A very rare cause of aortic regurgitation: pentacuspid aortic valve

A 54-year-old man with no previous history presented with a 4-month history of progressive exertional dizziness associated with chest pain and dyspnoea. The patient had no syncope, palpitations, orthopnoea, or paroxysmal nocturnal dyspnoea. His blood pressure was 139/71 mmHg and his heart rate was 80 beats per minute. Lung auscultation revealed normal vesicular breathing with bilateral equal air entry and no added sounds. Cardiac examination revealed a normal S1 and S2 with a grade III diastolic murmur heard at the location of the aortic valve. The electrocardiogram revealed a normal sinus rhythm and increased R-wave amplitude in precordial leads V3–V6, which is consistent with left ventricular hypertrophy. Transthoracic echocardiography suspected aortic regurgitation, but due to poor image quality, he was referred for transoesophageal echocardiography (TOE). TOE showed a pentacuspid aortic valve (PAV), with moderately severe aortic insufficiency and non-coaptation leaflets during diastole (Figure 1 and Supplementary material online, Images and Videos S1–S4). It also revealed left ventricular hypertrophy with a mass of 312 g and an ejection fraction of 55%. Additional TOE findings are listed in Table 1. Coronary angiography revealed normal coronary arteries. The patient underwent aortic valve replacement due to the valvular findings and the presence of cardiac symptoms (chest pain and dyspnoea). Due to patient preference, valve replacement was done using a 24 mm ATS AP360 supra-annular mechanical valve. The PAV was confirmed intraoperatively. He recovered without complications and discharged on the 6th day post-operatively. Upon follow-up, patient remained asymptomatic and echocardiography showed a well seated prosthetic valve with normal occlude motion and mean gradient across valve 8 mmHg. PAV is rarely seen and reported in clinical practice; with the first case reported in 1923 by Simonds J.P. Since then, there have been at least eight reported cases to our knowledge.^1–3 The main complaint of progressively worsening exertional dizziness, as seen in this case, was not reported before.

Figure 1.

Transoesophageal echocardiography image of the pentacuspid aortic valve, short-axis view. Transoesophageal echocardiogram was performed on Phillips IE 33 system (Phillips medicalvsystems, USA) with S7-2t transoesophageal echocardiography transducer. This shows mid-oesophageal short-axis view in diastolic frame at the level of aortic with unequal sized pentacusps. There are three large cusps of equal size (marked yellow) and two relatively small sized cusps (marked blue). There is a central regurgitant orifice in diastole.

Table 1.

Transoesophageal echocardiography findings and aortic valve insufficiency quantification

Left ventricular internal systolic dimension	40 mm
Left ventricular internal diastolic dimension	56 mm
Interventricual septal thickness	13.1 mm
Left ventricular posterior wall thickness	12.8 mm
Jet width to left ventricular outflow tract ratio	65%
Pressure half time (limited accuracy due to coexistent diastolic dysfunction)	400 ms
Vena contracta	9.8 mm
Effective regurgitant orifice area	0.35 cm2
Regurgitant volume	51 mL
Ejection fraction	55%
Left ventricular outflow tract	25.7 mm
Sinus of Valsalva	35.2 mm
Sinotubular junction	31 mm
Proximal ascending aorta	37 mm
Mid-ascending aorta	37.3 mm

Supplementary material

Supplementary material is available at European Heart Journal - Case Reports online.