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Can the technique for Doppler estimate of pulmonary stenosis gradient be simplified?

Although Doppler echocardiography has been demonstrated to accurately predict the pressure drop across the pulmonary valve in patients with pulmonary valve stenosis, prior reports have stressed the need to correct for beam-flow intercept angles, to use simultaneous imaging, and to utilize the subcostal approach. The purpose of this study was to determine the accuracy of estimating the pressure drop in pulmonary stenosis patients by means of nonimaging Doppler applied without angle correction from precordial examination. Pressure drop estimated by Doppler was compared to that measured by strain gauge manometry at catheterization. Data for 39 patients (21 simultaneous measurements; 18 nonsimultaneous) were evaluated. Results for the entire group showed a good correlation (r = 0.94; SEE = 7.9 mm Hg). The correlation for simultaneous measurement improved somewhat (r = 0.95; SEE = 5.9), but the difference was not significant. Comparison of the slope and intercept of data of this study to those of prior studies, which advocated more complex methodology, indicated that results were essentially similar and that use of the additional steps did not confer a significantly improved result. We conclude that the simplified methodology utilized in this study provides accurate Doppler estimates of pressure gradient in patients with pulmonary stenosis.

Adolescent↗

Punch pulmonary valvectomy: another alternative to treat critical pulmonary stenosis.

For neonates with severe valvar stenosis, or valvar pulmonary atresia with an imperforate pulmonary valve, we present a simple but effective closed procedure using a specially designed valvectomy punch. Seven neonates, who were not suitable for any type of transcatheter procedure, were treated. There were two late deaths, neither directly related to the operation; 4 patients are developing well. This approach using the valvectomy punch is a fast, safe, and effective procedure.

Cardiac Surgical Procedures↗

P wave dispersion is increased in pulmonary stenosis.

AIM: The right atrium pressure load is increased in pulmonary stenosis (PS) that is a congenital anomaly and this changes the electrophysiological characteristics of the atria. However, there is not enough data on the issue of P wave dispersion (PWD) in PS. METHODS: Forty-two patients diagnosed as having valvular PS with echocardiography and 33 completely healthy individuals as the control group were included in the study. P wave duration, p wave maximum (p max) and p minimum (p min) were calculated from resting electrocardiography (ECG) obtained at the rate of 50 mm/sec. P wave dispersion was derived by subtracting p min from p max. The mean pressure gradient (MPG) at the pulmonary valve, structure of the valve and diameters of the right and left atria were measured with echocardiography. The data from two groups were compared with the Mann-Whitney U test and correlation analysis was performed with the Pearson correlation technique. RESULTS: There wasn't any statistically significance in the comparison of age, left atrial diameter and p min between two groups. While the MPG at the pulmonary valve was 43.11 +/- 18.8 mmHg in PS patients, it was 8.4 +/- 4.5 mmHg in the control group. While p max was 107.1 +/- 11.5 in PS group, it was 98.2 +/- 5.1 in control group (p=0.01), PWD was 40.4 +/- 1.2 in PS group, and 27.2 +/- 9.3 in the control group (p=0.01). Moreover, while the diameter of the right atrium in PS group was greater than that of the control group, (38.7 +/- 3.9 vs 30.2 +/- 2.5, p=0.02). We detected a correlation between PWD and pressure gradient in regression analysis. CONCLUSION: P wave dispersion and p max are increased in PS. While PWD was correlated with the pressure gradient that is the degree of narrowing, it was not correlated with the diameters of the right and left atria.

Journal Article↗

[Calcification of the tricuspid annulus in association with pulmonary stenosis: report of a case].

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.

Calcinosis↗

[Pulmonary stenosis with tricuspid insufficiency in the child].

The authors report 3 cases of pulmonary stenosis with an intact interventricular septum and tricuspid incompetence. The tricuspid regurgitation was corrected in all 3 cases. After recalling the clinical features, they emphasize the importance of seeking for this condition and correcting it in every case if the immediate and long-term results are to be satisfactory.

Child↗

Morphologic and hemodynamic consequences after percutaneous balloon valvotomy for neonatal pulmonary stenosis: medium-term follow-up.

