[Experimental characteristics of the neurotropic spectrum of some antiepileptic agents]. / Eksperimental'naia kharakteristika neirotropnogo spektra nekotorykh antiépilepticheskikh preparatov.

Investigation of the psychotropic spectrum of phenobarbital, diphenin, milontin, pufemide, sodium valproate (depakine), and diazeparn showed that, besides antagonism with the corazole and electroshock effects, all these drugs prevented to a considerable degree the kindling seizures in rats on a "tonsil swing" (chronic electroencephalographic partial epilepsy) model. The mechanism of this effect probably involves exciting amino acids. All anticonvulsants except for diazepam also restored memory in experimental animals with retrograde electroshock amnesia. The administration of diazepam, diphenin, and sodium valproate to rats with kindling convulsions led to activation of the spontaneous horizontal motions in the open-field test, which can be explained by the presence of antianxiety component in the drug action.

Right ventricular endomyocardial fibrosis. Diagnosis and management.

The clinical course, noninvasive and invasive diagnostic findings in 4 patients (pt) with endomyocardial fibrosis are reported. All patients (16-50 years) were in functional class III-IV (NYHA). Central venous pressure with large a- and v-waves was elevated in all; liver enlargement and peripheral edema were also noticed in all pts, and ascites in 3. An apical 2/6 systolic murmur was present in 2, and a right parasternal pansystolic murmur 2-4/6 with positive Carvallo's sign in all pts. ECG was non-specific and chest x ray showed right atrial enlargement in every case. A right-sided diastolic plateau which was higher than left ventricular end-diastolic pressure was present in all pts. Echo-, angiocardiography and computed tomography of the heart revealed obliteration of the right ventricular cavity, predominantly localized at the apex. Two pts who underwent endocardial resection and tricuspid valve replacement are alive and well after 9 and 8 years, respectively. One pt died early in the postoperative period and one died waiting for surgical therapy. In conclusion, echo-, angiocardiography and computed tomographic findings are diagnostic. A satisfactory differentiation from other cardiac disorders with restrictive hemodynamics and right-sided heart failure is possible.

The effects of two strains of influenza virus on cardiac development in the chick embryo.

To investigate the teratogenic effects of influenza virus on cardiac development two strains of influenza virus (Ty/Calif/5142/66 and Ty/Calif/64/M. Meleagrium) were injected into the yolk sac of fertile white Leghorn eggs during stage 23. Eggs injected with normal saline served as controls. The number of live embryos was significantly reduced in eggs injected with the 5142 strain, whereas the Meleagrium strain did not affect the number of live embryos in a significant manner. Serial sections of the live embryo hearts, extracted during stage 35, showed no cardiac anomalies in control groups. However, cardiac malformations were increased significantly in the embryos infected with the influenza viruses. A comparison of the two viral strains showed that the 5142 strain produced significantly more abnormal hearts than the Meleagrium strain. We conclude that (1) influenza infection during critical stages of embryonic development can lead to the cardiac anomalies, and (2) the virulence of a viral strain has a significant bearing on its teratogenic potential, at least in chick embryo hearts.

Unusual cardiovascular manifestations in a case of pheochromocytoma.

A case of proven pheochromocytoma is reported of a patient who presented with attacks of apparent hypotension, pulmonary edema, and myocardial injury with typical ECG and enzyme change. In spite of unmeasurably low peripheral blood pressure recordings during the attack, central aortic pressure was well above 240/140 mmHg. A clinical shocklike state was in effect due to severe arterial vasoconstriction. The pulmonary artery pressure was 48/26 mmHg during attacks, wedge pressure was 26 mmHg, and cardiac index 1.8 liters/min/m2. The coronary angiogram showed abnormal pooling of dye in the myocardial capillary network. It is concluded that: (1) Apparent hypotensive episodes in pheochromocytoma may be misleading and central arterial measurements may be needed to demonstrate the hypertensive crisis. (2) The abnormal coronary angiogram in our patient may be due to myocardial necrosis and damage to the intact arteriolar and microvascular system of the myocardium.

Myocardial metal content in patients who expired from cyanotic congenital heart disease and acute rheumatic heart disease.

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.

Papillary muscle hypertrophy in chronic rheumatic mitral valve stenosis. A clinicopathologic study.

A quantitative pathologic study was performed on papillary muscles in 15 cases of chronic rheumatic mitral stenosis. The papillary muscles were grossly hypertrophied as compared with papillary muscles in 10 normal specimens. Hypertrophy of papillary muscles was associated with pronounced fibrosis. Angiographically, these hearts were remarkable in small left ventricular cavity with almost complete cavity elimination at end systole. Left ventricular wall dyskinesia was also present. It is probable that derangement of the mitral valve structure due to rheumatic process leads to more stress on the supporting elements, thus resulting in papillary muscle hypertrophy.

