Noninvasive assessment of pulmonary valve stenosis, aortic valve stenosis and coarctation of the aorta in critically ill neonates.
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OBJECTIVES: The aim of this study was to evaluate the effect of aortic valve replacement (AVR) on left ventricular (LV) function and LV remodeling, comparing patients with aortic valve stenosis to patients with aortic regurgitation. BACKGROUND: Aortic valve disease is associated with eccentric or concentric LV hypertrophy and changes in LV function. The relationship between LV geometry and LV function and the effect of LV remodeling after AVR on diastolic filling, in patients with aortic valve stenosis compared with aortic regurgitation, are largely unknown.Nineteen patients with aortic valve disease (12 aortic valve stenosis, 7 aortic regurgitation) were studied using magnetic resonance imaging to assess LV geometry and LV function before and 9 +/- 3 months after AVR. Ten age-matched healthy males served as control subjects. RESULTS: Before AVR, the ratio between left ventricular mass index (LVMI) and left ventricular end-diastolic volume index (LVEDVI) was only increased in patients with aortic valve stenosis (1.37 +/- 0.16 g/ml) compared with control subjects (0.93 +/- 0.08 g/ml, p < 0.05). After AVR, LVMI/LVEDVI decreased significantly in aortic valve stenosis (to 1.15 +/- 0.14 g/ml, p < 0.0001), but increased significantly in aortic regurgitation (1.02 +/- 0.20 g/ml to 1.44 +/- 0.27 g/ml, p < 0.0001). Before AVR, diastolic filling was impaired in both aortic valve stenosis and aortic regurgitation. Early after AVR, diastolic filling improved in patients with aortic valve stenosis, whereas patients with aortic regurgitation showed a deterioration in diastolic filling. CONCLUSIONS: Early after AVR, patients with aortic valve stenosis show a decrease in both LVMI and LVMI/LVEDVI and an improvement in diastolic filling, whereas in patients with aortic regurgitation, LVMI decreases less rapidly than LVEDVI, causing concentric remodeling of the LV, most likely explaining the observed deterioration of diastolic filling in these patients.
Calcified aortic valves were detected in 109 patients studied in hospital by an echocardiographic method (2D- and M-scanning). It was found that, unlike rheumatism, calcification starting in the aortic annulus and semilunar bases might result in formation of degenerative (non-inflammatory) calcific aortic stenosis. The latter differs from rheumatic aortic stenosis in commissural fusions. The authors defined clinical and echocardiographic differentially diagnostic criteria for degenerative (non-inflammatory) and rheumatic (inflammatory) calcific aortic stenosis. They also discussed pathogenetic aspects of aortic valve calcification and problems of terminology.
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Aortic stenosis is found in 15 to 25% of patients with gastrointestinal angiodysplasia. The usual treatment for haemorrhagic angiodysplasia associated with aortic stenosis is the same as for other types of gastrointestinal angiodysplasias: segmental intestinal resection, electrocoagulation and laser photocoagulation. The authors report the case of a 73 year old woman with a long history of gastro-intestinal bleeding and chronic anaemia requiring a number of hospital admissions for blood transfusions. The cause of this bleeding remained obscure for many years, as it was initially thought to be due to portal hypertension complicating cyrrhosis and a surgical porto-caval shunt was performed. Later, angiodysplasia of the colon was recognised and a segmental colonic resection was performed. These two surgical procedures had no effect on the chronic bleeding and finally the patient was referred for a gram negative endocarditis complicating aortic stenosis, previously considered to be non-surgical. After controlling the infection, the patient was sent for surgery of the aortic valve disease with mitral regurgitation in view of progressive degradation of left ventricular function. A double valve replacement with bioprostheses was undertaken with no complication. Finally, three years now after valve replacement, no further bleeding has occurred and control colonoscopy is normal. In the light of this case and a review of the literature of about 30 similar cases, the physiopathology and management of these patients is discussed with respect to the choice of valve prosthesis and the attitude to anticoagulant therapy. These observations suggest that in the presence of valvular heart disease at a surgical stage associated to an angiodysplasia, it is preferable to propose valve surgery to start with. Gastro-intestinal surgery is only indicated if haemorrhage persists after a period of observation.
