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Tricuspid and pulmonary valve involvement in carcinoid heart disease
Archives of Cardiovascular Disease (2009) 102, 591—592
IMAGE
Tricuspid and pulmonary valve involvement in carcinoid heart disease Atteinte des valves pulmonaires et tricuspide chez une patiente présentant une tumeur carcinoïde du grêle Stéphane Ederhy , Franck Engel , Ariel Cohen ∗ Cardiology Department, Saint-Antoine University and Medical School, université Pierre-et-Marie-Curie, Assistance Publique—Hôpitaux de Paris, 184, rue du Faubourg—Saint-Antoine, 75571 Paris cedex 12, France Received 5 March 2009; accepted 29 April 2009 Available online 28 July 2009
KEYWORD Carcinoid heart disease
MOT CLÉ Tumeur carcinoide
∗
A 55-year-old woman with a history of proven carcinoid syndrome due to an enterochromaffin ileal tumour was evaluated with transthoracic echocardiography for dyspnoea on exertion. The patient had flushing and severe diarrhoea. Urinary excretion of 5hydroxyindoleacetic acid was 250 mmol/L per 24 hours. Treatment with a somatostatin analogue had been initiated 5 months earlier. Physical examination revealed a 3/6 systolic ejection murmur at the left upper sternal border. No signs of right-sided heart failure were found. The electrocardiographic data were within normal limits. Transthoracic two-dimensional echocardiography revealed restricted motion of the septal and anterior tricuspid valve leaflets both during systole and diastole, leading to an eccentric jet and mild to moderate tricuspid regurgitation (Fig. 1 and Movie 1). Continuous wave (CW) Doppler documented an associated tricuspid stenosis with a mean diastolic gradient of 5 mmHg (Fig. 2). The pulmonary valve was also involved, defined by a limited excursion of the pulmonary valve leaflets without a complete coaptation. CW Doppler of the pulmonary valve demonstrated the presence of both pulmonary stenosis defined by a mean gradient of 40 mmHg (Fig. 3) and pulmonary regurgitation (Movie 2). No patent foramen ovale was found at contrast echocardiography. Pulmonary and tricuspid valves were replaced with bioprosthetic valves. A surgical view of the tricuspid and pulmonary valve revealed immobile, thickened and retracted pulmonary and tricuspid valves leaflets (Figs. 4 and 5). Characteristic echocardiographic features of advanced carcinoid heart disease include thickening, retraction of immobile tricuspid valve leaflets with associated tricuspid
Corresponding author. Fax: +33 1 49 28 28 84. E-mail addresses: [email protected], [email protected] (A. Cohen).
1875-2136/$ — see front matter © 2009 Published by Elsevier Masson SAS. doi:10.1016/j.acvd.2009.04.008
592
Figure 1. Apical 4-chamber view showing restricted motion of the septal and anterior tricuspid leaflet during systole and diastole.
S. Ederhy et al.
Figure 4. Surgical view of the tricuspid valve showing immobile, retracted and thickened valve leaflets.
Figure 2. Continuous wave Doppler on the tricuspid valve revealing a moderate tricuspid stenosis with a mean gradient of 5 mmHg.
regurgitation in 90% of cases and pulmonary involvement, mainly immobility of the pulmonary valve cusps, in at least 50% of cases [1]. Contrast transthoracic echocardiography should be performed systematically, searching for a right-to-left atrial shunt through a patent foramen ovale [2].
Figure 5. Surgical view of the pulmonary valve showing immobile, retracted and thickened valve leaflets.
Conflict of interests None.
Appendix A. Supplementary data Supplementary data associated with this article can be found, in the online version, at doi:10.1016/j.acvd. 2009.04.008.
References
Figure 3. Continuous Doppler of the pulmonary valve demonstrating pulmonary stenosis with a mean gradient of 40 mmHg.
[1] Pellikka PA, Tajik AJ, Khandheria BK, et al. Carcinoid heart disease. Clinical and echocardiographic spectrum in 74 patients. Circulation 1993;87:1188—96. [2] Mansencal N, Mitry E, Pilliere R, et al. Prevalence of patent foramen ovale and usefulness of percutaneous closure device in carcinoid heart disease. Am J Cardiol 2008;101:1035—8.
