Pathology of Congenital Heart Disease Flashcards
What are the three categories of congenital heart defects?
Left-to-right shunts, right-to-left shunts, obstructive/other anomalies
The aorta dilates in patients with patent ductus arteriosus. Why is this?
Shunting of blood into the pulmonary artery leads to increases blood returning to the right heart. This increases CO and leads to LV remodeling and dilation of the aorta as more blood is ejected.
This congenital anomaly is caused by obstruction at the pulmonary valve and results in RV hypertrophy.
Pulmonary stenosis and atresia
What complications are associated with ventricular septal defects?
RV hypertrophy, pulmonary hypertension and vascular disease, Eisenmenger syndrome
True/False. Patent foramen ovale is a type of atrial septa defect.
False - while a patent foramen ovale enables blood exchange through the atria, it is not a type of atrial septal defect
What is the most common category of congenital heart defects?
Left-to-right shunts
What is the clinical presentation of a patient with postductal coarctation?
Adolescent or adult with strong pulse and UE hypertension, but weak pulse and LE hypotension
DiGeorge syndrome is most associated with what congenital heart disorder?
Persistent Truncus Arteriosus
This congenital heart defect is characterized by narrowing of the aortic lumen, reducing blood flow to the body.
Coarctation of the aorta
In cases of atrial septal defect, S2 is split. Why is this?
Increased volume in the right ventricle increases RV systole and prolongs closure of the pulmonic valve
True/False. Left-to-right shunts present initially with cyanosis.
False. They present with no initial cyanosis. As patients decompensate, cyanosis may present with right-to-left shunting due to increased pulmonary pressure.
What heart changes are seen with Ebstein’s anonaly of the tricuspid valve?
Right atrium dilation and right ventricle hypoplasia due to tricuspid regurgitation
This congenital heart disorder is caused by failure of the pulmonary veins to join directly to the L atrium.
Total anomalous pulmonary venous return - blood is emptied into the RA or SVC
True/False. Right-to-left shunts present with initial cyanosis and hypoxemia.
True - severe cases may cause clubbing and polycythemia
Tetralogy of Fallow presents with cardinal features. What are they?
Pulmonary stenosis, right ventricle hypertrophy, overriding aorta, ventricular septal defect
This congenital heart defect is incompatible with life without surgical intervention.
Transposition of the Great Arteries
This congenital heart defect is due to incomplete closure of a fetal structure that allows blood from the aorta to enter the pulmonary artery.
Patent Ductus Arteriosus
Coarctation of the aorta is most associated with what chromosomal abnormality?
Turner Syndrome (45, X)
This congenital heart defect is caused by incomplete fusion of the superior and inferior endocardial cushions.
Atrioventricular septal defect
What is Eisenmenger Syndrome?
Complication of left-to-right shunts due to increased pulmonary blood flow and pressure that ultimately leads to an inverted right-to-left shunt and cyanosis.
This congenital heart defect is caused by complete occlusion or unequal division of the tricuspid valve.
Tricuspid atresia - there is little to no communication between the right atria and ventricle
This congenital heart defect is caused by the failure of the aorticopulmonary septum to develop normally.
Persistent truncus arteriosus - single artery for systemic, pulmonary, and coronary circulations
When and where will a murmur be heard in a patient with tetralogy of fallot?
Systolic ejection murmur at the left upper sternal border due to pulmonary stenosis
What is a ventricular septal defect?
Incomplete closure of the ventricular septum allowing blood flow between the ventricles
What is the clinical presentation of tetralogy of fallot?
Cyanosis, dyspnea, poor feeding, tet spells (cyanosis, tachycardia, and dyspnea after exercise)
This congenital heart defect presents with a continuous, machinery-like murmur at the upper left sternal border.
Patent ductus arteriosus
Nearly 1/3 of patients with atrioventricualr septal defect also present with this chromosomal abnormality.
Trisomy 21 - Down Syndrome
Hypoplastic left atrium is associated with what congenital heart disorder?
Total anomalous pulmonary venous return - blood is emptying into the RA or SVC. The LV is normal because it is still pumping blood to systemic circulation
This type of aortic coarctation presents in infancy and causes cyanosis in the lower half of the body.
Preductal coarctation
What are clinical symptoms of Eisenmenger syndrome?
Hypoxia, cyanosis, polycythemia (increased RBCs), digital clubbing, R heart failure
This is a congenital defect due to an opening in the atria septum that permits shunting of blood between the left and right atria.
Atrial septal defect
What is the most common type of atrial septal defect?
Ostium secundum
