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Supravalvular Aortic Stenosis in Aortic Dissection
Supravalvular Aortic Stenosis in Aortic Dissection Isidre Vilacosta, MD, Jose´ Alberto San Roma´n, MD, Paloma Aragoncillo, MD, Joaquı´n Ferreiro´s, MD, Ramiro Me´ndez, MD, Catherine Graupner, MD, Walter Stoermann, MD, Elena Batlle, MD, and Mario Baquero, MD he following report illustrates how an aortic dissection may T lead to false lumen thrombosis of the ascending aorta and cause supravalvular aortic stenosis. Magnetic resonance imaging and echocardiography facilitated the clinical recognition of this complication. This mechanism of aortic obstruction has not been previously described. •••
A 73-year-old woman with a long history of hypertension was referred to our hospital for evaluation of a systolic precordial murmur and angina on effort. Approximately 2 months before referral, the patient experienced an episode of severe substernal and interscapular back pain. At that time, she did not seek medical attention, but after this episode she became increasingly dyspneic and had 2 episodes of chest pain on exertion. When seen by her family doctor a prominent systolic murmur was heard, and she was sent to our hospital. Findings on physical examination revealed normal jugular venous pressure. Blood pressure was 150/80 mm Hg. Pulse rate was 90 beats/min with a regular rhythm. Coarse systolic vibrations were felt in the carotid arterial pulse. On palpation, the cardiac apical impulse was sustained and a presystolic distention was noted. A systolic thrill was readily appreciated on the left side of the sternum. On auscultation, there was a grade 4/6 midsystolic murmur at the base of the heart that was well transmitted to the jugular notch and along the carotid vessels. A prominent S4 was noted. The abdomen was normal. CASE 1:
From the Departments of Cardiology, Human Pathology, and Radiology, Hospital Universitario de San Carlos, and Department of Cardiology, Hospital Universitario de Valladolid, Madrid, Spain. Dr. Vilacosta’s address is: Serrano, 46, 28001 Madrid, Spain. Manuscript received October 16, 1997; revised manuscript received and accepted January 6, 1998. ©1998 by Excerpta Medica, Inc. All rights reserved.
All pulses were present and symmetric. Neurologic examination was normal. The electrocardiogram showed sinus rhythm with left ventricular hypertrophy. A chest roentgenogram disclosed left ventricular enlargement and mild dilatation of the ascending aorta. Transthoracic 2-dimensional echocardiography demonstrated a hypertrophied left ventricle with an ejection fraction of 75%. The aortic valve was normal. The ascending aorta was filled by a thrombus-like nonmobile echodense mass (Figure 1). A systolic jet through the ascending aorta with a maximal velocity of 3.8 m/s was recorded by continuous-wave Doppler. Transesophageal echocardiographic (TEE) study allowed the diagnosis of a DeBakey type I aortic dissection. The dissection flap was detected in the descending aorta, aortic arch, and in the distal segment of the ascending aorta. The false lumen of the proximal portion of the ascending aorta was completely thrombosed producing a slit-like narrowing of the true lumen (Figure 2A). The entrance tear could not be visualized. Magnetic resonance imaging (Figure 3) was concordant with the TEE findings and, in addition, the entrance tear was seen in the aortic arch (Figure 4). Operation was recommended but she refused surgery. Fourteen months later the patient is doing well with only mild dyspnea on exertion. The systolic murmur has markedly decreased and the systolic thrill disappeared. A new TEE study has demonstrated an increase in the size of the true lumen and a decrease in thrombosed false lumen (Figure 2B). There was no gradient along the ascending aorta by continuous-wave Doppler. CASE 2: A 65-year-old man with a history of hypertension was referred to our hospital for assessment and treatment of a carcinoma
of the pancreas. He had epigastric pain, anorexia, and weight loss. While he was admitted, nausea and vomiting occurred frequently and one day he experienced an episode of severe chest pain with mild dyspnea. Clinical examination showed no signs of heart failure. Blood pressure was 110/70 mm Hg. A prominent sustained apical impulse was noted. A systolic ejection murmur, grade 3/6, was present, loudest in the third left intercostal space and radiating to the carotids. All pulses were present. Neurologic examination was normal. An electrocardiogram showed sinus rhythm, left atrial enlargement, and left ventricular hypertrophy. A chest roentgenogram disclosed a mild dilatation of the ascending aorta. TEE study demonstrated a DeBakey type II aortic dissection with a severe thrombosis of the false lumen of the proximal segment of the ascending aorta; the aortic valve was normal and the entrance tear could no be visualized. A systolic jet along the ascending aorta with a maximal velocity of 3 m/s was recorded by continuous-wave Doppler. We believe that the patient was not a surgical candidate and did not undergo surgery. He developed a severe sepsis and died several days later. Pathologic examination of the aorta showed an entrance tear located in the distal segment of the ascending aorta. The descending aorta and the aortic valve were normal, and there was a severe thrombosis of the false lumen in the ascending aorta producing a slit-like narrowing of the true aortic lumen (Figure 5). •••
This is the first report of aortic dissection with false lumen thrombosis of the ascending aorta and supravalvular aortic stenosis. The physical examination, electrocardiogram, and chest radiograph 0002-9149/98/$19.00 PII S0002-9149(98)00101-5
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FIGURE 1. Transthoracic 2-dimensional echocardiography. Apical 5-chamber view. An echodense mass (asterisk) filling the ascending aorta is seen. AI 5 left atrium; VD 5 right ventricle; VI 5 left ventricle.
