Diffuse alveolar damage in a patient with Epstein Barr virus (EBV)-positive diffuse large B-cell lymphoma,severely low serum folate, and megaloblastic anemia: a case report of autopsy.

We report a case of a 49-year-old Japanese man, who was admitted to our hospital because of severe dyspnea. He was found to have severely low serum folate and megaloblastic anemia, which was at first suspected to cause his dyspnea. It was assumed that severely low serum folate might be related to his malnutrition, probably caused by habitual alcohol consumption. He died in several days because of acute respiratory distress syndrome. Autopsy revealed diffuse alveolar damage (DAD) in the lungs and systemic lymph node swelling by EBV-positive diffuse large B-cell lymphoma, in addition to megaloblastic anemia in the bone marrow. Together with histological hemophagocytosis and high level of serum iron and ferritin, DAD was considered to be caused by hypercytokinemia triggered by the presence of EBV-positive diffuse large B-cell lymphoma. On the other hand, pathological findings suggestive of habitual alcohol consumption were not apparent. We considered that low serum folate in this case was not by the low intake but by increased consumption of folate by rapid progression of the lymphoma.

Total arch replacement with long elephant trunk anastomosed at the base of the innominate artery: a single-centre longitudinal experience.

OBJECTIVE: Total arch replacement, with a long elephant trunk (ET) anastomosed at the base of the innominate artery using an undersized graft, is performed for a variety of arch aneurysms. We investigated the long-term clinical outcomes of this procedure, as well as its long-term effectiveness for preventing retrograde flow into the aneurysm and further dilation of the descending aorta. METHODS: We treated 127 consecutive patients with an arch aneurysm, who were divided into two groups according to the diameter of the descending aorta at the Th6-Th8 thoracic vertebral level: 35 mm or less (Single-ET, n = 94) and >35 mm (Staged-ET, n = 33). The graft diameter was undersized by 10-20% of the distal aortic diameter. ET length was determined by preoperative computed tomography (CT) to locate the distal end at Th6-Th8. Thrombosis around the ET and the descending aorta diameter around the distal end of the ET were evaluated using CT. RESULTS: Two patients (1.6%) died within 30 days, while seven (5.5%) died in the hospital, three (2.4%) had a new stroke, three (2.4%) had permanent paraplegia and one (0.8%) had paraparesis. CT demonstrated complete thrombosis of the perigraft space around the ET in 81 patients (86%) in the Single-ET group and 11 (33%) in the Staged-ET group within 1 month after surgery, but not in the remaining 35 patients. Twenty-seven of the 35 patients without complete thrombosis underwent a subsequent second-stage operation. In those, the descending aorta showed no further dilation around the distal end of the ET, while new-onset perigraft perfusion occurred in two patients in the Single-ET group at 14 and 126 months, respectively. Overall survival was 89, 86, 78 and 74% at 1, 3, 5 and 7 years, respectively. CONCLUSIONS: Our operative strategy for extensive thoracic aortic aneurysms using a long ET technique yielded satisfactory short- and long-term outcomes.

Optimal graft diameter and location reduce postoperative complications after total arch replacement with long elephant trunk for arch aneurysm.

OBJECTIVE: Total arch replacement with an elephant trunk is a standard treatment for arch aneurysm, but serious complications, such as paraplegia and peripheral embolization caused by flapping of the elephant trunk, remain. Moreover, dilation of the descending aorta and retrograde flow into the peri-graft space at the distal elephant trunk are frequent problems. We hypothesized that optimal graft diameter and location would reduce complications after total arch replacement with a long elephant trunk by achieving complete thrombosis and minimal dilation of the descending aorta around the elephant trunk. METHODS: We treated 65 patients with arch aneurysm by total arch replacement with a long elephant trunk anastomosed at the base of the innominate artery. The graft diameter was undersized (10%-20% of the distal aorta's diameter). Elephant trunk length was determined by preoperative computed tomography to locate the distal end at Th6 to Th8. Thrombosis around the elephant trunk, diameter of the descending aorta, and distance between the descending aorta and the graft near the distal end of the elephant trunk were evaluated using computed tomography. RESULTS: The distal end of the elephant trunk was located at Th 8 ± 1. There were no operative deaths, 3 patients (5%) died in the hospital, and 3 patients (5%) experienced spinal cord injury, including 1 in whom permanent paraplegia developed. Computed tomography revealed complete thrombosis around the elephant trunk in 58 patients (89%). The descending aorta did not dilate further, and distance between the descending aorta and the graft progressively decreased. CONCLUSIONS: Optimal graft diameter and location minimized postoperative complications, with complete thrombosis and no dilation of the descending aorta around the long elephant trunk in most patients.

