Ann Thorac Surg 2007;83:287-288
© 2007 The Society of Thoracic Surgeons
Case Reports
Successful Aortic Valve Replacement for Heyde Syndrome With Confirmed Hematologic Recovery
Atsutomo Morishima, MD,
Akira Marui, MD, PhD,
Takeshi Shimamoto, MD,
Yoshiaki Saji, MD,
Keiichi Tambara, MD, PhD,
Takeshi Nishina, MD, PhD,
Masashi Komeda, MD, PhD*
Department of Cardiovascular Surgery, Kyoto University Graduate School of Medicine, Kyoto, Japan
Accepted for publication May 16, 2006.
* Address correspondence to Dr Komeda, Department of Cardiovascular Surgery, Kyoto University Graduate School of Medicine, 54 Shogoin-Kawara, Sakyo, Kyoto 606-8507 Japan (Email: komelab{at}kuhp.kyoto-u.ac.jp).
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Abstract
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Aortic valve stenosis can be complicated by recurrent gastrointestinal bleeding, particularly that due to angiodysplasia, also called Heyde syndrome. Recently, acquired type 2A von Willebrand disease, which is characterized by the loss of the large multimer of von Willebrand factor by the shear stress of aortic valve stenosis, was reported to be associated with this hemorrhagic syndrome. A 78-year-old woman, with severe aortic stenosis, presented with advanced anemia due to recurrent gastrointestinal bleeding and was diagnosed Heyde syndrome. By perioperative supplementation of von Willebrand factor and factor VIII, aortic valve replacement was safely performed without gastrointestinal bleeding. After the operation, the multimer of von Willebrand factor was normalized and thereafter no gastrointestinal bleeding occurred. This case reports the successful aortic valve replacement for Heyde syndrome, with confirmed hematologic recovery.
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Introduction
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Heyde syndrome, which presents with aortic stenosis and recurrent gastrointestinal bleeding from angiodysplasia, is rare but sometimes life-threatening [1, 2]. High fluid shear stress of the blood components by aortic stenosis leads to a decrease in the large multimer of von Willebrand factor (vWF) and causes systemic tendency of bleeding [3]. Aortic valve replacement (AVR) is the radical treatment for this syndrome; however, to establish cardiopulmonary bypass (CPB) by itself carries a high risk of bleeding to the patient. We report a successful case of AVR for Heyde syndrome with postoperative hematologic recovery, where perioperative supplementation with vWF and factor VIII was effective.
A 78-year-old woman with severe aortic stenosis had advanced anemia due to recurrent gastrointestinal (GI) bleeding of unknown origin and needed repeated transfusion. She presented with heart failure caused by aortic stenosis and anemia; however, she could not undergo aortic valve surgery because of the severe GI bleeding, and she was referred to our hospital.
An echocardiogram showed severe aortic stenosis, with a peak aortic pressure gradient of 127 mm Hg. The left ventricular ejection fraction was 0.60. Multiple angiodysplasia on the ileum and colon were diagnosed by endoscopy (Fig 1). No other lesions except for the angiodysplasia were found. Although the abnormal laboratory findings were anemia (hemoglobin, 9.3 g/dL) and prolonged bleeding time (5 minutes), the platelet count and other coagulation functions were normal. Further hematologic examination by gel electrophoresis revealed loss of large multimer of vWF, and the diagnosis of Heyde syndrome was established (Fig 2).
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Fig 1. Multiple angiodysplasia with redness (white arrows) was observed on the ileum by endoscopy. Multiple angiodysplasia were located on the ileum and colon.
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Fig 2. Multimer analysis of von Willebrand factor (vWF) by gel electrophoresis. Preoperation (Preop): large multimer of vWF was absent. Postoperation (Postop): multimer of vWF was normalized 7 days after the aortic valve replacement (black arrows). (L = large multimer [molecular weight: about 5500 to 7500 kD]; M = medium multimer [molecular weight: about 3000 to 5000 kD]; S = small multimer [molecular weight: about 1000 to 2500 kD]; SS = smallest multimer [molecular weight: about 500 kD]; N = control A: this patient.)
