04b: Abnormal Heart Development Flashcards
Atrial septal defects are caused by problems with tissue (formation/breakdown). What are the types of ASDs?
Both;
- Ostium secundum (75%)
- Ostium primium (20%)
- Sinus venosus (5%)
Pathophysiologic consequences of ASDs typically begin in (fetal period/childhood/adulthood). List some of these symptoms.
Adulthood;
- Arrhythmia
- Paradoxical embolism
- Pulmonary hypertension
- RV failure
T/F: Patent foramen ovale is a type of ASD.
False
In the most common, (X), type of ASD, the size of (Y) is insufficient to cover entire fossa ovale and/or (Z) fails to fully form.
X = ostium secundum Y = septum primium Z = septum secundum
Ostium secundum defect is typically found in (dorsal/ventral/superior/inferior) portion of atrial septum.
Middle/center
The clinical course of patients diagnosed with isolated ASD ostium secundum defect is often how severe?
Benign; may be associated with few functional limitations
A larger, more severe ostium secundum defect may lead to (X) due to persistent (Y).
X = RA volume overload; Y = L to R shunting
ASD ostium primum defect is (more/less) common and (more/less) severe than ostium secundum defect.
Less common; more severe
ASD ostium primum defect results in (L/R) to (L/R) shunting between (X) chambers due to defective septation between which surfaces of (Y) and (Z)?
L to R;
X = atria
Y = inferior margin of septum primum
Z = atrial surface of endocardial cushion
Severe ASD ostium primum defect will manifest as (X) in which stage of life?
X = heart failure
First few months
ASD sinus venosus defect is found in (upper/middle/lower) portion of (X) septum, near (Y) vessel opening.
Upper;
X = atrial
Y = SVC
T/F: ASD sinus venosus defect has the most poor prognosis of the ASDs.
False - excellent prognosis with surgical repair
ASD sinus venosus defect: the (R/L) (X) vessel may faultily drain into which chamber?
R;
X = pulmonary vein
RA
T/F: Presence of patent foramen ovale doesn’t always imply active interatrial communication.
True
List some conditions in which patent foramen ovale unsealed flap may open.
Higher R side pressure:
Sustained pulmonary HT, sneezing, coughing
Most common form of congenital cardiac anomaly is (X), which is usually associated with other cardiac anomalies.
X = Ventricular Septal Defect
Most VSDs are related to problems with (formation/breakdown) of (muscular/membranous) part of IV septum.
Incomplete or only partial formation;
Membranous
Most VSDs are typically the size of (X) orifice.
X = aortic valve
Large and severe VSDs create (X) shunt, leading to (Y) and (Z) symptoms.
X = L to R Y = RV hypertrophy Z = pulmonary hypertension
In persistent truncus arteriosus defect, there’s essentially a common trunk of origin for which vessels?
- Aorta
- Pulmonary arteries
- Coronary arteries
Persistent truncus arteriosus defect results from incomplete partitioning of (X) by (Y).
X = truncus arteriosus Y = spiral septum
T/F: Persistent truncus arteriosus defect always accompanied by IV septal defect.
True
A Persistent truncus arteriosus receives blood from which chamber(s)?
Both ventricles
A Persistent truncus arteriosus defect presents with (increased/decreased) pulmonary blood flow, pulmonary (hypo/hyper)-tension, and (X).
Increase;
hypertension;
X = recurrent respiratory infections
ASD ostium secundum caused by (incomplete formation/breakdown) of:
- Incomplete formation of septum secundum OR
2. Excessive cell death/resorption of septum primum
(X) defect may result in anomalous pulmonary venous drainage into RA or SVC.
X = ASD (sinus venosus)
An incomplete seal of foramen ovale occurs in about (X)% of adults.
X = 25
List the categories of VSDs, depending on various sizes/locations.
- Small hole in membranous septum
- Large hole involving more than membranous septum
- Defect only in muscular portion
- Complete absence of muscular septum
A persistence truncus arteriosus is ALWAYS associated with which two features?
- VSD
2. Mixing of LV and RV blood
List the four changes that define tetralogy of Fallot.
- Pulmonary stenosis
- VSD
- Overriding aorta
- RV hypertrophy
(X) is the most common R to L congenital heart disease.
X = tetralogy of Fallot
The (ASD/VSD) associated with tetralogy of Fallot is incomplete closure of (X), affecting (Y) septum as well as (Z).
VSD;
X = membranous septum
Y = muscular septum
Z = endocardial cushions
T/F: In tetralogy of Fallot, the development of spiral septum is abnormal.
True
In tetralogy of Fallot, there is a (R/L) displacement of (X) vessel.
R;
X = aorta
Clinical consequences of tetralogy of Fallot depend primarily on severity of:
Pulmonary stenosis
A (R/L) to (R/L) shunt produces cyanosis.
R to L
Transposition of great vessels: aorta is (anterior/posterior) and (R/L) of pulmonary artery and arises from (X) chamber.
Anterior;
R;
X = RV
(X) congenital heart defect, if left untreated, is the leading cause of death in neonates and infants.
X = transposition of great vessels
(X) heart defect is actually incompatible with life in absence of accompanying (Y) septal defects.
X = transposition of great vessels; Y = interarterial, interventricular, or patent ductus arteriosus
List the types of congenital aortic stenosis, which are present (alone/in combination). Star the most common type.
- Valvular*
- Subvalvular
- Supravalvular
Either
In valvular aortic stenosis, the valve cusps may have which problematic characteristics?
May be: hypoplastic, dysplastic or bicuspid
Valvular aortic stenosis is due to abnormal development of which key embryological structures?
Endocardial cushions in outflow tract
T/F: Valvular aortic stenosis may be asymptomatic in kids.
True
Subvalvular aortic stenosis is due to abnormal development of which key embryological structures?
Band of subvalvular fibroelastic tissue or muscular ridge (below valve)
Subvalvular aortic stenosis results in (X) of the aortic cusps.
X = thickening or immobility
What would the surgical intervention to correct subvalvular aortic stenosis involve?
Removal of fibrous ring (immediately below aortic valve)
Supravalvular aortic stenosis is (more/less) common than subvalvular stenosis. Its defining feature is (X).
Less (rarest);
X = focal narrowing above aortic valve (and involving ascending aorta)
What would the surgical intervention to correct supravalvular aortic stenosis involve?
Enlargement of narrowed region
Atrial septal defects occur in various locations and are described based on
relationships to the (X) and (Y) septum.
X = atria Y = AV
