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Journal of the American College of... Mar 2020Tricuspid regurgitation (TR) is common among adults with corrected tetralogy of Fallot (TOF) or pulmonary stenosis (PS) referred for pulmonary valve replacement (PVR)....
BACKGROUND
Tricuspid regurgitation (TR) is common among adults with corrected tetralogy of Fallot (TOF) or pulmonary stenosis (PS) referred for pulmonary valve replacement (PVR). Yet, combined valve surgery remains controversial.
OBJECTIVES
This study sought to evaluate the impact of concomitant tricuspid valve intervention (TVI) on post-operative TR, length of hospital stay, and on a composite endpoint consisting of 7 early adverse events (death, reintervention, cardiac electronic device implantation, infection, thromboembolic event, hemodialysis, and readmission).
METHODS
The national Canadian cohort enrolled 542 patients with TOF or PS and mild to severe TR who underwent isolated PVR (66.8%) or PVR+TVI (33.2%). Outcomes were abstracted from charts and compared between groups using multivariable logistic and negative binomial regression.
RESULTS
Median age at reintervention was 35.3 years. Regardless of surgery type, TR decreased by at least 1 echocardiographic grade in 35.4%, 66.9%, and 92.8% of patients with pre-operative mild, moderate, and severe insufficiency. In multivariable analyses, PVR+TVI was associated with an additional 2.3-fold reduction in TR grade (odds ratio [OR]: 0.44; 95% confidence interval [CI]: 0.25 to 0.77) without an increase in early adverse events (OR: 0.85; 95% CI: 0.46 to 1.57) or hospitalization time (incidence rate ratio: 1.17; 95% CI: 0.93 to 1.46). Pre-operative TR severity and presence of transvalvular leads independently predicted post-operative TR. In contrast, early adverse events were strongly associated with atrial tachyarrhythmia, extracardiac arteriopathy, and a high body mass index.
CONCLUSIONS
In patients with TOF or PS and significant TR, concomitant TVI is safe and results in better early tricuspid valve competence than isolated PVR.
Topics: Adult; Canada; Child; Child, Preschool; Cohort Studies; Female; Heart Defects, Congenital; Heart Valve Prosthesis Implantation; Humans; Infant; Length of Stay; Male; Postoperative Complications; Pulmonary Valve Stenosis; Tricuspid Valve Insufficiency
PubMed: 32138963
DOI: 10.1016/j.jacc.2019.12.053 -
Acta Veterinaria Scandinavica Jul 2017Congenital pulmonary valve stenosis and patent ductus arteriosus are common congenital heart defects in dogs. However, concurrence of atypical pulmonary valve stenosis...
BACKGROUND
Congenital pulmonary valve stenosis and patent ductus arteriosus are common congenital heart defects in dogs. However, concurrence of atypical pulmonary valve stenosis and patent ductus arteriosus is uncommon. This report describes the anatomic, histopathologic, and echocardiographic features in a dog with concomitant pulmonary valve stenosis and patent ductus arteriosus with atypical pulmonary valve dysplasia that included a fibrous band of tissue.
CASE PRESENTATION
A 1.5-year-old intact female Chihuahua dog weighing 3.3 kg presented with a continuous grade VI cardiac murmur, poor exercise tolerance, and an intermittent cough. Echocardiography indicated pulmonary valve stenosis, a thickened dysplastic valve without annular hypoplasia, and a type IIA patent ductus arteriosus. The pulmonary valve was thick line-shaped in systole and dome-shaped towards the right ventricular outflow tract in diastole. The dog suffered a fatal cardiac arrest during an attempted balloon pulmonary valvuloplasty. Necropsy revealed pulmonary valve dysplasia, commissural fusion, and incomplete opening and closing of the pulmonary valve because of a fibrous band of tissue causing adhesion between the right ventricular outflow tract and the dysplastic intermediate cusp of the valve.
CONCLUSIONS
A fibrous band of tissue between the right ventricular outflow track and the pulmonary valve should be considered as a cause of pulmonary valve stenosis. Pulmonary valve stenosis and patent ductus arteriosus can have conflicting effects on diastolic and systolic dysfunction, respectively. Therefore, beta-blockers should always be used carefully, particularly in patients with a heart defect where there is concern about left ventricular systolic function.
