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Row# ID# Date Journal Article
1  23512010-03-06
Authors: Otto CM, Burwash IG, Legget ME, Munt BI, Fujioka M, Healy NL, Kraft CD, Miyake-Hull CY, Schwaegler RG.
Citation: Circulation. 1997 May 6;95(9):2262-70.
Abstract: BACKGROUND: Only limited data on the rate of hemodynamic progression and predictors of outcome in asymptomatic patients with valvular aortic stenosis (AS) are available. METHODS AND RESULTS: In 123 adults (mean age, 63 +/- 16 years) with asymptomatic AS, annual clinical, echocardiographic, and exercise data were obtained prospectively (mean follow-up of 2.5 +/- 1.4 years). Aortic jet velocity increased by 0.32 +/- 0.34 m/s per year and mean gradient by 7 +/- 7 mm Hg per year; valve area decreased by 0.12 +/- 0.19 cm2 per year. Kaplan-Meier event-free survival, with end points defined as death (n = 8) or aortic valve surgery (n = 48), was 93 +/- 5% at 1 year, 62 +/- 8% at 3 years, and 26 +/- 10% at 5 years. Univariate predictors of outcome included baseline jet velocity, mean gradient, valve area, and the rate of increase in jet velocity (all P < or = .001) but not age, sex, or cause of AS. Those with an end point had a smaller exercise increase in valve area, blood pressure, and cardiac output and a greater exercise decrease in stroke volume. Multivariate predictors of outcome were jet velocity at baseline (P < .0001), the rate of change in jet velocity (P < .0001), and functional status score (P = .002). The likelihood of remaining alive without valve replacement at 2 years was only 21 +/- 18% for a jet velocity at entry > 4.0 m/s, compared with 66 +/- 13% for a velocity of 3.0 to 4.0 m/s and 84 +/- 16% for a jet velocity < 3.0 m/s (P < .0001). CONCLUSIONS: In adults with asymptomatic AS, the rate of hemodynamic progression and clinical outcome are predicted by jet velocity, the rate of change in jet velocity, and functional status.
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2  23502010-03-06
Authors: Olsson M, Dalsgaard CJ, Haegerstrand A, Rosenqvist M, Rydén L, Nilsson J.
Citation: J Am Coll Cardiol. 1994 Apr;23(5):1162-70.
Abstract: OBJECTIVES. Cell-specific antibodies were used to identify immunocompetent cells in a comparison of valves from patients who had symptomatic tricuspid aortic stenosis with subjects who had no evidence of valvular heart disease. BACKGROUND. Nonrheumatic valvular aortic stenosis is the most common valvular heart disease among adults. The biologic processes involved in the development of this disease are poorly understood. METHODS. Tricuspid stenotic aortic valves were obtained from 19 patients undergoing surgery for nonrheumatic valvular aortic stenosis, and 10 control valves were collected at autopsy. The valves were fixed in formaldehyde, cryosectioned and stained with antibodies against fibroblasts, endothelial cells, macrophages, T lymphocytes and interleukin-2 receptors. A subset of valves were also analyzed with antibodies against T-helper cells and cytotoxic T cells. RESULTS. Stenotic valves were characterized by a basal accumulation of calcium deposits and a cell-rich subendothelial thickening. The immunohistologic analysis indicated that the cells in the subendothelial connective tissue were fibroblasts. T lymphocytes appeared to be the most common cell type in the vicinity of the calcium deposits and were also found close to the endothelial lining of the valves. T-helper cells were more frequent than cytotoxic T cells. Expression of interleukin-2 receptors occurred at the same location as T lymphocytes. Control valves lacked subendothelial thickening and contained only few cells reacting with antibodies against lymphocytes and macrophages. CONCLUSIONS. The presence of activated T lymphocytes in tricuspid stenotic valves suggests that immunologic mechanisms may be involved in the etiology of nonrheumatic aortic stenosis.
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3  23492010-03-06
Authors: Otto CM, Kuusisto J, Reichenbach DD, Gown AM, O'Brien KD.
Citation: Circulation. 1994 Aug;90(2):844-53.
