Spontaneous rupture of an aneurysm of the right subclavian artery as a first presentation of Ehlers Danlos syndrome in a 15-year old boy.

We report a case of a spontaneous rupture of a right subclavian aneurysm in a 15 year-old patient. This ruptured aneurysm was successfully treated in an endovascular manner by placing a covered stent-graft in the right subclavian artery via right brachial access. Subsequent work-up by skin biopsy and fibroblast culture and by DNA-screening revealed the diagnosis of Ehlers Danlos type IV. Meanwhile, the patient developed twice a spontaneous pneumothorax, treated with thoracoscopic pleurodesis. This article provides a clear overview of the clinical and genetic characteristics of a case of Ehlers Danlos type IV and illustrates the importance of avoiding surgery in patients with connective tissue disease because of the high risk of perioperative complications.

Balloon-expandable, radially reinforced ePTFE relining of the superficial femoral artery after remote endarterectomy.

AIM: The aim of this paper was to evaluate the mid-term results of remote endarterectomy and balloon-expandable, radially reinforced ePTFE endograft uplining of the superficial femoral artery (SFA) for long occlusions. METHODS: One hundred and two limbs in 92 patients were included in this prospective, multicenter study. Indications were disabling claudication (n=74), restpain (n=10) and tissue loss (n=16) due to an SFA occlusion of at least 10 cm in length. Remote endarterectomy of the SFA using a modified ringcutter in an over-the-wire technique, was followed by implantation of a balloon-expandable Enduring endovascular graft. Control visits were performed at 1, 3 and 6 months postprocedure and every half year from then on. Follow-up protocol included physical examination, Doppler ankle-brachial index measurement and, in case of doubt, duplex examination. RESULTS: A technical success rate of 98% was achieved. Mean follow-up was 36 months. Four patients were lost to follow-up and 17 patients died of nondevice-related causes. The primary, primary assisted and secondary patency rates were 68%, 73% and 97.5% at 1 year and 50%, 60%, and 89% at 3 years, respectively. CONCLUSION: Although technical success rate is high and early patency rates are good, mid-term results are inferior to those of conventional femoro-popliteal synthetic bypass grafts. In order to become competitive to conventional bypass surgery, further technical refinements will be necessary, especially to overcome the problem of stenoses at the distal transition zone.

Complex thoracic aortic aneurysm: a combined open and endovascular approach.

The authors wish to describe a combined open and endovascular approach to repair a complex thoracic aortic aneurysm. A 72-year-old man with chronic obstructive pulmonary disease, aortic valvular insufficiency and diffuse thoracic aortic aneurysm underwent aortic valve and ascending aorta replacement by a Bentall-procedure and replacement of arch aneurysm using the elephant trunk technique, performed in a first procedure. During the second procedure, endovascular stenting of the descending thoracic aorta was done. Only a few similar case reports have been presented. Endovascular repair after an elephant trunk procedure for complex thoracic aortic aneurysms is an elegant approach to deal with such mega aortas. Further research is necessary to compare open and endovascular repair and to determine long-term follow-up with regard to endoleaks and mortality.

Ventricular outflow tract obstruction secondary to leiomyosarcoma of the right ventricle.

Primary leiomyosarcomas of the heart, particularly those affecting the right ventricle, are uncommon. We report the case of a 70-year-old Belgian woman presenting with the symptoms of progressive exertional dyspnea and left-sided pleuritic pain. A leiomyosarcoma which originated from the right lateral ventricle wall, causing pulmonary outflow obstruction, was diagnosed. Pathology revealed a neoplasm with a myxoid stroma, high mitotic activity and nuclei expressing atypia. Immunohistochemical staining was positive for vimentine and desmin. Seven months after complete surgical resection the tumor relapsed. This case demonstrates the poor outcome, the high relapse rate and inefficiency of treatment associated with primary cardiac leiomyosarcomas. The current literature regarding the incidence, diagnostic techniques, treatment strategies and survival rates of this rare but terminal disease is reviewed.

Endoleaks after endoluminal repair of abdominal aortic aneurysms: diagnosis and treatment.

Endoleaks currently present one of the major problems related to endoluminal treatment of abdominal aortic aneuryms (AAA). The incidence and fate of endoleaks stress the importance of long term follow-up. In this article the authors discuss which imaging techniques can best be used for surveillance and how endoleaks should be classified. Furthermore the necessity of treatment as well as treatment options are discussed.

Traumatic rupture of the thoracic aorta: the diagnostic challenge.

The traumatic rupture of the thoracic aorta is a severe and lifethreatening entity. It is therefore important to diagnose this lesion. Classically, this is based on the detection of a widened superior mediastinum on chest X-ray. However, several factors like supine position, obesity, mediastinal haematoma,... might bias the diagnosis. The authors present four different clinical presentations of traumatic rupture of the thoracic aorta and describe how the lesion was detected. Subsequently, they review the literature on the diagnostic approach of this severe lesion.

Repair of descending thoracic aneurysms through a left posterolateral thoracotomy using deep hypothermic circulatory arrest.

Aneurysms of the descending thoracic aorta are usually treated under simple cross-clamping of the aorta. A shunt or partial bypass may be used to protect the heart and to maintain the distal circulation. However, situations exist where repair is only feasible using deep hypothermic circulatory arrest (DHCA). The aim of this retrospective study is to describe the technique of DHCA through a left posterolateral thoracotomy and to assess conditions in which this technique can be used. Fifteen patients with proximal descending thoracic aneurysms were treated using DHCA through a left posterolateral thoracotomy. Most of the interventions were urgent. The decision to use DHCA was made intraoperatively in 86% of the patients and was based mainly on local, unforeseen conditions. Early mortality was 13.3%, no late mortality was observed. The most frequent complications were respiratory (20%) and renal (13.3%). Paraplegia or other neurologic disorders, temporary or permanent, were not encountered. Deep hypothermic circulatory arrest is a helpful tool in the management of difficult descending thoracic aorta aneurysms through a left posterolateral approach. In cases where simple cross-clamping of the aorta is not possible, this technique offers a reliable alternative with acceptable morbidity and mortality.

Aneurysm of the left pulmonary artery: surgical allograft repair.

We present a case of left pulmonary artery aneurysm with pulmonary valve stenosis. The aneurysm was excluded and the valve replaced by the implantation of a pulmonary artery allograft. The short-term follow-up (20 months) is promising.

Surgical treatment of left ventricular aneurysm and ischemic mitral incompetence.

Chronic left ventricular aneurysm and ischemic mitral valve incompetence have been treated during the last 2 years with more physiologic techniques. Left ventricular reconstruction with the endoaneurysmorrhaphy technique was carried out in 20 patients. Sixteen patients had additional procedures. Early mortality was 5% and functional results are encouraging with 18 patients in NYHA class I of II. Mitral valve repair was carried out in combination with myocardial revascularization in 15 patients. All patients had a Carpentier Edwards annuloplasty ring implanted. Nine patients needed additional reconstructive procedures. There were no early or late deaths neither reoperations. Late functional results are good with all patients in NYHA, class I or II. Three patients present a mild mitral regurgitation on echo. This physiologic approach to restore volume, size and shape of the left ventricle and the mitral valve can be combined with CABG without additional operative risk and excellent results up to 2 years.