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PubMed1999

Combined aortic and mitral stenosis in mucopolysaccharidosis type I-S (Ullrich-Scheie syndrome).

The genetic mucopolysaccharidosis syndromes (MPS) are autosomal recessive inborn errors of metabolism. Heart valve involvement in MPS is not uncommon but only a few case reports of successful cardiac surgery are available. In particular, reports of combined aortic and mitral stenosis associated with MPS type I-S are very rare. Both type I and type VI MPS are associated with significant left sided valvar heart disease that requires surgical valve replacement because of irregular valve thickening, fibrosis, and calcification. A 35 year old man had severe mitral valve stenosis after successful surgical replacement of a stenotic aortic valve. Valvar heart disease was investigated by cardiac ultrasound and left heart catheterisation. Histomorphological characterisation of the affected mitral valve was performed. The case illustrates typically associated clinical features of cardiac and extracardiac abnormalities found in MPS type I-S.

Adult↗
PubMed2005

Comparison of accuracy of mitral valve area in mitral stenosis by real-time, three-dimensional echocardiography versus two-dimensional echocardiography versus Doppler pressure half-time.

Mitral valve area (MVA) in 30 patients with mitral stenosis (MS) and 34 normal controls was calculated by real-time, 3-dimensional echocardiography (RT3DE); MVA in patients with MS correlated well with the mitral area determined by 2-dimensional echocardiography (r = 0.98) and by pressure half-time (r = 0.90). MVA in normal controls on RT3DE correlated well with MVA on 2-dimensional echocardiography (r = 0.94) and pressure half-time (r = 0.91). There were significant differences between the orifice areas in patients with MS and normal controls. RT3DE can provide not only the anatomic structure of mitral valve apparatus, but also the optimal plane of the smallest mitral valve orifice, and can thus accurately measure the MVA.

Adult↗
PubMed1989

Aneurysm of atrial septum associated with rheumatic mitral stenosis simulating tumour of right atrium.

We present a case of aneurysm of interatrial septum associated with rheumatic mitral stenosis, which supports the theory that claims that the pressure gradient between the atriums plays an important part in its aetiology. The angiographic image of a filling defect in the right atrium may cause an erroneous diagnosis of atrial tumour. The levophase of right angiography and cross-sectional echocardiography confirmed the diagnosis of aneurysm of interatrial septum.

Angiography↗
PubMed2000

Effect of Inoue balloon mitral valvotomy on severe pulmonary arterial hypertension in 315 patients with rheumatic mitral stenosis: immediate and long-term results.

BACKGROUND AND AIMS OF THE STUDY: Despite advances in surgical techniques, mitral valve surgery in patients with severe pulmonary arterial hypertension (PAH) causes considerable mortality and morbidity. Balloon mitral valvotomy (BMV) is an established alternative to treat high-risk surgical patients with mitral stenosis (MS). The study aims were to evaluate immediate and long-term efficacy of BMV in patients with MS and severe PAH, compared to those with mild/moderate PAH. METHODS: Among 1,125 patients who underwent Inoue BMV, 315 had severe PAH (mean pulmonary artery (PA) pressure > or = 50 mmHg (group I; 79 of these patients had suprasystemic PAH). Results from this group were compared with those of patients with mild/moderate PAH (group II). RESULTS: Group I patients were younger and more symptomatic (mean PA pressure 62 +/- 10.6 mmHg versus 32.6 +/- 8.2 mmHg in group II). Before BMV, mean transmitral gradient (17.8 +/- 6.5 versus 14.4 +/- 5.4 mmHg) and pulmonary capillary wedge pressure (PCWP) (31.6 +/- 6.1 versus 22.8 +/- 6.2 mmHg) were significantly higher, while mitral valve area (MVA) (0.66 +/- 0.2 versus 0.85 +/- 0.2 cm2) was significantly lower in group I. After BMV, PA mean pressure was significantly reduced (34.8 +/- 11.2 and 21.1 +/- 8.4 mmHg), transmitral gradient (8.0 +/- 3.9 and 6.9 +/- 3.2 mmHg) and mean PCWP (12.8 +/- 5.8 and 11.0 +/- 5.1 mmHg) in groups I and II, respectively, with a comparable increase in MVA (1.77 +/- 0.4 and 1.84 +/- 0.5 cm2). Group I patients had worse baseline hemodynamic parameters than group II, but the former had a higher absolute gain in hemodynamic parameters. Residual severe PAH after BMV was seen in 9.8% of patients, with PA pressures normalized in 9.5%. Among 79 patients with suprasystemic PA pressure (mean PA systolic pressure 116.6 +/- 28.2 mmHg), 16.5% normalized their PA pressures and 25.3% had residual severe PAH. At mean follow up of 33 months, 80.4% were in NYHA class I. Mean PA systolic pressure in 161 patients was 39.0 +/- 14.2 mmHg compared with a post-BMV value of 55.0 +/- 16.9 mmHg; thus, a sustained fall in pressure was demonstrated at follow up. CONCLUSION: Inoue BMV is safe and effective in patients with MS and severe PAH. Although these patients have worse clinical and hemodynamic parameters before BMV, they achieve a greater absolute gain in terms of improvement in all hemodynamic parameters.

