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27 Cards in this Set

  • Front
  • Back
Left to Right Shunts
1. VSD --> Most common congenital cardiac abnormality. Most are small and close by themselves.
2. ASD -->
3. PDA --> Continuous Machine shop murmur.

Important in some early cyanotic HD (R-->L Shunts)
Eisenmenger's Syndrome
L-->R Shunt becomes a R-->L shunt.

Over time, the L-->R Shunt causes ↑ volume and pressure in pulmonary circulation, causes hypertrophy of sm. muscle in vessels and leads to 2° pulm. HTN. Once RV Pressure>LV Pressure, shunt switches and becomes R-->L. This is Eisenmenger's Syndrome.
Right to Left Shunts
5Ts
1. Tetralogy of Fallot
2. Transposition of Great Arteries
3. Truncus Arteriosus
4. Tricuspid Atresia
5. Total Anomalous Pulm. Venous Return (TAPVR)
Tetralogy of Fallot
R-->L Shunt
4 Defects
1. Pulm. Stenosis
2. RVH (due to Pulmonic stenosis)
3. VSD
4. Overriding Aorta (over VSD)

Caused by: anteroposterior displacement of infundibular septum

22.11q deletion syndrome.

Not as severe as Transposition of Great Arteries, but most common of R-->L Shunts
Transposition of Great Arteries
R-->L Shunt
RV --> Aorta
LV --> Pulm. Artery.
Venous Return is not altered. This leads to a complete separation of pulmonary and systemic circulation.

**ASD, VSD, or PDA required to be compatible with life**
Untreated babies die quickly.
Ass'd with diabetic mothers
Truncus Arteriosus
R-->L Shunt
1 overriding Great artery connects to both ventricles overriding VSD. Then the Great Vessel splits to Aorta and Pulm. Artery more distally. Leads to Pulm. Vasculature seeing increased BP --> Pulm. HTN

22.11 q deletion syndrome
Tricuspid Atresia
R --> L Shunt
Tricuspid valve fails to develop. RV normally hypoplastic or absent.
Embryological similarity for Truncus Arteriosus, Tetralogy of Fallot, and Transposition of Great Arteries
All due to defects in NEURAL CREST Cell Migration.
Total Anomalous Pulmonary Venous Return
R-->L Shunt
Pulmonary Veins Return to Somewhere other than LA (either RA or Aorta normally)
Coarctation of the Aorta
Infantile = Preductal
Adult = Postductal
**Pulses in LE much weaker than pulses in UE**
Ass'd with Turner's Syndrome
More Commonly Ass'd with Bicuspid Aortic Valve
Congenital Rubella Association with Congenital Heart Diseases
1. Septal Defects
2. PDA
3. Pulm. Artery Stenosis
Down's Syndrome Ass'n with Congenital Heart Disease
Endocardial Cushion Defects
ASD
VSD
AVSD
Osler-Weber-Rendu // Hereditary Hemorrhagic Telangectasia
Small Vessel Dz
Autosomal Dominant disorder
Dilated AV malformations throughout skin, mucus membranes, (kidneys?)
Normally present with NOSEBLEEDS and skin telangectasias (blanche when pressed).
Raynaud's Disease
Small Vessel Dz
Vasospasm of small vessels in distal extremities exacerbated by Stress or ↓ Temp.
Can be 1° (Raynaud's disease) or 2° due to a connective tissue disorder (CREST, MCTD, SLE, Scleroderma) [Raynaud's Phenomenon]
Wegener's Granulomatosis
C-ANCA Positive!!
Necrotizing Granulomatous Vasculitis
Triad:
1. Local Necrotizing Vasculitis
2. Necrotizing Granulomas of Lung & Upper Airway
3. Necrotizing Glomerulonephritis

Sx: EENT, Lung, Kidneys [ELK!]
Can cause perforation of nasal septum (not just cocaine use!)

Tx: Cyclophosphamide and Corticosteroids
Microscopic Polyangiitis
P-ANCA associated
Similar to Wegener's, but no Granulomas!
Churg-Strauss
p-ANCA Associated
Granulomatous inflammation of Skin, Lung, Kidney, Nerves, etc. with EOSINOPHILIA
Associated with patients with Atopic Dermatitis

Atopic Dermatitis is associated with allergies, i.e. EOSINOPHILIA. Make this connection to help this disease stick.
Sturge-Weber
PORT-WINE STAIN
AV Malformations in brain.

That's all you need to know. Oh yeah, and congenital.
Henoch Schonlein Purpura
MOST common vasculitis of children

Seen in Children AFTER URI

Associated with IgA complex deposition in vessels --> purpura and IgA deposition in kidneys --> IgA nephropathy.

Palpable purpura with common Triad of symptoms
1. Skin
2. Joints
3. GI
Small Vessel Diseases
Small Vessel Diseases
1. Osler-Weber-Rendu//HHT - Nosebleeds & Skin Lesions
2. Raynaud's - vasospasm in distal extremities. Cold & Stress
3. Vasculitises
a. Wegener's Granulomatosis - C-ANCA, Triad, ELK sx
b. Microscopic Polyangiitis - p-ANCA, no granulomas
c. Churg Strauss - p-ANCA, granulomas, Triad: Skin, Joints, GI
Buerger's Disease // Thromboangiitis Obliterans
SMOKERS, SMOKERS, SMOKERS!
Small/Medium Vessel Disease
PAINFUL, can cause gangrene and autoamputation
Tx: SMOKING CESSATION
Kawasaki's Disease
Infants & Children
Associated with Asians
Ass'd with CORONARY ANEURYSMS
Sx: Strawberry tongue & Fever
Polyarteritis Nodosa
Medium-Vessel Vasculitis
Affects primarily RENAL and other visceral arteries.
Ass'd with HEP B SeroPOSITIVITY

Sx: HTN, cutaneous eruptions of different ages, GI effects

Rads: Aneurysms and Narrowing on arteriogram "Beads on a string"

Tx: Cyclophosphamide & Corticosteroids
Small/Medium Artery Diseases
1. Buerger's//Thromboangiitis Obliterans - SMOKERS, Painful, gangrene, Tx: stop smoking
2. Kawasaki's dz - Asian Children, Fever & Strawberry Tongue, Coronary Aneurysms
3. Polyarteritis Nodosa - Type III Hypersensitivity, Hep B Seropositivity, Renal artery stenosis --> HTN, Beads on a string
Medium/Large Vessel Vasculitides
1. Takayasu's Arteritis - young ASIAN Females, Pulseless dz, inflamm of aortic arch/prox. great vessels, weak pulses in upper extremities
2. Giant Cell (Temporal) Arteritis - Granulomas, ↑ESR Sx: Jaw claudication, HA, IRREVERSIBLE BLINDNESS Tx: high dose Corticosteroids
Giant Cell (Temporal) Arteritis
M/L vessel vasculitis prim. branches of Carotid

Granulomatous inflammation

Sx: Unilateral HA, Jaw Claudication, & vision changes --> IRREVERSIBLE blindness

Labs: ↑ ESR

Tx: High Dose Corticosteroids
Takayasu's Arteritis
M/L vessel vasculitis of YOUNG (<40) ASIAN FEMALES.
Inflammation of aortic arch and proximal great vessels --> ↓ in pulse in UE --> name of "Pulseless disease"