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Dysplastic aortic valve with absence of aortic valve cusp: An unreported cause of congenital aortic insufficiency
Volume 91 Number 3
March, 1986
good alternative to spiral autogenous vein conduits for palliative surgical treatment of SVC syndrome. We thank Prof. Dr. B. Zawirska from the Department of Pathology at the Medical Academy of Wroclaw, Poland, for pathologic examination, and Mrs. Henriette Maat for secretarial assistance. REFERENCES
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3 4 5
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Doty BD: Bypass of superior vena cava. J THoRAc CARDIOVASC SURG 83:326-338, 1982 Dyet JF, Moghissi K: Role of venography in assessing patients with superior caval obstruction caused by bronchial carcinoma for bypass operations. Thorax 35:628-630, 1980 Doty DB, Baker WH: Bypass of superior vena cava with spiral vein graft. Ann Thorac Surg 22:490-493, 1976 Scherck JP, Kerstein MD, Stansel HC: The current status of vena caval replacement. Surgery 76:209-233, 1974 Haimovici H, Hoffert PW, Zinicola N, Steinman C: An experimental and clinical evaluation of grafts in the venous system. Surg Gynecol Obstet 131: 1173-1186, 1970 Smith ER, Brantigan 0: Bypass of superior vena cava using spiral vein graft. J Cardiovasc Surg 23:259-261, 1983 Avasthi RB, Moghissi K: Malignant obstruction of the superior vena cava and its palliation. J THoRAc CARDIOVASC SURG 74:244-247, 1977 Heydorn WH, Zajtchuk R, Miller J, Schuchmann GF: Gore-Tex grafts for replacement of the superior vena cava. Ann Thorac Surg 23:539-544, 1977 Smith DE, Hammon J, Anane Sefah J, Richardson RS, Trimble C: Segmental venous replacement. J THORAC CARDIOVASC SURG 69:589-598, 1975 Vincze K, Kulka F, Csorba L: Saphenous-jugular bypass as palliative therapy for superior vena cava syndrome caused by bronchial carcinoma. J THoRAc CARDIOVASC SURG 83:272-277, 1982 Danielson GK: Congress on Pediatric Cardiology (abstr), Rome, May, 1984, pp 21-23
Dysplastic aortic valve with absence of aortic valve cusp: An unreported cause of congenital aortic insufficiency Tran-Quang-Hoa, M.D., Aram Smolinsky, M.D., Henry N. Neufeld, and Daniel A. Goor, M.D., Tel Hashomer, Israel From The Department of Thoracic and Cardiovascular Surgery, The Chaim Sheba Medical Center, Tel Hashorner, Israel. Address for reprints: Daniel A. Goor, M.D., Director, Department of Thoracic and Cardiovascular Surgery, The Chaim Sheba Medical Center, Tel Hashomer 52621, Israel
Brief communications
471
Three patients are described in whom dysplasia of the aortic valve was associated with total absence of one of the semilunar aortic cusps and congenital aortic iffiufficiency. Two of the three survived aortic valve replacement.