Adult↗
PubMed1985

[Diagnosis of subvalvular adhesions in mitral stenosis by 2--dimensional echocardiography].

Echocardiographic signs of subvalvular fusions are discussed, as they frequently complicate the course of mitral stenosis. The diagnostic value of two-dimensional echo-cardiography is established for this abnormality. Subvalvular fusions of the chordal apparatus are shown to be associated with certain signs on the two-dimensional echocardiogram, such as additional echoes under mitral valve cusps in short-axis heart section (sensitivity 67.2%, specificity 90.7%), and mitral orifice drawn towards the interventricular septum (sensitivity 35.7%, specificity 93.5%), thickened subvalvular structures in the long-axis section of the heart's left compartments (sensitivity 40.7%, specificity 82.9%), and fissural or "coma-like" shape of the mitral orifice.

Adult↗
PubMed1993

[Catheter balloon valvuloplasty in mitral stenosis (choice of surgical methods and evaluation of its results)].

Clinico-experimental study was undertaken for the choice of the optimal method for radiosurgical correction of the disease. Catheter balloon valvuloplasty (CBV), suggested by V. A. Silin and V. K. Sukhov with the use of balloon catheters of their design was found to be the most effective and safe method. This method was used to operate on 265 patients with mitral stenosis and various degree of affection of the valvular apparatus and complications in the form of calcinosis of the valve and cardiac fibrillation. Thirteen of the patients were operated on in pregnancy. CBV produced a positive therapeutic effect in more than 95% of patients. Increase of the mitral orifice area by 2.2-3.4 times was attended by a corresponding reduction of the diastolic pressure gradient at the mitral valve and systolic pressure in the pulmonary artery. The increase in the area of the mitral orifice and the positive changes of the hemodynamics values were more marked in patients with severe stenosis. The small number of complications (up to 9.5%) and the low lethality (up to 0.86%) allow the CBV method to be recommended as one of the main methods of surgical correction of mitral stenosis.

Adult↗
PubMed1991

Percutaneous balloon mitral valvuloplasty in a pregnant woman with mitral stenosis, sickle cell crisis and acute pulmonary edema. A case report.

A woman with acute congestive heart failure secondary to mitral stenosis and sickle cell crisis was treated successfully with a combination of an exchange transfusion and percutaneous balloon valvuloplasty. That combination provided an alternative to surgical mitral commissurotomy, with its significant risks for both the mother and fetus. The patient was able to undergo an uncomplicated pregnancy course despite the increased risk of cardiac decompensation in the intrapartum and postpartum period.

Adult↗
PubMed1987

Effects of valvular surgery on overall and regional lung function in patients with mitral stenosis.

Changes in overall and regional pulmonary function after open heart surgery were studied in 23 patients with mitral stenosis, grouped according to hemodynamic abnormality at cardiac catheterization. In group 1 (pulmonary arterial pressure less than 25 mm Hg), no pulmonary function disorders were detected before or after surgery. In group 2 (pulmonary hypertension with no or slight tricuspid regurgitation) lung volumes increased significantly within normal ranges after surgery. However, group 3 patients (pulmonary hypertension and moderate or severe tricuspid regurgitation, restrictive and obstructive impairment, uneven alveolar ventilation, disturbed diffusing capacity) did not recover after surgery. The regional perfusion distribution by 133Xe, expressed as the upper over lower lung field ratio, showed significant changes after surgery in groups 1 and 2, but no change in group 3. In groups 1 and 2 it seems that lung parenchyma and pulmonary vessels were not greatly damaged, while in group 3 organic changes in the lung occurred.

Adult↗
PubMed1998

Left atrial energy in normal subjects, in patients with symptomatic mitral stenosis, and in patients with advanced heart failure.

