Abstract
Sinus of Valsalva aneurysm (SVA) is rare, and aneurysm of the left sinus of Valsalva is extremely rare cardiovascular disease. Clinical presentation can vary from mostly asymptomatic to catastrophic life-threatening emergency. We report four extremely rare cases of SVA with different manifestations of which one case involved left aortic sinus with large unruptured aneurysm causing severe mitral regurgitation (MR) and severe aortic regurgitation (AR). The second case aneurysm was from right sinus presented with trifascicular block with intermittent complete heart block (CHB). Third was a diagnosed case of unruptured right SVA and while awaiting for surgery he had sudden cardiac death (SCD) at home. Therefore, SVA can present from asymptomatic to life-threatening condition, such as SCD. To the best of our knowledge, there are individual case reports in literature, and this is the first case series of unruptured SVA in literature highlighting the rarity of this disease.
Keywords: complete heart block, unruptured sinus of Valsalva aneurysm, mitral regurgitation, aortic regurgitation, right ventricular outflow track, sudden cardiac death, aneurysm
Sinus of Valsalva aneurysm (SVA) is an enlargement of the aortic root area between the aortic valve annulus and the sinotubular ridge. Most of the aneurysms are congenital in origin. 1
Almost all of the unruptured aneurysms are discovered accidentally, but presentation to the hospital may occur because of valvular regurgitation, right ventricular outflow obstruction causing congestive heart failure, conduction disturbances such as complete heart block (CHB), resistant ventricular tachycardia, myocardial ischemia because of coronary artery compression, left/right ventricular inflow obstruction, and sudden cardiac death (SCD). 2 SVA arise mostly from the right and non-coronary sinuses, only 1 to 2% arising from the left. 3
To the best of our knowledge, no case reports of combined severe mitral and aortic regurgitation (AR) because of SVA are reported in the literature.
Case 1
A 45-year-old male presented with history of palpitation and breathlessness on exertion for 3 months. On examination, he had soft S1, early diastolic murmur in the left parasternal area, and pan systolic murmur in the apex. An electrocardiogram (ECG) revealed feature suggestive of left ventricular (LV) volume overload. The chest X-ray (CXR) revealed cardiomegaly. Echocardiogram (ECHO) showed dilated left atrium (LA) and LV. There was a large aneurysm arising from the left aortic sinus of size 4.63 × 3.62 cm. This large aneurysm had thin wall and was compressing the LA and anterior mitral leaflet (AML). Color Doppler showed severe MR because of distorted AML and also severe AR because of non-coapting aortic leaflet ( Fig. 1 ). The computed tomography angiography (CTA) showed large left SVA of size 5.2 × 3.2 cm, which is compressing the LA ( Fig. 2 ). Under general anesthesia, the aortic valve and most of the aneurismal sac was excised through the aortotomy and St. Jude Medical composite valve conduit graft sutured to the ends of the aorta. Reimplantation of coronary arteries into the graft is done (Bentall procedure). Mitral valve repairing is done through left atrial approach.
Fig. 1.
( A ) ECHO showing aneurysm compressing the left atrium (arrow). ( B ) Short-axis view at aortic level showing SVA from left sinus (arrow). ( C ) CW Doppler at aortic valve showing pressure half time of 168 ms. ( D ) CW Doppler showing severe MR. CW, continuous wave; ECHO, echocardiogram; MR, mitral regurgitation; SVA, sinus of Valsalva aneurysm.
Fig. 2.
( A ) CT angiography in coronal view showing dilated aortic root. ( B ) Showing sinotubular junction and ascending aorta normal. ( C ) Showing aneurysm size of 3.7 × 4.49 cm. ( D ) Showing normal descending aorta. CT, computed tomography.
Case 2
A 30-year-old female presented with complaints of palpitation for 3 months. One month back, she was admitted for syncope evaluation, and Holter study showed trifascicular block with intermittent CHB for which she underwent permanent pacemaker in some other hospital. Two-dimensional (2D) ECHO showed unruptured SVA aneurysm of size 2.8 cm and mild AR. On transesophageal echocardiogram (TEE), this aneurysm was compressing the septum and pushing the septum toward right ventricular outflow track (RVOT). Cath study showed large unruptured right SVA of size 2.58 × 2.5 cm compressing the septum and RVOT ( Fig. 3 ). There was grade I AR. After aortotomy, the right SVA was excised, and pericardial patch closure was done. After surgery, CHB improved, but trifascicular block persisted; therefore, the patient was in follow-up with pacemaker.
Fig. 3.
( A ) ECHO PLAX view showing right sinus aneurysm (arrow). ( B ) On TEE aneurysm compressing RVOT. ( C ) RAO view showing right SVA with pacing lead. ( D ) ECG showing return of sinus rhythm. ECG, electrocardiogram; ECHO, echocardiogram; PLAX, parasternal long axis view; RAO, right anterior oblique; RVOT, right ventricular outflow track; TEE, transesophageal echocardiogram.
