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Quadricuspid pulmonic valve and great pulmonary artery aneurysm by multimodality imaging

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Quadricuspid pulmonic valve and

great pulmonary artery aneurysm by

multimodality imaging

A 71-year-old man was admitted to our hospital with chest

tightness and progressive dyspnea. Physical examination

re-vealed normal left ventricular (LV) impulse, systolic and diastolic

murmur at the left sternal border.

Chest X-ray revealed enlarged contour of the vessel below

the aortopulmonary window (Fig. 1a). An aneurysmal dilation of

the main pulmonary artery and left pulmonary artery was

re-vealed through transthoracic echocardiography, and the right

pulmonary artery was seen slightly dilated (Fig. 1b). A

short-axis view of pulmonary valve in a higher intercostal window

demonstrated a quadricuspid anomalous valve (Fig.1c, Video

1). Moderate pulmonary stenosis (transvalvular peak velocity

of 3.3 m/s) and moderate regurgitation (Fig.1d, Video 2) were

seen through color Doppler flow imaging at the pulmonary

valve. Cardiac computed tomography and three-dimensional

volume-rendered computed tomography confirmed a

quadri-cuspid pulmonary valve with four equal-sized cusps, and great

aneurysm of the pulmonary artery trunk (73 mm) extended into

the left pulmonary artery (43 mm), while the right pulmonary

ar-tery was dilated (31 mm) (Fig. 2a–2d). Therefore, the diagnosis

of quadricuspid pulmonic valve complicated with pulmonary

artery aneurysm was made by multimodal imaging.

The quadricuspid pulmonary valve is a rare congenital

anom-aly with an incidence reported from 1 in 1000 to 1 in 20000 (1).

Pulmonary artery aneurysms are rare and infrequently

diag-nosed (2). Limited reports are available on the quadricuspid valve

in combination with pulmonary artery aneurysm (3, 4). Cardiac

computed tomography allowed an adequate characterization of

the valve morphology, and echocardiography provided the

in-formation about the function of the anomalous valve. This case

highlights the crucial role of multimodality imaging evaluation for

detailed noninvasive depiction of pulmonary valve disease.

Funding: The study was supported by National Key R&D Program of China (Grant Nos. 2018YFC0114600) and the National Natural Science Foundation of China (Grant Nos. 81727805, 81922033, 81401432).

Informed consent: The informed consent was obtained from this patient.

References

1. Davia JE, Fenoglio JJ, DeCastro CM, McAllister HA Jr, Cheitlin MD. Quadricuspid semilunar valves. Chest 1977; 72: 186-9.

2. Kreibich M, Siepe M, Kroll J, Höhn R, Grohmann J, Beyersdorf F. Aneurysms of the pulmonary artery. Circulation 2015; 131: 310-6. 3. Guijarro-Contreras A, Pardo-Martínez P, Cham M, Macaluso F,

Sanz-Salvo J. Quadricuspid Pulmonic Valve, Pulmonary Artery Aneurysm, and Apical Hypertrophic Cardiomyopathy: A Rare Combination. Circ Cardiovasc Imaging 2018; 11: e007698.

E-4

Figure 1. Chest X-ray revealed an enlarged contour of the vessel below the aortopulmonary window (a). Transthoracic echocardiographic image revealed aneurysmal dilation pf pulmonary artery trunk and left pulmonary artery (b). A short view of pulmonary valve obtained by transthoracic echocardiography showed a quadricuspid pulmonary valve (c). Color Doppler flow imaging demonstrated the moderate pulmonary insufficiency and systolic flow acceleration (d)

a

c

b

d

Figure 2. The computed tomography angiography (CTA) and three-dimensional volume-rendered computed tomography (3D VR CT). Axial image of the chest at the level of the bifurcation of the main pulmonary artery showed aneurysmal dilation of the main (73 mm) and left (43 mm) pulmonary arteries, while the right pulmonary artery was seen slightly dilated (31 mm) (a). Reconstructed CTA of the chest at the level of pulmonary valve demonstrated a quadricuspid pulmonary valve with four cusps of similar size (b). 3D VR CT confirmed that quadricuspid pulmonary valve had four cusps of similar size and showed the great aneurysm of the pulmonary artery intuitively (c and d)

a

c

b

(2)

Anatol J Cardiol 2021; 25: E-4-5 E-page Original Image

E-5

4. Nollen GJ, Kodde J, Beek AM, Res JC, van Rossum AC. Quadricus-pid pulmonary valve and left pulmonary artery aneurysm in an as-ymptomatic patient assessed by cardiovascular MRI. Neth Heart J 2013; 21: 196-8.

Video 1. A transthoracic echocardiography demonstrated the

quadricuspid pulmonary valve

Video 2. Color Doppler flow imaging indicated the moderate

pulmonary valve insufficiency and systolic flow acceleration

Ziming Zhang1,2 #, Yuman Li1,2 #, Bin Wang1,2 #, Li Zhang1,2,

Mingxing Xie1,2

1Department of Ultrasound, Union Hospital, Tongji Medical

College, Huazhong University of Science and Technology; Wuhan-China

2Hubei Province Key Laboratory of Molecular Imaging;

Wuhan-China

#These authors contributed equally to this manuscript.

Address for Correspondence: Mingxing Xie, MD, PhD, Department of Ultrasound,

Union Hospital, Tongji Medical College,

Huazhong University of Science and Technology; 1277 Jiefang Avenue,

Wuhan-China Phone: 86-27-85726430 E-mail: xiemx@hust.edu.cn

©Copyright 2021 by Turkish Society of Cardiology - Available online at www.anatoljcardiol.com

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