Double outlet right atrium and complete atrioventricular septal defect with abnormal findings of the biopsied lung

Suzuki, Kiyoshi; Kikuchi, Toshio; Mimori, Shigekazu

doi:10.1017/s1047951100002420

Cited by 5 publications

(3 citation statements)

References 9 publications

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“…Pacemaker implantation for immediate complete AV block was necessary due to the lesion of the aberrant AV node and AV conduction, located between the two RAVV orifices. We reviewed the literature and found 39 cases of DORA: 20 cases of DORA with malaligned AS and common AV valve 3,5,[12][13][14][15][16][17][18] ; 6 cases of DORA with malaligned AS and unique AV valve 3,5,[19][20][21][22] ; 8 cases of DORA with malaligned VS and adequate RV, as seen in our case 3,8,[23][24][25] ; and 5 cases of DORA with malaligned VS and inadequate RV. 7,9,[26][27][28] Of those, six were postmortem cases and one was an adulthood diagnosis.…”

Section: Case Reportmentioning

confidence: 99%

Double-Outlet Right Atrium: Review of a Rare Anomaly With an Exemplary Case

Miette

Nuri

Pomè

et al. 2019

World J Pediatr Congenit Heart Surg

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Background: Double-outlet right atrium (DORA) is a rare congenital cardiac defect, where the right atrium (RA) is connected to both the right ventricle (RV) and the left ventricle (LV). Double-outlet right atrium is classified into two types, each containing two subtypes: malaligned atrial septum with common or single atrioventricular (AV) valve and malaligned ventricular septum (VS) with adequate or inadequate RV. The VS type is characterized by straddling right AV valve (RAVV) with intact VS, resulting in two RAVV orifices. Methods: We report a case of “acquired DORA” in a 17-year-old male patient previously treated with subaortic VSD closure. At admission, we diagnosed DORA with VS malalignment and adequate RV. The patient had 2 orifices within the RAVV, connecting the RA to both the RV (via RAVV orifice 1) and the LV (via RAVV orifice 2). The latter was insufficient, with severe LV to RA shunt. A review of the literature indexed in PubMed and Scopus databases was undertaken. Results: The patient underwent biventricular repair through closure of the RAVV orifice 2 with a pericardial patch. Pacemaker implantation for complete AV block was necessary. Postoperative course and follow-up were regular. The literature review showed 39 cases of DORA, of which 8 had a malaligned VS and an adequate RV, as in our case. Conclusion: Acquired DORA is an interesting post-surgical variant of a rare congenital heart defect. Biventricular repair is a feasible and viable option, which involves closing the third AV orifice. Careful attention is required in order to avoid injury to the conduction system.

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Section: Case Reportmentioning

confidence: 99%

Double-Outlet Right Atrium: Review of a Rare Anomaly With an Exemplary Case

Miette

Nuri

Pomè

et al. 2019

World J Pediatr Congenit Heart Surg

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“…In such hearts, nonetheless, because of the commonality of the atrioventricular junctions, the atriums themselves must communicate through an ostium primum defect. [ 2 3 4 5 6 7 8 9 10 11 12 ] Double outlet atrium, however, is a non-specific term. It is always present, for example, when there is straddling and overriding of one or other of the two separate atrioventricular valves.…”

Section: Discussionmentioning

confidence: 99%

“…[ 1 ] A similar appearance can be seen in the setting of an atrioventricular septal defect with common atrioventricular junction. [ 1 2 3 4 5 6 7 8 9 10 11 12 ] The interatrial communication, however, is then through an ostium primum defect, rather than being found at the level of the oval fossa [ Figure 1 , right hand panel]. There is but one atrioventricular junction in both these situations.…”

Section: Introductionmentioning

confidence: 99%

Clarifying the atrioventricular junctional anatomy in the setting of double outlet right atrium

Gupta

Ramakrishnan

et al. 2015

Ann Pediatr Card

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Double outlet atrium is a rare cardiac anomaly wherein one of the atriums, most frequently the right atrium, opens into both the ventricles. Although seen more commonly in the setting of atrioventricular septal defect, this arrangement can also be found when one of the atrioventricular connections is atretic due to absence of the atrioventricular connection and the other atrioventricular valve straddles the muscular ventricular septum. It is the specific anatomy and connections of the atrioventricular junction that clarifies the situation and distinguishes between these two types of double outlet atrium. In this report, we present a case of double outlet right atrium co-existing with the absence of left atrioventricular connection. We then discuss the morphologic aspects of this interesting anomaly.

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2003

The Natural and Modifed History of Congenital Heart Disease

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Double outlet right atrium and complete atrioventricular septal defect with abnormal findings of the biopsied lung

Cited by 5 publications

References 9 publications

Double-Outlet Right Atrium: Review of a Rare Anomaly With an Exemplary Case

Double-Outlet Right Atrium: Review of a Rare Anomaly With an Exemplary Case

Clarifying the atrioventricular junctional anatomy in the setting of double outlet right atrium

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