Echocardiographic Diagnosis of Unruptured Aneurysm of Valsalva Dissecting into the Ventricular Septum
of the Sinus
Vishva Dev, MD, DM, and Savitri Shrivastava, MD, DM ongenital aneurysms of the sinus of Valsalva are uncommon and present mostly after their rupture.’ C Unruptured aneurysms of the sinus of Valsalva are rare and may present with arrhythmias, conduction abnormalities, right ventricular outflow obstruction, tricuspid regurgitation, aortic regurgitation or coronary conclusion 1,2Their diagnosis during life is even rarer and most caseshave beendetectedat autopsy or as incidental findings at angiography.2 There are isolated case reports of echocardiographic diagnosis of such patients.2-4We present herein 4 caseswith echocardiographic diagnosis of this entity that were later confirmed by angiography and/ or surgery. Over the last 4 years, 35 casesof sinus of Valsalva aneurysms have been detected by cross-sectional and Doppler echocardiography in our center. This report pertains to 4 unruptured aneurysms originatingfrom the right sinus of Valsalva and dissecting into the ventricular septum. In the 31 casesof ruptured aneurysms, the right sinus was involved in 29 (rupture into right ventricle in 28 and into left ventricle in I) and the noncoronary sinus in 2 (rupture into right atrium in both). There was no echocardiographic or clinical evidenceof endocarditis in any of the 4 unruptured patients, although vegetations weredetectedin 5 of the 31 patients with ruptured aneurysms. All 4 patients with unruptured aneurysms were men with an age range of 20 to 45 years. Three of these patients presented with syncope caused by complete heart block. Other manifestations included progressive dyspnea (in 3 patients), moderate aortic regurgitation (in 3 patients) and mitral regurgitation (in I patient). On 2-dimensional echocardiography, all 4 patients showed the aneurysm as a cystic mass in the upper part of the ventricular septum clearly communicating with the right coronary sinus of Valsalva (Figure I). The wall of the aneurysm showeddenseechoessuggestiveof calcification in 3patients. This was confirmed on cinefluoroscopy. The fourth patient had a huge noncalctjic aneurysm (8 cm X 5 cm X 3 cm in size) that projected into the left ventricular cavity and showed diastolic expansion and systolic collapse (Figure 2). Aneurysms with calcified walls did not change appreciably in size with the cardiac cycle. In the last 3 patients, pulsed-Doppler interrogation at the site of the communication of the aneurysm with the aorta showed a to and fro signal (Figure 2). Additional Doppler echocardiographic findings included moderate aortic regurgitation in 3 patients, mild mitral regurgitation in I (patient 2) and innominate artery block in 1 (patient 3); all were confirmed by angiogFrom the Department of Cardiology, CT Centre, All India Institute of Medical Sciences,New Delhi-l 10 029, India. Manuscript received December20,1989; revisedmanuscript receivedand acceptedApril 16, 1990.
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THE AMERICAN JOURNAL OF CARDIOLOGY VOLUME 66
raphy. Etiology of the innominate artery block is not clear. Left ventricular function was depressed(left ventricular ejectionfraction 0.3,0.4 and 0.45) in 3 patients (all with calcified walls). All 4patients weresubjectedto cardiac catheterization and angiography that confirmed the findings obtained by 2-dimensional and Doppler echocardiography. Twopatients (I and 2) wereoperated upon. The surgery involved closure of the aortic end of the aneurysm in both and aortic valve replacementin I. The echocardiographicfindings were confirmed at surgery. Permanent pacemaker implantation was required in the 3patientspresenting with syncope.All thepatients are doing well on follow-up. Unruptured aneurysmsof the sinus of Valsalva may dissect into the upper part of the ventricular septum, causing conduction abnormalities and aortic regurgitation.2-5There are 3 previousreports of unruptured aneu-
FIGURE 1. Twoechocardiography. Long-axis viowsofthe2pathtsshowingsinusofValsalvaaneurysms (A) timesting into ths upper part of intervenMcular septum. AO=aorta;RV=dghtvantricular; LV=k?ftv~.