In a population consisting of 45 subjects (15 patients with mitral stenosis, 15 patients with heart failure, and 15 normal subjects), the left atrial (LA) pressure-area relation was assessed and the correlations between LA stroke work, LA kinetic energy, and LA ejection force were studied. The area of the counterclockwise A loop expressing the LA external work was measured. LA kinetic energy was obtained from the formula: 1/2 x stroke volume x 1.06 x v2, where v = transmitral Doppler A velocity. An excellent correlation was found between LA stroke work index and LA kinetic energy when data from all patients were plotted together (r = 0.95, p <0.001). The Pearson's correlation similarity coefficient between LA ejection force and kinetic energy as well as between LA ejection force and stroke work index was r = 0.54 (p <0.001). The r value of 0.54 was significantly lower than r = 0.95 (p <0.03). LA kinetic energy extends the use of transmitral flow Doppler data to include assessment of LA work. LA work can be obtained noninvasively with a high degree of accuracy.

Atrial Function↗
PubMed1976

Relationship of the P loop measurements to pulmonary artery wedge pressure and left atrial volume in pure mitral stenosis.

Frank vectorcardiographic P loops with satisfactory signal to noise ratio were obtained from 28 patients with pure mitral stenosis by using computer-averaging technique of eight successive beats. Twenty-three parameters of the P loop automatically measured were compared with the mean pulmonary artery wedge pressure (mPAW) and the maximal left atrial volume (max. LAV) calculated from biplane angiocardiogram by the area-length method. There was a significant correlation between the m PAW and four P loop measurements. The positive amplitude in lead Z (the posterior component) showed the highest correlation with the mPAW (r=0.54, p less than 0.01). In the multiple regression analysis, the predicted m PAW using eight P loop measurements and the measured m PAW showed a high significant correlation (r=0.86, p less than 0.01). There were significant correlations between the max. LAV and fourteen P loop measurements. The magnitude of spatial maximal P-vector showed the highest correlation with the max. LAV (r=0.86, P less than 0.01). In the multiple regression analysis, the predicted max. LAV using four P loop measurements out of 23 showed the best correlation with the measured max. LAV (r=0.94, P less than 0.01).

Adolescent↗
PubMed1992

Reduced left ventricular compliance in human mitral stenosis. Role of reversible internal constraint.

BACKGROUND: The mechanisms of depressed left ventricular (LV) pump performance in human mitral stenosis (MS) remain poorly understood, because reduced filling alone affects many hemodynamic measurements. Therefore, pressure-volume relations were examined in nine subjects with MS and compared with eight age-matched normal controls. METHODS AND RESULTS: Data were obtained by conductance catheter/micromanometer technique with transient inferior vena cava occlusion used to alter load and generate pressure-volume relations. In a subset of patients (n = 5), data were obtained both acutely and at 3 months (n = 4) after balloon valvuloplasty. MS patients had reduced cardiac output (3.3 +/- 0.9 versus 5.6 +/- 1.7 l/min) and end-diastolic volume (68.0 +/- 6.9 versus 115 +/- 31 ml) versus controls (p less than 0.001), with a mean transvalvular gradient of 14 +/- 6 mm Hg and estimated valve area of 0.6 +/- 0.2 cm2. Systolic function as assessed by the end-systolic pressure-volume relation was virtually the same in MS and control subjects. In contrast, end-diastolic pressure-volume relations in MS were consistently shifted leftward and had an increased slope (lower compliance) at matched pressure ranges (6.5 +/- 3.0 versus 2.2 +/- 0.53 ml/mm Hg at a mean diastolic pressure of 8 mm Hg, p less than 0.001). This change was not a result of reduced LV filling or probably of increased right heart loading. Valvuloplasty acutely returned chamber compliance to near normal, a change that was sustained at 3-month follow-up. Systolic function was little altered at this time. CONCLUSIONS: These data indicate an impairment of diastolic function in human MS that can be acutely reversed by balloon valvuloplasty. Lowered LV compliance probably results from a functional restriction caused by ventricular attachment to a thickened and immobile valve apparatus.

Cardiac Catheterization↗
PubMed2004

Effect of age on atrial contribution to ventricular filling after balloon mitral valvuloplasty in mitral stenosis.

