[Secondary myocardial hypertrophy in newborn infants and infants without congenital heart defect]. / Sekundäre Myokardhypertrophie bei Neugeborenen und Säuglingen ohne Herzfehler.

Secondary hypertrophic cardiomyopathy (HCM) was diagnosed in 55 newborns and infants without heart disease. The 17 newborn infants of diabetic mothers, 23 infants given ACTH treatment for infantile spasms, 9 ventilated premature infants or babies with bronchopulmonary dysplasia and 6 infants with different underlying diseases comprising this group underwent 2-dimensional echocardiography. In almost all cases echocardiographic evidence of HCM resolved after removal of the exogenous or endogenous causative factor. Myocardial effects of several substances are known and their pathophysiological mechanisms are discussed. Since HCM may precede systemic disease or may often be the first pointer to a triggering agent, further diagnostic procedures are always indicated. Hence, the diagnosis of primary HCM should be made by exclusion.

Insulin-like growth factors and insulin increase the contractility of neonatal rat cardiocytes in vitro.

In the newborn several situations of hyperinsulinism can be associated with myocardial hypertrophy and increased contractility. Insulin and the insulin-like growth factors (IGF) are derived from a common ancestral molecule. Insulin exerts mainly metabolic action, whereas the IGFs promote cell multiplication and differentiation. Using an assay system of cultured neonatal myocardial cells the stimulatory action of insulin and the insulin-like growth factors I and II on myocardial cell contractility was investigated. Spontaneously beating aggregates of myocardial cells were synchronized by an electric impulse generator. Contractility was measured via the amplitude of contraction by an optoelectronic system. Insulin at a concentration of 6,250 and 12,500 microU/ml increased the contractility by 11 and 18%; IGF-I at a concentration of 12 and 25 ng/ml, and IGF-II at a concentration of 25 and 50 ng/ml increased the contractility by 16 and 22%, and 13 and 18%, respectively. Lower concentrations did not provoke a significant increase in contractility. Insulin only in supraphysiological doses increases the contractility of neonatal myocardial rat cells, whereas both insulin-like growth factors act in physiological concentrations. Therefore, during hyperinsulinism insulin may increase myocardial contractility via the IGF receptor and not via the insulin receptor.

Sensitivity, specificity and predictive value of clinical findings, m-mode echocardiography and continuous-wave Doppler sonography in the diagnosis of symptomatic patent ductus arteriosus in preterm infants.

By means of probability analysis we have compared the diagnostic value of clinical symptoms, m-mode echocardiographic measurements and peripheral arterial flow, assessed by continuous-wave Doppler, in preterm infants with symptomatic patent ductus arteriosus (PDA). Data were obtained in 29 infants with PDA and in 29 controls. The most sensitive clinical finding was a hyperactive precordium. Bounding pulses and a heart murmur were absent in 15% and 20%, respectively of the patients with PDA. M-mode echocardiographic measurements were rather specific for the detection of a PDA but less sensitive. Diastolic backflow in the brachial and femoral arteries was present in the majority of patients with PDA and absent in about 67% of the controls. The values in probability analysis, however, were too low to base a therapy on these findings. The highest sensitivity and specificity (100% each) was found for a disturbed cerebral blood flow with absent or retrograde diastolic perfusion estimated by Doppler sonography.

[Hypoplastic left heart with transposition of the great arteries]. / Hypoplastisches Linksherz mit Transposition der grossen Arterien.

Hypoplastic left heart syndrome (HLHS) is a rare congenital cardiovascular malformation with a fatal outcome, despite increasing cardiosurgical therapeutic options. Experiences in three cases are reported which were diagnosed as HLHS in the uncommon combination with transposition of the great arteries (TGA). Pathophysiology, clinical features and diagnostic approaches of the "classic" HLHS and the version with TGA are compared. In contrast to the poor clinical outcome, due to impaired systemic and coronary perfusion in patients with classic HLHS, HLHS with TGA leads to reduced pulmonary perfusion, which can be relieved by aortopulmonary shunt operation. After successful operation, two patients showed normal development during the follow-up period of 18 months and 2 years resp. Because of different therapeutic and prognostic consequences in the two lesions, the necessity to determine topographic relations of great arteries in HLHS is stressed.

[Long-term observation of ventricular extrasystole in children with normal hearts]. / Langzeitbeobachtung bei herzgesunden Kindern mit ventrikulärer Extrasystolie.

