[A neonate with the heart in the right hemithorax]. / Een pasgeborene met het hart in de rechter thoraxhelft.

Three neonates presented with the heart in the right hemithorax: a newborn girl with a chest deformity and secondary dextrocardia (Poland sequence), a prematurely born girl with uncomplicated Kartagener's syndrome, and a newborn boy with cyanosis and the heart in the right hemithorax, in whom a surgical correction was indicated. A diagnosis ofdextrocardia should include the differentiation between primary and secondary dextrocardia. Secondary dextrocardia, or dextroposition, is the result of chest deformities, diaphragmatic defects, severe lung disease, or the presence of a mass displacing the heart from its normal position, usually without any cardiac abnormalities. In primary dextrocardia, the position of the organs in the abdomen is important for the differential diagnosis and the prognosis. The incidence of congenital cardiac malformations in primary dextrocardia varies from 10%, in situs inversus totalis, up to 90% in solitary dextrocardia.

[Percutaneous closure of a perimembraneous ventricular septal defect: the first 4 patients in The Netherlands]. / Percutane sluiting van een perimembraneus ventrikelseptumdefect; de eerste 4 Nederlandse patiënten.

In four female patients, aged 30, 10, 8 and 2 years and 9 months (the latter with Down's syndrome), all with a perimembranous ventricular septal defect (VSD), percutaneous closure of the VSD was carried out using an Amplatzer endoprosthesis. These were the first 4 patients to undergo this procedure in The Netherlands. In 3 of the patients the procedure was without complications and complete occlusion of the defects was achieved. The last patient developed a left anterior hemiblock after implantation of the device and one week later an intermittent second degree atrioventricular block was detected, for which a pacemaker was implanted. A few days later the atrioventricular conduction time returned to normal. There was complete occlusion of the defect. Percutaneous closure of a perimembranous VSD using an Amplatzer prosthesis is a promising technique with good short-term results. In a selected group of patients this new technique can replace surgical treatment.

Severe myocardial ischaemia during mask induction of anaesthesia in an infant with unknown critical supravalvular aortic stenosis.

Congenital supravalvular aortic stenosis is an uncommon type of aortic obstruction. When critical, it represents an extreme variant of outflow tract obstruction with increased risk of cardiovascular instability during exercise or anaesthesia. We present a case of severe myocardial ischaemia during induction of anaesthesia with sevoflurane in a 3-month baby with a presumed diagnosis of valvular aortic stenosis for which a percutaneous balloon dilatation of the aortic valve was scheduled.

Multicore myopathy with restrictive cardiomyopathy.

A 10-y-old girl is presented who suffered mild muscular weakness and exercise intolerance from the age of 1 y onwards, with progression appearing from the age of about 8 y. Multicore myopathy and restrictive cardiomyopathy were diagnosed. Literature concerning the coexistence of multicore myopathy and cardiomyopathy is reviewed.

Surgical treatment of a fistula between the right pulmonary artery and the left atrium: presentation of two cases and review of literature.

OBJECTIVE: A direct communication between the pulmonary artery and the left atrium is a rare anomaly. On the basis of two cases of our own and a literature review of 49 cases, we focus on clinical presentation, anatomy, diagnosis, and the role of surgery. METHODS: Two cases of a fistula between the right pulmonary artery and the left atrium are described in a girl of 4 years and a boy of 15 years. Both presented with unexplained cyanosis. Diagnosis was made on echocardiography and angiography. The fistula was ligated using extracorporeal circulation in the first case and not in the second case. RESULTS: The surgical results were successful with resolution of the cyanosis. CONCLUSIONS: In newborns, urgent surgery may be necessary. In other patients, early elective surgical correction should be performed to prevent complications, especially systemic and cerebral emboli, cerebral abscesses, and rupture of aneurysmal fistulas. Complete cure can be achieved by ligation and possible division or by intracardiac repair.

[Chronic central cyanosis in children; always an indication for further diagnosis]. / Chronische centrale cyanose bij kinderen; altijd een reden voor nadere diagnostiek.

A girl and a boy, both aged 4 years, had displayed a blue discolouration of the skin for several years. In the girl, electrocardiography and roentgenography of the chest revealed no abnormalities; in the boy, the cardiac murmur was attributed to an insignificant ventricular septal defect. Further examinations were performed only when the children developed sleeping problems and decrease of exercise tolerance, respectively. In both, a right-left shunt was discovered caused by a direct communication between the right pulmonary artery and the left atrium, and tetralogy of Fallot, respectively. Both patients' condition improved after operation. Chronic central cyanosis in a child constitutes an indication for consultation of a paediatric cardiologist.

Pulmonary perfusion after endovascular stenting of pulmonary artery stenosis.

