Malignant fibrous histiocytoma of the heart complicated by factor XI deficiency in a Jehovah's Witness patient

We describe a 16-year-old girl with malignant fibrous histiocytoma (MFH) of the heart complicated by factor XI deficiency. The preoperative diagnosis was left atrial myxoma. We decided to perform the operation owing to a normal bleeding time. Operative findings suggested a malignant tumor. The patient was a Jehovah's Witness, and extensive excision was not performed because blood transfusion was not allowed. We resected as much of the tumor and left atrial appendage as possible. The pathologic diagnosis was MFH. Excessive bleeding was not observed during the operation. Bleeding time helps to determine whether a surgical procedure is indicated in patients with factor XI deficiency.     

Improvement of Liver Function After Surgery for Budd-Chiari Syndrome

Purpose We evaluated the relationship between liver histology and postoperative improvement of liver function after surgery for Budd-Chiari syndrome (BCS).Methods Over a period of 23 years, we operated on 46 patients with BCS by reconstructing the occluded inferior vena cava (IVC) and reopening as many occluded hepatic veins as possible. We divided the patients into a liver cirrhosis group (group I, n = 30) and a hepatic fibrosis or liver congestion group (group II, n = 16), and compared the ages, duration of illness, preoperative liver function, changes in liver function, and changes in esophageal varices (EV).Results There were no hospital deaths. In group I the patients were older, and the duration of illness was longer. The group I patients also had a lower thrombotest percentage and a higher serum ammonia. The indocyanine green clearance (ICG) test showed more remarkable improvement in liver function in group II. The rate of disappearance of EV was also higher in group II.Conclusion Surgery during the early stage of BCS is important in improving postoperative liver function.     

Prevention of postoperative paraplegia during thoracoabdominal aortic surgery

BACKGROUND: We present data showing the impact of sequential multisegmental aortic clamping accompanied by reimplantation of as many segmental arteries as possible on the prevention of postoperative paraplegia or paraparesis during thoracoabdominal aortic graft replacement. METHODS: Since 1987 we have performed graft replacements in 51 individuals undergoing thoracoabdominal aortic surgery using the technique of normothermic partial bypass with sequential multisegmental aortic clamping. The procedure was performed emergently in 10 patients and electively in 41 patients. The patients ranged in age from 22 to 82 years (mean, 57.6 +/- 13.8 years). Indications for surgery included dissecting thoracoabdominal aortic aneurysm (n = 19) and nondissecting thoracoabdominal aortic aneurysm (n = 32). The extent of aneurysm was Crawford type I in 19 patients, type II in 7 patients, type III in 12 patients, and type IV in 13 patients. Along the entire extent of aneurysm to be replaced, we reimplanted as many of the patent segmental arteries as feasible. RESULTS: Five patients died during hospitalization, for an in-hospital mortality rate of 9.8%. The number of aortic clampings per patient ranged from one to five (median, three). A total of 124 segmental arteries were reimplanted in 44 (86.3%) of 51 patients. Of the 124 arteries, 90 (72.6%) were distributed between T9 and L2. Postoperative paraplegia or paraparesis did not develop in any of the patients. CONCLUSIONS: Our results demonstrate that extensive reimplantation of segmental arteries using sequential multisegmental aortic clamping, accompanied by adequate intraoperative distal aortic perfusion, is effective in preventing spinal cord ischemia.     

Surgical treatment of aortic arch aneurysm combined with coronary artery stenosis.

OBJECTIVE: We present operative results of aortic arch aneurysm associated with coronary artery stenosis, and evaluate the operative risk of graft replacement of the aortic arch and concomitant coronary artery bypass grafting (CABG). PATIENTS AND METHODS: From January 1991 to December 2001, we treated 16 patients with aortic arch aneurysm and coronary artery stenosis. The patients, 3 women and 13 men (study group) ranged from 58 to 79 years of age, average 68.1 5.3 years. With the aid of deep hypothermic cardiopulmonary bypass, we performed graft replacement of the aortic arch aneurysm and concomitant CABG. We bypassed 31 coronary arteries. The bypass grafts included saphenous vein (n=16), left internal thoracic artery (n=4), right internal thoracic artery (n=1), right gastroepiploic artery (n=5) and inferior epigastric artery (n=2). The number of bypassed coronary arteries per patient ranged from 1 to 3, average 2.1 0.8/patient. A comparative study was performed between the study group and a control group of patients (n=39) who had undergone only graft replacement of the aortic arch. RESULTS: There was no significant difference between the two groups regarding: operation time, cardiopulmonary bypass time, cardiac arrest time, intraoperative bleeding volume, and early mortality rate. However, in the patients (n=4) of the study group who had undergone total arch graft replacement with three vessel CABG, the cardiopulmonary bypass time was significantly longer than that of the patients in the control group who underwent total arch graft replacement (n=19, P<0.05). Two of the 16 study group patients died in the early postoperative period, resulting in 12.5% early mortality rate. In the control group, four of 39 patients (10.3%) died in the early postoperative period. CONCLUSIONS: CABG combined with graft replacement of the aortic arch does not increase operative risk when the number of bypassed vessels is within two vessels, but may increase risk when three or more vessels are bypassed.     

