Characteristics of coronary artery blood flow waveforms and the mechanism of myocardial ischemia in aortic valve disease.
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Myocardial Na+,K(+)-ATPase was studied in patients with aortic valve disease, and myocardial Na+,K(+)- and Ca(2+)-ATPase were assessed in spontaneously hypertensive rats (SHR) and hereditary cardiomyopathic hamsters using methods ensuring high enzyme recovery. Na+,K(+)-ATPase was quantified by [3H]ouabain binding to intact myocardial biopsies from patients with aortic valve disease. Aortic stenosis, regurgitation and a combination hereof were compared with normal human heart and were associated with reductions of left ventricular [3H]ouabain binding site concentration (pmol/g wet weight) of 56, 46 and 60%, respectively (p < 0.01). Na+,K(+)- and Ca(2+)-ATPases were quantified by K(+)- and Ca(2+)-dependent p-nitrophenyl phosphatase (pNPPase) activity determinations in crude myocardial homogenates from SHR and hereditary cardiomyopathic hamsters. When SHR were compared to age-matched Wistar Kyoto (WKY) rats an increase in heart-body weight ratio of 75% (p < 0.001) was associated with reductions of K(+)- and Ca(2+)-dependent pNPPase activities (mumol/min/g wet weight) of 42 (p < 0.01) and 27% (p < 0.05), respectively. When hereditary cardiomyopathic hamsters were compared to age-matched Syrian hamsters an increase in heart-body weight ratio of 69% (p < 0.001) was found to be associated with reductions in K(+)- and Ca(2+)-dependent pNPPase activities of 50 (p < 0.001) and 26% (p = 0.05), respectively. The reductions in Na+,K(+)- and Ca(2+)-ATPases were selective in relation to overall protein content and were not merely the outcome of increased myocardial mass relative to Na+,K(+)- and Ca(2+)-pumps. In conclusion, myocardial hypertrophy is in patients associated with reduced Na+,K(+)-ATPase concentration and in rodents with reduced Na+,K(+)- and Ca(2+)-ATPase concentrations. This may be of importance for development of heart failure and arrhythmia in hypertrophic heart disease.
To determine the prevalence and significance of exercise-induced localized perfusion defects in symptomatic patients with aortic valve disease, thallium-201 rest and exercise studies were performed in a consecutive series of 29 such patients prior to left heart catheterization with coronary arteriography. Eight patients had repeat studies after aortic valve replacement. Twelve of 17 patients with predominant aortic regurgitation (AR) had distinct LV apical defects during exercise despite normal coronary arteries, while 10 of 12 patients with aortic valve disease and associated coronary artery disease (CAD) had localized perfusion defects in LV areas other than the apex. In patients with AR, reversible apical perfusion defects can occur without CAD; these apical defects are probably a reflection of severe LV volume overload in AR. LV perfusion defects in areas other than the apex are specific for CAD in aortic valve disease, and concomitant CAD may not provoke regional LV perfusion deficits in aortic stenosis patients with severe LV hypertrophy.
To evaluate the outcome of surgical intervention for end-stage aortic valve disease, we carried out a retrospective, longitudinal survey of 85 patients (65 males, 20 females; mean age 53 period. All the patients presented in New York Heart Association (NYHA) class IV in cardiac failure (3 had cardiogenic shock and 27 had bacterial endocarditis). In-hospital mortality was 9.4% (8/85) overall. Those with endocarditis had a significantly higher mortality, 6/27 (22%) vs 2/58 (3.4%), p < 0.01. In-hospital mortality was not significantly increased in those with renal failure, reoperation, simultaneous coronary artery surgery, age > 65 years nor was it related to the predominance of aortic regurgitation or stenosis. After a mean follow-up period of 5.9 years (range 0 to 12.5 years), the overall actuarial survival was 82% and 74% at 5 and 10 years respectively. For 66 late survivors, the NYHA status improved to class I in 51, to II in 10, to III in 4 patients, and one patient remained in class IV. The incidence of paraprosthetic leak, reoperation, thromboembolism, anticoagulant-related haemorrhage, and endocarditis were respectively 0.8, 0.8, 1.6, 1.4, and 0.2 per 100 patient-years. Aortic valve replacement in the patient with end-stage aortic valve disease is a high-risk procedure, the risk being higher in the presence of endocarditis. The favourable long-term survival, long-term improvement in functional class and the relatively low incidence of valve-related complications justify surgical intervention in such patients, who would otherwise have a very poor prognosis.
