Ritsu Matsukawa

Mortality And Cerebral Outcome In Patients Who Underwent Aortic Arch Operations Using Deep Hypothermic Circulatory Arrest With Retrograde Cerebral Perfusion: No Relation Of Early Death, Stroke, And Delirium To The Duration Of Circulatory Arrest

OBJECTIVE Our goal was to investigate factors for mortality and cerebral outcome in patients with aneurysm of the aortic arch. METHODS From 1993 to 1996, 148 patients with aortic arch aneurysm underwent operations involving deep hypothermic circulatory arrest with retrograde cerebral perfusion. Age was 63.9 +/- 11.6 years (mean +/- standard deviation) and 52 patients were older than 70 years. Twenty-eight had acute aortic dissection. Twelve had ruptured aneurysms. Fourteen had redo operations. Seventy had aortic dissection. The aneurysms were caused by atherosclerosis in 123 patients and by other causes in 25. Median sternotomy was used in 92 and left thoracotomy in 56. Twenty-eight patients underwent replacement of the ascending aorta to the proximal arch, 62 had total arch replacement, 38 had distal arch replacement, 12 had simultaneous replacement of the distal arch and the descending aorta or thoracoabdominal aorta, and 8 had patch repair. RESULTS Fifteen (10.1%) early deaths occurred. New stroke occurred in six (4.0%) patients and transient delirium in 37 (25.0%). The duration of deep hypothermic circulatory arrest plus retrograde cerebral perfusion was 49 +/- 17 minutes, and it was more than 60 minutes in 36 patients. Patients awoke 7.5 +/- 8.2 hours after the operation. Logistic regression analysis demonstrated that risk factors for mortality were ruptured aneurysm, chronic obstructive pulmonary disease, arterial cannulation in the ascending aorta, and stroke. Risks for stroke were ruptured aneurysm and replacement of the distal arch. Risks for delirium were age older than 70 years and atherosclerotic aneurysm. Duration of circulatory arrest plus cerebral perfusion did not correlate with length of time before the patient regained consciousness. No difference was found in mortality, stroke, and delirium between patients with and those without more than 60 minutes of circulatory arrest and cerebral perfusion. CONCLUSION Prolonged (> 60 minutes) deep hypothermic circulatory arrest with retrograde cerebral perfusion was not a risk factor for mortality and stroke in patients who underwent surgery for aneurysms of the aortic arch. However, the prevalence of transient delirium necessitates further investigations.

Mutations in a TGF-β Ligand, TGFB3, Cause Syndromic Aortic Aneurysms and Dissections

Background Aneurysms affecting the aorta are a common condition associated with high mortality as a result of aortic dissection or rupture. Investigations of the pathogenic mechanisms involved in syndromic types of thoracic aortic aneurysms, such as Marfan and Loeys-Dietz syndromes, have revealed an important contribution of disturbed transforming growth factor (TGF)-β signaling. Objectives This study sought to discover a novel gene causing syndromic aortic aneurysms in order to unravel the underlying pathogenesis. Methods We combined genome-wide linkage analysis, exome sequencing, and candidate gene Sanger sequencing in a total of 470 index cases with thoracic aortic aneurysms. Extensive cardiological examination, including physical examination, electrocardiography, and transthoracic echocardiography was performed. In adults, imaging of the entire aorta using computed tomography or magnetic resonance imaging was done. Results Here, we report on 43 patients from 11 families with syndromic presentations of aortic aneurysms caused by TGFB3 mutations. We demonstrate that TGFB3 mutations are associated with significant cardiovascular involvement, including thoracic/abdominal aortic aneurysm and dissection, and mitral valve disease. Other systemic features overlap clinically with Loeys-Dietz, Shprintzen-Goldberg, and Marfan syndromes, including cleft palate, bifid uvula, skeletal overgrowth, cervical spine instability and clubfoot deformity. In line with previous observations in aortic wall tissues of patients with mutations in effectors of TGF-β signaling (TGFBR1/2, SMAD3, and TGFB2), we confirm a paradoxical up-regulation of both canonical and noncanonical TGF-β signaling in association with up-regulation of the expression of TGF-β ligands. Conclusions Our findings emphasize the broad clinical variability associated with TGFB3 mutations and highlight the importance of early recognition of the disease because of high cardiovascular risk.

