Common Atrioventricular Valve Research Articles

Echocardiographic classification of complete atrioventricular canal defects

M-mode echocardiographic records of 26 patients with surgically proven complete atrioventricular canal defect were reviewed. Fragmentation of the interventricular septum and anterior displacement of the mitral annulus into the left ventricular outflow tract were noted in all patients. In 25 of 26 a common atrioventricular valve leaflet echo could be identified in the left ventricular outflow tract at the area of the crest of the interventricular septum. Recordings of echographic scans performed in the area of the left ventricle demonstrated separate “mitral” and “tricuspid” contributions to atrioventricular valve echoes in all 13 patients with type A defect, and a single common atrioventricular valve leaflet in 10 of 11 patients with type C complete atrioventricular canal. Two patients with type B defect had findings intermediate between these two patterns. M-mode echocardiography presumptively diagnosed complete atrioventricular canal in all 26 patients and diagnosis was definitive in 25 of 26. In addition, echocardiographic atrioventricular valve patterns permitted anatomic classification in the large majority of cases.

Contrast echocardiography in single or common ventricle.

Common ventricle is a rare congenital anomaly in which the ventricular chamber receives blood from two separate atrioventricular valves or from a common atrioventricular valve. We used contrast echocardiography during cardiac catheterization to confirm ventricular anatomy and to characterize blood flow dynamics in 35 patients with common ventricle. After injections of dye, a cloud of echoes anterior to the mitral valve echo during the rapid inflow phase of ventricular diastole is indirect evidence of common ventricle with two atrioventricular valves. Common ventricle with an outflow chamber is characterized by a smaller ventricular chamber visualized anterior to both atrioventricular valves which opacifies with subsequent ventricular systole. The arrival of all dye posterior to the only recorded atrioventricular valve further established the presence of a single atrioventricular valve. These contrast flow patterns gave greater specificity to the standard M-mode echocardiographic assessment of patients suspected of having common ventricle.

Common or single ventricle. An angiocardiographic and hemodynamic study of 42 patients.

To correlate anatomy with hemodynamics, the angiocardiographic findings were reviewed in 42 patients with common ventricle (CV). Nine had normally related great arteries (NRGA), 12 d-malposition, 21 l-malposition and 5 a common atrioventricular valve. Selective outlet chamber (OLC) angiocardiograms were available in 14 out of 29 patients with OLCs. OLC position varied from anterior and to the right of the CV to posterior and to the left of it; two categories (anterior and lateral OLC) were delineated by a line 45 degrees to theleft of anterior in the horizontal plane. The OLC was anterior in all patients with NRGA, lateral in most l-malpositions, and almost equally divided between anterior and lateral in d-malposition (P less than 0.05). Complete hemodynamic data were obtained in 29 patients. Complete mixing of venous return occurred in four patients with atresia of one valve. In the remainder complete mixing occurred in 36%, unfavorable streaming in 12% and favorable streaming in 52%. Semilunar valve position and pulmonary stenosis did not affect the nature of mixing. Systemic arterial (SA) minus pulmonary arterial O2 saturation was positive and significantly higher in patients with malposition with lateral OLCs than anterior OLCs (P less than 0.001). However 79% of SA O2 saturation variation could be predicted from pulmonary and systemic blood flow alone.

臨床 Floating type 完全型心内膜床欠損症手術例の検討

臨床 Floating type 完全型心内膜床欠損症手術例の検討

Single ventricle with transposition.

