Report of a new case of axial mesodermal dysplasia complex.

We present a female child with phenotypical and clinical features of the axial mesodermal dysplasia complex. Typical manifestations of both the Goldenhar syndrome and the caudal regression syndrome are present in this complex. Only a few reports have described patients with this pattern of malformations localized in both the cranial and caudal regions.

Effect of age, diet and sex on muscle energy metabolism and on related physicochemical traits in the rabbit.

Muscle energy metabolism and related physicochemical traits of the meat were studied in 84 Provisal hybrid rabbits of both sexes. From weaning (30 days) to 55 days of age, the rabbits were divided into 4 groups which were given ad libitum one of the following diets: H diet with high digestible energy (DE) level, L diet with low DE level, F diet with high DE level, obtained by supplementing with animal fats, and W diet with moderate DE level. The H, L and F diet were suitable for lactating does and weaning rabbits; the W diet for weaned young rabbits.

[Vitamin D-dependent type-1 rickets: diagnosis and treatment of a further case].

We describe a child with vitamin D dependent rickets type 1, who developed clinical signs of the disease at three months of age. The principal manifestations were hypocalcemia and seizure with EEG abnormalities. The circulating level of 1 alpha, 25 (OH)2D3 was low despite a normal level of 25 (OH)D3 and an adequate vitamin D supplementation.

[A transesophageal Doppler echo-color study of pulmonary venous flow before and after the correction of valvular defects].

Aim Of The Study: To verify changes of pulmonary venous flow pattern before and after surgical or percutaneous correction of valvular heart disease.

Methods: The pulmonary venous flow pattern was studied by transesophageal echocardiography in 27 patients affected with heart valve disease (11 mitral insufficiency, 10 mitral stenosis, 2 aortic stenosis and 4 pulmonary stenosis), before and after surgical or percutaneous correction. Pulmonary venous flow velocity variables measured included peak systolic and diastolic flow velocities (VmaxS and VmaxD), systolic and diastolic velocity time integrals (IS and ID) and their respective ratios (VmaxS/VmaxD and IS/ID).

Usefulness of transesophageal echocardiography in the pediatric catheterization laboratory.

Transesophageal echocardiography was performed in 51 children (aged 2 to 14 years, mean 4; weight 9 to 50 kg, mean 21) undergoing elective diagnostic or therapeutic cardiac catheterization. The interventional procedures were percutaneous balloon dilation of pulmonary (n = 8) and aortic (n = 2) valve stenosis, percutaneous closure of patent ductus arteriosus (n = 8), and attempted occlusion of Pott's anastomosis by the double umbrella device (n = 1). The diagnostic catheterizations were performed on preoperative children of whom 5 had undergone previous palliative procedures.

Plasma levels of atrial natriuretic factor (ANF) and urinary excretion of ANF, arginine vasopressin and catecholamines in children with congenital heart disease: effect of cardiac surgery.

We studied the changes in the plasma concentration of atrial natriuretic factor (ANF) and the urinary excretion of ANF, arginine vasopressin (AVP) and catecholamines in 22 children with congenital heart disease, divided into two groups. Group 1 included 11 children with congestive heart failure (CHF), treated with digitalis and diuretics. Group 2 included 11 children without CHF and without medical treatment.

Late left atrioventricular valve insufficiency after repair of partial atrioventricular septal defects: anatomical and surgical determinants.

Risk factors for late left atrioventricular (AV) valve insufficiency, which occurred in 16 (18%) of 90 patients evaluated after repair of partial AV septal defect, were examined. The operative findings in 9 patients undergoing reoperation were also examined. Preoperative left AV valve insufficiency was significantly more common in the group with late left AV valve incompetence, as were associated valvular malformations as a whole and fenestrations of valve leaflets in particular.

[Anomalous origin of the left coronary artery from the aorta. The surgical implications in a case].

A case of high take-off of the left coronary artery from the aorta in a patient with a large ventricular septal defect (VSD) is described. The preoperative detection of this coronary anomaly, enabled the surgeon to modify the technique of aortic cannulation in such a way that the anomalous left coronary artery could be adequately perfused with the cardioplegic solution. Furthermore, injury to the coronary artery during the operative procedure was carefully avoided by knowing the exact location and course of the left main coronary trunk, from the angiographic pictures.

