Congenital and Hereditary Diseases of the Thoracic and Cardiovascular Systems

What is cystic fibrosis

Hereditary disease caused by defective gene of chromosome 7 - secretion fo excessive viscous mucous by exocrine glands - thick mucus secreted by trachea and bronchi block airways (can lead to atelectasis)

What are significant signs and symptoms of Cystic fibrosis

poor growth or weight gain, greasy bulky stools. children sweat excessively, newborn obstruction

What is the radiographic appearance of cystic fibrosis

irregular thickening of lung markings - hyperinflation (large lung) - reticular pattern

What is this

Cystic Fibrosis

What is hyaline Membrane Disease (IRDS)

occurs in infants who are premature, have a diabetic mother, or are born c-section - evident within 6 hours after delivery - lack of surfactant and immature lungs

What is a main sign and symptom of hyaline membrane disease

cyanosis - grunting sounds when breathing

What is the radiographic appearance of hyaline membrane disease

under aeration - finely granular appearance of pulmonary parenchyma - air bronchogram required

What is this

Hyaline Membrane Disease

What are the 4 abnormalities of Tetralogy of Fallot

High ventricular septal defect - pulmonary stenosis - overriding of aortic orifice above ventricular defect - right ventricular hypertrophy

What are the main signs and symptoms of tetralogy of fallot

blueish colour skin, lack of alertness

What is the radiographic appearance of tetralogy of fallot

enlargement of right ventricle causing upward and lateral displacement of the apex of the heart - wooden shoe

What is this

tetralogy of fallot

What are septal defects? What are the 3 kinds?

left-to-right shunt most common - Atrial Septal, Ventricular Septal, and Patent Ductus Arteriosus

What is Atrial Septal

communication between the atria as result of patent foramen ovule - increased pulmonary blood flow and overloading of RV

What is ventricular septal

communication between the ventricles. Causes increased pulmonary venous return which overloads LA & LV

What is Patent Ductus Arteriosus

 shunts blood from pulmonary artery into systemic circulation during uterine life. Closes after birth normally. Blood from higher pressure aorta goes to pulmonary artery and overloads LA & LV

What are the signs and symptoms of septal defects

frequent respiratory infections, difficulty breathing, tiring when feeding, SOB, Skipped heartbeat

What is the radiographic appearance of Atrial septal defects

Because the left atrial pressure is higher that the RA, the blood shunts L to R causing enlargement RV, RA, pulmonary outflow tract - cardiomegaly and enlarged pulmonary outflow tract

WHat is the radiographic appearance of ventricular septal defect

Left ventricular pressure is higher that the pressure in the right ventricle > L to R shunt > ^pulmonary flow > ^ pulmonary venous return> overloading and enlargement of LA and LV - any blood directed to the RV immediately goes to the pulmonary artery therefore no overloading of RV so no enlargement on CXR - heart and pulmonary trunk enlarged

What is this

Atrial Septal Defect

What is this

Ventricular septal defect

What is dextrocardia

heart is on the right

What is this?

Dextrocardia