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Echocardiographic diagnosis of unruptured aneurysm of right sinus of Valsalva: An unusual cause of right ventricular outflow obstruction
BRIEF COMMUNICATIONS Echocardiographic diagnosis of unruptured aneurysm of right sinus of Valsalva: An unusual cause of right ventricular outflow obstruction A. G. Desai, M.D., S. Sharma, M.D., Anil Kumar, D.M., R. C. Hansoti, M.D., and B. R. Kalke, MS., PhD., Bombay,
India
A 32-year-old man presentedwith exertional dyspneaand palpitations for 3 years. Cardiovascular examination revealed a pulse rate of 90/min, blood pressure 150 to 70 mm Hg, apical impulse in the left sixth intercostal spaceof left ventricular type, a grade % ejection systolic murmur not varying with respiration, a grade 5/aearly diastolic murmur along the left sternal border, and a delayed soft pulmonary component. Chest x-ray showedcardiomegaly, prominent ascendingaorta, and normal pulmonary vascularity. ECG showed biventricular hypertrophy. M-mode echocardiography, performed with a commercially available DiasonicsCV-3400R phased-array system, showeda mildly dilated left ventricular cavity and fine fluttering of the anterior mitral leaflet. Pulmonic valve echocardiogram revealed marked systolic fluttering, normal ef slope, and absent a wave, suggestingthe presence of subpulmanic obstruction (Fig. 1). Two-dimensional echocardiography (2DE) in the parasternal long-axis view revealed a small perimembranous ventricular septal defect (VSD) and an aneurysmally dilated right sinusof Valsalva (SOV) protruding through it into the right ventricular outflow tract (RVOT) (Fig. 2). The parasternal short-axis view showeda dilated aortic root with a narrowed RVOT. An echocardiographicdiagnosisof the aneurysm of the right SOV, protruding through the perimembranousVSD, causing infundibular pulmonary stenosisand aortic regurgitation wasmade. The hemodynamic and oximetry data are summarized in Table I. The right ventriculogram in the posteroanterior and lateral views showed a well-circumscribed rounded structure projecting through the ventricular septumand causingsubpulmonic obstruction (Fig. 3). This structure was moving up and down during different phasesof the cardiac cycle. The left ventricular angiogram in the left anterior oblique long-axis view showeda small perimembranousVSD. The aortic root angiogramshowed grade % aortic regurgitation and an unruptured aneurysm of the right SOV (Fig. 4). At surgery, these findings were confirmed. The aneurysmal right sinus was excised, the VSD was closed, and the aortic valve was reconstructed. The postoperative course was uneventful. Postoperative 2DE in the parasternal long-axis view showed an intact
From
the Department
Reprint requests: Rd., Bombay-400
of Cardiology, A. G. Desai, 008 India.
M.D.,
B. Y. L. Nair B. Y. L. Nair
Hospital. Hospital,
Dr. A. L. Nair
Fig.
1.
M-mode echocardiogram of the pulmonic valve
(PV) showingnormal ef slope, absenta wave, and systolic
fluttering.
2. 2DE in parasternal long-axis view showingunruptured aneurysm of right sinus of Valsalva (RCC aneurysm) bulging through a subaortic ventricular septal defect (VSD), and producing right ventricular outflow tract (RVOZ’) obstruction. A0 = aorta; LA = left atrium; LV = left ventricle. Fig.
Fig. 3. Right ventriculogram in lateral (left) and posteroanterior (right) views showingsubpulmonicobstruction produced by the unruptured aneurysm. 363
February,
364
Brief Communications
American
Heart
1995 Journal
Fig. 5. Postoperative 2DE in parasternal long-axis view showingintact interventricular septum and disappearance of echoesfrom the RVOT. AV = aortic valve; AML = anterior mitral leaflet. Fig. 4. Aortogram in 60-degreeleft anterior oblique projection showingregurgitation of the dye into the ventricle and an unruptured aneurysm of the right SOV.
