[Myxoma of the pulmonary valve, respectively of the pulmonary artery].
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A neonate with pulmonary atresia and an intact ventricular septum with a tripartite right ventricle was successfully treated by percutaneous balloon dilatation of the pulmonary valve. This was facilitated by previous laser valvotomy with a hot tip Trimedyne laser wire. There were no major complications. Four weeks later the patient was discharged home on no medication with peripheral oxygen saturations of 70% in air.
Pulmonary artery catheter (PAC) is a commonly used monitor in cardiac surgery. Pulmonary valve injury from the balloon of the pulmonary artery catheter is exceedingly rare; its description by transesophageal echocardiography is nonexistent in the literature. A patient is reported who developed a flail anterior pulmonary leaflet several days after successful myectomy for hypertrophic cardiomyopathy. The temporal sequence of PAC use and four transesophageal echocardiography studies over 13 days, with the first three showing normal pulmonary valves, suggested pulmonary valve trauma, most likely from a Swan-Ganz catheter, as the etiology. Partial damage to the pulmonary valve from the original surgery may have predisposed the patient to further damage when the PAC was reintroduced in the intensive care unit.
Two patients with dysplastic pulmonary valves associated with Noonan's syndrome successfully underwent leaflet augmentation with pericardial membrane and annular enlargement with preservation of valve competence. Both patients are doing well at 36 and 37 months, respectively, postoperatively. Echocardiography and clinical assessment showed gradients of 12 and 16 mmHg, respectively, and negligible pulmonary valve insufficiency. The surgical technique is simple and provides an attractive alternative in patients with dysplastic pulmonary valve and small pulmonary orifice and annulus.
Calcification of the pulmonary valve is a uncommon condition. Non invasive diagnostic methods such as M-mode and two dimensional echocardiography have facilitated the identification of vegetations and calcifications on the pulmonary valve. Very few cases of isolated calcification on the pulmonary valve have been reported; a previous lesion of the valve almost always exists. The purpose of this communication is to report two cases with calcification of pulmonary valves without underlying pathology and to highlight the great utility of echocardiography in diagnosis and follow-up of such cases.
Premature opening of the pulmonary valve (opening independent of atrial or ventricular systole) was originally described in a case of sinus of Valsalva rupture into the right atrium. Since that time we have observed five additional cases in which the pulmonary valve opened prematurely. Entities encountered included: 1) constrictive pericarditis; 2) Loeffler's endocarditis; 3) Ebstein's anomaly with tricuspid regurgitation; 4) tricuspid regurgitation following tricuspid valvulectomy, and 5) pulmonary regurgitation accompanied by atrial septal defect. In the first two cases, premature pulmonary valve opening is felt to be due to restriction of diastolic filling of the right ventricle with subsequent early diastolic rise in pressure equalling or exceeding pulmonary artery diastolic pressure. In the latter three cases, the increased volume of blood entering the right ventricle again appeared to result in a rapid rise in initial right ventricular diastolic pressure and to produce premature opening of the pulmonary valve. Premature pulmonary valve opening, therefore, does not appear specific for any particular clinical entity but reflects the relative pressures in the right ventricle and pulmonary artery during diastole.
To clarify the determinants of pulmonary valve (PV) motion in pulmonary hypertension, we examined the correlations among PV echo patterns, the pulmonary artery (PA) flow curve just above the PA orifice and the pulmonary artery-right ventricle (PA-RV) pressure gradient. By constricting the PA, we could produce a variety of PV echo patterns, including midsystolic semiclosure in open-chest dogs. Throughout the experiments, the PV echo pattern and PA flow curve were similar in pattern and timing. When the PV echo showed midsystolic semiclosure with reopening. The PA flow curve showed a transient decrease followed by a transient increase during midsystole. The PA-RV pressure gradient became transiently positive (PA pressure greater than RV pressure) and then negative in midsystole only when the PV echo showed midsystolic semiclosure with reopening. In conclusion, PV motion during systole may be instantaneously determined by PA flow change and the PA-RV pressure gradient during the cardiac cycle in experimental pulmonary hypertension.
Percutaneous pulmonary balloon valvuloplasty (PBV) is a well-established treatment alternative to surgery in many cardiology centers. We described our experience with PBV in 25 adolescent and young adult patients with isolated pulmonary valve stenosis (PVS). Among 20 successful PBVs, there was a significant immediate decrease in right ventricular systolic pressure from 116.9 32.4 mmHg to 60.5 18.7 mmHg (p < 0.0001) and a decrease in transpulmonary valve pressure gradient from 93.5 32.8 mmHg to 33.5 9.7 mmHg (p < 0.0001) was noted. The follow-up period was 1-5 years (mean = 3.2 1.2 years), during which patients were periodically assessed by Doppler echocardiogram. During follow-up, the transpulmonary valve pressure gradient further decreased from 33.5 9.7 mmHg to 18.6 3.4 mmHg (p < 0.0001) mainly due to regression of infundibular hypertrophy. Thus, the study showed excellent short-term and intermediate-term results of PBV.
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Nine cases of imperforate tricuspid valve associated with dysplasia of the right ventricular myocardium, pulmonary valve, and right coronary artery are described. The mean frontal QRS axis of the electrocardiograms did not indicate left axis deviation in seven of the nine. Two-dimensional echocardiograms showed an imperforate tricuspid valve, normally aligned atrioventricular septum, and an irregular-shaped right ventricular cavity with a thin wall. In three cases with absence of the pulmonary valve, pulsed Doppler echocardiograms of the right ventricular outflow tract revealed antegrade flow during systole and retrograde flow during diastole, and selective angiography through a persistent ductus arteriosus showed retrograde filling of the blind-ended right ventricular cavity via the main pulmonary artery. Necropsy examinations showed an imperforate tricuspid valve with a thin-walled aneurysmal right ventricle in all six cases examined. Pulmonary atresia was observed in two cases, and the absence of the pulmonary valve in four. The right coronary artery was hypoplastic in four cases and absent in two. A shunt operation should be carried out in early infancy to prevent hypoxia after closure of the ductus arteriosus.
