An echocardiographic study of net atrioventricular compliance in juvenile and adult mitral stenosis

BACKGROUND: Left atrial compliance is an important determinant of symptoms in mitral stenosis. About one-third of patients with mitral stenosis have reduced left ventricular compliance. We measured the net atrioventricular compliance in rheumatic mitral stenosis patients noninvasively and analyzed if there were any clinical, electrocardiographic, roentgenographic or echocardiographic correlates of net atrioventricular compliance. METHODS AND RESULTS: Seventy-six patients with mitral stenosis were analyzed and as many normal subjects were taken as control group. Patients were divided into two groups--those 20 years and below were grouped as juvenile mitral stenosis and those above 20 years as adult mitral stenosis patients. The net atrioventricular compliance in patients with mitral stenosis was significantly impaired compared to normal population. Mean compliance in juvenile group was 4.66+/-2.18 ml/mmHg (range 2.17-9.6) and in adult group it was 4.79+/-1.99 ml/mmHg (range 2.04-8.9) (p = ns). There was no difference in net atrioventricular compliance between the juvenile and adult patients with mitral stenosis. Mitral valve area showed an independent positive correlation with net atrioventricular compliance. CONCLUSIONS: The net atrioventricular compliance was significantly reduced in patients with rheumatic mitral stenosis; however, there was essentially no difference in the net atrioventricular compliance between the juvenile and adult patients with mitral stenosis. The net atrioventricular compliance may not be responsible for the more severe symptoms observed in juvenile mitral stenosis.     

Two-dimensional and Doppler echocardiographic determinants of the natural history of mitral valve narrowing in patients with rheumatic mitral stenosis: implications for follow-up.

Fifty patients with rheumatic mitral stenosis were studied with serial two-dimensional and Doppler echocardiography to determine the natural history of changes in mitral valve area and its relation to transmitral gradients and mitral valve morphology. Over the 39-month observation period (range 7 to 74 months) the decline in valve area was 0.09 +/- 0.21 cm2/year. In addition, there were significant increases in total echocardiographic score (p = 0.0001), severity of mitral anulus calcification (p = 0.05) and severity of mitral regurgitation (p = 0.0007). Patients with an echocardiographic score greater than or equal to 8 had a more progressive course. In addition, patients with a more progressive course (decline in valve area greater than or equal to 0.1 cm2/year) had a significantly greater initial mean gradient (p = 0.01), peak gradient (p = 0.007) and total echocardiographic score (p = 0.0008). Initial valve area did not correlate with the rate of stenosis progression. Of 22 patients with an echocardiographic score less than 8 and a peak mitral gradient less than 10 mm Hg, only 1 patient (5%) had a more progressive course, compared with 80% of those with a total echocardiographic score greater than or equal to 8 and a gradient greater than or equal to 10 mm Hg. The rate of mitral valve narrowing in individual patients with rheumatic mitral stenosis is variable. Patients whose valve disease progresses rapidly are those with a greater mitral valve echocardiographic score and higher peak and mean transmitral gradients.(ABSTRACT TRUNCATED AT 250 WORDS)     

Assessment of rheumatic mitral valve disease. Value of echocardiography in patients clinically suspected of predominant stenosis.

The value of echocardiography as compared with cardiac catheterisation was evaluated prospectively in 33 consecutive patients clinically suspected of predominant mitral stenosis. Patients with clinical signs of accompanying mitral regurgitation, no matter how severe, and patients with clinical findings indicating insignificant aortic valve disease were included. Critical mitral stenosis was defined by a valve area of less than or equal to 1 cm2. Severe mitral regurgitation was diagnosed by echocardiography on the basis of left ventricular dilatation (more than 3.2 cm/m2 at end-diastole) if not explained otherwise. Significant aortic valve disease was suspected in cases with aortic valve deformity and left ventricular dilatation or hypertrophy as defined by echocardiography. Mitral valve area by echocardiography correlated well with mitral valve area calculated from catheterisation data and a good interobserver correlation was found for echocardiographic measurement. Mitral stenosis, critical or non-critical, may mask significant coexistent valve lesions; echocardiography failed to discover severe mitral regurgitation requiring valve replacement in two patients with non-critical stenosis, and significant aortic regurgitation needing valve replacement was underestimated in one patient with critical mitral stenosis. A correct echocardiographic classification with respect to surgery, however, was obtained in: (1) all patients with clinically pure mitral stenosis (nine patients), and (2) all patients with combined mitral stenosis and regurgitation when either critical stenosis or severe regurgitation was found at echocardiography (12 patients). It thus appears that two out of three patients with mitral valve disease in whom the clinical findings indicate predominant stenosis can be correctly evaluated with the echocardiogram.     