Table
I. Oximetry and hemodynamic data Oximetry (% saturation)
Site Superior vena cava Inferior vena cava Right atrium Right ventricle Main pulmonary artery Pulmonary artery wedge Left ventricle Aorta *Peak systolic gradient
75 76 75 75 75 96 96
Pressures (mm Hg)
iii 10 120*/10 37/10 iii 25 iii 12 145/l” 145170 75 100
of 83 mm across RVOT
ventricular septum and disappearanceof echoesfrom the RVOT (Fig. 5). The diagnosis of SOV aneurysms is usually delayed until they have ruptured or produced fatal complications. Right SOV aneurysmsmay occasionallyprotrude into the RVOT and produce significant obstruction.‘,’ Although, the echocardiographicfeatures of ruptured aneurysmsof the SOV have been reported in the literature,“5 there is little emphasison the noninvasive diagnosisof unruptured aneurysmscausingsubpulmonic obstruction. In the present case,precise delineation of anatomic details and hemodynamic consequencesof SOV aneurysm was possible by echocardiography. Widening of the aortic root demonstratedin the parasternal long-axis view, alongwith the curvilinear anterior protrusion through the VSD, can be highly suggestiveof aneurysm of the right SOV bulging into the RVOT. In addition, short-axis view delineates clearly the aneurysm of the SOV in most cases.The
associatedperimembranousVSD can be demonstrated as a discontinuity between the interventricular septum and the anterior aortic root in the parasternal long-axis view. Pulmonic valve systolic fluttering, normal ef slope, and absent a wave suggestedsubpulmonic obstruction produced by the anteriorly protruding aneurysm of the SOV. Premature opening of the pulmonic valve6 is a feature of rupture of SOV aneurysm into the right side of the heart, and its absencefavored the diagnosisof an unruptured aneurysmof the SOV. The finding of diastolic fine flutter of the anterior mitral leaflet supports the diagnosis of associatedaortic regurgitation. Ultrasound contrast study, performed either by peripheral venous or aortic root injection during cardiac catheterization, with the use of 5% dextrose or salinesolution, can delineate the anteriorly protruding right SOV aneurysm. The contrast study was not performed in our patient. Thus, 2DE along with its M-mode counterpart can provide detailed and accurate diagnostic information prior to cardiac catheterization and surgery.
REFERENCES
1. Sakakibara S, Konno S: Congenital aneurysm of sinus of Valsalva. AM HEART J 63:708, 1962. 2. Kerber RE, Ridges JD, Kyiss JP, Silverman JF, Anderson ET, Harrison DC: Unruptured aneurysm of sinus of Valsalva producing right ventricular outflow obstruction. Am J Med 53:775, 1972. 3. Rothbaum DA, Dhillon JC, Chang S, Feigenbaum H: Echocardiographic manifestation of right sinus of Valsalva aneurysm. Circulation 49:768, 1974. 4. Imiazumi T, Orita Y, Koiwaya Y, Hirala T, Nakamura M: Utility of two dimensional echocardiography in differential diagnosis of etiology of aortic regurgitation. AM HEART J 103:887,
1982.
5. Weyman A: Cross sectional echocardiography. Philadelphia, 1982, Lea & Febiger, Publishers, p 241. 6. Wann LS, Weyman AE, Dhillon JC, Feigenbaum H: Premature pulmonary valve opening. Circulation 55:128, 1977.
India
A 32-year-old man presentedwith exertional dyspneaand palpitations for 3 years. Cardiovascular examination revealed a pulse rate of 90/min, blood pressure 150 to 70 mm Hg, apical impulse in the left sixth intercostal spaceof left ventricular type, a grade % ejection systolic murmur not varying with respiration, a grade 5/aearly diastolic murmur along the left sternal border, and a delayed soft pulmonary component. Chest x-ray showedcardiomegaly, prominent ascendingaorta, and normal pulmonary vascularity. ECG showed biventricular hypertrophy. M-mode echocardiography, performed with a commercially available DiasonicsCV-3400R phased-array system, showeda mildly dilated left ventricular cavity and fine fluttering of the anterior mitral leaflet. Pulmonic valve echocardiogram revealed marked systolic fluttering, normal ef slope, and absent a wave, suggestingthe presence of subpulmanic obstruction (Fig. 1). Two-dimensional echocardiography (2DE) in the parasternal long-axis view revealed a small perimembranous ventricular septal defect (VSD) and an aneurysmally dilated right sinusof Valsalva (SOV) protruding through it into the right ventricular outflow tract (RVOT) (Fig. 2). The parasternal short-axis view showeda dilated aortic root with a narrowed RVOT. An echocardiographicdiagnosisof the aneurysm of the right SOV, protruding through the perimembranousVSD, causing infundibular pulmonary stenosisand aortic regurgitation wasmade. The hemodynamic and oximetry data are summarized in Table I. The right ventriculogram in the posteroanterior and lateral views showed a well-circumscribed rounded structure projecting through the ventricular septumand causingsubpulmonic obstruction (Fig. 3). This structure was moving up and down during different phasesof the cardiac cycle. The left ventricular angiogram in the left anterior oblique long-axis view showeda small perimembranousVSD. The aortic root angiogramshowed grade % aortic regurgitation and an unruptured aneurysm of the right SOV (Fig. 4). At surgery, these findings were confirmed. The aneurysmal right sinus was excised, the VSD was closed, and the aortic valve was reconstructed. The postoperative course was uneventful. Postoperative 2DE in the parasternal long-axis view showed an intact
From
the Department
Reprint requests: Rd., Bombay-400
of Cardiology, A. G. Desai, 008 India.