Primary aortic valve lesions causing congenital aortic insufficiency are rare. Total absence of the aortic valve,1,2 congenital disruption of the sinus CUSp, 3 and incomplete raphe in bicuspid aortic valve" have been described. In the present report, three cases of a previously undescribed condition are described, namely dysplastic aortic valve and total absence of only one of the three aortic cusps. Case reports. CASE 1. A 16-year-old girl had had aortic insufficiencyfrom the age of 4 years. She was referred for aortic valve replacement in April 1984. The clinical picture on admission showed a blood pressure of 120/40 mm Hg, bounding pulse, apical thrust palpable over the precordium, a Grade 3/6 systolic murmur and Grade 4/6 diastolic murmur along left sternal border, and normal splitting of the second sound. Left ventricular hypertrophy was apparent on the electrocardiogram. Cardiac catheterization showed an aortic pressure of 160/80 mm Hg, a left ventricular pressure of 160/0-10 mm Hg, and Grade 4 aortic insufficiency. The left ventricle was dilated and it contracted poorly. The operation was performed on April 12, 1984. The aorta was small and the aortic valve was dysplastic. The noncoronary cusp was thickened but otherwise not remarkable. The left coronary cusp was thick. The left sinus of Valsalva was deep and bottle-shaped, with a narrow aperture connecting the sinus with the aortic lumen. The right coronary cusp was absent, and a very thin rim of myxoid tissue was found at the supposed insertion of the right cusp. A triangular gap on the anulus, caused by the missing right cusp, was observed when the left cusp and noncoronary cusps were apposed. This gap was clearly the cause of the aortic insufficiency. A 21 mm mechanical tilting disc valve (Sorin Biomedica, Vercelli, Italy) was implanted after excision of the native aortic valve. The postoperative course was uneventful. Pathologic examination of tissue from the native aortic valve showed extensive myxomatous changes with abundant deposition of acid mucopolysaccharides. CASE 2. A 9Y2-year-old boy was known to have noncyanotic congenital heart disease at the age of 11 months. The diagnosis of aortic insufficiency was first made when he was 2Y2 years old. At that time he had cardiomegaly, a diastolic (aortic) murmur, and, according to the electrocardiogram, biventricular hypertrophy. On cardiac catheterization at age 2Y2, the pressure in the ascending aorta was 110/50 mm Hg, with no pressure gradient between the left ventricle and ascending aorta. There was marked aortic insufficiency but no mitral insufficiency. The second cardiac workup, done at age 9Y2 years (February, 1984), revealed a fast pulse, a diastolic thrill over the apex, a systolic thrill on the neck, visible pulsation on the right side of the neck, a Grade 2/6 systolic murmur over the apex, and systolic and diastolic murmurs (both Grade 4/6 in intensity) over the left sternal border. The second heart sound was normally split and the pulmonary second sound was normal. The electrocardiogram showed
472
Brief communications
sinus rhythm and left ventricular hypertrophy with strain. Findings on cardiac catheterization at 9V2 years were the same as at 2V2 except that, in addition, there was poor contraction of the left ventricle. A diagnosis of severe Marfan-like aortic insufficiency with aneurysmal dilatation of the aorta was made. At operation, the boy had cor bovinum. The aortic valve was dysplastic, with thick left and noncoronary cusps and deep sinuses of Valsalva. The right coronary cusp was absent. A thin rim of unorganized tissue was found at the area of supposed insertion of the right cusp. Apposition of the left and noncoronary cusps left a triangular gap because of absence of the right coronary cusp. This gap was clearly the cause of the aortic valve insufficiency. A 23 mm mechanical tilting disc valve (Sorin) was implanted after the native aortic valve was excised. The postoperative period was uneventful. Pathologic examination of valve tissue showed myxomatous degeneration. CASE 3. A 9-year-old Arab boy admitted in November, 1983, on an emergency basis. The patient was hospitalized in Chaim Sheba Medical Center because of pulmonary edema. An emergency catheterization revealed severe aortic insufficiency and significant dilatation of the ascending aorta. There was no past history of cardiac workup. Intensive medical treatment for 24 hours did not improve the situation and an emergency operation was performed. Significant cardiomegaly, with left ventricular hypertrophy
The Journal of Thoracic and Cardiovascular Surgery
