Abstract
The present report describes a rare case of aortic valve aneurysm without any vegetation with complete heart block. A 26-year-old man with severe acute aortic regurgitation was admitted to our admitted to our hospital. Transthoracic echocardiography showed right cusp aneurysm without any vegetations. Transoesophageal echocardiography confirmed these findings. Colour Doppler echocardiography revealed severe aortic regurgitation. For complete heart block, a transvenous permanent pacemaker was inserted as a first stage of treatment. Successful aortic valve replacement was performed as a second stage. The possible aetiology of this case is endocarditis.
BACKGROUND
Very few cases of aortic cusp aneurysm have been reported in the literature.1–7 To our knowledge, this is the first case of right cuspal aneurysm without any vegetation with complete heart block and probable aetiology of endocarditis. Our patient underwent aortic valve replacement, with a successful outcome.
CASE PRESENTATION
A 26-year-old man was admitted to our institute with the sole complaint of syncopal attacks for the past 3 months. There was no history of dyspnoea, chest pain and fever. He had no prior medical history and no past history of intravenous drug abuse or prolonged antibiotic treatment.
On clinical examination, he was of average built with no clubbing and cyanosis of fingers or toes. There were no enlarged lymph nodes, ascites or pedal oedema. His pulse rate was 40 beats/min, regular in rhythm, bounding with “water hammer” character. His blood pressure was 140/40 mm Hg and other clinical signs of severe aortic regurgitation were present. Cardiovascular examination revealed, on inspection, a prominent apical impulse with dancing carotids. On palpation, a heaving impulse was felt at the apex. Auscultation revealed normal S1 and S2 with early diastolic murmur at aortic area. There was no Austin Flint murmur and S3. There were no evidences of congestive cardiac failure. Neurological, respiratory and other systemic examination were unremarkable.
INVESTIGATIONS
Resting electrocardiography (ECG) showed complete heart block. Transthoracic two-dimensional and Doppler echocardiography was performed, and demonstrated a dilated left ventricle (left ventricular end diastolic dimension (LVEDD) was 6.65 cm, left ventricular end systolic dimension (LVESD) was 4.45 cm) with a grade 4 aortic regurgitation from a trileaflet aortic valve (figs 1 and 2). The aortic root was not dilated (aortic annulus 25 mm, sinus 32 mm, sinotubular junction 30 mm) but it showed a large cuspal aneurysm of the right aortic leaflet resulting in severe aortic regurgitation (fig 3). There was no evidence of vegetations on any valves, root abscess, fistulas, myocardial abscess or purulent pericarditis. No atrial or ventricular septal defects were seen. A transoesophageal echo was also performed, and it confirmed all the above findings.
Figure 1.
Transoesophageal short-axis view in systole showing the aneurysm of the right coronary cusp (R). Other structures are: LA, left atrium; RV, right ventricle; RA, right atrium; PA, pulmonary artery; TV, tricuspid valve; PV, pulmonary valve; N, non-coronary cusp of aortic valve; R, right coronary cusp of aortic valve; Av, aortic opening.
Figure 2.
Transoesophageal long-axis view in diastole showing the aneurysm of the right coronary cusp (RCC). Other structures are: LA, left atrium; LV, left ventricle; RV, right ventricle; MV, mitral valve; IVS, interventricular septum; SV, sinus of valsalva; AO, aorta.
Figure 3.
Transoesophageal short-axis view in diastole using colour Doppler, showing the eccentric aortic regurgitation jet (AR jet; marked by dashed lines). Other structures are: LA, left atrium; RV, right ventricle; RVOT, right ventricle outflow tract; PV, pulmonary valve; AV, aortic valve (arrowhead); RCC, aneurysmal right coronary cusp.
Routine blood investigations were within normal limits and blood cultures were sterile.
TREATMENT
After discussion with the cardiologist, we decided on a two-stage treatment. The patient underwent addition of a permanent pacemaker (VVI, Medtronic, Sigma series; Medtronic, Singapore) under local anaesthesia as the first stage. The pacemaker was inserted in right deltopectoral groove under full monitoring. The patient was discharged on the third day without any periprocedural morbidity. The patient was doing well at first follow-up at 1 week and was planned for aortic valve replacement after further 2 weeks.