Balloon valvuloplasty for pulmonary valve stenosis by static dilation is the treatment of choice for the treatment of isolated valvular pulmonary stenosis in the infant, child, and adolescent. The procedure is also effective in neonates; however, the complications of the procedure are significantly higher and the efficacy is less. This article reviews the background, indications, technique, acute and long-term results, and complications of balloon valvuloplasty for pulmonary valve stenosis in the infant, child, and adolescent with pulmonary valve stenosis. The application of this technique in neonates with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum is also discussed. The determinants of a successful outcome in each group are addressed.
The diagnosis of bicuspid pulmonary valve is usually established postmortem, occurring in 7% of patients with pulmonary stenosis and is usually associated to tetralogy of Fallot or ventricular septal defect. The authors report the case of a 14-year-old girl with patent ductus arteriosus in whom 2-D echocardiography disclosed a pulmonary valve formed of two slightly thickened leaflets that opened with the shape of a dome in systole, and moved under the valve ring plane during diastole. A Doppler examination showed a 21 mmHg transvalvar gradient. These characteristic features suggest that 2-D and Doppler echocardiography are the techniques of choice in the diagnosis of bicuspid pulmonary valve.
The choice of a valved conduit or a valve in the right ventricular outflow tract remains a problem. We propose to reconstruct the pulmonary valve inside the patient's own pulmonary trunk by autologous pericardium in tri-semilunar fashion with concomitant formation of the sinus of Valsalva and commissure. This operation was carried out on a 7-year-old girl suffering from Fallot's tetralogy with an absent pulmonary valve. The ventricular septal defect was repaired and the pulmonary valve reconstructed as above. The postoperative cross-sectional echocardiogram, color Doppler echocardiogram and angiogram showed a pliable tri-semilunar valve across the pulmonary outflow tract without regurgitation. It is concluded that firstly, the sinus of Valsalva is indispensable for a stentless semilunar valve to be competent; secondly, reconstruction of the semilunar valve inside the pulmonary trunk by autologous pericardium is recommended for the absent pulmonary valve syndrome, but the long-term fate of this pericardial valve awaits further evaluation.
Pulmonary valve stenosis was relieved by balloon dilatation during cardiac catheterisation on 27 occasions in 23 infants and children aged 7 days to 12 years, median 31 months (three aged less than 2 weeks). Pulmonary valve diameter was estimated by cross sectional echocardiography to assist in the choice of balloon size. Before dilatation the right ventricular systolic pressure ranged from 41 to 190 (median 92) mm Hg and was suprasystemic in 10 patients. There were significant reductions in the ratio of right ventricular to systemic systolic pressure and pulmonary systolic pressure gradients immediately after balloon dilatation. Twelve patients underwent recatheterisation (11 at six months and one at one week after balloon dilatation), which showed further improvement with significant reductions in right ventricular pressure or pulmonary valve gradient or both, particularly in the patients with the least satisfactory initial results. This improvement was attributed to resolution of the obstruction at infundibular level. Repeat pulmonary valve dilatation was successfully performed in four patients who had poor results after initial dilatation. Balloon pulmonary valvotomy appears to provide good short term and medium term relief of pulmonary valve stenosis and may obviate the need for surgery in many cases. An apparently poor immediate haemodynamic result does not preclude a good longer term result.
We experienced a case of a 38-year-old woman with a persistent cough, 24 years after repair of absent pulmonary valve syndrome with anomalous origin of the left pulmonary artery. The right pulmonary artery was massively dilated, thus it caused the compression of the bronchi, which was thought to result in her respiratory symptom. This dilatation of the right pulmonary artery seemed to have progressed because of the following two reasons. The first is the pulmonary hypertension caused by the late reconstruction of the left pulmonary artery. The second is residual pulmonary stenosis and regurgitation after the initial operation without a pulmonary valve insertion. We performed a reoperation consisting of reconstruction of the right ventricular outflow tract using a valved conduit and plication of the right pulmonary artery. Her postoperative course has been without any complications and satisfactory for the past 2 years.
The specific features of turbulent flow that are likely to be damaging to the blood cells and platelets are the stresses which are intrinsic to turbulence, known as Reynolds stresses. These include normal stresses as well as shear stresses. The purpose of this study is to determine the magnitude of the turbulent stresses that may occur during ejection in the vicinity of normal and diseased aortic valves near normal pulmonary valves. Both Reynolds normal stresses and Reynolds shear stresses were calculated from velocities obtained in vitro with a laser Doppler anemometer in the region of two severely stenotic and regurgitant human aortic valves. Reynolds normal stresses were also calculated from velocities obtained with a hot-film anemometer in 21 patients in the region of normal and diseased aortic valves. In seven of these patients, it was calculated in the region of the normal pulmonary valve. The Reynolds normal stress in patients with combined aortic stenosis and insufficiency was prominently higher than in patients with normal valves. In the former, the Reynolds normal stress during ejection transiently reached 18,000 dynes/cm2. This was in the range of the Reynolds normal stress observed in vitro. The Reynolds shear stress measured in vitro transiently reached 11,900 dynes/cm2 during ejection. Because the Reynolds normal stresses in the presence of the severely stenotic and regurgitant valves were comparable in vitro and in patients, it is likely that the Reynolds shear stress in patients is also comparable to values measured in vitro. These values were well above the stresses which, when sustained, have been shown to have a damaging effect upon blood cells and platelets.
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