scholarly journals The Role of Cell Tracing and Fate Mapping Experiments in Cardiac Outflow Tract Development, New Opportunities through Emerging Technologies

2021 ◽  
Vol 8 (5) ◽  
pp. 47
Author(s):  
Joshua C. Peterson ◽  
Tim P. Kelder ◽  
Marie José T. H. Goumans ◽  
Monique R. M. Jongbloed ◽  
Marco C. DeRuiter
Keyword(s):  
Congenital Heart ◽  
Emerging Technologies ◽  
Heart Diseases ◽  
Cell Lineage ◽  
Outflow Tract ◽  
Fate Mapping ◽  
Cell Tracing ◽  
Morphologic Variation ◽  
Tracing Techniques ◽  
Cardiac Outflow

Whilst knowledge regarding the pathophysiology of congenital heart disease (CHDs) has advanced greatly in recent years, the underlying developmental processes affecting the cardiac outflow tract (OFT) such as bicuspid aortic valve, tetralogy of Fallot and transposition of the great arteries remain poorly understood. Common among CHDs affecting the OFT, is a large variation in disease phenotypes. Even though the different cell lineages contributing to OFT development have been studied for many decades, it remains challenging to relate cell lineage dynamics to the morphologic variation observed in OFT pathologies. We postulate that the variation observed in cellular contribution in these congenital heart diseases might be related to underlying cell lineage dynamics of which little is known. We believe this gap in knowledge is mainly the result of technical limitations in experimental methods used for cell lineage analysis. The aim of this review is to provide an overview of historical fate mapping and cell tracing techniques used to study OFT development and introduce emerging technologies which provide new opportunities that will aid our understanding of the cellular dynamics underlying OFT pathology.

scholarly journals Single-cell RNA-seq of the Developing Cardiac Outflow Tract Reveals Convergent Development of the Vascular Smooth Muscle Cells at the Base of the Great Arteries

2018 ◽  
Author(s):  
Xuanyu Liu ◽  
Wen Chen ◽  
Wenke Li ◽  
James R. Priest ◽  
Jikui Wang ◽  
...  
Keyword(s):  
Single Cell ◽  
Single Molecule ◽  
Heart Diseases ◽  
Marker Gene ◽  
Outflow Tract ◽  
State Transitions ◽  
Intermediate Cell ◽  
Mesenchymal Transition ◽  
Cell Lineages ◽  
Cardiac Outflow

ABSTRACTRationaleCardiac outflow tract (OFT) is a major hotspot for congenital heart diseases (CHDs). A thorough understanding of the cellular diversity, transitions and regulatory networks of normal OFT development is essential to decipher the etiology of OFT malformations.ObjectiveWe sought to explore the cellular diversity and transitions between cell lineages during OFT development.Methods and ResultsWe performed single-cell transcriptomic sequencing of 55,611 mouse OFT cells from three developmental stages that generally correspond to the early, middle and late stages of OFT remodeling and septation. We identified 17 cell clusters that could be assigned to six cell lineages. Among these lineages, the macrophage and VSMC lineages of the developing OFT have seldom been previously described. Known cellular transitions, such as endothelial to mesenchymal transition, have been recapitulated. In particular, we identified convergent development of the VSMC lineage, where intermediate cell subpopulations were found to be involved in either myocardial to VSMC trans-differentiation or mesenchymal to VSMC transition. Through single-molecule in situ hybridization, we observed that cells expressing the myocardial marker Myh7 co-expressed the VSMC marker gene Cxcl12 in OFT walls, thus confirming the existence of myocardial to VSMC trans-differentiation. Moreover, we found that the Penk+ cluster c8, a relatively small mesenchymal subpopulation that was undergoing mesenchymal to VSMC transition, was associated with the fusion of OFT cushions. We also uncovered the expression dynamics and critical transcriptional regulators potentially governing cell state transitions. Finally, we developed web-based interactive interfaces to facilitate further data exploration.ConclusionsWe provide a single-cell reference map of cell states for normal OFT development, which will be a valuable resource for the CHD community. Our data support the existence of myocardial to VSMC trans-differentiation and convergent development of the VSMC lineage at the base of the great arteries.

scholarly journals Right Ventricular Outflow Tract Obstruction Caused by Double-Chambered Right Ventricle Presenting in Adulthood

2014 ◽  
Vol 41 (4) ◽  
pp. 425-428 ◽  
Author(s):  
John Kokotsakis ◽  
Efthymia G. Rouska ◽  
Leanne Harling ◽  
Hutan Ashrafian ◽  
Vania Anagnostakou ◽  
...  
Keyword(s):  
Right Ventricle ◽  
Right Ventricular ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Outflow Tract ◽  
Surgical Correction ◽  
Ventricular Outflow Tract Obstruction ◽  
The Right

Congenital heart diseases that cause obstruction of the right ventricular outflow tract are often difficult to diagnose. We report the case of a 49-year-old man who presented with long-standing shortness of breath on exertion. Imaging revealed right ventricular outflow tract obstruction caused by a double-chambered right ventricle, and he was referred for surgical correction. This case emphasizes both the detailed perioperative evaluation that is needed when diagnosing adults who present with manifestations of congenital heart disease and a method of successful surgical correction that resulted in symptom resolution.

Short and intermediate term outcome post right ventricle to pulmonary artery conduit surgery

QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Mohammed Abdullah Mohammed Mustafa Hegab ◽  
Amr Mansour Mohammed ◽  
Yasmine Abd Elrazek Ismail ◽  
Alaa Mahmoud Roshdy
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Artery ◽  
Right Ventricle ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Right Ventricular Outflow Tract ◽  
Outflow Tract ◽  
Term Outcome ◽  
The Right

Abstract Background Surgery for congenital heart disease has progressed by leaps and bounds in the last few decades, but the right ventricular outflow tract continues to pose a challenge to the congenital heart surgeon. Objective To describe short and intermediate term outcome in congenital heart disease patients undergoing surgical repair using right ventricle to pulmonary artery conduits. Patients and Methods Our study included 33 patients that were operated upon by putting a conduit between the right ventricle and the pulmonary artery in a single center (Al Agouza police hospital) between 2015 and 2019. Results This study was done in order to follow up patients who underwent surgery for conduit placement between the right ventricle (RV) and the pulmonary artery (PA), to observe the reintervention rates and to determine the most important determinants of re-operation. 33 patients were included in this study, with age range 1.5-17 years and mean age of 8.29 +/- 4.7 years. The mean age at placement of the first conduit was 3.57 +/- 3.18 years. The youngest patient at time of conduit placement for the first time was 0.2 years old and the oldest had 12.5 years. Conclusion The use of conduits to treat the RV to PA discontinuity is a cornerstone in the treatment of some congenital heart diseases requiring construction of the right ventricle outflow tract (RVOT). Nevertheless, conduit failure and replacement is inevitable, and depends on many factors: as age at first operation, type of conduit, mean and peak pressure gradient across the conduit. The higher the age at first conduit, the bigger the event / re-intervention free survival period.

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