scholarly journals Dysplastic pulmonary valve stenosis associated with unilateral absent first metacarpal: A rare association

2017 ◽  
Vol 5 ◽  
pp. 2050313X1772694 ◽  
Author(s):  
Ashwin Kodliwadmath
Keyword(s):  
Pulmonary Valve ◽  
Past History ◽  
Nyha Class ◽  
Pulmonary Stenosis ◽  
Pulmonary Valve Stenosis ◽  
Class 1 ◽  
Common Variety ◽  
Valvular Pulmonary Stenosis ◽  
Dysplastic Pulmonary Valve ◽  
Hand Anomalies

Context: Dysplastic pulmonary valve stenosis is a less common variety of valvular pulmonary stenosis. It is known to be part of Noonan syndrome. Bony hand anomalies in patients of pulmonary stenosis are very rare. Case report: A 50-year-old lady, with no significant past history, presented with slowly progressive breathlessness and fatigue, and had progressed from NYHA class 1 to 2 over 2 years. She had unilateral absent first metacarpal and diagnosed on workup to have dysplastic pulmonary valve stenosis and was treated with balloon valvuloplasty. Conclusion: Dysplastic pulmonary valve stenosis can rarely be associated with bony hand anomalies like absent first metacarpal.

scholarly journals Case Presentation - Biventricular Hypertrophy and Valvular Pulmonary Stenosis in Adult Patient with Noonan Syndrome: A Rare Case

2021 ◽  
Vol 2 (2) ◽  
pp. 77
Author(s):  
Tinton Pristianto ◽  
Rosi Amrilla Fagi
Keyword(s):  
Noonan Syndrome ◽  
Pulmonary Valve ◽  
Genetic Disorder ◽  
Pulmonary Stenosis ◽  
Pulmonary Regurgitation ◽  
Nasolabial Fold ◽  
Pulmonary Valve Stenosis ◽  
Case Presentation ◽  
Valvular Pulmonary Stenosis ◽  
Chest Deformities

Introduction: Noonan syndrome (NS) is a genetic disorder often accompanied by multiple congenital abnormalities. The prevalence of NS at live birth has been reported as one in 1000-2500 individuals. About 80% of patients with Noonan syndrome have abnormalities in the cardiovascular system.Case presentation:41-year-old Javanese male presented with chief complaint shortness of breath.  His Body Mass Index (BMI) was 18,3. He had an oval-shaped face with a short neck, thin hair, and prominent nasolabial fold. Echocardiography showed biventricular hypertrophy alongside pulmonary valve stenosis, pulmonary regurgitation and minimal pericardial effusion. Discussion: In 1962, Jacqueline Noonan, a pediatric cardiologist, identified 9 patients whose faces were very similar, had short stature, significant chest deformities, and with pulmonary stenosis. Noonan syndrome is a relatively common non-chromosomal syndrome that is similar to the phenotype of Turner's syndrome and presents with cardiovascular malformations. Adult with NS has distinctive facial features such as ptosis, wide eyes, low posterior rotation of ears and helical thickening, and a wide neck.Pulmonary stenosis is the most common heartdefect found in NS, besides HCM isalsoquitecommon inabout20% of patients. We reported a case of a patient with typical characteristics of NS such as pulmonary valve stenosis accompanied by biventricular ventricular hypertrophyand its typical face who survived through adulthood.Conclusion: Syndrome Noonan in the adult is quite rare and difficult to diagnose. We reported a case of an adult man with facial appearance and echocardiographic findings identical with Noonan Syndrome.

scholarly journals Results of balloon valvuloplasty in typical and dysplastic pulmonary valve stenosis: Doppler echocardiographic follow-up

1988 ◽  
Vol 12 (2) ◽  
pp. 476-479 ◽  
Author(s):  
Pablo M. Marantz ◽  
James C. Huhta ◽  
Charles E. Mullins ◽  
Daniel J. Murphy ◽  
Michael R. Nihill ◽  
...  
Keyword(s):  
Pulmonary Valve ◽  
Balloon Valvuloplasty ◽  
Pulmonary Valve Stenosis ◽  
Dysplastic Pulmonary Valve

scholarly journals Natural Course of Isolated Pulmonary Valve Stenosis in Pediatric Patients

2020 ◽  
Vol 3 (2) ◽  
pp. 01-07
Author(s):  
Güven Serçin ◽  
Öztarhan Kazım
Keyword(s):  
Pediatric Patients ◽  
Pulmonary Valve ◽  
Pulmonary Stenosis ◽  
Age Groups ◽  
Natural Course ◽  
Pulmonary Valve Stenosis ◽  
Valvular Stenosis ◽  
Patient Groups ◽  
The Impact