and an ascending aorta 4 em in diameter, was found. The aortic valve was dysplastic, with three sinuses of Valsalva. The right and the noncoronary cusps were large and thick, the left cusp was absent, and at the place of insertion was a rudimentary jelly-like substance. The valves were replaced by a 19 mm prosthetic mechanical tilting disc valve (Sorin). Hyperkalemia developedduring cardiopulmonary bypass, and the patient was treated while on the pump. However, he died 8 hours postoperatively of myocardial failure. Pathologic examination of tissue from the native aortic valve showed fibrosis with areas of myxomatous changes, but no evidence of active inflammation. REFERENCES Toews WH, Lortscher RH, Kelminson LLK: Double outlet right ventricle with absent aortic valve. Chest 68:381-382, 1975 2 Bierman FZ, Ming-Meng Teh, Swersky S, Martin E, Wigger JH, Fox H: Absence of aortic valve. Antenatal and postnatal two-dimensional and Doppler echocardiographic features. J Am Coll Cardiol 3:833-837, 1984 3 Dolara A, Manetti A, Magi-Diligenti L, Gori F: Aortic regurgitation in newborn. Br Heart J 42:606-607, 1979 4 Milo S, Neufeld HN, Goor DA: Incomplete commissure of the aortic valve. Thorax 37:636-637, 1982
March, 1986
good alternative to spiral autogenous vein conduits for palliative surgical treatment of SVC syndrome. We thank Prof. Dr. B. Zawirska from the Department of Pathology at the Medical Academy of Wroclaw, Poland, for pathologic examination, and Mrs. Henriette Maat for secretarial assistance. REFERENCES
2
3 4 5
6
7
8
9
10
11
Doty BD: Bypass of superior vena cava. J THoRAc CARDIOVASC SURG 83:326-338, 1982 Dyet JF, Moghissi K: Role of venography in assessing patients with superior caval obstruction caused by bronchial carcinoma for bypass operations. Thorax 35:628-630, 1980 Doty DB, Baker WH: Bypass of superior vena cava with spiral vein graft. Ann Thorac Surg 22:490-493, 1976 Scherck JP, Kerstein MD, Stansel HC: The current status of vena caval replacement. Surgery 76:209-233, 1974 Haimovici H, Hoffert PW, Zinicola N, Steinman C: An experimental and clinical evaluation of grafts in the venous system. Surg Gynecol Obstet 131: 1173-1186, 1970 Smith ER, Brantigan 0: Bypass of superior vena cava using spiral vein graft. J Cardiovasc Surg 23:259-261, 1983 Avasthi RB, Moghissi K: Malignant obstruction of the superior vena cava and its palliation. J THoRAc CARDIOVASC SURG 74:244-247, 1977 Heydorn WH, Zajtchuk R, Miller J, Schuchmann GF: Gore-Tex grafts for replacement of the superior vena cava. Ann Thorac Surg 23:539-544, 1977 Smith DE, Hammon J, Anane Sefah J, Richardson RS, Trimble C: Segmental venous replacement. J THORAC CARDIOVASC SURG 69:589-598, 1975 Vincze K, Kulka F, Csorba L: Saphenous-jugular bypass as palliative therapy for superior vena cava syndrome caused by bronchial carcinoma. J THoRAc CARDIOVASC SURG 83:272-277, 1982 Danielson GK: Congress on Pediatric Cardiology (abstr), Rome, May, 1984, pp 21-23
Dysplastic aortic valve with absence of aortic valve cusp: An unreported cause of congenital aortic insufficiency Tran-Quang-Hoa, M.D., Aram Smolinsky, M.D., Henry N. Neufeld, and Daniel A. Goor, M.D., Tel Hashomer, Israel From The Department of Thoracic and Cardiovascular Surgery, The Chaim Sheba Medical Center, Tel Hashorner, Israel. Address for reprints: Daniel A. Goor, M.D., Director, Department of Thoracic and Cardiovascular Surgery, The Chaim Sheba Medical Center, Tel Hashomer 52621, Israel
Brief communications
471
Three patients are described in whom dysplasia of the aortic valve was associated with total absence of one of the semilunar aortic cusps and congenital aortic iffiufficiency. Two of the three survived aortic valve replacement.
Primary aortic valve lesions causing congenital aortic insufficiency are rare. Total absence of the aortic valve,1,2 congenital disruption of the sinus CUSp, 3 and incomplete raphe in bicuspid aortic valve" have been described. In the present report, three cases of a previously undescribed condition are described, namely dysplastic aortic valve and total absence of only one of the three aortic cusps. Case reports. CASE 1. A 16-year-old girl had had aortic insufficiencyfrom the age of 4 years. She was referred for aortic valve replacement in April 1984. The clinical picture on admission showed a blood pressure of 120/40 mm Hg, bounding pulse, apical thrust palpable over the precordium, a Grade 3/6 systolic murmur and Grade 4/6 diastolic murmur along left sternal border, and normal splitting of the second sound. Left ventricular hypertrophy was apparent on the electrocardiogram. Cardiac catheterization showed an aortic pressure of 160/80 mm Hg, a left ventricular pressure of 160/0-10 mm Hg, and Grade 4 aortic insufficiency. The left ventricle was dilated and it contracted poorly. The operation was