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Biomedical subjects

J Apitz

Publications and source records attributed to J Apitz.

At least 37 records · Page 2Linked to original sources

Endomyocardial biopsy in infants and children: experience in 60 patients.

In 60 children, aged between 1 month and 22 years (median 3.54 years) and with a body weight of 3-67 kg (median 12.6 kg), transvascular endomyocardial biopsy (EMB) was performed from the right (35 children) or left ventricle (30 children). The specimens were investigated by light and electron microscopy. There were three indications for biopsy: (1) poorly functioning, dilated left ventricle (seven patients with endocardial fibroelastosis, 16 with dilated cardiomyopathy, six from healing/healed or chronic myocarditis); (2) unexplained left ventricular hypertrophy (10 with hypertrophic cardiomyopathy, four with secondary hypertrophy, three with storage diseases); (3) to answer certain questions in eight children (four with hypoxic and two with cytoxic myocardial damage). Retrospectively, there were five nonindicated biopsies. There were no serious complications. Biopsies were diagnostic in 11.7% of cases, helpful in 71.7%, and of no help in 16.6%. Thus even in childhood endomyocardial biopsy is a diagnostic tool which can add useful information on the etiology or pathogenesis of an underlying myocardial disease.

Adolescent↗

[Dynamic magnetic resonance tomography in pediatric cardiology: broadening of diagnostic possibilities by flow-rephasing gradient-echo sequences].

Based on the phase difference method as described by Nayler et al., we developed a gradient-echo sequence, which refocuses flow-related phase shifts. With regard to the higher peak velocity and higher acceleration in infants, we reduced the echo time (TE) to 5 ms. This is effective in rephasing the flow signals even for faster heart rates. Phase shifts are further minimized by reducing the voxel size. The slice thickness down to 2 mm also improves anatomic resolution. A repetition time (TR) of 15 ms provides high temporal resolution for dynamic studies. Modification of the flow-rephasing gradient-echo sequence allows blood flow measurements in the great arteries and the calculation of blood flow volume to assess left and right ventricular stroke volume. This can also be achieved by calculating the ventricular volume from contiguous slices of the whole heart; however, it results in excessive measuring times. Compared with conventional spin-echo techniques, anatomical analysis is improved. The size of atrial septal defects (ASD) can be measured more exactly. Small atrial or ventricular septal defects (VSD), which are not detectable on spin-echo images, are demonstrated on the gradient-echo images and semi-quantitative estimation of the pressure in the right and left ventricle can be made. Pulmonary arteries and veins are clearly differentiated from bronchial structures and are traced to subsegmental levels. Without ECG-gating, contiguous slices of interesting parts of the cardiovascular system can be obtained and successfully used for secondary angiographic reconstructions.

Child↗

[Secondary diseases of the heart muscle and their differential diagnosis in childhood].

Possible causes of specific cardiac muscle disease, diagnosis, follow-up and the therapeutic management are discussed on the basis of a series of cases. 8 out of 30 patients who showed the clinical picture of dilative cardiomyopathy (DCM) were found to have a specific cardiac muscle disease. 4 patients had DCM following adriamycin therapy. Fibromuscular dysplasia with renal hypertension, thalassaemia major with secondary haemosiderosis, long-overlooked and untreated athyroidism each caused one case of dilative specific cardiac muscle disease. Once DCM was preceded by the Kawasaki syndrome for over 2 years. Amongst 47 patients with hypertrophic cardiomyopathy there were two children who had undergone ACTH treatment, 6 children born of diabetic mothers, 4 cases of Pompe's disease, and one patient with hypothyroidism resulting in reversible hypertrophy of the cardiac muscle. Different neurodegenerative diseases were associated with cardiac muscle disease in 4 cases, partly dictating the clinical course. Extremely rare was the development over 6 years of cardiac hypertrophy following a burns injury.

Adolescent↗

Pulmonary hypertensive vasculopathy--no indication for corrective operation of isolated ventricular septum defect in babyhood.

The frequency of surgical closure of a ventricular septum defect (VSD) in the first year of life has risen from 10% to 30% in the last seven years in West Germany. Whereas there was a decrease of mortality in older children, mortality has stagnated at 8% for correction at age 12 months or less (early correction). Development of an irreversible pulmonary hypertensive vasculopathy (PHVP) and recurrent heart failure with growth disorders are given as reasons for operation in the first year of life. In the last six years, we have operated on 31 infants for isolated VSD with pulmonary hypertension (PH) and closed the VSD. In approximately equal preoperative pressure in the two ventricles with a pressure ratio of 0.88 +/- 0.16, there was an immediate fall in pressure in the right ventricle with pressure ratios of 0.43 +/- 0.24. Three patients (9.7%) died perioperatively. In the same period, a primary VSD closure was carried out in 120 children even after over 12 months with three deaths (2.5%). Pulmonary tissue removed in this operation did not show any PHVP in four patients (13%), grades 0 to I and I to II in 10 patients each (total 33%) and a PHVP grade II to III in six patients (20%). This PHVP is capable of full regression. An irreversible PHVP does not develop up to the end of the first year of life in isolated VSD with PH, so that the correction can be safely postponed to the beginning of the second year of life provided that recurrent heart failure with growth disorders does not compel earlier correction.(ABSTRACT TRUNCATED AT 250 WORDS)

Aging↗

[Aneurysm of the vein of Galen as a possible cause of congestive heart failure: a report of 5 new cases].

