Index
A
ACA26
Adaptive right ventricle
acute afterload—RV Anrep effect
in Eisenmenger syndrome
preserved fetal morphology
survival
miR 29 and 199b effects
ovine CHD model
fetal phenotype
RV nitric oxide signaling
AGC family kinases
Alternative mRNA splicing, in heart development
Alveolar bipotential progenitors
Alveolar epithelial progenitors (AEPs)
Alveolar epithelial stem cell niche
alveolar bipotential progenitors
alveolar epithelial progenitors
alveolospheres
Axin2
canonical Wnt signaling
compensatory regrowth
immune and stromal cells
mesenchymal alveolar niche cells
signaling pathways
type
Alveolar macrophages
Alveolar stem cell niche
Alveolospheres
AMP-activated protein kinase (AMPK)
Angiogenesis
Angiography
Apoptotic bodies
Arteriovenous fistula (AVF)
ATP-sensitive K channel (KATP)
Atrial appendage transcriptomes
Atrial fibrillation (AF)
genetics of
and pulmonary vein
transcription factors and
Atrio pulmonary connection (APC)
Axin2
B
Basigin (Bsg)
Betamethasone (BTM)
Blood-air barriers (BABs)
BMPR2 mutation
corrected HPAH iPSC-EC line
generation of iPSC Line
Bone marrow–derived macrophages
Bone morphogenetic protein 9 (BMP9)
Bone morphogenic protein receptor type 2 (BMPR2)
Brachyury
Bronchiolitis
Bronchopulmonary dysplasia (BPD)
C
Cadaveric lung transplantation
Calcium dependency
Candida albicans Cell Wall Extra (CAWE)
Cardiac catheterization
Cardiac cell specification
Cardiac conduction
Cardiac outflow tract development
Cardiac progenitor cells
Cardiac regulatory networks
Cardiac repair
Cardiomyocyte (CM)
differentiation
iPSC and differentiation into
proliferation
Cardiovascular diseases
Cardiovascular system
Cellular therapies
Ceramide
CHD
SeeCongenital heart disease
Chicken ductus arteriosus
cyclooxygenase, inhibition of
transcriptional profiles in
Chromosomal microarray
C-kit expression, in human induced pluripotent stem cells
Congenital cardiovascular disease
Congenital heart defects
Congenital heart disease (CHD)
elucidating, pathogenesis of
idiopathic pulmonary arterial hypertension
ovine model
palivizumab
pulmonary circulation
abnormalities of compliance
abnormal resistance
assessing RV-PA interaction/coupling
impedance analysis
non-pulsatile pulmonary flow
physiologic components
RSV infection
impact of
prevention
respiratory pathophysiology and
Congenital systemic-to-pulmonary shunt
Conotruncal heart defects (CTHDs)
COX-I
C-X-C chemokine receptor type 4 (CXCR4)
Cyclooxygenase contracts of chicken ductus arteriosus
Cyclooxygenase inhibitors
Cyclooxygenase (COX) pathway
Cyclophilin A (CyPA)
D
Diazoxide ductus arteriosus, dilatation of
Dicer
Distal angiogenesis
Distal muscularization process
DNA microarray analysis
Dorsal mesenchymal protrusion (DMP)
Dorsal pericardial wall (DPW)
Double outlet right ventricle (DORV)
Downstream analysis
Ductus arteriosus (DA)
chicken embryo as model for
clinical implications
closure of
constriction of
cyclooxygenase, inhibition of
dilatation of
genetic regulation
intimal thickening of
mediators
ceramide
H2S, vasoactivity of
isoprostanes
platelet-derived vasoactive mediators
molecular regulation
oxygen sensing and signaling
persistent patencyvs. delayed closure
pharmacological regulation
prostaglandin E-EP4-mediated fibulin-1
prostaglandin E1, long-term administration of
prostaglandin E2 receptor EP4 inhibition
pulmonary coarctation and proximal left pulmonary artery interruption
sixth pharyngeal arch formation and connections
transcriptional profiles in
treatment
decreased serum osmolality
glutamic acid
E
Early after-depolarizations (EADs)
Eisenmenger syndrome
adaptive right ventricle
preserved fetal morphology
survival
due to high pulmonary flow and idiopathic pulmonary arterial hypertension
preserved RV function
Embryonic stem cell (ESC)
Endocardial specific gene deletion, mouse models for