Abstract: BACKGROUND: Nonrheumatic stenosis of trileaflet aortic valves, often termed senile or calcific valvular aortic stenosis, is considered a "degenerative" process, but little is known about the cellular or molecular factors that mediate its development. METHODS AND RESULTS: To characterize the developing aortic valvular lesion, we performed histological and immunohistochemical studies on Formalin-fixed and methanol-Carnoy's-fixed paraffin-embedded aortic valve leaflets or on frozen sections obtained at autopsy from 27 adults (age, 46 to 82 years) with normal leaflets (n = 6), mild macroscopic leaflet thickening (n = 15), or clinical aortic stenosis (n = 6). Focal areas of thickening ("early lesions") were characterized by (1) subendothelial thickening on the aortic side of the leaflet, between the basement membrane (PAS-positive) and elastic lamina (Verhoeff-van Gieson), (2) the presence of large amounts of intracellular and extracellular neutral lipids (oil red O) and fine, stippled mineralization (von Kossa), and (3) disruption of the basement membrane overlying the lesion. Regions of the fibrosa adjacent to these lesions were characterized by thickening and by protein, lipid, and calcium accumulation. Control valves showed none of these abnormalities. Immunohistochemical studies were performed using monoclonal antibodies directed against macrophages (anti-CD68 or HAM-56), and contractile proteins of smooth muscle cells or myofibroblasts (anti-alpha-actin and HHF-35) or rabbit polyclonal antiserum against T lymphocytes (anti-CD3). In normal valves, scattered macrophages were present in the fibrosa and ventricularis, and occasional muscle actin-positive cells were detected in the proximal portion of the ventricularis near the leaflet base, but no T lymphocytes were found. In contrast, early lesions were characterized by the presence of an inflammatory infiltrate composed of non-foam cell and foam cell macrophages, occasional T cells, and rare alpha-actin-positive cells. In stenotic aortic valves, a similar but more advanced lesion was seen. CONCLUSIONS: The early lesion of "degenerative" aortic stenosis is an active inflammatory process with some similarities (lipid deposition, macrophage and T-cell infiltration, and basement membrane disruption) and some dissimilarities (presence of prominent mineralization and small numbers of smooth muscle cells) to atherosclerosis.
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4  23482010-03-03
Authors: Borger MA, Preston M, Ivanov J, Fedak PW, Davierwala P, Armstrong S, David TE.
Citation: J Thorac Cardiovasc Surg. 2004 Nov;128(5):677-83.
Abstract: OBJECTIVE: The optimal diameter at which replacement of the ascending aorta should be performed in patients with bicuspid aortic valve disease is not known. METHODS: We reviewed all patients with bicuspid aortic valves undergoing aortic valve replacement at our institution from 1979 through 1993 (n = 201). Patients undergoing concomitant replacement of the ascending aorta were excluded. RESULTS: Follow-up was obtained on 98% of patients and was 10.3 +/- 3.8 (mean +/- SD) years. The average patient age was 56 +/- 15 years, and 76% were male. The ascending aorta was normal (<4.0 cm) in 115 (57%) patients, mildly dilated (4.0-4.4 cm) in 64 (32%) patients, and moderately dilated (4.5-4.9 cm) in 22 (11%) patients. All patients with bicuspid aortic valves with marked dilation (>5.0 cm) underwent replacement of the ascending aorta and were therefore excluded. Fifteen-year survival was 67%. During follow-up, 44 patients required reoperation, predominantly for aortic valve prosthesis failure. Twenty-two patients had long-term complications related to the ascending aorta: 18 required an operative procedure to replace the ascending aorta (for aortic aneurysm), 1 had aortic dissection, and 3 experienced sudden cardiac death. Fifteen-year freedom from ascending aorta-related complications was 86%, 81%, and 43% in patients with an aortic diameter of less than 4.0 cm, 4.0 to 4.4 cm, and 4.5 to 4.9 cm, respectively ( P < .001). CONCLUSIONS: Patients undergoing operations for bicuspid aortic valve disease should be considered for concomitant replacement of the ascending aorta if the diameter is 4.5 cm or greater.
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5  23472010-03-03
Authors: McDonald ML, Smedira NG, Blackstone EH, Grimm RA, Lytle BW, Cosgrove DM.
Citation: J Thorac Cardiovasc Surg. 2000 Jun;119(6):1205-12.
Abstract: OBJECTIVE: We sought to investigate the relationship of female sex, aortic pathology, and left ventricular function to outcome after an operation for aortic regurgitation. METHODS: One hundred nine women underwent aortic valve replacement (n = 92) or repair (n = 17) for pure aortic regurgitation between 1985 and 1996. Mean follow-up was 5.7 +/- 2.6 years. New York Heart Association functional class III-IV symptoms were present in 70 patients, whereas left ventricular function was normal in 60 patients. Ascending aortic diameter in 97% exceeded the 90th percentile for a size-matched healthy population. A concomitant aortic operation was performed by means of root replacement in 31 patients and by means of interposition graft in 28 patients. Of 50 patients undergoing isolated valve procedures, 19 had aortas of 4.0 cm or larger. RESULTS: At 5 and 10 years, survival was 78% and 44%, respectively. Fatal aortic rupture occurred in 13 patients, and 2 others underwent emergency operations for impending aortic rupture, for a total of 15 late aortic events. Freedom from aortic events was 87% and 76% at 5 and 10 years, respectively. Risk factors for aortic events were older age (P =.07) and increasing ascending aortic diameter indexed to body surface area (P =.03) in women who had not undergone replacement of the ascending aorta. Rupture location was at the ascending aorta in 71% without ascending replacement and the descending aorta in 62% with ascending grafts. CONCLUSION: In women, late survival after an operation for aortic regurgitation is importantly decreased by coexisting aortic pathology with subsequent aortic rupture. Aortic replacement at the time of a valve operation should be considered on the basis of indexed aortic size.