We report on five newborns with an arterio-venous malformation of the vein of Galen. All newborns were cyanotic and in congestive heart failure without any evidence of congenital heart disease. Congestive heart failure in these cases was mainly due to an almost two-fold increase in cardiac output of approximately 8 l/min/m2 (normal: 4.5 l/min/m2). According to previous reports, mortality is very high in patients with this malformation when becoming symptomatic during infancy, and therapy by surgery or embolization is only successful in 10-30%. While three of our patients died shortly after diagnosis because of untreatable heart failure, the other two were operated on either by subtotal ligation of the draining vein or by ligation of 4 arterial feeders. In the first case secondary thrombosis of the aneurysm occurred and cardiac failure subsided. In the second case a large shunt remained and a balloon-embolization was performed successfully. However, in both patients severe neurologic defects occurred, the severity of which remains to be assessed later since the post-operative observation period is only 2 and 5 months, respectively.

Cerebral Angiography↗

[Special heart diagnosis in the 1st year of life. An analysis of 1,131 infants with congenital heart defects 1972-1983].

In the years 1972-1983, 1131 infants below one year of age have been investigated by means of heart catheterization and angiography, some only by echocardiography,--if necessary--operated and followed for at least one year. Ventricular septal defect was the most frequent congenital heart defect (16.6%), followed by transposition of the great arteries (12.1%), AV-canal (9.1%) and patent ductus arteriosus (8.1%). 58.3% of all patients had more than one lesion, 27.6% had critical lesions, which caused symptoms and had to be cured during the first month of life. 63.3% of patients with critical lesions were male. Clinical diagnosis could be improved considerably by means of echocardiography: in contrast to the years 1972-1974, where only in 49% of patients a clinical diagnosis was known before heart catheterization, this was the case in 73.1% in the years 1981-1983. Additional malformations of other organs were found in 28.6%, 19.9% had a birthweight below 2.5 kg. Concerning the various diagnoses, there were no regional or seasonal differences. The general rate of heart defects is said to be 0.8%, familiarity was 4.5%. Frequency of operations was 64.4%, mortality 37.4%, which could be improved by 10% in comparison of the beginning to the end of the time of observation.

Abnormalities, Multiple↗

[Differential diagnosis and prognosis of primary and post-myocarditis cardiomyopathy in childhood].

It is still difficult to differentiate between the various stages of myocarditis and primary dilated cardiomyopathy (DCM). Hence, we analyzed the history, as well as the laboratory and virological data of 22 children with a dilated, poorly functioning left ventricle, aged 2 months to 16.7 years (m = 4.2 y), on whom we had performed endomyocardial biopsies about 4 months after the beginning of the illness. Specimens were investigated by light and electron microscopy and, in addition immunoserological (n = 15) and immunohistological investigations (n = 7) were performed. On the basis of cellular infiltration in the histological examination we diagnosed resolving/resolved myocarditis in 6 patients and DCM in 15 patients. Previous respiratory infection or sudden onset were found in 40-50% of patients in both groups. Antimyolemmal and antisarcolemmal antibodies showed no preference. Follow-up (3.1 +/- 2.8 years) of the patients showed equal mortality (33% vs. 38%) in the post-myocarditis and DCM group. Complete normalization of all findings in 3 patients of the DCM group makes the classification among the post-myocarditis group probable, which would, then give a lower mortality rate (22% vs. 46%) and a chance of restitution in 55% of cases. In any one specific case all the diagnostic methods do not allow undoubted distinction between both entities. The prognosis of myocarditis seems to be better than that of DCM in childhood.

Adolescent↗

Dilated cardiomyopathy in childhood: problems of diagnosis and long-term follow-up.

Clinical profile and course of 13 infants and children (median age 2 years, range 2 months to 17 years) with dilated cardiomyopathy are presented. Endocardial fibroelastosis and myocarditis were excluded by transvascular endomyocardial biopsy. Elevated cardio-thoracic ratio (mean 0.67), depressed fractional shortening (mean 13%) and cardiac index (mean 2.52 l min-1 m-2) were the indicators of congestive heart failure. During a mean follow-up of 41 months (range 6 to 204 months) five patients died, four remained in a stable condition, four improved. The main complications were rhythm disturbances (23%) and thrombus formation (15%). We could not identify any predictors of survival.

