Peter Vlad

Anatomic types of congenital dextrocardia: Diagnostic and embryologic implications☆☆☆

Abstract The anatomic findings in 51 necropsied cases of congenital dextrocardia are presented. The diagnostic and embryologic implications of these findings are assessed. A new general anatomic classification is proposed which is equally applicable to right-sided and to left-sided hearts, either with two well-developed ventricles or with single (common) ventricle. The proposed classification, which is specifically designed to facilitate accurate diagnosis of the relative locations of the cardiac chambers by current investigative methods, is based upon two considerations: (1) the type of viscero-atrial situs present, and (2) the type of cardiac loop which coexists. The type of visceral situs, established radiologically, always is the same as the atrial situs. Thus, the visceral situs indicates the atrial locations with virtually complete accuracy. The type of relationship between the great arteries at the semilunar valves, determined angiocardiographically, indicates the type of cardiac loop present and, hence, the relative location of each ventricle. Thus, the great arteries identify the ventricles with great accuracy. Three types of relationship are considered, an appreciation of which greatly simplifies and clarifies ones understanding of congenital heart disease: (1) viscero-atrial; (2) bulboventricular (great arteries to ventricles); and (3) bulboventriculovisceral (types of cardiac loop relative to types of viscero-atrial situs). The malformation which recently has been regarded as “classic dextroversion” very probably is nonexistent. The incidence of the tetralogy of Fallot in dextrocardia has been greatly overestimated, probably because of confusion with transposition, pulmonary stenosis and ventricular septal defect. The mirror-imagery of inversion is considered to be a biologic illusion. Rudimentary spleen (polysplenia) appeared to be a forme fruste of asplenia. Posterior transposed aorta is described.

Sedation of children for cardiac catheterization with an ataractic mixture.

SummaryA mixture of chlorpromazine, promethazine and Demerol was administered intramuscularly as sedation to 670 children undergoing cardiac catheterization.With certain modifications, an arbitrary dosage schedule of 1 cc/20 lb. was employed Infants in poor condition or with cyanosis received 50–80 per cent of this arbitrary dose. A maximum dose of 15 cc was used in patients weighing more than 30 pounds. Those patients who were insufficiently sedated were supplemented with small diluted quantities of the mixture given via the catheter. Such injections should be made into a vena cava rather than into a chamber of the heart.Details of four deaths occurring in the per-catheter and post-catheter periods are presented. RésuméPour permettre de leur faire un cathétérisme car chaque, on a donné à 670 malades dont ľâge variait de 4 jours à 16 ans, un mélange de chlorpromazine, du chlorhydrate de promethazine et du. Demerol Ce mélange (une partie de chlorhydrate de promethazine et une partie de chlorpromazine avec 2 parties de Demerol) a été donné en injection intramusculaire à raison de 1 ml par 20 livres de poids sans toutefois dépasser 2 ml. Chez 26 malades, après ľintroduction du cathéter, on a donné des doses fractionnées par voie endovemeuse. Avant le cathétérisme, ľétat des malades était bon chez 31 9% des cas, passable chez 54.6% et mauvais chez 13 5% Chez 97 3% des cas, cette sédation a été adéquate et elle ne ľa pas été dans un pourcentage de 27%. Au delà de 70% de ces examens ont duré de une heure à deux heures. A la fin de ľexamen, 95.8% des malades étaient en bon état. Les arythmies les plus fréquemment observées furent des extrasystoles, des blocages nerveux et de la tachycardie paroxystique.

DIAGNOSIS OF THE ANATOMIC TYPES OF SINGLE OR COMMON VENTRICLE.

Abstract The present report seeks to determine whether or not one can accurately diagnose the various anatomic types of single ventricle by clinical and laboratory methods. The history, physical examination, chest roentgenograms and electrocardiogram usually were diagnostically nonspecific. Despite its lack of specificity, the electrocardiogram in single left ventricle with an infundibular outlet chamber usually could be understood in terms of the type of bulboventricular loop which was present. Cardiac catheterization often provided the first clue to the presence of a single ventricle: a large left to right shunt at the ventricular level, despite systemic pressure in the “right” ventricle; and an extraordinarily wide range of oxygen content from different sites in the ventricle, and even from the same site. Selective ventricular angiocardiography is the diagnostic method of choice. Two groups can be distinguished: (1) single ventricle with an outlet chamber, and (2) single ventricle without an outlet chamber. Those with an outlet chamber have absence of the right ventricular sinus (inflow tract). This results in a single left ventricle with an infundibular outlet chamber, the relatively frequent type of single ventricle (79% of this series).

Transposition of the Great Vessels

The vast majority of cases of transposition of the great vessels can be diagnosed in the first few weeks of life with electrocardiogram, x-ray and fluoroscope. The clinical pattern and the diagnostic features are described in detail. The degree of cyanosis, the characteristic heart shape and progressive increase in size coupled with generous hilar shadows all suggest this diagnosis. A new suggestion is made regarding possible surgical therapy using an extracorporeal circulation.

