Is scimitar syndrome rare?
Scimitar syndrome is a rare congenital disorder. It has a varied presentation. In adult life, it usually presents either as recurrent chest infection and/or exertional dyspnea. Pulmonary artery hypertension and hemoptysis both are uncommon features of this syndrome in adult life.
How common is scimitar syndrome?
Scimitar syndrome (SS) is a rare congenital heart malformation occurring in one to three per 100,000 live births with a 2:1 female predominance and accounts for the 3%–6% of partial anomalous pulmonary venous connection (PAPVR) [1,2]. The true incidence may be higher because many patients are asymptomatic.
Can scimitar syndrome be cured?
Less commonly, scimitar syndrome is discovered in adults, many of whom have mild or no symptoms at the time of diagnosis (3). Conventionally the syndrome is corrected surgically by redirecting central venous return toward the left atrium.
What are the symptoms of scimitar syndrome?
In its infant form, the scimitar syndrome is diagnosed within the first 2 months after birth, with symptoms of failure to thrive, tachypnea, heart failure and cyanosis. There is an associated mortality of about 45%.
What is Swyer James Syndrome?
Summary. Listen. Swyer-James syndrome is a rare condition in which the lung (or portion of the lung) does not grow normally and is slightly smaller than the opposite lung, usually following bronchiolitis in childhood.
What is a scimitar vein?
Scimitar syndrome is an association of congenital cardiopulmonary anomalies characterized by partial anomalous pulmonary venous return, in which an abnormal right pulmonary vein drains into the IVC either above or below the diaphragm.
What causes Papvr?
In many cases, we don’t know what causes TAPVR. It occurs because of abnormal development of the heart’s pulmonary veins during early fetal growth. Some congenital heart defects may have a genetic link, causing heart problems to occur more often in certain families.
What is Scimitar disease?
Scimitar, or pulmonary venolobar, syndrome is a rare but well-known congenital cardiovascular defect that includes a hypoplastic right pulmonary artery and right lung, which leads to displacement of cardiac structures into the right hemithorax, anomalous systemic arterial supply to the right lung, and a …
What is Mounier Kuhn syndrome?
Background: Mounier-Kuhn syndrome (MKS) is a rare disorder characterized by enlargement of the trachea and main bronchi and associated with recurrent respiratory tract infections.
What is Hyperlucent?
hy·per·lu·cent. (hī’pĕr-lū’sĕnt), A region on a chest film showing greater than normal film blackening from increased transmission of x-rays. See: unilateral hyperlucent lung.
What is horseshoe lung?
Horseshoe lung is a rare congenital malformation in which an isthmus of pulmonary parenchyma extends from the right lung base across the midline behind the pericardium and fuses with the base of the left lung.