[A 66-year-old woman with post-operative pulmonary valve stenosis showing spontaneous echo contrast in the right atrium].
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The presence of cyanosis following repair of congenital cardiac defects may result from several different mechanisms. We report two patients in whom early postoperative arterial hypoxemia manifested by cyanosis was caused by right-to-left interatrial shunting. Two-dimensional contrast echocardiography correctly identified the site and direction of shunting, leading to surgical correction of the complication.
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OBJECTIVE: To assess the efficacy of arterial duct angioplasty in maintaining adequate ductal patency in neonates with critical pulmonary valve stenosis. PATIENTS: Two neonates presenting with cyanosis due to critical pulmonary valve stenosis with severe right ventricular hypoplasia underwent percutaneous balloon dilatation of the pulmonary valve. Despite successful dilatation, both remained cyanosed while receiving prostaglandin E2 infusions. RESULTS: Angioplasty of the arterial duct resulted in increased arterial oxygen saturations. Adequate arterial duct patency was maintained for three months during which time right ventricular dimensions increased enough to allow sufficient pulmonary circulation without the duct. CONCLUSIONS: Arterial duct angioplasty is an effective additional treatment for patients with critical pulmonary valve stenosis whose right ventricle is too small to provide adequate forward flow after balloon dilatation of the valve. Arterial duct angioplasty sustains adequate patency of the duct while the right ventricle grows sufficiently to undertake the pulmonary circulation.
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.
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The adult with congenital heart disease who undergoes cardiac catheterization at the present time is most likely to have complex heart disease and is left with clinically important sequelae or residual defects, ventricular dysfunction or arrhythmias. Residual defects such as paravalvular leaks, coronary fistulas and pulmonary artery narrowings may be corrected with transcatheter techniques. Patients with simple forms of congenital heart disease (for example, atrial septal defect, patent ductus arteriosus, aortic valve stenosis, pulmonary valve stenosis) will go to the catheterization laboratory for treatment, not diagnosis. Certain lesions previously considered benign (for example, patent foramen ovale) may require definitive interventional therapy to reduce the risk of stroke from paradoxic embolism.
Balloon dilatation in infants with severe pulmonary valve stenosis may not be a straightforward procedure once the arterial duct has closed. Balloon dilatation was attempted in three neonates and infants. In an 11 week old infant hypotension and bradycardia developed shortly after a 5 French end hole catheter was passed through the severely stenosed pulmonary valve. An emergency Waterston shunt was subsequently performed, but he died three days later. After this experience the technique was modified so that progressively larger balloons were used for dilatation in two infants, aged one and three weeks, with severe pulmonary valve stenosis in whom the arterial duct had closed. It was successful in both.
Balloon pulmonary valvuloplasty (BPV) was attempted in 38 cases of congenital pulmonary valve stenosis. It was effective and was done without complication in 36 cases, however it was not effective in two cases of pulmonary valve dysplasia. The balloon used was 20-50% larger in diameter than the pulmonary valve annulus. In the seven cases in which the transvalvular pressure gradient was above 100 mmHg on cardiac catheterization, right ventriculography demonstrated that the functional obstruction of the right ventricular outflow tract increased immediately after BPV, however it subsequently improved at one year after the procedure. At between one and three months after BPV, two-dimensional echocardiography demonstrated that the morphological obstruction to the right ventricular outflow tract had diminished. In two cases, localized right ventricular septal hypertrophy with severe pulmonary valve stenosis was observed by two-dimensional echocardiography and right ventriculography, and persisted at one year. In all cases, two-dimensional echocardiography and angiography demonstrated doming pulmonary valves with valve stenosis, which was diminished by BPV. The pulmonary valve morphology was observed by two-dimensional echocardiography in three cases following BPV. In one case, partial relief of pulmonary valve obstruction was seen to be due to commissural splitting and in the other two, to tearing of cusp tissue. Inspection of the pulmonary valve at operation was made in one case who underwent elective surgery for repair of an atrial septal defect which was associated with pulmonary valve stenosis. It demonstrated partial relief of pulmonary valve stenosis by tearing of cusp tissue. In conclusion, BPV is as effective a treatment for congenital pulmonary valve stenosis as open valvulotomy. In our follow-up study, it has shown no apparent complications. The transient obstruction in the right ventricular outflow tract immediately after BPV improved within one month and improved further after three months and one year. However, in the more long-standing cases the localized right ventricular septal hypertrophy persisted and these cases will require, a long term follow-up.
