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Biomedical subjects

J Apitz

Publications and source records attributed to J Apitz.

At least 55 records · Page 3Linked to original sources

[Combination of ventricular septal defect with unilateral pulmonary artery stenoses and contralateral pulmonary hypertension].

The clinical features and hemodynamics of the triad ventricular septal defect, unilateral stenoses of the pulmonary artery and contralateral pulmonary hypertension are illustrated by means of 2 case histories. At the time of cardiac catheterization the discovery of severe pulmonary vascular disease of the left lung with inoperable multiple peripheral stenoses of the right pulmonary artery in the first patient, a 4-year old girl, meant that corrective surgery could no longer be undertaken. The second patient, a 10-month old infant with embryofetal alcohol syndrome, suffered from peripheral and two central stenoses of the right pulmonary artery with a total systolic pressure gradient of 62 mm Hg. In our opinion, if the contralateral pulmonary vascular bed has to cope largely single-handed with the increased cardiac output of the right ventricle as a result of additional unilateral pulmonary artery stenosis, the hemodynamic impact of a ventricular septal defect is considerably increased and the development of pulmonary vascular disease accelerated. Since there are strong indications that unilateral pulmonary artery stenosis has more than a mere mechanical effect causing regulative (humorally?, neurogenically?) pulmonary hypertension due also to increased resistance, we are in favour of early closure of the ventricular septal defect and additionally, if technically possible, of an angioplasty or a bypass operation of the stenosis. Since, to the best of our knowledge, an association between proved pulmonary artery stenoses and embryofetal alcohol syndrome has not yet been reported, we feel that our findings are worth sharing.

Child↗

[Aneurysm of the left ventricle in childhood. A contribution on classification and a review of the literature].

Two cases of left ventricular aneurysm in children offer the opportunity of classifying protrusions of the left ventricle into (a) embryonically malformed contractile diverticula and (b) aneurysms, which may be subvalvular, paraventricular, or apical in location. Numerous etiologies have been proposed, but none of these are supported by our examples. Clinical findings on an apical aneurysm of unknown etiology are presented. While in the case of the diverticulum surgical resection is strongly indicated, in the case of aneurysm conservative treatment may often be adequate.

Diagnosis, Differential↗

Endomyocardial biopsy in infants and children: technique; indications and results.

By modification of a miniaturized technique for transcatheter endomyocardial biopsy, it was possible to insert a bioptome through a 6 French catheter in 15 infants and children aged 40 days to 17 years and weighing 3.5 to 55kg. Eight right and 7 left ventricular biopsies were performed, and the specimens were examined by light and electron microscopy. In one child we failed to obtain a specimen. There were no complications. Diagnoses to be either proved or excluded were hypertrophic or dilated cardiomyopathy, endocardial fibroelastosis, glycogen and lipid storage disease, tumours and myocarditis. Morphological examination was diagnostic in 2 cases, helpful in 10 cases and of no help in 2 cases. by this technique it is possible to prove endomyocardial diseases with a high degree of reliability in infants and children without increasing risk or stress.

Adolescent↗

Primary rhabdomyosarcoma of the heart.

A primary rhabdomyosarcoma of the heart observed in a 3-week-old infant, provides an opportunity to review this rare clinical entity; 61 reported cases are collated. The more common sites are right ventricle, left atrium, and right atrium; the tumour may protrude into the cavity and cause obstruction. Echocardiography can demonstrate the tumour, which may also been seen as a filling defect on angiography. Primary heart tumours seldom occur, especially in infancy and childhood. According to Van der Hauwaert, the most common cardiac tumour in infancy is the rhabdomyoma. No primary malignant tumour was seen in 15 European centres within a 5-year period.

Angiocardiography↗

[Clinico-histologic-morphometric correlations in pulmonary arteries among patients with heart malformations ].

