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[Duroziez disease].

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Heart Defects, Congenital↗

Reproducibility of Doppler echocardiographic quantification of aortic and mitral valve stenoses: comparison between two echocardiography centers.

Doppler echocardiography has been widely used as a noninvasive method to quantify valvular heart diseases. This study assessed the variability between 2 echocardiography centers concerning 2-dimensional and Doppler echocardiographic results in the quantification of mitral and aortic valve stenoses. Forty-two patients were studied by 2 different echocardiography centers in a blinded, independent fashion. In patients with aortic and mitral valve stenosis, mean and maximal flow velocities were measured. The aortic valve orifice area was calculated according to the continuity equation. Mitral valve orifice area was determined by direct planimetry and by pressure half-time. In patients with an aortic valve stenosis, a close relation between the 2 centers was found for the maximal and mean flow velocities (coefficient of correlation, r = 0.72 to 0.92; coefficient of variation, 3.7 to 7.7%). A close correlation and a small observer variability was found for the flow velocity ratio determined by flow velocities measured in the left ventricular outflow tract and over the stenotic valve (r = 0.88; coefficient of variation, 0.01 +/- 0.009). In contrast, there was a poor correlation between the diameter of the left ventricular outflow tract and the aortic orifice area (r = 0.36 and 0.59, respectively). In patients with a mitral valve stenosis, mean and maximal velocities were closely correlated (r = 0.85 and 0.77, respectively). Velocities were not found to be significantly different between the 2 centers. Variability between the 2 centers for the mitral valve orifice area was 9.8% (2-dimensional echocardiography) and 5.7% (pressure half-time).(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

Ultrasonic-cardiographic long-term observations in patients with operated stenosis of the mitral valve.

135 patients operated for stenosis of the mitral valve were observed and treated 10.3 years, on the average. Three years of this time belong to the preoperative period and 7.3 years to the postoperative one. For evaluating the degree of stenosis, several ultrasound cardiograms were made yearly. After the operation one ultrasound examination was made every year for observing progress. In order to examine the effect of penicillin metaphylaxis on the process of restenosis, the patients were classified into different groups. The best operative result was maintained if continuous postoperative treatment with penicillin was made.

Adult↗

Mitral valve replacement for mitral stenosis secondary to Hunter's syndrome.

Hunter's syndrome is a rare, X-linked recessive, mucopolysaccharidosis. Survival into adulthood is uncommon. Mitral valve disease, predominantly regurgitation, has been reported in these patients. We have found no reports of mitral valve replacement for mitral stenosis secondary to Hunter's syndrome in the English literature. We report that mitral valve replacement for this pathology is a viable treatment option in an adult patient; however, specific precautions must be considered.

Adult↗

Beta-adrenergic receptor blockade in the management of pregnant women with mitral stenosis.

Twenty-five pregnant women with symptomatic mitral valve stenosis (mean valve area, 1.1 +/- 0.25 cm2) were managed by initiation or modification of beta-adrenergic receptor blockade with the use of either propranolol or atenolol. Significant improvement of symptoms occurred in 23 patients (92%) (p less than 0.01); the mean maternal heart rate was reduced significantly from 86 +/- 4 to 78 +/- 5 beats/min (p less than 0.0001). The overall fetal heart rate ranged between 130 to 150 beats/min during treatment. Only two patients required urgent closed mitral valvotomy, after pulmonary edema developed as a result of poor compliance to beta-blockade. All patients were safely delivered of infants at term. Fetal heart rates ranged between 120 to 140 beats/min at delivery. There was no maternal or fetal death. Pregnant woman with symptomatic mitral valve stenosis can be safely managed with beta-blockade, giving significant reduction in the incidence of pulmonary edema with no unwanted neonatal side effect.

Adrenergic beta-Antagonists↗

Correlation between the position of transducers and mitral valve gradient in mitral stenosis.

