Pulmonary Atresia and Pulmonary Stenosis

Miroslav Elek
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Pulmonary Atresia and Pulmonary Stenosis - Presentation
Pulmonary atresia with ventricular septal defect is synonymus with extreme form of Tetralogy of Fallot and Type IV truncus arteriosus or psueudotruncus. The amount of pulmonary blood supply makes a difference in treatment and outcome. Pulmonary circulation is provided by PDA, bronchal arteries and aorto-pulmonary collaterals. Newborns with PA and VSD are good in first hours of life until ductus arteriosus closes. Then occurs hypoxemia, cyanosis, acidosis and signs of respiratory distress and heart failure.
PA with intact interventricular septum (IVS) is very rare congenital heart defect and it presents in first hours of life with tachypnea and cyanosis of newborn. Blood from right ventricle has no way out except retrograde flow through the tricuspid valve. Often can be found communication between right ventricle and coronary vessels, through the persistent myocardial sinuses.
Mild and moderate pulmonary stenosis can be asymptomatic for a long time. Severe or critical stenosis manifests with cyanosis, tachypnea, exercise intolerance, arrhythmias, and signs of right heart failure (hepatomegalia, ascites, edema, neck veins distension). In older children can occur exercise induced syncope and symptoms similar to those of ischemic heart disease.


References
Pulmonary Atresia and Pulmonary Stenosis Pulmonary Atresia and Pulmonary Stenosis 05/14/2016
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