[Origin of the aorta and pulmonary artery from the right ventricle: anatomic and echocardiographic comparisons]. / Otkhozhdenie aorty i legochnoi arterii ot pravogo zheludochka (anatomo-ékhokardiograficheskie sopostavleniia).

The origin of the aorta and pulmonary artery from the right ventricle is a complicated and little studied congenital cardiac malformation. The A. N. Bakulev Institute of Cardiovascular Surgery, USSR Academy of Medical Sciences, have developed anatomic and morphological criteria for the malformation. With the criteria, echocardiograms from 64 patients were retrospectively analysed and their anatomic and echocardiographic comparisons were made. The findings revealed the echocardiographic measures of the aorta and pulmonary artery from the right ventricle and determines the optimal echocardiographic projections for their estimate.

[Echocardiographic diagnosis of the origin of the aorta and pulmonary artery from the right heart ventricle]. / Ekhokardiograficheskaia diagnostika otkhozhdeniia aorty i legochnoi arterii ot pravogo zheludochka serdtsa.

The origin of the aorta and pulmonary artery from the right ventricle is a congenital heart disease whose anatomical and echocardiographic criteria are the object of wide current discussion. The classification of the disease has been developed at the Bakulev Institute of Cardiovascular Surgery, USSR AMS. It is based on identification of the anatomical differences in the types of origin of the aorta and pulmonary artery from the right ventricle (RV OAPA). The present research was devoted to the study of the possibilities of echocardiography in the differential diagnosis of the types of the anomaly in the RV OAPA complex and the possibilities of noninvasive differential diagnosis of RV OAPA and adjacent anomalies of the conotruncus. The results of the study allow the conclusion that location of intracardiac structures with purposeful use of ultrasonic projections provides the possibility of making the differential diagnosis of the types of the anomaly in the RV OAPA complex and that the obtained echocardiographic criteria may serve for establishing the differential diagnosis of RV OAPA and other congenital anomalies of the conotruncus.

[Anatomic and hemodynamic comparisons in patients with aorta and pulmonary artery arising from the right ventricle]. / Anatomo-gemodinamicheskie sopostavleniia u bol'nykh s otkhozhdeniem aorty i legochnoi arterii ot pravogo zheludochka serdtsa.

The anatomy and hemodynamics were compared in 59 patients with aorta and pulmonary artery origin from the right ventricle. The patients' age was from 3 months to 23.5 years. The pathophysiology of the patients' circulation was determined by many factors, the most important factors were the following: the anatomic type of the malformation, the presence or absence of pulmonary and aortic stenoses and other concurrent congenital heart malformations. The oxygen saturation of the arterial blood was associated with the direction of physiological blood flows in the right ventricle. The most "successful" anatomic and hemodynamic variants of the malformation were types A and B, in which there was no higher blood oxygen saturation in the pulmonary artery as compared to the level of blood oxygenation in the aorta despite the presence or absence of pulmonary stenosis and the site of great vessels. Type C with the inverse site of great arteries is the less "successful" anatomic and hemodynamic variant.

[Surgical anatomy of subaortic conus of the normal heart]. / Khirurgicheskaia anatomiia podaortal'nogo konusa normal'nogo serdtsa.

One hundred and fourty-three normal hearts have been studied. The conoid septum, supraventricular crest, bulboventricular fold, area of the fibrous tissue between the aortal and mitral valves and the anterior interventricular septum make the walls (frontal view, in the direction of the hour hand) of the left ventricular cone. In the normal heart there is no real contact between the fibrous rings of the aortal and mitral valves. They are connected by means of a strip of the fibrous tissue, its size varies. The subaortal cone and deferent part of the left ventricular axes make an obtuse angle; the axes of the subpulmonary and subaortal cones have a cross direction. The geometrically definitive left ventricular cone is seen as obliquely-sectioned overturned isosceles cone, with its big base directed upward, right and back, and the small one forward and left.

[Classification of aortic and pulmonary artery origin from right ventricle]. / Klassifikatsia otkhozhdenia aorty i legochnoi arterii ot pravogo zheludochka.

