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MCID: PLM022
MIFTS: 45

Pulmonary Valve Insufficiency

Categories: Cardiovascular diseases, Respiratory diseases
Genes Variations Tissues Related diseases Publications Pathways Drugs
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Aliases & Classifications for Pulmonary Valve Insufficiency

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MalaCards integrated aliases for Pulmonary Valve Insufficiency:

Name: Pulmonary Valve Insufficiency 12 44 15 70 32
Pulmonary Regurgitation 12 54
Pulmonary Incompetence 12 6
Pulmonary Insufficiency Following Trauma and Surgery 12
Pulmonary Incompetence, Non-Rheumatic 12
Pulmonic Valve Regurgitation 12
Pulmonic Insufficiency 12
Pulmonary Regurg. 12

Classifications:

MalaCards categories:
Anatomical: Cardiovascular diseases Respiratory diseases
See all MalaCards categories (disease lists)
ICD10: 32


External Ids:

Disease Ontology 12 DOID:14265
MeSH 44 D011665
NCIt 50 C51447
SNOMED-CT 67 91434003
ICD10 32 I37.1
UMLS 70 C0034088
Sources

Summaries for Pulmonary Valve Insufficiency

About this section
Disease Ontology : 12 A pulmonary valve disease that occurs when the pulmonary valve is not strong enough to prevent backflow into the right ventricle. If it is secondary to pulmonary hypertension it is referred to as a Graham Steell murmur.

MalaCards based summary : Pulmonary Valve Insufficiency, also known as pulmonary regurgitation, is related to respiratory failure and pericarditis. An important gene associated with Pulmonary Valve Insufficiency is PMS2 (PMS1 Homolog 2, Mismatch Repair System Component), and among its related pathways/superpathways are Renin secretion and Cardiomyocyte Differentiation through BMP Receptors. The drugs Neostigmine and Acetazolamide have been mentioned in the context of this disorder. Affiliated tissues include heart, lung and liver, and related phenotypes are cardiovascular system and homeostasis/metabolism

Sources

Related Diseases for Pulmonary Valve Insufficiency

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Diseases in the Pulmonary Valve Disease family:

Pulmonary Valve Insufficiency

Diseases related to Pulmonary Valve Insufficiency via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 249)
# Related Disease Score Top Affiliating Genes
1 respiratory failure 30.8 REN PMS2 NPPB EDN1 CRP ACE
2 pericarditis 30.2 NPPB CRP ACE
3 right bundle branch block 30.0 NPPB BMPR2 ACE
4 pulmonary embolism 29.9 NPPB CRP ACE
5 pulmonary valve disease 29.8 NPPB FBN1
6 ischemia 29.7 PDE5A NPPB EDN1 ACE
7 acute myocardial infarction 29.7 NPPB EDN1 CRP ACE
8 constrictive pericarditis 29.5 REN NPPB CRP ACE
9 tetralogy of fallot 29.5 REN NPPB FBN1 EDN1 CRP ACE
10 atrial heart septal defect 29.3 NPPB FBN1 EDN1 CRP BMPR2 ACE
11 aortic valve insufficiency 29.2 REN NPPB FBN1 CRP ACE
12 mitral valve stenosis 29.2 REN NPPB EDN1 CRP ACE
13 congestive heart failure 29.1 REN PDE5A NPPB EDN1 CRP ACE
14 tricuspid valve insufficiency 29.0 REN PDE5A NPPB FBN1 EDN1 CRP
15 mitral valve insufficiency 28.9 REN NPPB FBN1 CRP ACE
16 patent ductus arteriosus 1 28.8 REN NPPB FBN1 EDN1 CRP ACE
17 vascular disease 28.4 REN NPPB EDN1 CRP BMPR2 ACVRL1
18 heart disease 28.2 REN PDE5A NPPB FBN1 EDN1 CRP
19 pulmonary hypertension 27.7 REN PTGIR PDE5A NPPB KCNK3 EDN1
20 rheumatic pulmonary valve disease 11.1
21 toxic myocarditis 10.3 NPPB ACE
22 chronic congestive splenomegaly 10.3 PMS2 ABCA3
23 anterolateral myocardial infarction 10.3 NPPB ACE
24 inferior myocardial infarction 10.3 NPPB ACE
25 eisenmenger syndrome 10.3 EDN1 BMPR2
26 vasculogenic impotence 10.3 PDE5A ACE
27 acute dacryoadenitis 10.3 CRP ACE
28 bladder neck obstruction 10.3 PDE5A ACE
29 mediastinum teratoma 10.3 CRP ACE
30 lipid pneumonia 10.3 CRP ABCA3
31 chronic mountain sickness 10.3 NPPB EDN1
32 anterior scleritis 10.3 CRP ACE
33 hydrops of gallbladder 10.3 NPPB CRP
34 multiple cranial nerve palsy 10.3 CRP ACE
35 cranial nerve palsy 10.3 CRP ACE
36 posterior scleritis 10.3 CRP ACE
37 tolosa-hunt syndrome 10.3 CRP ACE
38 chronic orbital inflammation 10.3 CRP ACE
39 silent myocardial infarction 10.3 CRP ACE
40 glossopharyngeal nerve disease 10.3 CRP ACE
41 granulomatous angiitis 10.3 CRP ACE
42 urinary tract infection 10.3 CRP ACE
43 pulmonary valves agenesis 10.3
44 pulmonary valve agenesis-intact ventricular septum-persistent ductus arteriosus syndrome 10.3
45 pulmonary valve agenesis-tetralogy of fallot-absence of ductus arteriosus syndrome 10.3
46 cerebral arteritis 10.2 EDN1 CRP
47 neuroretinitis 10.2 CRP ACE
48 optic papillitis 10.2 CRP ACE
49 granulomatous hepatitis 10.2 CRP ACE
50 diffuse scleroderma 10.2 EDN1 ACE