FIGURE 2. Transesophageal echocardiographic short-axis view of ascending aorta. A, superior image. Severe thrombosis of the false lumen (asterisk) and slit-like narrowing of the true lumen (color Doppler). B, inferior image, patient’s follow up. The thrombosis of the false lumen has decreased, and the size of the true lumen increased.
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FIGURE 3. Cine magnetic resonance imaging, coronal section. A, the dissection flap (arrow) is clearly seen and the thrombosis (asterisk) of the false channel nicely depicted. B, the turbulent jet along the true lumen (arrow) is well visualized.
FIGURE 5. Anatomic specimen of patient 2. Thrombosis of the false lumen (asterisk). Slit-like narrowing of the true lumen (arrow).
FIGURE 4. Spin-echo magnetic resonance imaging. Axial section in the aortic arch. The entrance tear is clearly detected (arrow).
were all consistent with aortic stenosis. Echocardiography and magnetic resonance imaging provided useful information for an ac-
curate diagnosis. Both cases had retrograde dissection; the entrance tear was distal to the proximal segment of the ascending
aorta, permitting thrombosis of the proximal false channel. Obstruction of the ascending aorta by a dissection flap with a ball-valve action has been previously published.1 Supravalvular aortic stenosis after replacement of the ascending aorta has also been described.2 Thus, this report illustrates an additional, previously unreported, potential mechanism by which an aortic dissection may produce supravalvular aortic stenosis. 1. Roan PG, Buja LM, Grammer JC. Ascending aortic obstruction produced by dissected intimal flap. Br Heart J 1981;46:452– 454. 2. Vilacosta I, Camino A, San Roma´n JA, Rolla´n MJ, De la Llana R, Gil M, Sa´nchez-Harguindey L. Supravalvular aortic stenosis after replacement of the ascending aorta. Am J Cardiol 1992;70:1505–1507.
CASE REPORTS
1273
A 73-year-old woman with a long history of hypertension was referred to our hospital for evaluation of a systolic precordial murmur and angina on effort. Approximately 2 months before referral, the patient experienced an episode of severe substernal and interscapular back pain. At that time, she did not seek medical attention, but after this episode she became increasingly dyspneic and had 2 episodes of chest pain on exertion. When seen by her family doctor a prominent systolic murmur was heard, and she was sent to our hospital. Findings on physical examination revealed normal jugular venous pressure. Blood pressure was 150/80 mm Hg. Pulse rate was 90 beats/min with a regular rhythm. Coarse systolic vibrations were felt in the carotid arterial pulse. On palpation, the cardiac apical impulse was sustained and a presystolic distention was noted. A systolic thrill was readily appreciated on the left side of the sternum. On auscultation, there was a grade 4/6 midsystolic murmur at the base of the heart that was well transmitted to the jugular notch and along the carotid vessels. A prominent S4 was noted. The abdomen was normal. CASE 1:
From the Departments of Cardiology, Human Pathology, and Radiology, Hospital Universitario de San Carlos, and Department of Cardiology, Hospital Universitario de Valladolid, Madrid, Spain. Dr. Vilacosta’s address is: Serrano, 46, 28001 Madrid, Spain. Manuscript received October 16, 1997; revised manuscript received and accepted January 6, 1998. ©1998 by Excerpta Medica, Inc. All rights reserved.
