Aortic Root Replacement with Composite valve graft conduit for Aortic root aneurysm in a Patient with Marfan Syndrome
Aortic Root Replacement with Composite valve graft conduit
for aortic root aneurysm in a Patient with Marfan Syndrome.
Mohammad Fazle Maruf
For reprint information contact: Mohammad Fazle Maruf, Department of Cardiothoracic Surgery, Chittagong medical College.
Abstract
A 34 yrs old man of marfan syndrome, visited us with the complaints of
palpitation, easy fatigability and chest discomfort for last 1year. He was
pointed out to have the aortic root enlargement with severe aortic
regurgitation & mild mitral regurgitation by echocardiography and CT
angiogram. We excised the aneurysm, implanted a composite graft with prosthetic
valve conduit (Bentall's operation), directly attached the coronary arteries to
the aortic graft, and made the distal anastomosis to the divided aorta.
Postoperative course was uneventful. To our knowledge, this is the first
successful case of Bentall’s operation in NICVD.
Introduction
Marfan Syndrome is an autosomal dominant hereditary connective tissue disorder with the clinical manifestations involving the ocular, skeletal and cardiovascular systems. The cardiovascular manifestations include aortic root dilatation,aortic valvular insufficiency, Mitral valve prolapse, Mitral regurgitation,aortic dissection and aortic rupture.Rupture of an ascending aortic aneurysm is the most common cause of death in patients with Marfan syndrome. We report our results of the surgical management of the Aortic Aneurysm with severe Aortic regurgitation & mild Mitral regurgitation.
Marfan Syndrome is an autosomal dominant hereditary connective tissue disorder with the clinical manifestations involving the ocular, skeletal and cardiovascular systems. The cardiovascular manifestations include aortic root dilatation,aortic valvular insufficiency, Mitral valve prolapse, Mitral regurgitation,aortic dissection and aortic rupture.Rupture of an ascending aortic aneurysm is the most common cause of death in patients with Marfan syndrome. We report our results of the surgical management of the Aortic Aneurysm with severe Aortic regurgitation & mild Mitral regurgitation.
Case Study
A man of 37 yrs come to us from Munshigonj with symptoms of palpitation,dyspnea and chest pain for 1 year. In addition to his positive family history of Marfan syndrome,he had the clinical findings of long slender body stature, arachnodactyly and high arch palate.
A systolic murmur was heard at the fourth left intercostal space and an early diastolic murmur was detected at the left sternal border.His BP was 120/20 mm of Hg.Chest radiography showed Cardiomegaly and a dilated ascending aorta.Transesophageal echocardiography showed severe aortic regurgitation and mild Mitral regurgitation. CT Angiogram revealed that the aortic root was measured 84mm x74mm x70mm sparing aortic arch. Coronary arteries are normal.
During surgery Cardio Pulmonary bypass was established through aortic and two stage single venous cannulation. The ascending aorta was separated from the main pulmonary artery. Moderate hypothermia achieved & aortic cross clamp applied. Ascending aorta opened longitudinally and selective antegrade cardioplegia was given to arrest the heart. Aortic valve was excised and aorta resected . Bental procedure (Composite graft replacement of the ascending aorta with aortic valve replacement and re-implantation of the coronary arteries in to the graft) was performed with 25 mm ATS composite bileaflet metalic Aortic valve conduit. Coronary orifice holes were made in the tube with a wire electrocautery. The Coronary ostia were anastomosed to the graft using 6-0 polypropylene running sutures. The aortic arch was found to be intact and it was anastomosed into the distal end of the graft. In addition, composite graft was wrapped with native aortic wall. Cross clamp time was 108 mins. Re-exploration was done on same day for post operative bleeding (2900ml in 5 hours). Generalized oozing was found and haemostasis was secured. Rest of the post operative period was uneventful & he was discharged with good recovery. He is still under my followup.
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| Fig 1. X-ray of chest P-A view showed dilatation of aortic root. |
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| Fig 2: CT angiogram of heart showing aneurismal dilatation of ascending aorta, sparing the arch. |
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| Fig3: Photograph of aortic aneurysm. |
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| Fig 4: Composite aortic graft with aortic valve also showing right coronary ostium. |
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