OBJECTIVE: Ventricular filling takes place during the conduit and pump functions of the atrium. While studying whether relief of mitral valve obstruction improves atrial filling, the effect of age on atrial contribution to ventricular filling was studied before and after balloon mitral valvuloplasty (BMV) and on follow-up at 1 year. METHODS: Patients with mitral stenosis (MS) and sinus rhythm (n = 59) were divided into group I (< 18 years, n = 13), group II (< 30 years, n = 29) and group III (> 30 years, n = 17). Two-dimensional mitral valve area (MVA in cm2), transmitral mean gradient (MG in mm Hg), velocity time integral (VTI in cm) of mitral valve flow, VTI contributed by atrial systole (A-VTI), difference between total VTI and A-VTI (E-VTI), percentage contribution of A-VTI to the total VTI (A-%) and difference between A-% before and after BMV (delta-A-%) were noted. Follow-up data was obtained at 1 year. The change in A-% at follow-up (A-%-FU) was calculated as the difference between A-% before BMV and A-% at follow-up. RESULTS: There was a similar increase in MVA with a reduction in MG among the three groups. Among the three groups, total VTI and E-VTI before and after BMV were similar. Before BMV, in all the groups, A-VTI and A-% were similar. After BMV, there was increase in A-VTI and A-% in all the groups with a trend to be more in younger patients. A-VTI was significantly higher in group I only. But E-VTI had decreased significantly in all groups and tended to be less in younger patients. In younger patients, delta-A-% after BMV was significantly higher (13.2 +/- 7.6, 7.9 +/- 5.1 and 6.5 +/- 4.5, respectively, in groups I, II and III; p < 0.01). Correlation coefficient of age against delta-A-% was -0.55 (p < 0.01). Correlation coefficients of delta-A-% against post-BMV-MVA and MG were not good. At follow-up of 11.3+/-1.2 months, changes achieved in total VTI, A-VTI, E-VTI and A-% were maintained. Total VTI, A-VTI, E-VTI and A-% were similar at the time of follow-up on comparing the three groups. But younger patients had significantly higher A-%-FU (12.1 +/- 5.8, 9.4 +/- 4.6 and 7.3 +/- 3.1, respectively, in groups I, II and III; p < 0.01). CONCLUSIONS: Prior to BMV, there is an age related reduction in atrial contribution to ventricular filling that improves with relief of MS. Advancing age reduces the immediate and late recovery of atrial contribution after BMV. This may be due to increasing left atrial fibrosis with age that prevents an improvement in atrial pump function. The differential improvement in atrial function in younger patients warrants earlier intervention in MS to achieve better recovery of atrial function.

Adolescent↗
PubMed1998

[Comparison between percutaneous balloon valvuloplasty and open commissurotomy for mitral stenosis].

PURPOSE: To compare immediate and late (12 months) follow-up of clinical and Doppler echocardiographic results between percutaneous mitral balloon valvuloplasty and open commissurotomy in a prospective and randomized trial. METHODS: Eighty eight symptomatic patients with severe mitral stenosis and favorable anatomy were randomized in a prospective trial comparing the two procedures. All patients were submitted to clinical and Doppler echocardiographic evaluation before the procedures and immediate and twelve months thereafter. RESULTS: Mean mitral gradient (mmHg) decreased from 12.2 +/- 5.8 to 5.80 +/- 2.7 (p < 0.001) in commissurotomy group (CG) and from 11.7 +/- 6.1 to 5.0 +/- 2.4 (p < 0.001) in the balloon valvuloplasty group (VG). Mitral valve are (cm2) increased from 0.98 +/- 0.21 to 2.52 +/- 0.46 in CG and from 1.05 +/- 0.25 to 2.18 +/- 0.40 in VG (p < 0.001). In both groups there was a slight decrease in mitral valve area at 12 month follow-up. There was no death in either group. One patient in the VG had moderate mitral regurgitation and underwent surgery. At the 12 month follow-up, all patients in CG and 97.7% of patients in VG were in New York Heart Association functional class I or II. CONCLUSION: Both procedures were safe and showed similar immediate improvement in mitral gradient and functional class. Mitral valve area had a greater increase immediately after commissurotomy, however, there was a significantly greater reduction in the CG after 12 months of follow-up, when compared to balloon valvuloplasty. In both groups, mitral gradient remained reduced and most patients did not change functional class during the follow-up.

Adolescent↗
PubMed1990

[A case of mitral stenosis with free floating ball thrombus].

A free floating thrombus in the left atrium is a rare complication of the mitral valvular disease. A 56-year-old woman with mitral stenosis was admitted for cerebral infarction and abdominal pain. A free floating ball thrombus was detected in the left atrium by echocardiography. We performed the emergent open heart surgery for removal of ball thrombus and mitral commissurotomy successfully. The size of thrombus was 4 x 3 x 2 cm. Postoperative course was uneventful.

Female↗
PubMed2003

Percutaneous balloon mitral valvuloplasty in patients with severe mitral stenosis and low transmitral diastolic pressure gradient.