Elephant trunk procedure for surgical treatment of aortic dissection

BACKGROUND In surgical intervention for aortic dissection, a highly radical operation can be performed by distal anastomosis with a true lumen resulting in thrombotic closure of the dissecting lumen. In this anastomosis, the elephant trunk procedure, in which a graft is inserted into the distal true lumen, prevents blood flow leakage into the dissecting lumen at the anastomosis site and also strengthens this area. METHODS We performed this procedure in 15 patients (8 men and 7 women). Acute aortic dissection was observed in 9 patients and chronic dissection in 6. Stanford type A dissection was diagnosed in 10 patients and type B in 5. RESULTS Graft replacement of the ascending aorta and total aortic arch was performed in 10 patients and descending aortic replacement in 5. A graft with a diameter of 16 to 24 mm was inserted into the true lumen of the descending aorta, and the false lumen was closed. Subsequently, distal anastomosis was performed on the true lumen. There were two hospital deaths. Postoperative digital subtraction angiography showed good results in living patients, and computed tomographic scanning showed thrombotic closure in the dissecting lumen of the descending aorta. CONCLUSIONS The elephant trunk procedure is useful for closing the false lumen of the distal aorta.

Transbrachial arterial insertion of aortic occlusion balloon catheter in patients with shock from ruptured abdominal aortic aneurysm.

OBJECTIVE Of 125 surgical patients with abdominal aortic aneurysm (AAA) treated from 1999, 11 patients with deep shock from ruptured AAAs who underwent aortic occlusion balloon catheter (AOBC) insertion before laparotomy were studied. METHODS With the patients under local anesthesia, the brachial artery was exposed and the balloon catheter was inserted into the thoracic aorta. The balloon was inflated halfway and pulled back gently to the orifice of the left subclavian artery, and was advanced with the aid of blood flow down to the abdominal aorta. After full inflation of the balloon, the catheter was pulled until the balloon was fixed at the proximal shoulder of the AAA. RESULTS AOBC insertion was completed within 16.1 +/- 5.1 minutes. Systolic blood pressure at presentation was 84.1 +/- 31.7 mm Hg, deteriorated to 60.9 +/- 15.4 mm Hg on arrival in the operating room, and increased significantly (P <.0001) to 123.4 +/- 25.3 mm Hg after AOBC insertion. The balloon burst in three patients. Embolic complications were observed in two patients. There were three deaths, two associated with the balloon bursting. In nine patients whose shock was successfully controlled by AOBC, operative mortality was 11%. CONCLUSION Transbrachial arterial insertion of an AOBC may be useful to ameliorate hemorrhagic shock in patients with ruptured AAAs.

Repair for aneurysms of the entire descending thoracic aorta or thoracoabdominal aorta using a deep hypothermia

OBJECTIVE Replacement of the entire descending aorta or of the thoracoabdominal aorta still has a significant risk for postoperative paraplegia. Surgical strategies using a deep hypothermia to protect the spinal cord or viscera are discussed. METHODS From April 1994, 25 patients underwent graft replacement of the entire descending aorta (13 patients) or thoracoabdominal aorta (12 patients) using a deep hypothermia. Five patients had atherosclerotic aneurysms and 20 had aortic dissection. There were 20 males and 5 females, whose age ranged from 26 to 72 years old, 47 years old in average. Surgery consisted with proximal anastomosis using deep hypothermia (18 degrees C) with retrograde cerebral perfusion by elevating central venous pressure to 20 mmHg, reconstruction of the intercostal arteries, and distal open anastomosis, while perfusing the brain and heart. Proximal open anastomosis was used with retrograde cerebral perfusion technique in 18 patients. Averaged number of reconstructed intercostal arteries was 2.1 for each patient. RESULTS No early mortality was found and one patient died of respiratory failure 6 months after surgery. One patient had a postoperative stroke and one had a delayed onset of paraplegia 2 days after operation. The cause of paraplegia was secondary hypoxemia and hypotension due to pneumonia. CONCLUSION Utilization of the deep hypothermia in surgery for aneurysms of the entire descending aorta or of the thoracoabdominal aorta provided an adequate protection of the spinal cord as well as the abdominal viscera, eliminated clamp injury or cerebral embolization of debris or thrombi, and afforded excellent surgical exposures.