Left ventricular performance following mitral valve replacement in patients with tight mitral stenosis combined with mild aortic regurgitation

The severity of aortic regurgitation is difficult to estimate prior to mitral valve replacement (MVR) in cases with tight mitral stenosis (MS), because low output state due to mitral obstruction masks signs of aortic regurgitation. This study clarified left ventricular performance, possibly affected by increased diastolic loading after MVR. The study subjects consisted of 12 patients with pure mitral stenosis (MS group) and 11 with combined mitral stenosis and aortic regurgitation (MSAR group). The diagnosis was made by cardiac catheterization preoperatively. The aortographic grade of aortic regurgitation was class 1 or 2 according to the AHA classification. Both groups were matched in terms of severity in mitral obstruction evaluated by mitral valve area. On preoperative echocardiographic evaluation, there was no difference in the mean values of LVDd, LVSd, and %FS between the groups MS and MSAR. After surgery, symptoms improved in each patient. Echocardiography performed three months after MVR revealed no differences in these parameters between both the groups. We concluded that aortic regurgitation evaluated as class 1 or 2 preoperatively does not increase in respect to left ventricular diastolic overloading and echocardiographic left ventricular performance remains unchanged.     

A critical appraisal of M-mode echocardiography in mitral stenosis

The reliability of M-mode echocardiography in confirming mitral stenosis and in assessing its severity was tested in 39 patients with mitral stenosis. None of the conventional echocardiographic signs of mitral stenosis is absolutely reliable. Unless at least 2 signs are combined, the diagnosis is not conclusive (sensitivity = 0.97, specificity = 1.00). Of a number of echocardiographic parameters and equations recommended (as tools) to determine the severity of mitral stenosis, the early diastolic velocity of anterior leaflet opening (EF) is the only one with a statistically significant relationship to the mitral valve area (MVA) calculated by Gorlin (r = 0.712, p less than 0.001), and this only in uncomplicated unoperated mitral stenoses, but not in mitral restenoses and combined mitral defects. Even the recently published Seitz's equation for calculating MVA from echocardiographic data is not reliable. M-mode echocardiography is an excellent technique for the primary diagnosis of mitral stenosis. However, it is not suitable to even an approximate assessment of its haemodynamic severity.     

Membranous supravalvular mitral stenosis: a treatable form of congenital heart disease

The clinical data, echocardiographic findings, operative anatomy and postoperative follow-up were assessed in 14 patients who had surgery for membranous supravalvular mitral stenosis between 1978 and 1985. The patients ranged in age from 6 weeks to 13 years at the time of operation, and 8 of the 14 had associated mitral valve abnormalities. Other associated lesions included ventricular septal defect (n = 7), coarctation of the aorta (n = 5), left superior vena cava (n = 6), subaortic stenosis (n = 3) and atrial septal defect (n = 1). Twelve of the 14 patients had successful removal of the supravalvular membrane, which was usually adherent to the valve, and 2 patients with associated mitral valve abnormalities underwent mitral valve replacement. There were no operative deaths. Review of preoperative two-dimensional echocardiograms, which were available in 11 patients, revealed two types of membranous supravalvular mitral stenosis in 10 patients. In four of these patients, the membrane was only evident after repeated stop action viewing from a single subcostal or parasternal location. The membrane was never seen in one patient. Eleven patients had follow-up in excess of 1 year, and there was one late death. Eight of the remaining 10 patients are asymptomatic, and 7 have no clinical evidence of residual mitral obstruction. Failure to recognize membranous supravalvular mitral stenosis can result in undue delay of cardiac surgery with resultant cardiopulmonary deterioration. Patients with evidence of left ventricular inflow obstruction should have extensive echocardiographic evaluation in an effort to detect membranous supravalvular mitral stenosis, which may be amenable to surgical repair.     

