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Current Cardiology Reviews 2023Quadricuspid pulmonary valve (QPV) is a rare congenital anomaly. Simple QPV had been mainly diagnosed at the time of autopsy before 2000, and the frequency rates of QPV...
BACKGROUND
Quadricuspid pulmonary valve (QPV) is a rare congenital anomaly. Simple QPV had been mainly diagnosed at the time of autopsy before 2000, and the frequency rates of QPV are approximately 0.02%-0.41%. QPV was initially diagnosed using transthoracic echocardiography (TTE) after 2000 and with contrast computed tomography (CT) or cardiac magnetic resonance imaging (CMR) after 2009. Obtaining the cross-sectional view of the pulmonary valve using TTE is difficult. We aimed to review the papers regarding the incidence, embryology, diagnosis, associated congenital heart anomalies, and prognosis in patients with QPV, and furthermore to compare with those in patients with quadricuspid aortic valve (QAV).
CASE PRESENTATION
We diagnosed QPV with mild stenosis in a 12-month-old infant. With a slight angulation of the transducer superiorly from the left high parasternal short-axis view, a short-axis view of QPV was obtained.
RESULTS
In QPV cases diagnosed at autopsy, Hurwitz's type-b with three equal cusps and one smaller cusp is dominant, whereas Hurwitz's type-a with four equal cusps is dominant in clinically diagnosed cases. Congenital heart anomaly and valvular stenosis are more frequent in patients with QPV than in patients with QAV. Coronary artery anomalies and infectious endocarditis are more frequent in patients with QAV than in patients with QPV. The incidence of PR is more common in type-a QPV than in type-b QPV. There is no difference between type-a QAV and type-b QAV with respect to the incidence of aortic regurgitation (AR). It is assumed that QPV is a risk factor for a Ross operation. However, QPVs have been used as autografts in certain patients.
CONCLUSION
Between QPV and QAV, various differences were found in frequency rates, diagnostic methods, valve morphology, valve function, associated congenital heart diseases, and frequencies of infectious endocarditis.
Topics: Infant; Humans; Aortic Valve; Quadricuspid Aortic Valve; Pulmonary Valve; Constriction, Pathologic; Cross-Sectional Studies; Heart Defects, Congenital; Endocarditis
PubMed: 35319379
DOI: 10.2174/1573403X18666220322092706 -
Ultrasound in Obstetrics & Gynecology :... Feb 2013To review the anomaly spectrum of prenatally detected absent pulmonary valve syndrome (APVS) and the outcome after diagnosis. Previous fetal studies reported survival... (Review)
Review
OBJECTIVE
To review the anomaly spectrum of prenatally detected absent pulmonary valve syndrome (APVS) and the outcome after diagnosis. Previous fetal studies reported survival rates of ≤ 25% for patients with intended postnatal care.
METHODS
Clinical data and echocardiograms of 12 cases with a fetal diagnosis of APVS between 2000 and 2010 were analyzed in this retrospective single-center study. Collected parameters included: gestational age at referral, associated fetal abnormalities, cardiothoracic ratio, maximum diameters of pulmonary annulus and main and branch pulmonary arteries, ventricular dimensions and function as well as ventricular Doppler flows. Karyotyping included fluorescence in-situ hybridization (FISH) analysis for microdeletion 22q11.2.
RESULTS
Median gestational age at diagnosis was 24 weeks. Three subtypes of APVS were observed: (1) with tetralogy of Fallot (TOF) and no arterial duct (n = 10; 83%); (2) isolated, with a large arterial duct (n = 1; 8%); and (3) with tricuspid atresia, right ventricular dysplasia and a restricted duct (n = 1; 8%). The cardiothoracic ratio and pulmonary artery dimensions were increased in all cases. The karyotype was abnormal in 70% of fetuses with TOF and their mortality rate was significantly higher due to pregnancy termination (n = 3) or perinatal demise (n = 2) (hazard ratio, 5; 95% CI, 0.87-28.9; P = 0.015). Of seven live births with active postnatal care, six children (86%) were alive without residual respiratory symptoms at a median follow-up of 4.7 (range, 2.1-10.6) years.
CONCLUSION
Outcome after fetal diagnosis of APVS was significantly better in this study compared with those of previous fetal series, with a low mortality rate for actively managed patients.
