Transannular Pulmonary Enlargement and Bioprostheses for Carcinoid Disease
Cliff K Choong, MD1,2,3,
Joseph E Arrowsmith, MD1,
Andrew A Klein, FRCA1,
Francis C Wells, FRCS1
1 Papworth Hospital NHS Foundation Trust, Cambridge, UK
2 Department of Surgery, The University of Cambridge, Cambridge, UK
3 Department of Surgery, Monash Medical Centre, Monash University, Melbourne, Victoria, Australia
Francis C Wells, FRCS, Tel: +44 1480 830541, Fax: +44 1480 364936, Email: francis.wells{at}papworth.nhs.uk, Papworth Hospital NHS Foundation Trust, Cambridge, CB23 8RE, UK.
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ABSTRACT
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A diminutive pulmonary artery and right ventricular outflow tract in a 46-year-old woman with a 10-year history of carcinoid syndrome required transannular pulmonary patch enlargement to allow replacement of the pulmonary and tricuspid valves with bioprostheses. The avoidance of anticoagulation permitted further hepatic arterial embolization without an increased risk of bleeding.
Key Words: Bioprosthesis • Carcinoid Heart Disease • Pulmonary Valve Stenosis • Tricuspid Valve Stenosis
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INTRODUCTION
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Carcinoid heart disease is a rare but important cause of intrinsic tricuspid and pulmonary valve disease, leading to significant morbidity and mortality secondary to right heart failure.1 When treated medically, and in appropriate cases surgically, significant benefits in overall quality of life and long-term survival can be achieved.1–3
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CASE REPORT
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A 46-year-old woman with a 10-year history of carcinoid syndrome and a 12-month period of progressive right heart failure secondary to severe tricuspid and pulmonary regurgitation, despite maximal medical therapy, presented acutely with severe right heart failure requiring urgent cardiac surgical intervention. The primary site of the carcinoid tumor had not been identified despite thorough investigations. Several successful palliative arterial embolizations of hepatic metastases had been performed, and further treatments were scheduled. Surgery was undertaken with care to prevent a carcinoid crisis during induction and maintenance of anesthesia.4 Intraoperative findings included markedly dilated right heart chambers and thickened and retracted tricuspid and pulmonary valve leaflets. The pulmonary artery (PA) and right ventricular outflow tract were contracted, narrowing to a diameter of 15 mm. Enlargement of the right ventricular outflow tract and PA annulus was undertaken with a 4-cm longitudinal incision and a patch of bovine pericardium. The PA was also incised longitudinally almost up to the bifurcation, and enlarged with another pericardial patch, resulting in the original PA forming the back wall of the neo-PA (Figure 1
). The pulmonary and the tricuspid valves were replaced with 21-mm and 27-mm stented pericardial tissue valves (Carpentier-Edwards Perimount; Edwards Lifesciences, Irvine, CA, USA), respectively. The patient was weaned from cardiopulmonary bypass with inotropic support, and her recovery in the intensive care unit was complicated by intermittent carcinoid crises characterized by flushing, nausea, and marked arterial hypertension. These were treated with octreotide infusion and analgesia. She was discharged from the hospital on the 14th postoperative day, and her ongoing care was closely managed by her endocrinologist. At the 6-month follow-up, she had made a good recovery with improvement of her symptoms from New York Heart Association class IV preoperatively to class I. At the 24-month follow-up, she remained well clinically, did not require anticoagulation, and had undergone 4 subsequent hepatic arterial embolizations for treatment of her hepatic metastases.
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Figure 1. Intraoperative picture showing reconstructed right ventricular outflow tract (RVOT) enlarged using a transannular pulmonary technique and patched with a sheet of bovine pericardium. The pulmonary artery (PA) was incised longitudinally almost up to the bifurcation and enlarged with another pericardial patch, resulting in the original PA forming the back wall of the neo-PA.
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DISCUSSION
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Carcinoid tumors are rare neuroendocrine malignancies arising from neural crest amine precursor uptake decarboxylation cells. Approximately 90% of carcinoid tumors are located in the gastrointestinal tract.1 The most aggressive of these tumors tend to arise from the ileum, and must invade or metastasize to produce carcinoid syndrome. The incidence of carcinoid tumors is approximately 1 in 75,000.5 Carcinoid syndrome occurs in half of these cases, and approximately 50% of patients with carcinoid syndrome develop carcinoid heart disease which typically causes abnormalities of the right side of the heart.5 Usually, only carcinoid tumors that invade the liver result in pathological changes to the heart. The cardiac manifestations are caused by paraneoplastic effects of vasoactive substances such as 5-hydroxytryptamine, tachykinins, histamine, and prostaglandins released by the malignant cells, rather than by direct cardiac metastatic involvement.1,5 The characteristic cardiac pathological findings are endocardial plaques of fibrous tissues. The preferential right heart involvement is most likely related to inactivation of vasoactive substances by the lung. In 5%–10% of cases with left-sided valvular pathology, a bronchial carcinoid or patent foramen ovale should be excluded.2
Mechanical valve prostheses have been advocated in patients with carcinoid heart disease because these valves are durable and relatively unaffected by vasoactive substances.1,5 A disadvantage is the fact that patients with carcinoid syndrome often have extensive liver metastases and hepatic dysfunction, and are therefore at high risk of bleeding due to the mandatory use of anticoagulation. Furthermore, the 3-year mortality data for patients with carcinoid heart disease shows a 31% survival rate, which is half of that in patients without cardiac involvement.1 It is therefore likely that the durability of modern bioprosthetic valves is greater than the life expectancy of patients with carcinoid heart disease.1,3 The introduction of more effective therapy, such as octreotide and hepatic artery interruption, might potentially protect the bioprosthetic valve from the adverse effects of vasoactive peptides. Although the use of bioprostheses in carcinoid heart disease has been reported previously, transannular pulmonary patch enlargement to enable placement of a stented tissue valve and thereby avoid using a small mechanical prosthesis has rarely been described. We believe that transannular pulmonary patch enlargement and use of bioprosthetic valves in this patient allowed her to undergo further hepatic arterial embolization without the increased risks of bleeding or valvular thrombosis. This strategy should be considered for patients with carcinoid syndrome who have a small right ventricular outflow tract and PA, requiring valve replacement and hepatic artery interruption.
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REFERENCES
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- Fox DJ, Khattar RS. Carcinoid heart disease: presentation, diagnosis and management. Heart 2004;90:1224–8.[Free Full Text]
- Connolly HM, Schaff HV, Mullany CJ, Rubin J, Abel MD, Pellikka PA. Surgical management of left-sided carcinoid heart disease. Circulation 2001;104(Suppl I)36–40.
- 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; 25:410–6.[Abstract]
- Prasad MK, Rosario CJ, Arrowsmith JE. Anesthetic management of the patient with carcinoid heart disease. In: Arrowsmith JE, Simpson J, editors. Problems in anesthesia: cardiothoracic surgery. Martin Dunitz, London, 2002:293–9.
- Basson MD, Ahlman H, Wangberg B, Modlin IM. Biology and management of the midgut carcinoid. Am J Surg 1993;165:288–97.[Medline]
Asian Cardiovasc Thorac Ann 2009;
17:510-512
© 2009 by SAGE Publications
DOI: 10.1177/0218492309348628
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ABSTRACT
INTRODUCTION