UNLABELLED: Between 1971 and 1980 uniform ventricular extrasystoles, classified as benign, were noticed in 47 children with normal hearts. After a mean observation period of 4.4 years 27 of these children, mean age 10.5 years, could be reexamined. This involved physical examination followed by resting and exercise ECG, 24-hour continuous ECG and echocardiography. Of the 27 children, 18 still had extrasystoles having the same morphological characteristics and identical behaviour under exercise as those seen on first examination. Nine children were free of extrasystoles in all ECG examinations. In none of the 27 children were there any complaints between the first and the second examination or signs of degeneration of the extrasystoles into threatening dysrhythmias. CONCLUSION: Uniform extrasystoles disappearing or decreasing on exercise in otherwise healthy children require no treatment even when occurring frequently and persisting over years.

Hypoplastic left heart with complete transposition of the great arteries.

A male newborn infant with hypoplastic left heart and transposition of the great arteries died on the second day of life. The haemodynamic consequences of this malformation are considerably different from those of ordinary hypoplastic left heart syndrome because of pulmonary rather than aortic atresia. In the absence of cross sectional echocardiographic equipment, cardiac catheterisation with angiography is necessary to delineate the anatomical picture. These two conditions have not apparently been reported before in association.

The protein requirement of preterm infants.

The daily fetal protein accretion rate (g/kg b.w.) was calculated from the daily fetal weight gain and the protein content of the fetus. Taking the net protein utilization of 80% in preterm infants (Snyderman et al., 1969) we calculated the protein requirement of preterm infants from the fetal protein accretion rate to be at a maximum of 2.7 g/kg b.w. day for the fetus growing on the 50th weight centile during the 30th to 31st postconceptional week. The more preterm infant has lower requirements.

Cor triatriatum masked by primary pulmonary hypertension.

In a 17-month-old boy with clinical signs of right heart failure, the diagnosis of primary pulmonary hypertension was made, based on cardiac catheterization findings with high pulmonary arterial pressure and normal pulmonary wedge pressure although two-dimensional echocardiography demonstrated a left atrial membrane. Postmortem examination confirmed a left atrial membrane (cor triatriatum), and the microscopic examination revealed the histological pattern of primary pulmonary hypertension. Due to reduced pulmonary blood flow, pulmonary venous obstruction was masked and could not be assessed by measuring pulmonary wedge pressure.

Cardiac hypertrophy secondary to ACTH treatment in children.

The usefulness of ACTH in the treatment of childhood epilepsy is assessed by improvement in the EEG and in the clinical condition. However, pronounced side effects, even serious ones, must be encountered. The most common complications are Cushing syndrome, infections, and arterial hypertension. We report on seven patients with infantile myoclonic seizures, who exhibited myocardial hypertrophy with increased left ventricular function during ACTH treatment. These changes were detected and followed by serial echocardiographic investigations. Within a period of 5 months after the termination of ACTH therapy the abnormal echocardiographic findings disappeared. We believe that the cardiac hypertrophy is ACTH-induced. Based on the various biological effects of ACTH different explanations are proposed: oedema or deposition of glycogen in the myocardial tissue, hyperinsulinism, arterial hypertension and increased inotropic stimulus. Because of our observations, we suggest careful monitoring of children treated with ACTH by performing serial echocardiographic investigations.

Left ventricular thrombi in three children with dilated cardiomyopathy: diagnostic procedure and clinical course.

In three of nine children with dilated cardiomyopathy (aged 1-9 years), left ventricular thrombi were diagnosed and followed by echocardiography. Thrombi recurred in two patients, in one of them, embolized to the cerebral arteries. Resolution of the thrombi was observed under therapy directed against platelet aggregation. However, this did not prevent thrombus formation.

[Value of 2-dimensional echocardiography in symptomatic heart diseases in the newborn infant and in early infancy]. / Stellenwert der zweidimensionalen Echokardiographie bei symptomatischen Herzerkrankungen im Neugeborenen- und frühen Säuglingsalter.

From January 1980 to March 1983, 73 distressed newborns and young infants, in whom congenital cardiac defects were suspected, were investigated by two-dimensional echocardiography. The diagnosis in these infants could be re-established later by cardiac catheterization and cineangiocardiography, operation or autopsy. Correct diagnoses were set in 97% of the patients (71 out of 73). Specific echocardiographic criteria for the diagnosis of the individual defects are discussed.

