- Email: [email protected]
Associated Subvalvular and Supravalvular Aortic Stenosis with Aortic Valve Anomaly
Associated Subvalvular and Supravalvular Aortic Stenosis with Aortic Valve Anomaly Harris B. Shumacker, Jr., M.D., and David L. Nahrwold, M.D.
S
ubvalvular and supravalvular aortic stenosis are both frequently associated with some abnormality of the aortic valve. On the other hand, the occurrence of subvalvular and supravalvular aortic stenosis in the same patient must be a rarity, and we have not been able to find such a combination in the literature. The patient, an asymptomatic 11%-year-oldboy, was admitted to the hospital on March 3, 1965. A 2-year-old sibling had died following an operation for pulmonary valvular stenosis at another institution. The patient had been thought normal at birth, but a heart murmur had been found when he was 6 months old. A loud holosystolic murmur was widely heard over the precordium. A mild aortic diastolic murmur also was present. The blood pressure was 128/80 in the right arm and approximately the same in the left. The electrocardiogram showed evidence of marked left ventricular hypertrophy. On fluoroscopic and radiographic study there was evidence suggestive of left ventricular enlargement. The aorta seemed normal in size. Cardiac catheterization and angiographic study showed severe discrete subvalvular stenosis, mild supravalvular aortic stenosis, and a deformity of the aortic valve permitting mild regurgitation. In addition the inferior vena cava was observed to be completely obstructed cephalad to the point of reception of the renal veins. Large collateral vessels drained into the azygos system. Simultaneously measured pressures were 263/14 in the left ventricle and 107/70 in the aorta distal to the area of supravalvular constriction. The pressure in the small ventricular segment between the subvalvular diaphragm and the aortic annulus was 122124. An operation was performed on March 10 with the aid of total cardiopulmonary bypass. There was evidence of left ventricular hypertrophy. The aorta was moderately dilated above the area of supravalvular stenosis, as were the coronary sinuses below. The coronary arteries were not enlarged. When the aorta was opened, the preoperative findings were confirmed. There was a mild discrete supravalvular stenosis, the aortic diameter in this area being approximately 15 mm. (Figure). The left coronary aortic cusp was very small, with a presenting edge no longer than 8 mm. and a rather shallow sinus. The right coronary aortic cusp was larger, the noncoronary cusp still larger, and both had From the Department of Surgery and the Heart Research Center, Indiana University School
of Medicine, Indianapolis, Ind. 46207.
Aided by US. Public Health Service Grants Nos. HE-06308-08 and HE-02035-14 and by the James Whitcomb Riley Memorial Association. Accepted for publication Nov. 26, 1969.
356
THE ANNALS OF THORACIC SURGERY
CASE REPORT:
Subualuular and Supraualuular Aortic Stenosis
Subualvular and supraualvular stenosis, and the aortic ualue as it appeayed front within the opened aorta. well-developed sinuses. The right and left cusps were very delicate and thinwalled. The greater part of the noncoronary cusp was also, but it had a rolled, thickened edge. Immediately below the valve leaflets could be seen a fibrous diaphragm which produced a marked stenosis and appeared to be attached to the junction of the left ventricle and the aortic annulus. Indeed, it was so firmly attached to the base of the aortic leaflets that it could be severed from them safely only by lifting up the leaflets and defining their base by gently pressing downward within the cusp with a blunt instrument or a fingertip. Though the supravalvular stenosis was mild, it was thought advisable to enlarge the aorta at this site with a patch. An oval piece of woven Teflon was fashioned with a maximum width of about 12 mm., and this was sutured into the aortic incision. When pressures were measured after repair, the aortic pressure was found to be 140 mm. Hg systolic, the left ventricular 160 mm. Hg, and the aortic immediately afterward 160 mm. Hg. The patient experienced an uneventful convalescence and was discharged from the hospital on the tenth postoperative day. I n June, 1966, on reexamination there was a relatively short grade 3 systolic ejection murmur heard at the base and a mild diastolic murmur of aortic insufficiency. The electrocardiogram showed striking reduction in the degree of previously present severe left ventricular overload. It was, indeed, only suggestive of left ventricular hypertrophy and was not definitely abnormal. The patient has remained well and has been normally active.
SUMMARY A N D CONCLUSION
Deformity of the aortic valve is common in supravalvular aortic stenosis. There were abnormalities of the valve in 13 of the 43 cases reported and collected from the literature by Peterson and his associates [l]. A mild degree of thickening of the aortic cusps and minimal regurgitation are very common in our experience in cases of membranous
SHUMACKER AND NAHRWOLD
subvalvular stenosis. Other cardiovascular anomalies are frequently associated with both supravalvular and subvalvular aortic stenosis [Z]. T h e patient reported had severe subvalvular stenosis, mild supravalvular stenosis, and an aortic valvular deformity which produced mild insufficiency but was not hemodynamically significant. Should subvalvular and supravalvular aortic stenosis occur in the same patient, it is important to recognize both lesions, T h e supravalvular constriction would of course be readily recognized during operation even if it had not been identified by catheterization and angiographic study. It is conceivable, however, that a subvalvular stenotic ring situated immediately adjacent to the aortic annulus, such as occurred in our case, might be missed on the preoperative study if the angiograms were of less than optimal quality. Consequently, in cases of supravalvular aortic stenosis it would be wise to inspect not only the aortic valve itself but the area immediately below it in order to recognize and deal with any subvalvular constriction that might be present. REFERENCES 1. Peterson, T. A., Todd, D. B., and Edwards, J. E.
Supravalvular aortic stenosis. J . Thorac. Cardiovasc. Surg. 50:734, 1965. 2. Putnam, T. C., Harris, P. D., Bernhard, W. F., and Gross, R. E. The surgical management of congenital aortic stenosis. J . Thorac. Cardiovasc. Surg. 48:540, 1964.