performed on April 12, 1984. The aorta was small and the aortic valve was dysplastic. The noncoronary cusp was thickened but otherwise not remarkable. The left coronary cusp was thick. The left sinus of Valsalva was deep and bottle-shaped, with a narrow aperture connecting the sinus with the aortic lumen. The right coronary cusp was absent, and a very thin rim of myxoid tissue was found at the supposed insertion of the right cusp. A triangular gap on the anulus, caused by the missing right cusp, was observed when the left cusp and noncoronary cusps were apposed. This gap was clearly the cause of the aortic insufficiency. A 21 mm mechanical tilting disc valve (Sorin Biomedica, Vercelli, Italy) was implanted after excision of the native aortic valve. The postoperative course was uneventful. Pathologic examination of tissue from the native aortic valve showed extensive myxomatous changes with abundant deposition of acid mucopolysaccharides. CASE 2. A 9Y2-year-old boy was known to have noncyanotic congenital heart disease at the age of 11 months. The diagnosis of aortic insufficiency was first made when he was 2Y2 years old. At that time he had cardiomegaly, a diastolic (aortic) murmur, and, according to the electrocardiogram, biventricular hypertrophy. On cardiac catheterization at age 2Y2, the pressure in the ascending aorta was 110/50 mm Hg, with no pressure gradient between the left ventricle and ascending aorta. There was marked aortic insufficiency but no mitral insufficiency. The second cardiac workup, done at age 9Y2 years (February, 1984), revealed a fast pulse, a diastolic thrill over the apex, a systolic thrill on the neck, visible pulsation on the right side of the neck, a Grade 2/6 systolic murmur over the apex, and systolic and diastolic murmurs (both Grade 4/6 in intensity) over the left sternal border. The second heart sound was normally split and the pulmonary second sound was normal. The electrocardiogram showed
472
Brief communications
sinus rhythm and left ventricular hypertrophy with strain. Findings on cardiac catheterization at 9V2 years were the same as at 2V2 except that, in addition, there was poor contraction of the left ventricle. A diagnosis of severe Marfan-like aortic insufficiency with aneurysmal dilatation of the aorta was made. At operation, the boy had cor bovinum. The aortic valve was dysplastic, with thick left and noncoronary cusps and deep sinuses of Valsalva. The right coronary cusp was absent. A thin rim of unorganized tissue was found at the area of supposed insertion of the right cusp. Apposition of the left and noncoronary cusps left a triangular gap because of absence of the right coronary cusp. This gap was clearly the cause of the aortic valve insufficiency. A 23 mm mechanical tilting disc valve (Sorin) was implanted after the native aortic valve was excised. The postoperative period was uneventful. Pathologic examination of valve tissue showed myxomatous degeneration. CASE 3. A 9-year-old Arab boy admitted in November, 1983, on an emergency basis. The patient was hospitalized in Chaim Sheba Medical Center because of pulmonary edema. An emergency catheterization revealed severe aortic insufficiency and significant dilatation of the ascending aorta. There was no past history of cardiac workup. Intensive medical treatment for 24 hours did not improve the situation and an emergency operation was performed. Significant cardiomegaly, with left ventricular hypertrophy
The Journal of Thoracic and Cardiovascular Surgery
and an ascending aorta 4 em in diameter, was found. The aortic valve was dysplastic, with three sinuses of Valsalva. The right and the noncoronary cusps were large and thick, the left cusp was absent, and at the place of insertion was a rudimentary jelly-like substance. The valves were replaced by a 19 mm prosthetic mechanical tilting disc valve (Sorin). Hyperkalemia developedduring cardiopulmonary bypass, and the patient was treated while on the pump. However, he died 8 hours postoperatively of myocardial failure. Pathologic examination of tissue from the native aortic valve showed fibrosis with areas of myxomatous changes, but no evidence of active inflammation. REFERENCES Toews WH, Lortscher RH, Kelminson LLK: Double outlet right ventricle with absent aortic valve. Chest 68:381-382, 1975 2 Bierman FZ, Ming-Meng Teh, Swersky S, Martin E, Wigger JH, Fox H: Absence of aortic valve. Antenatal and postnatal two-dimensional and Doppler echocardiographic features. J Am Coll Cardiol 3:833-837, 1984 3 Dolara A, Manetti A, Magi-Diligenti L, Gori F: Aortic regurgitation in newborn. Br Heart J 42:606-607, 1979 4 Milo S, Neufeld HN, Goor DA: Incomplete commissure of the aortic valve. Thorax 37:636-637, 1982