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 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 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 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 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.

scholarly journals Evolution of biventricular T1 values in patients with right-sided congenital heart disease

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
O Burdet ◽  
AG Pavon ◽  
J Bouchardy ◽  
C Blanche ◽  
P Monney ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Interstitial Fibrosis ◽  
Pulmonary Stenosis ◽  
Pulmonary Regurgitation ◽  
Native T1 ◽  
Funding Sources ◽  
Basal Slice ◽  
Relationship Of

Abstract Funding Acknowledgements Type of funding sources: None. Background Conflicting reports exist on the prevalence and clinical impact of interstitial fibrosis in right ventricular (RV) congenital heart disease (CHD). This study evaluates the longitudinal evolution of native myocardial T1 relaxation time (T1) in RV CHD. Methods On a 1.5T scanner, an ECG-triggered modified Look-Locker inversion recovery sequence (scheme 3(3)3(3)5) was acquired on a short-axis basal slice covering the RV and left ventricle (LV) on two consecutive CMR exams. Global and segmental (LV = 6, RV = 4) RV and LV T1 values  were calculated (Figure). Results Mean time between CMR exams for 36 included patients (age 34 ± 2y) was 22 ± 2 months. All LV segments and 81/88% of RV segments of first and second CMR could be analyzed, respectively.  T1 increased mildly but not significantly (table). There was no relationship of T1 to pulmonary regurgitation fraction, pulmonary stenosis or RV enddiastolic volume (p &gt; 0.05). Global RV T1 of the second CMR was related to RV ejection fraction (RVEF): r = 0.353, 3.0 ± 1.4, p = 0.038. T1 of the infero-septal LV segment of first and second CMR, global LV T1 of second CMR and increase of T1 of global LV, anterior, antero-lateral and –septal LV segments, were related to age at CMR: r = 0.333 - 0.463, p &lt; 0.05, respectively. Conclusions Native T1 values increased mildly in patients with stable RV CHD, which was not statistically significant probably due to the short to median follow-up. Global RV T1 appears to be related to RVEF which could be sign of increasing interstitial fibrosis whereas the relationship of LV T1 to age might be a physiological finding. First CMR native T1 (ms) Second CMR native T1 (ms) p LV Global 1007 ± 37 1014 ± 39 0.413 LV Anterior 994 ± 53 999 ± 54 0.710 LV Antero-lateral 965 ± 63 981 ± 58 0.186 LV Infero-lateral 1000 ± 52 1004 ± 63 0.695 LV Inferior 1035 42 1037 ± 50 0.744 LV Infero-septal 1028 ± 35 1036 ± 43 0.282 LV Antero-septal 1016 ± 38 1024 ± 48 0.347 RV Global 1091 ± 90 1096 ± 85 0.410 RV Inferior 1112 ± 104 1115 ± 118 0.696 RV Infero-lateral 1061 ± 130 1077 ± 115 0.425 RV Antero-lateral 1046 ± 127 1080 ± 109 0.088 RV Anterior 1088 ± 156 1108 ± 154 0.410 Abstract Figure. Determination of biventricular T1 values

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 Congenital heart disease in adults and its problems

2001 ◽  
Vol 41 (5) ◽  
pp. 237
Author(s):  
Teddy Ontoseno
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Septal Defect ◽  
Adult Congenital Heart Disease ◽  
Ductus Arteriosus ◽  
Pulmonary Stenosis ◽  
Adult Congenital ◽  
Education Achievement ◽  
Adult Lives

There were 40 adult congenital heart disease (CHD) patients seen in the Cardiology Division during 1 year (February 1993 - February 1994). The most frequently seen defect was atrial septal defect; however there were also cases with patent ductus arteriosus, pulmonary stenosis, ventricular septal defect, and tetralogy of Fallot. Hemodynamic disorder, serious hindrance to education achievement, and occupational threat due to limited physical capabilities as well as malnutrition are some of prominent issues to be closely anticipated. In general the older the patients the more serious hemodynamic disorder they suffer due CHD. It is worth thinking how to improve the quality of life of CHD patients who succeed to live their adult lives and minimize any possible fatal complication risks.

scholarly journals Double Inlet Left Ventricle with Eisenmenger Syndrome in an Adult – A Case Report

2017 ◽  
Vol 5 (1) ◽  
pp. 53-56
Author(s):  
Rahul Regi Abraham ◽  
Rahul Regi Abraham
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Left Ventricle ◽  
Congenital Heart ◽  
Pulmonary Stenosis ◽  
Eisenmenger Syndrome ◽  
Tertiary Center ◽  
History Of ◽  
Double Inlet Left Ventricle

Background: Patient diagnosed with double inlet left ventricle (prevalent in 5 – 10 in 100,000 newborns) complicated with Eisenmenger syndrome had a median survival age of 14 years without corrective surgery. Congenital heart disease such as this is usually treated by multiple surgeries during early childhood. A surgically uncorrected case in adults is not of common occurrence. Further, generalized itching after coming in contact with water (aquagenic pruritis) presented an interesting conundrum to treat. Case: A 29-year-old patient in India presented at a primary health care center with a history of difficulty breathing and discoloration of extremities since birth. He also gave a history of itching which commonly occurred after taking bath, hemoptysis and history of turning blue in color after birth. Patient had received no treatment besides regular phlebotomies. On examination, there was grade IV clubbing and conjunctival congestion. Cardiovascular examination revealed an enlarged heart, heaving apex beat and a pan-systolic murmur. A provisional diagnosis of a congenital cyanotic heart disease was made. Investigations revealed hemoglobin of 16.8g/dl. X–ray and electrocardiogram showed hypertrophy of the ventricles. An echocardiogram showed double inlet left ventricle with L-malposed vessels but without pulmonary stenosis. A final diagnosis of congenital heart disease; double inlet left ventricle, L-malposed vessels without pulmonary stenosis, Eisenmenger Syndrome and absolute erythrocytosis was made. Patient was advised for further management with a cardiologist in a tertiary center but the patient did not follow up. Conclusion: Unlike in high-income countries where most congenital heart diseases are detected and dealt with at birth whereas low-and middle-income nations often have to deal with cases that present much later and should often be included in the differential diagnosis. Inability to follow up cases, centers that are poorly equipped and lack of facilities for investigations, patient’s lack of medical awareness, and financial restrictions are major barriers to providing optimal treatment.

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