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6  23462010-03-03
Authors: Zehr KJ, Orszulak TA, Mullany CJ, Matloobi A, Daly RC, Dearani JA, Sundt TM 3rd, Puga FJ, Danielson GK, Schaff HV.
Citation: Circulation. 2004 Sep 14;110(11):1364-71. Epub 2004 Aug 16.
Abstract: BACKGROUND: This study evaluated long-term results of aortic root replacement and valve-preserving aortic root reconstruction for patients with aneurysms involving the aortic root. METHODS AND RESULTS: Two-hundred three patients aged 53+/-16 years (mean+/-SD; 153 male, 50 female) underwent elective or urgent aortic root surgery from 1971 to 2000 for an aortic root aneurysm: 149 patients underwent a composite valve conduit reconstruction, and 54 patients underwent valve-preserving aortic root reconstruction. Fifty patients had Marfan syndrome. In-hospital and 30-day mortality was 4.0% (8/203) overall: for a composite valve conduit procedure, the corresponding value was 4.0% (6/149) and for valve-preserving procedure, 3.7% (2/54) (P=NS). Morbidity included 3 strokes (1%), 10 perioperative myocardial infarctions (5%), and 8 reoperations for bleeding (4%). Actuarial survival at 5, 10, 15, and 20 years was 93% (95% confidence interval [CI] = 88% to 97%), 79% (95% CI = 71% to 87%), 67% (95% CI = 57% to 79%), and 52% (95% CI = 36% to 69%), respectively. Freedom from reoperation was 72% (95% CI = 54% to 86%) at 20 years. Complications with anticoagulation occurred in 29 patients; with valve thrombosis, in 2; and with hemorrhage, in 27 (4 life threatening and 23 minor). Freedom from thromboembolism was 91% (95% CI = 77% to 98%) at 20 years. Freedom from endocarditis was 99% (95% CI = 92% to 100%) at 20 years. Multivariate analysis revealed preoperative mitral valve regurgitation (+3 to 4) and older age to be significant predictors of late death (P< or =0.005), and Marfan syndrome, initial valve-preserving aortic root reconstruction, and need for a concomitant procedure at initial operation to be significant predictors of the need for reoperation (P< or =0.01). CONCLUSIONS: Aortic root replacement for aortic root aneurysms can be done with low morbidity and mortality. Composite valve conduit reconstruction resulted in a durable result. There were few serious complications related to the need for long-term anticoagulation or a prosthetic valve. Reoperation was most commonly required because of failure of the aortic valve when a valve-preserving aortic root reconstruction was performed or for other cardiac or aortic disease elsewhere.
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7  23452010-03-03
Authors: Kallenbach K, Hagl C, Walles T, Leyh RG, Pethig K, Haverich A, Harringer W.
Citation: Ann Thorac Surg. 2002 Dec;74(6):2026-32; discussion 2032-3.
Abstract: BACKGROUND: This study assesses the durability and clinical outcome of valve-sparing aortic root reconstruction using the reimplantation technique in a single center cohort. METHODS: From July 1993 to July 2001, 158 patients underwent replacement of the ascending aorta with native valve reimplantation. Mean age of patients was 52 +/- 17 years (9 to 84 years), 103 were men (65%). Thirty-four patients (22%) suffered from Marfan's syndrome. Aortic dissection Stanford type A was present in 29 patients (19%) (22 acute, 7 chronic), and concomitant partial or total arch replacement was necessary in 57 patients (36%). One or more additional procedures were performed in 28 patients (18%). Mean follow-up was 36 +/- 25 months (0.4 to 96 months). RESULTS: Thirty-day mortality was 3.8% (6 patients), but only 2.2% in elective patients. Mean bypass time was 169 +/- 50 minutes (99 to 440 minutes), aortic cross-clamp time was 129 +/- 31 minutes (79 to 205 minutes). In patients undergoing arch replacement, circulatory arrest was 26 +/- 18 minutes (7 to 99 minutes). During follow-up, there were 5 (3.3%) cardiac-related late deaths. Grade of aortic insufficiency (AI) decreased from 2.3 +/- 1.1 (0 to 4) preoperatively to 0.23 +/- 0.44 (0 to 2) postoperatively (p < 0.0001). Six patients required aortic valve replacement, 4 of those due to progressive AI. Average grade of AI increased significantly to 0.42 +/- 0.61 (0 to 3) at latest evaluation (p = 0.002). Two patients experienced a transient ischemic attack within the first postoperative week. No further thromboembolic complications were noticed. All patients presented with a favorable exercise tolerance. CONCLUSIONS: The aortic valve reimplantation technique achieves excellent clinical outcome with few complications even in complex pathologies. Lack of anticoagulation and favorable durability encourage wider and earlier use of this technique.
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8  23442010-03-03
Authors: David TE, Armstrong S, Ivanov J, Webb GD.
Citation: Ann Thorac Surg. 1999 Jun;67(6):1840-2; discussion 1853-6.
