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

Abstract

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.

Canine Animal as a Model in the Repercussion of Pulmonary Stenosis: Analysis by Two-Dimensional Echocardiography and Doppler Mode - Case Report

“…Such alterations are similar to echocardiographic findings in human PD patients, in which pulmonary stenosis, right ventricular hypertrophy, reduced RV systolic function, biatrial enlargement, aortic insufficiency, mitral and tricuspid insufficiency and shunts were reported. However, the severity of these varied changes in each case according to the degree of the change and the presence of other pathologies [15,23].…”

“…Such alterations are similar to echocardiographic findings in human PD patients, in which pulmonary stenosis, right ventricular hypertrophy, reduced RV systolic function, biatrial enlargement, aortic insufficiency, mitral and tricuspid insufficiency and shunts were reported. However, the severity of these varied changes in each case according to the degree of the change and the presence of other pathologies [15,23].Due to these findings, drug therapy [24] was chosen with the use of an angiotensin converting enzyme (ACE) inhibitor, benazepril, at a dose of 0.5 mg / kg for the treatment of heart failure, aiming at the relaxation of the right ventricle and, consequently, improvement in the transvalvar gradient [25]. Pharmacological blockade of ACE-activated hormones or their metabolic pathways prolongs survival in patients with Congestive Heart Failure (CHF).…”

Endovascular Methods of Treatment Pulmonary Artery Stenosis

“…Стеноз ЛА частіше вроджена патологія. Її причинами є: а) генетична схильність до розвитку порушень роботи серця; ризик виникнення захворювання зростає, якщо з подібними проблемами стикалася мати новонародженого; б) вживання майбутньою мамою психотропних, антибактеріальних або наркотичних засобів на ранніх термінах вагітності; в) робота вагітної жінки на шкідливому виробництві; г) перенесені під час вагітності вірусні інфекції (наприклад, краснуха); д) несприятливий вплив радіаційних випромінювань на організм жінки, яка виношує дитину; є) забруднена екологія в регіоні проживання майбутньої мами [10].…”