The valve of the foramen ovale in interatrial right-to-left shunt: echocardiographic cineangiocardiographic and hemodynamic observations.

In postpartum persistent right-to-left shunt at the atrial level, the valve of the foramen ovale fails to close. As a thin valve-flap the septum primum is pushed to the left during the phases of right atrial pressure predominance and closes to the septum secundum, when left atrial pressure exceeds right atrial pressure. Thus, it performs a marked movement during the cardiac cycle, reflecting the interatrial pressure-flow dynamics. With use of M-mode echocardiography, this movement pattern was studied in 24 patients: 13 with cyanotic heart disease (age 2 days to 21 years) and 11 newborns with persistent transatrial right-to-left shunt due to noncardiac disease. Cardiac defects were confirmed by cardiac catheterization and cineangiocardiography. Interatrial right-to-left shunts were proved by M-mode and 2-dimensional contrast echocardiography. The comparison of the M-mode echocardiographic findings in our patient groups with normal atrial septal movement studied in 20 healthy infants and children revealed considerable differences. The characteristic movement of the valve of the foramen ovale also was compared with results obtained by cineangiography and 2-dimensional echocardiography. Analysis of interatrial blood pressure difference provided a pathophysiologic explanation of the septum primum movement in transatrial right-to-left shunt.

[Hypertrophic cardiomyopathy during ACTH treatment]. / Hypertrophe kardiomyopathie unter ACTH-Behandlung.

We report on two infants (two and six month old) with infantile myoclonic seizures, who developed signs of hypertrophic cardiomyopathy (HCM), while receiving ACTH treatment (Tetracosactid=Synacthen Depot). The diagnosis of HCM was established by echocardiography. The first patient (R.M. female). showed signs of cardiac insufficiency and was treated with a beta-blocking agent (Propranolol=Dociton). This led to resolution of the clinical symptoms and reversal of myocardial muscle thickness as determined by echocardiography. The second patient (R.S. female) did not develop cardiac symptoms. A correlation between cardiomyopathy and ACTH treatment is discussed. Based on the various biological effects of ACTH different hypothetical explanations for this correlation are proposed: increased deposition of glycogen, enhanced protein synthesis, oedema of the myocardial tissue and systemic hypertension. Because of the correlation observed between ACTH treatment and the development of cardiomyopathy we recommend regular physical and echocardiographic examinations to detect cardiac involvement during treatment with ACTH.

[The right aortic arch with retrooesophageal component]. / Der rechte Aortenbogen mit retroösophagealer Komponente.

We report on 8 children with a right aortic arch and a retrooesophageal component of the upper descending aorta. The chest roentgenogram shows a right aortic arch and the oesophagogram a broad posterior indentation of the oesophagus in every case. Aortography was performed in 5 cases: in 2 children, the branches of the right aortic arch originate in a mirror image relationship to normal, in the other 3 children, the left subclavian artery originates as 4th branch from a retrooesophageal diverticulum of the upper descending aorta. Three children have an acyanotic congenital cardiac defect. Four of the children are asymptomatic while 3 have exertional dyspnoe and one suffers from dysphagia and frequent bronchitis. The symptoms are caused by compression of the oesophagus and the trachea by the right aortic arch, the retrooesophageal diverticulum and the patent or atretic ductus arteriosus originating from the latter. Only one child had to be operated for severe symptoms. There is a significant difference between the right aortic arch with or without a retrooesophageal component. The former mostly occurs alone and sometimes causes clinically significant oesophago-tracheal compression, while the latter is almost always combined with a cyanotic cardiac defect (tetralogy of Fallot, truncus arteriosus) but practically never causes a compression.

[Aortico-left ventricular tunnel. Report of 3 cases and review of the literature]. / Aortico-linksventrikulärer Tunnel. Bericht über drei Fälle und Literaturübersicht.

The aortico-left ventricular tunnel (AOLVT) is a rare abnormal communication between the ascending aorta and the left ventricle. The pathogenetic mechanism of this congenital anomaly is not completely understood. In addition to our 3 observations, 28 cases have been reported to date. In most cases the first symptoms appeared in early infancy. Clinical presentation and hemodynamics are identical to those of an aortic valve insufficiency. The correct diagnosis can be established by echocardiographic and angiocardiographic methods. Since the aortico-left ventricular tunnel is associated with congestive heart failure in infancy, in most cases surgical intervention is indicated in early childhood. The method of choice for surgical correction is a patch closure of the aortic orifice of the tunnel.