To assess the severity of mitral stenosis related to the level of transducers, the mitral valve gradient using pulmonary wedge and left ventricle was obtained from 15 patients. The mitral gradient was obtained with both transducers at mid chest level. Then, the level of each transducer was realigned with the catheter tip in the pulmonary capillary wedge and in the left ventricle using lateral fluoroscopy and the mitral gradient was re-measured. At the mid chest level, the mean mitral valve gradient was 14 +/- 6.2 mm Hg with a mitral valve area of 1.3 +/- 0.6 cm2. With the adjusted level of transducers, the mitral valve gradient was 18.7 +/- 6.8 mm Hg with a valve area of 1.0 +/- 0.5 cm2. The difference was that the level of catheter tip in the wedge was 3.5 cm below the mid chest level and the one in the left ventricle was 2.5 cm higher than the mid chest level. This result suggested that the mitral valve gradient obtained at mid chest level underestimated the severity of mitral stenosis.

Cardiac Catheterization↗

Mitral valve replacement in a patient with an extensively calcified mitral anulus: report of a case.

We report herein the case of a patient with chronic renal failure in whom mitral valve stenosis with extensive mitral anular calcification involving the entire anulus and leaflets was successfully treated surgically. Excision of both leaflets and partial resection of the anular calcification enabled the insertion of a 23-mm St. Jude Medical prosthetic valve. The technical difficulties involved with inserting the appropriate-sized prosthetic valve in a narrowed mitral anulus with heavy calcification are discussed following this case report.

Calcinosis↗

Plasma NT-proBNP is a potential marker of disease severity and correlates with symptoms in patients with chronic rheumatic valve disease.

BACKGROUND: A noninvasive marker of disease severity and presence of symptoms is required in patients with chronic rheumatic valve disease (RVD). AIMS: We sought to test the utility of measuring of N-terminal pro-B type natriuretic peptide (NT-proBNP) in chronic phase RVD. We also evaluated whether echocardiographic measures are interrelated with NT-proBNP levels. METHODS: The study comprised 92 patients with RVD (mean age of 40+/-14 years) and 50 age/gender-matched control subjects. Functional status was assessed. Detailed echocardiographic examination was performed and mitral valve score was estimated. Venous blood samples were taken for measuring the level of NT-proBNP. RESULTS: The plasma levels of NT-proBNP rose with increasing severity of mitral valve stenosis (p<0.001), increasing severity of mitral valve score (p<0.001), increasing severity of clinical symptom (p<0.001), increasing severity of mitral regurgitation (p<0.013), presence of mitral valve calcification (p<0.001), presence of tricuspid valve stenosis (p<0.001), increasing severity of tricuspid regurgitation (p<0.011), presence of aortic stenosis (p=0.043), decreasing left ventricular ejection fraction (p<0.001), presence of left atrial thrombus (p=0.0019), and with increasing left atrium dimensions (p=0.002). CONCLUSION: NT-proBNP levels in patients with chronic RVD are a potential marker of disease severity and correlates with symptoms.

Adolescent↗

Valve replacement for children: report of two cases.

The authors report on two children who underwent valve replacement. Case 1: A 10-year-old boy with high fever and severe heart failure was diagnosed as having aortic regurgitation and left ventriculo-right atrial fistula caused by active infective endocarditis. An aortic annular defect was repaired by a pericardial patch to enlarge the aortic annulus followed by aortic valve replacement with a 17-mm Björk-Shiley prosthetic valve. He is doing well 2 years after surgery. Case 2: A 3-year-old girl was diagnosed as having congenital mitral stenosis with severe pulmonary hypertension. Mitral valve stenosis was a commissural fusion type according to the Carpentier's classification. An open mitral commissurotomy was attempted initially, resulting in severe mitral regurgitation. Then, the mitral valve was replaced with a 16-mm CarboMedicus bileaflet valve. Her postoperative course was uneventful and residual pulmonary hypertension decreased gradually. The selection of prosthetic valves is important in its type and size in order to obtain as large an orifice as possible. Careful postoperative follow-up is mandatory.

Adult↗

[Incidence and concomitant factors of tricuspid valve insufficiency in patients with aortic and mitral valve diseases].

