Undiagnosed Severe Pulmonic Regurgitation in a Carcinoid Patient for Tricuspid Valve Replacement and Patent Foramen Ovale Closure

Hastie, Maya Jalbout MD; Souh, Jennifer MD; Ju, Albert MD; Shanewise, Jack S. MD

Anesthesia & Analgesia:
doi: 10.1213/ANE.0b013e3182753538
Cardiovascular Anesthesiology: Echo Rounds
Author Information

From the Department of Anesthesiology, Division of Adult Cardiothoracic Anesthesiology, Columbia University, New York.

Accepted for publication July 16, 2012.

The authors declare no conflicts of interest.

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Oral informed consent for the publication of this article was obtained from the patient.

Reprints will not be available from the authors.

Address correspondence to Maya Jalbout Hastie, MD, Department of Anesthesiology, Division of Adult Cardiothoracic Anesthesiology, Columbia University, 622 W 168th street, PH 5, Rm505, New York, NY 10032. Address e-mail to mj2081@columbia.edu.

Article Outline

A 67-year-old man with carcinoid heart disease presented for patent foramen ovale (PFO) closure and tricuspid valve (TV) replacement for severe tricuspid regurgitation (TR). The patient was referred for surgery with progressive dyspnea and signs of right ventricle (RV) dysfunction. The patient’s consent for the case report was obtained.

Preoperative transthoracic echocardiogram (TTE) revealed normal left ventricular function, enlarged RV with severely decreased function, and severe TR. Preoperative transesophageal echocardiogram (TEE) showed a moderately dilated RV, thickened anterior tricuspid leaflet with frozen posterior leaflet resulting in malcoaptation and severe TR, and PFO. Although not well vizualized, the pulmonic valve (PV) was reported to be grossly normal. Pulmonary artery pressures were reported as normal (24/10 mm Hg).

Baseline intraoperative TEE revealed normal left ventricle size and function and hypertrophied and dilated RV with mildly decreased function. TV leaflets were thickened and tethered open (Video 1, see Supplemental Digital Content 1, http://links.lww.com/AA/A492), resulting in 4+ TR with systolic reversal of hepatic vein flow. Three-dimensional TEE showed thickened, immobile TV leaflets with no central coaptation (Video 2, see Supplemental Digital Content 2, http://links.lww.com/AA/A493). The PV leaflets were difficult to see, but at least 2 appeared to be thickened and retracted. Severe pulmonary regurgitation (PR) was seen with color flow Doppler (CFD; Video 3, see Supplemental Digital Content 3, http://links.lww.com/AA/A494). Pulsed wave Doppler (PWD) across the PV showed a dense diastolic PR wave with rapid deceleration to zero before the onset of systole (Fig. 1). A PFO with a small left-to-right shunt was seen with CFD.

Given the new finding of severe PR, the patient underwent TV replacement, PV replacement, and PFO closure. Postcardiopulmonary bypass TEE showed a well-seated bioprosthetic TV and PV with good leaflet motion. Continuous wave Doppler (CWD) across the PV prosthesis showed a peak velocity of 150 cm/s, consistent with a prosthetic valve, but the diastolic PR wave below the baseline was gone (Fig. 2). A mirror image artifact of the systolic wave was noted below the baseline. PV pathology revealed proliferation of myointimal cells on intact, tan rubbery valvular tissue consistent with carcinoid plaque. The patient’s postoperative course was unremarkable.

Carcinoid heart disease occurs in more than 50% of patients with carcinoid tumors.1 Enterochromaffin cell-derived neuroendocrine tumors metastasize to the liver and secrete serotonin, which travels to the right heart causing deposits of fibrous material on valves.1 Left-sided valvular involvement is rare, and it is postulated that serotonin is inactivated in the pulmonary circulation.2,3 The presence of a PFO could lead to left-sided pathology2–4 and was therefore surgically closed in our patient. The fibrous plaque deposition causes valves and tendinous cords to thicken and shorten.4 The thickened valves are often retracted and immobile, resulting in regurgitation and stenosis. TR is most common (97%) followed by tricuspid stenosis (59%), PR (50%), and pulmonary stenosis (25%).2

