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[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↗

[A case report of successful mitral valve replacement for congenital mitral stenosis associated with coarctation of the aorta and ventricular septal defect].

A 5-month-old infant with coarctation of the aorta, ventricular septal defect and mitral stenosis known as "Shone's anomaly" is presented. He underwent the repair of coarctation of the aorta by means of the extended aortic arch anastomosis and banding of the pulmonary trunk at 1 month of age and the patch closure of ventricular septal defect and debanding of the pulmonary trunk at 3 months of age in our institution. About 2 months after second surgery, he had been admitted to our institution due to developing tachypnea and he needed the support of mechanical ventilation. The chest X-ray showed pulmonary congestion and the echocardiography revealed only one papillary muscle of mitral valve and pressure gradient about 30 mmHg through mitral valve. Mitral stenosis due to parachute mitral valve was suspected and he was subjected to an emergent surgery. Initially we performed mitral valve repair for parachute mitral valve but echocardiography during the surgery revealed moderate grade of mitral regurgitation and a hemodynamics was not satisfactory. Eventually mitral valve replacement was successfully done with Carbo-Medics mechanical valve (19 mm in diameter) in the position of left atrial wall because his mitral annulus was so small as 10 mm in diameter. The postoperative course was uneventful and the patient has been doing well.

Aortic Coarctation↗

[Acquired heart valve defects. Diagnostic and therapeutic strategies].

BACKGROUND: Diagnostic and therapeutic strategies in patients with acquired valvular heart disease, are determined by clinical symptoms, hemodynamics and empirical information. DIAGNOSIS: A quantitative assessment of cardiac disease can made largely on the basis of echocardiography or Doppler echocardiography with consideration also being given to the results of clinical findings. As a rule, cardiac catheterization should be done only once, immediately prior to operation. OUTLINE OF THERAPY: In chronic mitral and aortic valve insufficiency, the surgical indication is based primarily on the clinical symptoms, while in aortic and mitral valve stenosis, it is based primarily on the hemodynamic findings. Promising interventional procedures such as balloon valvuloplasty, represent useful alternatives to valve replacement in the case of mitral valve stenosis alone. In the event of significant aortic valve stenosis alone. In the event of significant aortic valve stenosis, balloon valvuloplasty is not a promising procedure. In patients with aortic or mitral valve insufficiency, but normal left ventricular function, medical treatment should first be attempted.

Catheterization↗

Echocardiographic evaluation of mitral stenosis in predicting mitral valve replacement vs commissurotomy. Relation to hemodynamic measurements.

Fifty-one patients with mitral stenosis were studied by M-mode echocardiograms to verify the possibility of predicting if they will require mitral valve replacement or commissurotomy. Fifteen of 18 patients with heavy calcification and restricted or poor valvular mobility underwent mitral valve replacement. Twelve of 14 patients with normal valve amplitude underwent mitral commissurotomy regardless of the presence of valvular calcification. A newly derived measurement, the MT/ST, which is the ratio between the maximal thickness of the widest echo from the mitral valve and the maximal thickness of the left ventricular margin of the interventricular septum, was used to assess valvular calcification. Values above 1.7 were present only in valves with restricted or poor mobility and indicated mitral valve replacement in 14 of 15 cases. All of the patients undergoing mitral valve replacement who had MT/ST ratios between 1.5 and 1.7 had restricted or poor valvular mobility. Of the patients with MT/ST ratios less than 1.5, ten of 12 with normal valvular amplitude underwent mitral commissurotomy, and four of five with restricted valvular mobility underwent mitral valve replacement. We conclude that echocardiographic assessment of mitral valvular calcification and amplitude is useful in predicting patients who will require mitral valve replacement vs mitral commissurotomy.

Adolescent↗

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↗

Echocardiographic assessment of commissural calcium: a simple predictor of outcome after percutaneous mitral balloon valvotomy.

OBJECTIVES: This study was undertaken to determine whether the presence of calcium in the mitral valve commissures, as demonstrated echocardiographically, could predict outcome and to compare this with an established echocardiographic scoring system. BACKGROUND: Percutaneous mitral balloon valvotomy is an effective form of treatment for mitral valve stenosis. It is important to identify patients who would benefit from this procedure. Commissural splitting is the dominant mechanism by which mitral valve stenosis is relieved by this technique, and thus commissural morphology may predict outcome. METHODS: One hundred forty-nine consecutive patients who underwent percutaneous mitral balloon valvotomy at the Mayo Clinic were evaluated retrospectively. The morphology of the mitral valve apparatus on the baseline echocardiograms was scored in blinded manner using a semiquantitative grading system of leaflet thickening, mobility, calcification and subvalvular thickening (Abascal score). Additionally, each of the medial and lateral commissures was graded for the presence or absence of calcification. End points were death, New York Heart Association functional class, repeat percutaneous mitral balloon valvotomy and mitral valve replacement at follow-up. RESULTS: The mean follow-up period was 1.8 years (maximum 7.9 years). Univariate predictors of death and all events combined included age, the use of a double-balloon technique, the presence of calcium in a commissure and the Abascal score, as continuous variables. Patients with an Abascal score < or = 8 showed a trend toward improved survival at 36 months free of death, repeat percutaneous mitral balloon valvotomy or mitral valve replacement (78 +/- 6% vs. 67 +/- 8%, p = 0.07) and free of all events combined (75 +/- 6% vs. 64 +/- 8%, p = 0.07) versus those patients with a score > 8. However, survival at 36 months free of death, repeat percutaneous mitral balloon valvotomy or mitral valve replacement (86 +/- 4% vs. 40 +/- 4%) and free of all events combined (82 +/- 5% vs. 38 +/- 10%) at follow-up was significantly different between patients without commissural calcium and those with commissural calcium (p < 0.001). In a Cox regression model with Abascal score and commissural calcium and their interaction, calcification emerged as the only significant variable (p < 0.01). CONCLUSIONS: The presence of commissural calcium is a strong predictor of outcome after percutaneous mitral balloon valvotomy. Patients with evidence of calcium in a commissure have a lower survival rate and a higher incidence of mitral valve replacement and all end points combined. Thus, the simple presence or absence of commissural calcification assessed by two-dimensional echocardiography can be used to predict outcome.

Calcinosis↗

Papillary fibroelastoma of the mitral valve associated with rheumatic mitral stenosis.

Papillary fibroelastoma of the mitral valve diagnosed and treated in life is extremely rare. There have been eight cases documented so far. We report the first case of a mitral valve papillary fibroelastoma associated with severe rheumatic mitral stenosis and tricuspid regurgitation with stenosis. The tumor arose from the posteromedial papillary muscle of the mitral valve. The mitral valve was replaced after excising the valve with the tumor and the tricuspid valve was repaired. The patient did well and remains asymptomatic.

Echocardiography, Transesophageal↗

Treatment with epoprostenol of pulmonary arterial hypertension following mitral valve replacement for mitral stenosis.

Pulmonary hypertension frequently complicates mitral stenosis. Increased pulmonary artery pressure results from raised left atrial pressure, pulmonary arteriolar constriction, and obliterative changes in the pulmonary vascular bed, and usually responds to surgical relief of mitral stenosis. However, severe pulmonary hypertension may persist after surgical treatment of mitral stenosis. We describe a patient whose severe pulmonary hypertension following mitral valve replacement was treated successfully with continuous intravenous epoprostenol.

Antihypertensive Agents↗