Is there another type of biventricular atrioventricular connection? (original) (raw)
Related papers
Morphologic features of the uniatrial but biventricular atrioventricular connection
Journal of Thoracic and Cardiovascular Surgery, 2007
Objectives: Hearts with an absent atrioventricular connection and a straddling of the solitary atrioventricular valve are rare but significant lesions. They are suitable only for Fontan-like palliation, in which atrioventricular valvar abnormalities play a significant role in determining the outcome. We studied the segmental arrangements in such lesions and clarified the valvar morphology, particularly its surgical implications.
A case of double-outlet left ventricle with atrioventricular discordance
The Turkish journal of pediatrics
The clinical, echocardiographic and angiographic aspects of a four-month-old boy with double-outlet left ventricle, atrioventricular discordance, L-malposition of the great arteries, ventricular septal defect and pulmonary stenosis are described. Additionally, in this patient, the right arcus aorta and the ligament of ductus arteriosus caused anterior compression of the trachea. The anterior position of the morphological left ventricle is the most interesting feature.
Straddling atrioventricular valve with absent atrioventricular connection. Report of 10 cases
Heart, 1982
Six hearts with absent right connection and four hearts with absent left connection in association with straddling of an atrioventricular valve are described. Their categorisation is made easier by adopting a segmental approach. The conduction tissue was studied in two hearts and the arrangements were unusual. Straddling of an atrioventricular valve may be defined as the arrangement in which its tension apparatus is attached to both sides of the ventricular septum.' 2 When defined as such, the most frequent example of a straddling valve is an atrioventricular septal defect with a common atrioventricular orifice ("complete endocardial cushion defect" or "complete atrioventricular canal malformation"), though some authorities question whether "straddling" should be used in this context.34 Less frequent are the hearts in which there are two atrioventricular valves and either the right valve or the left valve, or, exceptionally, both valves, have their tension apparatus attached to both sides of the septum.2-4 These hearts pose a major problem in surgical treatment.' Hearts have also been reported, however, in which either the right or left valve straddles when the other valve is atretic. These latter hearts are exceedingly rare,3 5-8 but they pose particular problems in classification, particularly concerning their relation to common atrioventricular orifice.9 We have now examined 10 such hearts from *RHA and FJM are supported by the British Heart Foundation together with the Joseph Levy and Vandervell Foundations, respectively; and SM was a Visiting Fellow from Chaim Sheba Medical Centre, Tel Aviv, Israel.
The spectrum of double-outlet right atrium including hearts with three atrioventricular valves
European Journal of Cardio-Thoracic Surgery, 2012
Double-outlet right atrium (DORA) is characterized by simultaneous right atrial emptying into both ventricles. Ventriculoatrial septal malalignment is the cardinal morphological feature. Three cases are presented to depict two major types of DORA-DORA with a malaligned atrial septum and DORA with a malaligned ventricular septum. We describe two subtypes of each form of DORA: DORA with a malaligned atrial septum presents with either a common atrioventricular (AV) junction (guarded by a common AV valve) or with a single AV junction (due to the absence of the left AV junction). DORA with a malaligned ventricular septum may be associated with a right ventricle (RV) that is adequate for biventricular repair or a severely hypoplastic RV not compatible with biventricular repair. DORA with a malaligned ventricular septum is closely related to typical straddling of the tricuspid valve. Peculiarly, DORA with a malaligned ventricular septum presents three AV valves at the AV junction and is associated with an abnormal disposition of the AV conduction axis. Clear understanding of the morphology of these lesions is important in preventing a surgical misadventure at the crux of the heart.
Isolated Atrioventricular Discordance: Surgical Experience
The Annals of Thoracic Surgery, 2008
Background. Isolated ventricular inversion (atrioventricular discordance with ventriculoarterial concordance) is an extremely rare presentation of cyanotic congenital heart disease. The mode of presentation is akin to Dtransposition of great arteries as systemic venous drainage and systemic arterial output connect to the same side of the cardiac septae, and pulmonary venous drainage and pulmonary arterial outflow to the opposite. Systemic oxygenation relies on intracardiac or extracardiac modes of mixing, as does survival, similar to transposition of the great arteries. Published literature is scant, mainly because of the rarity of this cardiac anomaly. We review our surgical experience with this lesion.