Invasive data about the frequency and associated factors of tricuspid regurgitation in normals and in patients with aortic and mitral valve disease are still rare. Thus, right ventricular biplane angiograms (RAO/LAO projection), the mean pulmonary artery pressure and the presence of atrial fibrillation were analyzed with regard to tricuspid regurgitation in 30 normals and 165 patients with pure mitral regurgitation, mitral stenosis, aortic regurgitation, aortic stenosis, combined mitral valve disease or combined aortic valve disease. Patients with tricuspid stenosis or coronary artery disease were excluded. In 52 of the 195 patients tricuspid regurgitation was present. Tricuspid regurgitation occurred statistically more often in patients with mitral stenosis (33%), mitral regurgitation (48%) or combined mitral valve disease (68%) than in patients with aortic regurgitation (4%) or combined aortic valve disease (3%). In patients with aortic stenosis and in normals tricuspid regurgitation was not present. In patients with combined mitral valve disease, tricuspid regurgitation was more often present than in patients with pure mitral stenosis (p less than 0.002), despite comparable values of the mean pulmonary artery pressure, the right ventricular enddiastolic and endsystolic volume indexes, the right ventricular ejection fraction and the frequency of atrial fibrillation. Only in patients with pure mitral regurgitation tricuspid regurgitation was associated with an elevated mean pulmonary artery pressure (p less than 0.02). Differences in the right ventricular size and function did not occur between normals and patients with mitral or aortic valve disease. Therefore, the mean pulmonary artery pressure, atrial fibrillation and the size and function of the right ventricle are not major determinants for the occurrence of tricuspid regurgitation.(ABSTRACT TRUNCATED AT 250 WORDS)

Aortic Valve↗

Transesophageal color Doppler evaluation of obstructive lesions using the new "Quasar" technology.

Due to the unavoidable problem of aliasing, color flow signals from high blood flow velocities cannot be measured directly by conventional color Doppler. A new technology termed Quantitative Un-Aliased Speed Algorithm Recognition (Quasar) has been developed to overcome this limitation. Employing this technology, we used transesophageal color Doppler echocardiography to investigate whether the velocities detected by the Quasar would correlate with those obtained by continuous-wave Doppler both in vitro and in vivo. In the in vitro study, a 5.0 MHz transesophageal transducer of a Kontron Sigma 44 color Doppler flow system was used. Fourteen different peak velocities calculated and recorded by color Doppler-guided continuous-wave Doppler were randomly selected. In the clinical study, intraoperative transesophageal echocardiography was performed using the same transducer 18 adults (13 aortic valve stenosis, 2 aortic and 2 mitral stenosis, 2 hypertrophic obstructive cardiomyopathy and 1 mitral valve stenosis). Following each continuous-wave Doppler measurement, the Quasar was activated, and a small Quasar marker was placed in the brightest area of the color flow jet to obtain the maximum mean velocity readout. The maximum mean velocities measured by Quasar closely correlated with maximum peak velocities obtained by color flow guided continuous-wave Doppler in both in vitro (0.53 to 1.65 m/s, r = 0.99) and in vivo studies (1.50 to 6.01 m/s, r = 0.97). We conclude that the new Quasar technology can accurately measure high blood flow velocities during transesophageal color Doppler echocardiography. This technique has the potential of obviating the need for continuous-wave Doppler.

Adult↗

Distinct down-regulation of cardiac beta 1- and beta 2-adrenoceptors in different human heart diseases.

Cardiac beta-adrenoceptor density and beta 1- and beta 2-subtype distribution were examined in human left ventricular myocardium from transplant donors serving as controls and from patients with mitral valve stenosis, aortic valve stenosis, idiopathic dilated cardiomyopathy, and ischaemic cardiomyopathy respectively. The total beta-adrenoceptor density was similar in transplant donors and patients with moderate heart failure (NYHA II-III) due to mitral valve stenosis, but was markedly reduced in all forms of severe heart failure (NYHA III-IV) studied. A reduction of both beta 1- and beta 2-adrenoceptors was found in patients with severe heart failure due to mitral valve stenosis or ischaemic cardiomyopathy. In contrast, a selective down-regulation of beta 1-adrenoceptors with unchanged beta 2-adrenoceptors and hence a relative increase in the latter was observed in idiopathic dilated cardiomyopathy and aortic valve stenosis. It is concluded that the extent of total beta-adrenoceptor down-regulation is related to the degree of heart failure. Selective loss of beta 1-adrenoceptors is not specific for idiopathic dilated cardiomyopathy but also occurs in aortic valve stenosis. Changes in beta 1- and beta 2-subtype distribution are rather related to the aetiology than to the clinical degree of heart failure.

