[Primary cardiac angiosarcoma in the left atrium with adrenal metastasis; report of a case].

We encountered a 61-year-old woman with primary cardiac angiosarcoma in the left atrium. On echocardiography, the tumor extended into the atrial septum and mitral valve, and mitral valve stenosis and regurgitation were significant. We resected the tumor protruding into left atrium, and affecting mitral valve. The surgical procedure was not radical, but on postoperative echocardiography, function of the mitral valve was improved. Three months later, elevation of her right diaphragma was observed on chest X-ray and a giant adrenal tumor was detected by magnetic resonance imaging. Tumor biopsy indicated that this tumor was adrenal metastasis from cardiac angiosarcoma. In addition, echocardiography showed the recurrence of angiosarcoma in the left atrium and the presence of mitral stenosis and regurgitation. She died of heart failure 185 days postoperatively.

Identification of ephrin-A3 and novel genes specific to the midbrain-MHB in embryonic zebrafish by ordered differential display.

Development of the tectum and the cerebellum is induced by a reciprocal inductive signaling between their respective primordia, the midbrain and the midbrain/hindbrain boundary (MHB). We set out to identify molecules that function in and downstream of this reciprocal signaling. Overexpression of LIM domain of the transcription factor Islet-3 (LIM(Isl-3)) leads to inhibition of this reciprocal signaling and to resultant defects in tectal and cerebellar development. We therefore searched for genes that may be either up- or down-regulated by overexpression of LIM(Isl-3) by comparing the gene expression profiles in the midbrain and the MHB of normal embryos and embryos in which Islet-3 function was repressed, using a combination of ordered differential display and whole-mount in situ hybridization. Among genes identified in this search, two cDNA fragments encoded Wnt1 and FGF8, which are already known to be essential for the reciprocal signaling between the midbrain and the MHB, confirming the effectiveness of our strategy. We identified four other partial cDNA clones that were specifically expressed around the MHB, ten cDNAs specifically expressed in the tectum, and three cDNAs expressed in neural crest cells including those derived from the midbrain level. The ephrin-A3 gene was specifically expressed in posterior tectum in a gradient that decreased anteriorly. Although ephrin-A2 and ephrin-A5 have been reported to be expressed in the corresponding region in mouse embryos, the superior/inferior colliculi, mouse ephrin-A3 is not expressed prominently in this region, suggesting that the role of ephrin-A3 in brain development may have been altered in the process of brain evolution.

Functional repression of Islet-2 by disruption of complex with Ldb impairs peripheral axonal outgrowth in embryonic zebrafish.

Islet-2 is a LIM/homeodomain-type transcription factor of the Islet-1 family expressed in embryonic zebrafish. Two Islet-2 molecules bind to the LIM domain binding protein (Ldb) dimers. Overexpression of the LIM domains of Islet-2 or the LIM-interacting domain of Ldb proteins prevented binding of Islet-2 to Ldb proteins in vitro and caused similar in vivo defects in positioning, peripheral axonal outgrowth, and neurotransmitter expression by the Islet-2-positive primary sensory and motor neurons as the defects induced by injection of Islet-2-specific antisense morpholino oligonucleotide. These and other experiments, i.e., mosaic analysis, coexpression of full-length Islet-2, and overexpression of the chimeric LIM domains derived from two different Islet-1 family members, demonstrated that Islet-2 regulates neuronal differentiation by forming a complex with Ldb dimers and possibly with some other Islet-2-specific cofactors.

[A case of teratoma in both the mediastinum and the intrapulmonary system].

A rare case of mature teratoma in both the mediastinum and the intrapulmonary system is presented. A 30-year-old male was admitted to our hospital due to tumor masses in the mediastinum and the left lung. We performed mediastinal tumor resection and left upper partial lobectomy. Neither tumor communicated with each other. Pathological findings revealed teratoma in the mediastinal lymph node and the intrapulmonary system including no malignant cells in either tumor. In this case, because metastasis and perforation were negative, we proposed that both tumors occurred at the same time in the early embryo.

Compartmentalized expression of zebrafish ten-m3 and ten-m4, homologues of the Drosophila ten(m)/odd Oz gene, in the central nervous system.

Zebrafish ten-m3 and ten-m4 encode proteins highly similar to the product of Drosophila pair-rule gene ten(m)/odd Oz (odz). Their products contain eight epidermal growth factor (EGF)-like repeats that resemble mostly those of the extracellular matrix molecule tenascin. During segmentation period, ten-m3 is expressed in the somites, notochord, pharyngeal arches, and the brain, while expression of ten-m4 is mainly restricted to the brain. In the developing brain, ten-m3 and ten-m4 expression delineates several compartments. Interestingly, ten-m3 and ten-m4 show expression patterns complementary to each other in the developing forebrain and midbrain along both rostrocaudal and dorsoventral axes, depending on developmental stages and locations.