Heart, 1979
Two pathological specimens are described in which a bizarre distortion of atrioventricular and ventriculoarterial relations existed. In both cases anterior and leftward displacement of the tricuspid valve coexisted with rightward displacement ofthe infundibular regions and semilunar valves. Abnormal expansion of the AV canal, possibly secondary to malrotation of the cardiac tube, is considered as a possible cause ofthe anomaly. One of the hearts showed severe subpulmonary stenosis produced by a valve-like structure which was well differentiated, with a complete annulus and chordae tendineae that inserted in a parachute-like fashion on the infundibular septum. The morphology of this structure was clearly different from that of the fibrous tags described in other anomalies. Its origin is uncertain. These anomalies have important implications in relation to the nomenclature and classification of congenital heart malformations, and illustrate the value ofrecent modifications ofthe segmental approach.
International Journal of Cardiology, 1992
Prospective echocardiographic diagnosis of absence of the left atrioventricular connexion, with the right atrium connected to a morphologic left ventricle through a bileatlet morphologically mitral valve, was made in six infants. The rudimentary right ventricle was left-sided in all patients, and separated from the left atrium by sulcus tissue. The ventriculoarterial connexions were discordant. Associated defects included subpulmonary stenosis (2 patients), pulmonary atresia (1 patient), and a patent duct (4 patients). All patients developed early left atrial hypertension due to a restrictive interatrial septum, and required transcatheter septostomy (5 patients), or surgical septectomy (3 patients). One patient who had a severely restrictive ventricular septal defect died following cardiac catheterization. In three others the ventricular septa) defect has become progressively restrictive on serial catheterization. Successful intermediate term palliation has been performed in two patients using a bidirectional Glenn anastomosis, together with enlargement of the ventricular septal defect and a Damus-Kay-Stansel procedure in one. It is possible to distinguish this malformation from "mitral atresia" using cross-sectional echocardiography. The long-term outlook is influenced by early relief of left atrial hypertension. Balloon atria) septostomy alone is usually inadequate, and either blade septostomy or surgical septectomy are required. Serial cardiac catheterization is mandatory for planning definitive palliation .
Atrioventricular discordance. Cross-sectional echocardiographic--morphological correlative study
Heart, 1983
Forty-nine patients with atrioventricular discordance were studied using cross-sectional echocardiography. The atrial arrangement was consistently predicted by echocardiographic analysis of either the patterns of pulmonary and systemic venous drainage or the arrangement of the great vessels within the upper abdomen. The ventricular arrangement was consistently predicted by direct identification of ventricular morphology. In hearts with two atrioventncular valves (47 patients) this was best derived by determining the pattern of implantation of the valve chordae to the ventricular myocardium. This proved to be more reliable in identifying ventricular morphology than either the analysis of trabecular patterns or the identification of valve leaflet morphology. In hearts with a common atrioventricular valve (two patients) ventricular arrangement had to be determined by identifying ventricular trabecular patterns.
Absence of one atrioventricular connexion — a terminologic problem
International Journal of Cardiology, 1991
We present a patient in whom the heart was right-sided, with usual atrial position, absence of the right-sided atrioventricular connexion, and a single outlet via the aorta from a dominant morphologically right ventricle. We use the case to illustrate potential problems in the terminology as used to describe the absence of one atrioventricular connexion. Our case shows why absence of one atrioventricular connexion must he classified according to the side of the missing connexion, and not the anatomy of the atrial or ventricular chambers involved. To simplify the approach to certain hearts, the use of the concept of one concordant or discordant atrioventricular connexion may be justified when the other atrioventrkular connexion is absent.