Eduardo Coelho

Cardiac involvement in a peculiar form of paramyloidosis

Abstract Heart disease is common in paramyloidosis. The clinical and pathologic features can be divided into two fundamental types. In the first type serious cardiac insufficiency is predominant and the patients die from congestive heart failure. The second type is familial, a peculiar peripheral neuropathy is the predominant lesion and patients die from cachexia. Patients with the first form may present a clinical, electrocardiographic and hemodynamic picture simulating ischemia or infarction of the myocardium or chronic constrictive pericarditis, or diffuse fibrosis of the myocardium. Patients of the second type have cardiac abnormalities in the electrocardiogram such as right or left bundle branch block, left ventricular hypertrophy, atrioventricular dissociation and signs of ischemia. The Congo red test is usually negative. It is almost always possible to diagnose cardiac paramyloidosis of the first or second form in living patients by biopsy of the skin.

Physiopathologic study (clinical and experimental) of the tricuspid valve

Abstract In a great number of cases clinical symptoms fail to give the diagnosis of tricuspid stenosis and insufficiency. The phlehogram can be used with advantage in the differential diagnosis of functional insufficiency and organic insuffi ciency, but stasis in congestive heart insufficiency may produce waves similar to these of tricuspid insufficiency. The most exact method is to register the auriculoventricular curves by catheterization. Tricuspid stenosis is almost always accompanied by insufficiency. At autopsy of patients with rheumatic valvular lesions, the percentage of cases with tricuspid stenosis was 22.5 per cent. For the diagnosis of stenosis the recommended criterion is the increase in the A-V gradient of pressure during the entire diastole (caused by increased auricular pressure), or the increase in the telediastolic gradient. The highest gradient observed was 23 mm Hg. Tricuspid endocarditis can exist without hemodynamic alterations, as occurred in one case of mitral and aortic endocarditis and tricuspid disease in which the stenosis was very severe. In 620 mitral patients who were catheterized, tricuspid insufficiency was found in 20 per cent. One-third of these did not present clinical symptoms sufficient for diagnosis, and the diagnosis was established by the cardiac catheterization. The early and marked elevation of the V wave, with or without a small plateau, generally corresponds to functional tricuspid insufficiency. Systolic regurgitation waves, more prolonged and with a more extensive plateau (waves of ventricularization), are characteristic of organic insufficiency. The configuration and amplitude of the tracings make it possible to appreciate the degree and the nature of tricuspid insufficiency as shown in several of the tracings. The development of auricular fibrillation does not provoke tricuspid insufficiency unless there is myocardial insufficiency. Certain curves of auricular pressure in auricular fibrillation do not present the x descent, since the auricle in fibrillation has neither systole or diastole. The shape of these pressure curves is therefore different from that of the sinus normal rhythm. A curve with no descent of x should not be interpreted as showing tricuspid insufficiency. On correlating tricuspid insufficiency with pulmonary hypertension it was found that the greater number of cases were encountered in the groups with the highest hypertension. There was no parallelism between the size of the regurgitation wave and the level of pulmonary pressure. There is likewise no correlation between the A-V gradient of diastolic pressure in tricuspid stenosis and the pulmonary pressure. The atrial pressure curves of tricuspid insufficiency provoked experimentally in dogs are similar to these of human tricuspid insufficiency. The pressure tracing of the right atrium with experimental auricular fibrillation (provoked by Mecholyl) may present a small x descent. In spite of this there may be, in some systoles, an escape of blood to the atrium resulting from the hingelike movement of the cusps, when these do not close completely. After tricuspid insufficiency has been provoked experimentally, the pressure tracings may not show the characteristic alterations. If we then provoke auricular fibrillation, these alterations appear immediately and continue even after the fibrillation ceases. If we provoke ventricular extrasystoles the auricular pressure tracing is completely modified and there appears a wave similar to the regurgitation wave, occupying the entire systole. The data observed in experimental tricuspid insufficiency in the dog following alterations in rhythm help us to understand the physiopathology of the tricuspid valve in man.

Electrocardiographic and vectorcardiographic alterations in chronic cor pulmonale

Abstract Study of the precordial electrocardiographic leads of 100 cases of chronic cor pulmonale establishes six patterns of the electrical alterations of the disease. The first four correspond to hypertrophy of the right ventricle; the fifth in part also corresponds to hypertrophy of the right ventricle; and the last, to associated disease. In some cases we were able to study the evolution of the electrocardiogram over long periods. The first electrical abnormality is the alteration of the P wave, but we have not included here cases only with this alteration. In 5 cases there was an arrhythmia. Detailed examination of the vectorcardiograms of 38 cases of cor pulmonale permits us to classify them in five groups according to the projection of the QRS loop and the direction of rotation in the horizontal plane. In the horizontal plane there is a progressive evolution of the orientation of the QRS loop from the front and left, to the front (left and right), to the front and right, to the right and posterior (group I and II); from the left and posterior, to posterior and right (group III) where the loop of the subgroup d (counterclockwise) almost coincides with the loop of group II (clockwise). For the frontal plane we note three stages in the projection of the loop, all clockwise: first, inferior, left and right; second, superior and predominantly right; third, right and predominantly superior. Comparative study of the electrocardiogram and vectorcardiogram during the course of the disease case by case enables us to understand the evolution of chronic pulmonary disease up to the appearance of cor pulmonale. There is overall a close correlation between the electrocardiogram and vectorcardiogram. The absence of a uniform pattern in either the electrocardiogram or the vectorcardiogram in cor pulmonale is due to the fact that this heart disease has many etiologies.