Others have reported TV involvement in carcinoid heart disease.3,5 We report a classic case of carcinoid heart disease in which both the TV and PV had become retracted and immobile resulting in regurgitation. Although our patient had preoperative TTE and TEE, PR had not been diagnosed before surgery. A careful intraoperative examination of all valves should be performed, especially in patients with carcinoid disease. Assessment of the PR severity is less validated than other valvular lesions, because it is uncommon.6 Using different modalities for evaluation is recommended.6,7 Structural abnormalities of the PV should be identified when possible. However, PV can be challenging to visualize,7 and usually only 1 or 2 of the 3 leaflets can be seen.6 In addition, because of its anterior location, the PV is more difficult to evaluate with TEE than TTE. Although the use of 3-dimensional TEE is not validated for examining PV pathology, it allows visualization of the 3 leaflets from a modified midesophageal long-axis view of the aorta, bringing the PV into view.8 Presence of PR can be detected by CFD, and severity of the regurgitation can be estimated by the jet’s duration and width relative to the RV outflow track.6 The jet width is measured in diastole, immediately below the PV6 in the midesophageal ascending aorta short-axis or upper esophageal aortic arch short-axis views with Nyquist limits set at 50 cm/s to 60 cm/s.6 A PR jet occupying more than 65% of RV outflow tract width is suggestive of severe PR.6 Jet density on CWD or PWD is a qualitative measure of severity and is proportional to the number of red cells involved in the regurgitation jet.6,7 The rate of deceleration of the regurgitant jet can be measured in CWD or PWD as the time from peak velocity to baseline; a rapid deceleration rate and hence a shorter jet duration, can be seen in severe PR;6 however, the rate of deceleration can be affected by filling and RV pressures,7 leading to earlier equalization of pressures between the pulmonary artery and RV. A dilated RV is not specific for severe PR; however, a normal size RV usually excludes it.6 Other quantitative measures of severity of regurgitation such as vena contracta and regurgitant fraction can be challenging to obtain and lack validation.6,7 Table 1 summarizes the echocardiographic findings in severe PR. In our patient, structural abnormality, a PR jet occupying the width of the RV outflow tract by CFD, a dense PR wave on PWD that approched zero velocity before systole (Fig. 1), and a dilated RV in the absence of pulmonary hypertension, were indicative of severe PR. The concomittant diagnosis of carcinoid with the newly identified pathology on TEE dramatically altered surgical management.

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Name: Maya Jalbout Hastie, MD.

Contribution: This author helped prepare the manuscript.

Name: Jennifer Souh, MD.

Contribution: This author helped prepare the manuscript.

Name: Albert Ju, MD.

Contribution: This author helped prepare the manuscript and edit the videos.

Name: Jack S. Shanewise, MD.

Contribution: This author helped prepare the manuscript and edit the videos.

This manuscript was handled by: Martin J. London, MD.

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Clinician’s Key Teaching Points

By Nikolaos J. Skubas, MD, Roman M. Sniecinski, MD, and Martin J. London, MD

* Carcinoid tumors secrete serotonin resulting in fibrin deposition with leaflet thickening and retraction, usually restricted to the tricuspid or pulmonic valves. The left-sided cardiac valves are not affected, unless a right-to-left communication is present, as serotonin is inactivated in the pulmonary circulation.

* The immobile valve leaflets cause stenosis and regurgitation of the affected valve. The tricuspid valve is more commonly affected than the pulmonary valve. Eventually, the right ventricle may dilate with impaired function.

* In this case of a patient with carcinoid-induced pulmonic regurgitation, intraoperative 2-dimensional (2D) and 3D transesophageal echocardiogram (TEE) examination revealed thickened and retracted leaflets (midesophageal [ME] ascending aorta short-axix [SAX]). Severe regurgitation was diagnosed based on qualitative characteristics of the pulmonary regurgitation jet with a wide neck (color Doppler) and dense diastolic spectral envelope with rapid deceleration to baseline (continuous wave Doppler). The right ventricle was enlarged and hypocontractile.

* A careful examination of all cardiac valves should be performed in patients with carcinoid disease. The anterior location of the pulmonic valve usually results in superior imaging with transthoracic echocardiography. However, 3D TEE may facilitate imaging of all leaflets (modified ME ascending aorta SAX). Associated findings, such as a normal size right ventricle, may be helpful in excluding pulmonic valve involvement

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