Adult↗

[Parachute mitral valve coexisting with complex congenital heart defect. Successful multistage surgical treatment].

Type parachute valve is a very rare bicuspid valve congenital anomaly. When signs of dysfunction are noted the valve has to be replaced with an artificial one. The case describes a 7-year old girl suffering from complex congenital heart defect: coarctation of the aorta followed by hypoplastic aortic arch, ventricular septal defect and parachute mitral valve. In the neonatal period the girl had been operated on by the subclavian floppy aortoplasty method due to coarctation of the aorta. At the age of 2, another surgical correction was performed: ventricular sept defect closure simultaneously with mitral valvuloplasty. In infancy she failed to thrive, also signs of heart failure had been observed which resulted from increasing mitral valve stenosis and III grade mitral insufficiency. These findings in with artificial valve replacement (type Sorin O 23 mm). Procedure has been performed by the left atrium. Post operational period was uneventful. Proper function of the implanted valve has been observed with no perivalvular leakage and left atrium diameter has been normalized.

Child↗

Surgical treatment for Scheie's syndrome (mucopolysaccharidosis type I-S): report of two cases.

Scheie's syndrome (mucopolysaccharidosis type I-S) is a rare genetic lysosomal storage disease affecting mucopolysaccharide metabolism, and is known to include cardiovascular disease. Surgical treatment was carried out in 2 patients with Scheie's syndrome. Patient 1 was a 56-year-old man with triple-vessel coronary artery disease, who successfully underwent coronary artery bypass grafting. Patient 2 was a 52-year-old man with aortic and mitral valve stenosis, who successfully underwent combined aortic and mitral valve replacement. The literature on Scheie's syndrome associated with valvular and coronary artery disease is also reviewed.

Aortic Valve Stenosis↗

[Non-invasive estimation of transmitral pressure gradient and mitral valve area in mitral stenosis by an ultrasonic pulsed Doppler technique].

We attempted to estimate transmitral pressure gradient and mitral valve area (MVA) noninvasively in mitral stenosis (MS) by a bi-directional pulsed Doppler flowmeter combined with an electronic two-dimensional echocardiograph. Eleven patients with MS in sinus rhythm were studied by cardiac catheterization. Fifteen healthy subjects (H) served as normal control. The pulsed Doppler flowmeter operated with a carrier frequency of 2.5 MHz, a pulse repetition rate of either 5 KHz or 10 KHz and a sample volume of 1 X 3 X 3 mm. The velocity of transmitral central flow was measured by this system, monitoring audible Doppler sounds and cardiac images which depict the anatomic location of the sampling site. The Doppler signal was analyzed by a sound spectrograph. In estimating the transmitral pressure gradient and MVA, we employed a Doppler parameter (half time) defined as the time for instantaneous maximal blood flow velocity to reduce to one-half from its rapid inflow peak, which is independent of the angle between the ultrasonic beam and blood flow. Transmitral pressure gradient (delta P100) was measured as the pressure gradient between either left atrial or pulmonary capillary pressure and left ventricular pressure at the point after 100 msec from the nadir of left ventricular early diastolic pressure [( LA or PC--LVDP]100). MVA was obtained using a Gorlin's formula. The transmitral blood flow velocity in both MS and healthy groups revealed a narrow frequency band pattern with two peaks, R and A, in diastole. The former peak occurred during rapid inflow phase and the latter following atrial contraction. In the healthy group, the descent rate of R wave was increased than that in the MS group. The square root of the pressure gradient also reduced linearly with transmitral flow velocity in the MS group. Thus in the MS group, the transmitral velocity was directly proportional to the square root of the pressure gradient as described by a Bernoulli theorem, and the half time was proportional to the transmitral velocity. The square of the half time (delta t2) was highly correlated with delta P100 (r = 0.97), and the inverse of the half time (delta t-1) was correlated with MVA (r = 0.76). There was no significant correlation between delta P100 and diastolic descent rate of anterior mitral leaflet (DDR). The present study indicates that the half time is useful in estimating transmitral pressure gradient and MVA in mitral stenosis.

Adult↗