scholarly journals The diagnosis and treatment of pulmonary valve stenosis in children

2015 ◽  
Vol 6 (6) ◽  
pp. 1-5 ◽  
Author(s):  
Shiv Kumar Yadav
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Recent Progress ◽  
Pulmonary Valve ◽  
Diagnosis And Treatment ◽  
Pulmonary Valve Stenosis ◽  
Medical Sciences ◽  
Percutaneous Balloon ◽  
First Choice

Pulmonary valve stenosis (PS) is a common congenital heart disease, its early diagnosis and treatment can improve growth and prognosis in children with pulmonary valve stenosis. With the development of the diagnostics and treatment, percutaneous balloon pulmonary valvuloplasty (PBPV) has replaced surgery as the first choice for isolated pulmonary valve stenosis. The article reviews the recent progress in diagnosis and treatment of pulmonary valve stenosis.DOI: http://dx.doi.org/10.3126/ajms.v6i6.12017Asian Journal of Medical Sciences Vol.6(6) 2015 1-5 

scholarly journals Long-term survival in patients with isolated pulmonary valve stenosis: a not so benign disease?

Open Heart ◽  
2021 ◽  
Vol 8 (2) ◽  
pp. e001836
Author(s):  
Kristofer Skoglund ◽  
Annika Rosengren ◽  
Georgios Lappas ◽  
Maria Fedchenko ◽  
Zacharias Mandalenakis
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Early Diagnosis ◽  
Congenital Heart ◽  
Pulmonary Valve ◽  
Survival Rates ◽  
Pulmonary Valve Stenosis ◽  
Term Survival ◽  
Risk Of Mortality

Background and objectivesDuring the last decades, the survival rates in patients with congenital heart disease have increased dramatically, particularly in patients with complex heart malformations. However, the survival in patients with simple defects is still unknown. We aimed to determine the characteristics and the risk of mortality in patients with isolated pulmonary valve stenosis (PS).MethodsSwedish inpatient, outpatient and cause of death registries were used to identify patients born between 1970 and 2017 with a diagnosis of PS, without any other concomitant congenital heart lesion. For each patient with PS, 10 control individuals without congenital heart disease were matched by birth year and sex from the total population registry. We used median-unbiased method and Kaplan-Meier survival analysis to examine the risk of mortality.ResultsWe included 3910 patients with PS and 38 770 matched controls. The median age of diagnosis of PS was 0.7 years (IQR 0.3–7.0). During a median follow-up of 13.5 years (IQR 6.5–23.5), 88 patients with PS and 192 controls died; 500 patients with PS (12%) underwent at least one transcatheter or surgical valve intervention. The overall mortality rate was significantly higher in patients with PS compared with matched controls (HR 4.67, 95% CI 3.61 to 5.99, p=0.001). Patients with an early diagnosis of PS (0–1 year) had the highest risk of mortality (HR 10.99, 95% CI 7.84 to 15.45).ConclusionsIn this nationwide, register-based cohort study, we found that the risk of mortality in patients with PS is almost five times higher compared with matched controls. Patients with an early diagnosis of PS appears to be the most vulnerable group and the regular follow-up in tertiary congenital heart units may be the key to prevention.

scholarly journals Severe Pulmonary Valvular Stenosis and Balloon Pulmonary Valvuloplasty (BPV): A Case Report

2015 ◽  
Vol 3 (1-2) ◽  
pp. 53-58
Author(s):  
Tahera Nazrin ◽  
CM Shaheen Kabir ◽  
Sahela Nasrin ◽  
Md Rokonujjaman ◽  
M Maksumul Huq ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Pulmonary Valve ◽  
Excellent Result ◽  
Pulmonary Stenosis ◽  
Pulmonary Valve Stenosis ◽  
Short Term ◽  
Valvular Stenosis ◽  
Congenital Cyanotic Heart Disease

Congenital pulmonary valve stenosis is a common congenital heart disease andIsolated pulmonary valve stenosis comprises 8-10% of all congenital heart disease. It is an acyanotic heart disease,but can present with severe cyanosis if it is associated with patent foramen ovale (PFO). Severe pulmonary stenosis with cyanosis can be misdiagnosed clinically. Proper evaluation and modern technique of treatment modality can save a life easily. We report a 5 years old boy with severe pulmonary valvular stenosis with PFO who was clinically misdiagnosed as a case of congenital cyanotic heart disease (Tetralogy of Fallot). After taking proper history, clinical examination and investigations we treated the baby by balloon pulmonary valvuloplasty (BPV) successfully without any complication. The short term (6 months) outcome of BPV showed excellent result. Ibrahim Cardiac Med J 2013; 3(1&2): 53-58

scholarly journals Adult Congenital Heart Disease Investigated with Cardiac Catheterization Over A 20-Year Period