To improve the evaluation of grades of pulmonary vascular lesions in congenital heart malformations, we studied the extent to which there is a measurable relationship between pressure conditions in the pulmonary circulation and the area of the media in small and medium-sized pulmonary arteries, and the possibility of a correlation between the subjective evaluation of grades of hypertensive pulmonary vascular lesions according to Heath and Edwards (1958). Material and methods. The lungs in 68 children (mean age, 22.7 months) with congenital malformations of the heart or great vessels resulting in pulmonary hypertension were examined. The lungs were fixed in a 4% formalin solution passed via the trachea under a constant filling pressure of 150 cm H2O. Peripheral as well as central tissue was removed from all lobes of the lungs; the specimens were stained with Elastica-van Gieson. The extent of hypertensive pulmonary vasculopathy was staged, without knowledge of the pressure conditions, according to the grading system developed by Heath and Edwards. The diameter and the area of all muscular arteries with diameters smaller or larger than 100 micron were measured using a semiautomatic measurement device for quantitative morphometric analysis (MOP/AM 01). Cardiac catheter values were available for all cases. The quotient of systolic pressures in the pulmonary artery and the aorta was taken as the measure of hemodynamic conditions in the pulmonary circulation. The Pearson-Bravais correlation coefficient (r) was computed from the respective area quotient and the corresponding pressure values. In addition, the coefficient of determination (r2) and regression functions were determined. Results. A linear correlation (r = 0.70) exists between the pressure quotient (Psyst. pulm. art./ Psyst. aorta) and the vessel area quotient (media area/total area). The correlation is expressed by the following functions: x = 1.89 y - 0.08 y = 0.26 x + 0.24 Using the Heath and Edwards grading, the following frequencies were obtained: Grade 0: 21, Grade I: 7, Grade II: 16, Grade III: 15, Grade IV: 6, and 3 children were undeterminable. No relationship exists between the grades and certain heart malformations. In spite of the fact that hypertensive vasculopathy becomes progressively more severe as the child grows older, we found four cases of Grade IV in children under the age of one. On the whole, a comparison of the measured area quotients and the Heath and Edwards grading showed a good correlation. Although we considered only pressure quotients and no other hemodynamic parameters, conclusions can be drawn about the operability of congenital heart malformations based on these findings. Using measured pressure values, the morphologic state of the pulmonary arteries can be approximately evaluated.

Aorta↗

[Haemodynamics of blood supply to the pulmonary or body circulation via a patent ductus arteriosus in infants (author's transl)].

To control the cardiopulmonary functioning of high risk newborn with congenital heart defects the authors apply computergenerated recording of vital parameters predominantly measured by non-invasive methods. It was found that periodic variations of the arterial partial oxygen pressure occur with occlusion of the outflow from both the right and the left ventricle, associated with patent ductus arteriosus. The authors interpret these periodic variations as intermittent contractions of the arterial duct. The pathophysiology of this phenomenon is discussed.

Blood Circulation↗

Primary heart tumors in infancy and childhood. Report of four cases and review of literature.

Primary heart tumors are rare. The findings in 4 children with heart tumors are reported: a rhabdomyosarcoma, a rhabdomyoma, a fibroma and a myxoma. The symptoms depend upon the location (intrapericardial, intramural or intracavitary). ECG and X-ray provide no essential diagnostic information; echocardiography can give a decisive diagnostic indication in parietal and intracavitary tumors. Surgery provides a specimen for histology, but the tumor cannot always be removed. The prognosis, therefore, depends upon the result of surgery, but malignant tumors, mostly being diagnosed too late, have a very bad prognosis.

Arrhythmias, Cardiac↗

[Computer aid in pediatric intensive care (author's transl)].

The application of computerized intensive care in pediatrics is described. After adaption of commercial software and the development of several processing units, it was possible to control the circulatory and pulmonary function, reliably and continuously almost only by noninvasive methods. The graphical drawing of the trend of the controlled vital parameters will give better informations about the patients condition than the indication or registration of the momentary values. Moreover typical trend patterns of the measured parameters are hereby recognizable, so that critical situations will be detected, treated and - if possible - abolished earlier than in the past.

Computers↗

[Transposition of the great arteries: results of treatment in the years 1967--1980 (author's transl)].

To demonstrate how the prognosis for a newborn with transposition of the great arteries (TGA) has improved, the clinical data of 121 children diagnosed between January 1967 and December 1979 were reviewed. 104 patients had an initial balloon atrial septostomy; 34 infants needed palliative operations (resection of ductus or coarctation, banding of the pulmonary artery and aortic-pulmonary anastomosis). Corrective surgery was according to Mustard (43), Senning (7), Rastelli (5) or an anatomic correction (2). 80% of the infants survived the first month of life. 25% died between the first month and corrective surgery. The mortality of the intraatrial switch operations decreased from 28% before 1976 to 12% in the following years. After Mustard's operation cardiac rhythm disturbances occurred in 39.4%, hemodynamic complications in 24,2% of the cases. Neurological complications developed more frequently in children with simple TGA (27.3%) than in children with combined TGA (18.2%). Owing to improvement of conservative and operative treatment the 2-years survival rate increased from 48.5% between 1967--1970 to 62.5% between 1977--1979.

