Clinicopathologic conference: interauricular septal defect with calcific pulmonary stenosis, dilatation of the pulmonary artery.
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BACKGROUND: Carcinoid heart disease typically results in pulmonary stenosis and tricuspid incompetence. Percutaneous balloon dilatation is an effective treatment for congenital pulmonary stenosis and has been applied successfully to tricuspid stenosis caused by carcinoid heart disease. The value of balloon dilatation of the pulmonary valve in carcinoid pulmonary stenosis was assessed. METHODS: Two patients with severe congestive heart failure secondary to carcinoid heart disease and with documented pulmonary stenosis had balloon dilatation of the pulmonary valve. In both cases tricuspid regurgitation was also present together with reduced cardiac output. RESULTS: The procedure was technically successful in both patients. One patient experienced symptomatic benefit for two months and the other experienced no improvement. Both patients subsequently required combined tricuspid and pulmonary valve replacement from which good results and symptomatic improvement were obtained. CONCLUSION: Though balloon dilatation of the pulmonary valve is technically feasible it is unlikely to provide useful palliation in carcinoid heart disease. Valve surgery should be considered in patients in whom the malignancy is controlled but carcinoid heart disease is producing drug resistant congestive heart failure.
A patient with acquired pulmonary stenosis due to lymphocytic lymphoma is presented. Three further patients with acquired pulmonary stenosis have been seen in our department in the past 15 years. A review of published cases reveals that there are more causes of this condition than have previously been enumerated.
The double outlet right ventricle is a rare malformation, and its surgical correction has been well defined. The authors present seven cases of double outflow of the right ventricle and stenosis of the pulmonary outflow. If the technical problems of those variants with a subaortic septal defect seem to have been overcome, those with a subpulmonary ventricular septal defect present a much more difficult problem. Of the seven cases presented, the authors report the death of one patient who had a right ventricle with a double outlet associated with pulmonary stenosis and a sub-pulmonary ventricular septal defect. The post-operative course of the other 6 patients, who had a subaortic ventricular septal defect, was simple. One patient suffered secondary dehiscence of the repaired septal defect, and was reoperated on. The maximum follow-up period has been 7 years.
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To evaluate the differences in patch-materials used to reconstruct the pulmonary artery in arterial switch operation for transposition of the great arteries, we compared mid-term results in 50 consecutive survivors who underwent arterial switch operation. In 35 patients (XP-group), the pulmonary artery was reconstructed using a glutaraldehyde-treated heterologous pericardial patch, while in 15 patients (AP-group) it was reconstructed using a fresh autologous pericardial patch. A W-shaped patch was used in 14 patients of the XP-group. In 21 patients of the XP-group and all those of the AP-group, a square patch was used. The mean length of follow-up has been 94.1 +/- 38.1 months after surgery in XP-group, and 16.7 +/- 11.8 months in AP-group, respectively. Four patients in XP-group required balloon angioplasty for pulmonary stenosis and 5 patients in XP-group underwent reoperation, but no patients in AP-group required balloon angioplasty or reoperation for pulmonary stenosis. The risk factors influencing postoperative pulmonary stenosis (sex, age at surgery, preliminary pulmonary artery banding, patch shape and material) were analyzed by multiple regression analysis. The patch material (heterologous patch) was the only identifiable risk factor for pulmonary stenosis. These data suggest that pulmonary artery reconstruction with an autologous pericardial patch may be effective to prevent postoperative pulmonary stenosis, although the long-term prognosis remains unknown.
Acquired pulmonary stenosis was diagnosed in a 53 year old woman presenting with the unusual feature of an oral murmur. Mediastinotomy showed extensive malignant tissue surrounding the pulmonary artery, although a mediastinal mass was not evident on chest radiographs. Histological examination confirmed a pleomorphic adenocarcinoma, which has not previously been reported as causing acquired pulmonary stenosis.
