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A patient who died after surgery for critical mitral stenosis was found to have underlying unrecognised plexogenic pulmonary arteriopathy and familial pulmonary hypertension. The importance of recognising familial pulmonary hypertension is discussed, together with the contribution of genetic and other risk factors to plexogenic pulmonary arteriopathy.
Pulmonary vein flow (PVF) pattern was studied with an electromagnetic flowmeter in 15 patients with mitral stenosis. ECG, left atrial and left ventricular pressure were recorded simultaneously. Commissurotomy was performed in 8 patients and prosthetic valve replacement in the others. Measurements were performed before and after the intracardiac procedure. The predominating forward flow occurred in ventricular diastole, in contrast to ventricular systole in normal hearts. A small retrograde PVF was observed in 11/15 patients in early ventricular systole, coinciding with the c-wave in left atrial pressure (LAP). The reversed flow did not exceed 13% and showed no correlation to a slight regurgitation demonstrated at left ventricular angiography. The maximum delay in forward systolic PVF after onset of ventricular systole was 0.10 sec. The PVF pattern remained unchanged after commissurotomy in the majority of patients, even after reduction of the mitral valve gradient to less than 5 mmHg. However, holosystolic reversed PVF occurred in one patient, indicating a severe mitral regurgitation. The method of PVF recording is simple and proved useful for haemodynamic analysis and as a control after mitral valve surgery for evaluating the degree of mitral regurgitation.
OBJECTIVES: The vena contracta is the narrowest region of the regurgitant or stenotic jet just downstream the orifice and reflects the size of that orifice. This study was performed to assess the accuracy of the vena contracta width (VCW) in evaluating the severity of mitral stenosis (MS) and to compare the mitral valve area (MVA) determined by VCW with MVAs obtained by other more traditional echocardiographic methods. METHODS: We studied 59 patients (43 females, 42 +/- 14 years) with MS. VCW was measured in the apical four chamber view by Doppler color flow mapping. The largest diameter of the VCW during diastole was measured for at least three cardiac cycles and averaged. MVA was calculated from the following equation: pir(2), where r = VCW/2. MVA was also determined by planimetry, the pressure half-time method, and by the Gorlin formula. RESULTS: In this study, the width of the vena contracta ranged from 0.89 to 1.73 cm (mean 1.30 +/- 0.21). MVA, calculated based on the VCW, ranged from 0.63 to 2.35 cm(2) (mean 1.36 +/- 0.41). MVA by VCW (1.36 +/- 0.41 cm(2)) showed good correlations with three comparative techniques: (1) the cross-sectional area by planimetry (1.35 +/- 0.36 cm(2), mean difference = 0.21 +/- 0.16 cm(2), y = 0.91x + 0.14, r = 0.79, SEE = 0.26 cm(2), p < 0.001); (2) the area derived from the Doppler pressure half-time (1.27 +/- 0.32 cm(2), mean difference = 0.22 +/- 0.19 cm(2), y = 0.97x + 0.13, r = 0.76, SEE = 0.27 cm(2), p < 0.001), and (3) the area derived from the Gorlin equation in the 18 patients who underwent catheterization (1.27 +/- 0.35 cm(2), mean difference = 0.19 +/- 0.16, y = 0.98x + 0.05, r = 0.81, SEE = 0.26 cm(2), p < 0.001). CONCLUSIONS: These findings suggest that Doppler color flow imaging of the MS jet in the vena contracta can provide an accurate estimation of MVA and appears to be potentially applicable in the assessment of the severity of MS.
