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We present a case of left pulmonary artery aneurysm with pulmonary valve stenosis. The aneurysm was excluded and the valve replaced by the implantation of a pulmonary artery allograft. The short-term follow-up (20 months) is promising.
The optimal method for balloon dilation valvuloplasty of congenital pulmonary valve stenosis has not been established. The cardiac damage produced by using a balloon the same size as the pulmonary anulus has not been described. The use of balloons that are larger than the anulus or of a dynamic dilation technique may enhance gradient reduction, but their added risks are not known. The pulmonary valve anuli of 14 normal newborn lambs were dilated with angioplasty balloons 20% smaller to 90% larger than the anulus; in 3 dilations, a dynamic technique was used, consisting of withdrawal of the fully inflated balloon from the anulus into the body of the right ventricle. Twelve lambs were killed immediately after the procedure and 2 were killed later, and detailed gross anatomic and microscopic observations of the heart were made. These observations support the following conclusions: (1) Dilation of the pulmonary valve anulus with overlarge balloons is "clinically" well tolerated in normal newborn lambs. (2) The major damage to the heart from any balloon is not the pulmonary anulus, but to the right ventricular outflow tract and free wall, with mural hemorrhages of varying size. (3) Trauma is minor if the balloon is 30% larger than the anulus, but is considerable when the balloon is 50% larger; (4) Trauma is worst subjacent to the proximal end of the balloon, so that longer balloons may cause greater damage; and (5) Resolution of acute right ventricular hemorrhage occurs with small, patchy areas of fibrosis.
In 12 months since March, 1975, 25 of 27 patients with tetralogy of Fallot have had corrective operations without ventriculotomy. Infundibular obstructions were excised and ventricular septal defects were closed through a right artiotomy with retraction of the anterior leaflet of the tricuspid valve. Pulmonary valve stenosis was relieved through a pulmonary arteriotomy. In five patients the pulmonary annulus was patched 0.5 to 1.5 cm. into the right ventricle. Immediately after repair peak right ventricular-pulmonary arterial systolic pressure difference averaged 17 mm. Hg and ranged between zero and 40 mm. Hg. Cardiac indices averaged 2.85 L. per square meter per minute 4 hours after operation. All but two patients developed right bundle branch block. One patient with severe pulmonary hypertension died. Fourteen patients have been recatheterized. Right ventricular-pulmonary peak systolic pressure differences ranged between zero and 45 mm. Hg (mean, 22). Cineangiograms show contraction of the free right ventricular wall during systole. Transatrial repair of tetralogy of Fallot is feasible technically in many patients, avoids muscle necrosis and coronary arterial injury, and improves cardiac output in the immediate postoperative period.
This report describes the results and follow-up of 27 patients undergoing pulmonary balloon valvuloplasty at Children's Hospital of Wisconsin where this procedure has been performed since 1985. The valvuloplasty data at catheterization as well as follow-up echocardiograms when available were retrospectively reviewed. For 26 patients, the transvalvar gradient changed from a mean of 72 mm Hg + 5.2 (SEM) to 29 mm Hg + 4.4 (SEM) (p 0.00001) a mean gradient reduction of 58%. No major complications were reported and no patient went on to surgical valvotomy. Follow up echocardiograms were available for 26 patients. Average estimated transvalvar pressure gradients by continuous wave Doppler echocardiography were 28 mm Hg at one day (13 patients), 20 mm Hg at mean 1.5 months (18 patients), 23 mm Hg at mean 8.8 months (12 patients), and 19 mm Hg at mean 26 months (8 patients). Percutaneous balloon valvuloplasty gives good results in nearly all patients with pulmonary valve stenosis.
A baby presented in cardiac failure with cyanosis on the first day of life. Investigations demonstrated severe pulmonary valve stenosis and duct-dependent coarctation of the aorta. This combination is impossible to explain using recognised embryological theories.