Percutaneous mitral commissurotomy: immediate and short-term results]

31 symptomatic patients with mitral stenosis were selected for percutaneous transvenous mitral commissurotomy using Inoue catheter. The patients were selected using the echocardiographic score for: leaflets mobility, leaflets thickening, subvalvular thickening, degree of calcifications. All patients had a score less than or equal to 8 and represented 17.5% of the patients studied in our echocardiographic laboratory for mitral stenosis. We were able to perform the commissurotomy in 30 of them. Mean left atrial pressure decreased from 26 +/- 5.2 mmHg to 14.6 +/- 6 mmHg (p less than 0.001). The mean mitral diastolic pressure gradient decreased from 8.9 +/- 3.1 mmHg to 3.9 +/- 1.3 mmHg (p less than 0.001). The mitral valve area, using the echocardiographic Pressure Half Time (PHT), increased from 0.94 +/- 0.17 cmq to 1.96 +/- 0.33 cmq (p less than 0.001). Mitral regurgitation, angiographically evaluated in 29 patients, increased in 11 (38%), being of degree + + + in 3 patients. There were 2 heart tamponades and 5 cases (16.6%) of left-to-right shunt with Qp / Qs less than 2. After 3 months, the follow-up showed improvement of one or more functional classes in 96.6% of all patients. The mitral valve area, determined after 6 months in 24 patients by PHT, was stable (1.98 +/- 0.31 vs 1.93 +/- 0.25) (p = 0.5); we did not find mitral stenosis recurrence in any instance. In the first 10 patients, after 1 year, the results are stable (1.85 +/- 0.28 cmq vs 1.93 +/- 0.21 cmq) (p = 0.5) without mitral stenosis recurrence. These data suggest that in selected tight mitral stenosis the percutaneous transvenous commissurotomy may be alternative to the open surgical solution. Using an Inoue catheter, the percutaneous transvenous mitral commissurotomy is easier and the complications are few. The major procedural hazards derive from the transseptal technique.     

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.     

Comparisons between female and male patients with mitral stenosis.

OBJECTIVE--To compare Doppler, echocardiographic, and clinical variables in female and male patients with mitral stenosis. DESIGN--Observational study in consecutive patients with mitral stenosis of cross sectional and Doppler echocardiographic and clinical variables and a retrospective search for a history of systemic embolism. SETTING--A medical centre with 3000 beds, serving both urban and rural populations. PATIENTS--500 consecutive patients with an echocardiographic mitral valve area of 2 cm2 or less. 331 (66.2%) were female and 169 (33.8%) male (mean (SD) ages of 49 (13) and 48 (14) respectively). MAIN OUTCOME MEASURES--Mitral valve areas by echocardiographic planimetry and Doppler pressure half-time method, peak early diastolic mitral velocity and pressure gradient, echocardiographic score of mitral valve, left atrial end systolic diameter, frequency of left atrial thrombus and smoky echoes as well as various valve lesions detected with Doppler and echocardiography, cardiac rhythm, symptomatic functional class of heart failure, and history of systemic embolism. RESULTS--The prevalence of significant tricuspid (22% v 9%, P < 0.001) and pulmonary regurgitation (5% v 1%, P = 0.018) was higher in the female patients than in the male patients. Female patients also had a higher peak regurgitant velocity (3.2 (0.7) v 2.9 (0.7) m/s, P = 0.007) and pressure gradient (41 (21) v 36 (19) mm Hg, P = 0.010) across the tricuspid valve. However, the male patients had a higher echocardiographic score (9.7 (2.4) v 7.0 (2.3), P < 0.001) and a smaller Doppler-derived mitral valve area (0.9 (0.4) v 1.0 (0.4) cm2, P = 0.027). There were no differences between the female and the male patients in mitral valve area measured by planimetry, peak early diastolic mitral velocity and pressure gradient, and left atrial end systolic diameter or in the prevalence of atrial fibrillation, left atrial thrombus, left atrial smoky echoes, significant aortic stenosis, aortic regurgitation, or heart failure of New York Heart Association class III or IV. CONCLUSIONS--Female patients not only had a higher prevalence of mitral stenosis but also had a higher prevalence of associated tricuspid and pulmonary regurgitation along with a higher velocity and gradient of tricuspid regurgitation. The echocardiographic score was higher in male patients, however. These findings suggest that the pathophysiology of mitral stenosis is different in the two sexes and that gender should be taken into account when therapeutic strategies are formulated.     