Topics: Animals; Diagnosis, Differential; Dogs; Ductus Arteriosus, Patent; Echocardiography; Female; Heart Defects, Congenital; Pulmonary Valve Stenosis
PubMed: 28697788
DOI: 10.1186/s13028-017-0314-z -
Journal of Veterinary Internal Medicine 2009Boxers are predisposed to subaortic and pulmonic stenosis (SAS, PS). Screening of puppies may be useful in estimating the risk of their developing a defect that...
BACKGROUND
Boxers are predisposed to subaortic and pulmonic stenosis (SAS, PS). Screening of puppies may be useful in estimating the risk of their developing a defect that potentially compromises life expectancy or exercise tolerance.
HYPOTHESIS
Presence of SAS or PS in adult Boxers can be predicted by auscultation and Doppler echocardiography at 9-10 weeks of age.
ANIMALS
Eighty-five Boxer puppies examined at 9-10 weeks of age and at 12 months of age.
METHODS
Prospective, longitudinal observational study. Auscultation by stethoscope and continuous wave-Doppler echocardiography for peak velocities (V(max)) in the aorta (Ao) and pulmonary artery (PA).
RESULTS
Intensity of heart murmurs in puppies correlated with V(max)Ao and V(max)PA in adults. V(max)Ao and V(max)PA in puppies correlated with V(max)Ao and V(max)PA in adults, respectively. From puppy to adult, V(max)Ao increased and V(max)PA remained unchanged. The negative predictive value for absent or only a soft (< or =II/VI) murmur in puppies being associated with V(max)Ao and PA < or = 2.4 m/s as an adult was 90% and < or =3.5 m/s 100%. The negative predictive value of a V(max)< or = 2.4 m/s as a puppy still being < or =2.4 m/s as an adult was 94% for Ao and 96% for PA, and of a V(max)< or = 3.5 m/s, 99% for Ao and 100% for PA.
CONCLUSIONS AND CLINICAL IMPORTANCE
Even though V(max)Ao increases during growth in Boxer puppies, indicating relative narrowing of the aorta, puppies with V(max)Ao < or = 2.4 m/s do not usually progress to clinically have relevant SAS at 12 months of age.
Topics: Animals; Aortic Valve Stenosis; Dog Diseases; Dogs; Echocardiography, Doppler; Genetic Predisposition to Disease; Heart Auscultation; Heart Murmurs; Predictive Value of Tests; Pulmonary Valve Stenosis
PubMed: 19175725
DOI: 10.1111/j.1939-1676.2008.0213.x -
Circulation. Genomic and Precision... Aug 2022Valvar pulmonary stenosis (vPS) accounts for 8% to 12% of congenital heart disease cases. Multiple genetic syndromes are associated with vPS, most commonly Noonan...
BACKGROUND
Valvar pulmonary stenosis (vPS) accounts for 8% to 12% of congenital heart disease cases. Multiple genetic syndromes are associated with vPS, most commonly Noonan syndrome, but the cause is unknown in most cases. We analyzed genomic data from a large cohort with vPS to determine the prevalence of genetic diagnosis.
METHODS
The Pediatric Cardiac Genomics Consortium database was queried to identify probands with vPS without complex congenital heart disease or aneuploidy and with existing whole exome or genome sequencing. A custom analysis workflow was used to identify likely pathogenic or pathogenic variants in disease-associated genes. Demographic and phenotypic characteristics were compared between groups with and without molecular diagnoses.
RESULTS
Data from 119 probands (105 trios) were included. A molecular diagnosis was identified in 22 (18%); 17 (14%) had Noonan syndrome or a related disorder. Extracardiac and neurodevelopmental comorbidities were seen in 67/119 (56%) of probands. Molecular diagnosis was more common in those with extracardiac and neurodevelopmental phenotypes than those without (18/67 versus 4/52, =0.0086).
CONCLUSIONS
Clinicians should have high suspicion for a genetic diagnosis in individuals with vPS, particularly if additional phenotypes are present. Our results suggest that clinicians should consider offering sequencing of at least the known congenital heart disease and RASopathy genes to all individuals with vPS, regardless of whether that individual has extracardiac or neurodevelopmental phenotypes present.
