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Background: Atrial ejection force (AEF) expresses the force exerted by the left atrium to the mass of blood passing through the mitral valve during atrial systole. It provides a diagnostic and predictive parameter for evaluating left ventricular diastolic abnormalities and a physiologic assessment of atrial systolic function., Methods: We obtained normal values of AEF in a group of 47 newborn infants with normal heart function and structure, using Doppler echocardiographic parameters of transmitral filling flow. AEF is defined as the product of the density of blood, the mitral valve area, and the square of peak A velocity [AEF = 0.5 x rho x mitral valve area x (peak A velocity)(2)]., Results: Mean and SD of AEF was 1.12 +/- 0.42 kilodynes. Atrial filling fraction (r = 0.74, P value = .000), A acceleration rate (r = 0.67, P value = .000), A deceleration rate (r = 0.64, P value = .000), and heart rate (r = 0.70, P value = .000) showed a positive correlation with AEF. Rapid filling fraction (r = -0.71, P value = .000) and E/A ratio (r = -0.6, P value = .000) had a negative correlation with AEF., Conclusion: AEF index in neonatal period is augmented and comparable with the values in adult population that could be explained by the specific pattern of slow ventricular relaxation in newborn infants. Complex aspects of diastolic function in newborn infants could be assessed beyond a simple E to A ratio by providing an estimate of normal values for AEF in this age group.

Ouabain-induced tachyarrhythmias in cats can be abolished by bolus injection of NaHCO3 solution. This effect is consistent, rapid and of short duration. The antiarrhythmic effect appears to be due to high sodium concentration of this solution, as hypertonic NaCl solution with equal concentration of sodium exerts the same effect. Alkalinity and hyper-osmolality cannot account for the antiarrhythmic effect of NaHCO3 as respiratory alkalosis and mannitol solution with equal osmolality did not relieve the arrhythmias. Overdrive suppression is not responsible for this effect as the heart rate slows down during normalization of the cardiac rhythm. It is postulated that high Na+ concentration abolished ouabain-induced arrhythmias by competition with calcium ion in the myocardial cell sarcotubular system thus replacing it. This hypothesis is supported by the known effect of other agents such as sodium-EDTA and magnesium chloride, in digitalis-induced arrhythmias, which exert their antiarrhythmic properties by removal or displacement of calcium in the myocardial cells.

The case histories of two children with echinococcal cysts of the left ventricular myocardium are reported. Both patients underwent successful enucleation of the cysts, after initial local treatment with 0.5 percent silver nitrate solution. A previous review of the world literature on the surgical treatment of echinococcal cyst of the heart, published in 1971, is brought up to date by the addition of 60 new cases to the 118 cases then described. Instillation of 0.5 percent silver nitrate solution into the cyst cavity is now a routine procedure in our institution to sterilize the cyst and to kill the scolices. This substance is preferable to formalin and hypertonic sodium chloride solution, because it acts rapidly and is nontoxic and nonabsorbable. The two children in this report are the first in whom silver nitrate solution was used to sterilize the myocardial cysts.

A comparative morphometric study was performed on the aortomitral valve apparatus of the embryonic and adult chicken hearts. It was found that the thickness of the aortic and mitral valve cusps diminishes dramatically as the embryo matures, however the relative length of the aortic valve, mitral valve and subaortic conus remains constant. A comparison of the aortomitral valve apparatus in chicken and man revealed that the subaortic conus is not completely resorbed in the fowl, whereas this process is well documented in the mammalian hearts. The implications of this observation on "the differential conal growth hypothesis" of transposition of the great arteries are discussed.