The authors made diagnosis of valvar pulmonary stenosis in a 1-day old neonate. The severe stenosis was treated by means of pulmonary balloon valvuloplasty at the age of 4 days. The initial suprasystemic pressure in the right ventricle decreased to normal after the procedure. Transluminal balloon valvuloplasty is an effective therapeutic method for severe and critical valvar pulmonary stenosis and casually may serve as an alternative of surgical treatment. This case was the first successful procedure of pulmonary balloon valvuloplasty in Hungary at neonatal age.
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OBJECTIVE: Evaluation of the preliminary results of stent implantation as a new technique of intervention cardiology to treat (residual) stenosis of pulmonary arteries in congenital heart disease. DESIGN: Descriptive. SETTING: Children's Heart Centre, University Hospital Nijmegen, the Netherlands. METHODS: Data about physical condition, heart catheterization/angiography and lung perfusion scan were collected. Special attention was paid to the lung perfusion scan as a potential parameter of evaluation. RESULTS: The physical condition was not a useful parameter to judge the result of the pulmonary intravascular stenting, due to a simultaneously present important pulmonary valve insufficiency in most cases. Angiography showed a good result immediately after the procedure in all cases, except in one with kinking of the stent. The increase of lung perfusion could be confirmed by lung scintigraphy in most cases. The relative perfusion of the affected lung increased from 22.7% (SD: 10.8) to 38.6% (12.3) (p < or = 0.001). CONCLUSION: Percutaneous implantation of endovascular stents in pulmonary artery branch stenosis is a welcome alternative to (reconstructive) cardiac surgery. Objective quantification of this stenting by lung perfusion scintigraphy, a technique without patient discomfort, is possible.
It is unclear whether pulmonary stenosis with intact ventricular septum is a secondary cardiac malformation. We report an infant with pulmonary stenosis (diagnosed by fetal echocardiography) with progressive obstruction in late gestation who presented with increasing transvalvular pressure gradients (15 mm Hg at 22 weeks' gestation to 47 mm Hg at 35 weeks). The tricuspid/mitral valve annulus ratio decreased from 1.25 at 24 weeks' gestation to 0.96 at 33 weeks. At 38 weeks' gestation, a male infant weighing 3,524 g, with Apgar scores of 9 and 9 at 1 and 5 minutes, respectively, was delivered by cesarean section. Critical pulmonary stenosis was confirmed by postnatal catheterization. These findings support the postulation that pulmonary stenosis is a progressive disorder. After percutaneous balloon dilatation, the transvalvular pressure gradient decreased and the right ventricular cavity increased gradually. The transvalvular pressure gradient had decreased to 15 mm Hg and the tricuspid/mitral valve annulus ratio was 0.93 at the age of 2 years.
Although balloon dilation of valvular pulmonary stenosis is established in infants and children, the techniques for and results of balloon dilation in neonates with critical pulmonary stenosis remain largely unreported. Since January 1, 1985, six successive neonates with critical pulmonary stenosis (aged 1 to 6 days) underwent attempted balloon dilation. Each was cyanotic and three of the six were on prostaglandin E1 therapy and three required tracheal intubation and ventilation. All had suprasystemic right ventricular pressures (mean 122.8 +/- 6.8 mm Hg). After hemodynamic evaluation and right ventricular angiography, the valve was crossed in five patients, and was first dilated with a low profile, 5 or 6 mm diameter, 2 cm long balloon. At least one more balloon was used in each patient, the largest being 95 to 133% of the diameter of the pulmonary valve anulus. The anulus size was 6.8 +/- 1.1 mm and the largest balloon size used was 6 to 10 mm. Right ventricular pressure decreased to nearly systemic level or less in five of five patients (58.8 +/- 6.7 mm Hg). Pressure gradients, measured in four infants, were 7, 12, 16 and 35 mm Hg, respectively, but were unreliable indicators of obstruction because of a patent ductus arteriosus. The five patients were discharged 3 to 8 days after balloon dilation. All are currently symptom free 10.6 +/- 11.7 months later, and all but one are believed clinically to have mild obstruction. Complications included iliac vein occlusion (n = 1) and complete right bundle branch block (n = 1). Although follow-up has been brief, neonates with critical pulmonary stenosis can safely undergo balloon dilation, usually with good short-term results.
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