Endocardial Tie2
Endocardium as ventricular trabeculation regulator
Endogenous erythropoietin (Epo)/Epo receptor (EpoR) system
EndoMT
SeeEndothelial-to-mesenchymal transition
Endothelial cell communications
adapted extracellular matrix remodeling
defective BMPR-2 signaling system
pulmonary endothelial dysfunction
targeting cell proliferation and cell accumulation
targeting metabolic changes
vasodilation and vasoconstriction
vicious cycle
Endothelial cells (ECs)
vs. smooth muscle cells
vascular
Endothelial function
Endothelial-to-mesenchymal transition (EndoMT)
clinical implications
pathogenesis
hypoxia
inflammation
loss of endothelial junctions
molecular pathways
PAH pathological environment
shear stress
vascular remodeling
pathological process
polarity and barrier functions
pulmonary arterial hypertension
transcription factors Snail/Slug/ZEB/Twist
Engineered cardiac tissues (ECTs)
Eomesodermin (Eomes)
EP4 antagonist
Epithelial cell types
Epithelial-to-mesenchymal transition (EMT)
Exosomes (Exo)
Extracellular matrix (ECM)
Extracellular vesicles (EV)
apoptotic bodies
endothelial and smooth muscle cells
exosomes
future direction and clinical implications
microRNAs
microvesicles
pulmonary hypertension
F
Feeder cells
Fetal splice isoforms
Field-potential duration (FPD)
First heart field (FHF)
Fontan or Glenn circulation
G
GATA4
Gene expression
Gene mutation
Gene ontology (GO) terms
Generalized linear model (GLM)
Genetic analysis
Glial cell line-derived neurotrophic factor receptor alpha(Gfra)
Glial cell line–derived neurotrophic factor receptor alpha 2(Gfra2)
Glibenclamide
Glucocorticoid receptor (GR)
GluR1 proteins
Glutamic acid
Great artery malformation mechanisms
H
Hatching DA, transcriptional profiles in
HCN4 channel protein
Heart defects, in zebrafish
Heart development
alternative mRNA splicing in
model
Heart outflow tract
Hereditary hemorrhagic telangiectasia (HHT)
Heritable pulmonary arterial hypertension (HPAH)
BMPR2
clinical characteristics
genetic studies
hemodynamic characteristics
outcomes
dead and surviving patients
mutation carriers and non-carriers
pathobiology
patients selection
statistical analysis
targeted drug therapy
Hey1
Holt-Oram syndrome
HPAH
SeeHeritable pulmonary arterial hypertension
Human induced pluripotent stem cell (H-iPSC)
c-kit expression in
linear ECTs
generation and characterization
incorporating human compatible biomaterials into
optogenetic pacing of
Human umbilical arterial smooth muscle cells (hUASMCs)
Hyaluronic acid
Hypertrophy
Hypoplastic left heart syndrome (HLHS)
Hypotonic stimulation
Hypoxia-induced pulmonary hypertension (HPH)
interleukin (IL)-6
interleukin (IL)-21
Hypoxia-inducible factors (HIFs)
I
Idiopathic pulmonary arterial hypertension (IPAH)
clinical characteristics
with congenital heart disease
gene mutations
genetic studies
hemodynamic characteristics
outcomes
dead and surviving patients
mutation carriers and non-carriers
patients selection
prognosis
pulmonary vasodilators
statistical analysis
targeted drug therapy
time of government approval
Immortalized B cell lines, iPSC
Impaired right coronary vasodilator function
Implantable human arterial graft fabrication
Induced pluripotent stem cells (iPSCs)
cell adhesion and migration defect
future direction and clinical implications
from immortalized B cell lines and differentiation
model arterial ECs and distal PASMCs
omics analysis
pulmonary vasculature
RNA-seq
in vitro LQT3 model using
Inflammatory cytokines
HPH
interleukin (IL)-6
interleukin (IL)-21
M2 macrophage markers
Inhaled nitric oxide (iNO) therapy
Innate immune cells
Inositol triphosphate receptor (IP3R)
Interleukin (IL)-6
Interleukin (IL)-21
Intimal thickening (IT)
formation
prostaglandin E-EP4-mediated fibulin-1
In vitro LQT3 model
IPAH
SeeIdiopathic pulmonary arterial hypertension