Arch aneurysm repair with long elephant trunk: a 10-year experience in 111 patients.

BACKGROUND: We studied the long-term outcome of arch aneurysm repair with a long elephant trunk (LET) anastomosed at the base of brachiocephalic artery. METHODS: Between 1998 and 2008, 111 patients underwent arch aneurysm repair with LET. A 4-branched graft was sutured to the sinotubular junction, the distal ascending aorta transected, and a LET inserted into the aortic arch while selective cerebral perfusion was maintained. The graft distal end was anastomosed to the LET, incorporating the distal ascending aorta, and arch vessels were anastomosed to graft branches. RESULTS: Concomitantly, 33 patients (30%) underwent other cardiac procedures, including 11 aortic root replacements. Two patients died (1.8%) within 30 days and 7 died (6.3%) after 30 days. Perioperative morbidity included 2 (1.8%) with stroke, 3 (2.7%) with paraplegia, and 1 (0.9%) with paraparesis. Postoperative computed tomography scans revealed complete aneurysmal thrombosis around the LET in 88 patients (79%), who were monitored without a second-stage procedure. Among 23 patients with incomplete thrombosis, 19 underwent a second-stage procedure to complete distal fixation of the LET. Overall survival was 88%, 83%, and 75%, at 1, 3, and 5 years after aneurysm repair with the LET. No aneurysm rupture or reexpansion occurred in patients with complete thrombosis. Four patients with incomplete thrombosis died of rupture before the second-stage procedure. CONCLUSIONS: Our results demonstrated safety and good durability of the LET technique and suggest that this technique is a simple and safe procedure that is applicable to a variety of arch aneurysms.

Elephant trunk anastomosis proximal to origin of innominate artery in total arch replacement.

PURPOSE: The purpose of this study was to describe our current technique for aortic arch replacement using a four-branched arch graft and a long elephant trunk. DESCRIPTION: Using our method, the ascending aorta is replaced with a four-branched Hemashield arch graft (Hemashield Platinum, Woven Double Velour [Boston Scientific Corp, Wayne, NJ]) while cooling the patient. When 25 degrees C is reached, selective cerebral perfusion is started and the elephant trunk is inserted under open distal conditions into the descending aorta using a catching catheter introduced through a femoral artery. A distal aortic anastomosis is then performed between the four-branched graft and distal aorta incorporating the elephant trunk at the base of the innominate artery. Arch vessels are reconstructed separately during rewarming. EVALUATION: Between October 1998 and December 2005, we performed the present technique in 52 patients with no operative deaths within 30 days after the procedure. The mean duration of hypothermic circulatory arrest was 24 +/- 5 minutes, whereas that of selective cerebral perfusion was 86 +/- 9 minutes. CONCLUSIONS: The technique described herein requires a shorter circulatory arrest time and is uniformly applicable to patients with extensive thoracic aortic aneurysms.

Simple and uniform technique for combined repair of aortic arch and root replacement.

We present herein a simple and uniform repair technique for combined aortic arch and root aneurysms. Our method is performed under an open distal procedure and includes selective antegrade cerebral perfusion, adequate myocardial preservation, a four-branched composite valve graft, and a long elephant trunk anastomosis proximal to the innominate artery. The technique was designed to reduce morbidity and mortality associated with aortic arch and root replacements, as well as allow for easier performance of subsequent downstream operations.

Single-stage repair of arch aneurysms with a long elephant trunk: medium-term follow-up of thromboexcluded aneurysms.

