Benjamin M. Gasul

Premature narrowing or closure of the foramen ovale

P remature narrowing or closure of the foramen ovale is an infrequent but not rare anomaly. We have found 25 cases reported in the literature, the details of which are presented in Table I.1-21 Analysis of 1,150 cases of congenital heart disease studied at the Congenital Heart Disease Research and Training Center revealed 10 examples of this anomaly. This is a report of the pathologic anatomy in these 10 cases as studied quantitatively by a method previously reported.22 In addition, this is a clinical-pathologic study of one case in which the diagnosis was strongly suspected clinically.

Congenital aplasia or marked hypoplasia of the myocardium of the right ventricle (Uhl's anomaly). Clinical, angiocardiographic, and hemodynamic findings.

Summary The previously unreported angiocardiographic and hemodynamic findings in a proved case of congenital aplasia or marked hypoplasia of the myocardium of the right ventricle are presented. The clinical picture consisted of progressive heart failure in infancy, marked cardiomegaly, feeble triple heart tones, no significant murmur, and absence of clinical evidences of right ventricular hypertrophy, pulmonary hypertension, or tricuspid regurgitation. The chest roentgenograms demonstrated marked cardiac enlargement with normal or diminished pulmonary vascular markings. The electrocardiogram showed right atrial hypertrophy and left ventricular hypertrophy. Cardiac catheterization revealed normal systolic pressures and markedly elevated presystolic waves in the right ventricular and pulmonary arterial pressure curves which were identical with the right atrial “a” waves. We have not encountered nor have we seen any reports in the literature showing identical curves. The angiocardiograms demonstrated enlarged right heart chambers, diminished pulmonary blood flow, unusual thinness of the right ventricular wall, and conspicuous absence of it trabeculae. We believe that the correct diagnosis may be derived from the clinical, cardiac catheterization, and angiocardiographic findings. It is suggested that a superior vena cavaright pulmonary artery anastomosis be performed for palliative treatment of this anomaly.

The Diagnosis of Bilateral Stenosis of the Primary Pulmonary Artery Branches Based on Characteristic Pulmonary Trunk Pressure Curves A Hemodynamic and Angiocardiographic Study

The cardiac catheterization and angiocardiographic findings in 12 cases with bilateral stenosis of the primary pulmonary artery branches are described. Characteristic pressure tracings were consistently obtained from the main pulmonary trunk in all cases. These typical curves were reproduced in animal experiments. Associated lesions with left-to-right shunts and mild pulmonary valvular stenosis did not alter the characteristics of the curves. An attempt is made to explain these hemodynamic phenomena. These tracings obtained from the main pulmonary trunk appear to us to be diagnostic of bilateral stenosis of the main pulmonary arteries even if, as frequently happens, one is unable to enter both pulmonary artery branches during cardiac catheterization or to demonstrate definitely the stenosis of both pulmonary arteries by angiocardiography.

FURTHER OBSERVATIONS ON THE NATURAL HISTORY OF ISOLATED VENTRICULAR SEPTAL DEFECTS IN INFANCY AND CHILDHOOD. SERIAL CARDIAC CATHETERIZATION STUDIES IN 75 PATIENTS.

Serial cardiac catheterization has been performed in 75 patients with isolated ventricular septal defect. At the time of the first cardiac catheterization, 39 were under 1 yearof age, 19 were between 1 to 3 years, 14 between 3 to 10 years, and three over 10 years. In 68 patients the second catheterization was performed 1 to 7 years after the first, and in seven patients a third cardiac catheterization was performed 2 to 5 years after the second.Normal pulmonary artery pressure was observed at the initial study in 25 patients. Of these, only one revealed a slight rise in pulmonary artery systolic pressure by 19 mm. Hg; the rest revealed no significant hemodynamic changes.Mild pulmonary hypertension (31 to 59 mm. Hg systolic pressure) was noted in 28 patients at first catheterization, at which time moderate or severe pulmonary hypertension (60 mm. Hg or more systolic pressure) was present in 22 patients. In 33 of these 50 cases, the initial investigation was done during infancy and not later than the second year. In three fourths of the cases with mild pulmonary hypertension, and in two thirds of those with moderate or severe hypertension studied at this very early age, a significant drop in pulmonary pressure and flow was observed at the subsequent catheterization. This was interpreted to indicate most likely relative reduction of the functional size of the defect during early childhood. In eight patients hemodynamic and angiocardiographic evidence of the ventricular septal defect disappeared, indicating probable functional closure.Progressive pulmonary vascular obstruction was observed in six patients, one of whom already had Eisenmengers complex at the initial examination. All but one, an 11-month-old infant, had significantly elevated pulmonary vascular resistance already present during the first cardiac catheterization. In seven patients with similar findings at the initial examination, striking reduction in pulmonary vascular resistance was observed. The progressive pulmonary vascular obstruction is interpreted to indicate failure of the pulmonary vascular bed to undergo maturation, whereas the diminution in pulmonary resistance observed in the other group is interpreted to indicate delayed onset of pulmonary vascular maturation.The therapeutic significance of these findings is discussed.