Patients with aortic valve stenosis (AS) and left ventricular (LV) dysfunction may dramatically improve after aortic valve replacement, but operative risk is high. In an earlier study, all patients with low preoperative wall stress and low ejection fraction, or with low aortic valve gradient, died or had persistent heart failure after operation. Because wall stress is difficult to calculate, we reassessed its effect and the effect of other preoperative characteristics on outcome in 66 consecutive catheterization patients with predominant aortic stenosis referred for valve replacement. Despite ejection fraction that was inordinately low compared with afterloading wall stress in nine patients, seven patients improved with surgery. All three patients with ejection fraction less than 20% improved after surgery. Two of three patients with mean aortic valve gradients of less than 30 mm Hg improved. Mortality was 33% in patients with mean gradient less than 30 mm Hg and 19% with mean gradient less than 50 mm Hg. In the 54 patients with calculated aortic valve areas of less than or equal to 0.8 cm2, 1 (2%) had continuing heart failure, while 6 of 12 (50%, P less than .01) patients with aortic valve areas of 0.9-1.2 cm2 had continued symptoms of or died of heart failure. Patients who died or failed to improve after operation were older (71 +/- 9 years) than those who improved (65 +/- 9 years, P = .02). We conclude that wall stress calculations do not predict which patients with aortic stenosis will benefit from aortic valve replacement and that poor left ventricular function and low mean aortic valve gradient do not absolutely preclude operation. On the other hand, low gradient, non-critical valve area, and advanced age are all relative contraindications to aortic valve replacement in aortic stenosis.
Calcific aortic valve stenosis (AS), the main heart valve disease in the elderly, is characterized by extensive remodeling of the extracellular matrix. Matrix metalloproteinases (MMPs) are upregulated in calcific AS and might modulate matrix remodeling. The regulatory mechanisms are unclear. As recent studies have suggested that calcific AS might result from an inflammatory process involving leukocyte invasion and activation, the present study aimed to elucidate the role of the pro-inflammatory cytokine interleukin (IL)-1 beta on MMP expression and cell proliferation in human aortic valves. Immunohistochemistry for leukocytes, IL-1 beta and MMP-1 was performed on aortic valves with (n=6) and without (n=6) calcification obtained at valve replacement or autopsy. Stenotic valves showed marked leukocyte infiltration and associated expression of IL-1 beta and MMP-1. In control valves only scattered leukocytes, low staining for MMP-1 and no staining for IL-1 beta were present. Double-label immunostaining localized IL-1 beta expression mainly to leukocytes and MMP-1 expression to myofibroblasts. Stimulation of cultured human aortic valve myofibroblasts with IL-1 beta lead to a time-dependently increased expression of MMP-1 and MMP-2 by Western blotting and zymography, whereas MMP-9 remained unchanged. Cell proliferation was increased by IL-1 beta as determined by bromodesoxyuridine incorporation. Thus, IL-1 beta may regulate remodeling of the extracellular matrix in calcific AS.
Aortic valve stenosis (AS) severity can be estimated by various modalities. Due to some of the limitations of the currently available methods, the usefulness of live three-dimensional transthoracic echocardiography (3D TTE) in the assessment of AS was explored. Live 3D TTE was able to visualize the aortic valve orifice in all 11 patients studied. Live 3D TTE correctly estimated the severity of AS in all 10 patients in whom AS severity could be evaluated at surgery. These included eight patients with severe AS and two with moderate AS. Two of these 10 patients with AS had associated hypertrophic cardiomyopathy and underwent myectomy at the time of aortic valve replacement. Aortic valve orifice area measurements by live 3D TTE correlated well with intraoperative three-dimensional transesophageal echocardiographic reconstruction measurements (r=0.85) but not as well with two-dimensional transesophageal echocardiography measurements (r=0.64). Live 3D TTE measurements of the aortic valve orifice area also did not correlate well with two-dimensional transthoracic echocardiography measurements (r=0.46) but the number of patients studied with two-dimensional transthoracic echocardiography was smaller (only seven) and four of these did not undergo two-dimensional transthoracic echocardiography at the authors' institution. Altogether, four patients with severe AS by live 3D TTE, and subsequently confirmed at surgery, were misdiagnosed as having moderate AS by two-dimensional transthoracic echocardiography. Because it is completely noninvasive and views the aortic valve in three dimensions, 3D TTE could be a useful complement to the existing modalities in the evaluation of AS severity.