To determine resting myocardial perfusion in 30 patients with aortic valve disease (AVD) TI-201 emission computer tomography (ECT) studies and heart catheterization were performed. 16 patients had a predominant aortic regurgitation (AR); an aortic stenosis (AS) was found in 14 patients at catheterization. Perfusion defects were documented in 10 of 16 patients with AR, and in 5 of 14 patients with AS. Regional determination demonstrated in 10 of these 15 abnormal cases at least one of the perfusion defects in the postero-basal segment. In patients with aortic valve disease and normal myocardial perfusion scintigraphy, the ejection fraction (EF) was significantly higher (p < 0.05) as compared to the patients with abnormal TI-201 ECT (EF 65.2 +/- 15.0% vs. 58.9 +/- 15.1%). Additionally, in the 15 patients with abnormal TI-201 ECT, the left ventricular end-diastolic volume index (LVEDVI) and the left ventricular end-systolic volume index (LVESVI) were both increased (LVEDVI in AVD: 130.3 +/- 15.3 ml/m2 vs. 107.4 +/- 13.6 ml/m2, LVESVI in AVD: 61.9 +/- 14.6 ml/m2 vs. 48.0 +/- 9.8 ml/m2). In conclusion, left ventricular perfusion defects at rest can be detected by TI-201 ECT in many patients with aortic valve disease. Such myocardial perfusion defects indicate left ventricular enlargement and impaired left ventricular function, especially in patients with aortic regurgitation.
127 patients with diagnosis of acquired mitral and aortic valve disease confirmed by cardiac catheterization were followed for 5-17 years (mean 10 years) under conservative management. At the end of the study, 80 patients were still alive, and 68 of them were examined again. 23 patients died, and no information was available on 24. Analysis of the natural course of the disease showed that results obtained with different classification systems (NYHA, clinical findings, hemodynamic data) were not comparable. However, the majority of mitral and combined mitral-aortic lesions remained stable over the years, independent on the initial classification of the lesion. Aortic valve disease became hemodynamically worse in the absence of subjective and clinical change.
A retrospective study of 196 patients with rheumatic mitral and aortic valve disease seen at a district general hospital was carried out. Patients having surgical intervention for their valvular disease had a better survival at 10 years (81 per cent +/- 7 per cent) compared to those managed medically (56 per cent +/- 8.5 per cent). This superiority was maintained, both for survival and symptomatic improvement, when individual valve lesions were considered.
Left ventricular function was analyzed by angiography in 31 patients with aortic valve disease and in 12 patients without heart disease (control group). Ejection fraction, percentage shortening of minor equator, mean velocity of fiber shortening and men left atrial pressure were considered as parameters of left ventricular function. Contractile reserve was tested by a single postextrasystolic beat. Patients with pure aortic stenosis and an increase of left ventricular muscle mass to 220% of the normal value showed no impairment of left ventricular function. Patients with pure aortic regurgitation and a left ventricular muscle mass of 260% of normal showed no significantly impaired function. Both groups increased ejection fraction and percentage shortening of the minor equator after premature beat comparable to the control group. Patients with combined lesions of the aortic valve had a left ventricular muscle mass of 360% of normal. This group showed decreased ejection fraction, percentage shortening of minor equator and mean velocity of fiber shortening as compared to the control group while mean left atrial pressure was significantly elevated. After premature beat all parameters remained depressed as compared to control group. We conclude that the degree of hypertrophy determinates cardiac function in aortic valve disease. Moderate hypertrophy shows normal function at rest, while severe hypertrophy shows impaired function.
Mediastinal irradiation has been reported to induce cardiac disease such as pericarditis, valvular dysfunction, conduction abnormalities, accelerated arteriosclerosis of the coronary arteries, and also calcifications of the ascending aorta. We herein describe a case of radiotherapy-induced porcelain aorta and aortic valve disease and their surgical treatment. The patient was diagnosed with myasthenia gravis (MG) in 1965 (Osserman's type II), and mediastinal irradiation was performed in 1970 for treatment of thymic tumor associated with MG. Thirty years after radiation therapy, complete atrioventricular block and aortic valve disease with severe calcification of the ascending aorta and aortic arch (porcelain aorta) were detected on echo cardiogram and cardiac catheterization. A permanent pacemaker was implanted via the left subclavian vein and aortic valve replacement was performed under extracorporeal circulation established by selective cerebral perfusion and balloon occlusion instead of aortic cross-clamping. As no risk factors of arteriosclerosis such as hypercholesterolemia, hyperglycemia and hypertension were apparent, we concluded that the aortic valve disease and porcelain aorta were primarily induced by radiotherapy.
