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Indian Heart Journal 2024There is conclusive evidence for relief of pulmonary valve obstruction immediately after balloon pulmonary valvuloplasty (BPV) and at follow-up. Development of... (Review)
Review
There is conclusive evidence for relief of pulmonary valve obstruction immediately after balloon pulmonary valvuloplasty (BPV) and at follow-up. Development of infundibular obstruction is seen in more severe PS cases and in older subjects. Reappearance of PS was observed in approximately 10 % of patients following BPV. The reasons for recurrence were found to be balloon/annulus ratio less than 1.2 and immediate post-BPV pulmonary valve peak gradients greater than 30 mmHg. Recurrent stenosis is successfully addressed by repeating BPV with lager balloons than used initially. Long-term results revealed continue relief of obstruction, but with development of pulmonary insufficiency, some patients requiring replacement of the pulmonary valve. It was concluded that BPV is the treatment of choice in the management valvar PS and that balloon/annuls ratio used for BPV should be lowered to 1.2 to 1.25. It was also suggested that strategies should be developed to prevent/reduce pulmonary insufficiency at long-term follow-up.
Topics: Humans; Balloon Valvuloplasty; Pulmonary Valve Stenosis; Pulmonary Valve; Treatment Outcome
PubMed: 38147974
DOI: 10.1016/j.ihj.2023.12.007 -
Pediatric Radiology Jul 2020There is a broad spectrum of congenital anomalies of the central pulmonary arteries including abnormalities of development, origin, course and caliber. These anomalies... (Review)
Review
There is a broad spectrum of congenital anomalies of the central pulmonary arteries including abnormalities of development, origin, course and caliber. These anomalies incorporate simple lesions such as isolated pulmonary valve stenosis to very complex anomalies with many associated abnormalities. Part 1 and Part 2 of this review describe the range of anatomical variations that are encountered as well as important aspects of anatomy, physiology and surgical correction. The authors summarize and illustrate both well-recognized and more complex anomalies to provide a broad and comprehensive understanding of these lesions and their appearances on CT and MR imaging. In Part 2 the authors review abnormalities in development, origin and course of the central branch pulmonary arteries as well as abnormal pulmonary artery caliber.
Topics: Abnormalities, Multiple; Anatomic Variation; Humans; Infant, Newborn; Magnetic Resonance Imaging; Pulmonary Artery; Respiratory System Abnormalities; Tomography, X-Ray Computed; Vascular Malformations
PubMed: 32500159
DOI: 10.1007/s00247-020-04703-3 -
Journal of Electrocardiology 2023Right ventricular hypertrophy can be caused by conditions such as pulmonary stenosis and pulmonary hypertension. ECG is a readily available and affordable test, the aim...
BACKGROUND
Right ventricular hypertrophy can be caused by conditions such as pulmonary stenosis and pulmonary hypertension. ECG is a readily available and affordable test, the aim of this study was the evaluation of the electrocardiographic aspects of pulmonary stenosis, and pulmonary hypertension.
METHODS
A list of patients diagnosed with isolated pulmonary stenosis and pulmonary hypertension patients hospitalized and treated between 2019 and 2021 were extracted from the hospital archives. Furthermore, the ECG of the patients was analyzed in terms of the prevalence of the variables in the study using FECG Caliper software. Finally, the data of 93 patients (in both groups) were analyzed.
RESULTS
In this study, 46 patients were in the severe pulmonary stenosis group, and 49 were in the severe or moderate-to-severe pulmonary hypertension group. The heart rate in the pulmonary hypertension group was significantly higher. R/S > 1 in precordial leads differs between the two groups and higher amplitude R wave in V1(p-value = 0.05). in the pulmonary stenosis group. While in the pulmonary hypertension group, R wave growth occurs later, and this ratio is greater than one after V4. Bundle block in the form of RBBB(p-value <0.001) and maximum QRS duration is more in the pulmonary stenosis group(p-value = 0.001).
CONCLUSION
Our findings show the different strains of the right ventricle in two groups. It can be concluded that the effects of severe pulmonary stenosis on the ECG are more on the QRS wave and in the form of a block, while severe pulmonary hypertension affects the ST segment and T wave.
Topics: Humans; Electrocardiography; Hypertension, Pulmonary; Arrhythmias, Cardiac; Heart Rate; Pulmonary Valve Stenosis
PubMed: 37688841
DOI: 10.1016/j.jelectrocard.2023.08.010 -
Frontiers in Genetics 2022Common cardiac abnormalities in Noonan syndrome (NS) include congenital heart diseases (CHD), pulmonary valve stenosis and hypertrophic cardiomyopathy (HCM). Molecular...
