L26- CVS Pathology V Flashcards
list the 8 common congenital heart diseases
- tetrology of fallot
- transposition of great arteries (or vessels)
- truncus arteriosus
- VSD
- ASD
- PDA
- coarctation of aorta
- cardiac neoplasm
Turner Syndrome is associated with (1) congenital heart disease
Trisomy 21 (Down syndrome) is associated with (2)
Rubella is the main environmental factor to cause congenital heart disease, associated with (3)
1- coartication of aorta
2- AV canal, ASD, VSD
3- PDA
______ is by far the most common congenital heart disease
VSD (42% of all, ASD is 2nd at 10%)
list the R to L shunts in utero
foramen ovale: RA –> LA
ductus arteriosus: pulmonary trunk –> aorta
describe the transition from fetal to adult circulation
- at birth, baby’s breathing inflate lungs –> dec pulmonary resistance
- pressure change closes flap valve of foramen ovale
- ductus arteriosus (15 hrs of life): PGs (via placenta) keep it open –> at birth PGs are metabolized in lung –> reduction via dec production and inc breakdown => closes DA
what are the categories of congenital heart malformations
- R-to-L shunts (cyanotic at birth or later)
- L-to-R shunts
- obstruction
describe pulmonary vascularity in relation to congenital heart disease
Dx clue:
-inc lung vascularity indicates L –> R shunt (plethoric lung fields)
-dec lung vascularity indicates R –> L shunt (oligemic lung fields)
what are the R-to-L shunt malformations seen at birth
(cyanosis at birth)- 5 T’s
- Tetrology of Fallot
- Transposition of the Great Vessels
- Truncus Arteriosus
- Tricuspid Atresia
- Total Anomalous Pulmonary Venous Connection
what are the R-to-L shunt malformations seen well after birth (mos-yrs)
(cyanosis later in life)- 3 D’s
- VSD
- ASD
- PDA
list the 4 features of Tetrology of Fallot
- VSD
- pulmonary stenosis (infundibular stenosis)
- overriding aorta
- RVH
the prognosis of Tetralogy of Fallot is dependent on…..
degree of pulmonary stenosis
-if severe, cyanosis is evident immediately
Tetrology of Fallot usually presents at (1), its timing is dependent on (2). It is often associated with (3) disorder.
1- w/in 6 mos
2- degree of severity of pulmonary stenosis
3- Down syndrome
- (1) is one of the hallmark symptoms of ToF as it causes (2)
- (3) is evident with increased activity
- (4) is evident on labs and can possibly cause (5)
- ToF is a (6) type shunt, therefore there is an increased risk of (7)
1/2- squatting- inc PVR / pressure in aorta –> pushes blood into pulmonary circulation
3- dyspnea
4/5- polycythemia inc risk of cerebral thrombosis
6/7- R-to-L, inc risk of infective endocarditis and systemic embolization
Transposition of the Great Arteries is defined as (1) and is only compatible with life if (2) is present. The dominant clinical finding is (3). The prognosis is dependent on (4).
1- aorta from RV, pulmonary trunk from LV
2- VSD, PDA
3- cyanosis
4- degree of mixing => tissue hypoxia /// ability of RV to maintain systemic circulation
Truncus Arteriosus is defined as (1), accompanied with (2) necessary for compatibility with life. The main blood flow abnormality is (3), possibly causing (4).
1- failure of partitioning of embryonic truncus (supposed to => aorta + pulm. trunk)
2- VSD (=> one ventricular chamber with mixed blood)
3- over-perfusion to lungs
4- pulmonary HTN
(1) is the most common heart lesion. It is associated with (2). It mostly comes in the (3) form/type. Most will progress to (4) in childhood.
1- VSD
2- Trisomy 13, 18, 21
3- membranous (90%- 1-% muscular)
4- closing spontaneous (if small enough)
The clinical features of VSD are (1), until (2) occurs, then (3) is evident.
1- pulmonary HTN, CHF, pansystolic murmur
2- shunt reversal (L-to-R switches to R-to-L)
3- cyanosis (Eisenmenger Complex)
ASD is defined as (1) due to (2). It is commonly diagnosed at (3) and presents with (4) symptoms. (5) is also a key sign if ASD is significant enough.