Percutaneous mitral valvuloplasty. Immediate results with the 30 mm diameter dilatation balloon]

Percutaneous balloon dilatation of the mitral valve is a promising alternative to surgical treatment for patients with mitral stenosis. We analysed echocardiographic and haemodynamic results in 62 consecutive patients who underwent mitral dilatation with a big and one chamber balloon (30 mm diameter). The relationship between the dilatation balloon area and body surface was 4.2 (in 98% of patients it was bigger than 3.5). In the whole series we get significant improvements in the haemodynamic parameters and mitral areas (0.91 cm2 versus 1.78 cm2)(p < 0.005). According to the echocardiographic score, patients were divided in two groups. Good results (mitral area > 1.5 cm2, and final, valve area 50% greater than the initial area), were obtained in 93% of patients with a score of 8 or less, and in 45% of patients with a score bigger than 8. Seventy-five percent of patients with bad results (mitral area < 1.5 cm2) were in atrial fibrillation versus 37% of patients where we get a good results. Mortality of procedure was nulle, and two patients required surgery because of procedure failure for mitral insufficiency. We can conclude: percutaneous mitral valvuloplasty with single balloon is valid alternative, and good results can be expected if there is a good relationship between the area of balloon and patient's body-surface. The echocardiographic score and presence of atrial fibrillation can predict immediate results of this technique.     

Long-term follow-up of iatrogenic atrial septal defect: after percutaneous mitral balloon valvuloplasty

During percutaneous mitral balloon valvuloplasty, the Inoue method leaves patients with an iatrogenic atrial septal defect. In this study, we evaluated the factors affecting the development of iatrogenic atrial septal defect and searched for the possible influence of this defect on long-term outcomes.We reviewed the medical records of 267 patients who had undergone successful percutaneous mitral balloon valvuloplasty for symptomatic moderate or severe mitral stenosis from January 2000 through March 2004. Sixty-three of the 267 patients were enrolled in a face-to-face follow-up study. We noted their clinical and demographic characteristics. All included patients were asked for the endpoints of repeat percutaneous mitral balloon valvuloplasty or mitral valve surgery, cerebrovascular accident or transient ischemic attack, and the need of intervention for the iatrogenic atrial septal defect. They underwent standard 2-dimensional and Doppler echocardiographic examination. The presence of iatrogenic atrial septal defect was evaluated via the color-Doppler technique in the subcostal view and via contrast echocardiography.Patients were subclassified in accordance with the presence (n = 15) or absence (n = 48) of echocardiographically proven persistent iatrogenic atrial septal defect. When we compared the 2 groups, there were no significant differences in baseline demographic characteristics or in pre- and postprocedural echocardiographic data.We conclude that the presence of persistent iatrogenic atrial septal defects might not be predicted from echocardiographic or demographic data in patients undergoing percutaneous mitral balloon valvuloplasty. Fortunately, these defects are small in size and low in shunt ratio. They appear not to be associated with serious long-term outcomes.     

Left Atrial Compliance and Pulmonary Venous Flow Velocities Are Related to Functional Status in Patients with Moderate‐to‐Severe Mitral Stenosis

Aim: In mitral stenosis (MS), left atrial (LA) compliance plays a crucial role in the occurrence of symptoms and pulmonary arterial hypertension and can alter pulmonary venous flows (PVF). The aim of present study is to compare LA compliance (net atrioventricular compliance‐Cn) and PVF velocities in patients with different functional status despite similar mitral valve area (MVA). Additionally, the relationships of the same variables with each other and other echocardiographic parameters showing the hemodynamic severity of mitral stenosis were investigated. Methods: Thirty‐one patients with moderate to severe mitral stenosis were studied. The patients were divided into two groups according to their NYHA functional classes (FC). The patients with lower FC (NYHA FC1 and FC2) were included in Group I(n = 15), and those with FC ≥ 3 included in Group II (n = 16). All patients underwent comprehensive transthoracic and transesophageal echo Doppler examination. Results: Despite similar LA size, MVA, and transmitral diastolic pressure gradients, systolic pulmonary artery pressure (SPAP) was significantly higher and LA compliance was significantly lower in Group II as compared to group I. Also, systolic PVF velocity (PVs), diastolic PVF velocity (PVd), and PVs/PVd ratio were significantly lower in Group II as compared to Group I. In whole group, significant positive correlations between LA compliance and PVs (r = 0.38, P = 0.035), as well as PVd (r = 0.40, P = 0.023) and, significant negative correlation between LA compliance and SPAP (r =– 0.36, P = 0.047) were noted. Conclusion: Our findings suggested that in patients with MS, when there is inconsistency between symptomatic status and conventional echocardiographic variables, simple and noninvasive Doppler parameters, LA compliance, and PVF could be measured before cardiac catheterization for more accurate and complete evaluation of patients. (ECHOCARDIOGRAPHY, Volume 26, November 2009)     

