Forme Fruste of Endocardial Cushion Defect

Sumner, Robert G.; Phillips, John H.; Jacoby, William J.; Tucker, Donald H.

doi:10.1097/00000441-196709000-00002

Cited by 8 publications

(5 citation statements)

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“…Our observations suggest that from the anatomical viewpoint, the group of anomalies termed transitional or intermediate types Edwards, 1956, 1958;Al Omeri et al, 1965;Sumner et al, 1967) are best considered as variants of the partial form in that they have discrete mitral and tricuspid annuli. It is true that the discrete annuli are in many instances produced as a consequence of a bridge of valve tissue extending across the septum from posterior to anterior leaflets and that the conjoined valves have features that resemble a common orifice (Fig.…”

Section: Discussionmentioning

confidence: 81%

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Morphology and classification of atrioventricular defects.

Piccoli¹,

Gerlis²,

Wilkinson³

et al. 1979

Heart

102

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Anatomical studies were made on 114 necropsy specimens of atrioventricular defects with atrioventricular concordance. The malformation is characterised by disproportion between the ventricular inlet and outlet dimensions and a malorientation of the aortic valve relative to the atrioventricular valve or valves. Associated with this there is a characteristic 'scopped-out' appearance of the muscular ventricular septum, gross abnormalities of the membranous components of the septum as compared with the normal heart, and narrowing of the aortic outflow tract. Hearts with these anatomical features can be divided into partial and complete forms depending on the morphology of the atrioventricular annuli. In the partial form the septal leaflets are conjoined to give separate mitral and tricuspid orifices, the conjoined leaflets being displaced into the ventricles and usually attached to the crest of the septum. In the complete form, anterior and posterior components of the 'septal' leaflets are separate, so that a single valve orifice connects the atrial to the ventricular chambers. Further subdivision of the complete form, apart from the morphology of the anterior leaflet, is dependent upon the presence or absence of an ostium primum atrial septal defect.

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Section: Discussionmentioning

confidence: 81%

“…It is evident from those investigations that opinion is by no means unanimous concerning several important features of these anomalies. For example, there is disagreement concerning the meaning of the so-called intermediate (Wakai and Edwards, 1958) or transitional type (Wakai and Edwards, 1956;Al Omeri et al, 1965;Sumner et al, 1967;Goor and Lillehei, 1975) …”

mentioning

confidence: 99%

Morphology and classification of atrioventricular defects.

Piccoli¹,

Gerlis²,

Wilkinson³

et al. 1979

Heart

102

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“…Conceivably, the anomaly might exist without a septal defect at all and thus suggest an Circulation, Volume XXXIV, November 1966 explanation for the finding of an abnormal VCG in normal relatives of children with A-V canal defects. 19 The course of the fibers destined to become the right bundle branch was not obviously elongated in any of the examples of VSD studied; however, comparison of the measurement ratios indicated differences similar to those found in the cases of tetralogy of Fallot. Thus, the distance from the A-V node to the origin of the right bundle branch was relatively greater in the two examples of VSD with VCG patterns similar to the A-V canal pattern than in the examples with the usual VCG pattern.…”

Section: Discussionmentioning

confidence: 54%

The Anatomy of the Atrioventricular Conduction System in Ventricular Septal Defect and Tetralogy of Fallot: Correlations with the Electrocardiogram and Vectorcardiogram

1966

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IN RECENT YEARS the morphological features of the major portions of the atrioventricular (A-V) conduction system of human hearts that were afflicted with various congenital anomalies have been described. [1][2][3][4][5][6] These reports have contrasted the anatomy of the A-V conduction system in examples of ventricular septal defect (VSD) and tetralogy of Fallot with that configuration found in defects of the persistent atrioventricular (A-V) canal type.7 8The vectorcardiographic (VCG) pattern found in the A-V canal has been considered to be characteristic.9-12 It is now known, however, that various congenital heart defects have a similar vectorcardiographic pattern, including occasional examples of VSD and tetralogy of Fallot.13-l5 Therefore, a study was made of the morphology of the A-V conduction system in instances of VSD and tetralogy of Fallot that had frontal plane VCG patterns similar to those usually found in the A-V canal. An effort was made to find a characteristic or characteristics of the anatomy of the conduction system that might be associated with the unusual VCG pattern observed in these examples. Material Tetralogy of Fallot Of 78 examples of tetralogy in which autopsy was performed from 1958 through 1962, five (6.4%) were associated with preoperative VCG From the Mayo Clinic and Mayo Foundation, Rochester, Minnesota. This investigation was supported in part by Training Grant HTS-5515 from the National Institutes of Health, U. S. Public Health Service. patterns similar to the A-V canal pattern, that is, an initially counterclockwise QRS loop in the frontal plane with the mean axis directed superiorly and to the left (fig. 1A). Two of these cases were studied; these were designated cases Al and A2. Two other examples of tetralogy (cases A3 and A4), which had VCG patterns more representative of tetralogy, also were studied (fig. 1C). None of the four examples selected had ventricular septal defects of the A-V canal type.8 The defects in all four cases were located in similar positions in the ventricular septum. Ventricular Septal Defect Seventy-nine examples of isolated VSD of the usual type came to autopsy from 1958 through 1962. Seven of the 79 (8.9%) had preoperative VCG patterns similar to the A-V canal pattern and two of these (cases Bi and B2) were studied (fig . 1B). Two other examples (cases B3 and B4) in which the VCG patterns were more typical of VSD also were studied ( fig. 1D). None of these four examples had the morphological features of VSD of the A-V canal type.8 Methods The morphology of the conduction system in each of the eight cases studied was reconstructed with the use of a previously established technique of multiple histological sections.5 The blocks of tissue for sectioning included the atrial and ventricular septa on each side of the atrioventricular rings and included the atrioventricular node, the common bundle of His, and the proximal portions of the right and left bundle branches. The more peripheral bundle branches were not studied.The tissue blocks were sectioned at ...

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“…6,8,[11][12][13] In cardiac classification, the focus was to the spectrum of AVCDs, and the other cardiovascular anomalies were considered as secondary diagnosis. Mutations found in the present series of patients with AVCD/related defects and in published series of AVCD were reviewed and analysed for possible genotype-phenotype correlations.…”

Section: Methodsmentioning

confidence: 99%

“…Cleft mitral valve is considered the less severe form of AVCD. [8][9][10][11][12][13] A defect of the extracellular matrix has been considered a likely pathogenic mechanism for the defects in the spectrum of AVCD. 14,15 AVCD is also the most common CHD found in children with Down syndrome and one of the structural heart defects most frequently associated with extracardiac anomalies in the setting of chromosomal and mendelian disorders.…”

Section: Introductionmentioning

confidence: 99%