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|Number 11-21: Utility of Cardiac MRI in Carcinoid Heart Disease|
Number 11-21: Utility of Cardiac MRI in Carcinoid Heart Disease
Case from: Rosica Panayotova, Simon G Ray, Matthias Schmitt, Nik Abidin
Institute: Univeristy Hospital of South Manchester, Manchester, UK.
Clinical history: A 65 year old patient presented with symptoms of worsening right heart failure.
Echo 2a & 2b - apical 4-chamber focused RV view
Transthoracic echocardiography showed right heart dilation and severe tricuspid regurgitation with grossly abnormal, thickened and retracted tricuspid valve leaflets (Echo 1a and 1b; Echo 2a and 2b).
Figure 1 - End systolic SSFP frame
The TV leaflets had typical appearance of thickening, shortening and retraction, characteristic of carcinoid TV disease. They were fixed in a partially open position throughout the cardiac cycle, with resultant severe tricuspid regurgitation (Movie 1, Fig 1, Movie 2, Movie 3, Movie 4).
Movie 1 Movie 2
Movie 3 Movie 4
An accurate calculation of RV volumes and ejection fraction was obtained from the RV transaxial stack SSFP cine views (Movie 5, Table 1). The normalized values to BSA indicate that the right ventricular volumes are mildly elevated.
Right ventricular trans-axial stack
Table 1 - Volumetric measurements for the Right Ventricle - Values normalized to BSA
There was interventricular septal flattening with typical D-shaped left ventricle (LV) in diastole seen in the short axis LV SSFP cine stack views suggestive of RV volume overload (Movie 6).
SSFP - RV & LV short axis cine
Movie 7a Movie 7b
The tricuspid regurgitation (TR) was quantified by the TR regurgitant volume, derived using the indirect method of subtracting the pulmonary forward flow (54 mls) from the right ventricular stroke volume (RV SV = 105 mls). This gave an estimated regurgitation volume of 51 mls and a regurgitation fraction of 49%, in keeping with severe TR. The pulmonary forward flow was estimated using through plane phase contrast velocity mapping positioned just above the level of the pulmonary valve whilst the RV SV was estimated by contouring volumes of the RV transaxial stacks in systole and diastole.
Sagittal views of the IVC and its drainage into the RA were obtained (Movie 8), with in-plane and through-plane velocity mapping of the RA inflow at the level of the proximal IVC for detection and quantification of venous flow reversal related to the severe tricuspid regurgitation.
Late gadolinium enchancement T1 inversion recovery and phase sensitive inversion recovery imaging was performed to look for hyperenhancement within the RV wall (Fig 3a and 3b, Fig 4a and 4b). Although there was no evidence of obvious late gadolinium uptake, there appeared to be thinning of the RV free wall with the appearance of chemical shift artefact at the myocardium - blood pool interface. There was no evidence of intracardiac metastatic disease. Of note, there is late gadolinium enhancement of the anterior tricuspid valve leaflet.
Conclusion: This study illustrates a typical case of right heart valve involvement in carcinoid syndrome with evidence of RV volume loading, dilation and thinning of the RV free wall. The preserved RV systolic function is a favourable prognostic factor for patients who are being worked up for valve replacement surgery.
Perspective: Cardiac carcinoid is a rare condition which affects up to 50% of patients with carcinoid syndrome. It is more common in those with metastatic disease and in particular liver involvement. The neuroendocrine tumours, usually originating from the GI tract, secrete vasoactive substances which promote the formation of endocardiac plaques of fibrous tissue. These most often affect the right sided heart valves, including the subvalvular apparatus and papillary muscles. Left sided heart valve involvement may occur if there is a PFO present or in the setting of pulmonary metastases [1,2].References:
The presence of cardiac involvement in carcinoid syndrome imparts a dramatic deterioration in prognosis with a 50% increase in mortality. There is evidence that surgical correction of the valve abnormalities not only improves symptoms but also overall prognosis and thus should be considered in appropriately selected patients [3,4].
CMR evaluation of RV function and volumes, as well as accurate calculation of valvular regurgitant fractions, is helpful for a more detailed pre-operative assessment and patient risk stratification .
1. Fox DJ, Khattar RS. Carcinoid heart disease: presentation, diagnosis, and management, Heart. 2004; 90(10):1224-1228
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Number 11-21: Carcinoid heart disease
Case open for discussion. Pretty impressive TR! ...
On: 11/10/2011 By: chiarabd Read more?