BACKGROUND: The circulating levels of adhesion molecules, such as vascular cell adhesion molecule-1 (VCAM-1) and intercellular adhesion molecule-1 (ICAM-1), have been demonstrated to be elevated in patients with rheumatic mitral stenosis (MS). However, the impact of percutaneous transluminal mitral valvuloplasty (PTMV) on the elevated circulating levels of VCAM-1 and ICAM-1 in patients with MS has never been investigated. METHODS: and results: A total of 19 patients with symptomatic MS undergoing PTMV were studied (group 1) [15 patients in chronic atrial fibrillation, and 4 patients in sinus rhythm]. The plasma levels of soluble VCAM-1 and ICAM-1 in the femoral vein and artery, and right and left atria before PTMV, and those in the peripheral venous blood at the 1-week and 4-week follow-ups after PTMV were determined by solid-phase sandwich enzyme-linked immunosorbent assay. The mitral valve area was calculated by means of the Doppler pressure half-time method. In addition, we measured plasma concentrations of soluble VCAM-1 and ICAM-1 in the peripheral venous blood samples obtained from 22 control patients (including 14 healthy volunteers in sinus rhythm [group 2] and 8 patients in chronic lone atrial fibrillation [group 3]). The plasma level of soluble VCAM-1 was significantly elevated in group 1 patients (1,205.4 +/- 462.4 ng/mL [mean +/- SD]) compared with group 2 (580.9 +/- 208.0 ng/mL) and group 3 patients (716.4 +/- 221.6 ng/mL) [p < 0.0001]. In group 1 patients, the plasma levels of soluble VCAM-1 and ICAM-1 in the left atrium did not differ from those in the right atrium, femoral vein, or femoral artery (p = 0.668 for VCAM-1, and p = 0.232 for ICAM-1). The area of mitral valve increased significantly after PTMV (1.08 +/- 0.14 cm(2) vs 1.48 +/- 0.33 cm(2), p < 0.0001). The mean left atrial pressure fell significantly after PTMV (22.9 +/- 5.2 mm Hg vs 17.7 +/- 6.0 mm Hg, p < 0.0001). The peripheral venous plasma level of soluble VCAM-1 obtained before PTMV fell significantly after PTMV (before, 1,205.4 +/- 462.4 ng/mL; 1 week after PTMV, 915.7 +/- 280.2 ng/mL; 4 weeks after PTMV, 859.0 +/- 298.7 ng/mL; p < 0.0001). CONCLUSIONS: In patients with moderate-to-severe MS, the venous plasma level of soluble VCAM-1 fell significantly after PTMV, and the elevated plasma soluble VCAM-1 concentration was associated with hemodynamic abnormality rather than with rheumatic activity.
The sensitivity of basal hemodynamic data and the value of those observed during exercise in the diagnosis of severe mitral stenosis (MS) were investigated by comparing the results under basal conditions and during dynamic exercise with the operative findings in 55 patients with pure MS, aged between 18 and 73 years (average, 43 years), 29 with mild stenosis (Group I) and 26 with severe stenosis (Group II). Under basal conditions, mean pulmonary capillary pressure (PCP) was significantly higher in Group II than in Group I (15.7 +/- 4.4 mm Hg compared to 12.2 +/- 3.5 mm Hg, p less than 0.01). The same difference was observed in mean pulmonary arterial pressure: Group I, 18.2 +/- 5.4 mm Hg; Group II: 23.0 +/- 6.1 mm Hg, p less than 0.01; but cardiac and systolic indices were comparable in the two groups. On exercise, only PCP (Group I: 29.6 +/- 6.8; Group II: 34.7 +/- 4.9 mm Hg) and systolic index (Group I: 39.9 +/- 10.0; Group II: 33.4 +/- 8.2 ml/m2) were significantly different (p less than 0.01). Pulmonary capillary and arterial pressures rose in the same proportion in the two groups on exercise but systolic index fell in severe MS (-4 p. 100) and rose in mild MS (+30 p. 100) (p less than 0.001). The formulae for calculating mitral valve surface area only give discriminating results between the two groups when the hemodynamic data on exercise are used.(ABSTRACT TRUNCATED AT 250 WORDS)
To evaluate the pathoanatomic findings of mitral valve stenosis and changes after percutaneous balloon valvuloplasty (PBV), magnetic resonance (MR) imaging was performed in 23 patients. The patients were imaged with a 2.0-T system within 1 week before and 3-10 days after PBV. The angle of the interatrial septum was measured on the transverse image to facilitate a successful transseptal puncture. On MR images, the mean transverse and anteroposterior diameters of the left atrium at the level of the aortic root in the ventricular diastolic phase decreased significantly after PBV. Areas of flow-related intraluminal signal intensity detected in the left atrial cavity of 17 patients (74%) before the procedure disappeared in 15 patients after the procedure. Other MR imaging findings after PBV were the disappearance of intraluminal signal intensity in the pulmonary artery, normal curvature of the interatrial and interventricular septa, and pericardial effusion as a complication. MR imaging was thought to provide useful information before and after PBV in patients with mitral stenosis.
