The morphology and diagnosis of atrioventricular septal defects

1991 ◽  
Vol 1 (4) ◽  
pp. 290-305 ◽  
Author(s):  
Robert H. Anderson ◽  
Edward J. Baker ◽  
Siew Yen Ho ◽  
Michael L. Rigby ◽  
Tjark Ebels
Keyword(s):  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Outflow Tract ◽  
Normal Heart ◽  
Atrioventricular Septal Defects ◽  
Septal Defects ◽  
Left Ventricular Outflow ◽  
Normal Mitral Valve ◽  
Atrioventricular Septation ◽  
Conduction Tissues

SummaryIn this review, we discuss and describe those features which distinguish hearts with abnormal atrioventricular septation from the normal heart. The hearts, best described as atrioventricular septal defects, are unified by having a common atrioventricular junction guarded by a valve having five leaflets. The left component has three leaflets and cannot be interpreted in terms of a cleft in a normal mitral valve. The papillary muscles supporting this valve are also markedly dissimilar from the arrangement seen in normal hearts. The subaortic outflow tract is displaced superiorly compared to the normal heart, and is no longer wedged between the left valve and the septum. There is marked discrepancy in the inlet and outlet lengths of the ventricular mass, these dimensions being equal in hearts with normal atrioventricular septation. Although having the above features in common, atrioventricular septal defects show anatomic variations related to the arrangement of the bridging leaflets and their relationship to the septal structures. There may be a common valvar orifice or separate right and left orifices. The anatomic potential for shunting may be at atrial or ventricular levels, or both. Rarely, the septal structures may be intact. Other important features include ventricular dominance, the left ventricular outflow tract, and the disposition of the atrioventricular conduction tissues.

Repair of Complete Atrioventricular Septal Defects With Decellularized Extracellular Matrix: Initial and Midterm Outcomes

2017 ◽  
Vol 8 (3) ◽  
pp. 310-314 ◽  
Author(s):  
Adeel Ashfaq ◽  
Tyler Brown ◽  
Brian Reemtsen
Keyword(s):  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Single Institution ◽  
Atrioventricular Septal Defects ◽  
Septal Defects ◽  
Decellularized Extracellular Matrix ◽  
Left Ventricular Outflow ◽  
Patch Technique ◽  
Complete Atrioventricular

Objective: Since April 2010, our institution has repaired complete atrioventricular septal defects (CAVSDs) with a two-patch technique utilizing CorMatrix extracellular material. This material is potentially an attractive patch because of its theorized eventual integration with the host tissue. We sought to analyze initial outcomes of CAVSD repair with CorMatrix. Methods: Data were collected on consecutive pediatric (age <18) patients receiving two-patch CAVSD repairs with CorMatrix at a single institution from April 2010 to July 2014. Baseline and perioperative characteristics were evaluated. Echocardiograms were evaluated in both the immediate postoperative period and the most recent postoperative follow-up. Variables analyzed included left AV valve performance, residual shunting, left ventricular outflow tract (LVOT) gradient, morbidity, and mortality. Results: Fifteen patients were identified. The average age at operation was 205 days, with mean follow-up time at 1,364 days. Echocardiograms revealed the following: 12 (80%) patients showed either improved or stable left AV valve performance remaining at “mild” or less insufficiency, while two (13%) declined from “none” to mild and one (7%) from mild to “severe,” which required reoperation. There was no residual shunting or LVOT obstruction at follow-up. The single (7%) reoperation was performed after three years due to left AV valve zone of apposition dehiscence. No permanent pacemakers were needed, and no deaths were reported. Conclusion: Our initial experience with CorMatrix in the repair of CAVSD in children has resulted in good initial and midterm outcomes. The CorMatrix patch remained stable through midterm follow-up, thus may be efficacious for use in CAVSD repair.

Abstract 2721: Linkage of Left Ventricular Outflow Tract Obstruction to Xq28 in 3 French-Canadian Pedigrees

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Sylvie Provost ◽  
Philippe Chetaille ◽  
Ana Siles ◽  
Maryse Thibeault ◽  
Nathalie Bureau ◽  
...  
Keyword(s):  
Linkage Analysis ◽  
X Chromosome ◽  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Outflow Tract ◽  
French Canadian ◽  
Ventricular Outflow Tract Obstruction ◽  
Septal Defects ◽  
Left Ventricular Outflow