OBJECTIVES: This study sought to evaluate ventricular and valvular morphologic changes, hemodynamic consequences and clinical outcomes of pulmonary balloon valvotomy performed in the neonatal period. BACKGROUND: Previous studies support percutaneous balloon valvotomy as the management option of choice for infants and children with valvular pulmonary stenosis. Less information is available to define the impact of valvotomy on the clinical course when performed in the neonatal period. METHODS: Patient records, catheterization data, cineangiograms and selected echocardiograms (initial and most recent studies) of 37 consecutive neonates undergoing attempted balloon dilation were reviewed. RESULTS: Dilation was accomplished in 35 (94%) of 37 attempts. Immediately after dilation, the transvalvular peak to peak systolic gradient decreased from 60 +/- 22 mm Hg (mean +/- SD, range 20 to 100) to 11 +/- 10 mm Hg (range 0 to 45) (p < 0.0001), and the right ventricular/aortic systolic pressure ratio decreased from 1.25 +/- 0.43 (range 0.5 to 2.6) to 0.66 +/- 0.22 (range 0.2 to 1) (p < 0.0001). Oxygen saturation measured by percutaneous oximetry increased from 80 +/- 7% to 92 +/- 4% (p < 0.0001). Three patients died (8%), and two required repeat balloon dilation. At the follow-up visit (median 31 months, range 6 months to 8 years), the estimated peak instantaneous Doppler gradient was 15 +/- 9 mm Hg (range 6 to 36). Thickening of valve leaflets, initially present in 93% of patients, was found in only 4%, and leaflet mobility improved in all. Hypoplasia of the right ventricle, initially present in 31%, was found in only 4% at the latest evaluation. Pulmonary annulus diameter Z score increased from -3 +/- 1.0 to 0 +/- 0.1 (p < 0.0001). Freedom from reintervention was 90%, 84% and 84% at 1, 2 and 8 years, respectively. CONCLUSIONS: These data support the application of balloon valvotomy as the initial intervention in the treatment algorithm for neonates with critical pulmonary valve stenosis. Medium-term follow-up observations demonstrate sustained hemodynamic relief and support maturation of the right ventricle and pulmonary valve annulus, with the expectation of a good long-term outcome.

Algorithms↗

The role of beta blocker in the post operative low output state following adequate valvotomy in cases of pure valvar pulmonary stenosis (clinical experience).

The role of infundibular resection in cases of pure valvar pulmonary stenosis is much debated. Some advocate aggressive approach especially in cases of supra systemic right ventricular pressures. Some consider it unnecessary, and feel that it is secondary hypertrophy which will regress in due course following a successful valvotomy. But all agree that the dynamic right ventricular outflow obstruction plays a very crucial role in the immediate post operative period which can cause significant morbidity and mortality. We advocate the use of beta blocker to relieve the infundibular spasm in the immediate post operative period, thereby relieving the dynamic outflow obstruction. We herewith present two cases to exemplify this.

Adolescent↗

Pulmonary stenosis and impaired myocardial function.

Two young Black female patients with pulmonary valve stenosis and intact ventricular septa are presented in protracted congestive cardiac failure with severe tricuspid insufficiency and, in one, atrial fibrillation. Right ventricular systolic dysfunction was manifested by peak systolic pressures below systemic level, raised end-diastolic pressures and low cardiac output, but without right-to-left shunt. These findings are in strong contrast to those found in most patients with pulmonary stenosis of long standing, where persistent impairment of right ventricular function is diastolic with a high end-diastolic pressure and reversal of an interatrial shunt which result from poor right ventricular compliance. Evidence of left ventricular dysfunction was also present in both cases. Protracted heart failure in these patients is believed to have been the result of coincidental cardiomyopathy in a racial group highly predisposed to this disorder. A diagnostic appreciation of this phenomenon is important in the evaluation of heart disease in the Black, since cardiomyopathy may modify or even mask the features of the underlying disorder.

Adolescent↗

[Noonan syndrome associated with atrial septal defect, ventricular septal defect and pulmonary stenosis--a case report].

A 10 year old girl with diagnosis of Noonan syndrome was admitted to our hospital because of easy fatiguability and cardiac murmur. She had a peculiar face with hypertelorism, saddle nose, webbed neck and kyphosis. Chromosomal analysis demonstrated normal 46 XX pattern. Associated cardiac anomalies in this case were atrial septal defect, ventricular septal defect and pulmonary stenosis caused by pulmonary valvular dysplasia. The cardiac anomalies were corrected successfully and postoperative clinical course was uneventful.

Child↗

[Leopard syndrome and pulmonary subvalvular stenosis].

Pulmonary valve stenosis is the most frequent congenital heart disease associated with Leopard syndrome, an inheritable condition with a low incidence that we should be aware of and diagnose early. We describe the evolution of a patient with Leopard Syndrome and pulmonary subvalvular stenosis, from birth to the surgical treatment.

Child↗