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Temporal single-cell transcriptomic profiling of cardiac differentiation in pulmonary atresia with intact ventricular septum (PA-IVS) derived induced pluripotent stem cells

ABSTRACT: Temporal single-cell transcriptomic profiling of cardiac differentiation in pulmonary atresia with intact ventricular septum (PA-IVS) derived induced pluripotent stem cells

PROVIDER: PRJNA926106 | ENA |

REPOSITORIES: ENA

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			Action	DRS
	SRR23176553_1.fastq.gz	Fastqsanger.gz
	SRR23176553_2.fastq.gz	Fastqsanger.gz
	SRR23176554_1.fastq.gz	Fastqsanger.gz
	SRR23176554_2.fastq.gz	Fastqsanger.gz
	SRR23176555_1.fastq.gz	Fastqsanger.gz

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Temporal single-cell transcriptomic profiling of cardiac differentiation in pulmonary atresia with intact ventricular septum (PA-IVS) derived induced pluripotent stem cells

Project description:Pulmonary atresia with the intact ventricular septum (PA-IVS) is a rare congenital cardiac disease characterized by atresia of the pulmonary valve, resulting in the absence of a connection between the right ventricular outflow tract and pulmonary arteries. PA-IVS is characterized by varying degrees of right ventricular hypoplasia: from single ventricle palliation (1v) to 1½-ventricle palliation (1.5v) and bi-ventricle repair (2v). To understand how cardiac development is impaired in PA-IVS-1v, we generated PA-IVS-1v patient-specific induced pluripotent stem cells (iPSCs). We then ran single-cell RNA-seq to temporally profile transcriptomic changes during cardiac differentiation in healthy control (Control) and PA-IVS-1v iPSCs. We collected differentiating cells at multiple time points corresponding to different development stages, ie. Day 5 (cardiac mesoderm), Day 10 (cardiac progenitor), Day 14 (early cardiomyocyte), and Day 30 (fetal cardiomyocyte). Single-cell transcriptomic analysis indicates that cell lineage commitment of cardiac progenitors is skewed towards epicardial and first heart field progenitors in the differentiating iPSCs from PA-IVS-1v.

2024-03-13 | GSE223414 | GEO

intact Hi-C from cardiac septum (ENCSR556QHB)

Project description:w61 cardiac septum intact dnase hic For data usage terms and conditions, please refer to http://www.genome.gov/27528022 and http://www.genome.gov/Pages/Research/ENCODE/ENCODE_Data_Use_Policy_for_External_Users_03-07-14.pdf

2023-07-24 | GSE238038 | GEO

Long-term outcomes after intervention for pulmonary atresia with intact ventricular septum.

Project description:OBJECTIVE:Pulmonary atresia with intact ventricular septum (PA/IVS) can be treated by various operative and catheter-based interventions. We aim to understand the long-term transplant-free survival of patients with PA/IVS by treatment strategy. METHODS:Cohort study from the Pediatric Cardiac Care Consortium, a multi-institutional registry with prospectively acquired outcome data after linkage with the National Death Index and the Organ Procurement and Transplantation Network. RESULTS:Eligible patients underwent neonatal surgery or catheter-based intervention for PA/IVS between 1982 and 2003 (median follow-up of 16.7 years, IQR: 12.6-22.7). Over the study period, 616 patients with PA/IVS underwent one of three initial interventions: aortopulmonary shunt, right ventricular decompression or both. Risk factors for death at initial intervention included earlier birth era (1982-1992), chromosomal abnormality and atresia of one or both coronary ostia. Among survivors of neonatal hospitalisation (n=491), there were 99 deaths (4 post-transplant) and 10 transplants (median age of death or transplant 0.7 years, IQR: 0.3-1.8 years). Definite repair or last-stage palliation was achieved in the form of completed two-ventricle repair (n=201), one-and-a-half ventricle (n=39) or Fontan (n=96). Overall 20-year survival was 66%, but for patients discharged alive after definitive repair, it reached 97.6% for single-ventricle patients, 90.9% for those with one-and-a-half ventricle and 98.0% for those with complete two-ventricle repair (log-rank p=0.052). CONCLUSIONS:Transplant-free survival in PA/IVS is poor due to significant infantile and interstage mortality. Survival into early adulthood is excellent for patients reaching completion of their intended path independent of type of repair.

| S-EPMC6571047 | biostudies-literature

Right ventricular thrombus-induced myocardial infarction after Fontan surgery in pulmonary atresia with intact ventricular septum.

Project description: Not available

| S-EPMC8669549 | biostudies-literature

Transcriptomic analysis (RNA-seq) of early iPSC-CMs between PA-IVS and healthy control.

Project description:Transcriptomic analysis by RNA-seq revealed that pathways associated with cell cycle and mitosis were downregulated in day 13 PA-IVS-1v iPSC derived cardiomyocytes.

2024-03-12 | GSE233349 | GEO

Generation of an induced pluripotent stem cell line NCHi003-A from a 11-year-old male with pulmonary atresia with intact ventricular septum (PA-IVS).

