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Case Reports

Aortic Valve Replacement in a Patient With Osler-Rendu-Weber Disease

^a Cardiothoracic Intensive Care Unit, Instituto Cardiovascular de Buenos Aires, Buenos Aires, Argentina
^b Department of Cardiology, Instituto Cardiovascular de Buenos Aires, Buenos Aires, Argentina
^c Department of Hematology, Instituto Cardiovascular de Buenos Aires, Buenos Aires, Argentina
^d Department of Cardiothoracic Surgery, Instituto Cardiovascular de Buenos Aires, Buenos Aires, Argentina

Accepted for publication February 6, 2009.

^_* Address correspondence to Dr Benzadón, Instituto Cardiovascular de Buenos Aires, Blanco Encalada 1543, Buenos Aires, C1428DCO, Argentina (Email: marianobenzadon{at}yahoo.com.ar).

	Abstract

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Osler-Rendu-Weber (hereditary hemorrhagic telangiectasia) disease is an uncommon disease characterized by the presence of abnormal telangiectasias and arteriovenous malformations that cause recurrent episodes of bleeding. We present a patient with Osler-Rendu-Weber disease, with a history of multiple major bleeding events and severe aortic valve stenosis, who underwent aortic valve replacement. Unexpectedly, the postoperative course was uneventful, and there was no untoward bleeding in the early or in the late postoperative follow-up.

	Introduction

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Osler Rendu-Weber (O-R-W) disease, or hereditary hemorrhagic telangiectasia, is characterized by an abnormality of the peripheral blood vessels that often causes recurrent epistaxis. We report aortic valve replacement in a patient with O-R-W disease with 12-month follow-up. To the best of our knowledge, this represents the second case published in the literature [1].

The patient was a 52-year-old man who had been diagnosed with O-R-W disease 20 years earlier and presented with gastrointestinal, nasal, and bronchi telangiectasias, and hepatic arteriovenous malformations (AVMs). He was being treated with a daily dose of danazol (200 mg), vitamin C (50 mg), and flavonoids (450 mg) to prevent bleeding, and ferrous sulfate (40 mg) for anemia. He had been hospitalized 80 times in the last 2 years because of severe bleeding from the respiratory and gastrointestinal tract.

The patient was sent to our institution after sustaining an acute myocardial infarction with shock, secondary to anemia due to a massive epistaxis that required multiple transfusions, mechanical ventilation, and support with inotropic drugs. A transthoracic echocardiogram showed a severe aortic valve stenosis, with a peak pressure gradient of 92 mm Hg, a velocity of 4.8 m/s, an area of 0.7 cm², and an ejection fraction of 0.51. No lesions were found on coronary angiography.

After the patient was stabilized, an urgent aortic valve replacement was performed. To avoid anticoagulation, a St Jude No. 23 (St. Jude Medical, St. Paul, MN) bioprosthesis was implanted within a total operation time of 250 minutes and a cardiopulmonary bypass time of 75 minutes. The patient recovered satisfactorily, without bleeding in the early postoperative period, and was discharged on day 5. He was not treated with aspirin because of his high hemorrhagic risk. After a 1-year follow-up, the patient did not experience any new bleeding complications.

	Comment

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O-R-W (hereditary hemorrhagic telangiectasia) is inherited as an autosomal dominant trait, with a prevalence of 1 in 8000. It can be caused by mutation of the endoglin gene (O-R-W 1) and the activin receptor gene (O-R-W 2), which are essential products for angiogenesis and normal vascular maturation [2]. These mutations produce the formation of telangiectasias and AVMs, which tend to rupture easily and bleed. They are usually localized in skin and mucosa, causing epistaxis and gastrointestinal bleeding, but AVMs also occur in the pulmonary and hepatic circulation in 30% of patients and in the cerebral circulation in 10% [3].

Cardiopulmonary bypass causes several changes in hemostasis that increase the risk of hemorrhage in the early postoperative period. These alterations tend to be worse with longer cardiopulmonary bypass time [4]. It is possible that the short cardiopulmonary bypass exposure in our patient might have helped to reduce the early postoperative blood loss. In addition with this patient, we carefully focused on keeping the activated clotting time at more than 400 seconds, a measure that seems to prevent bleeding due to lower coagulation system activation [5].

The lack of severe hemorrhagic complications observed in the ambulatory follow-up of our patient may have been related to aortic valve replacement. Aortic stenosis tends to increase venous pressure, which could enhance the risk of telangiectasias and AVM bleeding, similar to what happens in patients with Heyde syndrome (aortic valve stenosis and colonic vascular ectasias) [6]. As expected in our patient, left ventricle filling pressures were significantly reduced as estimated by conventional and tissue Doppler studies after the operation.

Aortic stenosis may also be associated with the von Willebrand acquired syndrome, which tends to resolve when the aortic stenosis is surgically corrected [7]. The disappearance of these mechanisms after the operation may explain the abrupt cessation of hemorrhages observed in our patient in the late follow-up. However, we cannot confirm this hypothesis because we did not test our patient for von Willebrand disease.

Our results are similar to those previously reported by Seike and colleagues [1], according to which a 75-year-old woman with aortic stenosis was operated on and recovered without any early bleeding complications. In contrast with our patient, Seike and colleagues did not report the late postoperative course.

The postoperative course of our patient was uneventful and there was no untoward bleeding. It appears that the surgical correction of aortic valve stenosis in patients with O-R-W disease can reduce episodes of hemorrhage in the late follow-up. This hypothesis calls for further evaluation.

	References

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1. Seike Y, Sato H, Nagashima M, et al. Aortic valve replacement for the patient with Osler-Rendu-Weber disease Kyobu Geka 2005;58:1141-1144.[Medline]
2. Payne RM, Jonhson MC, Grant JW, Srauss AW. Toward a molecular understanding of congenital heart disease Circulation 1995;91:494-504.[Abstract/Free Full Text]
3. Begbie ME, Wallace GM, Shovlin CL. Hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu syndrome): a view from the 21st century Postgrad Med J 2003;79:18-24.[Abstract/Free Full Text]
4. Khuri SF, Wolfe JA, Josa M, et al. Hematologic changes during and after cardiopulmonary bypass and their relationship to the bleeding time and nonsurgical blood loss J Thorac Cariovasc Surg 1992;104:94-107.[Abstract]
5. Despotis GJ, Joist JH, Hogue Jr CW, et al. The impact of heparin concentration and activated clotting time monitoring on blood conservation. A prospective, randomized evaluation in patients undergoing cardiac operation. J Thorac Cardiovasc Surg 1995;110:46-54.[Abstract/Free Full Text]
6. Greenstein RJ, McElhinney AJ, Reuben D, Greenstein AJ. Colonic vascular ectasias and aortic stenosis: coincidence or causal relationship? Am J Surg 1986;151:347-351.[Medline]
7. Vincentelli A, Susen S, Le Tourneau T, et al. Acquired von Willebrand syndrome in aortic stenosis N Engl J Med 2003;349:343-349.[Medline]

This article has been cited by other articles:

				F. Shikata, M. Nagashima, K. Kawachi, Y. Seike, S. Takano, and T. Hori Open Heart Surgery for Hereditary Hemorrhagic Telangiectasia Ann. Thorac. Surg., June 1, 2010; 89(6): 2074 - 2075. [Full Text] [PDF]

This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Guillermo Vaccarino Marcelo Trivi Daniel Navia Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Benzadón, M. N. Articles by Navia, D. Search for Related Content PubMed PubMed Citation Articles by Benzadón, M. N. Articles by Navia, D. Related Collections Valve disease