Abnormal CV Dev Flashcards
When do most congenital heart disease arise? and what from?
weeks 3-8
- Genetics- 9%
- Environment-1%
- Unknown- 90%
In terns of Congenital heart diseases what makes up the 9% genetic defects?
- Familial like Marfan 2-3
- Chromosomal defects (non-familial) 5%
What are the left to right shunts?
Atrial septal defect
Ventricular septal defect
Patent Ductus arteriosus
What are the symptoms of a left to right shunt?
Cyanosis (sats
What are the right to left shunts and its symptoms presentation?
Tetralogy of Fallot
Transposition of Great arteries
Truncus arteriosus
- Cyanosis early in postnatal life
What are the 2 anomalies that create most atrial septal defects?
- Excessive resorption of primary septum (Spider like appearance, Apoptosis)
- Incompetent foramen ovale due to hypoplastic growth of secondary septum
What are other ways to have a atrial septal defect?
- inadequate dev of the primary septum producing a basal opening
- Sinus venosus dev defect
What happens to the physiology in a ASD?
- Pulmonary flow 2-4x normal
- Large ones lead to pulmonary HTN and cyanosis tardive and cardiac failure
What are some complications of a ASD?
- Right ventricular failure
- Paradoxical embolism
How would one treat a ASD?
Surgery
- Secondary atrial septal percutaneous transvenous closure device
What do shunts do to the heart?
Normal pressure 1:6 Pulmonary:aorta
- increases right ventricular pressure and pulmonary artery pressure increase leading to pulmonary fibrosis
- Can lead to ventricular septal defects
What is the most common congenital heart defect?
Ventricular septal defect
(VSD)
- 40%
What is the most common cause of VSD?
Membranous portion of ventricular septum fails to close
- swiss- cheese septum
How does one fix a VSD?q
50% close spontaneously
- surgery
What other anomalies are VSD associated with?
- Pulmonary stenosis
- Tetraology of Fallow
- Coarctation of aorta
- Transposition of aorta
What does the ductus arteriosus derive from?
6th aortic arch
What is common flow of a patent ductus ateriosus after birth?
From aorta to pulmonary artery
- if reversed cause cardiac enlargement and increase pulmonary vascularity
- at day 30 DA should be a ligament
What is the most common cause of patent ductus arteriosus PDA?
Maternal rubella during early weeks of pregnancy
What are some other complications of maternal rubella?
- Cataracts
- Deafness
- Pulmonary stenosis and VSD
What does the murmur sound like and how is a PDA fixed?
Thrill
- Catheter occluder or Ibuprofen if premature
What are some clinical complications of a PDA?
Infective endocarditis
Pulmonary HTN
What are the defects in a Tetraology of Fallot?
- VSD
- Pulmonary stenosis
- Right ventricular hypertrophy
- Over ridding large aorta
What is clinical significant in T of F?
Cyanosis is size dependent
- murmur due to stenosis
What are some complications of T of F and how is it treated?
Paradoxical embolism
Infective endocarditis
- surgery
What is the cause of Transposition of the great arteries TGA?
Failure of normal spiraling of the aorticopulmonary septum (bulbotruncal septum)
Is TGA compatible with life and how is it fixed?
Yes if there is a VSD other no
- fixed via surgery
What other shunts are common in TGA?
ASD and PDA
What is the male to female ratio of TGA?
M:F 3:1
- offspring of a diabetic mother
What is Persistent Truncus arteriosus?
Aorta and Pulmonary trunk receive blood from both ventricles
- leads to irreversible Pulmonary HTN
- Cyanosis present
-
What is the cause of Persistent truncus arteriosus?
failure of bulbar and truncal ridges to to develop
What are the obstruction CHD?
- Coarctation of aorta
- pulmonary stenosis and atresia
- aortic stenosis and atresia
What is Coarctation of the aorta?
Narrowed aortic lumen
- Infolding of the aorta opposite the closed ductus arteriosus
What are the sx’s of coarctation?
- increase BP in arms, low in legs
- intercostal artery blood flow increased (notched ribs)
- Strong pulse in UE and decreased in LE
Is coartation more common in males or females? Postductal
males 2x
What the some complications of the a coartation Postducatal?
CHF
CVA
Rupture
Infection
How are coartations fixed?
Excision
Bypass
Ballon angioplasty
What causes pulmonary/aortic stenosis and atresia?
Asymmetrical division or spiraling of the aorticopulmonary septum
How often does a preductal coarctation of the aorta heppne?
5%
What is the cause of preductal coarctation?
Tubular hypoplasia of the aortic arch proximal to the PDA
What are some complications of a preductal coarctation?
CHF
PDA
Cyanosis, lower body
Mortality increased
What is diGeorge syndrome and what is its association?
Deletion of chromosome 22q11
- Tetralogy of Fallot
Truncus arteriosus (Persistent)
Down syndrome has association with what CHD?
ASD
VSD
Turner syndrome as ass with what CHD?
Coarctation of aorta
What is Marfan syndrome and what is its ass with (CHD)?
Aortic stenosis
- Fibrillin-1 glycoprotein abnormality, FBN1 gene
- Chromosomes 15q21.1 and 5q23.31