Supravalvular aortic stenosis: clinical and genetic study of a family group

We describe a family with a high frequency of supravalvular aortic stenosis. The family includes 5 generations and 80 subjects (prospective study in 66, on whom physical examination, ECG, M-mode and two-dimensional echocardiogram were performed, and retrospective analysis of available data in 14). This is the largest family group with this disease studied so far. Thirty-six subjects (45%) were found to be affected. On the basis of the echocardiographic image and of the haemodynamic gradient (when available), three different degrees of supravalvular aortic stenosis were identified. The disease was found to be severe in 8 subjects (22%), moderate in 6 (17%), mild in 13 (36%) and undefined in 8 (22%). In 4 cases multiple pulmonary stenoses were associated with supravalvular aortic stenosis, while in one subject multiple pulmonary stenoses were noted in the absence of aortic abnormalities. In the family we studied, the supravalvular aortic stenosis gene is transmitted with a pattern of inheritance consistent with an autosomal dominant trait with variable expressivity and penetrance (penetrance coefficient = 0.86). A high mortality rate in early childhood was observed, while symptoms and ECG abnormalities were not related to the degree of the stenosis. Furthermore, we found a high rate of mitral valve echocardiographic abnormalities, such as mitral prolapse and systolic anterior motion. The absence of Williams dysmorphic somatic features in the many generations as well as in the large number of patients we studied, appears to exclude the coexistence of Williams and Eisenberg's syndromes in the same family group.     

The heterogeneity of hypertrophic cardiomyopathy: An autopsy and one dimensional echocardiographic study

Although echocardiography is sensitive in detecting genetically determined idiopathic hypertrophic subaortic stenosis with the two major criteria of systolic anterior motion of the mitral valve and asymmetric septal hypertrophy, the predictive value of these findings in a nonselected population remains uncertain. A study was made of nine consecutive patients with an echocardiographic diagnosis of idiopathic hypertrophic subaortic stenosis who underwent autopsy between 1975 and 1979. The patients ranged in age from 1 month to 74 years (average 53), and six were women. Seven had systolic anterior motion of the mitral valve, and one pseudo systolic anterior motion, eight had asymmetric septal hypertrophy, and three had mid systolic aortic valve closure. All nine had a systolic murmur at the cardiac apex and an abnormal electrocardiogram. At autopsy only two patients had asymmetric septal hypertrophy and myocardial fiber disarray; the other seven had no evidence of hypertrophic Cardiomyopathy on gross examination or light microscopy. Of these seven, two had concentric left ventricular hypertrophy, one coronary atherosclerosis, one cardiac amyloidosis, and three no cardiac disease. These data suggest that systolic anterior motion of the mitral valve and asymmetric septal hypertrophy may be sensitive but are poorly predictive of idiopathic hypertrophic subaortic stenosis in the general hospital population. Because these two findings may be observed even in patients with a normal heart, an echocardiographic diagnosis of idiopathic hypertrophic subaortic stenosis based on these criteria should be made with caution.     

