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The congenital absence of the pulmonary valves is a rare malformation that mainly affects children, frequently is associated with other cardiac malformations and very rarely is present in adults. Elective or urgent surgery is indicated in the symptomatic child; on the other hand, surgical treatment in the asymptomatic adult with this anomaly is controversial. Based on the reported experience in the literature, in the present article, the authors discuss the therapeutic approach of an asymptomatic 22-year-old male with pulmonary valvular absence with practically no hemodynamic repercussion. Although there had been few reported cases of isolated pulmonary valvular absence, the authors concluded that surgery is not a satisfactory solution and that it might be postponed to the time that patients develop congestive heart failure and or respiratory insufficiency. In order to detect that on time, we recommend a follow up based on periodic echocardiographic studies.

We describe two patients with under-and-over ventricles. Both were diagnosed angiographically. One has atrial, situs solitus with discordant atrioventricular and ventriculoarterial connections and a ventricular septal defect. The second has atrial situs inversus with concordant atrioventricular connections and double-outlet right ventricle. We review the literature and analyze the probable embryogenesis of the malformation.

A case of congenital intrapericardial aneurysm of the left atrium associated with functional mitral insufficiency is described; it was resected successfully. Clinical, radiographic, vecto-electrocardiographic, ecocardiographic and angiocardiographic findings are shown. Those are compared with those of other nine similar cases. The finding of qR or QS complexes in L-I and a VL in the electrocardiogram as a sign of left atrial enlargement and eco-fre space posterior to the left ventricular endocardium in the ecocardiogram is mentioned as useful data in the diagnosis of left atrial aneurysmal dilatation not previously reported. Considering that the surgical result is always good, it is concluded that the congenital intrapericardial aneurysm of the left atrium is a rare malformation which needs to be resected irregardless of the presence or absence of arrhythmias, embolisms or heart failure.

We are reporting a case of Ebstein's anomaly of the tricuspid valve and another case of a congenital double tricuspid lesion, both associated with pulmonary valve stenosis and with similar hemodynamics and physiopathology. Given that there are few reported cases in the literature, we analyzed the clinical and electrocardiographic findings of both. The utility of the echocardiogram is discussed in the diagnosis of such abnormalities and the angiographic and hemodynamic data commented upon. It is emphasized that right ventricular hypertension (105 mm Hg, and 98 mmHg, respectively) modifies the cardiopathic hemodynamics and its natural history, that was favorably modified by isolated corrective surgery of the pulmonary obstruction. The postoperatory transvalvular gradients were measured at 27 and 11 mm Hg, respectively. After having revised the pertinent bibliographic literature, we commented upon the factors that give such a high mortality rate in Ebstein's surgery and the congenital abnormalities which are most frequently associated.

The authors analyze the pre and post-operative hemodynamic picture of 30 children and adolescents with mitral valve replacement, which corresponds to a group of 106 patients operated on from 1964 to 1974. At variable time periods, between 9 days and 48 months (average 28 months) from the valve replacement, a new catheterization was done. The majority of the studies were done in the period between 13 and 36 months. Of the 30 cases, 21 were carriers of the Starr-Edwards valve, and the rest of different types. The Starr-Edwards valves produced a lawering of the CVP from 23.0 to 11.0 mm. Hg (average values) whereas with the other types the average pre-operative figure was 21.0 and in the post-operative period it fell to 11.0 mm. Hg. In the group with SE valves, pre-operative SPP was an average of 64.3 and in the post-operative period it was 32.0 mm. Hg. In the cases of SE valves the pre-operative total pulmonary resistances were 12.3 units and post-operatively they fell to 3.7 units. With the other types of valves the pre-opeative average was 10.9 units and post-operatively it was 5.3 units. The hemodynamic results obtained over a period of time with valvular substitution were similar in the distinct types of valves. However in the SE the benefits resulted more constant. The value of radiological and electrocardiographic study in order to predict the early post-operative hemodynamic changes is discussed. These studies serve essentially for a long range evaluation, whereas the hemodynamic, and clinical improvement maintained a strict relationship. This work shows that, in children and adolescents, the presence of severe venous-capillary and arterial pulmonary hypertension, and the elevated pulmonary resistences, are not contraindications for surgical treatment. It equally proves the eficacy of the valvular replacement, when the surgical indication and the control of the patients are handled in the proper way.