Pericardial cyst diagnosed by two-dimensional echocardiography and computed tomography in a newborn.

A one-day-old full-term female infant with massive "cardiomegaly" developed marked respiratory distress soon after birth. The two-dimensional (2D) echocardiogram demonstrated a 4 X 5-cm cystic mass adjacent to the left ventricular free wall. Computed tomography (CT) confirmed the presence and dimension of the cyst and CT number indicated the fluid within the cyst to be serous.

Staged septation of double inlet left ventricle.

Complete septation of a double inlet left ventricle with left anterior subaortic outlet chamber was carried out successfully as a staged procedure during the first two years of life in a patient with severe pulmonary hypertension and an abnormal left atrioventricular valve. In contrast with isolated pulmonary artery banding, which rarely has led to a satisfactory septation or modified Fontan operation, this technique achieved good initial palliation and uncomplicated intracardiac repair. It should be considered for infants with univentricular atrioventricular connection, two atrioventricular valves, and excessive pulmonary blood flow.

Effects of dl-methadone on the response to physiological transmitters and on several functional parameters of the isolated guinea-pig heart.

The effects of high concentrations of dl-methadone (1-10 microM) on several parameters of cardiac function were studied in isolated guinea-pig heart preparations. The opioid agonist dose-dependently potentiated the inotropic cardiac response to sympathetic nerve stimulation; this effect was naloxone-insensitive and was antagonized by an increase in extracellular calcium concentration. Moreover, methadone enhanced the dose-inotropic response curve of exogenous noradrenaline.

In vitro evaluation of the beta-blocking and electrophysiological properties of mepindolol.

The electrophysiological and beta-blocking effects of mepindolol have been examined in isolated guinea-pig preparations and sheep cardiac Purkinje fibers. Mepindolol did not show selectivity for blocking the chronotropic and inotropic responses to isoprenaline on isolated guinea-pig preparations, and was practically equally potent in blocking the effect of isoprenaline on isolated guinea-pig trachea. Mepindolol 10(-7) M fully antagonized the positive chronotropic effect of isoprenaline (10(-7) M) in Purkinje fibers.

Spectrum of hearts with one underdeveloped and one dominant ventricle.

Hearts with one underdeveloped and one dominant ventricle form a spectrum of anomalies extending from the heart with two clearly adequate chambers to those with a true single ventricle. An angiographic concept of grouping such hearts is presented. 129 patients with unequal ventricles underwent catheterization and cineangiography between 1974 and 1983.

Overriding atrioventricular valves. An angiographic-anatomical correlate.

An analysis of 6 patients with overriding atrioventricular valve was performed in order to correlate the anatomic and angiographic features of this malformation. The atrial arrangement was usual (situs solitus) in each patient. The ventricular chambers were normally related in 4 and the mirror image of normal in 2.

Repair of congenital pulmonary venous stenosis with living autologous atrial tissue.

Repair of localized bilateral congenital pulmonary venous stenosis with living autologous atrial tissue was accomplished in two patients, 3 and 4 months of age. The tip of the left atrial appendage was opened and used as an onlay patch to widen the stenotic area of the left pulmonary veins, leaving a double pathway for venous return. A flap of interatrial septum was evaginated through an incision in the right side of the left atrium and used as an onlay patch to widen the stenotic area of the right pulmonary veins.

Congenital pulmonary vein stenosis.

Congenital pulmonary vein stenosis is a rare and serious form of congenital heart disease. Between 1969 and 1982 10 patients with this lesion were studied. In 2 patients the condition was diagnosed at autopsy; these patients died before the presence of congenital heart disease was suspected.

Tetralogy of Fallot with atrioventricular canal. An angiographic study.

Tetralogy of Fallot associated with atrioventricular (AV) canal is a well-recognized cardiac malformation; its surgical repair carries a high mortality, which is related partly to the complexity of the defect and partly to incomplete diagnosis. Hence an accurate preoperative diagnosis is essential. This communication describes the angiographic features observed in 17 patients with tetralogy of Fallot and AV canal with emphasis on the morphology of the right AV valve as seen during the right ventriculogram.

Investigation and management of primary cardiac tumors in infants and children.

Seven children with primary cardiac tumors are discussed. The diagnosis was achieved by cardiac catheterization in all seven patients. In four patients, surgical excision was undertaken with one long-term good result.