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Block 2 PATH Exam -- Congenital Heart Disease Lecture


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Congenital Heart Defects more common in MALES
Aortic coarctation
Aortic Stenosis
Congenital Heart Defects more common in FEMALES
Maternal diseases related to congenital heart disease
Poorly controlled diabetes
Lupus erythemaosus
Maternal medications related to congenital heart disease
Maternal infections related to congenital heart disease
Heart Defects with Normal Sequence and Position with SHUNTING
The Four D's:
asD; vsD; asvD; pDa

Overall: L-->R shunt; Acyanotic; Inc. PBF
Accounts for 2/3 of heart disease in Down Syndrome pts.
Complete ASVD
Heart Defects with Normal Sequence and Position with OBSTRUCTION
Aortic Stenosis/Atresia
Pulmonic Stenosis/Atresia
Aortic Coarctation
Hypoplastic Left Heart Syndrome
Associated with Williams Syndrome
Supravalvar AORTIC stenosis (also assoc. with accel. atherosclerosis)
Supravalvar PULMONIC stenosis (also assoc. with rubella)
Associated with Noonan syndrome
Valvar PULMONIC stenosis
Hypoplastic Left Heart Syndrome (HLHS)
Underdevelopment of the entire left side of the heart
RV hypertrophies, providing pulmonary and systemic flow
Pulmonary return flows through patent foramen ovale
Systemic flow is dependent upon PDA
Coronary flow is retrograde
Initial maintenance of PDA through PG therapy; reconstruction --> transplant
Heart Defects with Normal Sequence of Anomalous Valves
Ebstein Anomaly
Ebstein Anomaly
Abnormal attachments of the tricuspid valve leaflets
Downward displacement of the tricuspid valve
RV divided into atrialized and muscular portions
90% associated with ASD of patent foramen ovale; 85% die in utero
ASD with right to left shunting often complicates
Surgical Repair: Valvoplasty and creation of systemic-->pulmonary shunt
Heart Defects with DISCORDANCE
The Four T's:
Tetralogy of Fallot
Transposition of Great Arteries
Truncus Arteriosus
Total Anomalous Pulmonary Venous Return (TAPVR)
Pulmonary veins return to systemic venous circulation or RA directly
Often accompanied by venous obstruction
Associated ASD or patent foramen ovale helps maintain LV output
Severe obstruction --> pulmonary hypertension
No obstruction acts like large ASD with right heart failure
Transposition of Great Arteries (TGA)
Aorta arising from the RV; Pulm artery arising from the LV
Circulatory system is in PARALLEL instead of in series
Oxygenation depends upon ASD, VSD, or PDA
Aorta is anterior and to the right of the pulmonary artery
A-V concordance --> PARALLEL circulation
Aorta is anterior and to the left of the pulmonary artery
A-V discordance --> SERIES circulation ("corrected" transposition)
Truncus Arteriosus
Single arterial trunk arises from a single semi-lunar valve
Results from incomplete or failed septation
Truncal vessel typically overrides a VSD
Ductus Arteriosus is usually absent
Repair: closing VSD; committing truncus to the left; reconstructing right outflow
Tetralogy of Fallot
4 components: VSD; Pulm stenosis; Overriding aorta; RV hypertrophy
Pulmonary valve is usually abnormal
Repair: closure of VSD; widening of right outflow; repairing pulm valve
"Pink tet"
TOF that is NOT associated with cyanosis
"Tet" spells
Paroxysmal hypercyanotic spells
Increased pumonary stenosis murmur
Thought to be secondary to spasm of RV infunibulum
Therapy: bring baby's knees to its chest
Decreases pulmonary return and increases systemic resistance

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