Congenital dextrocardia: Clinical, angiocardiographic, and autopsy studies on 50 patients

Summary Fifty cases of congenital dextrocardia were studied and classified into 5 major types. In Type I dextrocardia (mirror-image dextrocardia) the anatomic right atrium and right ventricle are situated to the left of an anterior to the corresponding systemic chambers. A mirror-image arrangement of the cardiac chambers is present since the frontal relationship of the chambers is reversed, yet, the anteroposterior arrangement is normal. In Type II dextrocardia (dextroversion complex) the relations of the cardiac chambers are normal; the right atrium and right ventricle are situated to the right of and posterior to the corresponding systemic chambers. In complete dextroversion the cardiac apex is situated anteriorly and to the right; in incomplete dextroversion or mesoversion, it is located in the substernal region and the longitudinal axis of the heart is parallel to the midsagittal axis of the chest. Type III dextrocardia (mixed dextrocardia) is characterized by inversion of the atria alone or of the ventricles alone. The arrangement of the cardiac chambers is therefore in part similar to that of Type I and of Type II dextrocardia. In Type IV dextrocardia (congenital dextroposition) the heart is in the midchest, but the arrangement of the chambers is normal and the cardiac apex is still directed to the left and anteriorly. Types I, II, III, and IV represent the intrinsic group of dextrocardia, since the cardiac heterotaxy is caused by a developmental anomaly of the primitive heart tube. In Type V dextrocardia (congenital extrinsic) the abnormal position of the heart is due to its displacement by congenital anomalies of the lungs, diaphragm, or chest cage. The rightward displacement may be in the frontal plane only (simple dextroposition) or may occur in both the frontal and horizontal planes (dextroposition with pivotal rotation). The various types of dextrocardia are illustrated by angiocardiograms. The incidence of cardiac anomalies in theintrinsic group was 79 per cent, and in the extrinsic group 24 per cent. These anomalies were, as a rule, multiple and severe and together formed specific anatomic syndromes usually characterized clinically by dextrocardia, cyanosis, and diminished pulmonary blood flow. The most common entities encountered were tetralogy, single ventricle with pulmonary stenosis, and tricuspid atresia or stenosis. The chest roentgenologic findings in Types I, II, and III are similar in most respects, consisting of an elevated left diaphragm and identical cardiac configurations in the posteroanterior and in the oblique views which are the reverse of the normal. A right aortic arch is common in Type I dextrocardia. Situs inversus always accompanies this type. The previously reported cases of isolated mirror-image dextrocardia are probably Type II or possibly Type III dextrocardia. In Type IV dextrocardia the heart shadow is medially located in the frontal view and assumes normal configuration in the oblique chest roentgenograms. Negative P waves in Lead I generally indicate atrial inversion, as occurs in Type I and occasionally in Type III dextrocardia, but they may be observed in other conditions not associated with atrial inversion as in nodal rhythm, in levocardia, and in atrial tachycardia. On 2 occasions we have observed slightly negative P I waves in dextroversion with marked right atrial enlargement. The rSr′ ventricular pattern in Lead I as well as the interchanged tracings of Leads aVR and II with those of Leads aVL and III, respectively, is typical of uncomplicated Type I dextrocardia. In the presence of right ventricular hypertrophy a constantly observed change is a relatively tall R wave in Lead I of the QR or qR pattern. The tracings in complete dextroversion characteristically consist of flattened but upright P waves in Lead I and deep Q waves in Leads I, II, and aVL with negative T waves. Similar tracings are seen in Type V dextrocardia when the dextroposition is accompanied by severe pivotal rotation. The T waves in these cases are, however, more often upright than negative. Only 1 of the 5 cases with incomplete dextroversion had similar abnormal Q waves in Leads I and aVL; a qRs pattern in V 2 through V 6 was present in 3. There are no typical electrocardiographic findings in Type III dextrocardia. The tracings in Type IV dextrocardia are normal. Most of the electrocardiographic changes described may be explained by the abnormal position and location of the cardiac chambers. These are demonstrated by means of schematic vector analysis and illustrated by actual vectorcardiograms.