In the present study, we describe our experience in using live three-dimensional transthoracic echocardiography in the assessment of mitral stenosis.
During a 1-year period, eight patients with New York Heart Association Class III or IV mitral stenosis were studied throughout the peripartum period with a pulmonary artery catheter. All patients were delivered vaginally. Intrapartum management was based upon cautious diuresis for preload optimization and heart rate control with propranolol. A mean increase in pulmonary capillary wedge pressure of 10 mm Hg was observed in the immediate postpartum period. Only two patients demonstrated a significant increase in cardiac output during this same time period. Central venous pressure correlated poorly with pulmonary capillary wedge pressure in seven of eight patients. Neonatal outcome was uniformly excellent. With the management approach described, no patient exhibited deterioration of cardiopulmonary status during the peripartum period.
BACKGROUND: Spontaneous echo contrast (SEC) of the left atrium is associated with increased risk of thromboembolism in patients with mitral stenosis (MS). The determination of the presence and severity of left atrial (LA) SEC is of prognostic importance in these patients. Harmonic imaging (HI), a novel echocardiographic technique that differs from conventional fundamental imaging (FI) in that it involves transmitting ultrasound at one frequency and receiving at twice the transmitted frequency, produces better endocardial border definition and myocardial opacification. However, there are no data about its value for the detection of LA SEC. The purpose of this study was to investigate the utility of transthoracic noncontrast tissue HI in the detection of LA SEC in patients with MS. METHODS: Seventy-four consecutive patients with MS (49 women, mean age 51 years) underwent standard transthoracic echocardiography (TTE) in both HI and FI modes and transesophageal echocardiography (TEE) to determine the presence and severity of LA SEC. Left atrial SEC was graded by TEE as either mild (only seen at high gain) or severe (visible in the entire left atrium at normal gain control of the equipment). The control group comprised 30 patients randomly selected from patients who did not have LA SEC at the TEE examination. RESULTS: Atrial fibrillation was found in 46 patients (62.2%). The mean mitral valve area and mean mitral gradient were 1.0+/-0.3 cm(2) and 8.2+/-4.1 mm Hg, respectively. Nine patients (12.2%) had episodes of systemic embolism; 8 had stroke, and 1 had peripheral embolism. Left atrial thrombus was found in 11 patients (14.9%) by TEE. Left atrial SEC was present in all but one patient by TEE (mild in 35 patients, severe in 38). Fundamental imaging with TTE, however, revealed LA SEC in only 5 (6.8%) of the 73 patients. In contrast, with HI, LA SEC could be detected in 63 (86.3%) patients. In the detection of severe LA SEC, the sensitivities of FI and HI were 13.2% (5/38) and 100% (38/38), respectively. Left atrial SEC was not observed in control subjects by either FI or HI. CONCLUSIONS: Transthoracic HI significantly enhances the detection of LA SEC in patients with MS.