Right ventricular endomyocardial fibrosis simulating Ebstein's anomaly.

A case of right ventricular endomyocardial fibrosis simulating Ebstein's anomaly is described. The clinical features, chest x-ray, electrocardiogram, echocardiogram, intracardiac electrogram, and the angiogram were all compatible with Ebstein's malformation. A correct diagnosis was subsequently made in the operating room. Reasons for the difficulties in the differential diagnoses of these two entities are discussed.

Constrictive endocarditis. Report of a case with successful surgery.

An 18-year old white youth presented with severe right heart failure and was found to have an obliterated and funnel-shaped right ventricle, massive tricuspid regurgitation, and mitral regurgitation. The haemodynamic findings were those of cardiomyopathy with obliteration on the right side. He underwent surgery consisting of decortication and peeling off of a thickened right ventricular endocardium, tricuspid valve replacement, and mitral valve repair. After surgery there was pronounced haemodynamic as well as clinical improvement. The pathological picture was that of constrictive endocarditis. We recommend this method of treatment for patients with obliterate cardiomyopathy on either side of the heart.

Association of left dominant coronary arterial system with congenital bicuspid aortic valve.

Clinical angiographic studies have documented an association of left dominance of the coronary arteries with aortic stenosis and congenital bicuspid aortic valve. The postmortem arteriograms of 973 autopsy patients were reviewed for pattern of coronary dominance and the hearts examined for the nature of any aortic valve disease. There were 673 hearts (70 percent) with a right dominant pattern, 198 (20 percent) with equal dominance and 102 (10 percent) with left dominance. Of 34 hearts with congenital bicuspid aortic valve, 10 (29 percent) had left dominance, a difference significant at the 0.005 level. Of 44 hearts with calcific aortic stenosis, an acquired valve lesion, 9 (20 percent) had left dominance. Rheumatic aortic valve disease (47 cases) and aortic regurgitation (27 cases) had no apparent relation to the coronary arterial pattern. The results confirm the association of left coronary arterial dominance with congenital bicuspid aortic valve. It is suggested that a left dominant coronary system may arise as a consequence of disproportionately decreased blood flow in the left heart chambers, one cause of which is aortic valve stenosis, during early cardiogenesis.

Association of atrioventricular valve atresia with single ventricle, truncus arteriosus communis and transposition. A basic reorientation in the approach to the definition of congenital heart defects.

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.

Isolated unilateral absence of the pulmonary artery. Review of the world literature and guidelines for surgical repair.

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.

Pathology of chronic rheumatic mitral valvulitis in Iran and its surgical implications.

Sixty-two consecutive surgically removed chronic rheumatic mitral valves were classified and tabulated according to their pathological features in relation to age and sex of the patients. The pathological features of the valves are quite different from the cases seen in Western hemisphere. Three types of gross pathological features are described. First is fibrous stenotic type which has very thick cusps and extremely short chordae tendineae. It is the most frequent rheumatic vavle seen in children, and majority of the valves show pure stenosis, for which probably open commissurotomy is the procedure of choice. Those which have a combination of stenosis and insufficiency usually require valve replacement. The second is the elastic insufficient type, which has a unique gross pathological feature, consisting of a remarkable elasticity and moderately thickened cusps. A valve replacement or valve repair when feasible is the treatment of choice for this type. The third is the calcific stenotic type. In this type the cusps are moderately thickened with short chordae tendineae with various degrees of calcification. This type is seen usually in the 4th decades of life and is more frequent in males. This type of valve must be treated either by open commissurotomy in order to eliminate the risk of embolization of calcific material or with valve replacement.

Quantitative angiocardiographic recognition of atypical form of tetralogy of Fallot with absent pulmonic valve.

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.

Calcified right ventricular fibroma causing outflow obstruction. Report of a case with successful excision.

A 5-year-old boy with clinical findings of pulmonic stenosis was found to have a large calcified mass in the right ventricular outflow region and a gradient of 120 mm Hg between the right ventricle and the pulmonary artery. At surgery, an ovalshaped tumor attached to the interventricular septum and obstructing the right ventricular outflow tract was removed. The child survived and is doing well. Histologically, the tumor had the characteristics of fibroma. A hemodynamic study three months after surgery showed almost complete abolishment of the gradient. To our knowledge this is the fifth reported case of calcified right ventricular fibroma with successful operation. In childhood intracardiac calcifications, together with obstruction, are highly suggestive of this lesion.