Atrioventricular canal defect without Down syndrome: A heterogeneous malformation

Author(s):  
Maria Cristina Digilio ◽  
Bruno Marino ◽  
Alessandra Toscano ◽  
Aldo Giannotti ◽  
Bruno Dallapiccola
The Lancet ◽  
1985 ◽  
Vol 326 (8459) ◽  
pp. 834-835 ◽  
Author(s):  
N.J. Wilson ◽  
E. Gavalaki ◽  
C.G.H. Newman ◽  
Samuel Menahem ◽  
RogerB.B. Mee

2011 ◽  
Vol 16 (4) ◽  
pp. 403-406 ◽  
Author(s):  
Svjetlana Tisma-Dupanovic ◽  
Rengasamy Gowdamarajan ◽  
Ilan Goldenberg ◽  
David T. Huang ◽  
Timothy Knilans ◽  
...  

QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Sherief Azzab ◽  
Ahmed Samy ◽  
Hamdy Singab ◽  
Mohamed EL Ghanam ◽  
Ahmed Tarek

Abstract Background The optimal timing, surgical technique and the influence of Down syndrome on outcome of surgical repair of Complete Atrio-Ventricular Canal Defect (CAVC) remains uncertain. We reviewed our experience in repair of CAVC to identify the influence of these factors on operative outcome. Methods A prospective study included 70 patients who underwent repair of CAVC at Ain Shams university hospitals, academy of cardiothoracic surgery during the period from July 2016 to October 2019. Age at surgery (less than 6 months old versus later), surgical technique used [(modified single patch versus double patch technique), (use of posterior annuloplasty for repair of left AV valve or not)] and association of Down syndrome were evaluated for their impact on the outcome of surgical repair using multivariate analysis. Patients were followed up for 6 months; primary end point was mortality and secondary end point was reappearance of LAVV regurgitation. Results No significant difference between patients operated on, at the first 6 months of age versus later, regarding mortality or LAVV regurgitation. Down patients showed significant difference in the occurrence of postoperative compared to non-Down patients (LAVVR grade 2 + = 8.9% vs 24%, P value =0.005) respectively. Surgical repair by Modified single patch technique showed significant reduction in cross clamp time (mean = 47.6 ± 9.227 min vs 73.55 ± 21.087 min, P value 0.00), shorter bypass time (mean = 71.13 ± 13.507 min vs 99.19 ± 27.092 min, P value =0.00) and shorter duration of ICU stay (mean =3.2 ± 1.657 days vs 5.3 ± 2.761 days, P value=0.01) as compared to double patch technique. Posterior annuloplasty used for repair of LAVV compared to closure of cleft only resulted in significant reduction in the occurrence of post-operative valve regurgitation during the early period (LAVVR 2+ 43% vs 7%, P value=0.03) and at 6 months of follow up. (LAVVR 2+ 35.4% vs 0%, P value=0.01) respectively. Conclusion early intervention, in the first 6 months in patients with CAVC by surgical repair gives comparable acceptable results to later repair, Down syndrome was not found to be a risk factor for early intervention. Modified single patch and double patch techniques for repair, can be used both with comparable results even in large VSD component (8mm and larger), finally, repair of common AV valve by cleft closure with posterior LAV annulplasty showed better results with significant decrease in postoperative LAV regurgitation and early mortality in comparison to closure of cleft only.


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Ilias P Doulamis ◽  
Supreet P Marathe ◽  
Breanna L Piekarski ◽  
Rebecca S Beroukhim ◽  
Gerald R Marx ◽  
...  

Hypothesis: Biventricular conversion (BiVC) following takedown of Fontan circulation is feasible and results in improved hemodynamics. Methods: Retrospective analysis of patients who had takedown of Fontan circulation and conversion to BiV circulation at a single center from September 2007 to April 2020. Failing Fontan physiology was defined as Fontan circulation pressure >15 mmHg. Results: There were 23 patients (median age: 10.0 (7.5-13.0) years); 15 (65%) had failing Fontan physiology and 8 (35%) underwent elective takedown of their Fontan circulation. Of the 15 patients with failing Fontan physiology, 4 had exercise intolerance or cyanosis, 3 had hepatic congestion or cirrhosis, 3 had end-organ damage and 1 patient had protein losing enteropathy; the rest 4 patients had no other sign of SVP complications. A subset of patients (n=6) underwent recruitment of the non-dominant ventricle prior to conversion. HLHS (p<0.01) and sub-/aortic stenosis (p<0.01) were more common in these patients. BiVC with or without staged ventricular recruitment led to a significant increase in indexed end-diastolic volume (p<0.01), indexed end-systolic volume (p<0.01) and ventricular mass (p<0.01) of the non-dominant ventricle (14 RV, 9 LV). There were 1 (4%) early and 4 (17%) late deaths. All who underwent elective BiVC survived, while 2-year survival rate for patients with a failing Fontan circulation was 72.7% (95% CI: 37-90%) (Figure 1). The overall, 1-year reintervention free survival was 44.1% (95% CI: 21-65%). Left dominant atrioventricular canal defect (p<0.01) and early year of BiVC (p=0.02) were significant predictors for mortality. Conclusions: BiVC is feasible in patients with failing Fontans, and has promising outcomes after elective takedown of Fontan circulation. A staged approach for ventricular recruitment does not seem inferior to primary BiVC. The optimal timing for BiVC in Fontan patients needs further evaluation.


2018 ◽  
Vol 9 (6) ◽  
pp. 645-650
Author(s):  
David Blitzer ◽  
Jeremy L. Herrmann ◽  
John W. Brown

Background: Mitral valve replacement (MVR) with a pulmonary autograft (Ross II) may be a useful technique for pediatric and young adult patients who wish to avoid anticoagulation. Our aim was to evaluate the long-term outcomes of the Ross II procedure at our institution. Methods: Patients undergoing the Ross II procedure between June 2002 and April 2008 were included. Preoperative diagnoses included rheumatic disease (n = 5), congenital mitral valve (MV) pathology (partial atrioventricular canal defect [n = 2], complete atrioventricular canal defect [n = 1], Shone's complex [n = 1]), and myocarditis (n = 1). Results: Ten patients (eight females and two males) between 7 months and 46 years were included. Mean age at surgery was 25.2 ± 15.7 years. There were no in-hospital deaths. Mean follow-up was 11.7 ± 5.2 years. There were three late deaths at 11 months, 5 years, and 11 years, respectively. Causes of death included right heart failure, sepsis, and sudden cardiac arrest. Three patients required subsequent mechanical MVR a median of two years after the Ross II procedure (range: 1-4 years). There was no mortality with reoperation. Echocardiographic follow-up demonstrated mean MV gradients ranging from 2.2 to 9.6 mm Hg. Two patients had greater than mild MV regurgitation postoperatively, and all others had minimal mitral regurgitation or less. Two patients developed moderate MV stenosis. Conclusions: The Ross II procedure is an option for select older children and young adults desiring a durable tissue MVR to avoid long-term anticoagulation.


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