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Ann Thorac Surg 2006;81:e4-e5
© 2006 The Society of Thoracic Surgeons


Case report

Homograft Replacement of the Calcified Aortic Root in Familial Hypercholesterolemia

Tamer Elghobary, MD a , c , * , Joseph Allal, MD b , Paul Menu, MD b , Olivier Baron, MD a

a Department of Thoracic and Cardiovacular Surgery, Laënnec Hospital, Nantes, France
b Medico-Surgical Department of Cardiology, De la Milétrie Hospital, Potiers, France
c Department of Cardiothoracic Surgery, Ain-Shams University Center, Cairo, Egypt

Accepted for publication July 25, 2005.

* Address correspondence to Dr Elghobary, Thoracic Institute, Laënnec Hospital, Nantes, France, 44093 (Email: telghobary{at}yahoo.com).


    Abstract
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 Abstract
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A 39-year-old woman with a known history of homozygous familial hypercholesterolemia was admitted with chest discomfort. Preoperative echocardiography and coronary angiography showed a heavily stenotic aortic valve as well as a calcific hypoplastic aortic root. Aortic root replacement using an aortic homograft was done.


    Introduction
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Homozygous familial hypercholesterolemia is an autosomal dominant disorder caused by a mutation of the gene for the low-density lipoprotein receptor [1]. We report the case of a young woman with hypercholesterolemia who underwent aortic root replacement using an aortic homograft.

A 39-year-old patient with a known history of hypercholesterolemia was admitted for chest pain and dyspnea grade III. Twelve years prior, this patient had undergone coronary artery bypass grafting surgery using the left internal thoracic artery. Its proximal segment was used to anastomose the left anterior descending artery and its distal segment was used as a free vascular patch to perform an osteoplasty to the right coronary ostium. Also she had a past history of a porto-caval anastomosis, an operation that used to be done for the palliation of this medical condition.

The patient was undergoing weekly plasma low-density lipoprotein apheresis. Her medical history also included peripheral arterial disease, and a non-stenosing atheroma at the ostium of the left carotid artery.

The echocardiography showed a tight stenosis of the aortic valve with a transvalvular gradient of 70 mm Hg and a cusp opening of 0.76cm2. There was also an element of subvalvular stenosis due to concentric hypertrophy of the left ventricular wall. The diameter of the aortic annulus was 17 mm, and the diameter of the sinuses of Valsalva at the level of the coronary ostia was 25 mm. The diameters of the supracoronary ascending aorta and that of the aortic arch were 22 and 20 mm, respectively.

The coronary angiography showed diffusely infiltrated coronary vessels with no significant lesions. It also confirmed the patency of the left internal mammary artery–left anterior descending coronary artery anastomosis and the absence of stenosis in the right coronary ostium operated on previously.

Thoracic magnetic resonance imaging confirmed the diameters of the aorta as well as the valve surface area and the calcifications of the aortic wall.

During surgery, our policy is to establish the cardiopulmonary bypass through femoro-femoral cannulas in all redo cases. The femoral artery was atherosclerotic, but its cannulation was easy. After sternotomy, the left internal mammary artery was isolated and freed, the ascending aorta was clamped in an apparently healthy place, and the heart was arrested using warm blood anterograde cardioplegia through the aortic root.

The aortic root was moderately calcified and the aortic arch was less calcified. The aortic root was resected. To our surprise, the right coronary ostium, which was the site of the previous surgical angioplasty, was free of calcification; however, it was the left coronary ostium that was surrounded by calcium plaques. We were able to separate the two coronary buttons and resect the aortic valve that was calcified and tricuspid. A myomectomy was done down in the ventricular septum to relieve the subvalvular stenosis, and no subaortic membrane was found. A 21-mm aortic homograft was inserted in place with 3-0 continuous sutures without difficulty. Partial decalcification of the left coronary button and reimplantation of the two coronary buttons were done.

A short period of deep hypothermia (21°C) and total circulatory arrest (10 min) were used to perform the distal aortic anastomosis with the proximal part of the aortic arch at the base of the great vessels.

Aortic cross-clamp time was 131 minutes and total bypass time was 250 minutes. The heart regained a sinus rhythm after 1 direct current shock with no inotropes.

On decannulation, the femoral artery was partially dissected, and we performed a short femoro-femoral anastomosis using a Gore-Tex 6 tube with no postoperative complications.

The postoperative period was difficult with temporary acute renal failure that spontaneously resolved. A short period of left ventricular dysfunction was treated with diuretics, and a mild pericardial effusion disappeared with follow-up.


    Comment
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The aim of this article is to highlight this rare pathology of familial homozygous hypercholesterolemia and its complications on the aortic valve and on the ascending aorta, as well as to discuss the different modalities of dealing with these complications.

In the English literature we did not find a similar case of homozygous familial hypercholesterolemia treated with aortic homograft. Yasuda and colleagues [2] reported a similar case for which the aortic valve was replaced by a mechanical bi-leaflet valve, together with endarterectomy of the calcified aortic root under total circulatory arrest. In their case, the right coronary artery was also grafted using the gastroepiploic artery.

The choice of homograft was done preoperatively on the basis of echocardiographic findings. Replacing the aortic valve with a mechanical valve with endarterectomy of the ascending aorta was our second option. We would only have chosen this option if it seemed difficult to us to separate the two coronary buttons. However, as a result of the intraoperative findings previously described, we were able to resect the entire ascending aorta and separate the coronary buttons.

We preferred the option of the homograft because of the young age of the patient, the complications of lifelong anticoagulation therapy in mechanical valve cases, the diameter, and the calcification of the aortic annulus, which would not permit a mechanical valve size greater than 19, and also with the complications of aortic endarterectomy on the strength of the aortic wall.

In the case described by Yasuda and colleagues [2], they used retrograde cerebral perfusion during the whole procedure. In our case, we clamped the aorta in an area apparently free of calcification just at the base of the innominate artery. We only performed the distal aortic anastomosis under total circulatory arrest as the period was short, and we did not need to do any cerebral perfusion.

Yasuda and colleagues [2] used retrograde cardioplegia for their operation to avoid any embolization in the coronary arteries. We used warm intermittent anterograde blood cardioplegia; first through the aortic root and then directly through the coronary ostia. It did not seem to us that there would be any contraindication in using anterograde cardioplegia, as the right coronary ostium was free of calcification and the left coronary ostium was surrounded with fixed calcium plaques.

We believed that using a homograft to replace the aorta in this rare pathology was a good option. Preoperative echocardiography, thoracic computed tomography, or magnetic resonance imaging are of extreme importance in delineating the size of the ascending aorta, the diameter of the aortic annulus, as well as the extent and degree of calcification.

The patient is now being followed-up. She is having weekly sessions of plasma low-density lipoprotein apheresis. Echocardiography 6 months after the intervention showed a well-functioning aortic homograft with a transvalvular mean gradient of 27 mm Hg and good left ventricular function.


    References
 Top
 Abstract
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 Comment
 References
 

  1. Kawasuji M, Sakakibara N, Takemura H, Matsumoto Y, Mabuchi H, Watanabe Y. Coronary artery bypass grafting in familial hypercholesterolemia J Thorac Cardiovasc Surg 1995;109:364-369.[Abstract/Free Full Text]
  2. Yasuda T, Kawasuji M, Sakakibara N, Watanabe Y. Aortic valve replacement for the calcified ascending aorta in homozygous familial hypercholesterolemia Eur J Cardiothorac Surg 2000;18:249-250.[Abstract/Free Full Text]



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[Abstract] [Full Text] [PDF]


This Article
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Paul Menu
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Right arrow Valve disease


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