Cardiovascular- pathology Flashcards
Congenital heart diseases
- RIGHT-TO-LEFT SHUNTS
Early cyanosis—“blue babies.”
5 Ts:
- Truncus arteriosus (1 vessel)
- Transposition (2 switched vessels)
- Tricuspid atresia (3 = Tri)
- Tetralogy of Fallot (4 = Tetra)
- TAPVR (5 letters in the name)
Congenital heart diseases
- Persistent truncus arteriosus
Truncus arteriosus fails to divide into pulmonary trunk and aorta due to lack of aorticopulmonary septum formation; most patients have accompanying VSD.
Congenital heart diseases
- D-transposition of great vessels
Aorta leaves RV (anterior) and pulmonary trunk leaves LV (posterior). Not compatible with life unless a shunt is present to allow mixing of blood.
Not compatible with life unless a shunt is present to allow mixing of blood
Congenital heart diseases
- Tricuspid atresia
Absence of tricuspid valve and hypoplastic RV;
requires both ASD and VSD for viability.
Congenital heart diseases
- Tetralogy of Fallot
Caused by anterosuperior displacement of the
infundibular septum.
PROVe:
- Pulmonary infundibular stenosis (most important determinant for prognosis)
- Right ventricular hypertrophy (RVH)— boot‑shaped heart on CXR A
- Overriding aorta
- VSD
Most common cause of early childhood cyanosis.
Congenital heart diseases
- Total anomalous pulmonary venous return
Pulmonary veins drain into right heart circulation (SVC, coronary sinus, etc); associated with ASD and sometimes PDA to allow for right-to-left shunting to maintain CO.
Congenital heart diseases
- Ebstein anomaly
displacement of tricuspid valve leaflets downward into RV, artificially “atrializing” the ventricle.
Associated with tricuspid regurgitation, accessory conduction pathways, and right-sided HF. Can be caused by lithium exposure in utero.
Congenital heart diseases
- LEFT-TO-RIGHT SHUNTS
Ventricular septal defect
Atrial septal defect
Patent ductus arteriosus
*Acyanotic at presentation; cyanosis may occur
years later.
Congenital heart diseases
- Ventricular septal defect
Most common congenital cardiac defect.
Asymptomatic at birth, may manifest weeks
later or remain asymptomatic throughout life
Frequency: VSD > ASD > PDA.
Congenital heart diseases
- Atrial septal defect
wide, fixed split S2.
Ostium secundum defects most common and usually an isolated finding; ostium primum defects rarer and usually occur with other cardiac anomalies.
May lead to paradoxical emboli (systemic venous emboli use ASD to bypass lungs and become systemic arterial emboli).
Congenital heart diseases
- Patent ductus arteriosus
In fetal period, shunt is right to left (normal).
Associated with a continuous, “machine-like” murmur. Patency is maintained by PGE synthesis and low O2 tension.
can eventually result in late cyanosis in the lower extremities (differential cyanosis).
Congenital heart diseases
- Eisenmenger syndrome
Uncorrected left-to-right shunt, high pulmonary blood flow pathologic remodeling of vasculature pulmonary arterial hypertension. RVH occurs to
compensate shunt becomes right to left.
Causes late cyanosis, clubbing, and polycythemia.
Congenital heart diseases
- Coarctation of the aorta
Aortic narrowing near insertion of ductus arteriosus (“juxtaductal”).
Associated with bicuspid aortic valve, other heart defects, and Turner syndrome.
Hypertension in upper extremities and weak, delayed pulse in lower extremities (brachial-femoral delay). With age, intercostal arteries enlarge due to collateral circulation; arteries erode ribs and notched appearance on CXR
Congenital cardiac defect associations
- Alcohol exposure in utero (fetal alcohol syndrome)
- Congenital rubella
- Down syndrome
- Infant of diabetic mother
VSD, PDA, ASD, tetralogy of Fallot
PDA, pulmonary artery stenosis, septal defects
AV septal defect (endocardial cushion defect), VSD, ASD
Transposition of great vessels, VSD
Congenital cardiac defect associations
- Prenatal lithium exposure
- Turner syndrome
- Williams syndrome
- 22q11 syndromes
- Marfan syndrome
Ebstein anomaly
Bicuspid aortic valve, coarctation of aorta
Supravalvular aortic stenosis
Truncus arteriosus, tetralogy of Fallot
MVP, thoracic aortic aneurysm and dissection, aortic regurgitation
Hypertension
90% of hypertension is 1° (essential). Remaining 10% mostly 2° to renal/renovascular diseases such as fibromuscular dysplasia (characteristic “string of beads”
appearance of renal artery) and atherosclerotic renal artery stenosis or to 1° hyperaldosteronism.
Hypertensive urgency
Hypertensive emergency
severe (≥ 180/≥ 120 mm Hg) hypertension without acute end-organ damage.
severe hypertension with evidence of acute end-organ damage (eg, encephalopathy, stroke, retinal hemorrhages and exudates, papilledema, MI, HF, aortic dissection, kidney injury, microangiopathic hemolytic anemia, eclampsia).