Association of the sea urchin EGF-related peptide, EGIP-D, with fasciclin I-related ECM proteins from the sea urchin Anthocidaris crassispina.

Exogastrula-inducing peptides (EGIP) of the sea urchin Anthocidaris crassispina are endogenous peptides related to epidermal growth factor (EGF), which induce exogastrulation in the embryo. Recently, a protein(s) from sea urchin embryos that binds to one of the EGIP, EGIP-D (EGIP-D-binding protein, EBP) was purified. The isolation and characterization of the cDNA clones for two EBP proteins (EBP-alpha and EBP-beta) is reported. The two EBP proteins were highly similar in structure to each other; both possessed putative cell-binding sites and two repeated sequences characteristically seen in the insect neuronal cell adhesion protein, fasciclin I. The EBP showed similarity with other sea urchin proteins HLC-32, Bep1, and Bep4. It has been confirmed that bacterially expressed EBP proteins associate with EGIP-D as does native EBP, suggesting the interaction between EGF-related proteins and fasciclin I-related proteins. An EBP transcript of 1.4 kb was strongly expressed in immature ovaries but not in immature testes. A somewhat lower level of the transcript existed in unfertilized eggs and the amount gradually declined to an almost undetectable level by the pluteus stage. The EBP proteins were present throughout embryonic development at nearly constant levels. Although most of the proteins were distributed rather evenly in the cytoplasm, a small portion was detected on the apical surface of blastomeres and ectodermal cells, showing that EBP are components of the hyaline layer.

[A case of successful reoperation for aortic root pseudoaneurysm ruptured into right ventricle after modified Bentall operation].

The patient was a 51-year-old man with Marfan syndrome who had simultaneously undergone modified Bentall operation (Carrel patch method) and coronary artery bypass grafting. Prosthetic valve endocarditis (PVE) occurred 19 months after the operation. PVE had thereafter been treated by antibiotic therapy for 3 months, but echocardiography revealed prosthetic valve detachment and aortic root pseudoaneurysm ruptured into the right ventricle which appeared to have been caused by PVE. At reoperation there were no laboratory or intraoperative findings indicative of ongoing inflammation or infection. The composite graft, therefore, didn't require replacing, and it was possible to simply re-suture the composite graft and directly close the tear. The postoperative course has been uneventful with no further evidence of endocarditis.

Purification of EGIP-D-binding protein from the embryos of the sea urchin Anthocidaris crassispina.

Exogastrula-inducing peptides (EGIPs) are intrinsic factors that are present in eggs and embryos of the sea urchin Anthocidaris crassispina. They induce exogastrulation when added exogenously to the embryos. In the present study, we isolated an EGIP-D-binding protein (EBP) from a homogenate of mesenchyme blastulae. EBP had an apparent molecular weight of 33,000. The N-terminal amino acid sequence of EBP had a sequence homology to HLC-32 and bep4 identified in other sea urchin embryos. In addition to its ability of binding to EGIP-D, EBP also inhibited exogastrulation induced by EGIP-D. These results suggest that EBP plays an essential role in EGIP-D-induced exogastrulation.

[Single-stage surgery for annulo aortic ectasia, Stanford type-A aortic dissection and pectus excavatum in a patient with Marfan's syndrome].

A 43-year-old female was hospitalized on October 25, 1993, due to severe back pain. She was diagnosed with annulo aortic ectasis, Stanford type-A acute aortic dissection and pectus excavatum caused by Marfan's syndrome. On November 30, 1993, single-stage surgery was performed, including modified Bentall procedure, aortic arch replacement, elephant trunk method and sternoplasty. Concerning the procedure for pectus excavatum, sternoplasty was selected because we thought that sternoplasty could endure the long procedure which would cause infection compared with sternal turnover. Postoperative course was satisfactory, and she is well 18 months after surgery.

[Coronary artery bypass grafting for ischemic heart disease associated with acromegaly: a case report].

Coronary artery bypass grafting (CABG) for ischemic heart disease associated with acromegaly has never been reported in Japan, though coronary artery sclerosis is known as complicating factor in acromegaly. A 44-year-old man with ischemic heart disease was admitted to our hospital and emergency percutaneous transluminal coronary recanalization was performed. After admission, he was diagnosed as acromegaly from endocrinological studies and operated on CABG. The peri- and post-operative course was uneventful in spite of our fear for complications due to acromegalic heart muscle disease.

