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Aortic valve prolapse misdiagnosed as aortic sinus aneurysm in patients with
ventricular septal defect: analysis of the echocardiographic findings
Guobing Hu 1: MD, Fang Song 2: MD, Xiangming Zhu 1: MD.
All authors declare that they have no conflict of interest.
This article has been supported by funding from the First Affiliated Hospital of Wannan Medical
College Program: Grant No.WK2015ZF.
1: Department of Ultrasound, the First Affiliated Hospital of Wannan Medical College, Wuhu 241001,
Anhui, China
2: Department of Radiology, the Number Five People Hospital of Wuhu, Wuhu 241001, Anhui, China
Correspondence: Guobing Hu
Department of Ultrasound
the First Affiliated Hospital of Wannan Medical College
Wuhu 241001, Anhui, China
Tel: 00865535712869
Fax: 00865535738279
E-mail: [email protected]
Aortic valve prolapse misdiagnosed as aortic sinus aneurysm in patients with
ventricular septal defect: analysis of the echocardiographic findings
Abstract
Objective
To retrospectively analyze the preoperative echocardiographic findings of ventricular septal
defect (VSD) associated with aortic valve prolapse (AVP) in 9 patients who were misdiagnosed as
ventricular septal defect asscociated with aortic sinus aneurysm (ASA).
Methods
Between June 2005 and May 2015, 92 patients were diagnosed as VSD associated with ASA by
transthoracic echocardiography (TTE). Intraoperatively, 83 patients were confirmed as VSD
associated with ASA, 9 patients were confirmed as VSD associated with AVP. In this paper, we mainly
discuss the 9 patients who were misdiagnosed by TTE. All patients were performed with Philip IE33
echocardiograph.6 were males and 3 were females.
Results
Subarterial VSD was diagnosed in all patients by TTE and confirmed during surgery. Both color
and continuous Doppler showed continuous turbulence in the right ventricle outflow tract (RVOT) in all
patients. Aneurysm -like protrusions could be detected and diagnosed as AVP by two dimensional
TTE in RVOT in all patients. During surgery, all of the aneurysm -like protrusions were confirmed as
over enlarged right coronary cusps bulging into RVOT.
Conclusions
Occasionally, subaterial VSD associated with AVP would be misdiagnosed as subaterial VSD
associated with ASA by TTE. To reduce the misdiagnosis rate and provide a more precise
preoperative diagnosis, multisection, multi-angle observation of these diseases should be performed.
The ultrasonic doctors should also have a thorough hemodynamic knowledge.
Key words: aortic valve prolapse, ventricular septal defect, aortic sinus aneurysm, transthoracic
echocardiography, misdiagnosis.
Introduction
Subarterial VSD is frequently associated with AVP. Aortic sinus aneurysm can also be associated
with subarterial VSD. Previous studies of these diseases showed that echocardiography has become
an useful noninvasive technique for diagnosing subarterial VSD associated with AVP without the need
for cardiac catheterization [1], however a certain misdiagnosis rate could be made while using TTE[2],
and there is very little literature about the causes of echocardiographic misdiagnosis, therefore we
collected 9 patients who were diagnosed as subarterial VSD associated with ASA by TTE but later
confirmed as subarterial VSD associated with AVP in surgery. We retrospectively analyze the
preoperative echocardiographic findings in the 9 patients and hope that the misdiagnosis rate could be
reduced a more accurate preoperative diagnosis can been provided for clinical heart surgical
operation.
Methods
Between June 2005 and May 2015, 92 patients were diagnosed as subarterial VSD associated
with ASA by TTE. Intraoperatively, 83 patients were confirmed as subarterial VSD associated with
ASA, 9 patients were confirmed as subarterial VSD associated with AVP. In this paper, we mainly
discuss the 9 patients who were misdiagnosed by TTE. All patients were performed with Philip IE33
echocardiograph.6 were males and 3 were females. There were 6 women and 3 men, aged between 3
and 45 (mean 16.5). Auscultation revealed continuous murmur along the left sternal border in all
patients,
TTE was performed in all patients with an IE33 echocardiograph, A transducer of 3, 3.75, 5 was
used depending on the patient’s physique and the kind of echocardiograph used. All patients were
confirmed with operative findings. The comparison and analysis between the images of TTE and the
operative findings were made in the 9 patients.
Results
All of the 9 patients were diagnosed as subarterial VSD by TTE, and the intro-operative findings
confirmed the diagnosis. Both color and continuous Doppler showed continuous turbulence in the
RVOT in all patients, aneurysm -like protrusions were detected by two-dimensional TTE in RVOT in all
patients. Operative findings confirmed those aneurysm -like protrusions were over enlarged right
coronary cusps bulging into RVOT. Due to infective endocarditis, perforation of the right coronary cusp
was found in all patients. Other associated congenital heart diseases included double chambered right
ventricle, secondary atrial septal defect and patent ductus arteriosus. Aortic regurgitation could be
detected in various degrees in all patients by TTE.