Over the last 10 years, several advances have been made in paediatric cardiology and cardiac surgery. However, the major developments have been in non-surgical attempts at transcatheter treatment of congenital heart disease. Initially these concerned some simple defects such as pulmonary valve stenosis but lately much more high-risk and complex defects have been treated.
The increasing popularity of Doppler echocardiography in recent years has stemmed from the combination of Doppler with two-dimensional echocardiography, and from the Doppler capability for flow velocity measurement. In the past decade, Doppler instrumentation has evolved from single probe A- and M-mode systems with non-quantitative Doppler output, into multi-faceted instruments combining two-dimensional echo, M-mode echo, with both pulsed and continuous wave Doppler. In the field of pediatric cardiology, quantitative Doppler applications have proven most useful in noninvasive measurement of the severity of obstruction at aortic and pulmonic valves, and across pulmonary artery bands. High accuracy is enjoyed using either continuous wave, or high pulse repetition pulsed wave, Doppler. Additional quantitative applications include estimation of volume aortic flow (cardiac output), and comparison with volume pulmonic flow (pulmonary to systemic flow ratio). Through evaluation of specific disturbances of blood flow (timing, location, direction, duration), qualitative aspects of Doppler continue to provide sensitive and specific diagnosis of valvular disease, as well as shunt lesions such as atrial and ventricular septal defect, and patent ductus arteriosus. Qualitative Doppler offers 'semi-quantitation' of the severity of lesions based on demonstration of breadth and extent of the individual flow disturbances. The addition of Doppler flow information to the information available from conventional M-mode and two-dimensional echo has led to a comprehensive cardiac ultrasound capability. Information previously available only from invasive study (severity of gradient, shunt, regurgitation) is now available noninvasively; the impact upon current and long-term aspects of patient care should prove considerable.
We performed a balloon pulmonary valvuloplasty in a child with Ebstein's anomaly using the standard technique. The procedure reportedly has not been performed in the face of this defect and, although difficult, was safely and successfully accomplished. In patients with Ebstein's anomaly and right ventricular outflow obstruction compounded by pulmonary valve stenosis, application of this technique may delay the need for surgical intervention.
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The technique of normothermic caval inflow occlusion was introduced primarily for relief of pulmonary valve stenosis and continues to be used for this purpose with excellent results. However, the technique may also be applied to a number of other lesions such as aortic valve stenosis in neonates and infants, for placement of outflow patches or valve excision for pulmonary atresia with intact ventricular septum, for atrial septectomy in children with restrictive atrial septum, and for other conditions wherein a period of up to 2 minutes of intracardiac exposure is sufficient. This report examines the early and long-term results in 140 children who underwent normothermic caval inflow occlusion at The Children's Hospital, Boston, Massachusetts, over the past 11 years. Ninety-four children underwent pulmonary valvotomy (early mortality 0%), 21 had aortic valvotomy (19%), 10 with pulmonary atresia and intact ventricular septum underwent various procedures (50%), 11 had atrial septectomy (9%), and there were four miscellaneous procedures (50%). Apart from avoiding many of the potential complications of cardiopulmonary bypass, inflow occlusion has provided as good or better short-term and long-term results as those obtained with cardiopulmonary bypass, particularly in infants and neonates, and also has proved more cost effective.
Severe pulmonary valve stenosis in association with a large atrial septal defect is uncommon. Simultaneous percutaneous closure of the atrial septal defect and pulmonary valvuloplasty appears to be an attractive modality when compared to patients with these two lesions being subjected to surgery or being treated percutaneously at different sittings. We encountered two cases, both with significant pulmonary valvular stenosis associated with atrial septal defect (fossa ovalis type). Both lesions were successfully treated percutaneously in the same sitting. Pulmonary valvuloplasty was done initially and the atrial septal defect closed later to minimize catheter manipulation after device implantation.
