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At least 631 records · Page 35Linked to original sources

[Problems posed by percutaneous pulmonary valvuloplasty in children].

Percutaneous valvuloplasty with a balloon catheter was attempted in 41 young patients with pulmonary valve stenosis. There were 9 neonates (1 to 7 days), 6 infants (1.5 to 7 months) and 26 children (16 months to 17 years). The attempt failed in 8 cases, either because the balloon could not be inserted due to intravenous progression problems or to quasi-atresia of the orifice, or because serious accidents (air embolism in one patient, perforation of the infundibulum in another) forced us to abandon the procedure before dilatation. Failures mostly occurred in very young children: 7 out of 15 aged less than 1 year, 1 out of 26 older than 1 year. Dilatation was effective in 33 cases. Among 13 patients who had moderate stenosis with infra- or iso-systemic right ventricular systolic pressure (RVP) (mean: 70 +/- 13 mmHg) and modest ventriculo-pulmonary gradient (49 +/- 12 mmHg), there was only 1 failure due to coexistent biventricular myocardiopathy; success was immediate and complete in 8 cases, partial in 4 cases. Among 20 children presenting with tight stenosis with supra-systemic RVP (164 +/- 39 mmHg) and high ventriculo-pulmonary gradient (140 +/- 43 mmHg), there were 5 failures; success was partial in 8 cases, complete and immediate in 7 cases. Accentuation of the infundibular stenosis after dilatation was a frequent cause of failure, as we have shown by calculating the infundibular "reactivity index" on pre- and post-operative angiocardiograms. In such cases one may hope for spontaneous improvement, as was indeed subsequently observed in 2 of our patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

[Plexiform neurofibroma and basal ganglia anomaly in Watson syndrome].

A 4 year-old boy was referred for diagnostic reevaluation with known pulmonary valve stenosis. Physical examination revealed multiple cafe-au-lait spots, inguinal freckling and on the right side in supraclavicular region a softly, non-painful tumour. The boy showed a mild mental and language retardation. Ultrasound and MRT demonstrated supraclavicular a plexiform neurofibroma and intracranial increased intensity lesions in basal ganglia and mesencephalon. In our patient, we have diagnosed a Watson-Syndrome, the overlap and differences to neurofibromatosis type I is discussed.

Basal Ganglia↗

[Percutaneous bursting of the pulmonary valve].

In a 25-year-old female patient with pulmonary valve stenosis valvuloplasty could be successfully performed using a balloon catheter inserted via the femoral vein. Balloon measurements were 2 cm width and 3 cm length, the required pressure was 5 atm. There was a decrease of the pressure gradient form 60 to 25 mm Hg, systolic right ventricular pressure decreased from 88 to 50 mm Hg. Besides transluminal angioplasty of coronary stenoses and non-surgical occlusion of patent ductus arteriosus thus a further catheter technique for treatment of organic heart disease has become available, being able to replace surgery.

Adult↗

Seizures due to lidocaine toxicity in a child during cardiac catheterization.

A 17-month-old boy developed grand mal seizures secondary to lidocaine toxicity during balloon dilatation of a congenital pulmonary valve stenosis. Lidocaine at 38 mg/kg (nine times the recommended maximum dose of 4.5 mg/kg) was administered during a 90-min period in order to optimize local anesthesia. This resulted in toxic serum lidocaine levels (8.7 mg/L; therapeutic range, 1.5-5 mg/L) at the time of seizures. Caution should be exercised with local anesthetics during invasive cardiac catheterizations. Hypercarbia (which lowers the seizure threshold to local anesthetics) should be avoided and the temptation to exceed the maximum recommended dose resisted.

Anesthesia, Local↗

Pulmonary stenosis.

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Humans↗

[Late results following Rastelli corrective operations in transposition of great vessels].

From 1974 to 1985, 19 patients with transposition of the great arteries, ventricular septal defect and pulmonary valve stenosis or atresia underwent Rastelli correction. The mean age of the patients at operation was 9.7 years. Four patients died on the day of operation (early mortality: 21%). One patient died 7 years later due to dysrhythmias (overall mortality: 26%). Cardiac catheterization was performed on average 1.8 and 6.7 years after the operation. Gradients above 40 mm Hg were registered in 70% of the conduits, 6 years after the operation. Five patients underwent six reoperations for conduit stenosis. The actuarial survival rate was 75%; the event-free survival rate was only 20% after 12 years.

Adolescent↗

Fetal cardiovascular cross-sectional morphology of tetralogy of Fallot in rats.