Abstract: BACKGROUND: Aortic valve sparing operations in patients with ascending aorta and/or aortic root aneurysms have been performed for a decade in our institution. Initially only patients with normal aortic valve leaflets had these operations, but more recently we utilized them in patients with prolapse of a single leaflet and in those with a bicuspid aortic valve. This article is an update on the clinical results of these operations. METHODS: From May 1988 to December 1997, 126 patients with ascending aorta and/or aortic root aneurysms and aortic insufficiency underwent replacement of the ascending aorta with reconstruction of the aortic root and preservation of the native aortic valve. There were 85 men and 41 women, with a mean age of 54 years (range, 14 to 84). Thirty-two patients had the Marfan syndrome; 17 patients had acute and 10 had chronic type A aortic dissection; 23 had a transverse arch aneurysm; 26 had coronary artery disease, and 8 had mitral regurgitation. The aortic valve sparing operation consisted of simple adjustment of the sinotubular junction in 33 patients, adjustment of the sinotubular junction and replacement of one or more aortic sinuses in 60, and reimplantation of the aortic valve in a tubular Dacron (C.R. Bard, Haverhill, PA) graft in 33. Fifteen patients also had repair of aortic leaflet prolapse. Only 4 patients had a bicuspid aortic valve. RESULTS: There were 3 operative deaths due to cardiac failure. Patients were followed from 2 to 117 months, with a mean of 31. There were 11 late deaths: 7 cardiovascular and 4 from unrelated causes. The actuarial survival was 72 +/- 8% at 7 years. Two patients required aortic valve replacement; the freedom from aortic valve replacement was 97 +/- 2% at 7 years. Doppler echocardiography revealed absent, trivial or mild aortic insufficiency in most patients; only 9 patients had moderate aortic insufficiency. CONCLUSIONS: Aortic valve sparing operations are feasible in most patients with ascending aorta and/or aortic root aneurysms who have normal or near normal aortic leaflets. The functional results of the repaired aortic valve are excellent, and the repair appears to be durable.
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9  23432010-03-03
Authors: Johnston KW, Rutherford RB, Tilson MD, Shah DM, Hollier L, Stanley JC.
Citation: J Vasc Surg. 1991 Mar;13(3):452-8.
Abstract: The literature on arterial aneurysms is subject to potential misinterpretation because of inconsistencies in reporting standards. The joint councils of the Society for Vascular Surgery and the North American Chapter of the International Society for Cardiovascular Surgery appointed an ad hoc committee to address this issue. This communication, prepared in response to the need for standardized reporting, defines and classifies arterial aneurysms and recommends standards for describing the causes, manifestations, treatment, and outcome criteria that are important when publishing data on aneurysmal disease.
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10  23422010-03-03
Authors: Roman MJ, Devereux RB, Kramer-Fox R, O'Loughlin J.
Citation: Am J Cardiol. 1989 Sep 1;64(8):507-12.
Abstract: Two-dimensional echocardiography is increasingly used to measure aortic root dimensions, which provide prognostic information in aortic regurgitation and the Marfan syndrome. Aortic root dilatation is currently detected by nomograms based on M-mode echocardiographic data. Aortic root diameters measured by 2-dimensional echocardiography at the anulus, sinuses of Valsalva, supra-aortic ridge and proximal ascending aorta in 135 normal adults and 52 normal children were compared with age, gender, body habitus, blood pressure and stroke volume, and with M-mode findings and normal limits. Two-dimensional measurements at the sinuses of Valsalva were larger than M-mode aortic root values (p less than 0.001), and use of 2-dimensional values with M-mode nomograms falsely diagnosed aortic dilatation in 40% of normal children and 19% of normal adults. Two-dimensional measurements at the sinuses closely correlated with body surface area in children (r = 0.93, p less than 0.0005), moderately in adults younger than 40 years of age (r = 0.71, p less than 0.0005) and weakly in older adults (r = 0.40, p less than 0.0005). In adults, gender influenced aortic root size at all levels (p less than 0.001), but dimensions were similar when indexed for body surface area. Age strongly influenced supraaortic ridge and ascending aortic diameters; blood pressure and stroke volume had no independent effect on aortic size. In conclusion, (1) 2-dimensional echocardiographic aortic root dimensions are influenced by age and body size but not by blood pressure; (2) aortic root dilatation is overdiagnosed when aortic diameter at the sinuses of Valsalva is compared with M-mode nomograms; (3) nomograms comparing aortic diameter with body surface area should be used in children; and (4) although use of nomograms based on body size in adults should maximize sensitivity for aortic dilatation, 98% specificity is attained by use of an upper normal limit of 2.1 cm/m2 for aortic diameter at the sinuses of Valsalva in both men and women.
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11  23412010-03-03
Authors: Shores J, Berger KR, Murphy EA, Pyeritz RE.
Citation: N Engl J Med. 1994 May 12;330(19):1335-41.