65 patients out of 420 with aortic valve lesions (class III and IV NYHA) who underwent cardiac catheterization in 1967-1976 were not operated upon for different reasons. The fate of these 65 patients was analyzed retrospectively to elucidate the natural history of severe aortic valve disease. The five years survival rate was 26% (aortic stenosis 17%, aortic incompetence 37%). Patients with angina pectoris and congestive heart failure, ventricular ectopic beats, mean pulmonary artery pressure greater than 30 mm Hg, mean left atrial pressure greater than 16 mm Hg and left ventricular enddiastolic pressure greater than 20 mm Hg had a significant worse prognosis than those without these parameters. Otherwise patients who refused the operation by personal reasons had a high survival rate. The results of the study indicate that patients with aortic valve disease class III or IV (NYHA) have a serious prognosis when treated medically. In comparison with our patients who underwent aortic valve replacement surgery has proven to be a life-prolonging procedure in these highly endangered cases.
Anatomically isolated aortic valve disease accounted for 1.1% of all and 5.2% of cardiac autopsies over a 20 year period. Among a total of 100 symptomatic cases, 52% had congenitally bicuspid, 43% had tricuspid and 5% had congenitally unicuspid valves. Nineteen percent had undergone fibrous sclerosis, including 1% unicuspid, 3% tricuspid and 15% bicuspid valves. Thirty nine percent had been affected by infective endocarditis, including 20 bicuspid and 19 tricuspid valves. The remaining 42 showed fibrocalcification, including 4 unicuspid, 17 bicuspid and 21 tricuspid valves. The nature of the valvular disease showed a correlation with the age of the patient. Infective endocarditis, fibrosis and calcific disease occurred in an ascending age pattern, at average ages of around 30 years, mid to late thirties and mid to late forties, respectively. The lesions occurred much earlier on the bicuspid than on the tricuspid valves, except for infective endocarditis. The bicuspid deformity was not found to make the aortic valve more prone to infection, nor did infection occur earlier on it than on the tricuspid valve. Four of the 100 cases, all tricuspid, were considered to be of rheumatic origin, the reasons for which are discussed. Certain well established associated cardiac lesions were identified.
Passive diastolic properties were determined in 10 control patients and 21 patients with aortic valve disease before and 17.5 months after successful valve replacement. Ten patients had severe aortic stenoses (AS), five had combined aortic valve lesions (AS + aortic insufficiency [AI]), and six patients had severe AI. Left ventricular endomyocardial biopsies were obtained before and after surgery in patients with AS, AS + AI, and AI. Simultaneous echocardiographic and high-fidelity pressure measurements were made in all patients, and left ventricular chamber stiffness was calculated from a viscoelastic pressure-circumference relationship and left ventricular myocardial stiffness from a viscoelastic stress-strain relationship. The constant of chamber stiffness, beta', was slightly although not significantly increased in patients with AS (0.27 before and 0.24 after surgery), but was normal in those with AS + AI (0.22 before and 0.17 after surgery) and slightly decreased in those with AI (0.18 before and 0.16 after surgery) when compared with in control subjects (0.21). The constant of myocardial stiffness beta was normal in patients with AS (13.2), AS + AI (11.5), and AI (11.7) before surgery compared with in the control group (12.5). beta increased, however, significantly in those with AS (25.2; p less than .02), but not in those with AS + AI (16.3; NS) and AI (12.8; NS) after surgery. Myocardial morphologic characteristics showed a significant decrease in muscle fiber diameter in patients with AS, AS + AI, and AI, as well as a significant increase in interstitial fibrosis from 15% to 26% (p less than .05) in those with AS and a slight increase from 15% to 22% (NS) in those with AS + AI and from 19% to 24% (NS) in those with AI. Left ventricular fibrous content (left ventricular muscle mass index multiplied by interstitial fibrosis) remained, however, unchanged in all three groups after aortic valve replacement. In conclusion, left ventricular chamber stiffness is increased in AS but decreased in AI, whereas LV myocardial stiffness is normal in patients with aortic valve disease before surgery. After surgery, left ventricular myocardial stiffness increased significantly in AS patients but remained unchanged in those with AI. Postoperative changes in myocardial structure were characterized by a decrease in muscle fiber diameter and a relative increase in interstitial fibrosis, whereas fibrous content remained unchanged. Thus, regression of myocardial hypertrophy in aortic valve disease is accompanied by an increase of myocardial stiffness in concentric hypertrophy that is not seen in eccentric hypertrophy.