Previous reports from this laboratory have shown that penicillamine effectively reduces serum digoxin levels and is a clinically useful drug in correcting digoxin intoxication. To elucidate further the antidigitalis effects of penicillamine a prospective study was undertaken in 10 children aged 4--14 years with congestive heart failure. Plasma and intracellular erythrocyte concentrations of sodium, potassium, calcium as well as Na+/K+ and Na+/Ca++ ratios were measured before digitalization, 6 days after full digitalization while the patients were on maintenance doses of digoxin (0.02 mg/Kg/day, po, maximum 0.25 mg/day) and 6 hours after 1 Gm of oral penicillamine. After digitalization RBC Na+, Ca++, Na+/K+, and Na+/Ca++ increased, whereas RBC K+ levels decreased significantly. Administration of penicillamline not only reduced serum digoxin levels, but it also caused significant alterations in RBC electrolyte concentrations, toward pre-digoxin values. All values were significantly changed after penicillamine. Plasma and RBC magnesium levels were not altered significantly, neither after digitalization nor after penicillamine. It is concluded that in addition to RBC Na+ and K+ levels, intra-erythrocyte levels of calcium are sensitive indicators of digoxin effect; and that penicillamine reverses digoxin-induced RBC electrolyte alterations towards pre-digitalization values.

The electrophysiologic effects of verapamil, a slow channel blocker, were investigated during diagnostic cardiac catheterization in 24 children premedicated with lytic cocktail. The ages ranged from 50 days to 12 years. Twenty had congenital and 4 had rheumatic heart disease. Surface EKG, high intra-atrial and His bundle electrograms were obtained in all before and 5 min after a single dose of verapamil (0.15 mg/Kg, max 5 mg iv). In 14 cases complete electropysiologic studies were performed using the atrial pacing and extrastimulus technique. Due to variability of the resting heart rates and the effect of cycle length on refractory periods each paced with identical S1-S1 interval before and after verapamil, thus allowing each case to serve as his own control. Verapamil prolonged the corrected AH interval in all (mean +/- SD; from 116 +/- 37 to 152 +/- 41 msec, p less than 0.01) and shortened the HV interval in 15/24 (mean +/- SD: from 55 +/- 13 to 47 +/- 9.9 msec, p less than 0.05). The effective and functional refractory periods of the total conduction system, the AV node (ERPAVN) and atrium (ERPA) increased significantly in 10/14. The most profound effect was on ERPAVN and ERPA (25.54 +/- 29 and 19.27 +/- 21.81 percent mean percent increase +/- SD respectively, p less than 0.01 and p less than 0.02). Our findings show that verapamil prolongs the effective and functional refractory periods of the cardiac conduction system with maximal effects on the AV node, thus suggesting the mechanism of its effectiveness in the treatment of reentrant supraventricular arrhythmias.

Disopyramide has been successfully used to treat a variety of supraventricular and ventricular arrhythmias. The electrophysiologic effects of this drug were studied following diagnostic cardiac catheterization in 14 children aged 7 months to 14 years, suffering from congential heart disease. Surface EKG, intra-atrial, and His bundle electrogram as well as right atrial pacing with the use of extrastimulus technique were obtained before and after a single intravenous dose of disopyramide (2 mg/Kg, max 50 mg). The drug significantly prolonged the HV interval, effective and functional refractory periods of the atrium and the AV node. The most consistent change was the prolongation of the atrial refractory periods. The electrophysiologic effects of this drug on the conduction system have been variable and this variability has been attributed to the degree of vagal tone. The findings in this age group are similar to adults with simultaneous cholinergic blockade. This might be due to the direct depressant effects of disopyramide on the atrial and AV nodal tissue, and point to the potential usefulness of this drug in the pediatric age group.

A general discussion of arrhythmias as followed by a brief description of two of the commonly encountered dysrhythmias in the pediatric age group, i.e., paroxysmal supraventricular tachycardia and complete AV block. The use of verapamil a slow-channel blocker in the treatment of the former is described. The problems of permanent cardiac pacing in children are also dealt with.