Graphical network of the top 20 diseases related to Pulmonary Valve Insufficiency:



Diseases related to Pulmonary Valve Insufficiency
Sources

Symptoms & Phenotypes for Pulmonary Valve Insufficiency

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MGI Mouse Phenotypes related to Pulmonary Valve Insufficiency:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.85 ABCA3 ACE ACVRL1 BMPR2 CRP EDN1
2 homeostasis/metabolism MP:0005376 9.7 ABCA3 ACE ACVRL1 BMPR2 CRP EDN1
3 renal/urinary system MP:0005367 9.17 ABCA3 ACE ACVRL1 BMPR2 EDN1 FBN1
Sources

Drugs & Therapeutics for Pulmonary Valve Insufficiency

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Drugs for Pulmonary Valve Insufficiency (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 120)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Neostigmine Approved, Vet_approved Phase 4 59-99-4 4456
2
Acetazolamide Approved, Vet_approved Phase 4 59-66-5 1986
3
Rocuronium Approved Phase 4 119302-91-9, 143558-00-3 441290
4 Natriuretic Peptide, Brain Phase 4
5 Cholinesterase Inhibitors Phase 4
6 Carbonic Anhydrase Inhibitors Phase 4
7 Neuromuscular Nondepolarizing Agents Phase 4
8 Neuromuscular Blocking Agents Phase 4
9 Anticonvulsants Phase 4
10 Bromides Phase 4
11
Furosemide Approved, Vet_approved Phase 3 54-31-9 3440
12
Hydroxychloroquine Approved Phase 3 118-42-3 3652
13
Azithromycin Approved Phase 3 83905-01-5 447043 55185
14
Propofol Approved, Investigational, Vet_approved Phase 3 2078-54-8 4943
15
Rivaroxaban Approved Phase 3 366789-02-8
16
Doxapram Approved, Vet_approved Phase 3 309-29-5 3156
17
Nitric Oxide Approved Phase 3 10102-43-9 145068
18
Beractant Approved Phase 3 108778-82-1
19
Dobutamine Approved Phase 3 34368-04-2 36811
20 Anticoagulants Phase 3
21 Analgesics, Non-Narcotic Phase 3
22 Antirheumatic Agents Phase 3
23 Anti-Inflammatory Agents Phase 3
24 Anti-Inflammatory Agents, Non-Steroidal Phase 3
25 Anti-Arrhythmia Agents Phase 3
26 Gastrointestinal Agents Phase 3
27 Immunologic Factors Phase 3
28 Adjuvants, Immunologic Phase 3
29 Sympatholytics Phase 3
30 Enkephalins Phase 3
31 Anti-Ulcer Agents Phase 3
32 Antacids Phase 3
33 enkephalin-Leu, Ala(2)-Arg(6)- Phase 3
34 Hypnotics and Sedatives Phase 3
35 Serine Proteinase Inhibitors Phase 3
36 Antithrombin III Phase 3
37
protease inhibitors Phase 3
38 Factor Xa Inhibitors Phase 3
39 HIV Protease Inhibitors Phase 3
40 Antithrombins Phase 3
41 Central Nervous System Stimulants Phase 3
42 Respiratory System Agents Phase 3
43 Neurotransmitter Agents Phase 3
44 Antioxidants Phase 3
45 Endothelium-Dependent Relaxing Factors Phase 3
46 Anti-Asthmatic Agents Phase 3
47 Bronchodilator Agents Phase 3
48 Protective Agents Phase 3
49 glutamine Phase 2, Phase 3
50 Pulmonary Surfactants Phase 3