Atypical Tetralogy of Fallot: A Noncyanotic Form with Increased Lung Vascularity Report of Four Cases

Only recently has the association of pulmonic stenosis with increased pulmonary blood flow been recognized. Four symptomatic infant cases are described in which the clinical diagnosis was large interventricular septal defect. Hemodynamic study showed the presence of associated pulmonic stenosis. Features suggesting that there is an overriding aorta in these patients, and that they, therefore, form an atypical group within the tetralogy of Fallot, are presented. The diagnosis has been confirmed in one case at autopsy. Their differentiation from clinically similar malformations as well as the surgical problem they pose is discussed.

The electrocardiogram in primary endocardial fibroelastosis.

Primary endocardial fibroelastosis (EFE) in infancy has become a widely recognized pathological entity but it is only latterly that clinical diagnosis has been attempted. Although there are now numerous reports of the clinical picture of the disease, electrocardiographic studies are scanty. Before 1953 there were 30 electrocardiograms of previously reported cases (Weinberg and Himelfarb, 1943; Vulliamy, 1947; Craig, 1949; Glynn and Reinhold, 1950; Stadler et al., 1950; Lewis, 1951; Emery and Illingworth, 1951; Collier and Roshan, 1951; Hill and Reilly, 1951; Edmonds and Seelye, 1951; Blumberg and Lyon, 1952; Johnson, 1952; Bonham-Carter et al., 1952; Prec and Cassels, 1952). Rhyhtm disturbances were noted in fourteen: auricular tachycardia in two; nodal rhythm with short P-R interval in one; and paroxysmal multifocal ventricular extrasystoles in one instance. Alterations in conduction occurred in about one-third of the cases: prolonged P-R interval in six and atrio-ventricular dissociation in five cases; the latter finding was observed at, or shortly after, birth in four tracings. Bundle branch block was rare-left once and right three times. A slight or moderate degree of right axis deviation was present in 14 instances, left axis deviation occurring only once. Flattening or inversion of the T wave in two or all of the standard leads was reported in 10 patients. Since 1952 several complete electrocardiograms have been published (Adams and Katz, 1952, 1953; Clement et al., 1952; Schweisguth and Nouaille, 1952; Lambert et al., 1953; Leteer, 1953; Metianu et al., 1954). It has become increasingly clear in this period that the electrocardiogram will assist clinical diagnosis. Most authors note left ventricular hypertrophy to be present. The purpose of this paper is to analyse the electrocardiographic findings in a relatively large group of cases with post-mortem confirmation.

Selective Angiocardiography in Infants and Children

More complete information of an anatomical and hemodynamic nature has been derived from angiocardiography in cases of congenital heart disease in recent years. It has become clear that, while venous angiocardiography with rapid serial biplane technics and short exposure times usually gives excellent results in small infants (1), absolute clarity on some specific points, and in older patients, is not always obtained. These limitations may be overcome by injection of Diodrast through a cardiac catheter placed in a more central position. The feasibility of intracardiac angiocardiography was first demonstrated adequately by Chavez et al. (2). Jonsson et al. (3) introduced the term “selective angiocardiography” to describe the method of opacifying limited and predetermined portions of the heart and great vessels. This procedure has been used by groups in Europe but at present its value has not been recognized widely on the North American continent. The purpose of this paper is to re-emphasize the usefulness of...

Persistent truncus arteriosus: Report of two cases with right aortic arch

Abstract Two infants with persistent truncus arteriosus and right aortic arch proved by autopsy are described from their clinical and pathological aspects. The electrocardiogram shows in one case predominant right ventricular hypertrophy and in the other case predominant left ventricular hypertrophy, but both have features suggesting biventricular hypertrophy. A venous angiogram in one case reveals clear evidence of the malformation.

Severe systemic hypertension and muscular subaortic stenosis

Abstract The case of a 15-year-old girl with severe systemic hypertension secondary to chronic pyelonephritis and muscular subaortic stenosis is reported. The severity of the obstruction of the left ventricular outflow tract varied according to the blood pressure levels. Uncontrolled hypertension attenuated the auscultatory signs indicative of stenosis. Augmentation of the obstruction, evidenced by intensification of the murmur, was present when antihypertensive drugs produced orthostatic hypotension. No adequate form of palliation could be offered to this patient because of the coexistence of end-stage renal failure and the severe cardiac disease which led to her death.

Angiographic and Catheterization Findings in Patients with Difficulties After Total Correction of Tetralogy of Fallot

Tetralogy of fallot is an anatomically complex intracardiac defect resulting in right-to-left or bidirectional shunts at the ventricular level and decreased pulmonary blood flow. The peripheral arterial unsaturation varies with the size of the interventricular defect and its anatomic relationship to the enlarged aortic root, as well as with the severity of the right ventricular outflow obstruction. The muscular hypertrophy of the right ventricle may be pronounced. Total repair of the anomaly achieves physiologic correction by separation of the intracardiac streams and by relief of the pulmonic stenosis. The repair may or may not result in perfect anatomic or entirely normal intracardiac pressure relationships. Of 124 patients operated upon, there were 102 long-term survivors in this series. Twelve of these were restudied by cardiac catheterization and angiocardiography performed from two months to ten years after total correction of the defects. The most common indication for re-examination was the presen...