Topics: Exome; Heart Defects, Congenital; Humans; Noonan Syndrome; Prevalence; Pulmonary Valve Stenosis
PubMed: 35666834
DOI: 10.1161/CIRCGEN.121.003635 -
Journal of Veterinary Internal Medicine 2023Cardiovascular diseases with increased right ventricular (RV) afterload induce RV diastolic and systolic dysfunction, and myocardial fibrosis in humans. Studies in dogs...
BACKGROUND
Cardiovascular diseases with increased right ventricular (RV) afterload induce RV diastolic and systolic dysfunction, and myocardial fibrosis in humans. Studies in dogs with pulmonary stenosis (PS) evaluating RV diastolic function and markers of myocardial fibrosis are lacking.
HYPOTHESIS/OBJECTIVES
Dogs with PS have echocardiographic evidence of RV diastolic and systolic dysfunction and increased serum concentrations of galectin-3 (Gal-3), a surrogate biomarker for myocardial fibrosis.
ANIMALS
Forty client-owned dogs (10 controls, 30 with PS).
METHODS
Prospective study. All dogs had systemic blood pressure measurement, serum biochemical analysis, echocardiography, and measurement of serum Gal-3 concentration performed.
RESULTS
Variables of RV diastolic function were obtained in 39/40 dogs. Trans-tricuspid flow velocity in early diastole to trans-tricuspid flow velocity in late diastole ratios (RV E/A) were lower (P < .001) in dogs with PS (median, 0.94; range, 0.62-2.04) compared to controls (1.78; 1.17-2.35). Trans-tricuspid flow velocity in early diastole to tricuspid annular myocardial velocity in early diastole ratios (RV E/e') were higher (P < .001) in dogs with PS (11.55; 4.69-28) compared to control (6.21; 5.16-7.21). Variables of RV systolic function were lower in dogs with PS (P = <.001). Serum Gal-3 concentration was higher (P = .002) in dogs with PS (285.1 pg/mL; 94.71-406.97) compared to control dogs (162.83 pg/mL; 52.3-232.82).
CONCLUSIONS AND CLINICAL IMPORTANCE
Dogs with PS have RV diastolic and systolic dysfunction, and increased Gal-3 concentrations. These findings suggest the presence of RV myocardial fibrosis in dogs with PS, which could impact clinical management.
Topics: Humans; Dogs; Animals; Galectin 3; Diastole; Prospective Studies; Pulmonary Valve Stenosis; Fibrosis; Dog Diseases
PubMed: 37767953
DOI: 10.1111/jvim.16890 -
Thorax Jul 1967Three patients suffering from acquired pulmonary stenosis are described resulting from compression of the main pulmonary artery by anterior mediastinal teratoma,...
Three patients suffering from acquired pulmonary stenosis are described resulting from compression of the main pulmonary artery by anterior mediastinal teratoma, Hodgkin's disease, and aneurysm of the ascending aorta, respectively; also four others who had compression of the right or left primary branch of the pulmonary artery. The pulmonary artery compression was demonstrated angiographically in every case, and in the patient with right and left pulmonary artery compression the main pulmonary artery pressure tracing was characteristic of bilateral pulmonary artery stenosis. Cardiac catheterization and selective angiography are indicated in patients who have evidence of right ventricular outflow obstruction to determine the site and severity of the obstruction and to differentiate between extrinsic compression and intrinsic stenosis.
Topics: Adolescent; Adult; Aortic Aneurysm; Cardiac Catheterization; Female; Hodgkin Disease; Humans; Male; Mediastinal Neoplasms; Middle Aged; Pulmonary Artery; Pulmonary Valve Stenosis; Radiography; Teratoma
PubMed: 6035800
DOI: 10.1136/thx.22.4.358 -
Croatian Medical Journal Sep 2000This report describes 3 cases of pulmonary stenosis in the recipient twin in twin-twin transfusion syndrome. Fetal echocardiography showed cardiomegaly, tricuspid valve... (Review)
Review
This report describes 3 cases of pulmonary stenosis in the recipient twin in twin-twin transfusion syndrome. Fetal echocardiography showed cardiomegaly, tricuspid valve regurgitation, and increased reverse flow in the inferior vena cava, as signs of congestive heart failure in all 3 cases. We diagnosed 2 cases of pulmonary stenosis by fetal echocardiography prenatally and confirmed our findings in all 3 cases postnatally. Two cases underwent postnatal balloon valvuloplasty to release the pulmonary valvular stenosis in neonatal period. The third one died soon after delivery and autopsy showed a slightly thickened pulmonary valve. One of the cases was diagnosed in the early second trimester (20 weeks of pregnancy), the earliest detection of fetal pulmonary stenosis reported in literature. The presence of high peak velocity of the pulmonary artery at 20 weeks of pregnancy preceded the development of pulmonary stenosis in this case. This supports the hypothesis that alterations in fetal hemodynamics may result in structural cardiac abnormality.