The relationship of the great arteries with their respective ventricles in the three toncoconal morphologies depends on the orientation and rotation of the septum of the trunk and cone. In this work we study a case of crossed great arteries and another of partial distortion of the great arteries and one of transposition of the great arteries, all with arterioventricular concordance. In the anatomic and angiocardiographic examples an analysis is made of the arterioventricular relation in each type of arterial pedicle. We say that arterioventricular concordance exists with crossed great arteries when the pulmonary originated from the anterior infundibulum and its direction of right to left or from left to right in the frontal plane shows the spacial position of the anatomically right ventricle. In the transposition of the great arteries or in the partial distortion of the great arteries with arterioventricular concordance the aorta in the frontal plane shows the spacial position of the anatomically right ventricle. In the first group the aorta originates from the anterior infundibulum while in the second, from the posterior infundibulum. We review the examples of arterioventricular discordance diagnosed by necropsy or by laboratory studies. We study 5 cases; 1 with crossed great arteries, 3 with transposition of great arteries and the last with partial distortion of the great arteries. In the arterioventricular discordances with crossed great arteries in the lateral position, we observe that the pulmonary artery originates from the anterior infudnibulum whereas in the transposition, the aorta emerges from the anterior infundibulum. In the partial distortion of the great arteries the vessels are side by side or the aorta a little bit anterior to the pulmonary which is connected with the anterior infundibulum. In the posteroanterior incidence when there exists an arterioventricular discordance with crossed great arteries, the direction of the pulmonary does not indicate the spacial position of the anatomically right ventricle. In the same way, in the transposition and in the partial distortion of the great arteries the aorta does not indicate the spacial position of the anatomically right ventricle. On the basis of the evidence obtained by experimental and descriptive embriology, the morphogenetic processes responsible for the arterioventricular relations are presented. The anatomic specimens of each example is analyzed and the adequate parameter for the diagnosis of the distinct varieties of arterioventricular relations are presented. Finally a review is made of the few cases found in the literature and the differential diagsis between the concordant and discordant troncoconal malformation is established.

Ten patients with duplication of the atrioventricular valves were studied, nine of them with the double orifice located in the mitral valve and the other one in the tricuspid. In two cases the diameter of the secondary valve was the same as that of the normal valve, and in the other 8 cases, it was smaller; each had its own valvular apparatus with chordae tendinae attached to papillary muscles located in different sites. The principal malformation in nine was ostium primum. One of the cases with mitral duplication had pulmonary atresia, an association which has never before been published. In five patients regurgitation of the secondary orifice was present; the diagnosis was made during surgery in nine cases and at necropsy in one. The orifice was sutured in three cases because of regurgitation. Four patients died in the immediate post-operative period, including the one with pulmonary atresia. The other three had pulmonary hypertension. These findings agree with the literature. The etiological hypotheses are discussed and emphasis is given to the importance of the finding of a narrow jet of contrast material directed upward and to the right in the left angiocardiogram in frontal plane. The authors believe that this may be an important sign in diagnosing cases with ostium primum septal defect; taking into account the fact that double mitral orifice is the cause of valvular insufficiency, this can be the site of bacterial endocarditis and usually needs to be surgically closed.

This paper manifests the importance of the systemic-pulmonary collateral circulation in congenital heart diseases. It shows that through the angiographic and hemodynamic study you should recognize the number, origin, course, and size of each of these arteries because during the corrective surgical treatment of certain types of heart diseases, these arteries should be tied off to prevent cardiac overload during the procedure and to prevent intrapulmonary short circuits from left to right in the postoperative period. The knowledge of the central and peripheral pulmonary arteries is important and because of this it should be remembered that the area of the section of the pulmonary arteries, right to left, together should be at least the equivalent of half of the area of the section of the aorta in its diaphragmatic level, the diameter which permits an acceptable cardiac output after the intervention. If the arteries are adequate a plan of surgical intervention which permits the control of the collateral systemic-pulmonary great arteries should be drawn up.

We studied 72 healthy subjects; 31 of them were adults and 41 children. By means of two-dimensional echocardiography we obtained a short axis view at the papillary muscle level of the ratio of the thickness (h) of the ventricular wall and the radius (r) of the cavity. We analysed ventricular performance determinants (pre-load, after-load and contractility). This non-invasive method gives information similar to pressure-volume curves. Thus, we propose it for the study of left ventricular overloads.

A study was made of the electric activity of the atrioventricular (AV) conduction system in basal conditions and with atrial stimulation in order to discover the type, severity and prognosis of the AV disturbance in cases of total correction of tetralogy of Fallot. The authors studied thirteen patients from the Paediatric Department of the National Institute of Cardiology who had undergone such surgery, 7 males, 6 females from 3 to 22 years old. All presented advanced RBBB and sinus rhythm; in the immediate postoperative period, one presented complete transitory A-V block which required a stand by pacemaker; in 2 cases there was a first degree AV block; in two other, LAH and LPH. In 5 cases atrial stimulation was made with single charges and progressive frequency. Measurements were taken at the customary intervals. In basal conditions, prolongation of the AH interval was only found in 2 patients, both with first degree AV block in the peripheral EKG; both patients were taking digitalis. The HV interval was longer than normal in 3 cases, with one of these showing also a lengthened AH interval. The atrial stimulation showed prolonged ventricular activation in 2 cases, one of them with normal basal HV interval. The authors conclude that this procedure permits: 1) The discovery of alterations in the AV conduction system which could not be found in a peripheral EKG. 2) Localization of the site of the lesion. 3) The finding of disturbances of the ventricular conduction by atrial stimulation. 4) Identification of those patients who, after a complete correction of tetralogy of Fallot, show a potential high risk of developing complete complete AV block or of sudden death. 5) The establishment of a real prognosis in these patients.