Abstract: BACKGROUND. The aortic root enlarges progressively in Marfan's syndrome, and this enlargement is associated with aortic regurgitation and dissection. Long-term treatment with beta-adrenergic blockade, by reducing the impulse (i.e., the rate of pressure change in the aortic root) of left ventricular ejection and the heart rate, may protect the aortic root. METHODS. We conducted an open-label, randomized trial of propranolol in adolescent and adult patients with classic Marfan's syndrome (32 treated and 38 untreated [control] patients). Aortic-root dimensions and clinical end points (aortic regurgitation, aortic dissection, cardiovascular surgery, congestive heart failure, and death) were monitored for an average of 9.3 years in the control group and 10.7 years in the treatment group. All 70 patients were included in the analysis according to the intention-to-treat principle. RESULTS. The dose of propranolol was individualized; the mean (+/- SE) dose was 212 +/- 68 mg per day. The mean slope of the regression line for the aortic-root dimensions, which reflect the rate of dilatation, was significantly lower in the treatment group than in the control group (0.023 vs. 0.084 per year, P < 0.001). Clinical end points were reached in five patients in the treatment group and nine in the control group. The Kaplan-Meier survival curve for the treatment group differed significantly from that for the control group during the middle years of the trial and remained better for the treatment group throughout the study. CONCLUSIONS. Prophylactic beta-adrenergic blockade is effective in slowing the rate of aortic dilatation and reducing the development of aortic complications in some patients with Marfan's syndrome.
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12  23402010-03-03
Authors: Elefteriades JA.
Citation: Ann Thorac Surg. 2002 Nov;74(5):S1877-80; discussion S1892-8.
Abstract: BACKGROUND: The natural history of thoracic aortic aneurysm is incompletely understood. Over the last 10 years, at Yale University we have maintained a large computerized database of patients with thoracic aortic aneurysms and dissections. Analysis of this database has permitted insight into fundamental issues of natural behavior of the aorta and development of criteria for surgical intervention. METHODS: Specialized statistical methods were applied to the prospectively accumulated database of 1600 patients with thoracic aneurysm and dissection, which includes 3000 serial imaging studies and 3000 patient years of follow-up. RESULTS: Growth rate: the aneurysmal thoracic aorta grows at an average rate of 0.10 cm per year (0.07 for ascending and 0.19 for descending). Critical sizes: hinge points for natural complications of aortic aneurysm (rupture or dissection) were found at 6.0 cm for the ascending aorta and 7.0 cm for the descending. By the time a patient achieved these critical dimensions the likelihood of rupture or dissection was 31% for the ascending and 43% for the descending aorta. Yearly event rates: a patient with an aorta that has reached 6 cm maximal diameter faces the following yearly rates of devastating adverse events: rupture (3.6%), dissection (3.7%), death (10.8%), rupture, dissection, or death (14.1%). Surgical risks: risk of death from aortic surgery for thoracic aortic aneurysm was 2.5% for the ascending and arch and 8% for the descending and thoracoabdominal aorta. Genetic analysis: family pedigrees confirm that 21% of probands with thoracic aortic aneurysm have first-order family members with arterial aneurysm. CONCLUSIONS: In risk/benefit analysis the accumulated data strongly support a policy of preemptive surgical extirpation of the asymptomatic aneurysmal thoracic aorta to prevent rupture and dissection. We recommend intervention for the ascending aorta at 5.5 cm and for the descending aorta at 6.5 cm. For Marfan's disease or familial thoracic aortic aneurysm, we recommend earlier intervention at 5.0 cm for the ascending and 6.0 cm for the descending aorta. Symptomatic aneurysms must be resected regardless of size. Family members should be evaluated.
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13  23392010-03-03
Authors: Svensson LG, Kim KH, Lytle BW, Cosgrove DM.
Citation: J Thorac Cardiovasc Surg. 2003 Sep;126(3):892-3.
Abstract: No abstract available.
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14  23382010-03-03
Authors: Davies RR, Goldstein LJ, Coady MA, Tittle SL, Rizzo JA, Kopf GS, Elefteriades JA.
Citation: Ann Thorac Surg. 2002 Jan;73(1):17-27; discussion 27-8.
Abstract: BACKGROUND: Prior work has clarified the cumulative, lifetime risk of rupture or dissection based on the size of thoracic aneurysms. Ability to estimate simply the yearly rate of rupture or dissection would greatly enhance clinical decision making for specific patients. Calculation of such a rate requires robust data. METHODS: Data on 721 patients (446 male, 275 female; median age, 65.8 years; range, 8 to 95 years) with thoracic aortic disease was prospectively entered into a computerized database over 9 years. Three thousand one hundred fifteen imaging studies were available on these patients. Five hundred seventy met inclusion criteria in terms of length of follow-up and form the basis for the survival analysis. Three hundred four patients were dissection-free at presentation; their natural history was followed for rupture, dissection, and death. Patients were excluded from analysis once operation occurred. RESULTS: Five-year survival in patients not operated on was 54% at 5 years. Ninety-two hard end points were realized in serial follow-up, including 55 deaths, 13 ruptures, and 24 dissections. Aortic size was a very strong predictor of rupture, dissection, and mortality. For aneurysms greater than 6 cm in diameter, rupture occurred at 3.7% per year, rupture or dissection at 6.9% per year, death at 11.8%, and death, rupture, or dissection at 15.6% per year. At size greater than 6.0 cm, the odds ratio for rupture was increased 27-fold (p = 0.0023). The aorta grew at a mean of 0.10 cm per year. Elective, preemptive surgical repair restored life expectancy to normal. CONCLUSIONS: This study indicates that (1) thoracic aneurysm is a lethal disease; (2) aneurysm size has a profound impact on rupture, dissection, and death; (3) for counseling purposes, the patient with an aneurysm exceeding 6 cm can expect a yearly rate of rupture or dissection of at least 6.9% and a death rate of 11.8%; and (4) elective surgical repair restores survival to near normal. This analysis strongly supports careful radiologic follow-up and elective, preemptive surgical intervention for the otherwise lethal condition of large thoracic aortic aneurysm.