An original systematics of origin of the aorta and pulmonary artery from the right ventricle (OAPA RV) is suggested on the basis of examination of 63 heart specimens with the anomaly. Two main variants of OAPA RV are distinguished: (1) with and (2) without a formed infundibular septum (IS). Types A and DA belong to the first variant, types B, C, D, and DC to the second variant. Type A is characterized by drainage of the left ventricular (LV) outflow tract (OT) into the subaortic conus. Type DA differs from type A by the existence of an auxiliary opening between the ventricles due to a deficiency of tissue of the posterior (sinus part) of the septum. Type B is characterized by the absence (or marked hypoplasia) of the septum of the conus (SC). The LV OT in this case drains under both (aortic and pulmonary) coni. In type C the LV OT opens into the subpulmonary conus. In distinction from type C, type DC has an auxiliary defect in the sinus part of the interventricular septum (IVS). Type D is characterized by complete obturation of the exit and the absence of a defect in the sinus part of the IVS (type D1: OAPA RV with an intact IVS) or with a sinus defect in the IVS-DIVS (type D2: OAPA RV with noncommitted DIVS). According to the presence or absence of stenosis of the pulmonary artery (PA) or aorta, 4 subtypes are distinguished: (1) without stenosis; (2) with stenosis of PA; (3) with aortic stenosis; (4) with combined aortic and pulmonary stenosis.

[Angiocardiographic diagnosis of the position of the interventricular septal defect in a case of double outlet of the great vessels from the right ventricle]. / Angiokardiograficheskaia diagnostika polozheniia defekta mezhzheludochkovoi peregorodki pri dvoinom otkhozhdenii magistral'nykh sosudov ot pravogo zheludochka.

The paper is devoted to analysis of the results of angiocardiographic investigation of 237 patients with a double deflection of great vessels (DDGV) from the right ventricle (RV) (the patients ranged in age from 3 months to 23.5 years) to study the potentialities of the method in the diagnosis of a defect position of the interventricular septum (DIVS). Angiographic investigation permitted a sufficiently accurate determination of a DIVS position in DDGV from the RV. Of paramount importance for determining a DIVS position is the establishment of the interrelationships of the latter with the infundibular septum and the openings of major arteries. For diagnosis of a DIVS position one should necessarily use left and right ventriculography both in standard and axial projections.

Angiocardiographic diagnosis of ventricular septal defect localization in double outlet right ventricle.

Children with double outlet right ventricle, aged 3 months to 23.5 years, were examined to assess the possibility of angiocardiographic diagnosis of defect localization. It was found angiocardiography allows satisfactory localization of the defect in question. To be able localize the defect, it is critical to determine the interaction between the defect and the conus septum, and the origin of large arteries. To establish the diagnosis of ventricular septal defect, right and left ventriculography in standard and axial projections must be performed.

[Angiocardiography in the diagnosis of interventricular septum defects in a double displacement of great vessels from the right ventricle]. / Angiokardiograficheskaia diagnostika polozheniia defekta mezhzheludochkovoi peregorodki pri dvoinom otkhozhdenii magistralnykh sosudov ot pravogo zheludochka.

The paper is devoted to analysis of the results of angiocardiographic investigation of 237 patients with a double deflection of great vessels (DDGV) from the right ventricle (RV) (the patients ranged in age from 3 months to 23.5 years) to study the potentialities of the method in the diagnosis of a defect position of the interventricular septum (DIVS). Angiographic investigation permitted a sufficiently accurate determination of a DIVS position in DDGV from the RV. Of paramount importance for determining a DIVS position is the establishment of the interrelationships of the latter with the infundibular septum and the openings of major arteries. For diagnosis of a DIVS position one should necessarily use left and right ventriculography both in standard and axial projections.

[Anomalies of atrioventricular valves in origin of the aorta and pulmonary artery from the right heart ventricle]. / Anomalii atrioventrikuliarnykh klapanov pri otkhozhdenii aorty i legochnoi arterii ot pravogo zheludochka.