358
THE ANNALS OF THORACIC SURGERY
S
ubvalvular and supravalvular aortic stenosis are both frequently associated with some abnormality of the aortic valve. On the other hand, the occurrence of subvalvular and supravalvular aortic stenosis in the same patient must be a rarity, and we have not been able to find such a combination in the literature. The patient, an asymptomatic 11%-year-oldboy, was admitted to the hospital on March 3, 1965. A 2-year-old sibling had died following an operation for pulmonary valvular stenosis at another institution. The patient had been thought normal at birth, but a heart murmur had been found when he was 6 months old. A loud holosystolic murmur was widely heard over the precordium. A mild aortic diastolic murmur also was present. The blood pressure was 128/80 in the right arm and approximately the same in the left. The electrocardiogram showed evidence of marked left ventricular hypertrophy. On fluoroscopic and radiographic study there was evidence suggestive of left ventricular enlargement. The aorta seemed normal in size. Cardiac catheterization and angiographic study showed severe discrete subvalvular stenosis, mild supravalvular aortic stenosis, and a deformity of the aortic valve permitting mild regurgitation. In addition the inferior vena cava was observed to be completely obstructed cephalad to the point of reception of the renal veins. Large collateral vessels drained into the azygos system. Simultaneously measured pressures were 263/14 in the left ventricle and 107/70 in the aorta distal to the area of supravalvular constriction. The pressure in the small ventricular segment between the subvalvular diaphragm and the aortic annulus was 122124. An operation was performed on March 10 with the aid of total cardiopulmonary bypass. There was evidence of left ventricular hypertrophy. The aorta was moderately dilated above the area of supravalvular stenosis, as were the coronary sinuses below. The coronary arteries were not enlarged. When the aorta was opened, the preoperative findings were confirmed. There was a mild discrete supravalvular stenosis, the aortic diameter in this area being approximately 15 mm. (Figure). The left coronary aortic cusp was very small, with a presenting edge no longer than 8 mm. and a rather shallow sinus. The right coronary aortic cusp was larger, the noncoronary cusp still larger, and both had From the Department of Surgery and the Heart Research Center, Indiana University School
of Medicine, Indianapolis, Ind. 46207.
Aided by US. Public Health Service Grants Nos. HE-06308-08 and HE-02035-14 and by the James Whitcomb Riley Memorial Association. Accepted for publication Nov. 26, 1969.
356
THE ANNALS OF THORACIC SURGERY
CASE REPORT:
Subualuular and Supraualuular Aortic Stenosis
Subualvular and supraualvular stenosis, and the aortic ualue as it appeayed front within the opened aorta. well-developed sinuses. The right and left cusps were very delicate and thinwalled. The greater part of the noncoronary cusp was also, but it had a rolled, thickened edge. Immediately below the valve leaflets could be seen a fibrous diaphragm which produced a marked stenosis and appeared to be attached to the junction of the left ventricle and the aortic annulus. Indeed, it was so firmly attached to the base of the aortic leaflets that it could be severed from them safely only by lifting up the leaflets and defining their base by gently pressing downward within the cusp with a blunt instrument or a fingertip. Though the supravalvular stenosis was mild, it was thought advisable to enlarge the aorta at this site with a patch. An oval piece of woven Teflon was fashioned with a maximum width of about 12 mm., and this was sutured into the aortic incision. When pressures were measured after repair, the aortic pressure was found to be 140 mm. Hg systolic, the left ventricular 160 mm. Hg, and the aortic immediately afterward 160 mm. Hg. The patient experienced an uneventful convalescence and was discharged from the hospital on the tenth postoperative day. I n June, 1966, on reexamination there was a relatively short grade 3 systolic ejection murmur heard at the base and a mild diastolic murmur of aortic insufficiency. The electrocardiogram showed striking reduction in the degree of previously present severe left ventricular overload. It was, indeed, only suggestive of left ventricular hypertrophy and was not definitely abnormal. The patient has remained well and has been normally active.
SUMMARY A N D CONCLUSION
Deformity of the aortic valve is common in supravalvular aortic stenosis. There were abnormalities of the valve in 13 of the 43 cases reported and collected from the literature by Peterson and his associates [l]. A mild degree of thickening of the aortic cusps and minimal regurgitation are very common in our experience in cases of membranous
SHUMACKER AND NAHRWOLD
subvalvular stenosis. Other cardiovascular anomalies are frequently associated with both supravalvular and subvalvular aortic stenosis [Z]. T h e patient reported had severe subvalvular stenosis, mild supravalvular stenosis, and an aortic valvular deformity which produced mild insufficiency but was not hemodynamically significant. Should subvalvular and supravalvular aortic stenosis occur in the same patient, it is important to recognize both lesions, T h e supravalvular constriction would of course be readily recognized during operation even if it had not been identified by catheterization and angiographic study. It is conceivable, however, that a subvalvular stenotic ring situated immediately adjacent to the aortic annulus, such as occurred in our case, might be missed on the preoperative study if the angiograms were of less than optimal quality. Consequently, in cases of supravalvular aortic stenosis it would be wise to inspect not only the aortic valve itself but the area immediately below it in order to recognize and deal with any subvalvular constriction that might be present. REFERENCES 1. Peterson, T. A., Todd, D. B., and Edwards, J. E.
Supravalvular aortic stenosis. J . Thorac. Cardiovasc. Surg. 50:734, 1965. 2. Putnam, T. C., Harris, P. D., Bernhard, W. F., and Gross, R. E. The surgical management of congenital aortic stenosis. J . Thorac. Cardiovasc. Surg. 48:540, 1964.
358
THE ANNALS OF THORACIC SURGERY