Fifty-seven cases of single (common) ventricle with transposition of the great vessels are reviewed. The diagnosis had been confirmed by necropsy in 25 and by angiocardiography in 32 cases. Single (common) ventricle is defined as that condition in which both atrioventricular valves separately enter a single ventricular cavity. According to this definition, cases presenting with common atrioventricular valve, atresia of one atrioventricular valve, or straddling valves have been excluded. Two structural types of common ventricle are identified: the left ventricular type and the primitive type of single ventricle. The former showed anatomic features similar to a left ventricle, whereas in the latter, features characteristic of a left ventricle were not present. The latter was considered a more primitive condition than the former. A classification based upon the type of single ventricle and its outflow tract and upon the relationship of the great vessels is presented. Of the 25 cases of single ventricle with transposition studied pathologically, 21 were of the left ventricular type and four of the primitive type. Of the four cases with double conus three were among the four examples of primitive ventricle. Among the 21 cases with a single conus, the type of transposition was about equally divided between the d -type (ten cases) and the l -type (11 cases). Pulmonary stenosis or atresia was observed in seven of the 21 cases with a single conus and in one of the four cases with double conus. In the clinical cases, only angiocardiography could establish the diagnosis and delineate the different types of great vessel-ventricular relationships.

Twin valve: a new approach to single ventricle.

A twin valve with two mounted homograft semilunar valves was designed and constructed aiming to replace the common atrioventricular valve, particularly in case with single ventricle.

Asplenia associated with infradiaphragmatic total anomalous pulmonary venous return and esophageal varices.

SPLENIC AGENESIS is frequently associated with Smultiple systemic abnormalities in which some form of anomalous pulmonary venous return is a common manifestation. However, the association of splenic agenesis with infradiaphragmatic total anomalous pulmonary venous return and esophageal varices presented in this case report is quite unique. Case Report This 61b. 7 oz. male infant was born spontaneously following an uncomplicated thirty-seven-week gestational period. Cyanosis associated with irregular respirations was noticed almost immediately. Physical examination revealed poor aeration of the lungs and an enlarged left lobe of the liver. A systolic murmur became apparent at five days of age and congestive heart failure developed three days later. The electrocardiogram at this time demonstrated an axis of −120° and a qR pattern over the right precordial leads. Multiple x–ray examinations of the chest taken since the first day of life revealed a reticular appearance throughout both lung fields consistent with the diagnosis of total anomalous venous return (Fig. 1). A right ventricular angiogram demonstrated a single ventricle from which both aorta and pulmonary artery arose (Fig. 2, A). A right aortic arch was present and reflux into the hepatic veins occurred. Both veins were symmetrical and equal in size. The pulmonary veins formed a single channel that extended through the diaphragm into distended vessels within the left upper quadrant (Figs. 2, B and C). During this phase a large vessel, presumably an esophageal vein, was also noted within the upper mediastinum (Fig. 2, B). Gastrointestinal studies demonstrated huge esophageal varices (Fig. 3) as well as malrotation of the midgut. Peripheral blood smears, however, failed to reveal Howell-Jolley or Heinz bodies. Chromosomal study was also normal. The patient was treated with antibiotics, digoxin, mist, and oxygen. Despite therapy, severe respiratory distress and generalized cyanosis persisted, and the infant died at three weeks of age. Autopsy confirmed the clinical impression of splenic agenesis. A bilocular heart with a common atrioventricular valve was found. The aorta arose anteriorly and to the left of a transposed pulmonary artery. In addition, a persistent left superior vena cava was present. A large channel draining the anomalous pulmonary veins emptied into gastric varices along the lesser curvature. The distal segment of the gastric vein was atretic. Large esophageal varices arose from the gastric vessels and inserted into the left superior vena cava. Injection of contrast material into the gastric vessels confirmed many of these findings (Fig. 2, C). Both lungs were trilobar. Histologic examinations of the lungs showed dilatation of the lymphatics and venous congestion.

The anomalous relationship of the interior vena cava and abdominal aorta as a specific angiocardiographic sign in asplenia.