Topics: 22q11 Deletion Syndrome; Abortion, Induced; Echocardiography; Female; Fetus; Gestational Age; Humans; Pregnancy; Pregnancy Outcome; Pulmonary Valve; Tetralogy of Fallot; Ultrasonography, Prenatal
PubMed: 22605612
DOI: 10.1002/uog.11193 -
Scientific Reports Jul 2021Murine animal models are an established standard in translational research and provides a potential platform for studying heart valve disease. To date, studies on heart...
Murine animal models are an established standard in translational research and provides a potential platform for studying heart valve disease. To date, studies on heart valve disease using murine models have been hindered by a lack of appropriate methodologies due to their small scale. In the present study, we developed a multi-scale, imaging-based approach to extract the functional structure and geometry for the murine heart valve. We chose the pulmonary valve (PV) to study, due to its importance in congenital heart valve disease. Excised pulmonary outflow tracts from eleven 1-year old C57BL/6J mice were fixed at 10, 20, and 30 mmHg to simulate physiological loading. Micro-computed tomography was used to reconstruct the 3D organ-level PV geometry, which was then spatially correlated with serial en-face scanning electron microscopy imaging to quantify local collagen fiber distributions. From the acquired volume renderings, we obtained the geometric descriptors of the murine PV under increasing transvalvular pressures, which demonstrated remarkable consistency. Results to date suggest that the preferred collagen orientation was predominantly in the circumferential direction, as in larger mammalian valves. The present study represents a first step in establishing organ-level murine models for the study of heart valve disease.
Topics: Algorithms; Animals; Biomechanical Phenomena; Collagen; Heart Valve Diseases; Image Processing, Computer-Assisted; Mice; Models, Theoretical; Pulmonary Valve; Translational Research, Biomedical; X-Ray Microtomography
PubMed: 34234231
DOI: 10.1038/s41598-021-93513-0 -
The Journal of Thoracic and... Feb 2021
Topics: Animals; Heart Valve Prosthesis; Humans; Pericardium; Pulmonary Valve; Swine
PubMed: 32921441
DOI: 10.1016/j.jtcvs.2020.08.036 -
Open Heart Apr 2023Cardiac surgery may cause temporarily impaired ventricular performance and myocardial injury. We aim to characterise the response to perioperative injury for patients... (Observational Study)
Observational Study
OBJECTIVE
Cardiac surgery may cause temporarily impaired ventricular performance and myocardial injury. We aim to characterise the response to perioperative injury for patients undergoing repair or pulmonary valve replacement (PVR) for tetralogy of Fallot (ToF).
METHODS
We enrolled children undergoing ToF repair or PVR from four tertiary centres in a prospective observational study. Assessment-including blood sampling and speckle tracking echocardiography-occurred before surgery (T1), at the first follow-up (T2) and 1 year after the procedures (T3). Ninety-two serum biomarkers were expressed as principal components to reduce multiple statistical testing. RNA Sequencing was performed on right ventricular (RV) outflow tract samples.
RESULTS
We included 45 patients with ToF repair aged 4.3 (3.4 - 6.5) months and 16 patients with PVR aged 10.4 (7.8 - 12.7) years. Ventricular function following ToF repair showed a fall-and-rise pattern for left ventricular global longitudinal strain (GLS) (-18±4 to -13±4 to -20±2, p < 0.001 for each comparison) and RV GLS (-19±5 to -14±4 to 20±4, p < 0.002 for each comparison). This pattern was not seen for patients undergoing PVR. Serum biomarkers were expressed as three principal components. These phenotypes are related to: (1) surgery type, (2) uncorrected ToF and (3) early postoperative status. Principal component 3 scores were increased at T2. This increase was higher for ToF repair than PVR. The transcriptomes of RV outflow tract tissue are related to patients' sex, rather than ToF-related phenotypes in a subset of the study population.
CONCLUSIONS
The response to perioperative injury following ToF repair and PVR is characterised by specific functional and immunological responses. However, we did not identify factors relating to (dis)advantageous recovery from perioperative injury.
TRIAL REGISTRATION NUMBER
Netherlands Trial Register: NL5129.
Topics: Humans; Tetralogy of Fallot; Pulmonary Valve; Pulmonary Valve Insufficiency; Heart Valve Prosthesis Implantation; Ventricular Function, Right; Cardiac Surgical Procedures; Ventricular Function; Biomarkers
PubMed: 37024245
DOI: 10.1136/openhrt-2022-002238 -
Tissue Engineering. Part A Nov 2014Currently available replacement heart valves all have limitations. This study aimed to produce and characterize an acellular, biocompatible porcine pulmonary root...
