Interesting Cases
Title: A reversible and lethal cause of complete heart block which could be easily missed
A 43 year old lady was referred to our center for pacemaker implantation because of CHB.
She had a history of dizziness and one episode of syncope since two days ago. Physical examination did not disclose any significant abnormality. She did not have any risk factor of coronary artery disease. There was no significant past medical illness. Laboratory data including serum potassium and cardiac enzyme levels and sedimentation rate were within normal limits.
The ECG showed complete AV block with ventricular escape rate of 30-35 beat/min. The QRS complexes were wide with LBBB morphology pattern (Figure 1).
Transthoracic echocardiography showed mildly enlarged left ventricle with normal contractility, mild aortic regurgitation and an aneurysm of sinus of valsalva eroding into the upper part of interventricular septum (Figure 2).
Multislice CT scan disclosed a large sinus of valsalva aneurysm originating from right sinus of
valsalva (Figure 3).
Right sided heart catheterization and selective coronary angiography showed normal
pulmonary artery and wedge pressures and normal epicardial coronary arteries. Aortography in LAO and RAO projections showed a large aneurysm of right coronary sinus of valsalva and
mild aortic regurgitation (Figure 4).
The patient underwent surgery and the mouth of aneurysm was closed with a Gortex patch.
In addition, an epicardial pacemaker was implanted.
The ECG taken two weeks later disclosed sinus rhythm with prolonged AV interval and
bifascicular block and no pacing (Figure 5).
Discussion
Sinus of valsalva aneurysm is a rare anomaly that is more common in men.The most common involved sinus is the right sinus. 5 to 15 percent of aneurysms originate in noncoronary sinus and the left sided cusp is seldom involved.(1)They may be congenital or acquired. A separation or lack of continuity between the aortic media and aortic valve annulus may initiate aneurysm formation.(1) Acquired aneurysms are less common and more frequently affect the left sinus.(2) They may result from trauma, endocarditis, Behcet, Marfan, Syphilis, Tuberculosis and senile type dilation. (3) The most common presentation of sinus of valsalva aneurysm is subsequent to its rupture. An unruptured aneurysm can cause right ventricular outflow tract obstruction, infective endocarditis, malignant arrhythmia and myocardial infarction or ischemia due to coronary ostia distortion or coronary arteries compression.(3)
In rare cases, the aneurysm erodes into the interventricular septum. It has been suggested that intraseptal extension is caused by intramural rupture of a congenital aneurysm with subsequent formation of a hematoma and in fact it represents a pseudoaneurysm.(4) Direct pressure by the expanding aneurysm and low grade inflammation can lead to atrioventricular conduction defects.(4)
It is interesting in our patient that the rhythm returned to sinus after surgical decompressing of interventricular septum. It is reasonable that unruptured sinus of valsalva aneurysm eroding into the interventricular septum is operated as soon as possible, preventing development of complications such as heart block.the practical point in this case is to stress the need for complete evaluation of every patient who is candidate for PPM imlantation.in this regard presense of reversible causes of AVB such as drugs,ischemia,..should be considered.in this case if echocardiography has not been performed ,the patient woud be discharged after PPM implantation despite a large and ready for rupture aneurysm.
Dr A.Kharazi
Dr M.Alasti
References
1) Webb GD, Smallhorn JF, Therrien J, Redington AN. Congenital heart disease. In: Libby P, Bonow RO, Mann DL, Zipes DP,eds. Braunwald's Heart Disease. Eighth edition. Saunders Elsevier 2008: 1609.
2) El Hattaoui M, Charei N, Boumzebra D, Chraibi S, Bennis A. A large aneurysm of a left sinus of valsalva invading the interventricular septum- a rare cause of syncope. Can J Cardiol 2008; 24(5): e28-29.
3) Vural KM, Sener E, Tasdemir O, and Bayazit K. Approach to sinus of Valsalva aneurysms: a review of 53 cases. Eur J Cardiothorac Surg 2001;20:71-76.
4) Choudhary SK, Bhan A, Bose Reddy SC, Sharma R, Murari V, Airan B, et al. Aneurysm of Sinus of Valsalva Dissecting IntoInterventricular Septum. Ann Thorac Surg 1998;65: 735- 40.