iPSC-derived endothelial cell (iPSC-EC)
future aspects and clinical implications
gene editing
vs. PAH PAEC
unaffected BMPR2 mutation carriers
preserved EC function
preserved pP38 signaling pathway
iPSCs
SeeInduced pluripotent stem cells
Isoprostanes
K
Kawasaki disease
KEGG pathway
Kyoto Encyclopedia of Genes and Genomes (KEGG) pathways
L
lacZ reporter transgenic mouse line
Left atrial appendage (LAA)
Left ventricle noncompaction (LVNC)
Long QT syndrome (LQTS)
Lung
airways and epithelial components
ex vivo 4D imaging
morphogenesis and epithelial progenitors
NEB formation process
NE cell clusters at bifurcation point
NE cell marker CGRP
nodal/internodal NEBs
Notch-Hes1 signaling and NE cell development
Notch signaling pathways
organ culture
pseudoglandular stage
3D image analysis
3D mapping of epithelial cells
Lung biopsy
Lung mesenchyme
Lung-specific mesenchyme enhancer (LME)
M
Major aortopulmonary collateral arteries (MAPCAs)
Mammalian lung, alveolar epithelial stem cell niche
alveolar bipotential progenitors
alveolar epithelial progenitors
alveolospheres
Axin2
canonical Wnt signaling
compensatory regrowth
immune and stromal cells
mesenchymal alveolar niche cells
signaling pathways
type
Matrigel
MaxGel
Mesenchymal alveolar niche cells (MANCs)
Mesp1
MicroRNAs (miRNAs, miRs)
Microscopic lung airway abnormality
Microvesicles (MV)
mRNA splicing
Multicellular organismal signaling
Multiple peripheral pulmonary stenosis
Multipotent epithelial progenitors
Myocardial infarction (MI) model
N
N-cadherin
Neuroepithelial bodies (NEBs)
Next-generation sequencing (NGS)
Nitric oxide (NO)
Noncoding RNA (ncRNA)
Non-compaction cardiomyopathy
Non-pulsatile pulmonary flow
Fontan or Glenn circulation
protein-losing enteropathy
pulmonary artery pressure
pulmonary compliance or capacitance
pulsatile nature
“super-Fontan” strategy
total vascular impedance
vascular parameter
Non-steroidal anti-inflammatory drugs (NSAIDs)
Notch signaling
Novel cardiac progenitors
O
Optogenetic pacing (OP) of h-iPSC linear ECTs
Outflow tract (OFT)
Ovine CHD model
fetal phenotype
RV nitric oxide signaling
P
PAH
SeePulmonary arterial hypertension
Palivizumab, RSV infection
Patent ductus arteriosus (PDA)
Pdgfra-CreEr knock-in allele
Periodic hydrostatic pressure
Peripheral pulmonary stenosis (PPS)
Peri-pulmonary vein (PPV) myocardium
Peroxisome proliferator-activated receptors (PPARs)
Persistent truncus arteriosus (PTA)
Pharyngeal arch arteries (PAAs)
Platelet-derived vasoactive mediators
Polydimethylsiloxane (PDMS) molds
Primary cell lines
Primary spliceosome
Principal component analysis (PCA)
Private mutations
Progenitor cells
cardiac
during cardiac outflow tract development
resident
SHF cell regulation
SHF components
Progerin
Prostacyclin mimetics
ALK
cAMP generation
chemically stable analogues
clinical implications
cyclooxygenase pathway
highly proliferative phenotype
inhibition of cell proliferation
non-IP receptor targets
in PAH
BMPR2 and TGF-β signalling
pulmonary blood vessels
TASK-1, regulation of
Prostacyclin mimetics (cont. )
platelets, role of
potassium channel KCNK3 (TASK-1)
PPARs
prostanoid receptor expression profiles
prostanoids and downstream receptors
prostanoid synthesis and receptor expression
bronchial smooth muscle
downregulation of IP Receptors
EP 2 and EP 4 receptors
EP 3 receptors
pulmonary blood vessels
venous remodelling
prostanoidvs. non-prostanoid IP agonists
receptor selectivity
SMAD
vascular remodelling in vivo
Prostaglandin E1 (PGE 1 )
Prostaglandin E 2 (PGE 2 )
Prostaglandin E-EP4-mediated fibulin-1
Prostaglandin E2 receptor EP4 inhibition
Prostanoid synthesis and receptor expression
bronchial smooth muscle
in PAH
downregulation of IP Receptors
EP 2 and EP 4 receptors
EP 3 receptors
venous remodelling
pulmonary blood vessels
Proteaseactivated receptors (PARs)