[Repair of mitral valve prolapse by resection and sliding plasty].

There have been many techniques applied to the repair of mitral valve prolapse, and the method used in a particular case is usually selected according to the position and extent of the lesion. To simplify and standardize the technique of mitral valve repair, we have adopted the resection, sliding plasty and ring annuloplasty methods since December 1992. Of 10 consecutive surgical cases, 2 involved prolapse of the anterior leaflet, 1 the posteromedial commissural, and 7 the posterior leaflet. One patient with posterior leaflet prolapse required valve replacement due to dehiscence of the plastied site on the 3rd postoperative day, and one died because of sepsis. However, the remaining patients were doing well without mitral regurgitation at a mean of 20 months (range: 8-32) after the operation. The advantages of these techniques include easy adjustment of the height of the leaflet and a good chance of long-term durability, since the affected lesion is resected.

[Surgical repair of post-infarction ventricular septal defects--reconstruction with pericardial patch].

Despite improvements in the pre- and postoperative management of patients with post-infarction ventricular septal defects, the results after surgery have been variable. This is in part due to advanced patient age, multi-vessel coronary disease. In addition, resection of the infarcted ventricular septum results in significant compromise in left ventricular function. Since January of 1993, we have used a new technique for the reconstruction of the left ventricle using a single pericardial patch. In this method, which was introduced by Komeda and David in 1990, a single patch is sutured to the interventricular septum and the lateral ventricular wall, excluding the infarcted muscle from the left ventricular cavity. This method was used in the treatment of four patients. There was no bleeding along the suture lines at operation, all of the patients survived. Postoperatively, all four were functional New York Heart Association Class 1. One patient underwent re-operation on the 12th day using the same technique because of a residual shunt. Another two patients had small residual shunts, which spontaneously resolved in 2 to 7 months. Left ventricular function evaluated 1 month after the operation utilizing cardiac catheterization revealed a mean stroke volume index of 40 (range: 32-45 ml/m2, and a mean left ventricular ejection fraction of 71 (range: 70-73%). An excellent functional outcome has thus been achieved with the reconstruction of post-infarction ventricular septal defects using a single pericardial patch.

[Reoperation for complications of mitral valve surgery].

Among the early postoperative complications of mitral valve surgery. Mitral Regurgitation (MR) is one of the most dangerous complications, which has to be diagnosed and to be treated as early as possible. Four reoperations-two of which are of paraprosthetic regurgitation, and two of recurrent MR after mitral valve plasty-are reported. Their clinical courses and transesophageal echocardiographic findings are shown. And the differences their MR has when it is compared with the physiologic MR of the prosthetic valve and with the acceptable MR after mitral valve plasty are described.

[Safe and accurate coronary artery bypass grafting: combined use with single aortic clamp and retrograde coronary perfusion].

Neurological injury following myocardial revascularization may result from embolization of atheromatous debris from clamping the diseased aorta. The hazards of manipulating and clamping the aorta has been reported in some literatures. The proximal anastomoses with partial occluding clamp is conventional technique, but it may cause neurological injury, aortic tear or traumatic laceration. We developed a technique for coronary bypass grafting with single aortic cross clamp and combined antegrade/retrograde infusion of cardioplegia. Our method allows accurate performance of the proximal anastomosis without partial clamping and adequate protection of myocardium.

[Comparative study of CABG following PTCA and PTCA following CABG].

Forty-five cases of PTCA with prior CABG and 14 cases of CABG with prior PTCA were studied on their pathogenesis and results. Ninety-four PTCA procedures were performed electively after CABG on 45 patients and 145 lesions. Lesion success rates were 95.8% in stenosis and 55.6% in occluded vessel. The mortality was none and no emergent surgery was needed. Causes of PTCA following CABG included the progression of coronary artery disease (13.1%), graft stenosis (15.0%), graft occlusion (31.0%), incomplete revascularization (13.8%), restenosis after the initial procedure (26.2%). Especially percentage of the progression of coronary artery disease was increased with a lapse of time, and it was 31.7% in PTCA group over 3 years after CABG. Fourteen patients with prior PTCA were received CABG because of unsuccessful PTCA (50.0%), progression to LMCD (21.4%), restenosis (21.4%), and PTCA complication (7.1%). Both interventions were appropriate and useful to reduce the recurrent ischemia and cardiac events with each other. Myocardial revascularization should not be considered either PTCA or CABG, but PTCA and CABG. In conclusion the strategy that much more benefits can be obtained from supplementary use of PTCA and CABG makes it possible to reduce the mortality and morbidity associated with intervention.