We previously reported that pulmonary artery pressures can be assessed in children with chronic rheumatic mitral valve disease by measuring right maximum spatial vector from Frank vectorcardiogram. To simplify the calculation, pulmonary artery systolic pressure was correlated with maximum negative deflection on Frank scalar X-lead (SX), maximum negative deflection on scalar Z lead (SZ), sum total of SX and SZ (SX+SZ) and combined SX and SZ. The patient material consisted of 30 children with chronic rheumatic mitral valve disease, aged 8-14 1/2 years. Eleven were male and 19 were female. Sixteen had mitral stenosis, 8 had mitral regurgitation, and 6 had combined mitral regurgitation and stenosis, documented by cardiac catheterization and angiocardiographic study. The results showed a significant correlation between pulmonary artery systolic pressure and SX (r=0.782). As calculation of SX is considerably easier and less time consuming than that of right maximum spatial vector, this simplified method is preferable to right maximum spatial vector for prediction of the pulmonary artery systolic pressure of children with pulmonary hypertension due to chronic rheumatic mitral valve disease.

Vitamin E (E) and selenium (Se) are important antioxidant nutrients, preventing membrane damage by lipid hydroperoxides. In a previous study on E + Se deficient rats it was shown that plasma levels of lipoproteins increase dramatically. In continuation of this study a quantitative microscopic study was undertaken to determine the behavior of myocardial interstitial tissue space (ITS) in E + Se deficient rats as compared to control rats. Four rats fed on normal laboratory chow and 8 rats fed on basal diet supplemented with adequate E + Se served as control. Eight rats were fed on E + Se deficient diet. The animals were sacrificed after 13 to 20 weeks. Qualitative microscopic study showed patchy areas of hemorrhage, edema, infiltration of macrophages and myocardial damage consisting of pyknosis and coagulation necrosis in the deficient rats. Quantitative microscopic study of the apparently normal areas of myocardium of deficient rats showed a shrinkage of ITS (21.0 +/- 1.29% vs 16.51 +/- 4.62%, alpha = 0.01), whereas the ITS of normal laboratory chow and E + Se supplemented groups were similar. The myocardial fiber diameter was unchanged. Using Starling's hypothesis regarding fluid movement across capillary walls, several mechanisms could be advanced to explain this apparently paradoxical phenomenon. We contend that the interstitial fluid of normal areas of the myocardium is either absorbed by the adjacent damaged areas, or before extravasation of the intravascular proteins and swelling of ITS, the interstitial fluid is first absorbed by the intravascular compartment, due to capillary membrane damage.

Experience is presented of paediatric cardiology in a well-equipped and staffed centre during a six-year period in a developing country. Despite the success of the programme in offering first class medical investigation and surgical care to a large number of children, it is estimated that only about 2% of the existing cases in the country were operated upon. The main problems were the large numbers of cases, creating long waiting lists, and the lack of adequately trained paramedical personnel. Although paediatric cardiology in other developing countries is by no means a first priority, thousands of children suffering from heart disease should not be ignored. It appears that establishing similar centres for the care of children with heart disease in those countries is necessary; that they would contribute to patient care and medical education, would uncover the magnitude of the problem and would open ways to its future solution. The possibility of utilization of the facilities in the Western countries must also be considered.

The effects of intravenous administration of mexiletine on the refractory periods of the atrium and atrioventricular (AV) node were studied using His bundle recordings and the extrastimulus technique with atrial pacing. The drug was administered to 10 children with heart disease in an intravenous bolus dose of 3 mg/Kg, injected over a 5-min period, followed by an infusion of 1 mg/Kg/hr of mexiletine, achieving a mean therapeutic plasma concentration of 0.762 +/- 0.270 microgram/ml. The AH and HV intervals, the resting sinus cycle length, and the functional and effective refractory periods of the atrium and AV node were measured before and 5 min after initiation of the mexiletine infusion. The sinus cycle length was reduced significantly. However, neither the refractory periods nor the AH and HV intervals changed in a significant manner. Except for the heart rate, these results are similar to those reported in adults without conduction disturbances. In adults, mexiletine did not affect the sinus cycle length. However, in children, it consistently increased the heart rate. The lack of side effects in children is in contrast to adults, who usually suffer from gastrointestinal and neurologic symptoms upon receiving comparable doses parenterally.

