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Utility of Combined Assessment With 2- and 3-Dimensional Transesophageal Echocardiography for Acommissural Unicuspid Pulmonic Valve With Severe Pulmonic Valve Stenosis

Kuroda, Masataka MD, PhD*; Maeda, Takayasu MD; Usuda, Iwao MD; Mita, Norikatsu MD, PhD; Kagaya, Shin MD, PhD; Miyoshi, Sohtaro MD, PhD; Saito, Shigeru MD, PhD*; Yamashiro, Masahito MD

doi: 10.1213/XAA.0000000000000790
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From the *Department of Anesthesiology, Gunma University Graduate School of Medicine, Maebashi, Japan

Departments of Anesthesiology, Respiratory Center, Kumagaya, Japan.

Surgery, Cardiovascular Division, Saitama Prefectural Cardiovascular and Respiratory Center, Kumagaya, Japan.

Accepted for publication January 29, 2018.

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.

Address correspondence to Masataka Kuroda, MD, PhD, Department of Anesthesiology, Gunma University Graduate School of Medicine, 3-39-22 Showa-machi, Maebashi 8511, Japan. Address e-mail to m.kurohyou@gmail.com.

A 69-year-old woman was scheduled for pulmonic valve (PV) replacement for severe PV stenosis, closure of a large secundum atrial septal defect (ASD), and tricuspid valve annuloplasty for moderate tricuspid regurgitation secondary to annular dilation. Preoperative 2-dimensional (2D) transthoracic echocardiography revealed severe PV stenosis due to severely thickened leaflets and poststenotic dilation of the main pulmonary artery (PA). Cardiac catheterization demonstrated mild pulmonary hypertension with a mean PA pressure of 26 mm Hg. Consent for publication of this article has been obtained from the patient.

Intraoperative transesophageal echocardiography (TEE) was performed using a 3-dimensional (3D) echocardiographic matrix-array probe (X7-2t transducer; Philips Healthcare, Andover, MA). The midesophageal (ME) 4-chamber view with color-flow Doppler confirmed a large secundum ASD and a severely dilated right ventricle (RV) (Supplemental Digital Content 1, Video Clip 1A, http://links.lww.com/AACR/A190). The PV and surrounding structures were imaged.1,2 The probe was withdrawn from the ME 4-chamber view, resulting in the ME ascending aorta short-axis (SAX) view, which demonstrated the usual location of the PV in the left-anterior field, distant from the aortic root (Supplemental Digital Content 1, Video Clip 1B, http://links.lww.com/AACR/A190). Rotating the multiplane angle to 89° and turning the probe counterclockwise from this view, the long-axis view of the PV and surrounding structures was obtained (Figure 1A; Supplemental Digital Content 1, Video Clip 1C, http://links.lww.com/AACR/A190). With color-flow Doppler (Figure 1B; Supplemental Digital Content 1, Video Clip 1D, http://links.lww.com/AACR/A190), turbulent flow in the PA suggested pulmonic stenosis; however, the exact location of stenosis was uncertain due to acoustic shadowing. Next, the PV and transpulmonic valve pressure gradient were interrogated in the transgastric (TG) RV inflow–outflow view at a multiplane angle of 108° (Figure 1C, D; Supplemental Digital Content 2, Video Clip 2, http://links.lww.com/AACR/A191).1–3 There was systolic “doming” of the PV leaflet(s), flow acceleration originating just below the PV leaflet tips, a high trans-PV pressure gradient, and absence of RV outflow tract stenosis. For further detailed assessment of the PV, a real-time 3D TEE view was obtained with a wide-angle 3D acquisition mode from the ME aortic valve SAX view at 45°, and rotated to display an en face view of the PV from the PA side (Supplemental Digital Content 3, Video Clip 3A, http://links.lww.com/AACR/A192).1 We used multiplanar reconstruction of real-time 3D data set to assess the PV anatomy (Figure 2; Supplemental Digital Content 3, Video Clip 3B, http://links.lww.com/AACR/A192). The combined 2D and 3D echocardiographic examination identified an acommissural, unicuspid PV with severe stenosis. The 3D en face view of the PV from the PA side was consistent with the surgical view and excised specimen (Figure 3).