Interventional clinical trials:

(show top 50) (show all 256)
# Name Status NCT ID Phase Drugs
1 The Effect of Immunonutrition on Outcome and Postoperative Recovery in Patients Undergoing Elective Surgical Repair of a Thoraco (Abdominal) Aneurysm Aorta Unknown status NCT00339053 Phase 4 Immunonutrition ( Impact)
2 Non-Invasive Positive Pressure Mask Ventilation vs Extrathoracic Biphasic Cuirass Ventilation in Patients With Acute Respiratory Failure: A Randomized Prospective Study. Unknown status NCT00331656 Phase 4
3 Phase 4, Single Centre, Single Blinded,Prospective Randomised Cross Over Comparison on the Physiological Response of Post Extubation Intensive Care Patients to Non-invasive Ventilation Using Either Air O2 or Heliox21 Unknown status NCT01498432 Phase 4 Heliox21;Air O2
4 Effect of Neuromuscular Blockade and Reversal by Sugammadex Versus Neostigmine on Breathing When Hypoxic or Hypercapnic in Volunteers Completed NCT02845375 Phase 4 Sugammadex;Neostigmine;Placebo
5 Acetazolamide for Respiratory Failure in Combination With Metabolic Alkalosis Completed NCT00222534 Phase 4 Acetazolamide;Placebo
6 Does a Diagnostic Strategy Reduce Duration and Cost of Hospitalization in Patients With Acute Dyspnea? BASEL II Intensive Care Unit Completed NCT00130559 Phase 4
7 Effects of Remifentanil on Breathing Pattern, Respiratory Effort and Gas Exchange in Mechanically Ventilated Patients. Completed NCT00665119 Phase 4 remifentanil;placebo
8 Effect of Non-invasive Ventilation (NIV) on Cerebral Oxygenation Completed NCT02454582 Phase 4
9 Partial Neuromuscular Blockade to Facilitate Lung and Diaphragm Protective Mechanical Ventilation in Intensive Care Unit Patients: a Randomized Controlled Pilot Study Recruiting NCT03646266 Phase 4 Rocuronium Bromide
10 Assessment of the Effectivity of Total-face Mask for Noninvasive Ventilation During Acute Respiratory Failure: a Randomized Controlled Trial. Terminated NCT00243828 Phase 4
11 Randomized Controlled Trial Evaluating the Transcutaneous Carbon Dioxide Measurements in Obese Women Using Intrathecal Morphine Versus Patient-Controlled Intravenous Hydromorphone for Post-Cesarean Analgesia Withdrawn NCT03282669 Phase 4 Intrathecal Morphine;Intravenous hydromorphone
12 Multi-center Trial of Percutaneous Pulmonary Valve Implantation Using Venus-P Valve for Patients With Severe Pulmonary Regurgitation and Native Right Ventricular Outflow Tract After Previous Surgical Repair Unknown status NCT02590679 Phase 2, Phase 3
13 Efficacy and Safety Evaluation of Transcatheter Pulmonary Valve Implantation for Right Ventricular Outflow Tract (RVOT) Stenosis After Congenital Heart Defect Surgical Correction Complicated With Severe Pulmonary Regurgitation Unknown status NCT02071654 Phase 3
14 Hemofiltration for Respiratory Failure Following Hematopoietic Stem Cell Transplantation Unknown status NCT00120575 Phase 2, Phase 3
15 Study of Efficacy and Tolerance of Early Launching of Nocturnal Non Invasive Ventilation in Adults With Myotonic Dystrophy Type 1(DM1) Unknown status NCT01225614 Phase 3
