Quadricuspid Aortic Valve with Ruptured Sinus of Valsalva Aneurysm: a Case Report

Huang, Shuran; Liu, Xiaolong; Sun, Zhanguo

doi:10.21470/1678-9741-2020-0735

Shuran Huang

Department of Intensive Care Unit, Affiliated Hospital of Jining Medical University, Shandong, People’s Republic of China.

Xiaolong Liu

Department of Medical Imaging, Affiliated Hospital of Jining Medical University, Shandong, People’s Republic of China.

Zhanguo Sun Correspondence Address: Zhanguo Sun, https://orcid.org/0000-00001-8040-8986 Department of Medical Imaging, Affiliated Hospital of Jining Medical University, No. 89, Guhuai Road, Jining City, Shandong, People’s Republic of China, Zip Code: 272029, E-mail: yingxiangszg@163.com

Department of Medical Imaging, Affiliated Hospital of Jining Medical University, Shandong, People’s Republic of China.

https://orcid.org/0000-0001-8040-8986

This study was carried out at the Affiliated Hospital of Jining Medical University, Shandong, People’s Republic of China.

Correspondence Address: Zhanguo Sun, https://orcid.org/0000-00001-8040-8986 Department of Medical Imaging, Affiliated Hospital of Jining Medical University, No. 89, Guhuai Road, Jining City, Shandong, People’s Republic of China, Zip Code: 272029, E-mail: yingxiangszg@163.com

No conflict of interest.

Figures (3)
Tables (3)

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Fig. 1
A) Subcostal short axis view of transthoracic echocardiogram showing thickened sinus of the accessory aortic cusp (arrow) with a windsock (four-point star) deformity and a shunt from right-aortic cusp to right atrium during systole and diastole by color Doppler. B) Continuous wave Doppler showing the shunt from left to right, most evident during diastole and attenuated during systole; the velocity of the shunt was 5.7 m/s. AO=aortic root; LA=left atrium; RA=right atrium; RV=right ventricle

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Fig. 2
A) The right atrium has lesser amount of contrast material than the aorta, allowing visualization of a jet from the aorta to the right atrium though the windsock (four-point star). B) Multiplanar reconstruction in diastole by computed tomography angiography showing thickened aortic leaflets, especially the accessory one. A windsock (four-point star) developed from the accessory sinus. C) Virtual intra-aortic endoscopy image showing a quadricuspid aortic valve with three equal-sized cusps (1-3) and a smaller accessory cusp (4); the sinus of Valsalva aneurysm originated from the accessory sinus (arrow). D) Volume rendering image showing normal coronary arteries and the windsock (four-point star) with a shunt (arrow). 1=right coronary sinus; 2=left coronary sinus; 3=non-coronary sinus; 4=accessory sinus; LAD=left anterior descending artery; LCX=left circumflex coronary artery; RCA=right coronary artery

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Fig. 3
Timeline of the diagnosis and treatment process in the present case. CTA=computed tomography angiography; ECG=electrocardiography; QAV=quadricuspid aortic valve; SVA=sinus of Valsalva aneurysm; TTE=transthoracic echocardiography

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Abbreviations, acronyms & symbols 2D = Two-dimensional QAV = Quadricuspid aortic valve 3D = Three-dimensional RA = Right atrium AO = Aortic root RCA = Right coronary artery AR = Aortic regurgitation RV = Right ventricle CC = Cardiac catheterization RVAT = Right vertical infra-axillary thoracotomy CTA = Computed tomography angiography RVOT = Right ventricular outflow tract ECG = Electrocardiography SVA = Sinus of Valsalva aneurysm LA = Left atrium TEE = Transesophageal echocardiography LAD = Left anterior descending artery TTE = Transthoracic echocardiography LCX = Left circumflex coronary artery

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Table 1
Summary of previously reported cases of QAV associated with ruptured SVA (including the present case).

