Diseases Quiz 2 Flashcards
Left to right shunts
Blue kids (tardive cyanosis)
Atiral septal defect
Ventricular septal defect
Patent Ductus Arteriousus
Right to left shunts
Blue babies
Tetrology of Fallot
Transposition of the great arteries (TGA)
Truncus arteriosusu
Atrial Septal Defect (ASD)
L–>R = Acynotic
Abnormalities usually result following incorrect development of the atrial septum.
90% Patent Foramen Ovale (PFO):
Excessive resorption of primary septum (fenestrated oval fossa or large secondary foramen) or
Incompetent foramen ovale due to hypoplastic growth of the secondary septum (large foramen ovale)
5%: Inadequate development of the primary septum producing a basal opening known as a primary foramen defect (primum anomaly)
5%: Sinus venosus development defect w/ high atrial septal defect
In general left to right shunting of blood
Physiology:
- Pulmonary blood flow is 2-4x normal
- Only large ones lead to pulmonary HTN –> cyanosis tardive and cardiac failure
Complications:
- RV failure
- Paradoxical embolism (and brain infarction or abscess)
Rx: Surgery
Ventricular Septal Defect (VSD)
L–>R Acynotic
Most common congenital heart abnormality (40%)
Size variable; symptoms = size
Solitary: 90% involve membranous; muscular involvement may exhibit multiple sites (swiss-chees septum)
Combined w/ other abnormalitis:
- pulmonary stenosis
- tetrology of Fallot
- Coarctation of aorta
- Trasnsposition of aorta
Murmur/size: 50% close spontaneously –> cyanosis tardive in long standing/large cases
Rx: surgery
Patent (Persistent) Ductus Arteriosus (PDA):
L–>R Cyanosis
When patent the direction of blood flow is from the aorta to the PA. Reversed blood flow casues cardiac enlargement and increased pulmonary vascularity
Associated w/ maternal rubella during early weeks of pregnancy (along w/ congenital cataracts, deafness, and other congenital heart defects)
More common at high altitudes
Machinery like murmur/thrill
Clinical complicaitons:
- infective endocarditis
- pulmonary hypertension
Rx: catheter placed occluder or indomethacin (ibuprofen) in pre-matures
Tetrology of Fallot (TF)
R–>L = cyanotic
Defects:
- ) Ventricular septal defect
- ) Pulmonary stenosis
- ) Overriding (large) aorta
- ) Right ventricular hypertrophy (last to develop)
Clinical: Cyanosis dependent on size of ventricular septal defect; cyanosis may be absent early
Murmur due to pulmonary stenosis
Complications:
- paradoxical embolism (brain abscess)
- infective endocarditis
Rx: surgery
Transposition of the Great Arteries (TGA)
R–>L = cyanotic
Incompatible w/ life except when paired w/ PDA
Caused by a failure of the normal spiraling of the aorticopulmonary septum. As a result, aorta originates from the RV and the PA from the LV.
Shunts common
- atrial septal defect
- ventricular septal defect
- PDA
M/F = 3/1; malformation particularly common in offspring of type I diabetic mother.
Rx: early surgery
Truncus Arteriosus (persistent)
R –> L; cyanotic
A common aorticopulmonary outlet, receiving blood from both ventricles
Due to failure of bulbar and truncal ridges (aorticopulmonary septum to devlop
Sx: cyanosis and increased pulmonary blood flow and irreversible pulmonary HTN.
Coarctation of the Aorta
A cynotic
Characterized by a narrowed aortic lumen (obstructive congenital heart disease)- infolding of the aorta opposite the closed ductus arteriosus
Postductal: (adult form) distal to DA
-(95%); M/F = 2/1
BP arms increased; legs decreased
Intercostal artery blood flow increased (notched ribs)
Complications: CHF, CVA, rupture, infeciton
Rx: Excision, bypass.
Preductal: (infantile form 5%) proximal to PDA
- Early CHF
- PDA
- Cyanosis, lower body
- Mortality increased
Aortic or pulmonary stesnosis and atresia
Due to asymmetrical division of spiraling of the aorticopulmonary septum.
Varicose veins
Abnormally enlarged and tortuous veins
Usually affect superficial veins in legs of older individuals
Due to loss of muscle tone; wall degeneration; valvular incompetence
Other common locations: lower esophagus (esophageal varices); terminus of anal canal (hemorrhoids)
Familial Hypercholestemia (aka hyperlipidemia type IIa)
Autosomal Dominant
Homozygotes have severe cardiovascular disease:
cholesterol between 500-1,000 mg/dL (13-25.9 mmol/L), LDL-C > 600 mg/dL (15.5 mmol/L)
heterozygotes have Less severe problems:
total cholesterol between 310-550 mg/dL (9-14 mmol/L)
low-density lipoprotein cholesterol (LDL-C) between 190-400 mg/dL (5-10 mmol/L)
normal triglycerides
Pathophys: LDLR receptor (ATM Machine) gene is mutated OR
APOB (apolipoprotein B) gene mutated OR
pro-protein convertase subtilisin/kexin 9 (PCSK9) gene mutated
Prizmenthal Angina
Vasospasm in a coronary artery, which leads to ischemia and chest pain. It typically occurs at rest and in cycles and can occur in a diseased artery or a healthy one.
It can be seen on an EKG as ST segment elevation.
Contraction Band Necrosis
Reperfusion of irreversibly damaged cells results in calcium influx, leading to hypercontraction of myofibrils
Repurfusion injury
Return of oxygen and inflammatory cells may lead to free radical generation → further damaging mycocytes