Neurological Outcomes After Immediate Aortic Repair for Acute Type A Aortic Dissection Complicated by Coma

Background— Management of acute type A aortic dissection (AADA) complicated by coma remains controversial. We analyzed our experience in managing AADA complicated by coma to determine the relationship of duration of preoperative coma to postoperative neurological recovery. Methods and Results— Between September 2003 and October 2010, 181 patients with AADA were treated, including 27 presenting with coma (Glasgow Coma Scale <11) on arrival. Twenty-one patients were repaired immediately (immediate group); time from onset of symptoms to operating room was <5 hours. For brain protection, deep hypothermia with antegrade cerebral perfusion was used, and postoperative therapeutic hypothermia with magnesium treatment was performed. Six patients initially were managed medically, and 3 of them were followed by eventual repair because time from onset was >5 hours (delayed group). The preoperative National Institutes of Health Stroke Scale score was 31.4±6.6 in the immediate group and 28.3±9.5 in the delayed group. Hospital mortality was 14% in the immediate group and 67% in the delayed group. Full recovery of consciousness was achieved in 86% of patients in the immediate group and in 17% in the delayed group. In immediate group, the postoperative National Institutes of Health Stroke Scale score significantly improved to 6.4±8.4, cumulative survival rate was 71.8% in 3 years, and independence in daily activities was achieved in 52% (11/21). Conclusions— Aortic repair, if performed immediately from the onset of symptoms, showed satisfactory recovery of consciousness and neurological function in patients with AADA complicated by coma. In this patient population, immediate aortic repair is warranted.

Operation for chronic pulmonary thromboembolism accompanied by thrombophilia in 8 patients

BACKGROUND Medical therapy for chronic pulmonary thromboembolism is limited, and surgical treatment has become more frequent recently. We have performed pulmonary thromboendarterectomy on 8 patients with chronic pulmonary thromboembolism accompanied by thrombophilia. METHODS The patients were 6 men and 2 women aged 21 to 56 years (mean, 35 years). Five patients had antiphospholipid syndrome, 2 had protein C deficiency, and 1 had congenital antithrombin III deficiency. The preoperative condition was New York Heart Association functional class III in 5 and class IV in 3. Hypoxemia, marked pulmonary hypertension (mean pulmonary artery pressure, 47+/-6.7 mm Hg), and low cardiac output were observed in all patients. After a median sternotomy, deep hypothermia was induced using a cardiopulmonary bypass, and pulmonary thromboendarterectomy in the bilateral pulmonary arteries was performed under intermittent circulatory arrest. RESULTS There were no operative deaths. Long-term respiratory management was needed postoperatively by 3 patients. In the remaining 5 patients, no reperfusion injury was observed. The arterial blood oxygen concentration improved, and the mean pulmonary pressure decreased to 16+/-5.5 mm Hg. The cardiac output also increased, and New York Heart Association functional class improved to I in 4 and II in 4 patients. CONCLUSION Pulmonary thromboendarterectomy under deep hypothermic intermittent circulatory arrest was effective for chronic pulmonary thromboembolism accompanied by thrombophilia for which medical treatment is of limited value.

Impact of Controlled Pericardial Drainage on Critical Cardiac Tamponade With Acute Type A Aortic Dissection

Background— Cardiac tamponade is associated with fatal outcomes for patients with acute type A aortic dissection, and the presence of cardiac tamponade should prompt urgent aortic repair. However, treatment of the patient with critical cardiac tamponade who cannot survive until surgery remains unclear. We analyzed our experience of controlled pericardial drainage (CPD) managing critical cardiac tamponade. Methods and Results— Between September 2003 and May 2011, 175 patients with acute type A aortic dissection were treated surgically, including 43 (24.6%) who presented with cardiac tamponade on arrival. Eighteen patients, who did not respond to intravenous volume resuscitation, underwent CPD in the emergency department. An 8F pigtail drainage catheter was inserted percutaneously, and drainage volume was controlled by means of several cycles of intermittent drainage to maintain blood pressure at ≈90 mm Hg. After CPD, all of the patients were transferred to the operating room, and immediate aortic repair was performed. Systolic blood pressure before CPD was 64.3±8.2 mm Hg and elevated significantly in all of the cases after CPD. Systolic blood pressure after CPD was 94.8±10.5 mm Hg, and increase in systolic pressure was 30.5±11.7 mm Hg. Total volume of aspirated pericardial effusion was 40.1±30.6 mL, and 10 patients required only ⩽30-mL aspiration volume. All of the patients underwent aortic repair successfully. In-hospital mortality was 16.7%; however, there was no complications or mortality related to CPD. Conclusions— Preoperative pericardial drainage with control of volume is a safe and effective procedure for acute type A aortic dissection complicated by critical cardiac tamponade. In our patient population, timely controlled pericardial drainage is warranted.

