Discussion
Despite recent advancement in imaging modalities, the incident of QAV,
either alone or with associated malformations has remained exceedingly a
rare finding [1,2]. The categorization of QAV based on structure has
been well documented by earlier pioneers [3]. Commonly, QAV is
incidentally found amongst clients who present with diastolic murmur as
the only chief concern to seek medical attention. On the other hand, QAV
can present with various symptoms, however, central-jet aortic valve
regurgitation predominates. Complication of aortic regurgitation due
malcoaptation of QAV include: dilatation of aortic root, Sinus of
Valsalva and ascending aorta. Others include significant enlargement of
left ventricle and left atrium with subsequent conduction abnormalities
which include either atrial fibrillation or RBBB [3,4].
Our reported case is notably peculiar and worthy reporting not because
of the rareness of QAV alone, but the inability to elicit significant
cardiac chamber and great vessel enlargement despite one-year duration
of progressive exertional chest tightness (NYHA class-III-IV). The
progressive reduction in ejection fraction in such a young and
supposedly energetic young man rendered him incapacitated, as he was
unable to participate in strenuous activities. Additionally, untimely
valve replacement risked severe and sudden global hypoperfusion during
strenuous activities with subsequent sudden death [4]. The
unpredictability of age at which QAV may manifest severe clinical
symptoms despite insignificant great-vessel and cardiac remodeling
underscores the incomprehensive study of QAV at molecular level due to
its rareness.
Similar to other facilities world over, due to patients age we opted to
replace the native valve with a mechanical prosthetic valve via open
heart surgery and avoid repeat of surgery due to bioprosthetic valve
deterioration [5,6]. Although repair of native QAV has been
reported, our facility favored replacement due to lack of reliable data
on the longevity of repaired cases, and avoid unwanted repeat of
surgery. A month post-surgery, the patient was reviewed and had reverted
to (NYHA class-I). He was maintained on life-long anticoagulants with
reviews scheduled at his local health facility.
Acknowledgement: none.
Funding: none
Conflict of interest ; none declared.
Patient’s consent : written consent obtained.
Institutional Ethics Committee approval : waiver obtained.
References
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Figure legends:
1.Figure.1. Imaging results: A, B and D; Transoesophageal
echocardiography (TEE) short axis view showing Quadricuspid aortic valve
structure(3-D) and regurgitation. E, TEE long axis view showing severe
regurgitation. C; Computed tomography showing valvular structure, and F;
intraoperative findings.
2. Transoesophageal echocardiography motion showing valve structure and
regurgitation;
Video.1 and Video.2.