16 Fluids in Mechanically Ventilated Children With Acute Infectious Lung Disease: How Dry Should They be? Unknown status NCT02989051 Phase 2, Phase 3
17 Pulmonary Valve Replacement : Study of Comparison Between a Standard Surgical Approach With Extracorporeal Circulation and an Off-pump Hybrid Strategy. Completed NCT00259207 Phase 3
18 Efficacy and Safety of NIPPV to Increase Survival Without Bronchopulmonary Dysplasia in Extremely Low Birth Weight Infants Completed NCT00433212 Phase 3
19 Efficacy of Early Tracheostomy to Reduce Incidence of Ventilator Acquired Pneumonia (VAP) Completed NCT00262431 Phase 3
20 Weaning of Mechanical Ventilation Guided by the Natriuretic Peptide of Type B Completed NCT00473148 Phase 3
21 An Open Randomized Study of the Effectiveness of the Drug Dalargin for the Prevention and Treatment of Symptoms of Pulmonary Complications in Patients With Coronavirus Infection (SARS-COVID-19) Completed NCT04346693 Phase 3
22 Fiberoptic Bronchoscopy With Broncho-alveolar Lavage Under Noninvasive Ventilation With Propofol Target Controled Infusion in Patient With Acute Hypoxemic Respiratory Failure. A Randomized Controlled Study : SEDA-FIBRO. Completed NCT00741949 Phase 3 Propofol;Placebo
23 The Evaluation of the Astral VAPS AutoEPAP Treatment Algorithm Completed NCT02683772 Phase 3
24 Sedation Management in Pediatric Patients With Acute Respiratory Failure Completed NCT00814099 Phase 3
25 Efficacy and Safety of Targeting Lower Arterial Oxygen Saturations to Reduce Oxygen Toxicity and Oxidative Stress in Very Preterm Infants: The Canadian Oxygen Trial (COT) Completed NCT00637169 Phase 3
26 Medically Ill Patient Assessment of Rivaroxaban Versus Placebo in Reducing Post-Discharge Venous Thrombo-Embolism Risk Completed NCT02111564 Phase 3 Rivaroxaban, 10 mg;Rivaroxaban, 7.5 mg;Placebo
27 Doxapram Versus Placebo in Preterm Newborns: An International Double Blinded Multicenter Randomized Controlled Trial Recruiting NCT04430790 Phase 3 Doxapram;Placebo
28 Airway Clearance Using Non-Invasive Oscillating Device in Critically Ill Children Not yet recruiting NCT03821389 Phase 2, Phase 3
29 The Effects of Inhaled Nitric Oxide in the Treatment of Acute Hypoxemic Respiratory Failure (AHRF) In Pediatrics Terminated NCT00041561 Phase 3 Nitrogen gas;inhaled nitric oxide
30 Early Inhaled Nitric Oxide Therapy in Term and Near Term Infants With Respiratory Failure Terminated NCT00005773 Phase 3 Inhaled Nitric Oxide;Standard iNO therapy
31 Randomized Clinical Trial Comparing Enteral Glutamine Supplementation to Standard of Care Enteral Feeding in Critical Illness Terminated NCT00318331 Phase 2, Phase 3 Glutamine
32 Early Tracheotomy Versus Prolonged Endotracheal Intubation in Intensive Care Unit Patients Terminated NCT00127621 Phase 3
33 The Randomized Inhaled Nitric Oxide Study (NINOS) in Full-Term and Nearly Full-Term Infants With Hypoxic Respiratory Failure Terminated NCT00005776 Phase 3 Inhaled nitric oxide;Placebo
34 Early Surfactant Followed by Nasal CPAP to Reduce the Use of Mechanical Ventilation Without Additional Morbidity in Infants 1250- 2000 Grams With RDS Terminated NCT00005774 Phase 3 Early surfactant;Standard practice