OBJECTIVES: The purpose of this study was to investigate the medium-term results of arch aneurysms repaired by total arch replacement with a long elephant trunk and to evaluate whether this technique requires a subsequent distal anastomosis at the descending aorta when complete aneurysmal thrombosis is achieved around a long elephant trunk. METHODS: From June 1999 through May 2005, 32 consecutive patients with arch aneurysms underwent total arch replacement with a long elephant trunk anastomosed at the base of the innominate artery. Postoperatively, aneurysm size was evaluated by means of serial computed tomographic scanning. RESULTS: None of the patients experienced a new stroke, although there was 1 (3%) hospital mortality. Computed tomographic scanning demonstrated complete thrombosis of the aneurysm in 29 (91%) patients within 1 month after surgical intervention, and 3 patients with incomplete thrombosis of the aneurysm underwent a subsequent distal anastomosis in the descending aorta. The 3-year survival rate was 87%, with no aneurysm rupture or sudden death. In the 29 patients who showed complete thrombosis of the aneurysm surrounding a long elephant trunk, serial computed tomographic scanning revealed a significant reduction in the size of the thrombosed aneurysm (81% at 1 year and 76% at 2 years after surgical intervention), and there was no case that showed expansion of the aneurysm. CONCLUSIONS: Arch aneurysms were repaired safely by means of total arch replacement with a long elephant trunk, and successful shrinkage of the arch aneurysm suggests that this technique does not require subsequent distal anastomosis and could turn the 2-stage elephant trunk procedure into a single-stage repair when complete aneurysmal thrombosis is achieved.

Evaluation of the mitral valve leaflet morphology after mitral valve reconstruction with a concept "coaptation length index".

BACKGROUND: In clinical settings, information on morphology of mitral valve leaflet after mitral valve reconstruction is limited. METHODS: Between January 1996 and June 2000, 36 patients underwent mitral valve repair for mitral regurgitation (MR). The etiology of mitral insufficiency was prolapse, dilated annulus, and ischemia. Ring annuloplasty was performed in all cases. Mitral valve short-axis dimension (MVd), vertical distance between annular line and closing point (Vd), coaptation length (CL), and coaptation length index (CLI) were measured by two-dimensional transesophageal echocardiography for the present 11 cases. RESULTS: In 11 cases, residual MR, using a scale from 0 to 4, was 0 in 5 patients, 1 in 4 patients, 2 in 2 patients whose etiology of regurgitation was cardiomyopathy. MVd and Vd decreased significantly (38.7+/- 6.2 to 27.0 +/- 5.6 mm, 10.1 +/- 7.7 to 6.5 +/- 4.6 mm, respectively). CL and CLI increased significantly (6.4 +/- 2.4 to 11.6 +/- 4.6 mm, 0.16 +/- 0.06 to 0.44 +/- 0.21, respectively). Among those indices, only CLI has a statistically significant negative correlation with the degree of residual MR. CONCLUSION: Mitral valve ring annuloplasty produces the morphologic change of the mitral apparatus, especially increase of CLI, which may be one of the main factors in regulation of regurgitation.

Evaluation of the mitral valve leaflet morphology after mitral valve reconstruction with a concept "coaptation length index".

BACKGROUND: In clinical settings, information on morphology of mitral valve leaflet after mitral valve reconstruction is limited. METHODS: Between January 1996 and June 2000, 36 underwent mitral valve repair for mitral regurgitation (MR). The etiology of mitral insufficiency was prolapse, dilated annulus, and ischemic. Ring annuloplasty was performed in all cases. Mitral valve short-axis dimension (MVd), vertical distance between annular line and closing point (Vd), coaptation length (CL), coaptation length index (CL/MVd) were measured by the two-dimensional transesophageal echocardiography for the present 11 cases. RESULTS: In 11 cases, residual MR, using a scale from 0 to 4, was 0 in 5 patients, 1 in 4 patients, 2 in 2 patients whose etiology of regurgitation was cardiomyopathy. MVd and Vd decreased significantly (38.7 +/- 6.2 to 27.0 +/- 5.6 mm, 10.1 +/- 7.7 to 6.5 +/- 4.6 mm, respectively). CL and CLI increased significantly (6.4 +/- 2.4 to 11.6 +/- 4.6 mm, 0.16 +/- 0.06 to 0.44 +/- 0.21, respectively). Among those index, only CLI have a statistically significant negative correlation with the degree of residual MR. CONCLUSION: The mitral valve ring annuloplasty produce the morphologic change of mitral apparatus, especially the increase of CLI, which may be one of the main factors in regulation of regurgitation.

Mycotic aneurysm of the infrarenal abdominal aorta infected by Clostridium septicum: a case report of surgical management and review of the literature.

We report a surgical case of mycotic aneurysm of the infrarenal abdominal aorta infected by Clostridium septicum. The patient was first treated with an in situ prosthetic graft replacement. When the infection recurred 5 weeks after the aortic surgery, the patient was successfully treated by transposition of rectus abdominis muscle flap around the graft. Only 19 cases of mycotic aneurysm or aortic dissection caused by Clostridium septicum have been reported. Ten of 12 patients who underwent vascular surgery survived, whereas all 7 patients who did not undergo surgery died. Surgical treatment should be undertaken since the surgical results seem satisfactory.

