scholarly journals Double orifice and atrioventricular septal defect: dealing with the zone of apposition†

2019 ◽  
Vol 56 (3) ◽  
pp. 541-548
Author(s):  
Margaux Pontailler ◽  
Moussa Haidar ◽  
Mathilde Méot ◽  
Anne Moreau de Bellaing ◽  
Régis Gaudin ◽  
...  

AbstractOBJECTIVESA double orifice of the left atrioventricular valve (LAVV) associated with atrioventricular septal defects (AVSD) can significantly complicate surgical repair. This study reports our experience of AVSD repair over 3 decades, with special attention to the zone of apposition (ZoA) of the main orifice, and presents a technique of hemivalve pericardial extension in specific situations.METHODSWe performed a retrospective study from 1987 to 2016 on 1067 patients with AVSD of whom 43 (4%) had a double orifice, plus 2 additional patients who required LAVV pericardial enlargement. Median age at repair was 1.3 years. Mean follow-up was 8.2 years (1 month–32 years).RESULTSAssociated abnormalities of the LAVV subvalvular apparatus were found in 7 patients (5 parachute LAVV and 2 absence of LAVV subvalvular apparatus). ZoA was noted in 4 patients (9%): partially closed in 15 (35%) and completely closed in 24 (56%). Four patients required, either at first repair or secondarily, a hemivalve enlargement using a pericardial patch without closure of the ZoA. The early mortality rate was 7% (n = 3), all before 2000. Two patients had unbalanced ventricles and the third had a single papillary muscle. There were no late deaths. Six patients (14%) required 7 reoperations (3 early and 4 late reoperations) for LAVV regurgitation and/or dysfunction, of whom 4 (9%) required mechanical LAVV replacement (all before 2000). Freedom from late LAVV reoperation was 97% at 1 year, 94% at 5 years and 87% at 10, 20 and 30 years. Unbalanced ventricles (P = 0.045), subvalvular abnormalities (P = 0.0037) and grade >2 LAVV postoperative regurgitation (P = 0.017) were identified as risk factors for LAVV reoperations. Freedom from LAVV mechanical valve replacement was 95% at 1 year, 90% at 5 years and 85% at 10, 20 and 30 years. An anomalous LAVV subvalvular apparatus was identified as a risk factor for mechanical valve replacement (P = 0.010). None of the patients who underwent LAVV pericardial extension had significant LAVV regurgitation at the last follow-up examination.CONCLUSIONSRepair of AVSD and double orifice can be tricky. Preoperative LAVV regurgitation was not identified as an independent predictor of surgical outcome. LAVV hemivalve extension appears to be a useful and effective alternate surgical strategy when the ZoA cannot be closed.

2004 ◽  
Vol 14 (2) ◽  
pp. 215-218 ◽  
Author(s):  
Solly E. Levin ◽  
Kathy Vanderdonck

We report two more patients with the association of postaxial polydactyly and atrioventricular septal defects. Additional cardiac findings, not previously described, were tetralogy of Fallot in the first case, and double orifice right atrioventricular valve in the second child.


2004 ◽  
Vol 14 (1) ◽  
pp. 15-23 ◽  
Author(s):  
Kathryn A. Dunlop ◽  
H. Connor Mulholland ◽  
Frank A. Casey ◽  
Brain Craig ◽  
Dennis J. Gladstone

Objectives:To review all cases of atrioventricular septal defects in Northern Ireland from January 1990 to February 1999, examining clinical and morphological features, management, and outcome.Methods:A retrospective case note analysis of 106 subjects with comparisons between subgroups.Results:An atrioventricular septal defect was part of a more complex abnormality in 50 of the patients (47%). Down's syndrome was present in 57 (54%). Cardiac surgery was performed in 81%. The defects were unrestrictive in 69 patients (65%), 45 of whom had Down's syndrome. Complex associated abnormalities existed in 36 patients, and 10 of these died without cardiac surgery. Operative mortality was 9.5% for those with co-existing Down's syndrome group, and 14.3% for the chromosomally normal patients. The ventricular components of the septal defect were restrictive in 23 patients (22%), with 9 having Down's syndrome. Spontaneous closure occurred in more than half of these patients. Mortality was zero. The septal defect was exclusively at atrial level in 14 patients (“primum” defects – 13%), and 3 of these had Down's syndrome. Operative mortality was again zero. Median duration of postoperative follow-up was 3 and a half years. Overall, moderate to severe left atrioventricular valvar regurgitation was observed postoperatively in 23% at follow-up.Conclusions:Mortality was highest in the atrioventricular septal defects with an unrestrictive ventricular component. Uncomplicated cases had good outcomes. Patients without Down's syndrome tended to have more associated cardiac abnormalities, and to have more postoperative arrhythmias. Approximately half of the defects with restrictive ventricular components closed spontaneously. Moderate postoperative left atrioventricular valvar regurgitation was commonest in patients with the defect exclusively at atrial level.