Anomalies of the atrioventricular valves may influence essentially the choice of the method and volume of surgical intervention in origin of the aorta and pulmonary artery (PA) from the right ventricle (RV). The results of morphological study of 55 heart specimens with origin of the aorta and PA from the RV are analysed; structural anomalies of the atrioventricular valves were revealed in 19 of them (34.5%). The anomalies were surgically significant only in 16.4% of cases. Isolated anomalies of biventricular attachment, simultaneous biventricular attachment and displacement of one of the atrioventricular valves, and anomalous chordae and supernumerary fibrous structures connected to the atrioventricular valves, etc. were the most commonly encountered anomalies of the atrioventricular valves in origin of the aorta and PA from the RV. The frequency and complexity of anomalies of the atrioventricular valves in origin of the aorta and PA from the RV were determined by the morphology of the cardiac complex, the type of the anomaly, and the side involved in the pathology of the atrioventricular valves. The complexity of the atrioventricular valve disorders in such cases could influence noticeably the volume of the surgical intervention. Anomalies of the atrioventricular valves in origin of the aorta and PA from the RV were encountered most frequently in malposition of the heart or discordant atrioventricular connection. The frequency of anomalies of the tricuspid valve was much higher than that of mitral valve anomalies, but the last named were surgically significant in most cases as a rule.

Fontan-type operation for correcting complex congenital defects in a criss-cross heart.

Three cases of criss-cross heart with severe and complex congenital defects are reported. Precise preoperative diagnosis of the morphologic abnormalities made it possible to choose an optimal method of surgical treatment: all patients successfully underwent a Fontan-type surgical procedure.

[Origin of the aorta and pulmonary artery from the right ventricle]. / Otkhozhdenie aorty i legochnoi arterii ot pravogo zheludochka.

In the period of 1980 to February 1988, 60 patients aged 1 year 7 months to 23.5 years were operated on for concordant atrioventricular linkage and origin of the aorta and pulmonary artery from the right ventricle. Sixty-two heart specimens were examined to study the defect anatomy. The left ventricular outlet opened up in the subaortic cone (Type A defect) in 47 (78.3%) patients, in the common subaortic and subpulmonary cone (Type B) in 8 (13.4%) patients, in the subpulmonary cone (Type C) in 3 (5%) patients; 2 (3.3%) subjects exhibited nonconcomitant ventricular septal defect (Type D). Thirty-four (56%) patients had pulmonary stenosis. Concomitant heart diseases were found in 78%. Fifteen patients (25%) underwent radical operation after prior palliative interventions. The total fatal cases were 13.7% (out of 58 operated patients, 8 died) in the group of radically operated patients. The fatal outcomes were observed in Type A defect. The analysis showed that defect correction techniques, age, prior palliative interventions, presence or absence of pulmonary stenosis, concurrent heart diseases, and restrictive ventricular septal defect failed to affect the immediate result of surgeries. An inaccurate preoperative diagnosis and lack of surgical experiment were factors increasing the risk for correction from 1980 to 1986. In the 2 years, the death rates decreased from 17.9% (7 dead patients per 39 operated ones) to 5.3% (1 dead patient per 19 radically operated ones).

[The anatomical criteria of the origin of the aorta and pulmonary artery from the right ventricle]. / Anatomicheskie kriterii otkhozhdeniia aorty i legochnoi arterii ot pravogo zheludka.

New anatomical criteria of the above malformation are created on the basis of the study of 63 hearts with the origin of aorta and lung artery from the right ventricle: the presence of the bulboventricular fold separating the mitral and semilunar valves; complete or partial delay of the subaortal cone movement to the left ventricle; the presence of the completely or partially formed proximal cone; underdevelopment of the left ventricle due to the complete or partial absence of its cone region; the only issue from the left ventricle is the proximal cone apertures or a part of the primary bulboventricular orifice; when the primary bulboventricular orifice is obturated the left ventricle is either deprived of the opening or has it in the form of noncommitted defect in the sinusal part of the interventricular septum; the anterior margins of the bulboventricular fold and cone septum are open and supraventricular comb is not formed; the interventricular septum defect formed due to failure of fusion between bulboventricular fold and cone septum connects the aorta with the right ventricle; aorta and lung artery start completely or partially from the right ventricle.

[What is anomalous departure of the aorta and pulmonary artery from the right ventricle?]. / Chto takoe otkhozhdenie aorty i legochnoi arterii ot pravogo zheludochka.

Available literature data on terminological and anatomical definition of congenital combined valvular disease--anomalous right ventricular origin of the aorta and pulmonary artery--are reviewed. Not a single report analysed permitted an objective precise structural outline of the disease. The causes of this situation are elicited. The review of the 8 Soviet and 55 foreign original sources shows that the question of pathognomonicity of the anomaly anatomical signs remains disputable.