Arrhythmias, Cardiac↗

[Neurological complications in children with congenital cyanotic heart disease (author's transl)].

Figures of incidence and risk factors of neurological complications (NC) endangering children with cyanotic congenital heart disease (CCHD) are very different. We examined 308 children with CCHD over an 8 years' period finding 39 cases (= 12,7) of NC. There were 5 hypoxic attacks and 3 brain abscesses. In 31 patients a cerebro-vascular accident was proven or probable. 56% of NC occured during the first year of life, most frequently in children with simple TGA. Mortality was not influenced by NC. Children without NC, surviving the first year of life represent the control group for comparison of the following hematologic parameters: erythrocytes, hematocrit, MCHC, hemoglobin, O2-saturation, platelets count. Only the last two were significantly lower in patients with NC, but no parameter allows to predict NC. The hypothesis of hypochromic, microcytic anemia as essential pathogenetic factor for cerebro-vascular accidents has no base in our material.

Age Factors↗

[Echocardiographic findings and function analysis of the left ventricle in infants with endocardial fibroelastosis (author's transl)].

7 infants with the clinical picture and the typical hemodynamic and angiocardiographic findings of endocardial fibroelastosis were studied echocardiographically. The echocardiograms were digitized and analyzed by the method of Gibson and Brown and compared with those of 8 normal infants. The echoes of EFE-patients showed a nearly twice as large LV-diameter. The mitral valve was displaced posteriorly, the septal motion in 4 of them abnormal. The shortening fraction was significantly reduced, the LPEP/LVET quotient increased. The comparison of LPEP with the Q to mitral valve closing interval revealed a considerable prolongation of isovolumetric contraction time. The result of computer analysis was a decrease of peak Vcf and maximum lengthening rate. The echocardiographic pattern is typical but not pathognomonic, it gives no prognostic information.

Echocardiography↗

Clinical and angio- and echocardiographic findings in 45 children with mitral valve prolapse syndrome.

45 patients with an age of 3--15.5 yr are reported. Only 9 of them showed symptoms (palpitations, dyspnea, fatigue). The murmur was often uncharacteristic, only 42% had a click. ECG changes could be found in 60%. The associated cardiac lesions and the angiographic findings are represented. Correlating to an angiocardiographically LVOTO we found in 13 patients (28.9%) the echocardiographic signs of an ASH with a septum/LV posterior wall quotient of 1.45 +/- 0.15. We conclude, that myocardial disease is one pathogenetic factor in the MVPS.

Adolescent↗

[Diffuse, telangiectatic arteriovenous anastomosis in the lung as the cause of cyanosis in an infant with persistent ductus arteriosus (author's transl)].

Compared to the discrete, solitary or multiple pulmonary arteriovenous fistulas, the type of diffuse telangiectatic arteriovenous anastomoses occurs less frequently. Pathogenetically an association with the morbus Rendu-Osler-Weber and with chronic liver diseases is discussed. Based on the literature, and an own case with an additional patent ductus arteriosus, the clinical features are described. Diagnostic results of pulmonary angiography are less helpful than the dynamic radionuclide perfusion scintigraphy which is of great value in following up the disease. There is no successful therapy.

Arteriovenous Fistula↗

[Pulmonary hypertension and histological findings before and years after surgical treatment of congenital heart disease (author's transl)].

The coincidence of pulmonary hypertension and pulmonary vascular disease was investigated in 186 infants and children with congenital heart disease and left to right shunts. The correlation between pulmonary hypertension and pulmonary vasculopathy was highly significant. Hemodynamic pulmonary hypertension is completely suppressed by early pulmonary artery banding or total correction. Even pulmonary vascular alterations can be reversed by these measures.

Blood Vessels↗

[Peripheral stenoses of the pulmonary arteries: possible causes and syndromic relationships (author's transl)].

The catheterization results and additional malformations and diseases in 20 infants and children with pulmonary artery stenosis are reported. In 17 cases additional cardiovascular malformations were found. In 15 cases extracardiac malformations or developmental retardation were observed. Peripheral stenoses of the pulmonary arteries are frequently established in rubella embryopathy, in the syndrome of supravalvular aortic stenosis, in Down s syndrome, in different types of nanism, in thalidomide embryopathy and in the socalled arteriohepatic dysplasia (Watson and Miller) associated with hypoplasia of the bile ducts. In 3 cases an arteriophepatic dysplasia was confirmed by angiocardiography and biopsy. Coarctations of pulmonary arteries rarely need operative interventions.

Adolescent↗