Adolescent↗

[Continuous-wave Doppler velocimeters and blood pressure difference in aortic coarctation. A simultaneous comparative study of 52 children].

Sixty examinations in 52 children with coarctation of the aorta (eight pre- and postoperative studies) were performed to assess the relation of the Doppler derived gradient using the simplified Bernoulli equation (delta p = V2 X 4) with the blood pressure difference simultaneously measured with an automated oscillometer. There was a close correlation (r = 0.89, y = 16.1 + 0.73x, syx = 7.38 mmHg) with significant overestimation of the blood pressure difference in mild stenoses (p less than 0.0001). If the patients are divided in two groups, an operative (group 1: symptomatic patients, n = 24) and a nonoperative group (group 2: asymptomatic patients, in the majority recoarctations, n = 36) it becomes clear that the overestimation is due to the latter. The velocity superior to the coarctation was higher in the second than in the first group (152.1 +/- 31.5 vs. 114.5 +/- 43 cm/s, p less than 0.004) (mean +/- SD). If the prestenotic velocities are taken into account, the correlation is nearly the same (n = 37, r = 0.9, y = 9.4 + 0.73x, syx = 7.3 mmHg) with slight underestimation in high-grade and overestimation in mild coarctations. A pandiastolic increased velocity was found in 79% of the patients in the operative group, but in none of the nonoperative group. The reasons for the overproportional increased jet velocity in group 2 as discussed are that: the peak pressure gradient may exceed the peak-to-peak pressure gradient; the prestenotic velocity must be taken into account, if the modified Bernoulli equation is used; and the collateral vascularization may be substantial.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

[Endomyocardial biopsy in childhood--experiences in 60 pediatric patients].

In 60 pediatric patients, aged from 1 month to 22 years (median 3.54) and a bodyweight of 3 to 67 kg (median 12.6 kg) transvascular endomyocardial biopsy was performed from the right (35 patients) or left ventricle (30 patients). The specimens were investigated by light and electron microscopy. Immune serological investigations were performed in 22 patients, immune histological examinations in eight. There were three indications for biopsy: a. 29 children had a poorly functioning, dilated left ventricle. Of these, seven suffered from endocardial fibroelastosis, 16 from dilated cardiomyopathy, six (plus one control-biopsy) from healing/healed or chronic myocarditis. b. 17 children showed inadequate left ventricular hypertrophy. Of these, ten suffered from HCM, four from secondary hypertrophy, three from storage diseases. c. Various questions were answered in eight children - four with hypoxic, two with cytotoxic myocardial damage. There were five misindications, retrospectively. We observed no serious complications. Evaluation of biopsy revealed diagnostic findings in 11.7%, was helpful in 71.7% and of no help in 16.6%. Hence, even in childhood, endomyocardial biopsy is a diagnostic tool which can contribute useful information on the etiology or pathogenesis of the underlying myocardial disease.

Adolescent↗

[Quantitative evaluation of left heart obstructions (including aortic isthmus stenosis) in children using Doppler echocardiography].

To assess the accuracy of pulsed and continuous-wave Doppler echocardiography (DE) in evaluating pressure drops across left heart outflow obstructions we examined 27 children with valvular aortic stenosis (n = 13), fixed subaortic stenosis (n = 14) and 16 children with coarctation. Doppler examination was performed within 24 hours of cardiac catheterization in 15 patients, in 17 patients 2-42 months (mean 13 months) after catheterization. Simultaneous blood pressure measurements in both upper and lower limbs with an automated oscillometer were taken in 12 cases with coarctation. Using three standard positions (suprasternal, high right parasternal and apical) we found a close Doppler-catheter correlation in patients with aortic and subaortic stenosis (r = 0.94). The correlation in cases with coarctation was poor (r = 0.17) because of one patient with severe stenosis and another with atresia and a huge collateral vascularization, in whom a poststenotic jet could not be located. Surprisingly the postoperative pressure gradient was much overestimated in one of these patients by DE. We conclude that a "multi-channel system" due to collateral vascularization excludes the use of the simplified Bernoulli equation. Apart from these anatomically related false Doppler estimates we found a good correlation (r = 0.90), with slight overestimation in mild stenosis. Thus, Doppler ultrasound provides an accurate noninvasive method for estimating pressure gradients in patients with aortic and subaortic stenosis and to a lesser extent in patients with coarctation. In particular DE is helpful for determining subsequent clinical management.

Aortic Coarctation↗

[Quantitative evaluation of right heart obstructions in children using Doppler echocardiography].

