Pentacuspid aortic valve diagnosed by transoesophageal echocardiography

Abstract

Congenital aortic valve anomalies are quite a rare finding in echocardiographic examinations. A case of a 19 year old man with a pentacuspid aortic valve without aortic stenosis and regurgitation, detected by transoesophageal echocardiography, is presented. (Heart 2000;84:e9) Keywords: pentacuspid aortic valve; echocardiography Monocuspid, bicuspid or quadricuspid aortic valves are a rare congenital malformation of the aortic valve.1 However, to our knowledge there has been no previous report of a pentacuspid aortic valve in the literature. Although transthoracic echocardiography can delineate aortic valve structure, image quality may be suboptimal in some cases. In these cases, transoesophageal echocardiography may be required to clarify the aortic valve structure and function. We report a case of a pentacuspid aortic valve diagnosed by transoesophageal echocardiography. Case reportA 19 year old man was admitted to our hospital with palpitations. He had no syncope, chest pain, cyanosis, dyspnoea, orthopnoea, paroxysmal nocturnal dyspnea, oedema, history of rheumatic fever, or recent fever. He was taking no medications. His blood pressure was 105/70 mm Hg, and his pulse was 94 beats per minute. Cardiac examination revealed a normal S1 and S2 without S3 and S4. A grade I-II/VI systolic ejection murmur was heard at the left sternal border. His ECG revealed normal sinus rhythm and his chest x ray was also normal. Additionally, his routine blood and urine analyses were normal. However, transthoracic echocardiography showed mitral valve prolapse and suspicion defect imaging in the interatrial septum. The structure of the interatrial septum and interatrial shunt could not be adequately assessed because of suboptimal imaging quality. A tricuspid aortic valve with normal structure and function was seen. Transoesophageal echocardiography was performed and revealed a patent foramen ovale, mitral valve prolapse with mitral regurgitation (I/IV), and a pentacuspid aortic valve with three relatively equal cusps and two smaller cusps (fig 1). In spite of the diVerent aortic valve structure, no aortic stenosis and regurgitation was seen. DiscussionThe aortic valve is derived from mesenchymal swellings in the aortic trunk by the fourth week of gestation.2 Normally, three outgrowths bulge into the lumen of the great vessel and develop into three semilunar cusps that coapt in the centre of the valve orifice. It has been suggested that an abnormal number of cusps result from developmental changes in the early stages of truncal separation.3 Case reports of quadricuspid aortic valve describe patients ranging in age from 30-70 years.4 Aortic stenosis is rare, but approximately 50% of patients with quadricuspid aortic valve have aortic regurgitation. 4 Our patient was much younger, and we did not detect aortic stenosis or aortic regurgitation probably because these complications had not had time to develop yet. Initially, we reported a tricuspid aortic valve upon transthoracic echocardiography. Because the two supernumeracy ...

Diagnosis of Pentacuspid Aortic Valve with Severe Regurgitation Using Three‐Dimensional Transesophageal Echocardiography

“…In differential diagnosis, pseudo‐PAV may be seen secondary to bacterial endocarditis or rheumatic valvulitis . Physical and laboratory evaluation found no evidence of these in our patient at the time of admission to the hospital.…”

“…Normally, three outgrowths bulge into the lumen of the great vessel and develop into three semilunar cusps that coapt in the center of the valve orifice. It has been suggested that an abnormal number of cusps is caused by developmental changes in the early stages of truncal separation . Embryologic extrapolation offers 3 possible critical phases when supernumerary cusped aortic valves can be formed .…”

“…4 Yates, Bogers, Kamata, Kuroki, and Cemri et al and Wang have all reported patients with PAV. [1][2][3][5][6][7] In some cases where TTE is unable to provide adequate information on PAV due to poor acoustic window or angle, 3DTEE may be an effective alternative. Preoperative imaging results of TTE and TEE are comparable to intraoperative findings.…”

“…It has been suggested that an abnormal number of cusps is caused by developmental changes in the early stages of truncal separation. 7 Embryologic extrapolation offers 3 possible critical phases when supernumerary cusped aortic valves can be formed. 2 Supernumerary prevalvular pads in the truncus evolve into supernumerary aortic cusps.…”

Unicuspid Aortic Valve

“…Although reported infrequently, the natural history of unicuspid aortic valve is poorly understood. Developmental abnormalities of aortic valve cusps in decreasing order of frequency include bicuspid (0.9%-1.3%), unicuspid (0.02%), quadricuspid (0.008%-0.043%), and pentacupsid aortic valves (Cemri, 2000). Decreasing cusp number in the congenitally abnormal aortic valve has shown increasing male predilection, earlier valve failure and aggressive pathological changes compared to normal tricuspid aortic valve (Collins MJ, 2008).…”