Ann Thorac Surg 2005;79:2147-2149
© 2005 The Society of Thoracic Surgeons
Case report
Double Bioprosthetic Valve Replacement in Right-Sided Carcinoid Heart Disease
Pieter G. Voigt, MDa,*,
Jerry Braun, MDa,
Onno Y. Teng, MDa,
Dave R. Koolbergen, MD, PhDa,
Eduard Holman, MD, PhDb,
Jeroen J. Bax, MD, PhDb,
Vincent T.H.B.M. Smit, MD, PhDc,
Robert A.E. Dion, MD, PhDa
a Department of Cardiothoracic Surgery, Leiden University Medical Center, Leiden, the Netherlands
b Department of Cardiology, Leiden University Medical Center, Leiden, the Netherlands
c Department of Pathology, Leiden University Medical Center, Leiden, the Netherlands
Accepted for publication December 10, 2003.
* Address reprint requests to Dr Voigt, Department of Cardiothoracic Surgery, Leiden University Medical Center, Albinusdreef 2, Leiden 2333 ZA, the Netherlands (E-mail: p.g.voigt{at}lumc.nl).
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Abstract
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A patient with tricuspid and pulmonary regurgitation due to carcinoid syndrome successfully underwent double bioprosthetic valve replacement. This technique avoids anticoagulation treatment in a patient with hepatic dysfunction and facilitates future hepatic de-arterialization as a treatment option in carcinoid disease. Advances in treatment of carcinoid syndrome may have reduced the risk of early bioprosthetic degeneration.
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Introduction
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Carcinoid heart disease represents one of the most intriguing aspects of carcinoid syndrome. Carcinoid tumors are rare, with a reported incidence of 1.5 per 100,000 [1]. Cardiac involvement (due to systemic effects of circulating vasoactive amines produced by a metastatic carcinoid tumor [2]) is detected in 57% to 70% of patients [3]. Prognosis is poor due to development of heart failure and death [1].
Typically cardiac lesions are detected on the right side of the heart, mostly limited to the tricuspid valve [1]. Combined lesions of tricuspid and pulmonary valves are rare.
In this report, we describe a patient with carcinoid heart disease who presented with severe tricuspid and pulmonary regurgitation, and who subsequently underwent tricuspid and pulmonary valve replacement using two bioprostheses. We believe this is the first report of treatment of carcinoid heart disease using two bioprostheses.
A 58-year-old woman with known carcinoid syndrome was referred to our institution. She underwent partial resection of the jejunum for carcinoid disease in 1997. On that occasion synchronous hepatic metastases were diagnosed.
On admission she complained of worsening dyspnea and increasing body weight. Physical examination revealed no acute respiratory distress. There was marked peripheral edema, hepatomegaly, and an elevated central venous pressure. A systolic murmur was heard at the left fifth intercostal space. Electrocardiogram revealed low-voltage ventricular complexes. Laboratory measurements showed normocytic anemia (hemoglobin, 5.9 mmol/L; mean cellular volume, 83 fL). Liver enzymes were normal, except for an elevated 
-glutamyl transpeptidase (281 U/L) due to liver metastases. Blood urea nitrogen and serum creatinine levels were normal, whereas 5-hydroxyindoleaceticacid urinary level was elevated (2400 µmol/24 hours urine; normal < 52 µmol/24 hours urine).
Preoperative transthoracic echocardiography showed grade 4 tricuspid and grade 3 to 4 pulmonary regurgitation without stenoses (Figs 1A–C). The tricuspid and pulmonary valve leaflets were thickened, retracted, and immobile. The right ventricle was enlarged (end-diastolic dimension, 52 mm; normal < 45 mm) due to volume overload. Left ventricular dimensions and function were normal.
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Fig 1. (A) Transthoracic echocardiogram (4-chamber view) showing thickened and retracted tricuspid leaflets (arrow). (B) Similar view as in figure 1A, showing grade 4 tricuspid regurgitation (color Doppler imaging). (C) Transthoracic echocardiogram (parasternal short-axis) showing severe pulmonary regurgitation. (D) Transesophageal echocardiogram (direct postoperatively) showing the tricuspid valve prosthesis. (E) Similar view as in figure 1D, showing no residual tricuspid regurgitation.