1- abnormal opening in atrial septum
2- incomplete tissue formation
3- any age (most common malformation diagnosed in adulthood)
4- usually asymptomatic until adulthood
5- pulmonary vascularity (inc indicates L-to-R shunt)
describe the types of ASDs
Ostium Secundum (90%)- failure to grow to septum primum
Ostium Primum (5%)- failure to fuse to endocardial cushions => AV valve insufficiency / ASD + VSD
Sinus Venous (5%)
Ductus Arteriosus is opened and maintained due to (1) which is produced by (2) and upon birth is broken down in (3). In a PDA, (4) is used to close it.
1- PG-E (sometime administered after birth if it needs to stay open due to other anomalies)
2- placenta
3- lungs (in addition to high Oxygen tension)
4- indomethacin (NSAID)
PDA is found with other anomalies (1)% of the time. It is more common in (males/females). It is evident on auscultation by (3). It will only present with (4) once (5) occurs.
1- 10% (90% isolated anomaly) 2- females 3- 'machine-like' murmur 4- cyanosis 5- shunt reversal (L-to-R --> R-to-L)
PDA has a high incidence with (1) during gestation. It is also associated with (2) on blood work.
1- rubella infection
2- polycythemia
what are the malformations that cause obstruction
- coarctation (narrowing) of the aorta
- pulmonary stenosis and atresia
- aortic stenosis and atresia (hypoplastic L heart syndrome)
(1) is the narrowing of the aortic lumen, It is either an isolated anomaly is is accompanied with (2). It comes in (3) or (4) types [briefly explain]. It is also associated with (5) disorder.
1- coarctation of the aorta
2- (50% are isolated) bicuspid aortic valve (sometimes PDA, ASD, VSD)
3- Preductal (infantile), PDA must be present
4- Postductal (adult), ductus arteriosus has closed
5- Turner’s Syndrome
Preductal coarctation of the aorta presents during (1) age with (2), (3), and (4) as the key symptoms.
(PDA is present)
1- infancy
2- CHF
3- selective cyanosis of lower extremities due to PDA
4- weak femoral pulses compared to upper extremities
Postductal coarctation of the aorta present during (1) age. (2) will not be present in comparison to preductal type. (3), (4), (5), and (6) are the key signs and symptoms.
(no PDA)
1- older childhood / adulthood
2- cyanosis
3- HTN in upper extremities
4- low BP and weak pulses in lower extremities
5- rib notching (due to collaterals)
6- intermittent claudication (leg cramping due to arterial insufficiency)
rib notching is a key sign of (1) and develop because of (2)
1- Postductal Coarctation of the Aorta
2- collateral blood supply formation
Tumors of the heart are usually (primary/secondary) in nature. They will be (2) until (3) occurs and (4) will be the important associated tumors.
1- secondary / metastatic (primary is rare)
2- silent
3- function is impaired
4- sudden onset of severe rapidly progressive HF w/o apparent cause and or arrhythmia
(1) is the most common primary cardiac tumor that favors (males/females) in the (3) age group. It is often diagnosed via (4).
1- cardiac myoxma
2- females
3- 30-60 y/o
4- calcification seen of X-Ray
Cardiac Myxoma often presents as a (1) on gross inspection affecting the (2) most often. On histological inspection, (3) cells will be evident, surrounded by (4).
1- lobulated pedunculated mass/lesion
2- LA (fossa ovalis) –> extending in mitral orifice
3- multinucleated stellate cells
4- suspended in edematous mucopolysaccharide rich stroma
Cardiac Myxoma will present with (1) symptoms. (2) and (3) are the major risks / complications seen with these tumors.
1- asymptomatic
2- fragmentation and embolization
3- ball-valve obstruction of AV valve (if it extends to AV valve) => syncopal episodes, death
what are the common secondary heart tumors
(metastasis)
- direct extension via lung CA
- breast CA
- lymphoma
- malignant melanoma