35 Can Diaphragm Pacing Delay Non Invasive Ventilation in Amyotrophic Lateral Sclerosis ? a Randomized Controlled Study Terminated NCT01583088 Phase 3
36 Cardiac Imaging Under Exercise Stress Test for Early Assessment of Right Ventricular Function in Patients With Tetralogy of Fallot and Pulmonary Regurgitation Terminated NCT00564993 Phase 3 Dobutamin
37 Compassionate Use of the Hattler Respiratory Assist Catheter in Severe Respiratory Failure - A Clinical Trial Withdrawn NCT00288964 Phase 3
38 Non-invasive Respiratory Mechanics Assessment During Spontaneous / Assisted Breathing: The ERNI (Elastance, Resistance, Non Invasive Measurements) Study Unknown status NCT01530282 Phase 2
39 Impact of Pressure Support Ventilation (PSV) Versus Neurally Adjusted Ventilatory Assist (NAVA) Diaphragm Efficiency Completed NCT02473172 Phase 2
40 Electrical Activity of the Diaphragm in Mechanically Ventilated Patients During the Weaning Period Completed NCT01411722 Phase 2
41 Effectiveness and Safety of Telmisartan in Acute Respiratory Failure Due to COVID-19 Recruiting NCT04510662 Phase 2 Telmisartan
42 Senicapoc in COVID-19 Patients With Severe Respiratory Insufficiency - A Randomized, Open-Label, Phase II Trial Recruiting NCT04594668 Phase 2 Senicapoc
43 Impact of Totally Transdermal Sedation in the Weaning From Remifentanil Infusion Among Critically Ill Patients Undergoing Mechanical Ventilation: a Pilot Randomized-controlled Study (The TOES Trial) Recruiting NCT04204967 Phase 2 Fentanyl Transdermal System;Remifentanil
44 Revefenacin in Acute Respiratory Insufficiency in COPD (RARICO) Recruiting NCT04315558 Phase 2 Revefenacin Inhalation Solution [Yupelri];Ipratropium Bromide
45 Pilot Randomized Clinical Trial of Inhaled PGE1 in Neonates With Sub-Optimal Response to Inhaled Nitric Oxide Terminated NCT01467076 Phase 2 Aerosolized Normal Saline;Inhaled PGE1
46 A Randomized, Double-Blind, Parallel Group Study to Assess the Efficacy and Safety of Once Weekly Subcutaneous Injections of Pemziviptadil (PB1046), a Sustained-Release VIP (Vasoactive Intestinal Peptide) ANalogue, in Hospitalized COVID-19 Patients at HiGh Risk for Rapid Clinical Deterioration and ARDS (PB1046 VANGARD Study) Terminated NCT04433546 Phase 2 Pemziviptadil (PB1046);Low Dose (10 mg) Control
47 Selecting PEEP in Ventilated Premature Infants by Reducing Ventilation/Perfusion Mismatch: a Pilot Feasibility Study Terminated NCT03109613 Phase 1, Phase 2
48 Nasal Continuous Positive Airway Pressure in Preterm Infants After Extubation: Comparison of Different Levels of Pressure Terminated NCT00636324 Phase 2
49 Randomized Clinical Trial of Inhaled PGE1 (IPGE1) in Neonatal Hypoxemic Respiratory Failure. A Protocol for the NICHD Neonatal Research Network Withdrawn NCT00598429 Phase 2 Inhaled Prostaglandin E1
50 Humidified High Flow Nasal Cannula Versus Nasal Intermittent Positive Ventilation in Neonates as Primary Respiratory Support:a Randomized Controlled Trial Unknown status NCT02499744 Phase 1

