A ten year review of atrioventricular septal defects

2004 ◽  
Vol 14 (1) ◽  
pp. 15-23 ◽  
Author(s):  
Kathryn A. Dunlop ◽  
H. Connor Mulholland ◽  
Frank A. Casey ◽  
Brain Craig ◽  
Dennis J. Gladstone
Keyword(s):  
Cardiac Surgery ◽  
Septal Defect ◽  
Operative Mortality ◽  
Down's Syndrome ◽  
Down’S Syndrome ◽  
Atrioventricular Septal Defect ◽  
Retrospective Case ◽  
Atrioventricular Septal Defects ◽  
Septal Defects

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.

Cardiovascular malformations in Omani Arab children with Down's syndrome

2007 ◽  
Vol 17 (2) ◽  
pp. 166-171 ◽  
Author(s):  
Olufemi Jaiyesimi ◽  
Vijaymani Baichoo
Keyword(s):  
Septal Defect ◽  
Down's Syndrome ◽  
Coarctation Of The Aorta ◽  
Down’S Syndrome ◽  
Atrioventricular Septal Defect ◽  
Cardiovascular Malformations ◽  
Arab Children ◽  
Septal Defects ◽  
Appropriate Care ◽  
Echocardiographic Evaluation

Background and aim: Reports from several countries suggest that the prevalence and spectrum of the congenital cardiac malformations seen in the setting of Down's syndrome vary in different ethnic groups and countries, and at different periods in the same country. Data on Arab children are lacking. Our study aimed to fill that void by ascertaining the pattern in Omani Arabs. Methods: Prospective clinical and echocardiographic evaluation of consecutively recruited Omani children with Down's syndrome. Results: We studied 110 children, aged from one day to 11 years, with a median of 2 months, detecting 76 cardiovascular malformations in 63 (57%) of them. Atrioventricular septal defect, the most prevalent lesion, accounted for 24 (32%) of the 76 defects. Next were atrial septal defects within the oval fossa (29%), patency of the arterial duct (17%), and ventricular septal defect (14%). There were only two cases of Fallot's tetralogy, one each of coarctation of the aorta and right-sided heart, respectively; and none of discordant ventriculo-arterial connections. Conclusion: The prevalence of cardiovascular malformations is high, at 57%, in our studied population of Arab children. Overall, 92% of the malformations are defects which are characterized by a left-to-right shunt, and the potential for pulmonary hypertension. These findings underscore the need for early detection and prompt, appropriate care. To achieve this, if feasible, all babies born with Down's syndrome should have echocardiography in the first month of life.

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 ◽  
...  
Keyword(s):  
Valve Replacement ◽  
Septal Defect ◽  
Surgical Repair ◽  
Independent Predictor ◽  
Mechanical Valve ◽  
Atrioventricular Septal Defect ◽  
Atrioventricular Septal Defects ◽  
Septal Defects ◽  
Double Orifice

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.

Sizes of the descending aorta and pulmonary arteries in infants with atrioventricular septal defects

1994 ◽  
Vol 4 (1) ◽  
pp. 51-54
Author(s):  
Jae H. Ahn ◽  
Christopher J. Knott-Craig ◽  
Ronald C. Elkins ◽  
Mary M. Lane ◽  
Edward D. Overholt ◽  
...  
Keyword(s):  
Vascular Resistance ◽  
Primary Repair ◽  
Down's Syndrome ◽  
Pulmonary Arteries ◽  
Down’S Syndrome ◽  
Atrioventricular Septal Defect ◽  
Descending Aorta ◽  
Atrioventricular Septal Defects ◽  
Septal Defects ◽  
Pulmonary Arterial