The patient was scheduled for surgery, and all routine blood and urine investigations were in normal limits and blood, sputum, groin and urine cultures were sterile. The patient was taken up for surgery under general anaesthesia and a transoesophageal echo probe was inserted; we confirmed the preoperative findings. A midline sternotomy was performed followed by aortic and unicaval cannulation. The patient was put on standard cardiopulmonary bypass. The heart was arrested with hypothermic retrograde blood cardioplegia and the pacemaker was stopped with the help of a magnet and programming device.
On inspection, we found the ascending aorta was normal (32 mm) with normal walls, but friable intima of aorta. On aortotomy, there was aneurysmal dilatation with thickening of right aortic cusp and prolapse. There was also a 12 mm defect along the annular attachment of the right aortic cusp. No vegetations were seen and the other two cusps were normal. No ventricular septal defects were seen. The mitral valve was normal. The aortic valve was excised and replaced by a mechanical prosthetic valve (TTK chitra valve, size-21 mm, Monoleaflet tilting disc mechanical valve; TKK Healthcare, Trivandrum, Kerala, India). The pacemaker was restarted with the help of a programming device. Recovery after cardiopulmonary bypass was smooth.
OUTCOME AND FOLLOW-UP
There was no perioperative morbidity and the patient was discharged on the fifth day.
The excised aortic valve culture was negative. Fungal and anaerobic cultures were also negative. The histopathological examination (HPE) of the excised right cusp showed signs of inflammation. A piece of the aortic wall was also sent for HPE, which was proved to be normal. The patient has been doing well to date (1 month after discharge).
DISCUSSION
Aortic regurgitation may be caused by primary disease of the aortic valve leaflets and/or the wall of the aortic root. Primary valvular causes in our country (India) are rheumatic heart disease, degenerative calcific disease and congenital bicuspid valve problems. Cystic medial necrosis, annuloaortic ectasia, myxoid degeneration, aortic dissection and aortitis are other causes. Infective endocarditis may account for 12% of patients with aortic regurgitation, and typically produce aortic regurgitation by perforation/rupture of the aortic valve leaflets. Endocarditis commonly affects patients with underlying aortic valve disease but may also affect normal valves as well.2–7
In our patient, there was no clinical evidence, blood cultures were negative and transthoracic/transoesophageal echocardiography did not show any evidence of infective endocarditis. Echocardiographic pictures and our observations during surgery were that of a right cusp aneurysm with defect at the annular attachment of the leaflet, suspicious for an old abscess, and the margins of the defect and cusp were thickened suggesting a possible aetiology of endocarditis. Complete heart block in such a young patient also favours this aetiology. The presence of complete heart block, a thickened aneurismal right cusp with perforation at annulus and HPE revealing inflammation all favour the possible aetiology of endocarditis. Despite right cuspal aneurysm with defect, there were no vegetations.
Very few case reports of aortic cuspal aneurysm have been reported in the literature.1–7 As described by Kiroshita et al3 the echocardiographic findings typical of cuspal aneurysm were: ringed echo at the level of the aortic annulus in short axis view, turbulent flow within the ringed echo, and dome formation of the aortic valve that persists throughout the cardiac cycle. Transthoracic and transoesophageal echocardiography examinations are very good diagnostic investigations for these types of cases. These investigations can impart a great deal about pathophysiology and related complications, but can also give hints as to the aetiology. Other investigations such as blood cultures, intraoperative observations and histopathological examination of excised tissue can give an accurate diagnosis. For treatment planning, prior diagnosis can help but this can be modified according to intraoperative observations as well. A peculiarity of our case is the association of right cusp aneurysm with complete heart block and a possible aetiology of endocarditis.
LEARNING POINTS
Very few case reports of aortic cuspal aneurysm have been reported in literature.
Infective endocarditis commonly affects patients with underlying aortic valve disease, but may affect normal valves as well.
Aortic regurgitation is produced by leaflet perforation, rupture of aortic valve leaflets or lack of proper coaptation due to elongated leaflets.
Transthoracic and transoesophageal echocardiography examinations are very good diagnostics investigations for these types of cases.
Footnotes
Competing interests: None.
Patient consent: Patient/guardian consent was obtained for publication.
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