Search NIH Clinical Center for Pulmonary Valve Insufficiency

Cochrane evidence based reviews: pulmonary valve insufficiency

Sources

Genetic Tests for Pulmonary Valve Insufficiency

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Sources

Anatomical Context for Pulmonary Valve Insufficiency

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MalaCards organs/tissues related to Pulmonary Valve Insufficiency:

40
Heart, Lung, Liver, Brain, Spinal Cord, Ovary, Pituitary
Sources

Publications for Pulmonary Valve Insufficiency

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Articles related to Pulmonary Valve Insufficiency:

(show top 50) (show all 1415)
# Title Authors PMID Year
1
Plasma brain natriuretic peptide levels, right ventricular volume overload and exercise capacity in adolescents after surgical repair of tetralogy of Fallot. 54 61
17182138 2007
2
Effects of volume and/or pressure overload secondary to congenital heart disease (tetralogy of fallot or pulmonary stenosis) on right ventricular function using cardiovascular magnetic resonance and B-type natriuretic peptide levels. 54 61
16563914 2006
3
Pulmonary valve replacement via left anterior minithoracotomy: Lessons learned and early experience. 61
33533086 2021
4
Durability of the St. Jude Epic Supra Bioprosthetic Valve in the Pulmonary Position. 61
32505797 2021
5
Experience and Outcomes of Surgically Implanted Melody Valve in the Pulmonary Position. 61
32603706 2021
6
Unusual association of isolation of right pulmonary artery in absent pulmonary valve syndrome. 61
33789442 2021
7
Liver fibrosis in patients with tetralogy of Fallot, an unrecognised complication? 61
33719984 2021
8
Impact of operator experience and volume on outcomes after complete repair for tetralogy of Fallot. 61
33724399 2021
9
Staged Repair of Tetralogy of Fallot: A Strategy for Optimizing Clinical and Functional Outcomes. 61
33771498 2021
10
Determinants of QRS duration in patients with tetralogy of Fallot after pulmonary valve replacement. 61
33651426 2021
11
The reduced left ventricular stroke volume does not fully recover after pulmonary valve replacement in patients with repaired tetralogy of Fallot. 61
33739388 2021
12
Mid-Term Outcomes Following Percutaneous Pulmonary Valve Implantation Using the "Folded Melody Valve" Technique. 61
33726503 2021
13
Successful management of heart failure 45 years after surgical correction of tetralogy of Fallot. 61
33520028 2021
14
Short-term outcome of Polytetrafluoroethylene Membrane Valve versus Transannular Pericardial patch Reconstruction of Right Ventricular Outflow Tract in Tetralogy of Fallot: a Randomized Controlled Trial. 61
32759096 2021
15
Persistent end-diastolic forward flow after pulmonary valve replacement in patients with repaired tetralogy of Fallot. 61
33619521 2021
16
Impact of Annulus-Sparing on Surgical Adequacy of Pulmonary Valve in Complete Repair of Tetralogy of Fallot with Right Ventricular Outflow Tract Incision. 61
33156379 2021
17
Preventing Arrhythmic Death in Patients With Tetralogy of Fallot: JACC Review Topic of the Week. 61
33573746 2021
18
Reintervention rates after bioprosthetic pulmonary valve replacement in patients younger than 30 years of age: A multicenter analysis. 61
33069421 2021
19
Effect of In Utero Non-Steroidal Anti-Inflammatory Drug Therapy for Severe Ebstein Anomaly or Tricuspid Valve Dysplasia (NSAID Therapy for Fetal Ebstein anomaly). 61
33217351 2021
20
Pulmonary root translocation in corrected transposition of great arteries with ventricular septal defect and pulmonary stenosis with dextrocardia. 61
33331022 2021
21
Results of large pulmonary homograft implantation for right ventricular outflow tract reconstruction. 61
33319937 2021
22
Bilateral Percutaneous Pulmonary Valves for Severe Pulmonary Regurgitation in a Patient with Prior Valvotomy. 61
33644519 2021
23