The aortic valve orifice area was measured in 95 patients with valvular aortic stenosis by means of transthoracic and transesophageal echocardiography. These results were compared to invasively determined measurements. The aortic-valve orifice area could be measured by transesophageal echocardiography in 87 patients (92%), and in 13 patients (14%) by the transthoracic approach. A comparison of the valve-orifice area determined by transthoracic and transesophageal echocardiography revealed a correlation coefficient of r = 0.91. There was also a good agreement when the aortic-valve orifice area determined by transesophageal echocardiography was compared to the invasive findings (r = 0.82; p less than 0.001). The morphology of the aortic valve could be better delineated with the transesophageal approach.
Dyspnea, angor and syncope are the most characteristic symptoms in stenosis aortic valve disease. Sudden death, as part of natural history of symptomatic stenosis aortic valve, is well know. On the other hand, sudden death in asymptomatic stenosis aortic valve is rarer. Different guidelines recommend a conservative management of these patients. We present here the case of a 58 year old woman, previously healthy, who arrived at Hospital because of sudden dyspnea at rest. The patient was diagnosed of pulmonary edema and died two hours later. Necropsy showed a stenosis aortica valve with a valve area of less than 0.8 cm2. We make a short review in medical literature about the incidence of sudden death in asymptomatic stenosis aortic valve, the risk groups and their management.
OBJECTIVE: To determine the prognostic value of exercise testing, valve area, and maximum transaortic pressure gradient in asymptomatic patients with aortic valve stenosis. SETTING: The outpatient service of a tertiary referral centre for cardiology. DESIGN: Prospective clinical study. PATIENTS: 66 consecutive patients with isolated severe aortic stenosis (aortic valve area </= 1.0 cm(2)) were selected over a 58 month period. Mean (SD) follow up was 14.77 (11.93) months. INTERVENTIONS: At the initial visit Doppler echocardiography and exercise testing were performed to evaluate ST segment depression and the development of symptoms of aortic stenosis, ventricular arrhythmia, or inadequate rise of systolic blood pressure during exercise. Follow up clinical examinations were performed every three months thereafter to record the onset of symptoms. MAIN OUTCOME MEASURES: Sudden death or the development of symptoms. RESULTS: Eight patients developed dizziness during exercise testing but made a rapid and spontaneous recovery. No other complications of exercise testing occurred. Survival curves, with or without the occurrence of end point events for the variables studied, showed significant differences for positive versus negative exercise testing (p = 0.0001) and aortic valve area < 0.7 cm(2) v >/= 0.7 cm(2) (p = 0.0021). There was no relation between the end points and transaortic gradient (p = 0.6882). In multivariate analysis, a hazard ratio of 7.43 was calculated for patients with a positive versus a negative exercise stress test. Although asymptomatic in daily life, 6% of the patients (4/66) experienced sudden death; all these had a positive exercise test and an aortic valve area of </= 0.6 cm(2). CONCLUSIONS: Exercise testing is safe and is of prognostic value in asymptomatic patients with aortic stenosis.
The authors studied the responses of the main systems of sympathetic and hormonal regulation in valvular aortic stenosis, a special model of dissociation between arterial pressure and left ventricular function. The series comprised 14 patients with an average age of 70 +/- 9 years without diuretic therapy presenting with pure calcific aortic stenosis without other valvular or coronary disease. All were in sinus rhythm; 5 were taking an angiotensin converting enzyme inhibitor. Plasma concentrations of endothelin 1, atrial natriuretic factor (ANF), arginine vasopressin (AVP), catecholamines, plasma renin activity (PRA), angiotensin II and aldosterone were measured in resting, fasting patients, by blood samplings from a peripheral vein immediately before cardiac catheterisation. The results were compared with the severity of the aortic stenosis (aortic valve area greater or less than 0.7 cm2), the ratio of left ventricular work/myocardial mass (greater or less than 0.6) and treatment (with or without ACE inhibitors). Catecholamine levels were much higher in severe aortic stenosis (noradrenaline: 579 +/- 66 pg/ml when valve surface area > 0.7 cm2 versus 900 +/- 92 pg/ml when valve surface area < 0.7 cm2; p < 0.01). Endothelin -1 and AVP concentrations were normal. Whereas PRA was normal, aldosterone levels were increased in patients without treatment by ACE inhibitors. This treatment did not, however, normalise the noradrenaline levels. The increase in ANF concentration was large when left ventricular work decreased with respect to myocardial mass (190.8 +/- 42.3 pg/ml if W/M was decreased versus 82.7 +/- 15.4 pg/ml when W/M was normal): this could be related to the degree of left ventricular hypertrophy.
