Corresponding author:
He Huang, Department of cardiology, West China Hospital of Sichuan
University, 37 Guo Xue Xiang, Chengdu, Sichuan, 610041, China, +86 028
8542
2355,huanghe@wchscu.cn
Declaration of interst: We have no conflicts of interest to
disclose.
Abstract : We present a case of a 75-year-old woman with typical
myocardial infarction, however coronary angiogram was negative.
Echocardiography identified the rare cause of chest pain, as a mobile
mass of aortic valve was found to obstruct the coronary ostium.
histopathology revealed a papillary fibroelastoma (PFE). Chest pain was
relieved after surgical resection of the mass.
KEYWORDS: myocardial infarction,
papillary fibroelastoma, aortic valve, echocardiography
INTRODUCTION: For patients clinically diagnosed acute myocardial
infarction, but no significant stenosis of coronary arteries, the rare
causes, such as cardiac valve tumors, should be considered. We report an
unsual condition, in which obstruction of the coronary ostium by aortic
PFE caused acute myocardial infarction.
CASE PRESENTATION: A 75-year-old woman, with a history of type 2
diabetes and hypertension for 10 years, and atrial fibrillation for 2
years, presented with chest pain for 12 hours, accompanying with
dyspnea, sweating, and radiating pain in the shoulder and back. Physical
examination revealed a standard heart rate and blood pressure(heart rate
68 beats/min, blood pressure 106/71mmHg), and no murmur was detected.
Electrocardiogram showed sinus rhythm, ST-segment depression in V2 to
V6、 I、 AVL、 II、 III、 AVF and ST-segment elevation in AVR. The CK-MB rose
to 149.8 ng/mL (reference range <2.88 ng/mL) and troponin T
increased up to 4852 ng/L (reference range <14 ng/L).
Coronary angiography, performed on the first day after admission,
revealed that the left and right coronary arteries were not
significantly narrow (Figure 1). During angiography, the patient
developed shortness of breath and palpitations shortly, and ECG
monitoring showed bradycardia and decreased blood pressure. CT angiogram
on the second day after admission showed nodular filling defects in the
aortic valve (Figure 2). At the same day, the transthoracic
echocardiography (image not clear) revealed a mobile mass (size,
10mm×6mm) of aortic valve. On the third day after admission,
transesophageal echocardiography confirmed an isoechoic, regular,
well-defined clump (size, 18mm × 12mm) on the left coronary cusp,
suggesting of PFE (Figures 3 and 4, movie S1).
On the sixth day after admission, the aortic valve mass was removed
under general anesthesia, extracorporeal circulation, along with aortic
annuloplasty, and atrial fibrillation bipolar radiofrequency ablation.
During the operation, a “sea anemone” pedicled clump (size, 18mm ×
12mm), arising from the endocardium of the left coronary cusp very close
to the commissure between the left and right coronary cusps, was
suspected to intermittently block the left coronary opening (Figure 5).
Postoperative pathology revealed PFE (Figure 6). The symptoms of chest
pain was alleviated significantly after operation. No abnormality was
found on the postoperative echocardiography and chest CT.
DISCUSSION: Papillary fibroelastoma is the most common primary
cardiac valve tumor.1 As the analysis of 21 Cases by
Ikegami and colleagues,2 Lesions are usually single,
multiple lesions can involve solitary or multiple cusps.
The clinical manifestations of fibroelastomas range from asymptom to
severe complications of embolism. Astonishingly, the tumor rarely cause
valvular regurgitation or dysfunction.3,4 Most
symptomatic cases show severe complications such as myocardial
infarction, stroke, sudden death, and syncope.1,5previous studies have shown these symptoms are related to either a
mechanical obstruction, or embolisation of either papillary
fibroelastoma fragments,or a thrombus formed on the tumour
itself.1,5,6
ACS is usually accompanied with evidence of coronary artery stenosis,
but in our case, no stenosis in coronary artery. We puzzled the reason
for ACS, and ECHO gave an answer. PFE was the reason for ACS.
Previously, Logan et al .1 also reported a
similar case about the cardiac papillary fibroelastomas (CPFs)
presenting as AMI.
For the circumstance that patients developed bradycardia and hypotension
during coronary angiography, presented with shortness of breath and
palpitations, nevertheless without obvious chest pain, we speculate the
mechanism as the passive movement of catheter to be advanced or
retreated, rotated, pushed in the vascular cavity. these intravascular
interventions may interfere the natural activities of papillary
fibroelastoma and facilitate pushing the tumor to block the right
coronary ostia , as Erdoes et al .6 presented in
their case. The primary blood supply to sinoatrial node is affected,
resulting in dysfunction of the sinoatrial node.
Gender-related morbidity of CPFs is a matter of debate. A retrospective
study of Innsbruck Medical University held that CPFs occured principally
in men.7 however, some medical literature has shown
more common in female,5 the patient in our case is
female.
Up to now, It has not been in agreement which coronary artery ostium is
the most involved or which aortic
valve CPFs predominately occur on.
Erdoes et al. 6 deemed the right coronary artery
(RCA) was involved in the majority of the cases. In a retrospective and
single-center study, Ikegami and colleagues2 reported
that the noncoronary cusp of the AV was involved most often, consistent
with the findings of Steger et al .7 However,
fatal AMI even cardiogenic sudden death caused by CPF in many case are
mainly reported in the left coronary valve.1,2 Besides
our case report, in report of Logan and colleagues,1CPF causing myocardial infarction was also on left coronary cusp.
anatomical factors may explain for this. The fibroelastoma located at
the left coronary valve is closer to the left coronary ostia and easier
to obstruct it, thereby seventy percent blood supply to heart is lacking
and fatal cardiovascular events occur. In addition, Ikegami et
al .2 also repored a rare cardiac arrest caused by a
CPF from left coronary cusp.
The advances in echocardiographic technology have resulted in CPFs’
detection in an increasing number.2 Echocardiography
is the primary modality for imaging intracardiac
disease.8 CPFs’ characteristics of echocardiography
imaging-small size,pedical or stalk attachment to endocardium, mobile
valvular/endocardial masses-make it likened to a sea
anemone,3,6,8,9 which can help to identify a tumor as
a PFE. Echocardiography has high value on diagnosing cardiac tumors,
simultaneously assessing valve function with a CPF attached and
measuring left ventricle function.
Fibroelastomas are easily detected and are well visualized at
echocardiography as their distinguishable ,macroscopic, characteristic
echocardiographic features, however they are usually not seen at CT or
MR imaging because they are small and are attached to moving
valves,8,9 which indicating echocardiography has an
advantage over CT or MR imaging in identifying Fibroelastomas.