Would increasing stress on the heart decrease stress on the doctor? Decision-making in asymptomatic pulmonary regurgitation. 61
33219110 2021
24
Is Pulmonary Hypertension a Rare Condition Associated to Angiostrongylosis in Naturally Infected Dogs? 61
33484890 2021
25
Assessing right ventricular systolic function using ultrasonic speckle-tracking imaging in repaired Tetralogy of Fallot with different pulmonary artery branch angles. 61
33594857 2021
26
Comparison of right ventricular outflow tract gradient under anesthesia with post-operative gradient in patients undergoing tetralogy of Fallot repair. 61
33679057 2021
27
Outcomes of pulmonary valve leaflet augmentation for transannular repair of tetralogy of Fallot. 61
33685734 2021
28
Congenital right ventricular diverticulum formed by pulmonary regurgitative jet flow in a fetus with tetralogy of Fallot and absent pulmonary valve. 61
33002584 2021
29
Spectrum of changes on cardiac magnetic resonance in repaired tetralogy of Fallot: Imaging according to surgical considerations. 61
32717537 2021
30
Outcomes of Right Ventricular Outflow Tract Reconstruction in Children: Retrospective Comparison Between Bovine Jugular Vein and Expanded Polytetrafluoroethylene Conduits. 61
32968822 2021
31
Risk factors for severe pulmonary regurgitation after repair of tetralogy of Fallot with transannular patch. 61
33185178 2020
32
Impact of Pregnancy on Ventricular Strain in Women with Repaired Tetralogy of Fallot. 61
32915291 2020
33
Severe Cardiopulmonary Disease in a Parturient With Noonan Syndrome. 61
32746732 2020
34
Asymmetric Regional Work Contributes to Right Ventricular Fibrosis, Inefficiency, and Dysfunction in Pulmonary Hypertension versus Regurgitation. 61
33383122 2020
35
Echocardiography and cardiac magnetic resonance in children with repaired tetralogy of Fallot: New insights in cardiac mechanics and exercise capacity. 61
32702408 2020
36
Pulmonary Valve Replacement via Left Thoracotomy as an Alternative to Resternotomy. 61
32454022 2020
37
Interplay between right atrial function and liver stiffness in adults with repaired right ventricular outflow obstructive lesions. 61
33367540 2020
38
Evolution of pulmonary valve reconstruction with focused review of expanded polytetrafluoroethylene handmade valves. 61
33377488 2020
39
Transcatheter Pulmonary Valve Replacement With the Sapien Prosthesis. 61
33303074 2020
40
Physiologic effects and functional outcome after treatment of dysfunctional right ventricular outflow tract in congenital heart disease using a two-stage intervention. 61
32593726 2020
41
Mid- to long-term follow-up of pulmonary valve replacement with BioIntegral injectable valve. 61
33210126 2020
42
Risk Factors for Mortality and Ventricular Tachycardia in Patients With Repaired Tetralogy of Fallot: A Systematic Review and Meta-analysis. 61
32416063 2020
43
The V̇e/V̇co2 slope: a useful tool to evaluate the physiological status of children with congenital heart disease. 61
32909919 2020
44
Assessment of Disease Progression in Patients With Repaired Tetralogy of Fallot Using Cardiac Magnetic Resonance Imaging: A Systematic Review. 61
32653300 2020
45
Early clinical experience with the straight design of Venus P-valveâ„¢ in dysfunctional right ventricular outflow tracts. 61
32096924 2020
46
Tibetan patients with hepatic hydatidosis can tolerate hypoxic environment without incident increase of pulmonary hypertension: an echocardiography study. 61
32767023 2020
47
Transcatheter Versus Surgical Pulmonary Valve Replacement: A Systemic Review and Meta-Analysis. 61
32268142 2020
48
Alterra Adaptive Prestent and SAPIEN 3 THV for Congenital Pulmonic Valve Dysfunction: An Early Feasibility Study. 61
33069657 2020
49
Prognostic markers in patients undergoing transcatheter implantation of Venus P-valve: Experience in Taiwan. 61
33158698 2020
50
Arduous redo made simple by "lateral approach"-pulmonary valve replacement via mini left thoracotomy. 61
32789946 2020
Sources

