Staged Surgical Management of Anatomically Corrected Malposition of Great Arteries

2020 ◽  
Vol 11 (3) ◽  
pp. 352-354
Author(s):  
Fabian A. Kari ◽  
Saira Siddiqui ◽  
Kanwal M. Farooqi ◽  
Michael P. DiLorenzo ◽  
Emile A. Bacha
Keyword(s):  
Pulmonary Artery ◽  
Surgical Management ◽  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Ventricular Septal Defects ◽  
Septal Defects ◽  
Left Ventricular Outflow ◽  
Anatomically Corrected Malposition ◽  
Atrioventricular Discordance

A nine-month-old girl diagnosed with anatomically corrected malposition (atrioventricular discordance, ventriculoarterial concordance, and malposed great arteries) complicated by multiple ventricular septal defects (VSD) and multifactorial left ventricular outflow tract obstruction (LVOTO) presented for management after pulmonary artery banding. She underwent interim palliation in the form of bilateral cavopulmonary connections, a modified Damus-Kaye-Stansel-type anastomosis, and subsequent staged one-and-a-half ventricle repair (1.5 repair) at the age of three years in the form of VSD closure, hemi-Mustard, and LVOTO resection.

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.

Evaluation of Factors Associated With Achievement of Biventricular Repair After Bilateral Pulmonary Artery Banding in Patients With Interrupted Aortic Arch

2018 ◽  
Vol 9 (1) ◽  
pp. 54-59
Author(s):  
Yasuhiro Hirano ◽  
Noboru Inamura ◽  
Yukiko Kawazu ◽  
Hisaaki Aoki ◽  
Futoshi Kayatani ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Interrupted Aortic Arch ◽  
Left Ventricular ◽  
Subaortic Stenosis ◽  
Biventricular Repair ◽  
Bilateral Pulmonary Artery Banding ◽  
Left Ventricular Outflow ◽  
Group B

Background: At our institution, we perform bilateral pulmonary artery banding (BPAB) as the first-stage palliation for interrupted aortic arch (IAA) with low birth weight or severe subaortic stenosis (SAS). The present study aimed to identify factors that may influence the decision regarding the type of second-stage operation, that is, univentricular palliation or biventricular repair, in these patients. Methods: Cardiac catheterization and angiographic data of nine patients with IAA who underwent initial BPAB and subsequent univentricular or biventricular repair were retrospectively analyzed. Results: Between 2004 and 2014, of nine patients with IAA who underwent initial BPAB, biventricular repair was subsequently performed in six patients (group B) and univentricular repair in three patients (group U). All patients survived. There was no significant intergroup difference in IAA classification, location of ventricular septal defect, presence of 22q11.2 deletion, presence of aberrant right subclavian artery, band diameter, or post-BPAB pulmonary artery pressure and index. Timing of BPAB and the body weight at the time of BPAB, however, differed significantly between the groups ( P = .02). Catheter data before BPAB were not significantly different between the groups, with the exception of the degree of subaortic stenosis (or hypoplasia of the left ventricular outflow tract) expressed as percentage of the normal end-systolic aortic valve annular diameter for patient body surface area. This metric (%SAS before BPAB) was significantly higher in group B (60%-68%) than in group U (47%-60%; P = .04). Among patients for whom baseline %SAS was < 60%, the %SAS did not increase after BPAB. Conclusion: The most important factor that allowed biventricular repair was not the pulmonary artery pressure or diameter but the degree of SAS. Patients who initially had more severe SAS ultimately underwent univentricular repair due to lack of substantial improvement in dimensions of the left ventricular outflow tract after BPAB.

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