Case 3
A 23-year-old male presented with a history of breathlessness and palpitation on exertion for 1 year. On cardiac examination, he had soft S1, pan systolic murmur in apical area. ECG was suggestive of left ventricular volume overload. CXR showed cardiomegaly. A 2D ECHO showed dilated LA and LV. There was AML prolapse with severe MR and mild AR. There was 4.4 × 3.3 cm right SVA, which was compressing the RVOT ( Fig. 4 ). The patient expired at home suddenly while awaiting for surgery.
Fig. 4.
( A ) CXR showing cardiomegaly. ( B ) PLAX view showing large aneurysm from right sinus. ( C ) On short-axis view, aneurysm (arrow) compressing RVOT. ( D ) Color Doppler showing severe MR. CXR, chest X-ray; MR, mitral regurgitation; PLAX, parasternal long axis view; RVOT, right ventricular outflow track.
Case 4
A 24-year-old male presented with a history of palpitation and exertional breathlessness for 1 year. ECG showed features suggestive of right ventricular hypertrophy. ECHO showed double chambered right ventricle (DCRV) and right SVA of size 1.4 × 1.2 cm ( Fig. 5 ). He was taken for Cath study, which showed DCRV ( Table 1 ) with unruptured right SVA. This patient is waiting for surgery.
Fig. 5.
(A) ECHO showing right SVA (arrow). ( B ) LAO view aneurysm from right sinus (arrow). ECHO, echocardiogram; LAO, left anterior oblique; SVA, sinus of Valsalva aneurysm.
Table 1. Clinical and hemodynamic profile of patients.
| Case 1 | Case 2 | Case 3 | Case 4 | ||
|---|---|---|---|---|---|
| Symptom onset to diagnosis | 3 months | 3 months | 1 year | 1 year | |
| Diagnosis to surgery | 1 month | 2 month | Expired after 1 month of diagnosis | Awaiting surgery | |
| Hemodynamic (mmHg) |
RV | 28/8 | 24/8 | – | 110/10 |
| PA | 38/16 | 28/10 | – | 24/8 | |
| LV | 138/20 | 126/10 | – | 108/8 | |
| AORTA | 136/60 | 126/90 | – | 110/84 | |
| Blood pressure on follow-up (mmHg) | 130/88 | 120/90 | – | – | |
| Post-operative follow-up | 1 year | 3 years | Expired | Awaiting surgery | |
Abbreviations: LV, left ventricle; PA, pulmonary artery; RV, right ventricle.
Discussion
SVA is a rare congenital heart disease with reported incidences varying from 0.5 to 3% among all congenital heart diseases. These aneurysms may involve any of the three aortic sinus. 4
Chu et al found that SVA most frequently involved the right sinus (76.8%), followed by the noncoronary (20.2%), and least commonly, the left sinus of Valsalva (3%). 5 Rupture of SVA is five times higher in Asian population as compared with that in the Western population. 4 The usual manifestation of SVA is asymptomatic, and manifestation occurs because of extension into adjacent cardiac chambers, most commonly into the right ventricle followed by the right atrium according to the location of the lesions. In most cases, the symptom is associated with the ruptured aneurysm into cardiac chambers or concomitant diseases, such as valvular regurgitation, ventricular septal defect, or bacterial endocarditis. 6
Rarely, SVA has been reported to extend outside the heart, resulting in catastrophic rupture or serious anatomic, or physiologic derangement by mass and pressure effect. 7 There are many case reports of AR 8 because of non-coapting valve in the literature, but only few case reports of mitral regurgitation (MR) because of large aneurysm compressing the LA and mitral valve apparatus.
Management of unruptured SVA is mainly by surgery because unruptured SVA has high risk for rupture and is mostly associated with other cardiac condition, which will require surgical management, such as valvular regurgitation. Post operatively, these patients need to be monitored for residual AR and CHB, which may develop because of damage to His bundle and its branches. 5
We are reporting the first case (case 1) of large left SVA compressing the left atrium and anterior mitral leaflet producing severe MR and severe AR because of non-coapting aortic valve. There are few case reports of trifascicular block with CHB in literature because of right SVA as in case 2. The possible explanation for CHB associated with large aneurysm eroding interventricular septum (IVS) is compression of bundle of His or bundle branches when aneurysm of the right SVA grows caudally distorting or eroding into membranous part of IVS. That is why the aneurysm should be excluded from aortic root or excised carefully, to relieve the His bundle from compression, which may help the CHB to improve to normal sinus rhythm. 9 SVA can cause SCD as in our case 3 because of sudden rupture or ventricular tachycardia. 2
SVA is very rare and can vary in presentation from asymptomatic to SCD. SVA is to be ruled out in young patient with cardiac manifestations, such as chest pain, palpitation, breathlessness, CHB, etc. importantly once diagnosed it should be treated earlier. Limitation of this series is there is no standard guidelines for management of these patients, and there are no studies involving long-term follow-up of unruptured SVA to compare these patients indicating rarity of this disease in the population.
Footnotes
Conflicts of Interest None.
References
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