This article dicusses emergencies of cardiovascular origin in infants and children. Particular attention is given to congestive heart failure, hypoxic states, arrhythmias, cardiac tamponade, perinatal cardaic emergencies and conditions simulating cardiac crises. An outline of etiologic and therapeutic approach is included, and guidelines are provided for the practitioner who is called to care for such emergency situations.

A heart specimen is presented which showed a rare combination of atrioventricular valve atresia with single ventricle, truncus arteriosus communis with an anteriorly placed trunk, ie transposition. Because of the problems involved in the classification of this heart, the types of single ventricle are revised with particular attention to the rare cases of atrioventricular valve atresia and single ventricle reported in the literature. Also the rare type of truncus arteriosus communis with truncoatrioventricular valve discontinuity is excluded from the so-called transposition complexes, and properly classified as a form of truncus. The literal meaning of transposition is stressed and it is urged to approach congenital heart defects in a purely anatomic sense with definition of each segment as accurately as possible. Thus the present confusion in terminology and further plethora of new terms are avoided.

A 9-year-old boy is reported who had tetralogy of Fallot, rudimentary pulmonic valve, and supravalvar pulmonic stenosis. The patient lacked almost all of the clinical findings commonly encountered in this syndrome. Thus he never had signs of a large left-to-right shunt, or congestive heart failure. He had no murmur of pulmonic regurgitation and his pulmonary artery was not large on chest roentgenogram. Angiocardiography revealed moderate main pulmonary arterial dilation. The lack of the usual manifestations of tetralogy of Fallot with absent pulmonic valve was due to supravalvar pulmonic stenosis, acting as a natural pulmonary artery band. A quantitative angiocardiographic study was undertaken in order to find a clue for the diagnosis of these atypical cases. The ratio of the transverse diameters of the main pulmonary artery and the aortic root as measured on lateral angiocardiograms in 31 children with uncomplicated tetralogy of Fallot was 0.70 +/- 0.22, whereas this ratio was 1.70 in this particular patient (p less than 0.001). It is concluded that a quantitative evaluation of the ratio of the transverse diameter of the main pulmonary artery to the aortic root on lateral angiocardiogram allows differentiation of tetralogy of Fallot with absent pulmonic valve associated with supravalvular pulmonic stenosis from uncomplicated forms of tetralogy of Fallot.

The effects of verapamil, a slow channel blocker and a potent antiarrhythmic drug, on retrograde V-A conduction were investigated following diagnostic cardiac catheterization in 12 children premedicated with lytic cocktail. Surface EKG, intra-atrial and His bundle electrograms, as well as right ventricular pacing with use of extra stimulus technique were obtained before and after a single intravenous dose of verapamil (0.15 mg/Kg, max 5 mg). Verapamil prolonged the AH interval in 9/12 and abolished or prolonged the retrograde V-A conduction whenever present (abolition in 5/12 and prolongation in 1/12). The duration of retrograde V-A conduction exceeded the antegrade conduction time in all. It is concluded that verapamil abolishes retrograde V-A conduction whenever present and this phenomenon may further explain the antiarrhythmic effects of the drug on retrograde conduction dependent (reentrant) arrhythmias.

Congenital heart disease and pediatric cardiology problems exist all over the world. The approach, however, including diagnosis and management varies considerably depending on manpower available, the clinical facility and the sociophilosophical climate prevailing in different parts of the world. In this article the priorities existing in the developed and developing countries concerning the approach to pediatric cardiology problems are discussed from the point of view of patient load, facilities available, teaching and research in this large field of highly specialized pediatric practice.