Long-term outcomes after immediate aortic repair for acute type A aortic dissection complicated by coma

OBJECTIVES The management of acute type A aortic dissection complicated by coma remains controversial. We previously reported an excellent rate of recovery of consciousness provided aortic repair was performed within 5 hours of the onset of symptoms. This study evaluates the early and long-term outcomes using this approach. METHODS Between August 2003 and July 2013, of the 241 patients with acute type A aortic dissection brought to the Japanese Red Cross Kobe Hospital and Hyogo Emergency Medical Center, 30 (12.4%) presented with coma; Glasgow Coma Scale was less than 11 on arrival. Surgery was performed in 186 patients, including 27 (14.5%) who were comatose. Twenty-four comatose patients underwent successful aortic repair immediately (immediate group). Their mean age was 71.0 ± 11.1 years, Glasgow Coma Scale was 6.5 ± 2.4, and prevalence of carotid dissection was 79%. For brain protection, deep hypothermia with antegrade cerebral perfusion was used, and postoperative induced hypothermia was performed. Neurologic evaluations were performed using the Glasgow Coma Scale, National Institutes of Health Stroke Scale, and modified Rankin Scale. RESULTS In the immediate group, the time from the onset of symptoms to arrival in the operating theater was 222 ± 86 minutes. Hospital mortality was 12.5%. Full recovery of consciousness was achieved in 79% of patients in up to 30 days. Postoperative Glasgow Coma Scale and National Institutes of Health Stroke Scale improved significantly when compared with the preoperative score (P < .05), and postoperative activities of daily living independence (modified Rankin Scale <3) was achieved in 50% of patients. The mean follow-up period was 56.5 months, and the cumulative survival was 48.2% after 10 years. Cox proportional hazards regression analysis indicated that immediate repair (hazard ratio, 4.3; P = .007) was the only significant predictor of postoperative survival over a 5-year period. CONCLUSIONS The early and long-term outcomes as a result of immediate aortic repair for acute type A aortic dissection complicated by coma were satisfactory.

Long-term results of patch repair for saccular aneurysms of the transverse aortic arch.

OBJECTIVE Long-term results of patch repair in patients with a saccular aneurysm of the aortic arch were investigated. PATIENTS From December 1984, 43 patients with a saccular aneurysm of the arch underwent patch repair. Indications for patch repair were determined as orifice diameter of aneurysm being less than 1/3 of the total circumference of the aorta. METHOD Midsternotomy was used in 38 patients, and left thoracotomy in five. Selective cerebral perfusion was used in 28 patients, deep hypothermic circulatory arrest with retrograde cerebral perfusion in eight during the last 3 years, and partial cardiopulmonary bypass in seven. RESULTS There were five (11.6%) early deaths, and causes were respiratory failure in two patients, low cardiac output in two, and gastrointestinal bleeding in one. Stroke was found in three patients (6.9%). During follow-up, seven patients died, two due to rupture of a residual or pseudoaneurysm, one due to reoperation of pseudoaneurysm, one due to stroke, two due to respiratory failure, and one due to unknown cause. Postoperative survival, including early death, was 69.3% at 5 years and 43.3% at 9 years. Aortic reoperation was done in three patients with a pseudoaneurysm formation and two survived. Freedom from reoperation was 91.7% at 5 years and 38.2% at 9 years. Event free ratio was 79.3 +/- 9.8% at 5 years and 37.6 +/- 18.6% at 9 years. CONCLUSION Because of a high incidence of pseudoaneurysm or residual aneurysms after patch repair for a saccular aneurysm of the aortic arch, strict criteria for the patch repair should be applied or graft replacement of the aorta is recommended.