AbstractThe descending aorta appears small relative to unusually large pulmonary arteries in infants with atrioventricular septal defects. To determine whether this discrepancy in great arterial size influences the outcome after repair of atrioventricular septal defects, we reviewed our experience with 34 infants younger than one-year-old undergoing primary repair. There were four early deaths (11.8%), one in 16 patients with normal chromosomes (6.3%), and three of 18 with Down's syndrome (16.7%). There was no difference between survivors and non-survivors in terms of either the size of the aorta at the level of the diaphragm (descending aortic index, 149±34 versus 139±46 mm2/m2), the pulmonary arterial index (675±170 versus 745±182 mm2/m2), or the ratio of the descending aorta to the pulmonary trunk arterial (4.85±1.6 versus 5.93±2.8). Compared with survivors, non-survivors had higher ratios of vascular resistance (0.48±0.42 versus 0.23±0.14, p<0.05), and tended to have larger preoperative cardiothoracic ratios (0.63±0.05 versus 0.55±0.08, p=0.12) Compared to patients with normal chromosomes, the patients with Down's syndrome tended to have smaller descending aortas (140±34 versus 159±34 mm2/m2, p=0.12), higher ratios of vascular resistance (0.32±0.2 versus 0.18±0.1, p=0.08), and higher mean pulmonary arterial pressures (47±11 versus 39±18 mm Hg, p=0.12), suggesting that pulmonary vascular obstructive disease may be more severe in patients with Down's syndrome. The size of the descending aorta and pulmonary arteries do not influence outcome after repair of atrioventricular septal defect in infants.

Atrioventricular septal defect with tetralogy of Fallot

1991 ◽  
Vol 1 (4) ◽  
pp. 396-398
Author(s):  
Bharat Dalvi ◽  
Krishnagopal Gupta ◽  
Satyavan Sharma
Keyword(s):  
Tetralogy Of Fallot ◽  
Septal Defect ◽  
Down's Syndrome ◽  
Down’S Syndrome ◽  
Atrioventricular Septal Defect ◽  
Cross Sectional

SummaryWe present a case of atrioventricular septal defect associated with tetralogy of Fallot which was diagnosed by cross-sectional echocardiography and angiography. The diagnosis was confirmed at necropsy. This case is unusual for the absence of Down's syndrome. We discuss the role of clinical, echocardiographic, hemodynamic and angiographic studies.

Massive pericardial effusion with left-to-right intracardiac shunt

2001 ◽  
Vol 11 (4) ◽  
pp. 461-463
Author(s):  
A. J. Edwards ◽  
N. J. Ring ◽  
A. J. Marshall
Keyword(s):  
Pericardial Effusion ◽  
Septal Defect ◽  
Down's Syndrome ◽  
Down’S Syndrome ◽  
Atrioventricular Septal Defect ◽  
Right Heart ◽  
Alternative Strategies ◽  
Intracardiac Shunt

We describe a 31-year-old man with Down's syndrome who presented with severe chronic hypothyroidism and a massive pericardial effusion. Following partial aspiration of this effusion, he rapidly deteriorated and died. Findings at autopsy revealed him to have an atrioventricular septal defect with shunting at the atrial level. We postulate that, by releasing extrinsic pressure on his right heart by the aspiration, there was sudden shunting of blood from left-to-right, resulting in hypotension, shock, and subsequent death. We highlight the difficulties in management of such a case, and suggest alternative strategies.

Better surgical prognosis for patients with complete atrioventricular septal defect and Down's syndrome

2004 ◽  
Vol 78 (2) ◽  
pp. 666-672 ◽  
Author(s):  
Roberto Formigari ◽  
Roberto M Di Donato ◽  
Gaetano Gargiulo ◽  
Duccio Di Carlo ◽  
Cristiana Feltri ◽  
...  
Keyword(s):  
Septal Defect ◽  
Down's Syndrome ◽  
Down’S Syndrome ◽  
Atrioventricular Septal Defect ◽  
Complete Atrioventricular Septal Defect ◽  
Complete Atrioventricular

scholarly journals Complete Atrioventricular Septal Defect and Pulmonary Stenosis Diagnosed in a 49-Year-Old Woman after 10 Uneventful Births

2015 ◽  
Vol 42 (2) ◽  
pp. 166-168 ◽  
Author(s):  
Firat H. Altin ◽  
Okan Yildiz ◽  
Mehmet Karacalilar ◽  
Oyku Tosun ◽  
Ozgen Ilgaz Kocyigit ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Septal Defect ◽  
Medical Literature ◽  
Pulmonary Stenosis ◽  
Atrioventricular Septal Defect ◽  
Atrioventricular Septal Defects ◽  
Septal Defects ◽  
Complete Atrioventricular Septal Defect ◽  
Complete Atrioventricular ◽  
Spontaneous Delivery

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.

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