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Papillary Fibroelastoma of the Pulmonary Valve

The Value of Three-Dimensional Transesophageal Echocardiography

Rebaine, Zineb MD; Watremez, Christine MD, PhD; El Khoury, Gebrine MD; Momeni, Mona MD, PhD

doi: 10.1213/ANE.0000000000000987
Cardiovascular Anesthesiology: Echo Rounds
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From the Department of Anesthesiology, Cliniques Universitaires Saint Luc, Université Catholique de Louvain, Brussels, Belgium.

Accepted for publication July 27, 2015.

Funding: None.

The authors declare no conflicts of interest.

Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal’s website.

Reprints will not be available from the authors.

Address correspondence to Mona Momeni, MD, PhD, Department of Anesthesiology, Cliniques Universitaires Saint Luc, Université Catholique de Louvain, Avenue Hippocrate 10–1200, Brussels, Belgium. Address e-mail to mona.momeni@uclouvain.be.

A previously healthy and physically active 48-year-old man presented to our hospital for the resection of a mass at the level of the pulmonary valve (PV). The patient’s symptoms were dyspnea and increasing fatigue. Preoperative transthoracic echocardiography (TTE) had shown a mass at the level of the PV with moderate left ventricular dysfunction. There were no signs of right ventricular outflow tract obstruction. Preoperative tests were negative for tumor markers. There was no evidence of chronic thromboembolic disease. The blood cultures were negative. Positron emission tomography showed hyperfixation at the level of the main pulmonary hilus. The decision to resect the mass was made.

Figure 1

Figure 1

Figure 2

Figure 2

Intraoperative 3-dimensional (3D) transesophageal echocardiography (TEE) was performed. Full-volume (FV) acquisition of the midesophageal right ventricular inflow–outflow tract view revealed a well-defined, nonmobile mass in the proximal right ventricular outflow tract adjacent to the PV (Supplemental Digital Content 1, Video 1, http://links.lww.com/AA/B241). No pedicle was observed. Only trivial pulmonary regurgitation was present (Supplemental Digital Content 2, Video 2, http://links.lww.com/AA/B242). Video 3 (Supplemental Digital Content 3, http://links.lww.com/AA/B243) helped analyze the mass and its relationship to the pulmonary cusps in several multiplanar reconstruction views. Figure 1 illustrates the absence of attachments to any pulmonary cusp. The mass showed a specific appearance with the presence of cavities. This typical aspect with the areas of different echogenicity inside the mass is seen in Figure 2. After exclusion of a patent foramen ovale, the mass was resected under cardiopulmonary bypass with beating heart. The size of the mass was 17 mm × 15 mm. It showed the characteristic “sea anemone” shape once put into a water solution. Its histologic examination revealed a papillary cystic myxoid stroma surrounded by endothelial cells. The diagnosis of a papillary fibroelastoma was made. The patient was discharged on postoperative day 5. TTE after 1 month showed a normal functioning PV.

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DISCUSSION

The differential diagnosis of a mass at the level of the PV includes endocarditis, a noninfective thrombus, or the presence of a primary valve tumor. In an analysis of 22 autopsy series, Reynen1 reported a frequency of 0.02% of primary cardiac tumors. The most common histologic type of cardiac valve tumors are papillary fibroelastomas and myxomas.2 Cardiac papillary fibroelastomas are small, benign tumors derived from the normal components of the endocardium. They are usually attached to the endocardium by a short pedicle. The analysis of 725 cases of cardiac papillary fibroelastomas revealed that in 44% of the cases, the tumor was located at the aortic valve, followed by mitral valve position in 35%, the tricuspid valve in 13% and the PV in 8% of the cases.3 Although these tumors are benign, they can embolize, resulting in life-threatening side effects. Their surgical removal seems to be low risk compared with their potential complications and should be considered.4 In a systemic review of the PV papillary fibroelastoma, 26 cases were analyzed.5 As in our case, dyspnea on exertion was the most common clinical presentation. Besides the TTE, all patients had undergone a TEE. The study could not show any specific echocardiographic features that would allow making the diagnosis of fibroelastoma. The echocardiographic differential diagnosis of a mass at the level of the PV can be challenging. Right-sided endocarditis occurs primarily in patients with a history of drug abuse, with central venous lines or pacemakers, or in congenital heart disease. The tricuspid valve is the usual site of the right-sided infective endocarditis, but pulmonary and Eustachian valves may be affected as well.6 Vegetations exhibit motion distinct from the endocardial surface and have an irregular appearance and rough borders. A thrombus can present with similar echocardiographic characteristics as a fibroelastoma. Right ventricular hypokinesis is common in case of a right ventricle thrombus. Moreover, it is generally known that fibroelastomas appear speckled with echolucencies.

The visualization of the PV is difficult with the use of conventional 2-dimensional (2D) TTE. The feasibility of PV imaging using 3D TTE has been demonstrated in a prospective study.7 Although the image quality was excellent, good, and fair in 0.5%, 7%, and 51.5%, respectively, of the studied population, poor image quality was observed in 41% of the patients. Poor 3D image quality was the result of poor 2D initial images because of patient-related factors. Good quality imaging can be obtained with 2D TEE, but the latter does not allow the analysis of the 3 cusps. Three-dimensional TEE overcomes these problems. Although the transgastric basal right ventricle view and upper esophageal aortic arch short-axis view can highlight the PV, in this case, the midesophageal right ventricular inflow–outflow tract view gave the best 2D imaging of the PV. In our case, 1 3D FV acquisition from the midesophageal right ventricular inflow–outflow view permitted offline analysis of the valve in several orthogonal planes. In the setting of a PV mass, 3D FV data can be cropped to identify the exact valvular anatomy. The position of the mass with regard to the 3 pulmonary cusps can be evaluated to guide the surgeon in the excision of the mass and the performance of an eventual PV repair. Moreover, as illustrated in our case, 3D TEE facilitates the echocardiographic analysis of the mass in the search of any echolucency signs and the presence of any pedicle or stalk attachments.