Disordered left ventricular function and changes in the sub-valvular apparatus appear to be associated in patients with isolated mitral stenosis (MS). Left ventricular cine-angiography has demonstrated two groups of patients, according to the presence (group II) or absence (group I) of changes in the sub-valvular apparatus. There was no significant difference between the two groups in terms of age, heart rate, left ventricular end diastolic pressure, surface area of the mitral valve, or ventricular ejection time. However, group II patients had a lowered ejection fraction (EF), systolic ejection volume (SEB), speed of shortening of circular fibres (VCF), and systolic work index (SWI) compared with group I cases (respectively 0.49 +/- 0.06 and 0.58 +/- 0.04 for the EF, 36 +/- 7 ml.m-2 and 41 +/- 7 ml.m-2 for the SEV, 0.85 +/- 0.12 circ.s-1 and 1.09 +/- 0.16 circ.s-1 for the VCF, 45 +/- 11 gm.m-2 and 57 +/- 11 gm.m-2 for the SWI. Nevertheless, it appears that changes in the sub-valvular apparatus indicate rather than cause dysfunction of the left ventricle, which occurs in a certain number of patients who have a normal sub-valvular apparatus.
To determine whether low ejection fraction (EF) in mitral stenosis (MS) is the result of depressed contractility or is mediated by other factors, left ventricular (LV) function was analyzed by force-length and stress-shortening relationships. Thirty patients without heart disease served as normal controls (Group 1). Forty-three patients with MS were divided into 2 subgroups: Group 2 (n = 19) had EF within one standard deviation of the mean of Group 1, and Group 3 (n = 24) had EF below it. Normal EF (Group 2) was associated with low preload (end-diastolic stress) and low afterload (end-systolic stress), and preload and afterload were in the normal range in patients with low EF (Group 3). A significant negative correlation was observed in the whole group of patients with MS between EF and end-systolic stress (Y = -0.14X + 72.8, r = -0.61, p less than 0.001), and a positive correlation between end-systolic stress and volume (Y = 1.39X + 65.4, r = 0.45, p less than 0.01). These observations suggest that systolic shortening and end-systolic volume of the left ventricle are in part governed by afterload in this disease. It is concluded that low EF of MS is not mediated by reduced preload or inappropriately elevated afterload, and contractility of the ventricle is mildly depressed in MS.
1. In order to investigate the changes of reduced urinary free dopamine excretion (uDA) in heart failure, 15 patients with symptomatic mitral stenosis were investigated on their uDA, endogenous creatinine (Cr) clearance, urinary excretion of sodium (UNaV), fractional excretion of sodium (FENa), plasma noradrenaline (pNA) and plasma L-dopa concentration before and early after percutaneous transvenous mitral commissurotomy (PTMC) by the clearance study. The delivery of L-dopa to renal proximal tubules (plasma L-dopa x Cr clearance), and the conversion ratio of plasma L-dopa to urinary dopamine in the kidney [uDA/(plasma L-dopa x Cr clearance)] were also estimated. 2. After successful PTMC, uDA, UNaV and FENa showed a significant but incomplete improvement and the changes of uDA were correlated positively with those of cardiac index (CI) (r = 0.665, P < 0.01), not with changes of pulmonary wedge pressure. While plasma L-dopa and plasma L-dopa x Cr clearance improved, uDA/(plasma L-dopa x Cr clearance) was not significantly changed early after PTMC. 3. From these results, it was suggested that reduced uDA tended to increase incompletely in relation with functional recovery of heart, and that increased plasma L-dopa and a delivery of L-dopa to renal proximal tubules have some positive role on urinary dopamine excretion, at least, early after PTMC.
Congenital absence of aortic cusps leads to severe aortic regurgitation. We present a newborn with this rare entity with extreme mitral stenosis. Hemodynamic features were those of hypoplastic left heart syndrome. Surgical management consisted of initial modified Norwood procedure followed by orthotopic heart transplantation.
