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F1000Research 2019Tetralogy of Fallot (ToF) is the most common type of cyanotic congenital heart disease. Since the first surgical repair in 1954, treatment has continuously improved. The... (Review)
Review
Tetralogy of Fallot (ToF) is the most common type of cyanotic congenital heart disease. Since the first surgical repair in 1954, treatment has continuously improved. The treatment strategies currently used in the treatment of ToF result in excellent long-term survival (30 year survival ranges from 68.5% to 90.5%). However, residual problems such as right ventricular outflow tract obstruction, pulmonary regurgitation, and (ventricular) arrhythmia are common and often require re-interventions. Right ventricular dysfunction can be seen following longstanding pulmonary regurgitation and/or stenosis. Performing pulmonary valve replacement or relief of pulmonary stenosis before irreversible right ventricular dysfunction occurs is important, but determining the optimal timing of pulmonary valve replacement is challenging for several reasons. The biological mechanisms underlying dysfunction of the right ventricle as seen in longstanding pulmonary regurgitation are poorly understood. Different methods of assessing the right ventricle are used to predict impending dysfunction. The atrioventricular, ventriculo-arterial and interventricular interactions of the right ventricle play an important role in right ventricle performance, but are not fully elucidated. In this review we present a brief overview of the history of ToF, describe the treatment strategies currently used, and outline the long-term survival, residual lesions, and re-interventions following repair. We discuss important remaining challenges and present the current state of the art regarding these challenges.
Topics: Cardiac Surgical Procedures; Humans; Pulmonary Valve Insufficiency; Survival Rate; Tetralogy of Fallot; Ventricular Dysfunction, Right
PubMed: 31508203
DOI: 10.12688/f1000research.17174.1 -
Vascular Health and Risk Management 2023Pulmonary stenosis (PS) is mainly a congenital defect that accounts for 7-12% of congenital heart diseases (CHD). It can be isolated or, more frequently, associated with... (Review)
Review
Pulmonary stenosis (PS) is mainly a congenital defect that accounts for 7-12% of congenital heart diseases (CHD). It can be isolated or, more frequently, associated with other congenital defects (25-30%) involving anomalies of the pulmonary vascular tree. For the diagnosis of PS an integrated approach with echocardiography, cardiac computed tomography and cardiac magnetic resonance (CMR) is of paramount importance for the planning of the interventional treatment. In recent years, transcatheter approaches for the treatment of PS have increased however, meaning surgery is a possible option for complicated cases with anatomy not suitable for percutaneous treatment. The present review aims to summarize current knowledge regarding diagnosis and treatment of PS.
Topics: Humans; Pulmonary Valve Stenosis; Echocardiography; Magnetic Resonance Imaging
PubMed: 37416511
DOI: 10.2147/VHRM.S380240 -
Circulation Research Apr 2021Aortic stenosis is the most common valvular disease requiring valve replacement. Valve replacement therapies have undergone progressive evolution since the 1960s. Over... (Review)
Review
Aortic stenosis is the most common valvular disease requiring valve replacement. Valve replacement therapies have undergone progressive evolution since the 1960s. Over the last 20 years, transcatheter aortic valve replacement has radically transformed the care of aortic stenosis, such that it is now the treatment of choice for many, particularly elderly, patients. This review provides an overview of the pathophysiology, presentation, diagnosis, indications for intervention, and current therapeutic options for aortic stenosis.
Topics: Aged; Aged, 80 and over; Allografts; Aortic Valve Stenosis; Balloon Valvuloplasty; Bioprosthesis; Clinical Decision-Making; Heart Valve Prosthesis; Heart Valve Prosthesis Implantation; Humans; Middle Aged; Prosthesis Design; Prosthesis Failure; Pulmonary Valve; Symptom Assessment; Transcatheter Aortic Valve Replacement
PubMed: 33914604
DOI: 10.1161/CIRCRESAHA.121.318040 -
Echocardiography (Mount Kisco, N.Y.) Jan 2022
Topics: Biomarkers; Humans; Pulmonary Valve Stenosis
PubMed: 34913198
DOI: 10.1111/echo.15218 -
Journal of the American College of... Oct 2022
Topics: Humans; Hypertension, Pulmonary; Aortic Valve Stenosis; Transcatheter Aortic Valve Replacement; Aortic Valve; Treatment Outcome; Risk Factors; Heart Valve Prosthesis
PubMed: 36265956
DOI: 10.1016/j.jacc.2022.09.005 -
CJC Pediatric and Congenital Heart... Dec 2023Tetralogy of Fallot with pulmonary stenosis has a diverse clinical spectrum with the degree of right ventricular outflow tract obstruction (RVOTO) and size of the branch... (Review)
Review
Tetralogy of Fallot with pulmonary stenosis has a diverse clinical spectrum with the degree of right ventricular outflow tract obstruction (RVOTO) and size of the branch pulmonary arteries driving clinical management. Optimal surgical management involves consideration of patient clinical status and degree and location (subvalvar, valvar, and supravalvar) of RVOTO. Timing of repair requires multidisciplinary decision-making and complete surgical repair with relief of RVOTO by either transannular patch or valve sparing repair techniques. The central goals of contemporary surgical management of tetralogy of Fallot incorporate maximizing survival, minimizing reintervention, and preserving right ventricular function across the lifespan.