In order to clarify cross-sectional morphology of tetralogy of Fallot in the fetus, 202 rat fetuses with tetralogy of Fallot induced by maternal administration of bis-diamine were studied using cesarean section, the whole body freezing method and by photographing cross sections of the fetal heart on a freezing microtome. Some characteristic features such as large subaortic ventricular septal defects, aortic overriding over the ventricular septum, aortomitral fibrous continuity, and narrow infundibulum and pulmonary valve were found. Moreover, three subtypes were noticed. In classic tetralogy of Fallot with stenotic infundibulum, the size of the pulmonary arteries was smaller in proportion to the infundibular stenosis. In the second type with absent infundibular septum and severe valvular pulmonary stenosis, the pulmonary arteries were most hypoplastic. In the third type with absent pulmonary valve, the pulmonary arteries were dilated forming aneurysms. In the last type only, ventricles were dilated and grossly hypertrophic.

Animals↗

[Levocardia with ventricular inversion and a single atrium in normal position].

A 12-year child had complete heart block, an abdominal situs inversus with laevocardia. Further investigation demonstrated in addition an inferior vena cava ending into an azygos vein, a shunt at the atrial level and a pulmonary valve stenosis. At operation there was a lone atrium in normal position, a ventricular inversion without transposition of the great vessels. In spite of a corrective operation of the Mustard type, the child died the following day through haemorrhage and low output.

Autopsy↗

Plasma atrial natriuretic peptide in patients with congenital heart diseases.

The plasma level of human alpha-atrial natriuretic peptide was measured in healthy children and patients, 1 month to 15 years of age, with congenital heart diseases. Significant increases were found in patients with a ventricular septal defect, tricuspid valve atresia, patent ductus arteriosus, and atrial septal defect but not in those with pulmonary valve stenosis or tetralogy of Fallot. The levels were significantly higher in children with ventricular septal defects (221 +/- 123 pg/mL) or patent ductus arteriosus (124 +/- 38 pg/mL) than in those with atrial septal defects (65 +/- 42 pg/mL) (P less than .01). The increased levels appeared to be correlated with enlargement of the left atrium (r = .85, P less than .01) but not with the right atrial size or the mean right atrial pressure. They were higher in younger than in older healthy infants, but this age difference did not affect the results. These findings indicate that human alpha-atrial natriuretic peptide is released into the circulation in response to chronic atrial expansion in patients with congenital heart disease and may have an important role in volume homeostasis.

Adolescent↗

Haemangiosarcoma of the pulmonary valve presenting as a pulmonary stenosis. A case report.

Intractable congestive cardiac failure, unexplained arrhythmias, changes in the cardiac silhouette and murmurs which change with position are all features which should arouse suspicion of a cardiac tumour. A case of intracardiac haemangiosarcoma of endothelial origin is discussed. The tumour presented as a pulmonary stenosis and extended into the right ventricular myocardium, causing a right bundle-branch block. At first the tumour seemed to be restricted to the endothelium of the pulmonary vascular bed, but it later infiltrated the interstitium and bronchi, cuasing dyspnoea and haemoptysis. After surgical removal of the tumour and reconstruction of the pulmonary outflow tract, the patient was free of symptoms for 14 months. However, rapid tumour growth in the pulmonary arterial system, lung interstitium and right ventricle subsequently recurred. Deep radiotherapy did not cause the tumour to regress.

Female↗

[Percutaneous intraluminal balloon dilatation of valvular pulmonary stenoses in infancy and childhood. Presentation of results with special reference to balloon size].

34 percutaneous balloon valvuloplasties (BVP), including four repeat procedures, were performed in 30 patients (aged 3 months to 19.4 years, mean 5.8 years) with congenital pulmonary valve stenosis. Out of six failures four were due to dysplastic thickened valves; in two children a significant part of obstruction was on the subvalvular level. After first BVP a significant reduction of the right ventricular outflow tract (RVOT) gradient (mean 58.8 +/- 13.2%) was achieved in 24 of 30 cases. The mean pre-BVP RVOT gradient of 67 +/- 24.4 mm Hg was reduced to 27.2 +/- 10.2 mm Hg. Of 24 patients, 14 had a residual RVOT gradient of 25 mm Hg or less. Of the 24 patients, nine were recatheterized 12 to 24 months later, and a further RVOT gradient reduction (33.4 +/- 9.9 mm Hg to 24.2 +/- 14.5 mm Hg) was observed. In four patients additional RVOT gradient reduction (37.3 +/- 11.6 mm Hg to 18.5 +/- 10.5 mm Hg) was achieved by repeat BVP. Balloon size exceeded valve annulus diameter by up to 58% (mean 22%). No definite correlation between balloon size related to pulmonary valve annulus diameter and magnitude of right ventricular systolic pressure reduction was observed. Even using a maximal balloon size of 158%, no significant complications were noted, not even pulmonary valve insufficiency.

Adolescent↗