Aim: The aim of our study is to be able to predict the prognosis of patients with isolated pulmonary valvular stenosis on the basis of age and degree of stenosis. Identification of the course of pulmonary stenosis of different age groups will significantly contribute both to the physicians and the relatives of the patient. Material and Methods: 105 pediatric patients diagnosed with isolated pulmonary valvular stenosis were included in our study. We investigated the impact of the gradient of stenosis and the age at the time of diagnosis on the natural course of pulmonary stenosis. Mean follow-up time of the children was 19 months, 25.45±22.48 months. The patients were divided into four groups over their trans-valvular gradient degrees and<20 mmHg was defined as transient, 20-39 mmHg mild, 40-59 mmHg moderate, 60 mmHg and over as severe pulmonary stenosis. Results: Between two to five months, none of the moderate stenosis cases progressed unlike other patient groups. The decline in the final gradient versus initial gradient was significant in children between two to five months and six months to two years in our study, and yet there was no significant change of initial and final gradients in patients under one month, and at two years and over. Conclusion: It would be reasonable to conclude that the progression of pulmonary valvular stenosis is benign in patients with pulmonary valvular stenosis under 40 mmHg of systolic gradient diagnosed after 6th month of life.

Treatment of severe refractory valvar pulmonary stenosis with primary transcatheter pulmonary valve implantation

2017 ◽  
Vol 27 (6) ◽  
pp. 1232-1234
Author(s):  
Kalyani R. Trivedi ◽  
Laurence Robinson ◽  
Alain Fraisse
Keyword(s):  
Noonan Syndrome ◽  
Surgical Repair ◽  
Pulmonary Valve ◽  
Pulmonary Stenosis ◽  
Balloon Dilation ◽  
Balloon Valvuloplasty ◽  
Pulmonary Valve Stenosis ◽  
Valve Implantation ◽  
Valvar Pulmonary Stenosis ◽  
Pulmonary Valve Implantation

AbstractSome patients with pulmonary valve stenosis do not respond to balloon valvuloplasty and must undergo surgical repair. We report the case of a 12-year-old child with pulmonary valve stenosis and Noonan syndrome in whom we performed transcatheter Melody pulmonary valve implantation after balloon dilation failed. The result was excellent. This technique can be proposed as an alternative to surgery in such cases.

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 Estenosis pulmonar con válvula Unicúspide. Diagnóstico por Resonancia Magnética Cardiaca. Informe de caso.

2018 ◽  
Vol 1 (38) ◽  
Author(s):  
Isabel Pitti ◽  
Mathew Tong ◽  
Ronald William ◽  
Robert Biederman
Keyword(s):  
Cardiovascular Magnetic Resonance ◽  
Magnetic Resonance ◽  
Pulmonary Valve ◽  
Pulmonary Stenosis ◽  
The Elderly ◽  
Pulmonary Valve Stenosis ◽  
Resonance Case ◽  
Adjacent Structures ◽  
Common Etiology ◽  
The Right

<p>[Pulmonary stenosis with Unicuspid valve. Diagnosis by Cardiac Magnetic Resonance. Case report.]</p><p>Resumen<br />La etiología más común de estenosis de la válvula pulmonar es congénita. La evaluación de la morfología de la válvula y de las estructuras adyacentes son importantes para correlacionar los síntomas del paciente y determinar el tratamiento. La estenosis pulmonar suele estar asociada con algún grado de obstrucción muscular subvalvular debido a la hipertrofia del miocardio del ventrículo derecho.  <br /><br />La morfología unicúspide de la válvula pulmonar es rara y su identificación es muy difícil en la ecocardiografía especialmente en los ancianos que tienen calcificación valvular.  Presentamos el caso de una paciente femenina de 56 años de edad con estenosis pulmonar sintomática de etiología confusa en la que la evaluación por resonancia magnética cardiovascular (RMC) define la morfología de la válvula, severidad de la estenosis pulmonar y del tracto de salida del ventrículo derecho, tamaño y función ventricular derecha y dilatación postestenótica de arteria pulmonar. <br /><br />Abstract<br />The most common etiology of pulmonary valve stenosis is congenital. The evaluation of the morphology of the valve and of the adjacent structures is important to correlate the patient's symptoms and determine the treatment. Pulmonary stenosis is usually associated with some degree of subvalvular muscle obstruction due to hypertrophy of the myocardium of the right ventricle. <br /><br />The unicuspid morphology of the pulmonary valve is rare and its identification is very difficult in echocardiography, especially in the elderly who have valvular calcification. We present the case of a 56-year-old female patient with symptomatic pulmonary stenosis of confused etiology in which the evaluation of cardiovascular magnetic resonance (CMR) defines the morphology of the valve, severity of the pulmonary stenosis and the outflow tract of the ventricle, right, size and right ventricular function and poststenotic dilatation of the pulmonary artery.</p>

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