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We present the case of a 64 year old man, previously of good health, who presented in November 1995 with a short history of presyncope and one witnessed episode of syncope. On admission he was clinically in complete heart block with a ventricular rate of 40 beats/min but had a well maintained blood pressure at 150/80 mm Hg. Auscultation revealed a soft systolic murmur in the aortic area. The remainder of the examination was unremarkable. Resting ECG confirmed complete AV block with a slow regular broad complex ventricular escape rhythm. A Paragon III (Pacesetter, Coventry, UK) dual chamber pacemaker was subsequently implanted with complete resolution of cardiac symptoms.
He remained well for three months when he presented with sudden onset dyspnoea in January 1996. Further examination at this time revealed signs of pulmonary congestion and a cardiac murmur throughout systole and diastole that had changed in character from the previous evaluation. There were no peripheral signs of infective endocarditis. Initial management with intravenous diuretics resulted in rapid clinical improvement and allowed the following sequence of investigations:
ECG confirmed normal AV sequential pacing.
Transthoracic echocardiography revealed a left ventricle of normal dimension and function. A subaortic membrane of uncertain aetiology was seen and appeared to be attached to the interventricular septum. Aortic regurgitation was confirmed.
Transoesophageal echocardiography revealed an abnormal aortic valve with a large apparently cystic mass under the aortic root, attached inferiorly to the interventricular septum (fig1). This structure was felt to be a large aneurysm of the right coronary sinus extending into the interventricular septum.
Cardiac catheterisation demonstrated a normal coronary arterial tree but confirmed the presence of a large saccular aneurysm of the right coronary sinus, shown best in the lateral position. Severe aortic regurgitation was seen.
The patient was referred for cardiac surgery and at operation a large aneurysm of the right coronary sinus invading the interventricular septum was confirmed. The walls of the sac were intact. The aortic valve itself was tricuspid and normal except that the right coronary cusp was noticeably redundant and was sagging into the left ventricular cavity (fig 1). The aortic valve and aneurysm were excised and the valve replaced with a Carbomedics mechanical prosthesis (East Crawley, West Sussex, UK) under full cardiopulmonary bypass. The patient made an uncomplicated recovery and remains well.
Sinus of Valsalva aneurysm is an uncommon condition usually caused by a congenital defect of continuity between the aortic media and the aortic fibrous ring. Secondary causes are rare but include bacterial endocarditis, syphilis, tuberculosis, and Behçet’s disease.1 ,2 Surgical treatment is required in nearly all cases with the long term results being favourable; one large series showed a 95% 20 year survival.3
The classic presentation of a ruptured sinus of Valsalva aneurysm is described as chest pain of sudden onset associated with the clinical findings of a continuous “to and fro” murmur best heard at the base of the heart. As the aneurysm often ruptures into the right heart structures (right atrium or ventricle), right sided cardiac failure often follows quickly. The pattern of presentation does however vary widely, and unruptured aneurysms are often “silent” or present only with vague non-specific symptoms. However, cerebrovascular embolisation4 and angina5 have been reported. In our patient the unruptured aneurysm had extended into the interventricular septum and, presumably through compression, had compromised normal AV nodal/His bundle function and resulted in complete heart block. Case reports of the latter association are very rare, and to our knowledge only one case of an antemortem diagnosis has been reported previously.6 Therefore, sinus of Valsalva aneurysm deserves to be considered as a rare cause of complete heart block.