To assess the accuracy of pulsed and continuous-wave Doppler echocardiography (DE) for estimating pressure drops across right ventricular outlet obstruction, we examined 27 children (valvular pulmonary stenosis n = 16, complex congenital heart lesion with prior pulmonary banding n = 11). The Doppler evaluation was performed within 24 hours of cardiac catheterization in 17 patients, in 10 it was done 2-24 months (mean 12.2 months) after catheterization. We found a good correlation (r = 0.93) with slight underestimation of pulmonary valve gradient. Using three windows (left parasternal, suprasternal and subcostal) the ultrasound beam can be better aligned parallel to the excentric jet of a malformed valve than using the left parasternal position alone. In children with complex congenital heart disease and normally related great arteries Doppler ultrasound reliably estimates the gradient across a pulmonary artery band using the left parasternal transducer position (r = 0.92). In patients with malposition of the great arteries the use of multiple windows is necessary. Thus, DE provides an accurate noninvasive method of estimating pressure gradients in patients with right ventricular outflow obstructions and is helpful in determining the subsequent clinical management.

Blood Pressure↗

[Hypertrophic cardiomyopathy in childhood: studies of the clinical picture and course].

30 children with hypertrophic cardiomyopathy and a mean age of 4.9 +/- 4.4 years, 11 infants and 19 children, were followed up for an average of 6.6 +/- 5.6 years. Apart from a systolic murmur in nearly all patients, only a few had symptoms. 7 patients from 5 families had a positive family history. The ECG often showed left ventricular hypertrophy, twice a deep Q wave, and no cases of giant T wave. A subaortic pressure gradient greater than or equal to 40 mm Hg was measured in 6 children, a secondary cardiomyopathy was excluded by biopsy in 8. Associated lesions were coarctation (n = 2) and patent ductus arteriosus (n = 2). 7 children died: 1 infant in congestive heart failure, 3 children suddenly, and 3 postoperatively. The annual mortality rate was 3.5%. Also in childhood, beta-blocker therapy may not prevent sudden cardiac death, nor may operation prevent late complications. In spite of the lack of pressure gradients, the children did not fulfil the criteria of hypertrophic non-obstructive cardiomyopathy.

Cardiac Catheterization↗

[Prognosis of children with congenital tricuspid and pulmonary atresia 1967-83 in comparison with the natural course].

46 Children with tricuspid atresia, 56 with pulmonary atresia and ventricular septal defect (VSD) and 33 with pulmonary atresia and intact ventricular septum were diagnosed and treated at the University's Children's Hospital Tübingen between 1967 and 83. Their age distribution and mortality is compared to 91 autopsy-proven cases of tricuspid atresia, 123 cases of pulmonary atresia with VSD and 41 cases without VSD, reported in the time between 1783 and 1945 showing the natural history of these malformations. Inspite of intensive medical and operative therapy, the results of which are reported in detail, mortality continues to be high: 55% of our patients with tricuspid atresia die within the first year of life, 60-62% within the first 2 years (in the bibliographical collective 64% and 73%), 53-61% of patients with pulmonary atresia and VSD die within the first, 61-68% within the first 2 years (in the bibliographical collective 74% and 78%), and 78% of patients with pulmonary atresia without VSD within the first year, 88% within the first 2 years of life (in the bibliographic collective 95% and 97%).

Child, Preschool↗

[Dilated cardiomyopathy in childhood: intravital differentiation of endocardial fibroelastosis using transvascular endomyocardial biopsy].

Possibly due to the lack of clinical reports concerned with dilated cardiomyopathy in childhood, pediatric cardiologists may inadvertently designate the angiographic finding of a dilated left ventricle as endocardial fibroelastosis. We report historical and noninvasively-obtained data from twelve children, aged seven months to 17 years, with poorly-functioning dilated left ventricles, in whom no differentiation between the two diseases was enabled. Only by means of transvascular endomyocardial biopsies, performed during cardiac catheterization with a 6-F guide-catheter and a Machida bioptome for light and electron microscopal examination, was differentiation achieved among six patients with endocardial fibroelastosis and six with the nonspecific histological findings of muscular hypertrophy, interstitial fibrosis and marked degenerative changes. After exclusion of noxious and inflammatory etiologies, even in childhood, the diagnosis of primary dilated cardiomyopathy must be regarded as established. Since experience previously reported with endocardial fibroelastosis has not been based on histologic documentation of the diagnosis, questions with respect to natural history and prognosis remain unanswered.

Adolescent↗

[Computer-assisted monitoring in intensive care].

Computerized intensive care in children and infants is possible. But it requires: 1. a modification and extension of the commercially available soft-ware, 2. a sampling rate of 2 values/min or more [7,8] and 3. the noninvasive measurement of the parameters, especially for long-time recordings. - The graphical presentation of the parameters for a longer time shows typical patterns, which lead to an earlier recognition of critical situations. Thus earlier therapy becomes possible. Other characteristic patterns no doubt will be discovered by further research.

Blood Gas Analysis↗