IMAGE
Tricuspid and pulmonary valve involvement in carcinoid heart disease Atteinte des valves pulmonaires et tricuspide chez une patiente présentant une tumeur carcinoïde du grêle Stéphane Ederhy , Franck Engel , Ariel Cohen ∗ Cardiology Department, Saint-Antoine University and Medical School, université Pierre-et-Marie-Curie, Assistance Publique—Hôpitaux de Paris, 184, rue du Faubourg—Saint-Antoine, 75571 Paris cedex 12, France Received 5 March 2009; accepted 29 April 2009 Available online 28 July 2009
KEYWORD Carcinoid heart disease
MOT CLÉ Tumeur carcinoide
∗
A 55-year-old woman with a history of proven carcinoid syndrome due to an enterochromaffin ileal tumour was evaluated with transthoracic echocardiography for dyspnoea on exertion. The patient had flushing and severe diarrhoea. Urinary excretion of 5hydroxyindoleacetic acid was 250 mmol/L per 24 hours. Treatment with a somatostatin analogue had been initiated 5 months earlier. Physical examination revealed a 3/6 systolic ejection murmur at the left upper sternal border. No signs of right-sided heart failure were found. The electrocardiographic data were within normal limits. Transthoracic two-dimensional echocardiography revealed restricted motion of the septal and anterior tricuspid valve leaflets both during systole and diastole, leading to an eccentric jet and mild to moderate tricuspid regurgitation (Fig. 1 and Movie 1). Continuous wave (CW) Doppler documented an associated tricuspid stenosis with a mean diastolic gradient of 5 mmHg (Fig. 2). The pulmonary valve was also involved, defined by a limited excursion of the pulmonary valve leaflets without a complete coaptation. CW Doppler of the pulmonary valve demonstrated the presence of both pulmonary stenosis defined by a mean gradient of 40 mmHg (Fig. 3) and pulmonary regurgitation (Movie 2). No patent foramen ovale was found at contrast echocardiography. Pulmonary and tricuspid valves were replaced with bioprosthetic valves. A surgical view of the tricuspid and pulmonary valve revealed immobile, thickened and retracted pulmonary and tricuspid valves leaflets (Figs. 4 and 5). Characteristic echocardiographic features of advanced carcinoid heart disease include thickening, retraction of immobile tricuspid valve leaflets with associated tricuspid
Corresponding author. Fax: +33 1 49 28 28 84. E-mail addresses: [email protected], [email protected] (A. Cohen).
1875-2136/$ — see front matter © 2009 Published by Elsevier Masson SAS. doi:10.1016/j.acvd.2009.04.008
592
Figure 1. Apical 4-chamber view showing restricted motion of the septal and anterior tricuspid leaflet during systole and diastole.
S. Ederhy et al.
Figure 4. Surgical view of the tricuspid valve showing immobile, retracted and thickened valve leaflets.
Figure 2. Continuous wave Doppler on the tricuspid valve revealing a moderate tricuspid stenosis with a mean gradient of 5 mmHg.
regurgitation in 90% of cases and pulmonary involvement, mainly immobility of the pulmonary valve cusps, in at least 50% of cases [1]. Contrast transthoracic echocardiography should be performed systematically, searching for a right-to-left atrial shunt through a patent foramen ovale [2].
Figure 5. Surgical view of the pulmonary valve showing immobile, retracted and thickened valve leaflets.
Conflict of interests None.
Appendix A. Supplementary data Supplementary data associated with this article can be found, in the online version, at doi:10.1016/j.acvd. 2009.04.008.
References
Figure 3. Continuous Doppler of the pulmonary valve demonstrating pulmonary stenosis with a mean gradient of 40 mmHg.
[1] Pellikka PA, Tajik AJ, Khandheria BK, et al. Carcinoid heart disease. Clinical and echocardiographic spectrum in 74 patients. Circulation 1993;87:1188—96. [2] Mansencal N, Mitry E, Pilliere R, et al. Prevalence of patent foramen ovale and usefulness of percutaneous closure device in carcinoid heart disease. Am J Cardiol 2008;101:1035—8.