The visceral anomaly known as agenesis of the spleen (herein referred to as asplenia) has been adequately described (2). The condition is almost universally associated with multiple and complex anomalies of the heart. These generally include common atrium, common atrioventricular valve, common ventricle, transposed great vessels, severe pulmonary stenosis or atresia, and anomalies of the pulmonary veins. The complex nature of the intracardiac malformations usually precludes correction by surgical technics. Nevertheless, it is important to recognize the syndrome of asplenia in order to exclude other forms of cyanotic heart disease which are amenable to surgical repair. Upon review of the forward angiocardiograms in these cases, it became apparent that the relationship between the inferior vena cava and abdominal aorta was anomalous. Because of this preliminary observation, we were motivated to review the angiocardiograms in several other cases to determine whether or not this anomalous relationship was dia...

Roentgenographic features of common ventricle.

Single or common ventricle is a rare and complex form of congenital heart disease, usually associated with other malformations of the heart and great vessels. At present, no surgical procedure is available to correct this anomaly, and thus its accurate diagnosis is of prime importance. Angiocardiography is the only procedure by which this may be accomplished. This presentation is concerned with the radiographic features of common or single ventricle and their correlation with the abnormal anatomy. Emphasis is placed on the identification of the internal ventricular anatomy, the interrelation of the great vessels as they are seen on the angiocardiogram, and on how these features relate to the different forms of single or common ventricle. Definitions and Terms The pathologic findings in 60 patients with single or common ventricle were reported recently (1), and we shall adhere closely to the definitions and the classification proposed in that report. A single or common ventricle is a ventricular chamber that receives both atrioventricular valves or one common atrioventricular valve. Patients with tricuspid or mitral atresia are excluded from this group. An outlet chamber may or may not be present. If present, both or either one of the great arteries may arise from it. The designation “right atrium” is given to the atrium attached to the inferior vena cava. This structure serves as an important landmark, since the limbus of the foramen ovale, the classic anatomic identification, cannot be identified on angiograms. It is understood that this is not an absolute criterion but a useful approximation, with the possibility kept in mind of anomalous connection or absence of the inferior vena cava. The designation “left atrium” is given the atrium which, unless there is partial or total anomalous pulmonary venous drainage, receives the pulmonary veins. The position of the atria depends on that of the abdominal viscera and is referred to as situs solitus if the right atrium is on the right of the spine, or situs inversus if the right atrium is on the left of the spine. The ventricular chambers are designated right or left, not according to their position in the chest, but according to anatomic characteristics. Thus the term “right ventricle” refers to a ventricular chamber that exhibits some of the following features: (a) a tricuspid atrioventricular valve; (b) a trabecular pattern of the ventricular sinus consisting of relatively few large and coarse trabeculae crossing each other at right angles and located along the free wall of the ventricle as well as on the septal surface; (c) the presence of a bulbar ring consisting of the parietal and septal bands of the crista supraventricularis and the moderator band. This ring separates the right ventricular sinus from the infundibulum; (d) an infundibulum extending from the bulbar ring to the annulus of the aortic or pulmonary valves, of which the septal portion is smooth but the free wall is trabeculated.

THE ROENTGENOLOGIC FINDINGS IN COMMON VENTRICLE WITH TRANSPOSITION OF THE GREAT VESSELS.