M.D.,
B. Y. L. Nair B. Y. L. Nair
Hospital. Hospital,
Dr. A. L. Nair
Fig.
1.
M-mode echocardiogram of the pulmonic valve
(PV) showingnormal ef slope, absenta wave, and systolic
fluttering.
2. 2DE in parasternal long-axis view showingunruptured aneurysm of right sinus of Valsalva (RCC aneurysm) bulging through a subaortic ventricular septal defect (VSD), and producing right ventricular outflow tract (RVOZ’) obstruction. A0 = aorta; LA = left atrium; LV = left ventricle. Fig.
Fig. 3. Right ventriculogram in lateral (left) and posteroanterior (right) views showingsubpulmonicobstruction produced by the unruptured aneurysm. 363
February,
364
Brief Communications
American
Heart
1995 Journal
Fig. 5. Postoperative 2DE in parasternal long-axis view showingintact interventricular septum and disappearance of echoesfrom the RVOT. AV = aortic valve; AML = anterior mitral leaflet. Fig. 4. Aortogram in 60-degreeleft anterior oblique projection showingregurgitation of the dye into the ventricle and an unruptured aneurysm of the right SOV.
Table
I. Oximetry and hemodynamic data Oximetry (% saturation)
Site Superior vena cava Inferior vena cava Right atrium Right ventricle Main pulmonary artery Pulmonary artery wedge Left ventricle Aorta *Peak systolic gradient
75 76 75 75 75 96 96
Pressures (mm Hg)
iii 10 120*/10 37/10 iii 25 iii 12 145/l” 145170 75 100
of 83 mm across RVOT
ventricular septum and disappearanceof echoesfrom the RVOT (Fig. 5). The diagnosis of SOV aneurysms is usually delayed until they have ruptured or produced fatal complications. Right SOV aneurysmsmay occasionallyprotrude into the RVOT and produce significant obstruction.‘,’ Although, the echocardiographicfeatures of ruptured aneurysmsof the SOV have been reported in the literature,“5 there is little emphasison the noninvasive diagnosisof unruptured aneurysmscausingsubpulmonic obstruction. In the present case,precise delineation of anatomic details and hemodynamic consequencesof SOV aneurysm was possible by echocardiography. Widening of the aortic root demonstratedin the parasternal long-axis view, alongwith the curvilinear anterior protrusion through the VSD, can be highly suggestiveof aneurysm of the right SOV bulging into the RVOT. In addition, short-axis view delineates clearly the aneurysm of the SOV in most cases.The
associatedperimembranousVSD can be demonstrated as a discontinuity between the interventricular septum and the anterior aortic root in the parasternal long-axis view. Pulmonic valve systolic fluttering, normal ef slope, and absent a wave suggestedsubpulmonic obstruction produced by the anteriorly protruding aneurysm of the SOV. Premature opening of the pulmonic valve6 is a feature of rupture of SOV aneurysm into the right side of the heart, and its absencefavored the diagnosisof an unruptured aneurysmof the SOV. The finding of diastolic fine flutter of the anterior mitral leaflet supports the diagnosis of associatedaortic regurgitation. Ultrasound contrast study, performed either by peripheral venous or aortic root injection during cardiac catheterization, with the use of 5% dextrose or salinesolution, can delineate the anteriorly protruding right SOV aneurysm. The contrast study was not performed in our patient. Thus, 2DE along with its M-mode counterpart can provide detailed and accurate diagnostic information prior to cardiac catheterization and surgery.
REFERENCES
1. Sakakibara S, Konno S: Congenital aneurysm of sinus of Valsalva. AM HEART J 63:708, 1962. 2. Kerber RE, Ridges JD, Kyiss JP, Silverman JF, Anderson ET, Harrison DC: Unruptured aneurysm of sinus of Valsalva producing right ventricular outflow obstruction. Am J Med 53:775, 1972. 3. Rothbaum DA, Dhillon JC, Chang S, Feigenbaum H: Echocardiographic manifestation of right sinus of Valsalva aneurysm. Circulation 49:768, 1974. 4. Imiazumi T, Orita Y, Koiwaya Y, Hirala T, Nakamura M: Utility of two dimensional echocardiography in differential diagnosis of etiology of aortic regurgitation. AM HEART J 103:887,
1982.
5. Weyman A: Cross sectional echocardiography. Philadelphia, 1982, Lea & Febiger, Publishers, p 241. 6. Wann LS, Weyman AE, Dhillon JC, Feigenbaum H: Premature pulmonary valve opening. Circulation 55:128, 1977.