Systolic clicks: A clinical, phonocardiographic, and hemodynamic evaluation

Abstract This study was comprised of 809 phonocardiographic tracings on a total of 598 patients, of whom 135 revealed early systolic clicks, and 11 more showed mid or late systolic clicks. Detailed clinical and phonocardiographic characteristics of the systolic clicks were noted, and the more significant early systolic clicks differentiated from the comparatively benign ones occurring just before mid-systole, in mid-systole, and in late systole. Clicks of the latter type were encountered mostly in normal hearts, but they were also found in abnormal states. The early systolic clicks were noted to occur in anomalies involving congenital malformations of the stenotic type in the aortic and pulmonary valves, and in those involving dilatation of the aorta and pulmonary artery. The aortic clicks generally tended to be of maximal intensity at the apex and varied little during respiratory cycles. The pulmonic clicks were maximal at the second left intercostal space parasternally and were loudest during expiration, sometimes disappearing completely with inspiration. The incidence of early systolic clicks was appreciably high in congenital aortic stenosis, while they were present in about half the cases of congenital isolated pulmonary stenosis. The latter anomaly in a majority of the cases was of the mild to moderate type. In these conditions the early systolic clicks always preceded the ejection murmurs. In congenital aortic stenosis the aortic closure was unusually loud in most instances; in congenital pulmonary stenosis a few cases revealed an abnormally loud pulmonic closure. Of the anomalies involving the aorta the early systolic clicks were found to be quite consistently present in cases of truncus communis, but were also noted in extreme cases of tetralogy of Fallot and only occasionally in coarctation of the aorta, transposition of the great vessels, tricuspid atresia, aortic regurgitation, and atherosclerosis of the aorta. Dilatation of the pulmonary artery associated with Eisenmengers physiology, Taussig-Bing complex, and idiopathic dilation of the pulmonary artery revealed that early systolic clicks were a relatively constant finding in these conditions. Pulmonary hypertension at less than systemic levels and pulmonary dilatation secondary to large left-to-right shunt with normotensive pressures were sometimes noted to be associated with early systolic clicks. With respect to timing the ejection component of the first heart sound, as well as the aortic clicks, the concept of isometric rise of aortic pressure as reflected in simultaneous indirect arterial tracings is discussed. The early systolic clicks were considered to be a pathologic manifestation of the second major, or ejection, component of the first heart sound and, depending on their origin, to reflect the isometric contraction period or beginning of the ejection phase of either ventricle. In congenital aortic stenosis and valvular pulmonary stenosis the early systolic clicks seemed to originate at the valvular level, occurring after the atrioventricular valve closure at a mean time interval of 0.055 and 0.033 second, respectively. In other conditions the vessel wall of the aorta or the pulmonary artery appeared to be their seat of origin. The mode of occurrence of the more benign mid and late systolic clicks could not be ascertained. The presence of early systolic clicks was considered an abnormal finding in itself, and their proper evaluation was regarded as being of significant diagnostic importance. Their absence did not imply exclusion of any given entity or hemodynamic state.

PULMONARY ARTERIOVENOUS FISTULA AND TELANGIECTASIA

Excerpt A fascinating chapter in the diagnosis and treatment of an uncommon pulmonary vascular lesion—pulmonary arteriovenous fistula—has been written since the turn of the century. In this disease...

The Diagnosis of Aortic Septal Defect by Retrograde Aortography Report of a Case

This rare congenital malformation is a round or oval opening between the ascending aorta and the main pulmonary artery above the semilunar valves. It is practically impossible to differentiate this malformation from a patent ductus arteriosus clinically because both malformations may present the same physical, fluoroscopic, roentgen and electrocardiographic findings. Even angiocardiography and cardiac catheterization do not differentiate these congenital malformations. The authors have studied 2 patients with aortic septal defects. One was operated at another clinic for a suspected patent ductus arteriosus, and the other case presented here was diagnosed by retrograde aortography.

Evolution of the frank vectorcardiogram in normal infants

HE Frank system of electrode placement for vectorcardiographyl combines theoretical accuracy and ease of clinical application. The normal evolution of the vectorcardiogram during infancy and childhood using Grishman’s cube electrode arrangement2 has been reported on by several workers.3-7 Because of the increasing frequency with which the Frank method is applied in children, an attempt was made to determine the changes in the Frank vectorcardiogram associated with growth and to define the normal characteristics in different age periods in infancy.

A case of tetralogy of Fallot with a patent foramenovale (pentalogy) showing a marked left ventricular hypertrophy and left axis deviation

Summary The clinical and pathologic findings in a case of tetralogy of Fallot associated with patent foramen ovale are presented. It is believed that this is the first reported case of a tetralogy of Fallot with a hypertrophy of the left ventricle and a left axis deviation substantiated by autopsy.