Cross-sectional echocardiographic localization of sites of left ventricular outflow tract obstruction

Real time two-dimensional echocardiographic studies of left ventricular outflow tract cross-sectional anatomy were obtained by the multicrystal echocardiographic method (Bom system) in 35 patients with various types of outflow obstruction as delineated by clinical, hemodynamic and angiographic studies. In each patient the noninvasive test allowed prediction of the site of obstruction. In valvular aortic stenosis, echocardiographic diagnostic findings included poststenotic dilatation of the ascending aorta, thickened aortic cusp tissue and increased superior-inferior cusp excursion (doming). The site of supravalvular aortic stenosis was readily observed although echocardiographic findings often underestimated the degree of obstruction recorded at cardiac catheterization. In patients with discrete subvalvular aortic stenosis, the major finding was a localized thickening of the septum and anterior mitral anulus producing a narrowing of the left ventricular outflow tract that was present in diastole and persisted throughout the cardiac cycle. The combination of discrete subvalvular and valvular aortic stenosis could be identified as well as mitral valve abnormalities associated with left ventricular outflow tract obstruction. Asymmetric septal hypertrophy and systolic anterior motion of the mitral leaflets were noted in six patients with idiopathic hypertrophic subaortic stenosis. This new echocardiographic approach allowed accurate localization of the site of left ventricular outflow tract obstruction and detection of associated malformations. The method has substantial merit as an initial test to establish diagnosis and allows more appropriate planning of a subsequent hemodynamic study.     

Echocardiographic predictors of pulmonary hypertension in patients with severe aortic stenosis.

BACKGROUND: Pulmonary hypertension complicating severe aortic stenosis increases morbidity and mortality. Causes and mechanisms of this are unclear. METHODS: This is a retrospective observational study of 626 patients with severe aortic stenosis who had measurable pulmonary arterial pressure by Doppler echocardiography. Clinical, echocardiographic and pharmacological data were related to the presence of pulmonary hypertension. RESULTS: Of the 626 patients, 119 (19%) had severe pulmonary hypertension defined as pulmonary artery systolic pressure > or =60 mmHg. Patients with severe pulmonary hypertension had a smaller aortic valve area (P < 0.0001), a lower left ventricular ejection fraction (P < 0.0001), a higher mitral E/A velocity ratio (P < 0.0001) indicating a higher filling pressure and a higher prevalence of 3 or 4+ mitral regurgitation (P < 0.001). They were less likely to be on a beta blocker (P = 0.05) or a statin (P = 0.02). Smaller aortic valve area, left ventricular dysfunction, mitral regurgitation and lack of statin use were independent predictors of severe pulmonary hypertension. CONCLUSIONS: Severity of aortic stenosis, left ventricular dysfunction, and mitral regurgitation are risk factors for the genesis of pulmonary hypertension and statins may potentially be protective in patients with severe aortic stenosis.     

A comparison of the assessment of mitral valve area by continuous wave Doppler and by cross sectional echocardiography.

Transmitral pressure half time (PHT) was assessed by continuous wave Doppler in 44 patients with rheumatic mitral valve stenosis (14, pure mitral valve stenosis; 15, combined mitral stenosis and regurgitation; and 15 with associated aortic valve regurgitation). The mitral valve area, derived from transmitral pressure half time by the formula 220/pressure half time, was compared with that estimated by cross sectional echocardiography. The transmitral pressure half time correlated well with the mitral valve area estimated by cross sectional echocardiography. The correlation between pressure half time and the cross sectional echocardiographic mitral valve area was also good for patients with pure mitral stenosis and for those with associated mitral or aortic regurgitation. The regression coefficients in the three groups of patients were significantly different. Nevertheless, a transmitral pressure half time of 175 ms correctly identified 20 of 21 patients with cross sectional echocardiographic mitral valve areas less than 1.5 cm2. There were no false positives. The Doppler formula significantly underestimated the mitral valve area determined by cross sectional echocardiography by 28(9)% in 19 patients with an echocardiographic area greater than 2 cm2 and by 14.8 (8)% in 25 patients with area of less than 2 cm2. In thirteen patients with pure mitral valve stenosis Gorlin's formula was used to calculate the mitral valve area. This was overestimated by cross sectional echocardiography by 0.16 (0.19) cm2 and underestimated by Doppler by 0.13 (0.12) cm2. Continuous wave Doppler underestimated the echocardiographic mitral valve area in patients with mild mitral stenosis. The Doppler formula mitral valve area = 220/pressure half time was more accurate in predicting functional (haemodynamic) than anatomical (echocardiographic) mitral valve area.     

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.     