UNLABELLED: Pulmonary artery stenosis is a well-known condition after surgical correction of tetralogy of Fallot. Endovascular stenting of the stenosis is a new technique for correction without surgical intervention. Objective evaluation of the procedure, however, is often hampered by moderate or severe pulmonary valve insufficiency. This disadvantage does not apply to 99mTc-macroaggregates of albumin (MAA) scintigraphy of the lungs. Moreover, quantification can be performed relatively easy. METHODS: Seven patients with surgically corrected tetralogy of Fallot (4 men, 3 women, mean age 15.7 yr, range 5-24 yr) were studied. The mean diameter decrement of a pulmonary artery was 69% +/- 8.7%. Before and after stenting, relative uptake in the left and right lung was assessed after injection of 37-55 MBq 99mTc-MAA. Three patients were studied twice after stenting. RESULTS: In all patients, perfusion of the affected lung increased significantly: before 22.7% +/- 10.8%, after 38.6% +/- 12.3% (p < 0.0001). All patients claimed clinical improvement of their condition after stenting. The perfusion gain did not correlate with the prestenting diameter decrement or with the pressure gradient over the stenosis. Lung uptake in the patients studied twice after stenting was similar between the initial and poststenting study (< or = 2% difference). CONCLUSION: Quantitative 99mTc-MAA lung imaging is a noninvasive technique without patient discomfort that objectively measures the effect of stenting pulmonary artery stenosis. The final outcome of stenting with regard to increment of pulmonary blood flow is not predicted by the severity of the stenosis or the pressure gradient over the stenosis.

[Endovascular stent in the treatment of residual pulmonary stenosis in patients operated on for congenital heart defects; initial results]. / Endovasculaire stent ter behandeling van resterende pulmonalistakstenose bij patiënten geopereerd wegens aangeboren hartafwijking; eerste ervaringen.

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.

Right-sided valvular regurgitation in normal children determined by combined colour-coded and continuous-wave Doppler echocardiography.

Using Doppler echocardiography, the prevalence of tricuspid and pulmonary valve regurgitation was determined prospectively in 173 normal children, aged 8.3 +/- 2.7 (range 5-14) years. Pulmonary regurgitation was defined as a red-yellow or mosaic coloured regurgitant flow, continuing to end-diastole with continuous-wave Doppler. It was found in 84% of the children. Tricuspid regurgitation was defined as a blue-green or mosaic coloured regurgitant flow from the tricuspid valve into the right atrium lasting > 0.5 systole, as determined by continuous-wave Doppler. Tricuspid regurgitation was present in only 8% of the children. Tricuspid regurgitation flow of very short duration, considered to be due to valve closure, was found in 75%. No effect of age, presence of a vibratory innocent heart murmur or gender on the prevalence of right-sided valvular regurgitation could be demonstrated. All regurgitations were haemodynamically insignificant. Thus right-sided valvular regurgitation in normal schoolchildren is a normal physiological finding with relatively high prevalence. In the absence of functional reasons for these regurgitations and in the absence of structural pulmonary or tricuspid valve disease, these signals should be considered physiological in order to avoid iatrogenic heart disease.

Influence of dialysate exchange on cardiac left ventricular function in children treated with CAPD.

The influence of peritoneal dialysate exchange on the ventricular function in 11 children on continuous ambulatory peritoneal dialysis (CAPD) therapy was studied before and after instillation dialysate. Systolic blood pressure, pulse rate, and echocardiographical data (shortening fraction and pre-ejection period/left ventricle ejection time-ratio) were obtained before and after instillation. There were no differences present with respect to the measured parameters after the abdomen was filled. It is concluded that in children treated with CAPD the exchange of normal volumes of dialysate has no influence on the function of the left ventricle.

[Detection of ventricular septal defects using pulsed Doppler echocardiography]. / Met pulsed Doppler echocardiografie op zoek naar het ventrikel septum defect.

Introduction of Echo-Doppler methods has changed diagnostic procedures in case of a VSD. Case reports of four children are described. In future E/D-examination is to be expected highly valuable in evaluation VSD severity, while heart catheterisation will be less necessary.

[Perinatal course of fetal supraventricular tachycardia; case report and literature review]. / Het perinatale beloop van de foetale supraventriculaire tachycardie; casuïstiek en literatuur overzicht.

Perinatal outcome of fetal supraventricular tachycardia. Report of 5 cases and review of the literature. Fetal supraventricular tachycardia (fsVT) is a rare cardiac arrhythmia and is associated with a mortality of 12%. The last decade fsVT has been detected more frequently probably on account of routine fetal monitoring. The outcome of 108 previously reported cases has been reviewed and 5 cases are added. Congestive heart-failure due to fsVT was seen quite frequently. Delivery by caesarean section was performed in 33% of fsVT-cases. Treatment of the condition before and after birth is discussed; digitalis is the drug of choice. A guide-line for delivery is suggested. fsVT should be listed among the possible causes of hydrops foetalis. On request a complete list of references concerning fetal supraventricular tachycardia is available from the authors.

Idiopathic arterial calcification of infancy.

A description is given of a 15-month-old girl with idiopathic arterial calcifications, detected during life time by X-ray. Radiological examination revealed calcifications in medium-sized arteries, histopathological examination showed distinct abnormalities of small vessels. The patient also had a metageria-like outward and retarded mental and motor development with myolysis. No cardiac failure was present.