rysms dissecting into the ventricular septum, diagnosed on echocardiography.3-5The aneurysms present like a “cyst” in the upper part of the ventricular septum. The diagnosisis basedupon demonstrationof the communication of the aneurysm with the sinus of Valsalva.3-5This was demonstrablein all our patients. Enlargement of the aneurysmduring diastole and collapseduring systolehas beenfound to be a useful tinding.3-4 We found this to be useful in only 1 of our patients. Calcification of the aneurysm wall possiblypreventschangein its sizewith cardiac contraction. A to and fro Doppler signal at the mouth of the aneurysmwas presentin 3 of our patients in whom it was specifically looked for. We believe the echocardiographic diagnosisof this entity should be basedupon the demonstration of a cystic spacein the ventricular septum communicating with a sinusof Valsalva, and a to and fro Doppler signal at the site of communication. Conduction abnormalities occurred in all our patients (complete heart block in 3 and bifascicular block in 1) and are causedby disruption of the conduction tissue by the dissecting aneurysm. Aortic regurgitation was present in 3 casesand was related largely to the distortion of the aortic valve by the aneurysm, as the valve leaflets appeared anatomically normal. The cause of poor left ventricular function in thesepatients is unclear. Mechanical interference with the septal motion becauseof the aneurysm,aortic regurgitation and extensionof the calcification into the myocardium may be contributory. Combined cross-sectionaland Doppler echocardiography is valuable in the diagnosis of this entity,
1. Nowicki GR, Aberden E, Friedman S, Rashkind WJ. Congenital left aortic sinus:left ventricle fistula and review of aortic-cardiac fistulas. Ann Thorac Surg 1977;23:378-388.
FIGURE 2. A, M-mode cut at the level of mitral valve showing systolic collapse and the diastolic expansion of the aneurysm in the interventricular septum. 6, short-axis view at the level of aortic valve clearly showing communication of the aneurysm with right coronary sinus (R). C, to and fro Doppler signal at site of the communication of aneurysm with right coronary sinus. ASOV = aneurysm of the sinus of Valsalva; L = left coronary sinus; LA = left atrium; N = noncoronary sinus; RA = right atrium; RV = right ventricle.
2. Fishbein MC: Obma R, Roberts WC. Unruptured sinus of Valsalva aneurysms. Am J Cardiol 1975;35:918-922.
3. Hands ME, Lloyd BL, Hung J. Cross-sectionalechocardiographicdiagnosisof unruptured right sinus of valsalva aneurysmdissectinginto the interventricular septum.Int J Cardiol 1985,9:380-383. 4. Ahmad RAS, Struman S, Watson RDS. Unruptured aneurysmof the sinusof Valsalva presentingwith isolated heart block: echocardiographicdiagnosisand successfulsurgical repair. Br Heart J 1989;61:375S377. 5. Lewis BS,AgathangelouNE. Echocardiographicdiagnosisof unrupturedsinus of Valsalva aneurysm.Am Heart J 1984:107:1025~1027.
THE AMERICAN JOURNAL OF CARDIOLOGY AUGUST 15, 1990
503
of the Sinus
Vishva Dev, MD, DM, and Savitri Shrivastava, MD, DM ongenital aneurysms of the sinus of Valsalva are uncommon and present mostly after their rupture.’ C Unruptured aneurysms of the sinus of Valsalva are rare and may present with arrhythmias, conduction abnormalities, right ventricular outflow obstruction, tricuspid regurgitation, aortic regurgitation or coronary conclusion 1,2Their diagnosis during life is even rarer and most caseshave beendetectedat autopsy or as incidental findings at angiography.2 There are isolated case reports of echocardiographic diagnosis of such patients.2-4We present herein 4 caseswith echocardiographic diagnosis of this entity that were later confirmed by angiography and/ or surgery. Over the last 4 years, 35 casesof sinus of Valsalva aneurysms have been detected by cross-sectional and Doppler echocardiography in our center. This report pertains to 4 unruptured aneurysms originatingfrom the right sinus of Valsalva and dissecting into the ventricular septum. In the 31 casesof ruptured aneurysms, the right sinus was involved in 29 (rupture into right ventricle in 28 and into left ventricle in I) and the noncoronary sinus in 2 (rupture into right atrium in both). There was no echocardiographic or clinical evidenceof endocarditis in any of the 4 unruptured patients, although vegetations weredetectedin 5 of the 31 patients with ruptured aneurysms. All 4 patients with unruptured aneurysms were men with an age range of 20 to 45 years. Three of these patients presented with syncope caused by complete heart block. Other manifestations included progressive dyspnea (in 3 patients), moderate aortic regurgitation (in 3 patients) and mitral regurgitation (in I patient). On 2-dimensional echocardiography, all 4 patients showed the aneurysm as a cystic mass in the upper part of the ventricular septum clearly communicating with the right coronary sinus of Valsalva (Figure I). The wall of the aneurysm showeddenseechoessuggestiveof calcification in 3patients. This was confirmed on cinefluoroscopy. The fourth patient had a huge noncalctjic aneurysm (8 cm X 5 cm X 3 cm in size) that projected into the left ventricular cavity and showed diastolic expansion and systolic collapse (Figure 2). Aneurysms with calcified walls did not change appreciably in size with the cardiac cycle. In the last 3 patients, pulsed-Doppler interrogation at the site of the communication of the aneurysm with the aorta showed a to and fro signal (Figure 2). Additional Doppler echocardiographic findings included moderate aortic regurgitation in 3 patients, mild mitral regurgitation in I (patient 2) and innominate artery block in 1 (patient 3); all were confirmed by angiogFrom the Department of Cardiology, CT Centre, All India Institute of Medical Sciences,New Delhi-l 10 029, India. Manuscript received December20,1989; revisedmanuscript receivedand acceptedApril 16, 1990.