BACKGROUND: Statistical genetic analysis of cohorts with left ventricular outflow tract obstruction (LVOTO) shows high heritability of such lesions. We have identified three X-linked French-Canadian pedigrees with a total of 54 family members with multiple cases of LVOTO and septal defects. AIMS: The aim of this study was to evaluate the three pedigrees with LVOTO and septal defects for genetic linkage to the X chromosome. METHODS: Detailed family history, physical exam, ECG, echocardiography and chart review was performed. Informed consent was obtained from all participants. Genomic DNA was isolated from peripheral blood samples. Twenty-four STR markers from the X chromosome were genotyped. We used a dominant genetic model with the following penetrances: 0.99 for homozygous women, 0.75 for heterozygous women, 0.99 for hemizygous men. Phenocopy rate was set to 0.01 and disease allele frequency to 0.001. Reference allele frequencies from the Quebec population were used. Analyses were performed with MLINK and MERLIN. RESULTS: Sixteen family members were affected (13 males, 3 females). Predominant lesions were atrial (n = 5) and ventricular (n = 6) septal defects, coarctation (n = 4) and aortic lesions (stenosis (n = 4), bicuspid/abnormal aortic valve (n = 6)), as well as mitral valve lesions (n = 4). Males had a high incidence of supraventricular arrhythmias postoperatively (4 with atrial flutter/fibrillation, one with ICD implantation). LOD scores above 2 were obtained in parametric two-point linkage analysis for 3 markers in the Xq28 region. Multipoint non-parametric linkage analysis confirmed these results with a NPL score of 9.1 (P < .00001) over a STR marker in intron 13 of the F8C gene. Haplotype analysis allowed the definition of a candidate interval flanked by marker DXS8069 spanning to the telomeric end of Xq. CONCLUSIONS: By analysing 3 multi-generation French-Canadian pedigrees, we mapped a syndrome of LVOTO and septal defects to the Xq28 region. Further work will refine our candidate region with dense SNP coverage to search for a shared haplotype identical by descent between the three French Canadian families, to complete the sequencing of candidate genes including FLNA, and to search for a possible founder effect in our population.

Mortality and complications in 3495 children with isolated ventricular septal defects

2016 ◽  
Vol 101 (9) ◽  
pp. 808-813 ◽  
Author(s):  
Jarle Jortveit ◽  
Elisabeth Leirgul ◽  
Leif Eskedal ◽  
Gottfried Greve ◽  
Tatiana Fomina ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Aortic Regurgitation ◽  
Left Ventricular Outflow Tract ◽  
Heart Defects ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Outflow Tract ◽  
Cardiac Complications ◽  
Septal Defects ◽  
Left Ventricular Outflow

BackgroundVentricular septal defects (VSDs) are the most common congenital heart defects (CHDs). Previous studies indicate an increased risk of endocarditis, aortic regurgitation, left ventricular outflow tract obstructions, pulmonary hypertension, arrhythmias and sudden death in patients with isolated VSDs. The present nationwide cohort study reports mortality and cardiac complications requiring hospitalisation or intervention in children with isolated VSDs.Methods and resultsMedical information concerning all 943 871 live births in Norway in 1994–2009 was retrieved from the Medical Birth Registry of Norway, the Cardiovascular Disease in Norway project, the Oslo University Hospital's Clinical Registry of Congenital Heart Defects and the Norwegian Cause of Death Registry. Isolated VSDs were identified in 3495 children without known chromosomal aberrations or extracardiac malformations. Surgical or catheter-based treatment of VSD was performed in 181 (5.2%) cases. Twelve (0.3%) children with VSDs died before 2013. There was no operative mortality, and no excess mortality in children with isolated VSDs compared with children without VSDs (adjusted HR 0.8 (0.5 to 1.4), p=0.48). The following conditions were recorded as possible cardiac complications of the VSDs: endocarditis in 3 children (0.9‰), aortic regurgitation in 12 children (3.4‰), left ventricular outflow tract obstructions in no children (0.0‰), pulmonary hypertension in 1 child (0.3‰) and arrhythmias in 16 children (4.6‰).ConclusionsThe entire group of children with isolated VSDs had a favourable prognosis without excess mortality. Cardiac complications requiring hospitalisation or intervention, including endocarditis, aortic regurgitation, left ventricular outflow tract obstructions, pulmonary hypertension and arrhythmias, were infrequent during childhood.Trial registration numberNCT02026557.

scholarly journals Active mitral valve endocarditis with widespread interventricular septal abscesses in a paediatric patient

2020 ◽  
Author(s):  
Kazuhiko Ishimaru ◽  
Shigemitsu Iwai ◽  
Sanae Tsumura ◽  
Hiroaki Kawata
Keyword(s):  
Postoperative Complications ◽  
Mitral Valve ◽  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Outflow Tract ◽  
Anterior Leaflet ◽  
Valve Repair ◽  
Septal Defects ◽  
Left Ventricular Outflow

Abstract We present the case of a previously healthy 2-year-old boy with extensive infective mitral valve endocarditis, with 2 huge, mobile vegetations attached to the anterior leaflet of the mitral valve and to the left ventricular outflow tract, and interventricular septal abscesses extending into the left ventricular outflow tract without any septal defects. He underwent mitral valve repair and simultaneous drainage of the interventricular septal abscesses excluding the inlet portion to avoid postoperative complications.

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