Currently available replacement heart valves all have limitations. This study aimed to produce and characterize an acellular, biocompatible porcine pulmonary root conduit for reconstruction of the right ventricular outflow tract e.g., during Ross procedure. A process for the decellularization of porcine pulmonary roots was developed incorporating trypsin treatment of the adventitial surface of the scraped pulmonary artery and sequential treatment with hypotonic Tris buffer (HTB; 10 mM Tris pH 8.0, 0.1% (w/v) EDTA, and 10 KIU aprotinin), 0.1% (w/v) sodium dodecyl sulfate in HTB, two cycles of DNase and RNase, and sterilization with 0.1% (v/v) peracetic acid. Histology confirmed an absence of cells and retention of the gross histoarchitecture. Immunohistochemistry further confirmed cell removal and partial retention of the extracellular matrix, but a loss of collagen type IV. DNA levels were reduced by more than 96% throughout all regions of the acellular tissue and no functional genes were detected using polymerase chain reaction. Total collagen levels were retained but there was a significant loss of glycosaminoglycans following decellularization. The biomechanical, hydrodynamic, and leaflet kinematics properties were minimally affected by the process. Both immunohistochemical labeling and antibody absorption assay confirmed a lack of α-gal epitopes in the acellular porcine pulmonary roots and in vitro biocompatibility studies indicated that acellular leaflets and pulmonary arteries were not cytotoxic. Overall the acellular porcine pulmonary roots have excellent potential for development of a tissue substitute for right ventricular outflow tract reconstruction e.g., during the Ross procedure.
Topics: Animals; Bioprosthesis; Cell-Free System; Equipment Failure Analysis; Heart Valve Prosthesis; Prosthesis Design; Pulmonary Valve; Swine; Tissue Engineering; Tissue Scaffolds
PubMed: 24786313
DOI: 10.1089/ten.TEA.2013.0573 -
Journal of the American College of... Mar 2020
Topics: Adult; Cardiac Surgical Procedures; Heart Defects, Congenital; Humans; Pulmonary Valve; Tricuspid Valve
PubMed: 32138964
DOI: 10.1016/j.jacc.2020.01.006 -
Journal of Cardiothoracic Surgery Jan 2021Isolated pulmonary valve endocarditis (IPE) is rare, accounting for 1.5-2% of all cases of infective endocarditis. Herein, we describe a case of isolated pulmonary valve... (Review)
Review
BACKGROUND
Isolated pulmonary valve endocarditis (IPE) is rare, accounting for 1.5-2% of all cases of infective endocarditis. Herein, we describe a case of isolated pulmonary valve endocarditis with rapid progression in a 28-year-old male. Unlike most patients reported previously who were cured with only anti-infective therapy, without surgery at an early stage, multiple complications occurred in this patient in less than 2 weeks.
CASE PRESENTATION
The patient was diagnosed with pulmonary valve endocarditis with blood cultures showing Staphylococcus aureus and echocardiography revealing 2 masses (measuring 14*13 mm、11*16 mm in size). Only 12 days later, acute massive pulmonary embolism occurred. Then, repeated echocardiography revealed multiple masses attached to the pulmonary valve with severe pulmonary insufficiency and the possibility of pulmonary valve destruction. Finally, pulmonary valve replacement, vegetation removal, and right pulmonary thromboendarterectomy together with resection of the middle and lower lobes of the right lung were performed.
CONCLUSIONS
The role of surgery at an early stage might need to be reconsidered, and it may be viable to combine medical and surgical approaches.
Topics: Adult; Cardiac Surgical Procedures; Disease Progression; Echocardiography; Endocarditis, Bacterial; Humans; Male; Pulmonary Valve; Staphylococcal Infections; Staphylococcus aureus
PubMed: 33509229
DOI: 10.1186/s13019-020-01375-w -
The Journal of Thoracic and... Jan 2019
Topics: Aortic Valve; Autografts; Pulmonary Valve
PubMed: 30100491
DOI: 10.1016/j.jtcvs.2018.06.051 -
JACC. Cardiovascular Interventions May 2022
Topics: Cardiac Catheterization; Coronary Vessels; Heart Valve Prosthesis; Heart Valve Prosthesis Implantation; Humans; Pulmonary Valve; Pulmonary Valve Insufficiency; Treatment Outcome
PubMed: 35512923
DOI: 10.1016/j.jcin.2022.03.031