The importance of metals in normal and pathologic cardiovascular function has been recognized. Significant derangements in myocardial Ca2+, Mg2+, and Cu2+ have been reported in ischemic heart injury. We studied 3 groups of hearts: 1) fifteen specimens obtained from patients who had no heart disease, 2) nine specimens from patients who had expired from cyanotic congenital heart disease, and 3) ten specimens from patients who had expired from acute rheumatic heart disease with carditis and severe heart failure. None of the patients had undergone cardiac surgery. Left ventricular lateral wall Mg2+, Ca2+, Cu2+, and Zn2+ contents were measured by atomic absorption spectrometry. The results showed a significant decrease in myocardial Mg2- (Group I 177.06 +/- 32.71; Group II 155.66 +/- 14.79; Group III 149.00 +/- 13.29, p less than 0.05 and p less than 0.01, respectively), and Cu2+ contents (Group I 3.22 +/- 0.37; Group II 2.94 +/- 0.22; Group III 2.56 +/- 0.32, p less than 0.02 and p less than 0.001, respectively), and a rise in myocardial Ca2+ content (Group I 36.06 +/- 10.72; Group II 43.22 +/- 7.01; Group III 46.30 +/- 4.85, p = not significant, and p less than 0.01, respectively). The myocardial Zn2+ content did not change significantly (Group I 26.53 +/- 3.99; Group II 26.00 +/- 4.15; Group III 26.40 +/- 3.53). The myocardial Mg2+/Ca2+ ratio was reduced markedly in both groups (Group I 5.328 +/- 1.879; Group II 3.685 +/- 0.735; Group III 3.135 +/- 0.291, p less than 0.001 for both Groups II and III vs Group I). The latter results correlated closely with the myocardial Mg2+/Ca2+ ratios reported in experimental models in peri-infarction zones. Thus, the myocardium of patients who had expired from cyanotic congenital heart disease and acute rheumatic carditis is jeopardized by ischemia, with metal contents similar to the border areas in myocardial infarction.

Atrioventricular conduction characteristics were examined by atrial extrastimulus technique in 45 children aged 50 days to 14 years following diagnostic cardiac catheterization. None of the patients had a history of supraventricular tachycardia and none had evidence of pre-excitation on routine electrocardiogram. By plotting the A2H2 and H1H2 intervals against corresponding S1S2 intervals 2 types of conduction curve were obtained. Eight of the 45 children (17.7%) showed discontinuous conduction curves, characteristic of dual atrioventricular nodal pathways, whereas 37 patients had continuous curves. Of the 8 patients with discontinuous curves 3 showed unsustained echo beats following extrastimuli, after the effective refractory period of the fast pathway was reached. This study showed that, as in adults, the atrioventricular node was frequently functionally dissociated in children.

The Frank vectorcardiogram (VCG) and the electrocardiogram (ECG) were used to predict pulmonary arterial pressures in 30 pediatric patients with rheumatic mitral valve disease. The patients' ages ranged from eight to 14 1/2 years. Eleven were male and 19 were female. Sixteen had mitral stenosis, eight had mitral regurgitation and six had mitral stenosis and regurgitation. Mean pulmonary arterial wedge pressure ranged from 5-32 mmHg. All patients underwent complete catheterization and angiocardiographic study. None had significant gradient across the right ventricular outflow tract. Right maximum spatial vector (Rmsv) was calculated using Frank VCG.R in V1 and S in V5 of ECG were also measured. Rmsv, RV1, and SV5 were correlated with pulmonary arterial pressures (systolic, diastolic and mean). Pulmonary artery systolic pressure and Rmsv bear the best correlation (r=0.773). The correlation coefficient for pulmonary artery diastolic pressure and Rmsv was 0.698. Rmsv as calculated from Frank VCG is useful in prediction of pulmonary arterial pressures (systolic and diastolic) in pediatric patients with rheumatic mitral valve disease.