502
THE AMERICAN JOURNAL OF CARDIOLOGY VOLUME 66
raphy. Etiology of the innominate artery block is not clear. Left ventricular function was depressed(left ventricular ejectionfraction 0.3,0.4 and 0.45) in 3 patients (all with calcified walls). All 4patients weresubjectedto cardiac catheterization and angiography that confirmed the findings obtained by 2-dimensional and Doppler echocardiography. Twopatients (I and 2) wereoperated upon. The surgery involved closure of the aortic end of the aneurysm in both and aortic valve replacementin I. The echocardiographicfindings were confirmed at surgery. Permanent pacemaker implantation was required in the 3patientspresenting with syncope.All thepatients are doing well on follow-up. Unruptured aneurysmsof the sinus of Valsalva may dissect into the upper part of the ventricular septum, causing conduction abnormalities and aortic regurgitation.2-5There are 3 previousreports of unruptured aneu-
FIGURE 1. Twoechocardiography. Long-axis viowsofthe2pathtsshowingsinusofValsalvaaneurysms (A) timesting into ths upper part of intervenMcular septum. AO=aorta;RV=dghtvantricular; LV=k?ftv~.
rysms dissecting into the ventricular septum, diagnosed on echocardiography.3-5The aneurysms present like a “cyst” in the upper part of the ventricular septum. The diagnosisis basedupon demonstrationof the communication of the aneurysm with the sinus of Valsalva.3-5This was demonstrablein all our patients. Enlargement of the aneurysmduring diastole and collapseduring systolehas beenfound to be a useful tinding.3-4 We found this to be useful in only 1 of our patients. Calcification of the aneurysm wall possiblypreventschangein its sizewith cardiac contraction. A to and fro Doppler signal at the mouth of the aneurysmwas presentin 3 of our patients in whom it was specifically looked for. We believe the echocardiographic diagnosisof this entity should be basedupon the demonstration of a cystic spacein the ventricular septum communicating with a sinusof Valsalva, and a to and fro Doppler signal at the site of communication. Conduction abnormalities occurred in all our patients (complete heart block in 3 and bifascicular block in 1) and are causedby disruption of the conduction tissue by the dissecting aneurysm. Aortic regurgitation was present in 3 casesand was related largely to the distortion of the aortic valve by the aneurysm, as the valve leaflets appeared anatomically normal. The cause of poor left ventricular function in thesepatients is unclear. Mechanical interference with the septal motion becauseof the aneurysm,aortic regurgitation and extensionof the calcification into the myocardium may be contributory. Combined cross-sectionaland Doppler echocardiography is valuable in the diagnosis of this entity,
1. Nowicki GR, Aberden E, Friedman S, Rashkind WJ. Congenital left aortic sinus:left ventricle fistula and review of aortic-cardiac fistulas. Ann Thorac Surg 1977;23:378-388.
FIGURE 2. A, M-mode cut at the level of mitral valve showing systolic collapse and the diastolic expansion of the aneurysm in the interventricular septum. 6, short-axis view at the level of aortic valve clearly showing communication of the aneurysm with right coronary sinus (R). C, to and fro Doppler signal at site of the communication of aneurysm with right coronary sinus. ASOV = aneurysm of the sinus of Valsalva; L = left coronary sinus; LA = left atrium; N = noncoronary sinus; RA = right atrium; RV = right ventricle.
2. Fishbein MC: Obma R, Roberts WC. Unruptured sinus of Valsalva aneurysms. Am J Cardiol 1975;35:918-922.
3. Hands ME, Lloyd BL, Hung J. Cross-sectionalechocardiographicdiagnosisof unruptured right sinus of valsalva aneurysmdissectinginto the interventricular septum.Int J Cardiol 1985,9:380-383. 4. Ahmad RAS, Struman S, Watson RDS. Unruptured aneurysmof the sinusof Valsalva presentingwith isolated heart block: echocardiographicdiagnosisand successfulsurgical repair. Br Heart J 1989;61:375S377. 5. Lewis BS,AgathangelouNE. Echocardiographicdiagnosisof unrupturedsinus of Valsalva aneurysm.Am Heart J 1984:107:1025~1027.
THE AMERICAN JOURNAL OF CARDIOLOGY AUGUST 15, 1990
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