A 58-year-old woman was admitted to our hospital suffering from repeated chest pain. Selective coronary angiography (CAG) showed severely stenotic lesion in the left main coronary artery. There was no additional lesion in the left anterior descending and circumflex arteries. Aortography showed severe calcification in the aortic valve. The aortic was incised and divided completely at the aortic root 1 cm distal to the aortic valve. The incision was then extended toward the opening of the left coronary artery. An atheromatous plague was found and resected at the ostium. A saphenous vein patch was used to enlarge the internal diameter of the artery. The aortic valve was replaced using #21 SJM prosthetic valve. The patient recovered the surgery well, and anginal attacks have disappeared. Postoperative coronary angiograms revealed no stenotic lesion in the left main coronary artery.
Although aortic and pulmonary valve stenosis are among the most common congenital heart defects, the combination of both aortic and pulmonary valve stenosis in the same patient appears to be very uncommon. Accurate diagnosis of combined valvular stenosis is imperative prior to surgical correction, otherwise surgery of one of the lesions may result in an insufficient hemodynamic improvement. A seven-year-old girl with congenital aortic and pulmonary valve stenosis associated with hypoplastic aortic annulus underwent Konno's operation, pulmonary valvotomy and resection of anomalous muscle of the right ventricular outflow tract simultaneously. The operation was successfully performed and postoperative course was uneventful.
A patient was a 65-year-old female who had a complaint of palpitation was diagnosed aortic valve stenosis due to congenital bicuspid aortic valve with pseudotendon by the echocardiographic examination. We suspected left single coronary artery by the aortography and the coronary artery angiography. Aortic valve replacement and resection of pseudotendon was performed with Carbomedics supra-annular aortic valve (21 A). During surgery, persistent left superior vena cava was detected. High-posterior take-off right coronary artery was casually detected at aortic closure. Ventricular fibrillation due to insufficient supply of cardioplegic solution at right coronary area frequently occurred after cardio-pulmonary bypass and percutaneous cardiopulmonary support was required. The patient was discharged 32 days after the operation. Preoperative and intraoperative evaluation was important in the case of aortic valvular disease.
To investigate the relation of left ventricular (LV) afterload, hypertrophy, geometry and systolic pump function, 17 normal persons, 24 patients with aortic stenosis (AS), 20 with aortic regurgitation (AR) and 15 with idiopathic dilated cardiomyopathy (DC) were studied. Two-dimensional echograms were used to assess end-systolic meridional and circumferential stresses and their ratio, LV mass, relative wall thickness (h/R ratio) and the ratio of LV minor axis to length, used as an index of shape. Independently obtained ejection fraction (EF) was used to determine which patients had normal (EF greater than or equal to 55%) and which had depressed (EF less than 55%) pump function. Patients with AS and low EF had similar LV mass (228 vs 215 g) but larger LV cavity (5.6 vs 4.5 cm), lower h/R ratio (0.53 vs 0.73, p less than 0.01), and therefore higher circumferential stress (336 vs 268 kdyne/cm2, p less than 0.05). Compared with normal persons, patients with DC had a lower h/R ratio (0.28 vs 0.38, p less than 0.01), higher circumferential stress (362 vs 215 kdyne/cm2, p less than 0.01) and more uniform stress distribution (meridional to circumferential stress ratio 0.57 vs 0.39, p less than 0.01), implying that meridional stress overestimates effective afterload. Afterload excess and LV shape change may be important to pump function in patients with AS or DC. In contrast, in those with AR, no significant shape differences were noted, although LV mass was higher in those with low EF (279 vs 211 g, p less than 0.05). Depressed pump function may result from impaired myocardial performance in AR without afterload excess.
Calcified stenotic aortic valve are usually replaced by the artificial valve. Recently, we are trying to decalcify the calcification of the aortic valve by ultrasonic energy. This case report reviewed a small physique, 47 year old female, who had the calcified stenotic bicuspid aortic valve and the narrow aortic valve ring (18 mm diameter). The ultrasonic decalcification was performed by the ultrasonic surgical instrument, SUS-201D (ALOKA), ultrasonic energy output range from 25% to 45%. After the ultrasonic decalcification, the movability of the aortic cusps were improved and the aortic opening was enlarged. The systolic pressure gradient across the aortic valve was improved from 100 mmHg to 20 mmHg. This method "ultrasonic decalcification" is very useful technique for the repair of the calcified aortic valve stenosis.