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Authors' roles & responsibilities SH Substantial contributions to the conception or design of the work; and acquisition and analysis of data for the work; drafting the work; final approval of the version to be published XL Substantial contributions to the conception or design of the work; and acquisition and analysis of data for the work; final approval of the version to be published ZS Substantial contributions to the conception or design of the work; and analysis and interpretation of data for the work; final approval of the version to be published

Abbreviations, acronyms & symbols
2D	= Two-dimensional	QAV	= Quadricuspid aortic valve
3D	= Three-dimensional	RA	= Right atrium
AO	= Aortic root	RCA	= Right coronary artery
AR	= Aortic regurgitation	RV	= Right ventricle
CC	= Cardiac catheterization	RVAT	= Right vertical infra-axillary thoracotomy
CTA	= Computed tomography angiography	RVOT	= Right ventricular outflow tract
ECG	= Electrocardiography	SVA	= Sinus of Valsalva aneurysm
LA	= Left atrium	TEE	= Transesophageal echocardiography
LAD	= Left anterior descending artery	TTE	= Transthoracic echocardiography
LCX	= Left circumflex coronary artery

Author	Age (years)	Sex	Symptoms	Type of QAV	AR	Origin of SVA	Ruptured into	Diagnostic method	Operation
Unger P[²2 Unger P, Preumont N, Stoupel E. Images in cardiology. Ruptured sinus of Valsalva aneurysm with right ventricular obstruction, quadricuspid aortic valve, and ventricular septal defect. Heart. 2000;84(4):424. doi:10.1136/heart.84.4.424. https://doi.org/10.1136/heart.84.4.424... ]	41	Male	Exertional dyspnea and peripheral edema for 8 days	Not described	Mild	Right sinus	RVOT	TTE	Not described
Aggarwal SK[³3 Aggarwal SK, Lingan A, Reddy KK, Swamy M, Iyer VR, Srivatsa SS. Quadricuspid aortic valve with ruptured sinus of valsalva aneurysm to the right atrium. Echocardiography. 2009;26(8):977-9. doi:10.1111/j.1540-8175.2009.00930.x. https://doi.org/10.1111/j.1540-8175.2009... ]	32	Male	Sudden onset of chest pain 5 months earlier followed by exertional dyspnea	Type A	Mild to moderate	Right anterior non-coronary sinus	RA	2D and 3D TTE, CC	Correction of SVA
Yang EH[⁴4 Yang EH, Rawal M, Pillutla P, Criley JM. Quadricuspid aortic valve with sinus of Valsalva rupture. Congenit Heart Dis. 2011;6(2):170-4. doi:10.1111/j.1747-0803.2010.00467.x. https://doi.org/10.1111/j.1747-0803.2010... ]	22	Female	Gradually worsening dyspnea on exertion, orthopnea, and cough for 2 weeks	Type B	Severe	Right sinus	RA	TTE	Resection of the SVA; aortic valve replacement
Akerem Khan SK[⁵5 Akerem Khan SK, Tamin SS, Burkhart HM, Araoz PA, Young PM. Quadricuspid aortic valve with ruptured sinus of Valsalva. Cardiol Young. 2013;23(1):154-6. doi:10.1017/S104795111200042X. https://doi.org/10.1017/S104795111200042... ]	24	Female	Intermittent chest pain, palpitations, and progressive dyspnea on exertion for several weeks	Not described	Not described	Right anterior non-coronary sinus	RA	TTE, TEE,	Correction of SVA (via median sternotomy)
	24	Female		Not described	Not described	Right anterior non-coronary sinus	RA	CTA	Correction of SVA (via median sternotomy)
Present case	61	Male	Chest distress and shortness of breath for 7 months; symptom aggravation for 10 days	Type B	Mild	Right anterior non-coronary sinus	RA	TTE, CTA	Repaired SVA (via RVAT)

Authors' roles & responsibilities
SH	Substantial contributions to the conception or design of the work; and acquisition and analysis of data for the work; drafting the work; final approval of the version to be published
XL	Substantial contributions to the conception or design of the work; and acquisition and analysis of data for the work; final approval of the version to be published
ZS	Substantial contributions to the conception or design of the work; and analysis and interpretation of data for the work; final approval of the version to be published

[1] This study was carried out at the Affiliated Hospital of Jining Medical University, Shandong, People’s Republic of China.

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Quadricuspid Aortic Valve with Ruptured Sinus of Valsalva Aneurysm: a Case Report

Abstract