PubMed: 38161666
DOI: 10.1016/j.cjcpc.2023.09.003 -
Pediatric Cardiology Apr 2021Supravalvular pulmonary stenosis (SVPS) is considered a rare form of pulmonary stenosis (PS) and represents both a diagnostic and therapeutic challenge. There currently...
Supravalvular pulmonary stenosis (SVPS) is considered a rare form of pulmonary stenosis (PS) and represents both a diagnostic and therapeutic challenge. There currently exist no reliable echocardiographic criteria to accurately predict the supravalvular form. The aims of the study were to describe the response to treatment of the different PS presentations and to outline the diagnostic capacity of echocardiogram to differentiate the SVPS from valvular PS (VPS). This retrospective study included 106 patients who underwent percutaneous angioplasty between 2006 and 2017. Interventional outcomes of patients with SVPS were compared to those of patients with VPS. Diagnosis of VPS vs. SVPS by echocardiogram was compared to diagnosis obtained by angiogram. Echocardiogram yielded a sensitivity of 56%, a specificity of 82.5%, a positive predictive value of 50%, and a negative predictive value of 85.7%. Patients with SVPS had a significantly smaller pulmonary artery to pulmonary valve (PA:PV) ratio. At 6-12 months of follow-up, the VPS group had a mean right ventricular to pulmonary artery (RV-PA) gradient of 21.68 ± 19.85 mmHg compared to 45.27 ± 24.58 mmHg in the SVPS group. Patients with SVPS had a higher rate of reintervention than patients with VPS (32% vs. 6.2%, p < 0.001). There was no difference in major complications between groups, whereas VPS patients had a higher proportion of pulmonary insufficiency. Percutaneous angioplasty for PS is less effective in patients with a supravalvular component. A better understanding of the underlying histopathology of different PS subtypes could lead to development of different techniques to improve outcomes, with fewer reinterventions, in this population.
Topics: Angiography; Angioplasty; Angioplasty, Balloon; Child; Child, Preschool; Dilatation; Echocardiography; Female; Humans; Infant; Male; Predictive Value of Tests; Pulmonary Artery; Pulmonary Valve; Pulmonary Valve Insufficiency; Pulmonary Valve Stenosis; Retrospective Studies; Sensitivity and Specificity
PubMed: 33464372
DOI: 10.1007/s00246-021-02545-w -
Cardiology in the Young Dec 2022Noonan syndrome is a genetic disorder with high prevalence of congenital heart defects, such as pulmonary stenosis, atrial septal defect and hypertrophic cardiomyopathy....
BACKGROUND
Noonan syndrome is a genetic disorder with high prevalence of congenital heart defects, such as pulmonary stenosis, atrial septal defect and hypertrophic cardiomyopathy. Scarce data exists regarding the safety of pregnancy in patients with Noonan syndrome, particularly in the context of maternal cardiac disease.
STUDY DESIGN
We performed a retrospective chart review of patients at Yale-New Haven Hospital from 2012 to 2020 with diagnoses of Noonan syndrome and pregnancy. We analysed medical records for pregnancy details and cardiac health, including echocardiograms to quantify maternal cardiac dysfunction through measurements of pulmonary valve peak gradient, structural heart defects and interventricular septal thickness.
RESULTS
We identified five women with Noonan syndrome (10 pregnancies). Three of five patients had pulmonary valve stenosis at the time of pregnancy, two of which had undergone cardiac procedures. 50% of pregnancies (5/10) resulted in pre-term birth. 80% (8/10) of all deliveries were converted to caesarean section after a trial of labour. One pregnancy resulted in intra-uterine fetal demise while nine pregnancies resulted in the birth of a living infant. 60% (6/10) of livebirths required care in the neonatal intensive care unit. One infant passed away at 5 weeks of age.
CONCLUSIONS
The majority of mothers had pre-existing, though mild, heart disease. We found high rates of prematurity, conversion to caesarean section, and elevated level of care. No maternal complications resulted in long-term morbidity. Our study suggests that women with Noonan syndrome and low-risk cardiac lesions can become pregnant and deliver a healthy infant with counselling and risk evaluation.
Topics: Infant; Infant, Newborn; Humans; Pregnancy; Female; Pregnancy Outcome; Noonan Syndrome; Cesarean Section; Retrospective Studies; Heart Defects, Congenital; Pulmonary Valve Stenosis
PubMed: 35034678
DOI: 10.1017/S104795112100514X