Total arch replacement for coarctation of the aorta in an adolescent.

A 16-year-old boy suffered a subarachnoid hemorrhage and underwent open head surgery. He was subsequently diagnosed with coarctation of the aorta and referred to our hospital. The coarctation was at the distal transverse arch, just at the site of branching of the subclavian artery. Total arch replacement with selective cerebral perfusion was selected because of the short hypoplastic arch. The patient had an uneventful postoperative course, and was doing well 3 years after the surgery.

Constrictive pericarditis caused by immunoglobulin G4-related disease.

An 81-year-old man with congestive heart failure was admitted to our hospital. Computed tomography of the chest revealed pericardial thickening without calcification. Ultrasonic echocardiography and cardiac magnetic resonance imaging demonstrated diastolic dysfunction. Cardiac catheterization showed bilateral elevation of ventricular end-diastolic pressure with a square root sign. The preoperative serum immunoglobulin G4 (IgG4) level was abnormally high. Pericardiectomy and pericardiotomy were considered to be indicated and were performed. Histopathologic examination revealed tissue infiltration by IgG4-positive plasma cells, and constrictive pericarditis caused by IgG4-related disease was diagnosed. Postoperatively, the patient received additional corticosteroid therapy. The latest cardiac magnetic resonance image showed further improvement in diastolic function.

Successful endovascular treatment of a ruptured superior mesenteric artery in a patient with Ehlers‒Danlos syndrome.

The purpose of this study was to describe covered-stent treatment of a ruptured dissection of the superior mesenteric artery (SMA) in a patient with Ehlers‒Danlos syndrome. The patient was a 13-year-old girl initially presenting with abdominal pain. Dissection and rupture of the SMA were diagnosed on detailed examination. Conservative treatment was performed initially because open surgery was considered high risk. However, the abdominal pain recurred, and we decided to perform endovascular therapy. A coronary artery covered stent was placed in the true lumen to close the entry site of the dissection. The false lumen was obliterated using a post-dilation technique, completing treatment of the rupture. The patient recovered uneventfully after surgery. Classic-type Ehlers‒Danlos syndrome was diagnosed on the basis of physical findings and genetic analysis. The stent has remained adequately patent as of 2 years after surgery. This case report shows that dissection and rupture of the SMA can be treated successfully using a covered coronary artery stent in a patient with Ehlers‒Danlos syndrome.

Acute aortic dissection associated with sleep apnea syndrome.

BACKGROUND: Sleep apnea syndrome (SAS) is an independent risk factor for hypertension which is a major risk factor for acute aortic dissection. The purposes of this study were to assess the prevalence of SAS in patients with acute aortic dissection, delineate the characteristics of patients who have acute aortic dissection with SAS. METHODS: Of 95 consecutive patients with acute aortic dissection, 13 had episodes of sleep apnea and nocturnal hypoxemia. A portable sleep monitoring system was used to assess sleep status in the 13 patients. RESULTS: The SAS-positive group consisted of 12 patients (12.6%), 8 with type A dissection and 4 with type B dissection. Age was significantly lower in the SAS-positive group (47.2 ± 8.5 years) than in the SAS-negative group (64.9 ± 10.3 years)(p <0.001). The male:female ratio was significantly higher in the SAS-positive group than in the SAS-negative group (p <0.001). The body mass index was significantly greater in the SAS-positive group than in the SAS-negative group (p <0.001). All 12 patients in the SAS-positive group had hypertension. CONCLUSIONS: Patients who have acute aortic dissection with SAS are characterized by being tall, fat, and relatively young men with hypertension. Sleep apnea syndrome may be a risk factor for acute aortic dissection in middle-aged men.

Evaluation of the vertebrobasilar system in thoracic aortic surgery.

BACKGROUND: We evaluated the probability of vertebrobasilar system malperfusion due to occlusion of the left subclavian artery as assessed by preoperative magnetic resonance angiography in patients scheduled to undergo thoracic aortic surgery. METHODS: (Study 1) From January 2000 through March 2009, we studied variations of vertebral arteries in 301 patients scheduled to undergo thoracic aortic surgery. We classified vertebral artery variations into 3 categories according to the findings on preoperative magnetic resonance angiography: connection type, interrupted right vertebral artery, and interrupted left vertebral artery. (Study 2) From February 2007 through January 2010, we evaluated the cerebral complication in 41 patients who had occlusion of the left subclavian artery with a stent graft. RESULTS: (Study 1) On preoperative magnetic resonance angiography, the vertebral artery was classified as connection type in 247 patients, interrupted right vertebral artery in 34, and interrupted left vertebral artery in 20. (Study 2) We performed subclavian obstruction test, left-right subclavian artery bypass, or left subclavian artery-left common carotid artery bypass to the 3 patients with interrupted right vertebral artery, respectively. Forty patients (98%) out of 41 patients had no complication after occlusion of the left subclavian artery. CONCLUSIONS: Preoperative magnetic resonance angiography is useful for detection of the patients with high risk of vertebrobasilar system malperfusion due to occlusion of the left subclavian artery.