Diffuse endomyocardial fibrosis

Abstract Two morphologic types of endomyocardial fibrosis are described. The roentgenographic aspects and the anatomic and histologic data which define them are indicated, making it possible to establish the differential diagnosis between the two anatomic typesduring the lifetime of the patient. The first type includes the cases in which there is cardiomegaly and predominant myocardial fibrosis, independent of endocardial fibrosis. The second type includes the cases in which the heart is normal or nearly normal in outline and in which there is extensive endocardial fibrosis which invades the myocardium irregularly. The hemodynamic alterations are identical in both types.

MALFORMATIONS OF THE PULMONARY ARTERY AND ITS BRANCHES, INCLUDING TWO CASES OF ABSENCE OF THE RIGHT PULMONARY ARTERY: ANGIOCARDIOGRAPHIC AND HEMODYNAMIC STUDY.

Abstract A review of 300 angiocardiograms of cases of congenital heart disease shows the following malformations of the pulmonary artery associated with Fallot-type complex, pulmonary stenosis or atrial septal defect: 2 cases of agenesis of the right pulmonary artery; 19 cases presenting isolated or multiple stenoses of the branches of the pulmonary artery, or changes of caliber and direction of its main branches, or shortness of the pulmonary artery. In one case of atypical Marfans syndrome the pulmonary angiograms showed a peculiar disposition of the peripheral branches, resembling the hanging branches of the weeping willow. The different postvalvular anomalies (agenesis, hypoplasia, coarctations, decreased caliber, and alterations of the division of the branches) result in a unilateral or regional decrease of pulmonary circulation which is proportional to the importance of the vascular branch and the severity of the anomaly. The comparison between the right ventricular and pulmonary artery pressures in the cases of agenesis of one of the main pulmonary branches or in the cases of coarctation of one or several branches shows that the decrease in lung circulation, irrespective of the unilateral or regional changes due to the postvalvular anomalies, depends more upon the low pulmonary artery pressure than upon the right ventricular-pulmonary artery systolic pressure gradient. The association of postvalvular coarctations with obstruction of the ouflow tract of the right ventricle does not influence the right ventricular or the pulmonary artery pressures.

Tetralogy of Fallot: Angiocardiographic, electrocardiographic, vectorcardiographic and hemodynamic studies of the Fallot-type complex

Abstract Clinical, angiocardiographic, electrocardiographic, vectorcardiographic and hemodynamic data concerning patients with tetralogy of Fallot showed that the tetralogy of Fallot must be regarded as a part of a large “spectrum” syndrome (the complex of the Fallot type) which includes the following six groups: (1) classic tetralogy of Fallot; (2) extreme tetralogy of Fallot; (3) acyanotic tetralogy of Fallot; (4) stenosis of the outflow tract of the right ventricle or pulmonary stenosis with interventricular septal defect and no overriding of the aorta; (5) partial transposition of the great vessels with stenosis of the outflow tract of the right ventricle or pulmonary stenosis and interventricular septal defect; (6) single ventricle with pulmonary stenosis. There is a common anatomic denominator: the existence of interventricular septal defect and stenosis of the right ventricular outflow tract or pulmonary stenosis. The groups differ from one another only in the anatomico-functional interrelations of these two factors. The third factor (aortic overriding) may be absent. All groups show a comparable clinical and pathophysiologic picture. This report shows the results of the angiocardiographic, electrocardiographic, vectorcardiographic and hemodynamic studies in patients included in each group of the complex of the Fallot type which illustrate the respective pathophysiologic features.

Intracavitary left ventricular myxoma

Abstract A patient with a proved myxoma of the left ventricle is described. The importance of a well documented phonocardiographic study for a reliable clinical diagnosis, and of left ventriculography for a correct preoperative evaluation of the nature of the lesion is emphasized.

Angiocardiographic studies of valvular malformations in pulmonary stenosis. Relationship to physiologic alterations.

Abstract Selective angiocardiographic study of the pulmonary valves in 28 cases of pulmonary stenosis with normal ventricular septum established five anatomic types of stenosis: dome, diaphragm, deformation of valves, incomplete opening of valves and subvalvular. The anatomic type may influence the degree of dilatation of the pulmonary artery. The poststenotic dilatation of the pulmonary artery is not related to the severity of the stenosis but is related to the direction of the blood jet and, therefore, to the topography of the valvular orifice. In the angiocardiograms of the first type of valvular deformation, the corkscrew effect during ejection is attributed to hypertrophy of the spiral muscles of the infundibulum caused by the strong resistance opposed by the valves to the blood flow. This phenomenon occurs only in the type where the valves still have fair mobility. In all the anatomic types, the pulmonary circulation depends more on the level of the systolic pulmonary pressure than on the systolic pressure gradient between the right ventricle and the pulmonary artery. The morphology of the valvular malformations is a factor to consider in evaluating the hemodynamic changes of pulmonary stenosis.

Selective angiocardiography in the diagnosis of atrial septal defect (ostium secundum type)

Abstract The authors studied twenty cases of atrial septal defect (ostium secundum type), simple or associated with anomalous drainage of the pulmonary veins. The position of the catheter at the level of the septum when it is introduced via the saphenous or antecubital veins may permit the differential diagnosis between ostium primum and ostium secundum. The angiocardiographic characteristics of atrial septal defect, when the contrast medium is injected into a pulmonary vein, left auricle, left ventricle or right ventricle, are described. Angiocardiography makes it possible to establish the size of the atrial septal defect. The results of angiocardiography are more valuable if supplemented by a study of the position of two catheters introduced simultaneously via the saphenous and antecubital veins.