She had a history of dizziness and one episode of syncope since two days ago. Physical examination did not disclose any significant abnormality. She did not have any risk factor of coronary artery disease. There was no significant past medical illness. Laboratory data including serum potassium and cardiac enzyme levels and sedimentation rate were within normal limits.
The ECG showed complete AV block with ventricular escape rate of 30-35 beat/min. The QRS complexes were wide with LBBB morphology pattern (Figure 1).
Transthoracic echocardiography showed mildly enlarged left ventricle with normal contractility, mild aortic regurgitation and an aneurysm of sinus of valsalva eroding into the upper part of interventricular septum (Figure 2).
Multislice CT scan disclosed a large sinus of valsalva aneurysm originating from right sinus of
valsalva (Figure 3).
Right sided heart catheterization and selective coronary angiography showed normal
pulmonary artery and wedge pressures and normal epicardial coronary arteries. Aortography in LAO and RAO projections showed a large aneurysm of right coronary sinus of valsalva and
mild aortic regurgitation (Figure 4).
The patient underwent surgery and the mouth of aneurysm was closed with a Gortex patch.
In addition, an epicardial pacemaker was implanted.
The ECG taken two weeks later disclosed sinus rhythm with prolonged AV interval and
bifascicular block and no pacing (Figure 5).
Discussion
Sinus of valsalva aneurysm is a rare anomaly that is more common in men.The most common involved sinus is the right sinus. 5 to 15 percent of aneurysms originate in noncoronary sinus and the left sided cusp is seldom involved.(1)They may be congenital or acquired. A separation or lack of continuity between the aortic media and aortic valve annulus may initiate aneurysm formation.(1) Acquired aneurysms are less common and more frequently affect the left sinus.(2) They may result from trauma, endocarditis, Behcet, Marfan, Syphilis, Tuberculosis and senile type dilation. (3) The most common presentation of sinus of valsalva aneurysm is subsequent to its rupture. An unruptured aneurysm can cause right ventricular outflow tract obstruction, infective endocarditis, malignant arrhythmia and myocardial infarction or ischemia due to coronary ostia distortion or coronary arteries compression.(3)
In rare cases, the aneurysm erodes into the interventricular septum. It has been suggested that intraseptal extension is caused by intramural rupture of a congenital aneurysm with subsequent formation of a hematoma and in fact it represents a pseudoaneurysm.(4) Direct pressure by the expanding aneurysm and low grade inflammation can lead to atrioventricular conduction defects.(4)
It is interesting in our patient that the rhythm returned to sinus after surgical decompressing of interventricular septum. It is reasonable that unruptured sinus of valsalva aneurysm eroding into the interventricular septum is operated as soon as possible, preventing development of complications such as heart block.the practical point in this case is to stress the need for complete evaluation of every patient who is candidate for PPM imlantation.in this regard presense of reversible causes of AVB such as drugs,ischemia,..should be considered.in this case if echocardiography has not been performed ,the patient woud be discharged after PPM implantation despite a large and ready for rupture aneurysm.
Dr A.Kharazi
Dr M.Alasti
References
1) Webb GD, Smallhorn JF, Therrien J, Redington AN. Congenital heart disease. In: Libby P, Bonow RO, Mann DL, Zipes DP,eds. Braunwald's Heart Disease. Eighth edition. Saunders Elsevier 2008: 1609.
2) El Hattaoui M, Charei N, Boumzebra D, Chraibi S, Bennis A. A large aneurysm of a left sinus of valsalva invading the interventricular septum- a rare cause of syncope. Can J Cardiol 2008; 24(5): e28-29.
3) Vural KM, Sener E, Tasdemir O, and Bayazit K. Approach to sinus of Valsalva aneurysms: a review of 53 cases. Eur J Cardiothorac Surg 2001;20:71-76.
4) Choudhary SK, Bhan A, Bose Reddy SC, Sharma R, Murari V, Airan B, et al. Aneurysm of Sinus of Valsalva Dissecting IntoInterventricular Septum. Ann Thorac Surg 1998;65: 735- 40.
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