Topics: Adult; Blood Flow Velocity; Female; Fetofetal Transfusion; Humans; Pregnancy; Pregnancy Outcome; Pulmonary Valve Stenosis; Ultrasonography, Prenatal
PubMed: 10962042
DOI: No ID Found -
Ultrasound in Obstetrics & Gynecology :... Mar 2014Fetuses with pulmonary outflow tract obstruction (POTO) have altered blood flow to the pulmonary vasculature. We sought to determine whether pulmonary vascular... (Comparative Study)
Comparative Study
OBJECTIVES
Fetuses with pulmonary outflow tract obstruction (POTO) have altered blood flow to the pulmonary vasculature. We sought to determine whether pulmonary vascular impedance, as assessed by the pulsatility index (PI), is different in fetuses with POTO compared with normal controls.
METHODS
Branch pulmonary artery PI was evaluated in age-matched normal control fetuses (n=22) and 20 POTO fetuses (pulmonary stenosis n=15, pulmonary atresia n=5). Pulsed-wave Doppler was performed in the proximal (PA1), mid (PA2) and distal (PA3) branch pulmonary artery. The direction of flow in the ductus arteriosus was noted. The study and control groups were compared with Student's t-test and ANOVA. A linear mixed model evaluated the relationship between PI and ductus arteriosus flow patterns.
RESULTS
There was no difference in PI between control, pulmonary stenosis and pulmonary atresia subjects at PA1 and PA2; however, there was a significant difference at PA3. Subjects with pulmonary atresia had a lower PI at PA3 than did controls (P=0.003) and pulmonary stenosis subjects (P=0.003). Subjects with retrograde flow in the ductus arteriosus had lower PIs in PA2 and PA3 than did those with antegrade flow (P=0.01 and 0.005, respectively). The PI in PA3 was lower in fetuses that required prostaglandin postnatally than in those that did not (P=0.008).
CONCLUSIONS
Fetuses with pulmonary atresia or severe pulmonary stenosis with retrograde flow in the ductus arteriosus have decreased PI in the distal pulmonary vasculature. Our findings indicate the capacity of the fetal pulmonary vasculature to vasodilate in response to anatomical obstruction of flow.
Topics: Alprostadil; Blood Flow Velocity; Ductus Arteriosus; Echocardiography, Doppler, Pulsed; Female; Gestational Age; Humans; Infant, Newborn; Male; Pregnancy; Pulmonary Artery; Pulmonary Atresia; Pulmonary Valve Stenosis; Retrospective Studies; Sensitivity and Specificity; Ultrasonography, Prenatal; Vasodilator Agents; Ventricular Outflow Obstruction
PubMed: 23554091
DOI: 10.1002/uog.12472 -
Journal of the American College of... Apr 2008The purpose of this study was to determine the morphologic and physiological predictors of post-natal surgical pathway in a longitudinal series of fetuses with pulmonary...
OBJECTIVES
The purpose of this study was to determine the morphologic and physiological predictors of post-natal surgical pathway in a longitudinal series of fetuses with pulmonary atresia with intact ventricular septum (PAIVS) and/or critical pulmonary stenosis with reversal of ductal flow (CPS) using statistical modeling.
BACKGROUND
Pulmonary atresia with intact ventricular septum is rarely associated with chromosomal or extra cardiac malformations, so decisions about continuing a pregnancy are strongly influenced by the prediction of univentricular (UV) or biventricular (BV) circulation.