Project description:Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare congenital heart defect defined by membranous or muscular atresia of the right ventricular outflow tract where patients display varying degrees of hypoplasia of the right ventricle. This condition results in cyanosis due to an inability of blood to flow from the right ventricle to the pulmonary arteries, thus requiring immediate surgical intervention after birth. An iPSC line was generated from peripheral blood mononuclear cells of a 11-year-old male patient diagnosed with PA-IVS through Sendai virus-mediated reprogramming. This disease-specific iPSC line was characterized by immunocytochemistry, STR analysis, karyotype analysis, and mycoplasma testing.

| S-EPMC9629876 | biostudies-literature

A method for cryopreservation and single nucleus RNA-sequencing of normal adult human interventricular septum heart tissue reveals cellular diversity and function

Project description:We developed a method for cryopreserving human heart tissue for later snRNA-seq analysis. We profiled four unused donor hearts to generate a representative snRNAseq dataset. This is the first dataset that focuses on adult human interventricular septum tissue, and our results reveal extensive cellular diversity. IVS cardiomyocytes and fibroblasts show divergent biological pathway activity.

2021-06-01 | GSE161921 | GEO

Elabela: A Novel Biomarker for Right Ventricular Pressure Overload in Children With Pulmonary Stenosis or Pulmonary Atresia With Intact Ventricular Septum.

Project description:Background: Assessing right ventricular overload in children is challenging. We conducted this study involving children with pulmonary valvular stenosis (PS) or pulmonary atresia with intact ventricular septum (PA/IVS) to evaluate the potential of a new endogenous ligand of apelin receptor, Elabela (ELA), as a potential biomarker for right heart overload. Methods: In this prospective cohort study, a total of 118 congenital heart diseases patients with right ventricle outflow tract obstruction were recruited from 2018 to 2019. Among them, 44 isolated PS and 7 PA/IVS patients were selected. Their venous blood was collected, and all patients underwent an echocardiographic examination. Among them, post-operative blood was collected from 24 patients with PS after percutaneous balloon pulmonary valvuloplasty. The plasma ELA concentration was measured using enzyme-linked immunosorbent assay. Results: The ELA was significantly associated with the peak transvalvular pulmonary gradient (r = -0.62; p = 0.02), thus reflecting the severity of PS or PA/IVS. The ELA significantly increased at 3 days after intervention, when mechanical obstruction of the right outflow tract was relieved. Based on the receiver-operator characteristic curve results, ELA could be a risk factor for duct dependence in patients with critical PS or PA/IVS who are younger than 6 months (AUC: 0.82). Conclusion: ELA concentration and severity of PS or PA/IVS had a significant negative correlation, indicating that ELA might be a novel biomarker for right ventricular afterload and reflect the immediate pressure changes in the right heart. Furthermore, ELA could predict duct-dependency in PS and PA/IVS patients, as valuable as classical echocardiographic indexes.

| S-EPMC7688667 | biostudies-literature

Mini-Konno procedure using a 10 mm handmade valve for pulmonary atresia with intact ventricular septum and severe aortic stenosis.

Project description:We herein report the case of a neonate with pulmonary atresia with intact ventricular septum, right ventricular hypoplasia and severe aortic stenosis. Repetitive aortic valve surgeries in early infancy resulted in early relapse of the aortic stenosis due to the small aortic annulus, bicuspid configuration and duct-dependent pulmonary circulation. The infant underwent a successful mini-Konno procedure using a 10 mm handmade valve and bidirectional Glenn shunt at 6 months of age.

| S-EPMC4051372 | biostudies-literature

Pulmonary Atresia with Intact Ventricular Septum associated with Ventriculo-Coronary Arterial Communication in a Fetus at 21 Weeks of Gestation.

Project description:Pulmonary atresia with the intact ventricular septum (PA-IVS) is a rare anomaly that has an absent communication between the right ventricle and pulmonary arterial circulation. PA-IVS has a hypoplastic and hypokinetic and thickened right ventricle with the intact ventricular septum. It can be diagnosed with PA-IVS in routine obstetric ultrasound examination because the four-chamber view of PA-IVS is generally abnormal. The size of the right ventricular cavity is proportional to the Z value of the diameter of the tricuspid valve. The essential associated cardiac finding of PA-IVS is ventricular-coronary arterial communications (VCAC). The hypertensive RV forces blood through the intramyocardial sinusoids with continuous fistulous contact with the epicardial branches of RCA. It is called the VCAC. A color Doppler can detect VCAC due to its location in the pericardium along the coronary artery. If patients have VCAC and tricuspid Z score below -4, all of these conditions are infallible signs of high mortality rates in their fetal or postnatal lives. Our aim in presenting this case is to remind perinatologists if they detect an abnormal four-chamber view of the fetus's heart, they should also carefully examine whether VCAC exists. Herein, we report a case of PA-IVS and VCAC at 21 weeks' gestation with fetal echocardiographic images.

| S-EPMC7378622 | biostudies-literature

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