In conclusion, 3D TEE is a helpful tool in the evaluation of any cardiac mass at the level of the PV. Offline analysis of the 3D images helps in the exact evaluation of the mass and the pulmonary cusps.

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Clinician’s Key Teaching Points

By Nikolaos J. Skubas, MD, A. Stephane Lambert, MD, and Martin J. London, MD

  • A right ventricular (RV) mass at the level of the pulmonic valve (PV) is most likely to be a thrombus, endocarditis lesion, or tumor. The position of the mass relative to the PV cusps will guide the surgical procedure.
  • A thrombus is usually associated with the regional or global RV hypokinesis and, if organized, may be strongly echogenic because of calcification. Vegetations tend to have irregular surface and erratic motion, are mostly attached on the tricuspid valve, and are found in patients with catheters or pacemaker wires or with history of IV drug abuse. Papillary fibroelastomas of the PV are rare benign tumors with speckled appearance because of cavities that result in echolucencies. The tumors are attached to the endocardium with a short stalk.
  • In this case, a healthy patient with a history of dyspnea and fatigue underwent excision of a PV papillary fibroelastoma. The relationship of the mass to the PV was investigated with 3-dimensional transesophageal echocardiography in the midesophageal RV inflow–outflow view. In a full-volume data set, multiplane cropping axes were rotated to investigate whether any of the 3 PV cusps were attached to the mass. The typical echolucency of these lesions was also imaged. The mass was resected uneventfully.
  • The anterior location of the PV and its proximity to the chest wall makes transthoracic echocardiography the ideal noninvasive imaging modality, but abnormal body habitus may decrease spatial resolution. With the use of transesophageal echocardiography in upper esophageal aortic arch short-axis views, midesophageal RV inflow–outflow or transgastric basal RV views, a full-volume data set can be acquired to allow detailed imaging of all PV cusps.
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DISCLOSURES

Name: Zineb Rebaine, MD.

Contribution: This author was a senior anesthesia resident assisting with the management of the current case and helped in the preparation of the manuscript.

Attestation: Zineb Rebaine approved the final manuscript and attests to the integrity of the original data.

Name: Christine Watremez, MD, PhD.

Contribution: This author helped prepare the video clips.

Attestation: Christine Watremez approved the final manuscript and attests to the integrity of the original data.

Name: Gebrine El Khoury, MD.

Contribution: This author was the attending cardiac surgeon responsible for the surgical management of the current case.

Attestation: Gebrine El Khoury approved the final manuscript and attests to the integrity of the original data.

Name: Mona Momeni, MD, PhD.

Contribution: This author was the attending anesthesiologist responsible for the management of the current case. She helped write the manuscript and prepare the video clips.

Attestation: Mona Momeni approved the final manuscript and attests to the integrity of the original data.

This manuscript was handled by: Martin London, MD.

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REFERENCES

1. Reynen K. Frequency of primary tumors of the heart. Am J Cardiol. 1996;77:107
2. Edwards FH, Hale D, Cohen A, Thompson L, Pezzella AT, Virmani R. Primary cardiac valve tumors. Ann Thorac Surg. 1991;52:1127–31
3. Gowda RM, Khan IA, Nair CK, Mehta NJ, Vasavada BC, Sacchi TJ. Cardiac papillary fibroelastoma: a comprehensive analysis of 725 cases. Am Heart J. 2003;146:404–10
4. Ngaage DL, Mullany CJ, Daly RC, Dearani JA, Edwards WD, Tazelaar HD, McGregor CG, Orszulak TA, Puga FJ, Schaff HV, Sundt TM III, Zehr KJ. Surgical treatment of cardiac papillary fibroelastoma: a single center experience with eighty-eight patients. Ann Thorac Surg. 2005;80:1712–8
5. Hakim FA, Aryal MR, Pandit A, Pandit AA, Alegria JR, Kendall CB, Click RL. Papillary fibroelastoma of the pulmonary valve—a systematic review. Echocardiography. 2014;31:234–40
6. Habib G, Hoen B, Tornos P, Thuny F, Prendergast B, Vilacosta I, Moreillon P, de Jesus Antunes M, Thilen U, Lekakis J, Lengyel M, Müller L, Naber CK, Nihoyannopoulos P, Moritz A, Zamorano JLESC Committee for Practice Guidelines. . Guidelines on the prevention, diagnosis, and treatment of infective endocarditis (new version 2009): the Task Force on the Prevention, Diagnosis, and Treatment of Infective Endocarditis of the European Society of Cardiology (ESC). Endorsed by the European Society of Clinical Microbiology and Infectious Diseases (ESCMID) and the International Society of Chemotherapy (ISC) for Infection and Cancer. Eur Heart J. 2009;30:2369–413
7. Kelly NF, Platts DG, Burstow DJ. Feasibility of pulmonary valve imaging using three-dimensional transthoracic echocardiography. J Am Soc Echocardiogr. 2010;23:1076–80

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