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15  23372010-03-03
Authors: Roberts CS, Roberts WC.
Citation: J Am Coll Cardiol. 1991 Mar 1;17(3):712-6.
Abstract: The association of congenital aortic valve malformation and aortic dissection is analyzed. Over a 30 year period, 186 patients with non-iatrogenic aortic dissection were studied at necropsy. The aortic valve was tricuspid in 170 (91.4%), bicuspid in 14 (7.5%) and unicuspid in 2 (1.1%). Among the 16 patients with aortic dissection and a congenitally malformed valve, the age at death ranged from 17 to 82 years (mean 52) and 13 (81%) were men. The entrance tear of the aortic dissection was located in the ascending aorta in all 16 patients with a malformed valve but in only 68% of those with a tricuspid aortic valve. The aortic valve was stenotic in 6 of the 16 patients with a congenitally malformed valve. Fatal rupture of the false channel occurred after acute ascending aortic dissection in each of the 11 patients (none with healed dissection) who did not have operative therapy for the dissection. Two of the 16 patients with a malformed valve compared with no patient with a tricuspid aortic valve had aortic isthmic coarctation. Histologic sections of aorta from 10 patients disclosed severe degeneration of the elastic fibers of the media in 9 patients. Thus, a congenitally malformed aortic valve appears to be present at least 5 times more frequently in adults with than in those without aortic dissection, and in our patients the entrance tear was always in the ascending aorta, which usually had severe loss of elastic fibers in its media.
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16  23362010-03-03
Authors: Ferencik M, Pape LA.
Citation: Am J Cardiol. 2003 Jul 1;92(1):43-6.
Abstract: Bicuspid aortic valve (BAV) is associated with premature valve dysfunction and abnormalities of the ascending aorta. Limited data exist regarding serial changes of aortic dilation in patients with BAV. We studied paired transthoracic echocardiograms of 68 patients with BAV (mean age 44 years) and with at least 2 examinations >12 months apart (mean follow-up 47 months) to characterize the progression of aortic dilation and the natural history of valve function. We measured aortic root and ascending aortic diameters at baseline and follow-up. We measured aortic gradients and severity of aortic regurgitation (AR). During follow-up, aortic diameters increased at the sinuses of Valsalva by 1.9 mm (95% confidence interval [CI] 1.3 to 2.5), at the sinotubular junction by 1.6 mm (95% CI 0.8 to 2.3), and at the proximal ascending aorta by 2.7 mm (95% CI 1.9 to 3.6). Mean rate of diameter progression was 0.5 mm/year at the sinuses of Valsalva (95% CI 0.3 to 0.7), 0.5 mm/year at the sinotubular junction (95% CI 0.3 to 0.7), and 0.9 mm/year at the proximal ascending aorta (95% CI 0.6 to 1.2). Progression was observed regardless of hemodynamic function at baseline. Mean aortic valve gradient increased significantly from baseline to follow-up (17.6 mm Hg vs 25.7 mm Hg, p <0.001). The degree of AR increased during follow-up in 17 patients (25%). In addition, progression of aortic diameter dilation occurred irrespective of baseline valve function in adult patients with BAV. We also observed considerable progression of aortic gradients and AR over time.
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17  23352010-03-03
Authors: Fedak PW, Verma S, David TE, Leask RL, Weisel RD, Butany J.
Citation: Circulation. 2002 Aug 20;106(8):900-4.
Abstract: No abstract available.
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18  23342010-03-03
Authors: Keane MG, Wiegers SE, Plappert T, Pochettino A, Bavaria JE, Sutton MG.
Citation: Circulation. 2000 Nov 7;102(19 Suppl 3):III35-9.