Figure 1.

Figure 1.

Figure 2.

Figure 2.

Figure 3.

Figure 3.

The PV was replaced with a bioprosthetic valve, tricuspid ring annuloplasty was performed, and the ASD was closed with autologous pericardium.

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DISCUSSION

PV stenosis accounts for 7%–10% of cases of congenital heart disease.2 In 95% of cases, it may be isolated or associated with other congenital cardiac anomalies, such as tetralogy of Fallot (most common), double-outlet RV, atrioventricular canal defect, or Noonan syndrome, a complex of congenital heart defects due to an autosomal dominant disorder, while in the remaining 5% it is acquired, secondary to rheumatic, carcinoid, or infective endocarditis.2,4 Congenitally abnormal PVs may present as unicuspid (acommissural or unicommissural, dome shaped), bicuspid, tricuspid (dysplastic), and quadricuspid.4 Subpulmonary obstruction,2,3 supravalvular stenosis, and double-chambered RV2 may mimic PV stenosis.

Echocardiography, cardiac magnetic resonance imaging, and computed tomography are useful imaging modalities for the diagnosis of PV stenosis. With TEE, ME views demonstrate doming valve leaflets typically associated with PV stenosis, while TG and upper esophageal views allow more accurate measurement of pressure gradients using spectral Doppler.1–3 Visualization of the PV by TEE can be challenging because of its distant anterior location relative to the probe position and thinner leaflets compared to the aortic valve.2

In our case, TG imaging and spectral Doppler assessment were useful for visualizing the PV leaflet anatomy and origin of flow acceleration avoiding the acoustic shadowing from calcium deposits in the aortic and pulmonary roots. Intraoperative 3D TEE and multiplanar reconstruction helped understand the unusual PV anatomy: (1) the en face view from the PA side showed an acommissural unicuspid PV; (2) the long-axis plane revealed leaflet “doming,” similar to what was imaged in the 2D TG view; and (3) the SAX plane enabled measurement of the stenotic area. In case of inadequate imaging, epicardial echocardiography is useful for the assessment of PV.

In conclusion, comprehensive assessment with 2D and 3D TEE is necessary for the preoperative assessment of congenital unicuspid PVs with severe pulmonic valvular stenosis.

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DISCLOSURES

Name: Masataka Kuroda, MD, PhD.

Contribution: This author helped conduct the study, collect and analyze the data, and prepare the manuscript.

Name: Takayasu Maeda, MD.

Contribution: This author helped prepare the manuscript.

Name: Iwao Usuda, MD.

Contribution: This author helped prepare the manuscript.

Name: Norikatsu Mita, MD, PhD.

Contribution: This author helped prepare the manuscript.

Name: Shin Kagaya, MD, PhD.

Contribution: This author helped prepare the manuscript.

Name: Sohtaro Miyoshi, MD, PhD.

Contribution: This author helped prepare the manuscript.

Name: Shigeru Saito, MD, PhD.

Contribution: This author helped prepare the manuscript.

Name: Masahito Yamashiro.

Contribution: This author helped conduct the study and prepare the manuscript.

This manuscript was handled by: Nikolaos J. Skubas, MD, DSc, FACC, FASE.

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REFERENCES

1. Hahn RT, Abraham T, Adams MSAmerican Society of Echocardiography; Society of Cardiovascular Anesthesiologists. Guidelines for performing a comprehensive transesophageal echocardiographic examination: recommendations from the American Society of Echocardiography and the Society of Cardiovascular Anesthesiologists. Anesth Analg. 2014;118:2168.
2. Russell IA, Rouine-Rapp K, Stratmann G, Miller-Hance WCCongenital heart disease in the adult: a review with internet-accessible transesophageal echocardiographic images. Anesth Analg. 2006;102:694723.
3. Kurup V, Perrino A Jr, Barash P, Hashim SWInfundibular pulmonary stenosis. Anesth Analg. 2007;104:507508.
4. Waller BF, Howard J, Fess SPathology of pulmonic valve stenosis and pure regurgitation. Clin Cardiol. 1995;18:4550.

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