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Octreotide scintigraphy did not show cardiac metastases. Because of the severity of cardiac symptoms and given an estimated life expectancy of more than 2 years, and based on the course of her carcinoid disease, the patient was accepted for surgery. Full normothermic cardiopulmonary bypass was established through a midline sternotomy. The tricuspid valve was exposed through a right atriotomy. The leaflets were thickened, retracted with commissural fusion, and covered by pearly-white plaques extending into the right ventricle. The valve was excised and replaced by a size 29 mitral Carpentier Pericardiac Perimount bioprosthesis (Edwards Lifesciences, Irvine, CA). Transesophageal echocardiography during surgery showed no residual tricuspid regurgitation (Fig 1D, 1E). The pulmonary valve was inspected through a longitudinal incision into the main pulmonary artery. The cusps were thickened and retracted, and they were excised followed by a pulmonary root replacement with a Medtronic Freestyle stentless bioprosthesis (Medtronic Inc, Minneapolis, MN). Weaning from cardiopulmonary bypass was uneventful. The patient recovered without complications. Transthoracic echocardiography at discharge showed normal function of both bioprostheses. No regurgitation was observed. The mean gradients over the tricuspid and pulmonary valve prostheses were 7 mm Hg and 12 mm Hg, respectively.
Histopathologic evaluation of the excised valves and right atrial tissue revealed typical carcinoid-related fibrous plaques, showing collagen-rich connective tissue with proliferating (myo-)fibroblasts (Fig 2). Twenty months after double valve replacement the patient is in stable cardiac condition. Echocardiography reveals no regurgitation or stenosis of either bioprosthesis. Right ventricular function is normal and the dimension decreased to 41 mm.
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Fig 2. Histologic examination of the resected pulmonary valve. (Left) Normal valve tissue containing elastic fibers demonstrated by Verhoeff von Gieson staining (x40). (Right) Fibrous valve thickening without elastic fibers, characteristic of carcinoid disease.
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Comment
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Cardiac surgery is the only effective treatment for symptomatic patients with metastatic carcinoid heart disease, although perioperative mortality is high [1, 4]. Controversy exists about the valvular substitute that should be used. Early reports recommend using a mechanical prosthesis based on the assumed damage to bioprosthetic valves by circulating vasoactive tumor substances [1]. Indeed, premature bioprosthetic degeneration has been reported in carcinoid patients [5]. However, the introduction of more effective therapies (eg, somatostatin analogues, hepatic artery interruption) may potentially protect prosthetic valve tissue from the adverse effects of serotonin and other vasoactive peptides [1]. Furthermore, mechanical prostheses are not ideal because of life-long anticoagulation therapy, inducing an increased risk if hepatic de-arterialization is considered. In addition, the risk of thrombosis in mechanical tricuspid prostheses is 4% per year [6].
In our patient, two bioprostheses were implanted (a stentless Freestyle prosthesis [Medtronic Inc] in the pulmonary position and a stented pericardial valve [Edwards Lifesciences] in the tricuspid position). The rationale for this approach was to avoid anticoagulation therapy in a patient with a high risk of bleeding, due to hepatic dysfunction, who was still a candidate at the time of surgery for hepatic de-arterialization. A stentless bioprosthesis was preferred in the pulmonary position because of the extreme friability of the annulus and root. At 2 years follow-up, both bioprostheses are functioning well without right ventricular dysfunction. Thus, double bioprosthetic valve replacement in right-sided carcinoid heart disease appears to be a feasible therapeutic option.
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References
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- Connolly HM, Nishimura RA, Smith HC, Pellikka PA, Mullany CJ, Kvols LK. Outcome of cardiac surgery for carcinoid heart disease J Am Coll Cardiol 1995;25:410-416.[Abstract]
- Møller JE, Connoly HM, Rubin J, Seward JB, Modesto K, Pellikka PA. Factors associated with progression of carcinoid heart disease N Engl J Med 2003;348:1005-1015.[Abstract/Free Full Text]
- Robiolio PA, Rigolin VH, Harrison JK, et al. Predictors of outcome of tricuspid valve replacement in carcinoid heart disease Am J Cardiol 1995;75:485-488.[Medline]
- Connolly HM, Schaff HV, Mullany CJ, Rubin J, Abel MD, Pellikka PA. Surgical management of left-sided carcinoid heart disease Circulation 2001;104(Suppl 1):I-36-I-40.
- Ridker PM, Chertow GM, Karlson EW, Neish AS, Schoen FJ. Bioprosthethic tricuspid valve stenosis associated with extensive plaque deposition in carcinoid heart disease Am Heart J 1991;121:1835-1838.[Medline]
- Thornburn CW, Morgan JJ, Shanahan MX, Chang VP. Long-term results of tricuspid valve replacement and the problem of prosthetic valve thrombosis Am J Cardiol 1983;51:1128-1132.[Medline]
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Abstract
Introduction