Author(s):  
Jing Sun ◽  
Hongxia Qi ◽  
Hongyuan Lin ◽  
Wenying Kang ◽  
Shoujun Li ◽  
...  

Abstract OBJECTIVES Aortico-left ventricular tunnel (ALVT) is an extremely rare, abnormal paravalvular communication between the aorta and the left ventricle. Few studies have identified the characteristics and long-term prognosis associated with ALVT. METHODS The data of 31 patients with ALVT from July 2002 to December 2019 were reviewed. Echocardiography was performed in all patients during the follow-up period. RESULTS The median age of the patients was 11.5 years. Bicuspid aortic valve and dilatation of the ascending aorta were found in 13 patients, respectively. The aortic orifice in 20 patients showed a close relation to the right sinus and the right–left commissure. Of the 31 patients, 26 were operated on. Mechanical valve replacement was performed in 4 patients and aortic valve repair, in 6 patients. Ascending aortoplasty was performed in 5 patients and aortic replacement was done in 2 patients. One patient died of ventricular fibrillation before the operation. Follow-up of the remaining 30 patients ranged from 1 to 210 months (median 64 months). There were 4 deaths during the follow-up period: 1 had mechanical valve replacement and 3 did not undergo surgical repair. In the 26 patients without aortic valve replacement, 6 had severe regurgitation and 2 had moderate regurgitation. In the 28 patients without replacement of the ascending aorta, 11 had continued dilatation of the ascending aorta, including those who had aortoplasty. CONCLUSIONS The aortic orifice of ALVT showed an association with the right sinus and the right–left commissure. For patients who did not have surgery, the long-term survival rate remained terrible. Surgical closure should be done as soon as possible after ALVT is diagnosed. The main long-term complications after surgical repair included aortic regurgitation and ascending aortic dilatation.


2015 ◽  
Vol 42 (2) ◽  
pp. 166-168 ◽  
Author(s):  
Firat H. Altin ◽  
Okan Yildiz ◽  
Mehmet Karacalilar ◽  
Oyku Tosun ◽  
Ozgen Ilgaz Kocyigit ◽  
...  

Atrioventricular septal defects constitute 4% of all congenital cardiac malformations. Patients with complete atrioventricular septal defect rarely survive for decades without surgical treatment. Pulmonary stenosis can provide a delicate balance between the pulmonary and systemic circulations and thereby increase longevity. We present the case of a 49-year-old woman whose complete atrioventricular septal defect and associated pulmonary stenosis were diagnosed only after she had given birth to 10 live children through uneventful spontaneous delivery. We discuss her successful surgical treatment in terms of the available medical literature.


2009 ◽  
Vol 138 (5) ◽  
pp. 1167-1171 ◽  
Author(s):  
Gerard J.F. Hoohenkerk ◽  
Arnold C.G. Wenink ◽  
Paul H. Schoof ◽  
Dave R. Koolbergen ◽  
Eline F. Bruggemans ◽  
...  

1995 ◽  
Vol 5 (3) ◽  
pp. 230-237 ◽  
Author(s):  
Ling Han ◽  
Soon Ung Kang ◽  
Sang C. Park ◽  
Jose A. Ettedgui ◽  
William H. Neches

AbstractLong-term left atrioventricular valvar function was evaluated in 95 of 110 survivors following surgical repair of atrioventricular septal defect between 1975 and 1984. A common or complete form was present in 40 and a partitioned or partial form in 55 patients. The patients have been followed for three to 13 years with a mean of 8.3 years. Pulmonary arterial banding was performed in 17 patients with a common atrioventricular valve prior to complete repair. The left atrioventricular valvar regurgitation was evaluated by clinical examination, Doppler and/or angiography. Three patients required valvar replacement postoperatively. Previous pulmonary arterial banding, pulmonary hypertension or pulmonary-to-systemic flow ratio did not affect the incidence or severity of left atrioventricular valvar regurgitation postoperatively. In this series left atrioventricular valvar regurgitation increased in the early postoperative period but rarely progressed at late follow-up.


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