The roentgenologic findings in corrected transposition and complete transposition of the great vessels have received sufficient attention to make them relatively accessible to clinical identification (1–7). Little attention, however, has been directed to the radiologic findings of common ventricle, a condition which, while sharing a somewhat similar interrelationship between the great vessels, does not have the same intracardiac characteristics. The purpose of this communication is to describe the roentgenologic findings in the 16 cases of common ventricle available to the authors. As the diagnostic and surgical problems concerned with common ventricle are specific, it is essential that we define the condition as used in this report. In common ventricle with transposed great vessels, there is one ventricular chamber into which both (when there are two) atrioventricular (AV) valves empty. Lying anteriorly at the basal portion of the ventricle, a subdivision, the infundibular chamber, communicates with the main part of the common ventricle. The pulmonary trunk arises from the ventricular chamber, while the aorta arises in a transposed position from the infundibulum. The AV valves do not communicate with the infundibular chamber (Fig. 1). In each case of common ventricle studied at necropsy and angiocardiography, an infundibular chamber was present. This was located anteriorly at the base of the heart in one of two positions. When the infundibulum was at the right aspect of the base, it was considered to be in a “noninverted” position, when at the left aspect, it was considered “inverted” (Fig. 1). These terms seem appropriate since common ventricle with infundibular inversion is the mirror image of common ventricle with noninverted infundibulum. In some cases of common ventricle, additional pathologic conditions may occur, including obstruction to pulmonary flow caused by pulmonary valvular or sub-pulmonary stenosis. As reported by us in an earlier communication (8), common ventricle may occasionally be associated with other abnormalities of the AV valves, such as common AV valve, tricuspid atresia, or mitral atresia. This report, however, deals solely with patients having two functioning AV valves. It is essential, we believe, not to confuse the condition under discussion with others in which there is transposition of the great vessels. Our definition of common ventricle with inversion of the infundibulum does not include corrected transposition, a condition with two ventricles, which anatomically allows a normal route of blood flow. Neither does our definition of common ventricle with noninversion of the infundibulum include complete transposition, a condition with two functioning ventricles, the aorta arising entirely from the right ventricle and the pulmonary trunk entirely from the left ventricle.

Diagnosis of ostium primum defects of the atrial septum

Four cases thought at operation to represent varieties of ostium primum defect and one similar unexplored case are presented together with clinical data, electrocardiograms, x-rays, fluoroscopy, phonocardiograms and catheterization findings. The differentiation of the ostium primum from the more common type defect of the atrial septum is discussed. The majority of the criteria for differentiation depend on the presence of either a cleft mitral leaflet or an incompetent common atrioventricular valve. An interesting but incompletely explained finding is the presence of a remarkably soft apical first sound. It appears likely that the diagnosis can be made preoperatively in the majority of cases. It is probably impossible to distinguish the rare ostium primum defect with normal A-V valves from the usual type of atrial septal defect prior to surgical exploration.

Incomplete division of the atrioventricular canal with patent interatrial foramen primum (persistent common atrioventricular ostium): Report of five cases and review of the literature

This paper is based on a report of five cases ∗ ∗ Since the time of preparation of the manuscript of this paper, we have observed a sixth example. The anomaly was found in a stillborn full-term female fetus. Both the septal leaflet of the tricuspid valve and aortic leaflet of the mitral valve were cleft, and a common atrioventricular valve was present. There was patency of the interatrial foramen primum and a defect in the posterior portion of the interventricular septum. In this connection, the atrioventricular junction was virtually identical to that in Cases 1, 2, and 3. There was also an anatomic patency of the foramen ovale. of the congenital cardiac disease termed incomplete division of the atrioventricular canal with patent interatrial foramen primum (persistent common atrioventricular ostium) and on a review of the essential pathologic and clinical features in an additional fifty cases reported in the literature. In the entire group of fifty-five cases, the median age of the patients at the time of death was 10 months. More than half of the patients died before they were 1 year of age. Only five of the patients lived beyond the age of 30 years. The anomaly apparently does not have any predilection for either sex. In the fifty-five cases, it appears that the lesion under consideration acts essentially as does a simple interatrial septal defect. Enlargement of the right side of the heart and widening of the pulmonary artery orifice are common associated secondary lesions. Cyanosis may be present at birth, but is usually acquired. When this manifestation develops, it is a sign of failure of the right side of the heart, pulmonary disease, or both. Cardiac murmurs, usually systolic, are frequently observed; they occurred in twenty-two of twenty-five cases in which an adequate history was given with reference to this sign. Monogolism is a relatively common associated finding in cases of incomplete division of the atrioventricular canal with patent interatrial foramen primum. In those case reports in which there was an adequate history, mongolism was recorded as having been present in seventeen cases and its absence was recorded or could be assumed in eight cases. Bacterial endocarditis was observed in three of the fifty-five cases.