their normal closure, forming a zone of apposition by using a single stitch. Leaflet apposition resulted in a defect between the apposed leaflets and the tricuspid annulus, which was patched with autologous pericardium. The tricuspid valve was reconstructed to function as a unicommissural bicuspid valve. The patient was stable during the follow-up period of two years without any medical treatment.

A 48-year-old woman with an abnormal shadow in chest radiography during an annual physical examination was found by chest computed tomography to have a large fatty mass lesion found to be diagnosed as a gigantic lipoma. Histopathological diagnosis was found to be benign thymolipoma consisting of mature fatty tissue and hyperplastic thymic tissue structures with Hassall,s corpuscles. Although the diagnosis is supported by imaging studies that demonstrate fat and soft tissue within the tumor, variations occur in computed tomography appearance. We suggest that surgical excision be considered when a gigantic intrathoracic lipomatous mass is in scanning as in this case.