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Twenty-seven infants with ventricular septal defects and in cardiac failure were followed regularly under medical treatment for an average period of 17 months. The diagnosis was proved in all cases by cardiac catheterization.At the end of the follow-up period, the patients could be classified as follows: Sixteen patients showed no change in their cardiovascular status, and six had signs of pulmonary infundibular stenosis. One child had died from bronchopneumonia. Another was classified as having probable functional closure of the defect. Finally, three other children, because of failure of medical treatment, underwent banding of the pulmonary artery. One died during the operation.It is concluded that the great majority of patients with ventricular septal defects and cardiac failure can be carried successfully under medical management until the age at which corrective surgery is feasible. Palliative procedures which in themselves carry a fairly high mortality risk should be used only as a last resort.
BACKGROUND: Though acute and follow-up benefits of pulmonary valve balloon dilatation (PVBD) for pulmonic valve stenosis are well known, the late course of residual gradients at individual valvular and infundibular levels is not well described. Furthermore, the factors influencing this late course have not been studied. MATERIALS AND METHODS: We assessed the transpulmonary gradients by echo-Doppler in 96 patients (61 male, mean age 10.7 years) at a mean follow-up interval of 58.8+/-32.1 months (minimum 2 years) following PVBD. The patients were divided into three groups based on the residual gradients at valvular and infundibular levels immediately following PVBD as assessed by pull-back of an end-hole catheter across the right ventricular outflow tract: Group A with minor pressure gradients at both valvular and infundibular levels of less than 30 mmHg (n=60, 62.5%), Group B with predominantly infundibular gradients of more than 30 mmHg with a valvular gradient of less than 30 mmHg (n=27, 28.1%), and Group C with a residual valvular gradient of greater than 30 mmHg irrespective of the infundibular gradient (n=9, 9.4%). Demographic characteristics, hemodynamic parameters and procedural variables were correlated with the change in gradient at follow-up (late fall) and with long-term results. RESULTS: The mean follow-up trans-pulmonary gradient was 20+/-14 mmHg which was significantly lower than that immediately post-PVBD (43+/-32 mmHg), P<0.001. The late fall (mean 24+/-29, range -55 to 110 mmHg) varied widely depending upon the acute result group: patients in Groups A and B showed significant late fall of 9+/-12 mmHg (P<0.05 for follow-up gradient compared to that following PVBD) and 58+/-31 mmHg (P<0.0001), respectively, while patients in Group C showed an insignificant late fall of 14+/-37 mmHg (P=0.21). In particular, each one of the patients in Group B showed decrease in trans-pulmonary gradients. On multivariate analysis, the extent of infundibular gradient emerged as the most important predictor of late fall (coefficient of determination 75%, P<0.0001). Patients who underwent PVBD at less than 2 years of age had a significantly greater late fall (41+/-33 mmHg) as compared to older patients (22+/-25 mmHg), P<0.05. A sub-optimal long-term result (transpulmonary gradient >25 mmHg, n=24) was significantly related to older age (P<0.001), dysplastic valve morphology (P=0.002), greater baseline trans-pulmonary gradients (P<0.001) and higher post-PVBD gradients (P=0.04). CONCLUSIONS: The long-term course of patients following PVBD depends upon the site and magnitude of the residual gradients. Even high residual infundibular gradients show marked reduction at follow-up, especially in infancy.
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In 1967, Watson described children in three families with pulmonary valve stenosis, dull intelligence, short stature, and cafe-au-lait spots. We present the case of an undiagnosed adult with coronary artery disease, the Watson syndrome characteristics, and a pulmonary artery aneurysm secondary to pulmonic stenosis.
This report describes a case involving an 82 year old patient with mild renal insufficiency, severe pulmonary valve stenosis (PVS), severe anterior descending artery stenosis and complete atrioventricular block, who successfully underwent, in a single session, coronary angioplasty and a stent implant, pulmonary valvotomy and a permanent pacemaker implant.