Mitral valve prolapse in patients with prior rheumatic fever

It is known that rheumatic heart disease frequently results in isolated mitral regurgitation without concomitant mitral stenosis, especially in countries with a high prevalence of rheumatic fever. However, more recent surgical pathologic data also have demonstrated a high incidence of mitral valve prolapse in cases of rheumatic heart disease, which suggests that rheumatic fever may be a cause of mitral valve prolapse. To determine whether this association of mitral valve prolapse and rheumatic heart disease is present in a stable clinic population, we studied 30 patients who had an apical systolic murmur and a well-documented history of rheumatic fever with dynamic auscultation, two-dimensional echocardiography, and pulsed Doppler examinations. Twenty of the 30 patients (67%) had findings on physical examination consistent with isolated mitral regurgitation and 25 patients (84%) had mitral regurgitation by Doppler examination. Echocardiography demonstrated mitral valve prolapse in 24 patients (80%), whereas only one of the total study group had echocardiographic findings consistent with mitral stenosis. We conclude that (1) the presence of an isolated systolic murmur in patients with a history of rheumatic fever frequently represents pure mitral regurgitation secondary to mitral valve prolapse and (2) postinflammatory changes in valvular tissue resulting from rheumatic fever may be the etiology of mitral valve prolapse in these patients.     

Echokardiographische Diagnostik angeborener Herzfehler im Erwachsenenalter

Echo and Doppler echocardiographic procedures have gained special importance in the diagnostics of congenital diseases in adults. These procedures permit detailed visualization of the pathomorphology of the heart as well as reliable evaluation of the hemodynamic changes. There are differentiated indications for the various procedures, such as transthoracic and transesophageal echocardiography, Doppler and color-Doppler echocardiography, contrast echocardiography and 3-dimensional echocardiography. This article discusses the opposition of the various echo and Doppler echocardiographic procedures with respect to the diagnostics of the most frequent non-operated congenital diseases in adults. The pathomorphology of the various congenital diseases will be summarized and then the important echocardiographic criteria presented which are decisive for the diagnostic procedure. In simple congenital malformation of cardiac valves, such as bicuspid aortic valve (Figure 1: aortic ring abscess), pulmonary valve stenosis (Figure 2), Ebstein's anomaly (Figure 3) or malformations of the mitral valve (Figure 4: cleft in the anterior mitral cusp), the diagnosis can often be made using transthoracic echo and Doppler echocardiography, and the severity of the defect determined. However, the sonographic conditions, especially in adults, are frequently too limited to permit recognition of detailed smaller changes, so that transesophageal examination is required to finally confirm the diagnosis in these patients. In the diagnostics of diseases of the left ventricular outflow tract and the thoracic aorta, such as subvalvular aortic valve stenosis (Figure 5), the sinus of Valsalva aneurysm or the coarctation of the aorta (Figure 6), the left ventricular outflow tract can be evaluated morphologically from a transthoracic procedure and the accelerations of flow can be recorded by continuous wave Doppler. If there is no sclerosis of the fibrous membrane, these can often not be depicted by transthoracic procedures, so that a supplementary transesophageal examination is meaningful. This is required in any case for diseases of the descending thoracic aorta. In the case of congenital lesions, such as atrial septal defects (Figure 7: anomalous pulmonary venous return, Figure 8: 3-dimensional visualization of an atrial septal defect, Figure 9: sinus venosus defect), ventricular septal defect or a patent ductus arteriosus Botalli (Figure 10), color-Doppler and contrast echocardiography have become especially important. Transesophageal examination is also indicated for these congenital diseases for direct depiction of the defect as well as for precise evaluation of the shunt. Moreover, in atrial septal defects, it has been shown that a 3-dimensional echocardiography provides additional advantage with respect to spatial relationship of the defect to the other cardiac structures, as well as presenting dynamic changes during a heart cycle. Extensive knowledge of complex congenital heart disease, such as tetralogy of Fallot (Figure 11), complete transposition of the great arteries, congenitally corrected transposition of the great arteries (Figure 12), the double-outlet right ventricle, truncus arteriosus communis, the cor triatriatum, tricuspid atresia (Figure 13) or the univentricular heart (Figure 14) usually requires performance of a transthoracic echo- and Doppler echocardiographic examination to assess the pathomorphological changes and to examine hemodynamics. In the majority of patients, supplementary transesophageal echocardiography and an echo contrast examination are important. Initial examinations using 3-dimensional echocardiography are very promising in this connection and with respect to the exact spatial presentation of pathoanatomical structures.