Abstract: BACKGROUND: Bicuspid aortic valves (BAVs) are associated with premature valve stenosis, regurgitation, and ascending aortic aneurysms. We compared aortic size in BAV patients with aortic size in control patients with matched valvular lesions (aortic regurgitation, aortic stenosis, or mixed lesions) to determine whether intrinsic aortic abnormalities in BAVs account for aortic dilatation beyond that caused by valvular hemodynamic derangement alone. METHODS AND RESULTS: Diameters of the left ventricular outflow tract, sinus of Valsalva, sinotubular junction, and proximal aorta were measured from transthoracic echocardiograms in 118 consecutive BAV patients. Annular area was measured by planimetry, and BAV eccentricity was expressed as the ratio of the right leaflet area to the total annular area. Seventy-seven control patients with tricuspid aortic valves were matched for sex and for combined severity of regurgitation and stenosis. BAV patients (79 men and 39 women, aged 44.1+/-15.5 years) had varying degrees of regurgitation (84 patients [71%]) and stenosis (48 patients [41%]). Within the bicuspid group, multivariate analysis demonstrated that aortic diameters increased with worsening aortic regurgitation (P:<0.001) and advancing age (P:<0.05) but not with the severity of aortic stenosis. BAV patients had larger aortic diameters than did control patients at all ascending aortic levels measured (P:<0.01), despite advanced age in the control patients. CONCLUSIONS: Aortic dimensions are larger in BAV patients than in control patients with comparable degrees of tricuspid aortic valve disease. Although more severe degrees of aortic regurgitation are associated with aortic dilatation in BAV patients, intrinsic pathology appears to be responsible for aortic enlargement beyond that predicted by hemodynamic factors.
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19  23332010-03-03
Authors: Nistri S, Sorbo MD, Marin M, Palisi M, Scognamiglio R, Thiene G.
Citation: Heart. 1999 Jul;82(1):19-22.
Abstract: OBJECTIVE: To evaluate the dimensions of the aortic root in a selected population of young males with isolated normally functioning bicuspid aortic valve. DESIGN AND SETTING: Echocardiographic and Doppler evaluation of conscripts with bicuspid aortic valve at the time of military pre-enrolment screening in two military hospitals. SUBJECTS AND METHODS: 66 consecutive young men with a normally functioning bicuspid aortic valve were studied to assess aortic size at four aortic levels: annulus, sinuses of Valsalva, supra-aortic ridge, and proximal ascending aorta; 70 consecutive normal young subjects, matched for age and body surface area, were used as controls. RESULTS: In men with a bicuspid aortic valve, the diameter of the aortic root was significantly larger than in controls at the sinuses (3.16 (0.37) v 2.87 (0.31) cm, p < 0.001), at the supra-aortic ridge (2.64 (0.46) v 2.47 (0.28) cm, p = 0.01), and at the level of the proximal ascending aorta (3.12 (0.48) v 2.69 (0.28) cm, p < 0.001). The prevalence of aortic root dilatation was 7.5% at the annulus (5/66), 19.6% at the sinuses (13/66), 15% at the supra-aortic ridge (10/66), and 43.9% at the ascending aorta (29/66); 32 subjects (48%) had aortic root dimensions comparable with controls, while 34 (52%) had definitely abnormal aortic root dimensions. CONCLUSIONS: Aortic root enlargement in people with a bicuspid aortic valve occurs independently of haemodynamic abnormalities, age, and body size. However, there appear to be different subgroups of young adults with bicuspid aortic valves, one of which is characterised by aortic dilatation, possibly caused by a congenital abnormality of the aortic wall.
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20  23322010-03-03
Authors: Hahn RT, Roman MJ, Mogtader AH, Devereux RB.
Citation: J Am Coll Cardiol. 1992 Feb;19(2):283-8.
Abstract: To determine whether aortic root dilation associated with a bicuspid aortic valve occurs independently of valvular hemodynamic abnormality, aortic root dimensions were measured by two-dimensional echocardiography in 83 adults with a functionally normal (n = 19), mildly regurgitant (n = 26), severely regurgitant (n = 27) or stenotic (n = 11) bicuspid aortic valve and compared with findings in normal subjects matched for age and gender. Aortic root measurements were made at four levels: anulus, sinuses of Valsalva, supraaortic ridge and proximal ascending aorta. Seventy-one percent of patients with a bicuspid aortic valve were men. When compared with control subjects, all hemodynamic subgroups showed a significantly larger aortic root size at three levels: sinuses of Valsalva, supraaortic ridge and proximal ascending aorta (p less than 0.05 to p less than 0.001). The prevalence of aortic root enlargement among all hemodynamic subgroups ranged from 9% to 59% at the level of the anulus, 36% to 78% at the sinuses, 47% to 79% at the supraaortic ridge and 50% to 64% in the ascending aorta. Thus, there is a high prevalence of aortic root enlargement in patients with a bicuspid aortic valve that occurs irrespective of altered hemodynamics or age. These findings support the hypothesis that bicuspid aortic valve and aortic root dilation may reflect a common developmental defect.
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21  23312010-03-03
Authors: Braverman AC, Güven H, Beardslee MA, Makan M, Kates AM, Moon MR.
Citation: Curr Probl Cardiol. 2005 Sep;30(9):470-522.