Fibrous sub-aortic stenosis in 19 patients surgically treated is discussed. The clinical, radiologic, EKG and cardiac catheterization data in the pre and post-operative evaluations are reviewed. There were no surgical deaths in this series. In 14 cases post-operative cardiac catheterization was carried out, in 11 patients a significant decrease in the gradient was found. In 4 cases pharmacological testing was done during catheterization before and after surgery, without significant change in gradients.

Due to the lack of uniformity in the criteria for formulating the diagnosis of the syndrome of heterotaxy, 12 cases with this abnormality were reviewed. The patients were selected on the following basis: symmetrical liver, changeable P waves in consecutive electrocardiograms, bronchial isomerism, hematological disturbances, anomalous relationship of the inferior vena cava and abdominal aorta, anomalous systemic and pulmonary venous drainage and complex cardiac malformations. The most frequent findings were: symmetrical liver, changeable P waves, anomalous systemic venous return, anomalies of the atrio-ventricular valves, particularly atrioventricular canal, aorto-cava juxtaposition, single atrium, anomalous pulmonary venous return, transposition of the great arteries and pulmonary stenosis. Less frequent anomalies were: atrial and ventricular septal defects, atrial isomerism, truncus arteriosus and partial distortion of the great arteries. The hematological disturbances as well as the radioisotope scanning of the liver and the spleen were of little help. Suggestions are given for the diagnosis of the syndrome and for the evaluation of present diagnostic procedures making necessary to use the data gathered with more precision.

Four hundred patients with atrial septal defect treated surgically were reviewed. Thirty five (8.7%) developed arrhytmias post-surgery which persisted for over a year. Sinus bradycardia was found in 10 patients, nodal rhythm in 21, and atrial fibrilation and flutter in 4 patients. Thirty five per cent of the patients with late arrhythmias developed related symptomatology. In 14 patients the function of the sinus node was studied with electrical stimulation of the atrium and with His registry. The interatrial conduction time, AV node and His Purkinje were analized employing various stimulation frequencies. All the cases studied had normal intra-atrial conduction; the response of the atrio-ventricular node to increasing frequencies was normal, an the intraventricular conduction remained constant. In 8 patients (52%), alterations of the sinus node were found; these consisted of prolonged post-stimulation pauses, Wenckebach's type sinoatrial block and suppression of sinus automatism employing vagal procedures or through electrical stimulation. A patient with severe bradycardia detected by dynamic electrocardiography had to be treated with a permanent pacemaker. We confirm that these arrhytmias are not produced by lesions of the internodal tracts, and that an alteration of the sinus node is frequent without a concomitant lesion of the intraventricular pathway. The lesion to the nutrient artery could be due to trauma and/or surgically induced. The response to anticholinergic drugs was good. Prolonged observation of these patients could increase the morbility of these arrythmias and raise doubts of the surgical indications in cases with moderate hemodynamic repercussion.

The authors reviewed 40 cases of endocardial cushion defect, 26 with the complete type and 14 incomplete. In 34 cases the diagnosis was corroborated anatomically and in the rest through angiocardiogram. 2. There is a discussion of proposed classifications and in accordance with the abnormality of the endocardial cushions, they outline the anatomic varieties. 3. They show that the complete and partial forms have notable differences in their clinical picture and in the prognosis. Particularly when there are associated malformations. They also give a detailed account of the essential data for the differential diagnosis. 4. In those cases with the partial type, the electrocardiogram showed typical behavior, whereas, in those with complete type the results were related to the associated malformations. 5. The anatomy of the persistence of endocardial cushion defect is analyzed and taking this into consideration, the problems which occur with mitral valve replacement are discussed. 6. They emphasize the importance of an excellent angiographic study, which besides its diagnostic value, is fundamental in making a decision in regard to surgical selection and technique. 7. They show the high frequency of malformations which may coexist with Endocardial cushion defect and remind us that as long as we continue to wait for classic data, especially of tb electrocardiogram, and ignore the left ventriculogram to establish the diagnosis, an important number of cases will remain undiagnosed. 8. They show that in the incomplete type there habitually exits situs solitus and the associated malformations are rare and simple; on the other hand in the complete, situs ambiguo was proven in 69.2% and solitus in the rest, and in 92.3% there were complex associated malformations. They affirm that with the suspicion or the confirmation of the complete type consider the possibility of it is imperative to heterotaxia. If this exist and there is low pulmonary flow we should suspect the coexistence with tetralogy of Fallot.

On an experimental basis the authors studied the process of repolarization and depolarization in high frequency electrocardiograms of 30 healthy children. The negativity of the T wave in V1 and V2 corresponds with the repolarization of the left ventricle and the positivity with that of the right ventricle. The vectors of repolarization of both ventricles were identified and found to be situated on a horizontal plane. In all tracings the electrical systole of the right ventricle was found to be greater than that of the left ventricle. The number of components of high frequency tracings in the intrinsecoid deflexion was greater on the right ventricle. The authors suggest that the high frequency electrocardiogram is a valuable procedure in clinical investigation.