[Surgical treatment of acquired tricuspid stenosis].

There is no definitive surgical procedure for acquired lesions of the tricuspid valve (TV). From Feb, 1978, through March, 1990, the surgical treatment for the organic lesions of TV was performed in 10 patients, repair in 6 and TV replacement in 4. TV was repaired by commissurotomy, annuloplasty or valvuloplasty, or combination of them. When residual significant tricuspid regurgitation (TR) and/or stenosis (TS) was detected by intraoperative pulsed Doppler echocardiography after reparative procedures, TV was replaced. Follow-up periods ranged from 1 to 12 years (mean, 45.3 months). There was no early death, and late death was noted in one patient 32 months after operation. Preoperatively, 7 patients were in NYHA class IV and 3 in class III. Out of survivors, 7 are in class I and 2 in class II because of progression of mitral stenosis or coronary artery disease. Following surgery, the patients exhibited significant decrease in the cardiothoracic ratio (69.3 +/- 7.2 to 56.9 +/- 6.4%; p less than 0.01) and in the mean right atrial pressure (11.4 +/- 3.6 to 8.6 +/- 3.1 mmHg; p less than 0.05). The postoperative right ventriculography showed mild to moderate TR in 3 of 6 patients who underwent TV repair. In conclusion, TV repair could be a reasonable procedure for the organic TV lesions, although careful follow-up is recommended for residual TR.

[Clinical application of total body retrograde perfusion to aortic dissection operation].

Open distal anastomosis technique proposed by Cooley facilitates operative management of aortic dissection. Usually this procedure is performed under circulatory arrest, therefore, prolonged management is hazardous to cerebral and visceral organ systems. We have utilized profound hypothermia with total body retrograde perfusion: 1) myocardial protection through coronary sinus, 2) cerebral perfusion through superior vena cava with pretreatment of cerebral protective drugs, 3) abdominal visceral perfusion through inferior vena cava). We have obtained good results with this method in consecutive four patients with aortic dissection.

[Tricuspid annuloplasty--modified technique].

A modification of the DeVega's tricuspid annuloplasty (TAP) in the treatment of tricuspid regurgitation (TR) is described. Using a double-ended 2-0 Ethibond suture buttered with a Teflon felt pledget, a double suture line is begun at the center of the annulus of the anterior tricuspid valve leaflet. The two suture lines 2 to 3 mm apart are run around the edge of the annular ring of the anterior and posterior tricuspid valve leaflets, going over the posteroseptal commissure by 1.5 cm. A tiny piece of Teflon felt is placed at the end of the sutures. The annulus is narrowed to sungly allow passage of a prove with a diameter of 28 or 30 mm. The tricuspid function is assessed by injecting saline into the right ventricle through the right atrium. Between March 1986 and July 1989, 28 patients with functional TR secondary to mitral valve diseases have been operated on by this technique. There are one early and one late deaths, none being related to tricuspid valve annuloplasty. All 26 survivors had a significant drop in right atrial pressure and an associated improvement in clinical status. Follow-up of the 27 patients who survived this TAP ranges from 2 to 38 months (mean 17 months). This annuloplasty is a safe, effective and readily teachable method for the surgical management of TR.

[Mitral valvuloplasty for mitral regurgitation due to torn chordae of the posterior leaflet].

Four patients with mitral regurgitation due to torn chordae of the mitral posterior leaflet underwent mitral valvuloplasty. Standard operation was McGoon's procedure which was sometimes combined with Kay's annulorrhaphy. There were no sign of mitral insufficiency and/or stenosis in all patients revealed by the post-operative cardiac catheterization and all of them are on good health. Then, our plans of mitral posterior valvuloplasty for mitral regurgitation are as follows: A resected area is not beyond 33% of the posterior leaflet and the target mitral valve area after valvuloplasty is 2.8 cm2.

[A case of successful surgery for atrial septal defect accompanied with angina pectoris].

A 59-year-old patient with atrial septal defect (ASD) and angina pectoris due to 99% stenosis of the anterior descending coronary artery (LAD) was successfully treated by closure of ASD and coronary artery bypass grafting (CABG) to the LAD #7 with a saphenous vein graft. Preoperatively, the right ventricle was overloaded with high pulmonary arterial pressure (52 mmHg) and anterior segmental wall motion of the left ventricle was reduced probably due to myocardial ischemia. Combined operation, ASD closure and CABG, made these impairments normal and resulted in disappearance of the chest pain and reinstatement to his former job.