Variations for Pulmonary Valve Insufficiency

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ClinVar genetic disease variations for Pulmonary Valve Insufficiency:

6
# Gene Name Type Significance ClinVarId dbSNP ID Position
1 PMS2 NM_000535.7(PMS2):c.1927C>T (p.Gln643Ter) SNV Pathogenic 91320 rs63751422 GRCh37: 7:6026469-6026469
GRCh38: 7:5986838-5986838
2 ABCA3 NM_001089.3(ABCA3):c.875A>T (p.Glu292Val) SNV Likely pathogenic 203381 rs149989682 GRCh37: 16:2367764-2367764
GRCh38: 16:2317763-2317763
Sources

Expression for Pulmonary Valve Insufficiency

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Search GEO for disease gene expression data for Pulmonary Valve Insufficiency.
Sources

Pathways for Pulmonary Valve Insufficiency

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Pathways related to Pulmonary Valve Insufficiency according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Renin secretion 36
10.64 REN EDN1 ACE
2
Cardiomyocyte Differentiation through BMP Receptors 63
10.3 NPPB BMPR2
Sources

GO Terms for Pulmonary Valve Insufficiency

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Cellular components related to Pulmonary Valve Insufficiency according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.23 REN NPPB FBN1 EDN1 CRP BMPR2

Biological processes related to Pulmonary Valve Insufficiency according to GeneCards Suite gene sharing:

(show all 23)
# Name GO ID Score Top Affiliating Genes
1 kidney development GO:0001822 9.77 REN FBN1 ACE
2 negative regulation of cell growth GO:0030308 9.74 NPPB BMPR2 ACVRL1
3 regulation of blood vessel diameter GO:0097746 9.61 NPPB ACE
4 regulation of vasoconstriction GO:0019229 9.61 EDN1 ACE
5 activin receptor signaling pathway GO:0032924 9.59 BMPR2 ACVRL1
6 vasoconstriction GO:0042310 9.58 EDN1 CRP
7 transmembrane receptor protein serine/threonine kinase signaling pathway GO:0007178 9.58 BMPR2 ACVRL1
8 negative regulation of DNA biosynthetic process GO:2000279 9.56 BMPR2 ACVRL1
9 amyloid-beta metabolic process GO:0050435 9.55 REN ACE
10 positive regulation of renal sodium excretion GO:0035815 9.54 NPPB EDN1
11 positive regulation of urine volume GO:0035810 9.52 NPPB EDN1
12 negative regulation of systemic arterial blood pressure GO:0003085 9.51 NPPB BMPR2
13 cellular response to transforming growth factor beta stimulus GO:0071560 9.5 FBN1 EDN1 ACVRL1
14 angiotensin maturation GO:0002003 9.49 REN ACE
15 lymphangiogenesis GO:0001946 9.48 BMPR2 ACVRL1
16 artery development GO:0060840 9.46 BMPR2 ACVRL1
17 retina vasculature development in camera-type eye GO:0061298 9.43 BMPR2 ACVRL1
18 body fluid secretion GO:0007589 9.4 NPPB EDN1
19 lymphatic endothelial cell differentiation GO:0060836 9.37 BMPR2 ACVRL1
20 response to drug GO:0042493 9.35 REN PMS2 KCNK3 EDN1 ABCA3
21 blood vessel remodeling GO:0001974 9.33 BMPR2 ACVRL1 ACE
22 venous blood vessel development GO:0060841 9.26 BMPR2 ACVRL1
23 regulation of blood pressure GO:0008217 9.02 REN NPPB EDN1 ACVRL1 ACE

Molecular functions related to Pulmonary Valve Insufficiency according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 hormone activity GO:0005179 9.43 NPPB FBN1 EDN1
2 transmembrane receptor protein serine/threonine kinase activity GO:0004675 9.16 BMPR2 ACVRL1
3 transforming growth factor beta-activated receptor activity GO:0005024 8.96 BMPR2 ACVRL1
4 BMP receptor activity GO:0098821 8.62 BMPR2 ACVRL1
Sources

Sources for Pulmonary Valve Insufficiency

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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