CV System Abnormal Development Flashcards
CHD is the most common cardiac condition in childhood. What is the most frequent cause of CHD?
Unknown causes
What are left to right shunts?
Cyanosis several months or years after birth
Atrial Septal Defect
Ventricular Septal Defect
Patent (persistent) Ductus Arteriosus
What are right to left shunts?
Cyanosis early in postnatal life (O2 stats below 75%)
- Tetralogy of Fallot
- Transposition of the great arteries (TGA)
- Truncus arteriosus (persistent)
What does the foramen ovale usually serve as in the fetus?
R –> L shunt!
What is the physiology of ASD?
- Pulmonary blood flow 2-4X normal
- Only large ones lead to pulmonary hypertension that leads to cyanosis and heart failure
What are complications of ASD?
- RV failure
- Paradoxical embolism (& brain infarction or abscess)
Atrial septal defects (ASDs) are common congenital heart anomalies. What is the most common form of ASD?
Patent foramen ovale
VSDs account for 40% of congenital heart defects. Which of the following is responsible for most forms of VSDs?
Abnormal formation of the endocardial cushions and aorticopulmonary septum (these make up the membranous portion of the ventricular septum).
What does the reversed blood flow in PDA cause?
Cardiac enlargement and increased pulmonary vascularity.
What aortic arch does the ductus arteriosus develop from?
6th - left side
What maternal problem is related to patent ductus arteriosus (PDA)?
Maternal rubella (german measles) during early weeks of pregnancy
What are other defects caused by maternal rubella?
- Congenital cataracts
- Deafness
- Other congenital heart defects - pulmonary stenosis (PS), ventricular septal defect (VSD)
What type of murmur does PDA produce?
Machinery-like
What are clinical complications associated with PDA?
- Infective endocarditis common
- Pulmonary hypertension common
What are treatments for PDA?
Surgery
-Catheter placed occluder
Also give ibuprofen to pre-matures
After birth, the ductus arteriosus usually closes. If the ductus remains patent, what clinical sign or symptom is most diagnostic of a patent ductus?
Machinery-like murmur
What is Tetralogy is Fallot?
(1) Pulmonary stenosis
(2) Overriding (Large) aorta
(3) Right ventricular hypertrophy
(4) Ventricular septal defect
What might you observe in clinic with Tetralogy of Fallot?
- Murmur due to pulmonary stenosis
- Cyanosis (depending on size of defect it may be present early on)
What complications are associated with TF?
- Paradoxical embolism (brain abscess)
- Infective endocarditis
Which of the four developmental consequences of the tetralology of Fallot would be the last to occur?
Right ventricular hypertrophy
What clinical sign would be most obvious on examination of a patient with TF?
Cyanosis
TF is a cardiac malformation that involves during early development, which of the following septa?
Aorticopulmonary septum
What causes transposition of the great arteries?
Failure of normal spiraling of the aorticopulmonary septum. This means that the aorta comes off the right ventricle and the pulmonary trunk comes off the left ventricle.
When would TGA be compatible with life?
If an atrial and ventricular septal defect and an associated patent ductus arteriosus is present
In what groups is TGA common?
More common in males of diabetic mothers.
Transposition of the great arteries (TGA) is a cardiac malformation that involves during early development which septa?
Aorticopulmonary septum
What is Truncus Arterioles (Persistent)?
- Common aorticopulmonary outlet, receiving blood from both ventricles
- Due to failure of bulbar and truncal ridges (aorticopulmonary septum) to develop
- Cyanosis present
- Increased pulmonary blood flow that leads to irreversible pulmonary hypertension
A persistent or common truncus arteriosus is a cardiac malformation that involves during early development, which septa?
Aorticopulmonary septum
What three CV congenital defects involve bulbar and truncal ridges/aorticopulmonary septum?
- Common/Persistant Truncus
- Tetralogy of Fallot
- Transposition of the great arteries (TGA)
What is coarctation of the aorta?
Narrows aortic lumen infolding of the aorta opposite the closed ductus arteriosus (DA)
What are clinical signs of coarctation of the aorta?
- BP in arms increased
- BP in legs decreased
- Intercostal artery blood flow increased (NOTCHED RIBS)
- HTN in upper extremities
- Weak pulse & lower bp in lower extremities
What are complications associated with coarctation of the aorta?
- CHF
- Cerebrovascular accident
- Rupture
- Infection
How to best treat Coarctation of the aorta?
-Excision
-Bypass
[Experimental: balloon angioplasty]
What can infantile (5%) (preductal) form of coarctation of the aorta cause?
It is tubular hypoplasia of the aortic arch proximal to a patent ductus arteriosus
- Early CHF
- Patent ductus arteriosus
- Cyanosis, lower body
- Mortality increased
What causes aortic or pulmonary stenosis or atresia?
-Asymmetrical division or spiraling of the aorticopulmonary septum (bulbar and truncal ridges)
13 yr old boy has no femoral pulses, increased BP in upper extremities, enlarged intercostal vessels. What abnormality is suspected?
Postductal coarctation of the aorta
What is involved in aortic or pulmonary stenosis?
Aorticopulmonary septum
What congenital defects are associated with blue babies?
- Tetralogy of Fallot
- Transposition of great vessels
- Truncus arteriosus (persistent or common trunk)
What is DiGeorge Syndrome?
Deletion in chromosome 22q11 - involved with neural crest cells
- Tetralogy of Fallot
- Truncus arteriosus (Persistent)
What is down syndrome associated with?
Trisomy 21
- Atrial septal defect
- Ventricular septal defect
What is turner syndrome involved in?
45, XO
-Coarctation of the aorta
What is Marfan syndrome involved in?
Fibrillin-1 glycoprotein abnormality; FBN1 gene; chromosomes 15q21.1 and 5q23.31
What is heard upon auscultation with ASD?
Fixed split S2!!
-Pulmonary valve takes longer to close due to increased volume/right ventricular filling (widened fixed split)
