Fibrous skeleton and ventricular outflow tracts in double-outlet right ventricle

Howell, C. E.; Ho, Siew Yen; Anderson, Robert H.; Elliott, Martin J.

doi:10.1016/0003-4975(91)90852-h

Cited by 22 publications

(4 citation statements)

References 21 publications

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“…Although 50% override rule is well accepted in diagnosing DORV, some researchers use a more restrictive criterion (90% overriding). In addition, other researchers propose the absence of fibrous continuity between the mitral and aortic valves as a definition of DORV (19, 20). We follow the STS‐EACTS International Nomenclature classification and a 70% override rule, and classify DORV into four subtypes: VSD type, Fallot type, TGA type and non‐committed VSD type (21).…”

Section: Discussionmentioning

confidence: 99%

Novel ZFPM2/FOG2 variants in patients with double outlet right ventricle

Huang

et al. 2011

Clinical Genetics

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Congenital heart defects (CHDs) occur in about 0.5-1% of all newborns and are the most common birth defects. Double outlet right ventricle (DORV) accounts for approximately 1-3% of all CHDs. Similar to Tetralogy of Fallot (TOF), DORV is a subtype of contruncal heart defects (CTDs) and is anatomically characterized by a malposition of the great arteries. We described a boy with chromosomal translocation: 46, XY t (8; 18) (q22; q21) that may disrupts the ZFPM2/FOG2 locus. The coding sequences of ZFPM2/FOG2 were determined in 38 patients with sporadic DORV, 95 patients with TOF, and 12 patients with transposition of the great arteries. Five DNA sequence variants affecting variably conserved residues of ZFPM2/FOG2 were identified in patients with TOF type or ventricular septal defect type of DORV. Three novel mutations (p.V339I, p.K737E, and p.A611T) were reported for the first time. The other two mutations (p.M703L and p.Q889E) were reported in patients with congenital diaphragmatic hernia but not in patients with CHD. Our finding suggests that variants of the ZFPM2/FOG2 gene might be a common cause of DORV.

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Section: Discussionmentioning

confidence: 99%

Novel ZFPM2/FOG2 variants in patients with double outlet right ventricle

Huang

et al. 2011

Clinical Genetics

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“…The relationship between double-outlet right ventricle and tetralogy of Fallot has long been contentious, largely centred on the dogma that bilateral infundibulums are needed to make the diagnosis of double outlet. Other disagreements have been related to the required degree of aortic overriding, with authors recommending from more than 50% to approximately 85–90% [7, 21, 22]. Double outlet, nonetheless, is a type of ventriculo-arterial connection rather than a distinct anomaly and can be found in the setting of tetralogy [23].…”

Section: Discussionmentioning

confidence: 99%

Tetralogy of Fallot: morphological variations and implications for surgical repair

Khan

Drury

Stickley

et al. 2019

European Journal of Cardio-Thoracic Surgery

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OBJECTIVES Tetralogy of Fallot is characterized by anterocephalad deviation of the outlet septum, along with abnormal septoparietal trabeculations, which lead to subpulmonary infundibular stenosis. Archives of retained hearts are an important resource for improving our understanding of congenital heart defects and their morphological variability. This study aims to define variations in aortic override, coronary arterial patterns and ventricular septal defects in tetralogy of Fallot as observed in a morphological archive, highlighting implications for surgical management. METHODS The Birmingham Children’s Hospital archive contains 211 hearts with tetralogy of Fallot, of which 164 were analysed [69 (42.1%) unrepaired and 95 (57.9%) operated specimens]. A detailed morphological and geometric analysis was performed using a rigorous 5-layer review process. RESULTS Anomalies were observed in the orifices, origins and course of the coronary arteries: 20 hearts (13.0%) had more than 2 orifices and 3 hearts (1.9%) had a single orifice. In 7 hearts (4.3%), a coronary artery crossed the right ventricular outflow tract. The extent of aortic override ranged from 31.0% to 100% (median of 59.5%). The ventricular septal defect was most often perimembranous (139, 84.8%), but we also found muscular (14, 8.5%), atrioventricular (7, 4.3%) and doubly committed juxta-arterial (2, 1.2%) variants. CONCLUSIONS Anatomical variations are common and can impact surgical management. Anomalous coronary arteries may require a conduit rather than a transannular patch. Variability in aortic override determines the size of patch used to baffle blood to the aorta. The type of ventricular septal defect affects patch closure and the risk of postoperative conduction defects.

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“…The rightward and posterior aorta to the PA is common in DORV with AV concordance, in which the VSD is subaortic, subpulmonary, or doubly committed. 36,44,71 A significant number of cases of DORV with subpulmonary VSD have truly side-byside great arteries that ascend parallel to each other rather than spiraling around each other, as in normally related great vessels. 5,36 DORV with noncommitted VSD is more likely to have the aorta anterior, either directly or slightly to the right.…”

Section: Relation Of Great Arteriesmentioning

confidence: 99%

“…36,44,71 A significant number of cases of DORV with subpulmonary VSD have truly side-byside great arteries that ascend parallel to each other rather than spiraling around each other, as in normally related great vessels. 5,36 DORV with noncommitted VSD is more likely to have the aorta anterior, either directly or slightly to the right. Despite the utility of these generalizations, it remains true that any type of VSD can occur with any arrangement of the great arteries and any type of subarterial conus.…”

Section: Relation Of Great Arteriesmentioning

confidence: 99%