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To avoid perioperative GI bleeding, we intravenously administered 1000 units of Confact F (combination of vWF and factor VIII, Chemo-Sero-Therapeutic Research Institute, Kumamoto, Japan) 1 hour before the operation. The patient was systemically heparinized (300 U/kg).
Under standard CPB with mild hypothermia, the ascending aorta was cross-clamped. The aortic valve had 3 cusps, all heavily calcified. The aortic ring was narrow and measured 19 mm. The valve and calcium was excised, and a 19-mm bovine pericardial bioprosthesis was implanted. Aortic cross-clamp time was 58 minutes and CPB time was 93 minutes.
This patient received two 500-unit doses of Confact F, after CPB and when the patient returned to the intensive care unit. Total amount of the chest tube drainage after the operation was 220 mL. Two days after the operation, bleeding time was normalized (2 minutes), and anticoagulation therapy with warfarin and aspirin was started. Seven days after the operation, vWF multimer was normalized (Fig 2). Additional supplementation of Confact F was unnecessary. The patient’s postoperative course was uneventful, and there was no anemia or GI bleeding.
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Comment
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In 1958, Heyde [1] initially reported an association between aortic stenosis and GI bleeding. In 1992, Olearchyk and colleagues [3] defined a combination of aortic stenosis and recurrent GI bleeding from angiodysplasia as Heyde syndrome; however, the cause of the syndrome had been unclear. Recently, Vincentelli and colleagues [2] reported that acquired von Willebrand disease type 2A causes Heyde syndrome. As it passes through the aortic stenotic valve, vWF is subjected to high fluid shear stress that renders the multimers susceptible to cleavage by ADAMTS13 (A disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) [4] and leads to loss of the large multimer. In addition, reduction in perfusion pressure by aortic stenosis may cause ischemia of mucous membrane of the intestine and venous dilation of angiodysplasia [5].
Although there were several reports of surgical treatment of Heyde syndrome, not a few patients had perioperative bleeding, which were sometimes life threatening. Endoscopic treatment of angiodysplasia may be effective. As observed in the present case, however, the angiodysplasia is untreatable by endoscopy if it is located at multiple sites of the intestine. Therefore, AVR, followed by normalization of the large multimer of vWF, is essential to patients with Heyde syndrome who have untreatable GI bleeding.
CPB itself, however, places those patients at high risk. If patients with aortic stenosis show GI bleeding, they should be suspected of having Heyde syndrome, and multimer of vWF should be evaluated. If the large multimer of vWF is absent, supplementation of vWF might be effective to prevent perioperative hemorrhagic shock. We perioperatively administered Confact F, which is combination of vWF and factor VIII. Although there was no protocol for the administration of Confact F to patients with Heyde syndrome, we used the same protocol as used for cardiac surgery for von Willebrand disease [6].
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References
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- Heyde EC. Gastrointestinal bleeding in aortic stenosis N Engl J Med 1958;259:196.
- Olearchyk AS. Heyde’s syndrome J Thorac Cardiovasc Surg 1992;103:823-824.[Medline]
- Vincentelli A, Susen S, Le Tourneau T, et al. Acquired von Willebrand syndrome in aortic stenosis N Engl J Med 2003;349:343-349.[Abstract/Free Full Text]
- Tsai HM, Sussman II, Nagel RL. Shear stress enhances the proteolysis of von Willebrand factor in normal plasma Blood 1994;83:217-219.[Abstract/Free Full Text]
- Boley SJ, Sammartano R, Adams A, DiBiase A, Kleinhaus S, Sprayregen S. On the nature and etiology of vascular ectasias of the colon Gastroenterology 1977;72:650-660.[Medline]
- Sekino Y, Hata M, Endo M, Tabayashi K, Suzuki Y, Mohri H. Aortic valve replacement in a patient with von Willebrand’s disease—a case report Nippon Kyobu Geka Gakkai Zasshi 1990;38:1183-1187Japanese.[Medline]
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Abstract
Introduction