Usefulness of continuous air tonometry for evaluation of splanchnic perfusion during cardiopulmonary bypass.

Although gastric mucosal tonometry has been reported as a useful method to assess splanchnic perfusion during cardiovascular surgery, the conventional discontinuous method of tonometry (saline tonometry) was cumbersome and prone to systematic errors. A new automated system of air tonometry (Tonocap; Datex Ohmeda, Helsinki, Finland) allows for frequent (every 10 minutes) measurement of gastric regional CO2 (PrCO2) and may be more suitable as a monitoring system in cardiac patients. We evaluated the usefulness of continuous air tonometry as a marker of splanchnic perfusion during cardiopulmonary bypass (CPB). In 19 patients (53-79 years, mean 63 years) who underwent cardiovascular surgery under standard CPB with mild hypothermia (32 degrees C) from January 2001 to May 2002, the PrCO2 and calculated intramucosal pH (pHi) of gastric tonometry was monitored using Tonocap, and their relation to postoperative visceral organ function was evaluated. The pHi significantly increased after initiation of CPB from 7.32 +/- 0.07 to 7.43 +/- 0.10 (p < 0.05) and then consistently decreased in all patients to 7.39 +/- 0.09 at the end of CPB. The value of PrCO2 significantly (p < 0.01) correlated with the value of pHi. The lowest value of pHi during CPB was significantly related to blood urea nitrogen (r = -0.75, p < 0.05), serum creatinine (r = -0.78, p < 0.05), creatinine clearance (r = 0.68, p < 0.05) on postoperative day 1, and blood urea nitrogen (r = -0.84, p < 0.01) on day 3. In contrast, arterial blood lactate level, venous oxygen saturation, and routinely measured hemodynamics (e.g., pump flow, arterial pressure) during CPB were unrelated to the postoperative visceral organ function. These results suggest that continuous monitoring of gastric regional CO2 and pHi by air tonometry system is useful for the evaluation of splanchnic perfusion during CPB and may contribute to improve CPB technique by allowing the early detection of visceral malperfusion.

An alternative approach using long elephant trunk for extensive aortic aneurysm: elephant trunk anastomosis at the base of the innominate artery.

BACKGROUND: Although a staged elephant trunk procedure has been widely used, the early mortality of the first stage operation as well as the interval mortality between operations remains unsatisfactory. We developed an alternative elephant trunk procedure to reduce mortality and morbidity. METHODS AND RESULTS: Ascending aorta and arch vessels were minimally dissected. During systemic cooling, a four-branched arch graft with a sewing "collar" and a long "elephant trunk" was prepared. The ascending aorta was opened under selective brain perfusion with moderate hypothermia (25 degrees C), and the elephant trunk was then pulled down into the descending aorta using the catching catheter introduced via a femoral artery. The elephant trunk anastomosis using the collar was made at the base of the innominate artery. The arch vessels were divided and closed at aortic stump, and grafted separately as a consequence of the very proximal site for the elephant trunk anastomosis. Between October 1998 and September 2001, 17 patients, ranging in age from 25 to 79 years (mean 67 years) with extensive aortic aneurysm underwent this operation. Preoperative cardiac complications included coronary artery disease in 5, aortic regurgitation in 3, and 3 of these 8 patients had poor left ventricular function with an ejection fraction less than 40%. Nine patients underwent a second stage operation, in 1 of them the permanent elephant trunk procedure was initially attempted but the second stage procedure was done because of increasing endo-leakage. The mean interval between operations was 8 days (range 1 to 14 days) in the remaining 8 patients. In 5 of 6 patients who underwent the permanent elephant trunk procedure, a decrease in the size of the aneurysm based on thromboexclusion was observed using serial computed tomography scans. A single stage repair was performed in 1 patient. The 30-day survival rate of all operations was 100%, however, there was 1 in-hospital death (6%) after the second operation. There was no stroke, however, paraplegia occurred after the first operation in 1 patient (6%) of the in-hospital death. No new phrenic or recurrent laryngeal nerve palsy occurred as a result of surgery. CONCLUSIONS: The present technique using a modification of the elephant trunk technique for extensive aortic aneurysm provides acceptable mortality and morbidity. The present strategy would be an alternative for the standard elephant trunk procedure in some high-risk patients with advanced age and comorbidities.