Protein-losing enteropathy (PLE)
Pulmonary arterial endothelial cells (PAECs)
Pulmonary arterial hypertension (PAH)
CXCR4
IP3Rs
Pulmonary arterial smooth muscle cells (PASMCs)
Pulmonary artery (PA)
Pulmonary artery capacitance
Pulmonary blood vessels
in distal pulmonary artery
in distal pulmonary veins
endothelium
pulmonary artery
Pulmonary capillaries
Pulmonary circulation
congenital heart disease
abnormalities of compliance
abnormal resistance
assessing RV-PA interaction/coupling
impedance analysis
non-pulsatile pulmonary flow
physiologic components
Pulmonary coarctation (PC)
Pulmonary endothelial dysfunction
Pulmonary endothelium
Pulmonary hypertension associated with postoperative tetralogy of fallot
Pulmonary hypertension of the newborn (PPHN)
Pulmonary microvasculature
Pulmonary vascular disease associated with congenital systemic-to-pulmonary shunt
Pulmonary vascular inflammation
Pulmonary vascular remodeling
Pulmonary vasculature
Pulmonary vasodilators
Pulmonary vein (PV)
atrial fibrillation and
development
R
Rat ductus arteriosus
Respiratory syncytial virus (RSV) infection
clinical characteristics of
impact of
palivizumab
prevention of
respiratory pathophysiology and
Retinoic acid
Reverse transcription quantitative polymerase chain reaction (RT-qPCR)
Right coronary circulation
Right ventricular contractile force
Right ventricular ejection fraction (RVEF)
Right ventricular end-diastolic volume (RVEDV)
Right ventricular hypertrophy (RVH)
Right ventricular stroke volume (RVSV)
Risk stratification
cadaveric lung transplantation
multidimensional
patient impact
pulmonary arterial hypertension
causes
of children
vascular burden
ventricular function
RV Anrep effect, potential mechanisms
S
Sall1 gene
SCARNA1
scaRNABase database
scaRNAs
knockdown of
Second heart field (SHF)
at cardiac poles pass through transition zone
clinical implications
emergence of
epithelial properties of
Secreted frizzled-related protein 5(Sfrp5)
Selenoprotein P (SeP), PAH
Sema3c, temporo-spatial regulation of
Serum/glucocorticoid-regulated kinase 1 (SGK1)
Single-nucleotide polymorphisms (SNPs)
Small nucleolar RNAs (snoRNAs)
Smooth muscle cell (SMC)
Specialized smooth muscle cell progenitors
clinical implications
distal muscularization process
hypoxia-induced distal pulmonary arteriole
monoclonal expansion
signaling pathways regulating primed cells
Sphingomyelinases (SMases)
Splice isoforms
Stem cell antigen-1 (Sca-1)
Stem cell-derived cardiomyocytes
Stem cell factor (SCF) receptor
Sulfonylureas (SUs)
Surface antigen, cardiac progenitor cells
Synchrotron radiation (SR) microangiography
Systemic venous capacitance
T
TBX1
TBX5
TBX20
Tbx5 -dependent expression patterns
Tbx5 -dependent transcriptome
Tbx4 expression
TBX4 mutations
Temporo-spatial regulation, of Sema3c
Tetralogy of Fallot (TOF)
pulmonary hypertension associated with
splice isoforms in
Three-dimensional echocardiography
Thrombin
Tie receptors
Tie2 ventricular chamber development, essential for
Total cavo-pulmonary connection (TCPC)
Transcriptional profiling
Transcription factor AP-2 beta (tfap2b)
Transcription factors (TF)
Transforming growth factor beta (TGF-β) receptor family
Transforming growth factor-β (TGF-β) pathway
Transition zone (TZ)
Transplacental fetal death
Transposition of great arteries (TGA)
TRP melastatin 3 (TRPM3)
Tube formation assay
Type 2 associated stromal cells (TASCs)
U
UMC
SeeUnaffected BMPR2 mutation carriers
Unaffected BMPR2 mutation carriers (UMC)
preserved EC function
preserved pP38 signaling pathway
V
Vascular casts
Vascular endothelial cells
Vascular endothelial growth factor (VEGF) receptors
Vasculogenesis
W
Whole-exome sequencing (WES)
Wnt5a
X
X-ray phase contrast tomography (XPCT)
Z
Zebrafish
scaRNAs, knockdown
splice isoforms change during development in