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We studied ten patients with unilateral absence of main branch of pulmonary artery (UAMBPA). Six patients with absent right pulmonary artery (ARPA) and four with the left pulmonary artery (ALPA). The presence of either atrial septal defect, ventricular septal defect and patent ductus arteriosus alone or combinated were associated with pulmonary hypertension (mean pressure of the pulmonary artery greater than 48 mm Hg). The patients without associated cardiac defects were asymptomatic and with little elevation of the pulmonary arterial pressure (mean pressure of the pulmonary artery less than 26 mmHg).

It is presented a pulmonary artresia case with untouched interventricular partition wall surgically treated. It was established the continuity between right ventricle and pulmonary artery's trunk with duramater ceiling, for which it was made a pulmonary valve. Postoperation evolution was excelent and it was obteined a frank diminution of the right ventricle's systolic pression. It is discussed about therapeutic conducts in this kind of congenital badformation.

Four cases of crossed atrioventricular connections are described. All of them were diagnosed at cardiac catheterization by angiocardiography and one was examined pathologically. Two possessed situs solitus, one with concordant connections and the other with discordant connections; the other had two situs inversus, both of them with concordant connections. Two had double-outlet right ventricle, one had transposition of the great arteries, and the other had normally related and connected great arteries. These cases have been interpreted as representing abnormal rotation of the ventricles following sepatation. A review of 36 cases previously reported on and our own cases, suggests that most patients have concordant atrioventricular connections. There are many types of ventriculo-arterial connections, the most frequent being transposition of the great arteries. There has not been any case reported with persistent truncus arteriosus. On the basis of atrioventriculo-arterial connections, we propose a classification for this malformation. We discuss the importance of the bulboventricular loop in the type of atrioventricular connections, some clinical implications for the diagnosis, and analyze the value of the rules to localize the ventricles by means of the position of the great arteries.

The results and value of conventional and axial angiocardiography were studied in 26 cases of atrioventricular discordance (AVD). The angiographic anatomy of this malformation was analyzed to determine the precise characteristics of atrioventricular and ventriculoarterial connections. The left and right ventricles in atrial situs solitus and levocardia show angiographic features that differ from those seen in atrial situs solitus and dextrocardia. The same differences are found in atrial situs inversus. Regardless of the type of ventriculoarterial connections, the ventricles maintain the same angiographic characteristics. The ventricular septum in AVD in situs solitus and levocardia and situs inversus and dextrocardia has a peculiar orientation. The use of axial angiocardiography facilitates detection and localization of the ventricular septal defect. In cases of malposition of the heart, the conventional frontal view allows visualization of the ventricular septum in all of its length. The axial projection is useful in diagnosing left ventricular outflow tract obstruction.

We studied 15 patients with the diagnosis of tetralogy of Fallot by means of conventional and axial angiocardiography. Axial studies were performed in the four chamber view (vertical and horizontal X-ray beams) and in the sitting-up view (vertical X-ray beam). With the right ventriculogram we analyzed the anatomic characteristics of this chamber, infundibular stenosis and the pulmonary branches. Left ventriculograms were performed in order to rule out other less common associated defects. In the sitting-up view we analyzed the characteristics of the right ventricular infundibulum and the pulmonary valve, artery and branches. None of our patients underwent aortography or coronary artery angiography. We conclude that utilization of axial angiocardiography is useful in tetralogy of Fallot because it permits a better appreciation of the anatomy of this malformation, the presence or absence of stenosis of the pulmonary branches and other less frequent associated defects. Aortography or coronary artery angiography must be performed in children in which the anatomy of the coronary arteries cannot be determined by either left or right ventriculograms.

We studied 15 cases of the scimitar syndrome. The diagnosis was suspected in 87% of the patients by x-rays studies. Bronchography is useful in order to detect bronchial anomalies, such as hypoplasia or agenesis of the medial lobe which is responsible for the left isomerism. The hemodynamic study is important in symptomatic patients in order to demonstrate associated congenital heart disease as well as the degree of pulmonary hypertension. Angiography is fundamental in determining the anomalous venous drainage of the right lung, whether complete or partial, and in demonstrating the exact site of the connection which may be to the right atrium or more frequently to the inferior vena cava above or below the diaphragm. There are variant forms in so far as the pathway, extension and connection of the anomalous venous trunk which gives rise to the scimitar sign. This syndrome is due to a persistent connection of the right pulmonary veins to the proximal portion of the right vitelin vein which normally forms the suprahepatic segment of the inferior vena cava. Asymptomatic patients should have medical treatment symptomatic ones with an associated congenital heart disease and moderate pulmonary hypertension must be subject to surgery.

The "inverted" pattern of the normal ventricular pressure curves (VPCs) -- as herewith defined -- is considered a characteristic finding in patients with uncomplicated transposition of the great arteries (TGA). In more than 2,000 consecutive cases of congenital heart disease, this pattern was recorded in 6 patients. In all of them situs solitus was present with a large atrial septal defect (ASD) as the sole and obligatory defect. It is shown by angiocardiography in the "systemic" (venous) ventricle in all patients, that this ventricle is: I) the angiographic right ventricle in 5 patients, and II) the angiographic left ventricle in 1 patient. In I TGA with atrio-ventricular concordance (classic TGA) is diagnosed, while in II atrio-ventricular discordance with no TGA (isolated ventricular inversion) is diagnosed. In both instances a Mustard-type of operation is indicated. "Inverted" patterns of different magnitude are found. Therefore, as long as the pattern of VPCs is "inverted", differential diagnosis should be made during life between I classic TGA, and II isolated ventricular inversion.