D. Madec , S. Bernheim , A. Desgrange , N. Boddaert , O. Raisky
{"title":"A 3D analysis of ventricle position in patients with heterotaxy shows high frequency of ventricle malposition in D-Loop hearts","authors":"D. Madec , S. Bernheim , A. Desgrange , N. Boddaert , O. Raisky","doi":"10.1016/j.acvd.2024.07.014","DOIUrl":null,"url":null,"abstract":"<div><h3>Introduction</h3><p>Congenital heart defects in the context of heterotaxy are severe, with a complex anatomy. These defects are described using the binary concept of clinical loop (D-Loop or L-Loop), which implies that ventricle position is determined during heart looping. Recent work in the mouse has shown unexpected plasticity of ventricle position after heart looping. Based on a analysis of Nodal mutants with heterotaxy and right bronchus isomerism, 27% of D-Loop mutants at birth underwent leftward embryonic heart looping. These mice with a revertant loop, all display abnormal ventricle position.</p></div><div><h3>Objective</h3><p>Analysis of 3D ventricle position in human patients with heterotaxy and right bronchus isomerism, using the strategy developed in the mouse based on the 3D orientation of the interventricular septum.</p></div><div><h3>Methods</h3><p>Constitution of an heterotaxy cohort from the Necker database. Controls were selected as patients with transposition of the great arteries who had a systematic CT scan at 6 years. Three dimensions reconstructions of CT scans and quantitative analyses were performed using the Imaris software.</p></div><div><h3>Expected results</h3><p>Control patients had their right ventricle-left ventricle axis in the expected anteroposterior and left-right orientation. We collected 506 patients with heterotaxy syndrome, 186 of whom had CT scan. Forty patients had right bronchial isomerism. Among them, we found 29 (72%) patients who had an orientation of the interventricular septal axis similar to control, 7 (18%) patients with abnormal supero-inferior ventricles and 4 (10%) patients with abnormal left-right ventricles (<span><span>Fig. 1</span></span>). In total, 32% of patients in D-Loop have a malpositioned interventricular septum whereas 0 of patients in L-Loop.</p></div><div><h3>Perspectives</h3><p>There is a high proportion of malpositioned ventricles in patients with D-Loop (32%), similar to what is observed in Nodal mouse mutants. This result supports conserved mechanisms in the mouse and human, including plasticity of ventricle position after heart looping.</p></div>","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":"117 8","pages":"Pages S226-S227"},"PeriodicalIF":2.3000,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Archives of Cardiovascular Diseases","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S1875213624002353","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"CARDIAC & CARDIOVASCULAR SYSTEMS","Score":null,"Total":0}
引用次数: 0
Abstract
Introduction
Congenital heart defects in the context of heterotaxy are severe, with a complex anatomy. These defects are described using the binary concept of clinical loop (D-Loop or L-Loop), which implies that ventricle position is determined during heart looping. Recent work in the mouse has shown unexpected plasticity of ventricle position after heart looping. Based on a analysis of Nodal mutants with heterotaxy and right bronchus isomerism, 27% of D-Loop mutants at birth underwent leftward embryonic heart looping. These mice with a revertant loop, all display abnormal ventricle position.
Objective
Analysis of 3D ventricle position in human patients with heterotaxy and right bronchus isomerism, using the strategy developed in the mouse based on the 3D orientation of the interventricular septum.
Methods
Constitution of an heterotaxy cohort from the Necker database. Controls were selected as patients with transposition of the great arteries who had a systematic CT scan at 6 years. Three dimensions reconstructions of CT scans and quantitative analyses were performed using the Imaris software.
Expected results
Control patients had their right ventricle-left ventricle axis in the expected anteroposterior and left-right orientation. We collected 506 patients with heterotaxy syndrome, 186 of whom had CT scan. Forty patients had right bronchial isomerism. Among them, we found 29 (72%) patients who had an orientation of the interventricular septal axis similar to control, 7 (18%) patients with abnormal supero-inferior ventricles and 4 (10%) patients with abnormal left-right ventricles (Fig. 1). In total, 32% of patients in D-Loop have a malpositioned interventricular septum whereas 0 of patients in L-Loop.
Perspectives
There is a high proportion of malpositioned ventricles in patients with D-Loop (32%), similar to what is observed in Nodal mouse mutants. This result supports conserved mechanisms in the mouse and human, including plasticity of ventricle position after heart looping.
期刊介绍:
The Journal publishes original peer-reviewed clinical and research articles, epidemiological studies, new methodological clinical approaches, review articles and editorials. Topics covered include coronary artery and valve diseases, interventional and pediatric cardiology, cardiovascular surgery, cardiomyopathy and heart failure, arrhythmias and stimulation, cardiovascular imaging, vascular medicine and hypertension, epidemiology and risk factors, and large multicenter studies. Archives of Cardiovascular Diseases also publishes abstracts of papers presented at the annual sessions of the Journées Européennes de la Société Française de Cardiologie and the guidelines edited by the French Society of Cardiology.