All pulses were present and symmetric. Neurologic examination was normal. The electrocardiogram showed sinus rhythm with left ventricular hypertrophy. A chest roentgenogram disclosed left ventricular enlargement and mild dilatation of the ascending aorta. Transthoracic 2-dimensional echocardiography demonstrated a hypertrophied left ventricle with an ejection fraction of 75%. The aortic valve was normal. The ascending aorta was filled by a thrombus-like nonmobile echodense mass (Figure 1). A systolic jet through the ascending aorta with a maximal velocity of 3.8 m/s was recorded by continuous-wave Doppler. Transesophageal echocardiographic (TEE) study allowed the diagnosis of a DeBakey type I aortic dissection. The dissection flap was detected in the descending aorta, aortic arch, and in the distal segment of the ascending aorta. The false lumen of the proximal portion of the ascending aorta was completely thrombosed producing a slit-like narrowing of the true lumen (Figure 2A). The entrance tear could not be visualized. Magnetic resonance imaging (Figure 3) was concordant with the TEE findings and, in addition, the entrance tear was seen in the aortic arch (Figure 4). Operation was recommended but she refused surgery. Fourteen months later the patient is doing well with only mild dyspnea on exertion. The systolic murmur has markedly decreased and the systolic thrill disappeared. A new TEE study has demonstrated an increase in the size of the true lumen and a decrease in thrombosed false lumen (Figure 2B). There was no gradient along the ascending aorta by continuous-wave Doppler. CASE 2: A 65-year-old man with a history of hypertension was referred to our hospital for assessment and treatment of a carcinoma
of the pancreas. He had epigastric pain, anorexia, and weight loss. While he was admitted, nausea and vomiting occurred frequently and one day he experienced an episode of severe chest pain with mild dyspnea. Clinical examination showed no signs of heart failure. Blood pressure was 110/70 mm Hg. A prominent sustained apical impulse was noted. A systolic ejection murmur, grade 3/6, was present, loudest in the third left intercostal space and radiating to the carotids. All pulses were present. Neurologic examination was normal. An electrocardiogram showed sinus rhythm, left atrial enlargement, and left ventricular hypertrophy. A chest roentgenogram disclosed a mild dilatation of the ascending aorta. TEE study demonstrated a DeBakey type II aortic dissection with a severe thrombosis of the false lumen of the proximal segment of the ascending aorta; the aortic valve was normal and the entrance tear could no be visualized. A systolic jet along the ascending aorta with a maximal velocity of 3 m/s was recorded by continuous-wave Doppler. We believe that the patient was not a surgical candidate and did not undergo surgery. He developed a severe sepsis and died several days later. Pathologic examination of the aorta showed an entrance tear located in the distal segment of the ascending aorta. The descending aorta and the aortic valve were normal, and there was a severe thrombosis of the false lumen in the ascending aorta producing a slit-like narrowing of the true aortic lumen (Figure 5). •••
This is the first report of aortic dissection with false lumen thrombosis of the ascending aorta and supravalvular aortic stenosis. The physical examination, electrocardiogram, and chest radiograph 0002-9149/98/$19.00 PII S0002-9149(98)00101-5
1271
FIGURE 1. Transthoracic 2-dimensional echocardiography. Apical 5-chamber view. An echodense mass (asterisk) filling the ascending aorta is seen. AI 5 left atrium; VD 5 right ventricle; VI 5 left ventricle.
FIGURE 2. Transesophageal echocardiographic short-axis view of ascending aorta. A, superior image. Severe thrombosis of the false lumen (asterisk) and slit-like narrowing of the true lumen (color Doppler). B, inferior image, patient’s follow up. The thrombosis of the false lumen has decreased, and the size of the true lumen increased.
1272 THE AMERICAN JOURNAL OF CARDIOLOGYT
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FIGURE 3. Cine magnetic resonance imaging, coronal section. A, the dissection flap (arrow) is clearly seen and the thrombosis (asterisk) of the false channel nicely depicted. B, the turbulent jet along the true lumen (arrow) is well visualized.
FIGURE 5. Anatomic specimen of patient 2. Thrombosis of the false lumen (asterisk). Slit-like narrowing of the true lumen (arrow).
FIGURE 4. Spin-echo magnetic resonance imaging. Axial section in the aortic arch. The entrance tear is clearly detected (arrow).
were all consistent with aortic stenosis. Echocardiography and magnetic resonance imaging provided useful information for an ac-
curate diagnosis. Both cases had retrograde dissection; the entrance tear was distal to the proximal segment of the ascending
aorta, permitting thrombosis of the proximal false channel. Obstruction of the ascending aorta by a dissection flap with a ball-valve action has been previously published.1 Supravalvular aortic stenosis after replacement of the ascending aorta has also been described.2 Thus, this report illustrates an additional, previously unreported, potential mechanism by which an aortic dissection may produce supravalvular aortic stenosis. 1. Roan PG, Buja LM, Grammer JC. Ascending aortic obstruction produced by dissected intimal flap. Br Heart J 1981;46:452– 454. 2. Vilacosta I, Camino A, San Roma´n JA, Rolla´n MJ, De la Llana R, Gil M, Sa´nchez-Harguindey L. Supravalvular aortic stenosis after replacement of the ascending aorta. Am J Cardiol 1992;70:1505–1507.
CASE REPORTS
1273