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Keutel syndrome (KS) is a rare autosomal recessive genetic disorder characterized by abnormal diffuse cartilage calcification, hypoplasia of the mid-face, peripheral pulmonary stenosis, hearing loss, short distal phalanges (tips) of the fingers and mild mental retardation.
The right ventricle is more muscular than normal, causing a characteristic boot-shaped appearance as seen by chest X-ray. This enlargement is generally a secondary condition, resulting from increased pressure. Pulmonary valve defects resulting in tricuspid regurgitation, a common effect of pulmonary stenosis, can cause this increase in muscle mass.
It can occur at the pulmonary valve (valvular stenosis) or just below the pulmonary valve (infundibular stenosis). [4] Infundibular pulmonic stenosis is mostly caused by the overgrowth of the heart muscle wall (hypertrophy of the septoparietal trabeculae), [ 41 ] however, the events leading to the formation of the overriding aorta are also ...
Pulmonary artery stenosis (PAS) is a narrowing of the pulmonary artery.The pulmonary artery is a blood vessel moving blood from the right side of the heart to the lungs. . This narrowing can be due to many causes, including infection during pregnancy, a congenital heart defect, a problem with blood clotting in childhood or early adulthood, or a genetic ch
The Blalock–Thomas–Taussig shunt (BTT shunt), [1] previously known as the Blalock–Taussig Shunt (BT shunt), [2] is a surgical procedure used to increase blood flow to the lungs in some forms of congenital heart disease [3] such as pulmonary atresia and tetralogy of Fallot, which are common causes of blue baby syndrome. [3]
Congenital cardiac anomalies including pulmonary artery stenosis and patent ductus arteriosus can be seen in infants with CRS. Infants should undergo cardiac evaluation soon after birth and those with confirmed cardiac lesions will require specialized care with a pediatric cardiologist for any interventions and follow-up care. [4]
When pulmonic stenosis (PS) is present, resistance to blood flow causes right ventricular hypertrophy. If right ventricular failure develops, right atrial pressure will increase, and this may result in a persistent opening of the foramen ovale, shunting of unoxygenated blood from the right atrium into the left atrium, and systemic cyanosis.
Pulmonary valve stenosis (PVS) is a heart valve disorder. Blood going from the heart to the lungs goes through the pulmonary valve, whose purpose is to prevent blood from flowing back to the heart. In pulmonary valve stenosis this opening is too narrow, leading to a reduction of flow of blood to the lungs. [1] [5]