2009 ◽  
Vol 3 (1) ◽  
pp. 124-127 ◽  
Author(s):  
George D Giannoglou ◽  
Antonios P Antoniadis ◽  
Yiannis S Chatzizisis ◽  
George E Louridas
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Atrial Septal Defect ◽  
Cardiac Catheterization ◽  
Congenital Heart ◽  
Septal Defect ◽  
Pulmonary Valve ◽  
Adult Congenital Heart Disease ◽  
Pulmonary Valve Stenosis ◽  
Adult Congenital

Background: Recent advances in diagnosis and treatment have increased the life expectancy of patients with congenital heart disease. Methods: To investigate the prevalence of adult congenital heart disease (ACHD) in a large registry of patients over a 20-year period, we retrospectively assessed data of 14,012 males and 4,461 females who underwent clinically indicated cardiac catheterization from 1984 to 2003. Results: ACHD was recorded in 234 subjects aged from 18 to 66 years, [95 males (40.7%) and 139 females (59.3%)]. Females were more likely to present with ACHD than males (p<0.001). Atrial septal defect was the most common defect (43.3%) followed by partial anomalous pulmonary venous return (12.0%), pulmonary valve stenosis (11.3%) ventricular septal defect (8.0%), coarctation of aorta (5.5%) patent ductus arteriosus (4.0%) and Fallot’s tetralogy (3.3%). Atrial septal defect was more common in females (p<0.01), while pulmonary valve stenosis was more frequent in males (p<0.05). No difference across sexes was found in the other forms of ACHD. Females with ACHD were significantly older than males at the time of catheterization (median age 41 years, interquartile range 26 to 53 years vs. median age 35 years, interquartile range 22 to 48 years, p<0.05). Conclusions: In adulthood ACHD is found more commonly in females and is diagnosed later in life than in males. Atrial septal defect is the most prevalent form of ACHD and occurs most commonly in females.

scholarly journals Prevalence, Type, and Molecular Spectrum of NF1 Mutations in Patients with Neurofibromatosis Type 1 and Congenital Heart Disease

Genes ◽  
2019 ◽  
Vol 10 (9) ◽  
pp. 675 ◽  
Author(s):  
Pinna ◽  
Daniele ◽  
Calcagni ◽  
Mariniello ◽  
Criscione ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Neurofibromatosis Type 1 ◽  
Odds Ratio ◽  
Congenital Heart ◽  
Pulmonary Valve ◽  
Neurofibromatosis Type ◽  
Study Cohort ◽  
Pulmonary Valve Stenosis

The aim of this study was to assess the prevalence and type of congenital heart disease (CHD) and the associated mutation spectrum in a large series of patients with neurofibromatosis type 1 (NF1), and correlate the mutation type with the presence and subgroups of cardiac defects. The study cohort included 493 individuals with molecularly confirmed diagnosis of NF1 for whom cardiac evaluation data were available. CHD was reported in 62/493 (12.6%) patients. Among these patients, 23/62 (37.1%) had pulmonary valve stenosis/dysplasia, 20/62 (32.3%) had mitral valve anomalies, and 10/62 (16.1%) had septal defects. Other defects occurred as rare events. In this NF1 subcohort, three subjects carried a whole-gene deletion, while 59 were heterozygous for an intragenic mutation. A significantly increased prevalence of non-truncating intragenic mutations was either observed in individuals with CHD (22/59, 37.3%) or with pulmonary valve stenosis (13/20, 65.0%), when compared to individuals without CHD (89/420, 21.2%) (p = 0.038) or pulmonary valve stenosis (98/459, 21.4%) (p = 0.002). Similarly, patients with non-truncating NF1 mutations displayed two- and six-fold higher risk of developing CHD (odds ratio = 1.9713, 95% confidence interval (CI): 1.1162–3.4814, p = 0.0193) and pulmonary valve stenosis (odds ratio = 6.8411, 95% CI: 2.6574–17.6114, p = 0.0001), respectively. Noteworthy, all but one patient (19/20, 95.0%) with pulmonary valve stenosis, and 18/35 (51.4%) patients with other CHDs displayed Noonan syndrome (NS)-like features. Present data confirm the significant frequency of CHD in patients with NF1, and provide further evidence for a higher than expected prevalence of NF1 in-frame variants and NS-like characteristics in NF1 patients with CHD, particularly with pulmonary valve stenosis.