Abstract: The bicuspid aortic valve affects 1 to 2% of the population and may be complicated by aortic stenosis or aortic insufficiency and infective endocarditis. The bicuspid aortic valve is associated with abnormalities of the aortic wall such as coarctation of the aorta, aortic dissection, and aortic aneurysm. Most patients with a bicuspid aortic valve will develop some complication during life. Individuals with a bicuspid valve may be unaware of its presence and are at risk for unsuspected complications. Aortic wall abnormalities associated with bicuspid aortic valve are due to cystic medial necrosis. This process is associated with increased metalloproteinase activity and apoptosis of vascular smooth muscle cells. The clinical correlates of aortopathy in the bicuspid aortic valve include significant enlargement of the ascending aorta with aneurysm formation and dissection. This process continues after valve replacement. The person with bicuspid aortic valve requires continuous surveillance to treat associated lesions and prevent complications.
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22  23302010-03-03
Authors: Nataatmadja M, West M, West J, Summers K, Walker P, Nagata M, Watanabe T.
Citation: Circulation. 2003 Sep 9;108 Suppl 1:II329-34.
Abstract: BACKGROUND: Marfan syndrome (MS) is a genetic disorder caused by a mutation in the fibrillin gene FBN1. Bicuspid aortic valve (BAV) is a congenital heart malformation of unknown cause. Both conditions are associated with ascending aortic aneurysm and premature death. This study examined the relationship among the secretion of extracellular matrix proteins fibrillin, fibronectin, tenascin, and vascular smooth muscle cell (VSMC) apoptosis. The role of matrix metalloproteinase (MMP)-2 in VSMC apoptosis was studied in MS aneurysm. METHODS AND RESULTS: Aneurysm tissue was obtained from patients undergoing surgery (MS: 4 M, 1 F, age 27-45 years; BAV: 3 M, 2 F, age 28-65 years). Normal aorta from subjects with nonaneurysm disease was also collected (4 M, 1 F, age 23-93 years). MS and BAV aneurysm histology showed areas of cystic medial necrosis (CMN) without inflammatory infiltrate. Immunohistochemical study of cultured MS and BAV VSMC showed intracellular accumulation and reduction of extracellular distribution of fibrillin, fibronectin, and tenascin. Western blot showed no increase in expression of fibrillin, fibronectin, or tenascin in MS or BAV VSMC and increased expression of MMP-2 in MS VSMCs. There was 4-fold increase in loss of cultured VSMC incubated in serum-free medium for 24 hours in both MS (27+/-8%) and BAV (32+/-14%) compared with control (7+/-5%). CONCLUSIONS: In MS and BAV there is alteration in both the amount and quality of secreted proteins and an increased degree of VSMC apoptosis. Up-regulation of MMP-2 might play a role in VSMC apoptosis in MS VSMC. The findings suggest the presence of a fundamental cellular abnormality in BAV thoracic aorta, possibly of genetic origin.
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23  23292010-03-03
Authors: Elayda MA, Hall RJ, Reul RM, Alonzo DM, Gillette N, Reul GJ Jr, Cooley DA.
Citation: Circulation. 1993 Nov;88(5 Pt 2):II11-6.
Abstract: BACKGROUND. Forty percent of 7.4 million Americans aged 80 years and older have symptomatic heart disease. Controversy exists as to whether the health care resources allotted to this patient subset represent a cost-effective approach to attaining a meaningful quality of life. Although aortic valve surgery carries greater risks in older than in younger patients, published studies reveal that the elderly should not be denied this procedure. METHODS AND RESULTS. To determine the results of aortic valve replacement (AVR) in an elderly population, we retrospectively analyzed 171 consecutive patients aged 80 to 91 years (mean, 82.6 years; 86 men and 85 women) who underwent AVR at the Texas Heart Institute between 1975 and 1991. Seventy-seven patients had AVR only, and 94 patients had concomitant surgical procedures (coronary artery bypass graft surgery, 75 patients; mitral valve replacement, mitral valve repair, aneurysm repair, 19 patients). The overall 30-day early mortality was 17.5%. The early mortality was 5.2% for patients with AVR only and 27.7% for those with concomitant surgical procedures. Statistical analysis of 17 perioperative variables revealed that left ventricular ejection fraction of less than 45%, hypertension, congestive heart failure, angina, and concomitant surgical procedures were significant univariate predictors of early mortality. Multivariate analysis revealed that left ventricular ejection fraction of less than 45%, hypertension, and concomitant surgical procedures were independent predictors of operative mortality. Mean follow-up of survivors was 39 months. The overall actuarial survival at 1, 3, and 5 years was 90.8%, 84.2%, and 76.0%, respectively. CONCLUSIONS. These results show that AVR can be performed with acceptable operative risks in the elderly. This study further shows that isolated AVR can be done with low operative mortality and that the performance of concomitant surgical procedures exposes elderly patients to higher operative risks.
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24  23282010-03-03
Authors: Schuler G, Peterson KL, Johnson AD, Francis G, Ashburn W, Dennish G, Daily PO, Ross J Jr.
Citation: Am J Cardiol. 1979 Oct;44(4):585-94.
Abstract: No abstract available.
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25  23272010-03-03
Authors: Boucher CA, Bingham JB, Osbakken MD, Okada RD, Strauss HW, Block PC, Levine FH, Phillips HR, Pohost GM.
Citation: Am J Cardiol. 1981 May;47(5):991-1004.
Abstract: No abstract available.
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