A 43-year-old woman in whom anomalous origin of the left coronary artery from the pulmonary artery, associated with rheumatic mitral stenosis, was surgically corrected. She underwent aortic implantation of anomalous left coronary artery and mitral valve replacement. It was feasible to obtain a sufficient length of the left main trunk excising a large cuff of pulmonary artery wall surrounding the ostium of anomalous left coronary artery, without transecting pulmonary artery. This surgical case is first reported about BWG syndrome associated with rheumatic valvular disease.
To assess the incidence, pathophysiology and influence on operative outcome of a depressed left ventricular (LV) ejection fraction (EF) in patients with mitral stenosis (MS), demographic, hemodynamic and cineangiographic data on 72 patients (16 men, 56 women, aged 19 to 75 years) with isolated MS were reviewed. Of the 45 who had mitral commissurotomy or replacement, operative course and functional class before and after surgery were assessed. Of the 72 patients, 21 (29%) had an LVEF < or = 0.50. These 21 were similar to the 51 with an LVEF > 0.50 in age, gender, heart rate, intracardiac pressures, transvalvular gradient and valve area, but they had larger LV end-diastolic (79 +/- 19 [mean +/- SD] vs 59 +/- 15 ml/m2, p < 0.001) and end-systolic volumes (46 +/- 13 vs 23 +/- 8 ml/m2, p < 0.0001). Of the 45 subjects undergoing surgery, operative outcome was similar in the 14 with a depressed and the 31 with a normal LVEF. Thus, about 1/3 of patients with isolated MS have a depressed LVEF. Compared with those with MS and a normal LVEF, these subjects have hemodynamic derangements of similar severity, but they have larger LV end-diastolic and end-systolic volumes, suggesting that impaired LV contractile function or excessive afterload (rather than diastolic underfilling), or both, is the cause of a low LVEF. Those with an LVEF < or = 0.50 who undergo valve surgery have a similar operative outcome as those with an LVEF > 0.50.
Normal gestation is associated with a hyperdynamic adaptive state. The circulatory changes represent an additional burden on the cardiovascular system of women with rheumatic mitral stenosis (MS). Life-threatening complications can occur in pregnant women with severe MS. We successfully performed percutaneous balloon mitral valvotomy (PMV) in eight pregnant patients (mean age 28 +/- 6.9 years, range 21-38 years). Pregnancy mean age was 24.6 +/- 6.5 weeks. Five patients were in NYHA functional class III and three patients were in class IV. Emergency PMV seemed to be life saving to both mother and foetus in one case. All patients but one had pliable valves. PMV was achieved using the double balloon transseptal technique. To protect the foetus from radiation, the patient's pelvic-abdominal area was shielded and left ventriculography was not performed. The total mean time of PMV was 72 +/- 19 min and that of fluoroscopy was 16.6 +/- 7.8 min. Gorlin's mitral valve area increased from 0.83 +/- 0.15 to 2.4 +/- 0.34 cm2 and the cardiac index from 3.1 +/- 0.77 to 4.2 +/- 0.79 l.min-1.m-2. Left atrium pressure decreased from 29 +/- 10 to 14 +/- 5 mmHg and mitral valve gradient from 21 +/- 7 to 6 +/- 3 mmHg. There were no complications or residual MS. At a mean follow-up of 13.2 +/- 9.4 months, all patients were in NYHA class I and had a normal course of pregnancy. The eight women delivered healthy full-term babies. At 1-25 months post-partum follow-up, the eight infants had shown normal growth and development.(ABSTRACT TRUNCATED AT 250 WORDS)
The aim of this study was to assess whether there is blood coagulation activation in patients with mitral stenosis (MS) and sinus rhythm (SR) and to investigate the value of left atrial spontaneous echo contrast (LASEC) as a predictive sign of increased coagulation activity. Using thrombin-antithrombin III complexes and prothrombin fragment 1+2 as in vivo hemostatic markers, we concluded that there is a hypercoagulable state in patients with MS and SR when LASEC is present.
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