Forty-one children with advanced chronic rheumatic disease of the mitral valve had preoperative and postoperative hemodynamic studies. Twenty-three cases had open mitral commissurotomy, and 20 had mitral valvular replacement. After surgery the average pulmonary arterial systolic pressure in the group with valve replacement decreased from 78 to 42 mm Hg, the pulmonary vascular resistance decreased from 974 to 313 dynes sec cm 5, and the cardiac index rose from 2.29 to 4.15 L/min sq m. In the group with mitral commissurotomy, the average pulmonary arterial systolic pressure decreased from 90 to 63 mm Hg, the pulmonary vascular resistance decreased from 1,201 to 616 dynes sec cm-5, and the cardiac index rose from 2.5 to 3.5 L/min sq m. While all patients with mitral valve replacement showed a drop in pulmonary arterial pressure, the postoperative pressures were higher after commissurotomy in four patients, two of whom required a second operation of valve replacement. The study shows that in children, even severe degrees of pulmonary hypertension secondary to mitral valve abnormality is corrected.

Frank vectorcardiograms (VCGs) in four patients with left ventricular-right atrial (LV-RA) communication were analyzed and compared with published values of VCGs of normal individuals and those from a group of patients with membranous ventricular septal defect. It was observed that the QRS-loops in the frontal and sagittal planes of the patients with LV-RA communication are shifted more superiorly than usual. Thus, almost 50% of the QRS-loop area was superior to the X- and Z-coordinates in the frontal and sagittal planes, whereas in the controls less than 10% of the frontal and sagittal plane QRS-loops were superior to the horizontal axes. The QRS-loops of patients with LV-RA communication thus seem to be halfway between normal and endocardial cushion defect loops. An unusual degree of superior orientation of the QRS-loop in a patient with clinical findings of a ventricular septal defect should arouse suspicion of a LV-RA communication. Also in patients with an isolated ventricular septal defect but with an exaggerated superior orientation of the QRS-loop in the frontal and sagittal planes, the interatrial septum should be examined at the time of operation to exclude the possibility of an associated LV-RA communication.

A 10-month-old boy is presented who had isolated unilateral absence of the right pulmonary artery. He suffered from hemoptysis and severe congestive heart failure. The patient underwent prosthetic anastomosis of the right to the main pulmonary artery. Although the hemodynamic response was favorable, his oxygenation did not improve due to diffuse pulmonary arteriovenous fistulae of the affected lung. The patient succumbed 3 months after operation due to massive uncontrollable hemoptysis from the right lung. Isolated unilateral absence of the pulmonary artery is a rare lesion. In our review of the world literature as of November 1976, 47 cases (including this report) of the unilateral absence of the pulmonary artery have been reported. Of these 25.5% had pulmonary hypertension and only 4 cases underwent successful repair of the lesion. Though repair of this defect can be carried out, the result may not be always gratifying. Our experience with this case has led us to consider a lung biopsy before proceeding to the surgical repair of the lesion. If the affected lung shows arteriovenous abnormalities the operation should not be recommended.

Hypertrophic cardiomyopathy (HCM) is most probably a genetically transmitted disease with different clinical and hemodynamic features. In hypertrophic obstructive cardiomyopathy (HOCM) the obstruction is predominantly in the left ventricular outflow tract (IHSS). In a minority of cases the obstruction is strictly located in midventricle (midventricular obstruction, MO). Hypertrophic nonobstructive cardiomyopathy (HNCM) includes asymmetric septal hypertrophy (ASH) and apical hypertrophy (AH). Right ventricular hypertrophic obstruction (RVHO) is an uncommon type of HCM and is almost always combined with other types of left ventricular HCM. We describe in the present report 1 case of RVHO with IHSS, 2 cases with MO and, to our knowledge, the first case with AH.

The effects of intravenous lidocaine on the refractory periods of the atrium, AV node and right ventricular myocardium were studied using His bundle recordings and the extrastimulus technique with and without atrial and ventricular pacing. The drug was administered in an intravenous bolus dose of 1 mg/Kg followed by an infusion of 100 micrograms/Kg/min. The AH, HV intervals and resting sinus cycle length as well as functional and effective refractory periods of atrium, AV node and right ventricle were measured before and 5 min afrer bolus injection of lidocaine. Neither of the refractory periods nor AH and HV intervals changed significantly following administration of lidocaine. The result of this first study in children is in agreement with previous reports of the effects of lidocaine on the cardiac conduction system in adults.