Although an uncommon lesion, when pulmonary stenosis is considered, pulmonary artery and right ventricular pressures should be assessed simultaneously on two-catheter pullback to appreciate the precise location of pulmonary-right ventricular pressure gradients. The case examples demonstrate that the peripheral pulmonic stenosis can mimic pulmonary valve stenosis and that pulmonary artery insufficiency may be difficult to delineate on pressure alone (as is often the case with the hemodynamics of aortic insufficiency). These hemodynamic tracings are complemented by the echocardiographic and angiographic characterization of pulmonic valve lesions. Conduction defects or ventricular hypertrophy can affect the right ventricular pressure tracing and either delay or increase the timing of pressure rise and decline depending on the conduction disturbance and abnormality of myocardial contraction.
We describe our experience in 6 cases with multiple congenital heart defects treated by percutaneous intervention. Their age ranged from 2.3 to 10 years (mean 6.1), with follow-up from 1 to 84 months (mean 28.8). Two cases had coarctation of the aorta (AC) and persistent ductus arteriosus (PDA). Two patients had pulmonary valve stenosis (PVS) and PDA, one case with aortic stenosis (AE) and PDA and one case with AC, mitral stenosis and subaortic stenosis (Shone's Syndrome). Ductus arteriosus was occluded in all patients with Gianturco coils or Rashkind occluder. The valvular gradient post balloon decreased in cases with AC from 46 to 9 mmHg, with PVS from 110 to 10 mmHg and with AE from 40 to 14 mmHg. In a 8 year old boy with Shone's syndrome, we performed angioplasty of aortic coarctation, mitral valvuloplasty with Inoue catheter. He was referred to surgery for subaortic repair stenosis. All are asymptomatic at follow-up. In conclusion; percutaneous intervention is possible in patients with multiple congenital heart defects.
Percutaneous transluminal balloon catheterization was performed in 38 patients, age 2 to 38 years (mean 13) between October, 1985 and May, 1987. Nineteen with pulmonary valve stenosis (PVS), three with small atrial septal defect (ASD) and one with ventricular septal defect (VSD); six with aortic valve stenosis (AVS); eight with Coarctation of the aorta (CoAo), in two with VSD and one with small ductus arteriosus; and four with rheumatic mitral stenosis (RMS). In PVS the peak systolic gradient (PSG) decreased significantly immediately after the procedure (92 +/- 40 vs 20 +/- 19 mmHg P less than 0.01). Nine patients were evaluated three months and seven one year later and no significant change occurred (19 +/- 11 and 20 +/- 19 mm Hg). The right ventricular systolic pressure (RVSP) and the ratio RVSP/left ventricular systolic pressure (LVSP) decreased immediately after the procedure (114 +/- 37 vs 69 +/- 36 mmHg P less than 0.15, and 0.98 +/- 0.29 vs 0.62 +/- 0.36, P less than 0.15). Three months later the RVSP and ratio RVSP/LVSP decreased more (57 +/- 16 mmHg, P less than 0.05, and 0.47 +/- 0.18, P less than 0.05) and one year later (54 +/- 27 mm Hg, P less than 0.05 and 0.46 +/- 0.24, P less than 0.05). In one case we repeated de dilatation one year later for restenosis. In CoAo PSG decreased immediately after dilation (59 +/- 21 vs 26 +/- 18 mmHg, P less than 0.1). Three months later the gradient was 38 +/- 20 mmHg. One patient was sent to surgery for significant residual gradient and one was dilated again 4 months after the first dilatation. One case was evaluated at cardiac catheterization one year later with gradient of 28 mmHg and blood pressure and femoral pulses were normal. In AVS PSG decrease immediately in all (91 +/- 25 vs 37 +/- 22 mmHg, P less than 0.02). Recatheterization in 4 patients three months later showed improvement in two. Two cases were sent to surgery for significant residual gradient. In these two patients we did not performed a dilatation because one had thrombosis in the puncture site and in the other we suspect subvalvular obstruction. The other two patients have shown clinical, echocardiographic and radiographic improvement.(ABSTRACT TRUNCATED AT 400 WORDS)
