Chest

Question 1

Scimitar Syndrome

Answer 1

Hypoplastic lung + PAPVR - almost exclusively on right

Drain into IVC (most common), also RA or portal vein

Essentially a L to R shunt

Associations
Congenital heart disease, e.g. ASD, VSD, tetralogy of Fallot, patent ductus arteriosus
Ipsilateral diaphragmatic anomalies, e.g. accessory diaphragm, diaphragmatic hernia
Localised bronchiectasis
Horseshoe lung
Vertebral anomalies, e.g. hemivertebrae 
Genitourinary tract abnormalities 
Pulmonary sequestration

Question 2

Coronary artery aneurysm common causes (adults vs paeds)

Answer 2

Adults - 50% due to atherosclerotic disease

Paeds - Kawasaki disease most common cause

Question 3

Dilated cardiomyopathy

Answer 3

Progressive cardiac dilation and systolic dysfunction, usually with concomitant hypertrophy. Mostly familial.

Can be primary or secondary.

Question 4

Dilated cardiomyopathy causes

Answer 4

Causes
Genetic - familial in up to 50% cases - Autosomal dominant inheritance mostly
Myocarditis
Alcohol and other toxins
Childbirth - peripartum cardiomyopathy. Can occur up to 5 months post partum
Iron overload - hemochromatosis or from multiple transfusions
Supraphysiologic stress - persistent tachycardia, hyperthyroidism. Takotsubo cardiomyopathy

Ischemic
Valvular
Congenital causes
Hypertensive

Question 5

Hypertrophic Cardiomyopathy

Answer 5

Clinically heterogenous, genetic disorder characterized by myocardial hypertrophy, poor compliant LV myocardium leading to abnormal diastolic filling, and (in about 1/3 cases) intermittent ventricular outflow obstruction.

Leading cause of LVH. Causes mainly diastolic dysfunction. Systolic function usually preserved

HCM most commonly caused by mutations in any one of several genes that encode sarcomeric proteins

Question 6

Hypertrophic cardiomyopathy morphology

Answer 6

Massive myocardial hypertrophy without ventricular dilation.

Usually asymmetric septal hyperytrophy. 10% of cases is concentric and symmetrical

Question 7

Restrictive cardiomyopathy

Answer 7

Decrease in ventricular compliance, resulting in impaired ventricular diastolic filling.
Systolic function of LV usually not affected.
Can be idiopathicor associated with amyloidosis, sarcoidosis, radiation induced fibrosis, metastatic tumors or accumulation of metabolites from inborn errors of metabolism

Question 8

Common cardiac tumors in descending order

Answer 8

Myxomas (most common)
Fibroma
Lipoma
Papillary fibroelastoma
Rhabdomyomas

These 5 are benign and accounts for 90% of all heart tumors