Evaluation of the results of the combined maze procedure for chronic atrial fibrillation with organic heart disease]

To examine the surgical indications and the results of the maze procedure, we reviewed 30 cases who underwent the maze procedure concomitantly with other open heart surgery from October 1995 to October 1997.: the average age was 60.9 years (37 to 75 years) and mean follow up period was 12.3 months (1 to 25 months). The modified maze procedure described by Kosakai and associates was applied in all patients. Twenty one patients (72.4%) regained atrial rhythm and eight patients (27.6%) sustained atrial fibrillation in the follow up periods. The left atrial diameter measured by echocardiography and cardiothoracic ratio were significantly larger in the latter group of patients, compared with those who recovered normal sinus rhythm (63.8 +/- 19.5 versus 51.2 +/- 7.8 mm and 67.7 +/- 8.1 versus 59.2 +/- 5.4%). In order to perform the combined maze procedure, cardiac arrest time and cardiopulmonary bypass time were extended for 56.9 minutes and 65.9 minutes, respectively, compared with the cases without a maze procedure. Four patients (brady atrial fibrillation 2, brady junctional rhythm 1, and complete AV block 1) required permanent pacemaker implantation. There was no operative death, but one patient who underwent the maze procedure and AVR + MAP + TAP died after 4 months due to pulmonary infection, sepsis and multiple organ dysfunction. This patient had shown low output syndrome for 3 days postoperatively. Having considered the data that the preoperative ejection fraction was 51%, cardiopulmonary bypass and cardiac arrest time were 200 min and 165 min respectively, occurrence of low cardiac output had been influenced by prolonged aortic cross-clamp. No late deaths have been observed in follow up period. In conclusion, maze procedure should not be performed in patients who have enlarged left atrium or complex cases requiring relatively longer operative time.     

An evaluation of cases carried out by both root reconstruction using carrel patch method and concomitant procedure

During the past 7 years from January 1991 to November 1997, we experienced 31 cases of aortic root reconstruction utilizing Carrel patch method. Concomitant procedure were performed in 9 cases of them due to another cardiovascular disease. Complicated cardiovascular disease included 3 cases of ischemic heart disease, 3 cases mitral regurgitation and one case of Aortic arch aneurysm. Several concomitant procedures were performed; 5 cases of CABG, 2 cases of mitral annuloplasty, one case of CABG with mitral valve replacement and one case of aortic arch replacement. The mean extra corporeal circulation time was 190.6 ± 39.3 minutes and aortic clamp time was 147.8 ± 34.2 minutes in these 9 cases. There were no significant differences of operative results between the simple root reconstruction group and the concomitant procedure group. We concluded that the aortic root reconstruction using the Carrel patch method has few complications. Although further long-term follow-up is required, our experiences suggest that the aortic root reconstruction with the concomitant procedure can be carry out safely with the aid of appropriate assistance method.     

Surgical treatment of Budd-Chiari syndrome induced by Behcet's disease.

OBJECTIVE: Although Budd-Chiari syndrome in Japanese is usually chronic, and of unknown etiology and idiopathic, Behcet's disease is rare as an underlying disorder of Budd-Chiari syndrome in Japanese. To clarify the Behcet-induced Budd-Chiari syndrome, the clinical course and pathologic findings of patients with Behcet-induced Budd-Chiari syndrome were compared with those of patients with idiopathic Budd-Chiari syndrome. PATIENTS AND METHODS: We treated 45 patients (15 women and 30 men) with our devised surgical procedure. With normothermic partial bypass, the occluded vena cava and hepatic veins were reopened. The age of the patients ranged from 24 to 76 years (mean, 48.9 13.0 years). In two patients, Budd-Chiari syndrome was induced by Behcet's disease (Behcet group). The other 43 patients (control group) had no distinct underlying disorder. The Behcet group was compared to the control group with regards to (1) onset of symptoms and duration of illness prior to medical treatment, (2) preoperative laboratory data including liver function, (3) intraoperative findings, (4) microscopic findings of liver tissue, and (5) postoperative course. RESULTS: (1) In the Behcet group, duration of illness from diagnosis to surgical treatment was markedly shorter (P=0.027, 8.5 months vs. 10.1 10.6 years). (2) The preoperative laboratory data of liver function were similar in both groups with moderately impaired hepatic function. (3) The Behcet group had no patent hepatic vein (P=0.025 vs. 1.22 0.57). (4) Microscopic examination of the liver tissue showed liver cirrhosis or liver fibrosis in the control group, and centrilobular marked congestion only in the Behcet group. (5) During hospitalization, one patient of the control group died due to preoperative severe hepatic failure. One patient with Behcet's disease underwent reoperation due to reocclusion by Behcet-induced vasculitis, and the other died of peritonitis by intestinal Behcet's disease. CONCLUSION: In Budd-Chiari syndrome in Japanese, the Behcet-induced Budd-Chiari syndrome had an acute clinical course, and its postoperative prognosis depends on the prognosis of the Behcet's disease.     