The Pediatrician and Congenital Heart Disease—1979

PEDIATRICS ◽  
1979 ◽  
Vol 64 (2) ◽  
pp. 180-186
Author(s):  
Thomas W. Rowland
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Early Identification ◽  
Congenital Heart ◽  
Diagnosis And Treatment

Technologic advances in the diagnosis and treatment of children with congenital heart disease have created new, expanded roles for the practicing pediatrician. Early identification of infants with serious lesions continues to be vital, and support of children and their families following surgery is central to the solution of emotional, employment, and childbearing problems these patients may eventually face.

scholarly journals Right ventricular restoration during pulmonary valve implantation in adults with congenital heart disease☆

2006 ◽  
Vol 29 ◽  
pp. S279-S285 ◽  
Author(s):  
Alessandro Frigiola ◽  
Alessandro Giamberti ◽  
Massimo Chessa ◽  
Marisa Di Donato ◽  
Raul Abella ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Right Ventricular ◽  
Congenital Heart ◽  
Pulmonary Valve ◽  
Valve Implantation ◽  
Ventricular Restoration ◽  
Pulmonary Valve Implantation

scholarly journals P1582 Outcomes, predictors and treatment of intraatrial reentrant tachycardias in Adult with Congenital Heart Disease

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
E Cambronero Cortinas ◽  
P Moratalla-Haro ◽  
A E Gonzalez-Garcia ◽  
P Avila-Alonso ◽  
M Bret-Zurita ◽  
...  
Keyword(s):  
Risk Factors ◽  
Congenital Heart Disease ◽  
Risk Factor ◽  
Heart Disease ◽  
Tricuspid Regurgitation ◽  
Cardiovascular Events ◽  
Congenital Heart ◽  
Late Complication ◽  
Systolic Pressure ◽  
First Choice

Abstract Introduction and objectives Atrial tachycardia (AT) is a major late complication in congenital heart disease (CHD) after surgery. These arrhythmias arise between areas of anatomical barriers or in fibrotic tissue/scars. In our study we examined the clinical predictors of AT. Methods A retrospective study of cases and controls in adult patients with CHD was performed. We have included consecutively 71 patients with AT and 71 controls matched by gender, age and type of CHD located in the same database. Medical records, electrocardiograms and echocardiograms were reviewed. Multivariate analysis of the risk factor (logistic regression) and analysis of adverse cardiovascular events (Kaplan-Meier) were performed. Results Systolic pressure of subpulmonary ventricle ≥40mmHg (HR:7, 95%CI:2.4-18, p = 0.001), right atrium (RA) dilatation≥21cm2 (HR:3, 95%CI:1.2-7.6, p = 0.005), significant tricuspid regurgitation (HR:4, 95%CI:1.3-10, p = 0.018) were identified as main risk factors for AT. Patients with AT had worse outcomes with more major adverse cardiovascular events (86% vs 14%, P = 0.006), and a 58% free survival events compared to 98% of patients without AT at 8 years of follow-up (p = 0.01). The treatment of the first episode of AT was electrical cardioversion (38%), pharmacological cardioversion (13%), heart rate control (20%). Antiarrhythmic treatment was not been given in 30% of the patients 73% of them had percutaneous ablation as first choice treatment. Ablation was performed in 40 pacientes and 6 patients have recurrences. In total, 24 patients have recurrences of AT. Previous Maze (OR 9 (IC al 95% 1.5, 50), p = 0.016) and surgical paliative shunt (OR 21 (IC al 95% 2.3,192), p = 0.007) were identified as main risk factor for AT recurrences. Conclusions The main risk factors identified for AT were RA dilatation, raised systolic pressure of subpulmonary ventricle and significant tricuspid regurgitation. The main risk factors for AT recurrences were previous Maze and surgical paliative shunt. Early treatment of these arrhythmic problems is mandatory in order to improve the prognosis in this group of patients. Abstract P1582 Figure. Central illustration of AT in CHD

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