METHODS
Predictive scores were derived, using a combination of z-scores of fetal cardiac measurements (for femoral length) and tricuspid/mitral valve (TV/MV) ratios, to facilitate early prediction of UV or BV circulation in 21 fetuses with PAIVS (18 fetuses) or CPS (3 fetuses) between 1998 and 2004. We also assessed the predictive value of coronary fistulae and right atrial pressure (RAP) score (comprising the tricuspid valve, foramen ovale, and ductus venosus Doppler).
RESULTS
One-half of the cohort was first assessed before 23 gestational weeks (range 15.7 to 33.7 weeks). The TV z-score was a good predictor at all gestations, but the best predictive scores for specific gestations were pulmonary valve (PV) z-score (<23 weeks), median TV z-score (<26 weeks), the combination of median PV z-score and the median TV/MV ratio (26 to 31 weeks), and the combination of median TV z-score and median TV/MV ratio (>31 weeks). The RAP score and coronary fistulae were good independent predictors: RAP score >3 predicted BV with area under the curve of 0.833, and detection of fistulae usually predicted a UV route.
CONCLUSIONS
The best predictive scores for post-natal outcome in fetal PAIVS/CPS are a combination of morphologic and physiological variables, which predict a BV circulation with a sensitivity of 92% and specificity of 100% before 26 weeks.
Topics: Area Under Curve; Female; Fetal Diseases; Health Status Indicators; Heart Septum; Heart Ventricles; Humans; Infant, Newborn; Male; Models, Statistical; Postnatal Care; Predictive Value of Tests; Pregnancy; Prospective Studies; Pulmonary Atresia; Pulmonary Valve; Pulmonary Valve Stenosis; Time Factors; Ultrasonography, Prenatal
PubMed: 18371563
DOI: 10.1016/j.jacc.2007.08.073 -
JACC. Cardiovascular Interventions Oct 2018The aim of this study was to describe and analyze data from patients treated in France with the Edwards SAPIEN transcatheter heart valve (Edwards Lifesciences LLC,... (Observational Study)
Observational Study
OBJECTIVES
The aim of this study was to describe and analyze data from patients treated in France with the Edwards SAPIEN transcatheter heart valve (Edwards Lifesciences LLC, Irvine, California) in the pulmonary position.
BACKGROUND
The Edwards SAPIEN valve has recently been introduced for percutaneous pulmonary valve implantation (PPVI).
METHODS
From April 2011 to May 2017, 71 patients undergoing PPVI were consecutively included.
RESULTS
The median age at PPVI was 26.8 years (range 12.8 to 70.1 years). Primary underlying diagnoses were conotruncal malformations (common arterial trunk, tetralogy of Fallot and variants; n = 45), Ross procedure (n = 18), and other diagnoses (n = 8). PPVI indication was pure stenosis in 33.8% of patients, pure regurgitation in 28.1%, and mixed lesions in 38.1%. PPVI was successfully implemented in 68 patients (95.8%). Pre-stenting of the right ventricular outflow tract was performed in 70 patients (98.6%). Early major complications occurred in 4 subjects (5.6%), including 1 death, 1 coronary compression, and 2 pulmonary valve embolizations. Three of the 4 major complications occurred in the first 15 operated patients. No significant regurgitation was recorded after the procedure. Transpulmonary gradient was significantly reduced from 34.5 to 10.5 mm Hg (p < 0.0001). No patient died during a 1-month follow-up period. At 1-year follow-up, the death rate was 2.9%, and 3 patients had undergone surgical reintervention (44%).
CONCLUSIONS
Early results with the Edwards SAPIEN valve in the pulmonary position demonstrate an ongoing high rate of procedural success.
Topics: Adolescent; Adult; Aged; Cardiac Catheterization; Child; Female; France; Heart Valve Prosthesis; Heart Valve Prosthesis Implantation; Humans; Male; Middle Aged; Postoperative Complications; Prospective Studies; Prosthesis Design; Pulmonary Valve; Pulmonary Valve Insufficiency; Pulmonary Valve Stenosis; Recovery of Function; Registries; Risk Assessment; Risk Factors; Time Factors; Treatment Outcome; Young Adult
PubMed: 30219326
DOI: 10.1016/j.jcin.2018.05.050