Myocardial protective effect of hypothermia during extracorporeal circulation -- by quantitative measurement of myocardial oxygen consumption -.

OBJECTIVE: To evaluate the effect of systemic temperature on myocardial protection during extracorporeal circulation, we quantitatively evaluated the relationship between myocardial oxygen consumption and rectal temperature. MATERIALS AND METHODS: Myocardial oxygen consumption during cardiac arrest was calculated via blood gas analysis of venous blood samples collected from the coronary sinus. The rectal temperatures of the patients during extracorporeal circulation ranged from 16.0 degrees C to 33.5 degrees C. The patients were classified into three groups according to their rectal temperature: group I (n=10; rectal temperature: 20.3+/-1.80 degrees C), group II (n=24; rectal temperature: 29.4+/-0.97 degrees C), and group III (n=29; rectal temperature: 31.7+/-0.72 degrees C). The myocardial oxygen consumption of each group was then compared. RESULTS: The average of the myocardial oxygen consumption of all cases was 62.5+/-64.0 O(2) ml/min/100 mm(3) left ventricle volume, and the averages of the individual groups were 26.9+/-28.8 in group I, 72.2+/-71.8 in group II, and 69.3+/-62.6 in group III. There was a significant difference in the oxygen consumption between group I and the other two groups. There was a positive correlation between the rectal temperature and myocardial oxygen consumption, as reflected in the following formula: Y=-0.3 x X +1.10 x X(2)-0.02 x X(3) (Y, myocardial oxygen consumption; X, rectal temperature; R(2)=0.533; P<0.0001). CONCLUSION: This study suggested that rectal temperature below 22.5 degrees C is advantageous due to the resultant myocardial protection such hypothermia affords.     

Selective visceral perfusion during thoracoabdominal aortic aneurysm repair.

PURPOSE: To evaluate the effectiveness of selective visceral perfusion during repair of an thoracoabdominal aortic aneurysm (TAAA), we compared the postoperative renal and hepatic functions (blood urea nitrogen, serum creatinine, total bilirubin, glutamate pyruvate transaminase) between the two groups with and without perfusion. PATIENTS AND METHODS: We operated on 52 patients with TAAA. Among them, the visceral vessels were reconstructed in 22 patients with selective visceral perfusion and in 12 patients without perfusion. The average selective perfusion time was 49.5+/-25.5 min. in the celiac and superior mesenteric arteries and 32.8+/-18.8 min. in the renal arteries. The average perfusion flow rate per each visceral vessel was 155.4+/-97.4 ml/min. RESULTS: There were five hospital deaths. There was no significant difference between the groups in the postoperative value of four factors. The selective perfusion time for vessel reconstruction in the selective visceral perfusion group was significantly longer than the arterial clamp time for vessel reconstruction in the non-perfusion group (49.5+/-25.5 min. vs. 25.6+/-13.4 min.). CONCLUSION: Our selective visceral perfusion method is not only beneficial for organ protection, but also provides us with the necessary time to reimplant the visceral as well as intercostal or lumbar arteries.     

One-stage operation for descending thoracic aortic aneurysm and left lung cancer: a case report.

We performed concomitant graft replacement for descending thoracic aortic aneurysm and pulmonary resection for squamous cell carcinoma of the left upper lobe in a 79-year-old man. The tumor reached the parietal pleura. No distance metastasis was found, and the tumor was diagnosed preoperatively as a stage IIB (N0, M0, T3) tumor. The descending thoracic aortic aneurysm was saccular, with greatest diameter being 55 mm, and extending from TH5 to TH8. A left upper lobectomy was performed, and after irrigation with a large volume of saline diluted with povidone iodine, graft replacement for the aortic aneurysm was performed under femoro-femoral partial bypass. To prevent postoperative graft infection, the greater omentum was dissected and placed over the resected pulmonary hilum and the graft. The patient's postoperative course was uneventful. There was no sign of infection, and the patient was discharged 1 month after surgery. Artificial graft wrapping with the greater omentum was useful for the prevention of the postoperative graft infection in this case of surgical treatment of lung cancer and descending thoracic aortic aneurysm.