Comparison between TTE diagnosis and operative findings in the nine patients (table 1)
Discussion
VSD is one of the most common congenital heart diseases. There are many subtypes of VSD [3],
among them, sub-arterial VSD is a unique subtype which is always seen in Asians [4,5]. Different from
other types of VSD, sub-arterial VSD is known for low tendency for spontaneous closure as well as
progressive AVP and aortic regurgitation due to: “venturi pressure effect” of the left to right shunt and
a lack of anatomical support of the aortic valve [6,7]. Open-heart surgery has been advocated as the
gold treatment of sub-arterial VSD. The introduction of echocardiography has made it possible to
diagnose the location and presence of VSD and aortic valve deformity noninvasively [8]. RCSA is
often associated with VSD [9], although we know that the echocardiographic criteria for diagnosis of
RCSA include:1) the root of the aneurysm is above the aortic annulus,2) the aneurysm is saccular,3)
continuous turbulence and high velocities could be detected by continuous wave Doppler distal to the
area of rupture,4) color flow shows mosaic turbulence across the ruptured aneurysm in real time [10].
We could still occasionally misdiagnose AVP as RCSA in subarterial VSD while using TTE.
In all patients, the size of subarterial VSD measured by TTE was smaller than that seen in surgery.
This is because the prolapsed coronary cusps were found tethered to the VSD margin, overlaid part of
the VSD and had reduced the size of the VSD [10]. In addition, the jet of VSD was not parallel to the
ultrasound beam [11]. Ultrasonic doctors should be aware of this phenomenon because it is very
common.
In all 9 patients, the pronounced distortion and translocation of aortic annulus resulting from the
lack of anatomical support due to large subarterial VSD made it difficult in judging the exact location of
the root of the aneurysm -like protrusions while using two –dimensional TTE. Valve excrescences can
always form on the surface of aortic valves as a result of infective endocarditis, they can appear not
only in the shape of nodular masses, but also in the shape of long strips [9, 10]. Excrescences could
be detected by TTE in all patients, TTE clearly displayed the excrescences floating around VSD,
covering the aortic annulus, making it even more difficult to demarcate the root of aneurysm -like
protrusions. We suggest that the continuity of the coronary cusp and the root of aneurysm -like
protrusion should be carefully observed while the aneurysm -like protrusions are suspected of
originating from the prolapsed coronary cusps, in addition, because the coronary cusp which
prolapses into RVOT through VSD is always enlarged, its movement is not as stable as that of a
normal coronary cusp in cardiac cycles.
Perforation of the aneurysm -like protrusions triggered by too thin coronary cusps and infective
endocarditis could be detected by TTE in all 9 patients, a continuous turbulent flow which was also the
typical characteristics of rupture of ASA could be detected in the RVOT by Continuous-wave Doppler
[11], in addition, auscultation revealed a continuous harsh murmur along the left sternal border in all
patients, so ultrasonic doctors tended to make the diagnosis of rupture of ASA. We should keep this in
mind that the continuous turbulent flow detected in RVOT is not unique to RASA.
Apart from the main causes of misdiagnosis mentioned above, other causes including: (1) The
irregular shape of VSD, the complex structure around VSD and the associated abnormalities such as
double-chambered right ventricle could also affect the diagnosis [12, 13]. (2) Inappropriate gain
adjustment of the ultrasound machine, poor imaging of two-dimensional TTE, and anamorphose of
color flow signal could also cause misdiagnosis [14, 15]. (3) Due to a lack of skill or understanding of
the disease, or limited experience, ultrasonic doctors only observed large VSD and failed to scan the
exact location of the aneurysm -like protrusion,
Conclusion
Sometimes it is very difficult to make an accurate diagnosis of subarterial VSD associated with
AVP while using TTE. To reduce misdiagnosis and to provide a precise preoperative diagnosis of
these diseases, multisection, multi-angle observation of the aneurysm -like protrusions should been
performed and the ultrasonic doctors should also have thorough hemodynamic knowledge.
Authors' Contributions
Concept/design: Guobing Hu; data analysis/interpretation: Fang Song, drafting article: Xiangming Zhu;
critical revision of article: Guobing Hu, approval of article：Guobing Hu, Fang Song, Xiangming Zhu.
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Figure 1. A. Continuous-wave Doppler showed a continuous turbulent flow during both systole
and diastole. B. The over enlarged right coronary cusp appeared to be bulbous and bulging into the
RVOT, valve excrescence could be displayed in the shape of small masses. C. Color Doppler showed
blood flow across large VSD during systole. D.RVOT view displayed perforation of right coronary
cusp.
Figure 2. A. Continuous-wave Doppler showed a continuous turbulent flow during both systole
and diastole. B. The excrescence appeared in the shape of long strips in the RVOT. C. The over
enlarged right coronary cusp appeared to be bulbous and bulging into the RVOT, perforation of right
coronary cusp could also be displayed. D Color Doppler showed blood flow across large VSD during
systole.
Figure 3. A. Continuous-wave Doppler showed a continuous turbulent flow during both systole
and diastole. B.RVOT view displayed perforation of right coronary cusp. C. The over enlarged right
coronary cusp appeared to be bulging into the RVOT, perforation of right coronary cusp could also be
displayed.
D
Color
Doppler
showed
blood
flow
across
large
VSD
during
systole..
RVOT=right ventricular outflow tract; LA=left atrium; RA=right atrium, LV=left ventricle, RV=right
ventricle; AVP=aortic valve prolapse. VSD=ventricular septal defect.