Common cardiac abnormalities in Noonan syndrome (NS) include congenital heart diseases (CHD), pulmonary valve stenosis and hypertrophic cardiomyopathy (HCM). Molecular diagnoses are enabling earlier and more precise diagnosis of patients who have a subtle or atypical presentation. The aims of this study were to investigate genotype-phenotype associations with respect to Noonan syndrome (NS)-associated cardiac abnormalities and catheter or surgery-based interventions conditions. From January 2019 to December 2021, 22 children with a confirmed molecular diagnosis of NS combined with cardiovascular abnormalities were consecutively enrolled into the current study. A comprehensive review was carried out of echocardiography and electrocardiogram results, second-generation whole-exome sequencing results and catheter or surgery-based interventions conditions. The main manifestations of electrocardiogram abnormalities were QTc prolongation, abnormal Q wave in the precordial lead and limb lead, right ventricular hypertrophy and left or right deviation of the electrical axis. The most commonly detected abnormality was pulmonary valve dysplasia with stenosis, seen in 15 (68.2%) patients, followed by atrial septal defect in 11 (50%) patients. Seven genes (, , , , , , and ) were found to contain disease-associated variants The most commonly observed genetic mutations were (27%) and (27%) Each genotype was associated with specific phenotypic findings. , , , and had common echocardiography features characterized by pulmonary valve stenosis, while was characterized by HCM. Interestingly, patients with mutations were not only characterized by HCM, but also by pulmonary valve stenosis. In the cohort there was only one patient carrying a mutation characterized by left ventricle globose dilation. Ten cases underwent catheter or surgery-based interventions. All the operations had immediate results and high success rates. However, some of the cases had adverse outcomes during extended follow-up. Based on the genotype-phenotype associations observed during follow-up, and genotypes seem to be poor prognostic factors, and multiple interventions may be required for NS patients with severe pulmonary stenosis or myectomy for HCM. The identification of causal genes in NS patients has enabled the evaluation of genotype-cardiac phenotype relationships and prognosis of the disease. This may be beneficial for the development of therapeutic approaches.
PubMed: 35770001
DOI: 10.3389/fgene.2022.915129 -
Archives of Cardiovascular Diseases Feb 2020Fetal cardiac intervention (FCI) is a novel and evolving technique that allows for in utero treatment of a subset of congenital heart disease. This review describes the... (Review)
Review
Fetal cardiac intervention (FCI) is a novel and evolving technique that allows for in utero treatment of a subset of congenital heart disease. This review describes the rationale, selection criteria, technical features, and current outcomes for the three most commonly performed FCI: fetal aortic stenosis with evolving hypoplastic left heart syndrome (HLHS); HLHS with intact or restrictive atrial septum; and pulmonary atresia with intact ventricular septum, with concern for worsening right ventricular (RV) hypoplasia.
Topics: Aortic Valve Stenosis; Cardiac Catheterization; Cardiac Surgical Procedures; Echocardiography; Female; Fetal Heart; Fetal Therapies; Gestational Age; Heart Defects, Congenital; Humans; Hypoplastic Left Heart Syndrome; Pregnancy; Pulmonary Atresia; Risk Factors; Treatment Outcome; Ultrasonography, Prenatal
PubMed: 32113817
DOI: 10.1016/j.acvd.2019.06.007 -
Pediatric Investigation Dec 2022Surgical repair of a variety of congenital heart diseases involves repair of the right ventricular outflow tract (RVOT) with valved or non-valved conduit to connect the... (Review)
Review
Surgical repair of a variety of congenital heart diseases involves repair of the right ventricular outflow tract (RVOT) with valved or non-valved conduit to connect the right ventricle (RV) to the pulmonary artery (PA) or just patch enlargement of the native RVOT. With time, this RV-PA conduit will degenerate with deterioration of function, either causing pulmonary stenosis or pulmonary regurgitation. This RVOT dysfunction may result in RV dilation, RV dysfunction, and eventual RV failure and arrhythmias. Multiple surgical pulmonary valve replacement (PVR) is often required throughout the patient's lifetime. Patients are subjected to increased risks with each additional cardiac operation. Transcatheter PVR (TPVR) has been developed over the past two decades as a valuable non-surgical alternative to restore the RVOT and RV function, and hence reduce patients' lifetime risks related to surgery. This article will discuss the long-term results of TPVR which are demonstrated to be comparable to surgical results and the latest development of large pulmonary valves which will allow TPVR to be performed on native or larger RVOT.
PubMed: 36582274
DOI: 10.1002/ped4.12359 -
The Veterinary Clinics of North... Nov 2023There have been recent advancements in understanding the genetic contribution to pulmonary valve stenosis (PS) in brachycephalic breeds such as the French Bulldog and... (Review)
Review
There have been recent advancements in understanding the genetic contribution to pulmonary valve stenosis (PS) in brachycephalic breeds such as the French Bulldog and Bulldog. The associated genes are transcriptions factors involved in cardiac development, which is comparable to the genes that cause PS in humans. However, validation studies and functional follow up is necessary before this information can be used for screening purposes.
Topics: Animals; Dogs; Humans; Pulmonary Valve Stenosis; Craniosynostoses; Breeding; Dog Diseases
PubMed: 37423844
DOI: 10.1016/j.cvsm.2023.05.014 -
Frontiers in Cardiovascular Medicine 2022
PubMed: 35924216
DOI: 10.3389/fcvm.2022.970515 -
Pediatric Radiology Jul 2020There is a broad spectrum of congenital anomalies of the central pulmonary arteries including abnormalities of development, origin, course and caliber. These anomalies... (Review)
Review
There is a broad spectrum of congenital anomalies of the central pulmonary arteries including abnormalities of development, origin, course and caliber. These anomalies incorporate simple lesions such as isolated pulmonary valve stenosis to very complex anomalies with many associated abnormalities. Part 1 and Part 2 of this review describe the range of anatomical variations that are encountered as well as important aspects of anatomy, physiology and surgical correction. The authors summarize and illustrate well-recognized as well as more complex anomalies to provide a broad and comprehensive understanding of these lesions and their appearances on CT and MR imaging. Part 1 covers anomalous development or origin of the main pulmonary artery.
Topics: Abnormalities, Multiple; Anatomic Variation; Humans; Infant, Newborn; Magnetic Resonance Imaging; Pulmonary Artery; Respiratory System Abnormalities; Tomography, X-Ray Computed; Vascular Malformations
PubMed: 32474775
DOI: 10.1007/s00247-020-04690-5 -
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