Pulmonary valvulotomy and infundibulotomy in infants.
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BACKGROUND: Dilatation of the ascending aorta in aortic stenosis may be partly explained by intrinsic wall structure changes, but the relative contribution of altered hemodynamics is unclear. The aim of this study was to assess the association between ascending aortic dimensions and valve stenosis severity. METHODS AND RESULTS: An analysis of echocardiographic examinations was conducted in 296 patients with aortic stenosis (179 males, mean age 71 years), 57 with bicuspid and 239 with tricuspid aortic valve, mean transaortic gradient 43+/-20 mmHg, and not more than moderate aortic regurgitation. Aortic dimensions at the level of annulus, sinuses of Valsalva, sinotubular junction and proximal ascending aorta were measured. Only height (p<0.001), degree of aortic regurgitation (p<0.01) and presence of bicuspid aortic valve (p<0.001) were independent predictors of ascending aortic dimensions. CONCLUSIONS: An independent association between aortic pressure gradients and proximal ascending aortic dimensions was not observed in patients with bicuspid or tricuspid aortic valve stenosis. Therefore, the poststenotic dilatation of the ascending aorta is not explained by aortic stenosis severity itself. Possible nonhemodynamic causes deserve detailed study at the time of diagnosis.
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A 51-year-old woman was admitted to our hospital because of palpitation, dyspnea and anterior chest discomfort. She had had a heart murmur since childhood. On physical examination, a rough grade 6/6 ejection systolic murmur was maximally audible at the second left intercostal space with a systolic thrill. The chest X-ray film showed the cardiothoracic ratio of 54% and the electrocardiogram demonstrated right ventricular hypertrophy. Calcification of the tricuspid annulus was recognized by chest X-ray films (anteroposterior and lateral views), M-mode and cross-sectional echocardiograms and the computed tomogram (CT) of the chest. Cardiac catheterization revealed the presence of valvular pulmonary stenosis and atrial septal defect. These findings were confirmed by surgical operation. Since the first description of Paktovskii in 1960, nine cases of calcification of the tricuspid annulus were reported, but no case has been described in the Japanese literature. Contrary to the mitral annulus, the echocardiographic and computed tomographic features of the calcification of the tricuspid annulus has not been reported previously. In this paper, we reported a middle aged patient with circumferential calcification of the tricuspid annulus in association with severe pulmonary stenosis and atrial septal defect, and the features of the echocardiogram and CT images were illustrated.
We present a 36-year-old woman with a malignant carcinoid syndrome, liver metastases and right heart involvement with severe tricuspid and pulmonic valve stenosis. She was admitted with severe heart failure and poor general status. A combined tricuspid and pulmonic valvuloplasty was performed and she exhibited great clinical improvement that was maintained during the follow-up. We point out the importance of two-dimensional echocardiography in the diagnosis of this pathology and the role of Doppler measurements for the evaluation of the valvuloplasty's results.
A case of severe stenosis of a tricuspid bioprosthesis was successfully dilated in a 13 year old young male with Ebstein's anomaly. The patient had congestive heart failure and developed cardiogenic shock; angiography revealed absence of emptying of the right atrium. Multiple size balloons (#4 to 23) were sequentially introduced with successful bioprosthesis dilation and shock resolution. Tricuspid bioprosthesis can be dilated even in critical conditions and, although a palliative procedure, balloon valvuloplasty can be a lifesaving procedure in patients with cardiogenic shock.
Clinical and morphologic observations are described in 12 patients who underwent simultaneous replacement of the tricuspid, mitral and aortic valves. All 12 patients had mitral stenosis, 10 aortic valve stenosis and 2 pure aortic valve regurgitation; 5 had tricuspid valve stenosis and 7 pure tricuspid valve regurgitation. Of the 10 patients who died within 60 days of triple valve replacement, 7 had the low cardiac output syndrome, which in 4, and possibly 5, of the 7 was attributed to prosthetic aortic valve stenosis. In none of the 12 patients was the ascending aorta dilated, and in the 4 (possibly 5) patients with low cardiac output, the space between the surface of the caged poppet (4 patients) or margins of the tilting disc (1 patient) in the aortic valve position and the aortic endothelium appeared inadequate to allow unobstructed flow despite small-sized prostheses in all but 1 patient. Thus, aortic valve replacement in the setting of triple valve dysfunction is hazardous or potentially so. The relative small sizes of the hearts in these patients also make valve replacement more difficult (and hazardous) compared to hearts with larger ventricles and aortas.
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Thirty-five patients with atrioventricular (AV) discordance underwent repair of major intracardiac defects. Ventricular septal defect (VSD) was the most frequently encountered lesion, present alone or in combination with other lesions in 86% of these patients. Pulmonary stenosis (51%) and tricuspid insufficiency (37%) were the other lesions encountered. The initial operative mortality of 8.6% is approaching that for these same defects when repaired in the patient with normal atrioventricular connections. However, the late mortality of 19% with an average follow-up of 4.4 years is higher than expected in the absence of AV discordance. Reoperation for residual or recurrent defects was required in 9 of the 32 survivors and was associated with a mortality of 33%. Seven of the 9 reoperations were required for tricuspid valve dysfunction. Elective repair of major anomalies in association with AV discordance can be accomplished safely, but these patients require careful long-term follow-up in anticipation of late problems.
Cardiac suction has been previously demonstrated in the normal heart and in cardiac diseases characterized by a normal or reduced end-diastolic ventricular pressure. We report a patient with tight mitral stenosis and severe tricuspid regurgitation who provides the evidence of cardiac suction, despite the presence of increased end-diastolic pressures. The negative diastolic pressures observed in our patient appear to be related to the preservation of a vigorous contraction of both ventricles resulting in small end-systolic volumes. In this setting the elastic potential energy stored in the myocardium during each vigorous contraction is released during diastole, thus creating a negative pressure which sucks blood into the ventricles.
A 62-year-old man was admitted to our hospital because of general malaise. The chest X-ray film showed severe cardiomegaly (CTR = 81%). The electrocardiogram demonstrated absence of P wave in 12-leads and regular junctional rhythm with incomplete right bundle branch block. Echocardiogram and color Doppler led to the diagnosis of mitral stenosis and regurgitation, tricuspid regurgitation with enlarged left and right atrium. Cardiac catheterization study revealed elevation of the pulmonary artery wedge pressure and the right atrial pressure without A wave. In the electrophysiologic study, no atrial electrical activity was recorded in the right atrium and the coronary sinus. However, when atrial pacing was attempted at multiple sites with stimulum strength of 10 V, there was no atrial activity at various sites. We considered that the atrial overload due to combined valvular disease for seventeen years had resulted in total and persistent atrial standstill. In this report, we discussed atrial standstill in 67 cases recorded in the medical literatures in our country.
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This report describes a 5-year-old girl with congenital tricuspid regurgitation associated with an atrial septal defect and peripheral pulmonary stenosis. The girl was diagnosed with the heart murmur at birth and recently developed the cardiomegaly. Cardiac echocardiography and catheterization showed severe tricuspid regurgitation, an atrial septal defect of the secundum type and peripheral pulmonary stenosis. In the operative findings, the tricuspid annulus was dilated to 33 mm in diameter, and leaflets were attached normally to the antomic annulus. There was a large cleft of the anterior leaflet of the tricuspid valve. Suture of the cleft and annuloplasty of the tricuspid valve, suture closure of the atrial septal defect and patch dilatation of peripheral pulmonary stenosis were successfully performed. Including this case, 19 other cases with congenital tricuspid regurgitation undergoing surgery were reported to date.