BACKGROUND: Immediate and long-term results after balloon dilatation of pulmonary valve stenosis in our unit. METHODS AND PATIENTS: All 111 patients (1 day-18 years) who have had balloon dilatation of a pulmonary valvar stenosis between 12/1987 and 8/1997 were divided into 4 groups: Typical valvar pulmonary stenosis (group A; n = 78), stenosis with dysplastic pulmonary valve (group B; n = 10), critical pulmonary stenosis (group C; n = 16) and pulmonary atresia after transcatheter or operative opening of the valve (group D; n = 7). Patients with pulmonary stenosis and complex congenital heart disease were excluded. RESULTS: The average systolic transvalvular gradient was reduced from 68.5 to 27.2 mmHg (60%) immediately after balloon dilatation. After a follow up of 48.8 +/- 37 months 101 patients could be reevaluated. In group A (n = 69 at FU) and C (n = 16 at FU) 81% showed a systolic transvalvular gradient < 30 mmHg after one and 83% (A) respective 94% (C) after two balloon dilatations. In group B (n = 9 at FU) 44% exhibited a systolic gradient < 30 mmHg after one and 56% after two balloon dilatations. In group D (n = 7 at FU) 57% showed a systolic gradient < 30 mmHg with no further improvement by a second dilatation. Over all, 80% of our patients could be treated sufficiently by transcatheter means. The rate of major complications was 7.3% with no lasting residuals at follow up and no deaths. CONCLUSION: Balloon dilatation of the pulmonary valve is secure and effective. Best results are obtained in patients with typical pulmonary valve stenosis and in newborns and infants with critical pulmonary valve stenosis.
The response to intravenous administration of propranolol hydrochloride was studied in 24 children undergoing operation for isolated or complex right ventricular outflow tract obstruction. Ten had pulmonary valve stenosis, 4 had pulmonary valve stenosis plus ventricular septal defect, 8 had tetralogy of Fallot, and 2 had complex lesions. Propranolol (0.01 to 0.2 mg per kilogram of body weight) was given to patients with a residual right ventricular to left ventricular systolic pressure ratio greater than 0.75. Four children did not respond to propranolol and required further surgical intervention. Twenty patients responded, 2 of whom died. Fifteen were restudied by cardiac catheterization 3 weeks to 27 months postoperatively. The right ventricle-pulmonary artery gradient was 25 mm Hg or less in 13 patients. We conclude that a small dose of propranolol given intraoperatively can identify patients in whom a reduction in the right ventricle-pulmonary artery gradient to acceptable levels will occur in the months following operation. This may reduce the need for placement of a subannular or transannular patch in some patients.
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OBJECTIVES: Percutaneous balloon valvotomy was studied retrospectively in newborns with critical pulmonary valve stenosis or atresia to assess its potential role as an alternative therapy to operation. BACKGROUND: Severe right ventricular outflow tract obstructions are life-threatening conditions requiring prostaglandin infusion immediately after birth and then relief of the valvular obstruction. To avoid surgical hazards at this age, it would be useful to extend to newborns the balloon valvotomy so effective in older patients. METHODS: Ninety-seven newborns (82 with critical pulmonary valve stenosis, 15 with atresia) underwent balloon valvotomy, provided that they had a well developed right ventricle, including an infundibulum close to the pulmonary artery. In patients with atresia, the outflow tract membrane had to be perforated with a wire needle or a radiofrequency probe. RESULTS: Balloon valvotomy could be performed in 81 patients and was effective in 77. It caused 3 fatal and 16 nonfatal complications. Ten patients with persistent poor right ventricular compliance despite an effective valvotomy required a surgical shunt. Among the 81 patients in whom the procedure could be performed, right ventricular surgery was avoided in 5 (55%) of the 9 patients with atresia (95% confidence interval [CI] 28% to 80%) and 55 (76%) of the 72 patients with stenosis (95% CI 66% to 86%) at the end of the follow-up period (9.7 years). CONCLUSIONS: Balloon pulmonary valvotomy is not always feasible in newborns, but it is relatively safe and effective and should be considered a valid alternative to operation.
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