Fibromuscular dysplasia associated with simultaneous spontaneous dissection of four peripheral arteries in a 30-year-old man.

A 30-year-old man had a sudden bout of severe abdominal pain. An enhanced computed tomographic scan revealed dissections of the celiac artery, superior mesenteric artery, left renal artery, and right external iliac artery; stenosis of the right renal artery; and left kidney infarction. After careful evaluation, the patient was diagnosed with fibromuscular dysplasia (medial dysplasia), based on the findings obtained from the enhanced computed tomographic scan. This case is extremely rare because fibromuscular dysplasia occurred concurrently with simultaneous spontaneous dissections of four peripheral arteries in a young man.

Idiopathic left atrial appendage rupture.

Cardiac rupture is mainly caused by myocardial infarction or blunt chest trauma. We present a case of idiopathic left atrial appendage rupture.

A Case of LMT Reconstruction Using Superficial Femoral Artery Graft in the Patient with Type A Acute Aortic Dissection.

A 76-year-old woman underwent a Bentall procedure for acute aortic dissection. A dissection involving half of the proximal portion of the left main coronary artery trunk was confirmed. The dissected site was resected, and a section of the superficial femoral artery was harvested and used as an interposition graft between conduit and the residual left main trunk. Two years after surgery, the graft remained well patent. If the coronary dissection involves only the left main artery trunk, the superficial femoral artery should be used as an artery graft for the anatomical reconstruction, potentially leading to better early and late outcomes.

Risk analysis for hospital mortality in patients with acute type a aortic dissection.

BACKGROUND: Stanford type A acute aortic dissection is a fatal condition requiring emergency surgery. This study was designed to evaluate risk factors for hospital mortality in patients with Stanford type A acute aortic dissection. METHODS: We studied consecutive 301 patients (163 men and 138 women; mean age, 63.3 years) who underwent emergency surgery for Stanford type A acute aortic dissection from January 1997 through December 2007. The subjects were divided into two groups: patients who were discharged from the hospital, and those who died during hospitalization. Preoperative and operative clinical factors were compared between the groups. RESULTS: Overall, 41 patients (13.6%) died during hospitalization. On univariate analysis, significant preoperative risk factors for hospital mortality were cardiopulmonary resuscitation, coagulopathy, renal dysfunction, elevated aspartate aminotransferase levels, myocardial ischemia, and lower-extremity ischemia. As for factors related to surgery, the duration of operation, cardiopulmonary bypass time, aortic cross-clamp time, and volume of blood transfusion were greater among patients who died during hospitalization than in those who were discharged from the hospital. On multivariate analysis, independent preoperative risk factors were cardiopulmonary resuscitation, renal dysfunction, and lower-extremity ischemia. Shock or cardiac tamponade were not risk factors. CONCLUSIONS: Risk factors for hospital mortality in patients with Stanford type A acute aortic dissection were cardiopulmonary resuscitation, renal dysfunction, and lower-extremity ischemia.

Endovascular repair of ascending aortic rupture: effectiveness of a fenestrated stent-graft.

PURPOSE: To present a technique for endovascular treatment using a fenestrated stent-graft in a patient with ascending aortic rupture in the setting of methicillin-resistant Staphylococcus aureus infection. CASE REPORT: A 62-year-old woman had undergone mastectomy and radiotherapy twice for breast cancer and then coronary artery bypass grafting (CABG). She developed sternal osteomyelitis 5 years after the CABG. Sternectomy and negative-pressure wound drainage were performed, but the infection did not resolve. Ascending aortic rupture occurred 5 months after sternectomy. Endovascular therapy was considered the only effective means of achieving hemostasis. A custom-designed fenestrated stent-graft was deployed from the ascending aorta to the proximal descending aorta via a femoral artery approach without transient cardiac arrest. Bleeding completely stopped after surgery. The postoperative course was uneventful, and the inflammatory activity subsided on antibiotic therapy. At 7 months after surgery, the patient's recovery has been uneventful. CONCLUSION: Rupture of the ascending aorta associated with infection was successfully treated by stent-graft repair. The use of a custom-made, fenestrated stent-graft was an effective, lifesaving procedure for the management of this ascending aortic lesion.

Acute aortic dissection occurring during the butterfly stroke in a 12-year-old boy.

A 12-year-old boy had severe chest and back pain of sudden onset while practicing the butterfly stroke in a swimming class. Computed tomography revealed an intimal flap in the descending thoracic aorta with massive right hemothorax. A ruptured type B acute aortic dissection was diagnosed, and then he collapsed. We totally replaced the descending aorta with a woven polyester prosthetic graft during deep hypothermic circulatory arrest. Hemostasis was achieved, but consciousness was not regained after operation, and multiple organ failure occurred. He died on the fifth postoperative day. He and his family had no history of cardiovascular disease. It seems that the swimming provoked a severe Valsalva maneuver, raising blood pressure acutely and thereby leading to dissection. This is then analogous to the propensity for dissection during intense isometic exercise such as weightlifting.