An elevated left atrial pressure and high diastolic pressure gradient (DPG) across the mitral valve are the major hemodynamic abnormalities in mitral stenosis (MS). However, a subgroup of patients with severe MS is characterized by low initial DPG. The authors reviewed the clinical, echocardiographic and hemodynamic data as well as procedural results in 180 patients who underwent percutaneous balloon mitral valvuloplasty (PBMV). An initial mean DPG > 10 mmHg was found in 144 patients (80%) (group A) and mean DPG < or = 10 mmHg in 36 patients (20%) (group B). Patients in group A had higher left ventricular ejection fraction (LVEF) than in group B (61 +/- 5% versus 42 +/- 6%, respectively) and higher cardiac index (2.8 +/- 0.4 versus 2.0 +/- 0.3 l/min/m(2) ). In group B 12 patients (33%) had normal LVEF, whereas 24/36 (67%) had reduced LVEF. All the latter had wall motion abnormalities on ventriculography. Unlike group A, intraprocedural echocardiography was essential for monitoring and evaluating immediate results of PBMV in group B. On follow-up of three years, 75% of group A patients and 55% in group B were in functional class I (p < 0.05). PBMV did not significantly improve symptoms in patients in group B who had preprocedure LVEF < or = 35%.

Adult↗
PubMed2005

Serum N-terminal pro-BNP levels correlate with symptoms and echocardiographic findings in patients with mitral stenosis.

This study is designed to evaluate the N-terminal pro-BNP (NTproBNP) levels in patients with mitral stenosis (MS) and its possible correlation with clinical and echocardiographic parameters of the disease. The study group consisted of 29 patients with isolated MS (patients with greater mild regurgitation were excluded) and 20 normal control subjects of similar age and gender distribution. Blood samples for NTproBNP were collected at the time of clinical and echocardiographic examination. NTproBNP levels were elevated in patients with MS compared to controls (325 +/- 249 pg/dL [19.9-890] versus 43 +/- 36 pg/dL [5.76-193.3], P < 0.001). Patients with atrial fibrillation had significantly higher NTproBNP levels compared to those with sinus rhythm (561 +/- 281 pg/dL versus 254 +/- 194 pg/dL, P = 0.044). MS patients with sinus rhythm also had higher NTproBNP levels compared to controls (254 +/- 194 pg/dL versus 43 +/- 36 pg/dL, P = 0.00011). NT pro BNP levels correlated to the LA (R = 0.73, P < 0.0001) and RV (R = 0.41, P = 0.042) diameters, mitral valve area (R =-0.45, P = 0.025), mean mitral gradient (R = 0.57, P = 0.003), peak PAP (R = 0.7, P = 0.03), and NYHA functional class (R = 0.61, P = 0.007). In conclusion, serum NTproBNP levels correlate well with echocardiographic findings and functional class in patients with MS and can be used as a marker of disease severity. Additionally, it may have a potential use as an additional noninvasive and relatively cheap method in monitoring disease progression especially in patients with poor echocardiographic windows.

Case-Control Studies↗
PubMed2000

Impact of atrioventricular compliance on pulmonary artery pressure in mitral stenosis: an exercise echocardiographic study.

BACKGROUND: The decay of the pressure gradient across a stenotic mitral valve is determined by the size of the orifice and net AV compliance (C(n)). We have observed a group of symptomatic patients, usually in sinus rhythm, characterized by pulmonary hypertension (particularly during exercise) despite a relatively large mitral valve area by pressure half-time. We speculated that this discrepancy was due to low atrial compliance causing both pulmonary hypertension and a steep decay of the transmitral pressure gradient despite significant stenosis. We therefore tested the hypothesis that C(n) is an important physiological determinant of pulmonary artery pressure at rest and during exercise in mitral stenosis. METHODS AND RESULTS: Twenty patients with mitral stenosis were examined by Doppler echocardiography. C(n), calculated from the ratio of effective mitral valve area (continuity equation) and the E-wave downslope, ranged from 1.7 to 8.1 mL/mm Hg. Systolic pulmonary artery pressure (PAP) increased from 43+/-12 mm Hg at rest to 71+/-23 mm Hg (range, 40 to 110 mm Hg) during exercise. There was a particularly close correlation between C(n) and exercise PAP (r=-0.85). Patients with a low compliance were more symptomatic (P<0.025). Catheter- and Doppler-derived values for C(n), determined in 10 cases, correlated well (r=0.79). CONCLUSIONS: C(n), which can be noninvasively assessed, is an important physiological determinant of PAP in mitral stenosis. Patients with low C(n) represent an important clinical entity, with symptoms corresponding to severe increases in PAP during stress echocardiography.

Adult↗