Thirty-four patients underwent isolated aortic valve replacement with mean pulmonary artery pressures greater than 40 mmHg between 1972 and 1988. The aortic valve disease was stenotic in 10 cases, regurgitant in 14 cases and mixed in 10 cases. Thirty patients (88%) had invalidating cardiac failure (NYHA Classes III and IV). The mean preoperative ejection fraction was 44 +/- 15%. The hospital mortality was 17.6%. Ten patients died secondarily, five with terminal cardiac failure. The 5 year actuarial survival was 70 +/- 16%; the 10 year survival was 60 +/- 18% with an average follow-up of 115 +/- 61 months. None of the patients was lost to follow-up. Fifteen of the 18 survivors (83%) are asymptomatic or pauci-symptomatic after a follow-up of 126 +/- 62 months. Doppler echocardiography (n = 12) showed normal prosthetic valve function in 11 cases and aortic regurgitation in 1 case. Eight patients had tricuspid regurgitation with pulmonary artery systolic pressures less than 30 mmHg in 6 cases and between 30 and 40 mmHg in 2 cases. Severe pulmonary hypertension is therefore a poor early postoperative prognostic factor in aortic valve replacement surgery due to the associated left ventricular dysfunction. However, the long-term results are satisfactory: clinical improvement is usually related to a reduction of pulmonary hypertension.
OBJECTIVE: The validity of the Ross operation as freestanding root replacement in adult patients with bicuspid aortic valve disease has lately been questioned. We have analyzed retrospectively our results in 23 adult patients (19 males) operated for bicuspid aortic valve disease ad modum "Ross" employing a freestanding root replacement technique. DESIGN: In 9 patients the dominant aortic valve lesion was stenotic (aortic stenosis group) and in the remaining 14 patients it was aortic insufficiency (aortic insufficiency group). The fate of the pulmonary autograft in the two groups was studied. The intraoperatively measured aortic and pulmonary annuli diameters from the two groups were compared with those from a population of normal looking aortic and pulmonary valves matched for body surface area. RESULTS: The aortic insufficiency group needed significant reduction of the aortic annulus diameter to conform to the size of the pulmonary autograft. The pulmonary autograft annuli in this group were significantly larger in diameter than the ones in the aortic stenosis group. The mean pulmonary annulus diameter in the aortic stenosis group was, on the other hand, significantly smaller when compared with that in the normal matched population. After a mean follow-up period of about 19 months, the aortic insufficiency group showed significant dilatation of the neo-aortic sinuses. Between the two groups, the remaining echocardiographic variables remained either stable or improved at follow-up. CONCLUSION: Pre-existing larger diameters of the aortic and pulmonary annuli in the aortic insufficiency group combined with the significantly increased left ventricular end-diastolic diameters, may predispose these patients to significant dilatation of the unsupported aortic sinuses after a Ross operation. This dilatation does not, however, lead to increase in the autograft valve insufficiency at short-term follow-up if the aortic annulus and the distal ascending aorta are tailored to the size of the pulmonary autograft. Ross operation, employing freestanding aortic root replacement technique, may therefore be recommended in adult patients with bicuspid aortic valve disease with excellent short-term results.
In 14 patients (pts) with aortic valve disease (AVD) left ventricular (LV) relaxation was assessed by the time constant (T) of LV pressure (tipmanometer) fall before and 19 months after successful aortic valve replacement (AVR). 12 control pts (CO) were studied by the same technique. Preoperative LV ejection fraction in AVD (64%) and in CO (69%) did not differ. In AVD T was increased (60 ms) as compared to the CO (38 ms, P less than 0.05). During handgrip (HG) there was a similar increase of LV peak systolic pressure (LVSP), heart rate and peak measured contractile element velocity of shortening in AVD and in the CO. LV end-diastolic pressure varied minimally in both groups. T decreased during handgrip in CO (38 to 33 ms, P less than 0.01) and remained unchanged in AVD. Following AVR T at rest decreased insignificantly to 52 ms, but remained increased (P less than 0.025) as compared with CO. During postoperative HG however, a decrease to 47 ms (P less than 0.05) was noted. Postoperative angiographic LV muscle mass (105 g/m2) and LVSP at rest (137 mmHg) remained elevated (P less than 0.02) as compared to CO (72 g/m2; 119 mmHg). It is concluded that (1) in AVD with normal ejection performance LV relaxation at rest is prolonged and the reaction of relaxation to HG is abnormal despite preserved contractile response, (2) following AVR the response of LV relaxation to HG becomes normal and (3) elevated postoperative T at rest appears to be related to residual hypertrophy and probably also to the still increased LVSP rather than to intrinsic disturbances of myocardial relaxation.
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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