complex and acquired anomalies

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Coronary fistulas – Most common connections and/or drainage sites (in order of frequency) are:

RV, RA, PA, CS, LV, & SVC (least common)

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Coronary Fistulas occur rarely and involve the:

RCA 50% LCA 42% Both 5%

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4 Complex Congenital Anomalies:

• Double Outlet Right Ventricle • Tetralogy of Fallot • Transposition of the Great Vessels (complete) • Congenitally Corrected TGV

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4 different DORV VSD’s:

a) Subaortic – below Aorta b) Subpulmonary – below PA c) Doubly committed – (large to feed both) d) Remote (noncommitted) – distant from vessels (down more toward the apex)

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DORV - 4 great vessel orientations:

• Aorta posterior to the PA (Normal, other than coming from RV) • Aorta right of PA with valves side by side • Aorta to the right and anterior to PA (transposed) • Aorta to the left and anterior to PA

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2 DORV problems:

• DORV children without pulmonary stenosis will have increased pulmonary blood flow and therefore suffer from CHF • DORV children with RVOT obstruction (pulmonary stenosis) will have diminished pulmonary blood flow and be cyanotic

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3 steps in DORV Echo Exam:

1. Utilize all appropriate views 2. Determine placement and size of VSD and great vessel relationship 3. Use 2D, color, and Doppler to evaluate VSD and determine whether PS or AS are present

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DORV Treatment – Surgery to establish 3 goals:

1. Establish left ventricle to aorta continuity 2. Establish right ventricle to pulmonary artery continuity 3. Repair associated defects (i.e. PS & VSD)

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Most common malformation in children born with cyanotic heart disease:

Tetralogy of Fallot

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Tetralogy of Fallot consists of 4 defects:

1. Malalignment VSD 2. PS (commonly infundibular) 3. Large overriding aorta 4. RV hypertrophy (seen after birth)

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3 criteria determining severity of tetralogy of Fallot:

1. Size of VSD 2. Degree of PS 3. Position of Aorta

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With tetralogy of Fallot, RVH and PS cause VSD to shunt right to left causing 3 things:

1. Decreased O2 saturation 2. Cyanosis 3. Reduced pulmonary flow

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Tetralogy of Fallot Echo Exam, 2 steps:

1. Utilize all views that visualize IVS, PV, Ao, & RV (Parasternal good view!) 2. Use 2D/color/Doppler to evaluate VSD, PS, over-riding Ao, and RVH

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Transposition of the Great Vessels – Two types:

1. Complete transposition 2. Congenitally corrected

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Complete transposition: 1. __% with transposition have complete 2. ______-looping (__-looping) occurs during development causing great arteries to be transposed

1. 80% 2. Dextro-looping (d-looping)

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Congenitally Corrected Transposition of the Great Vessels: __% of transposition consists of this type

20%

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Congenitally Corrected TGV: 1. ____-looping (__-looping) 2. Morphologic LV is on the ____and connects to pulmonary artery 3. Morphologic RV is on the ____and connects to Aorta 4. TV on ____ w/RV; MV on ____ w/ LV 5. Atria are in ______position

1. Levo-looping (L-looping) 2. right 3. left 4. left, right 5. normal

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Congenitally Corrected TGV Circulation: 1. Pulmonary venous flow returns to __, then anatomic __, then body 2. Systemic flow returns to __, then anatomic __, then ____, then __ 3. **_____ _____ blood is circulating through body!

1. LA, RV 2. RA, LV, then lungs, then LA 3. **Appropriate oxygenated blood is circulating through body!

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Congenitally Corrected TGV Circulation Treatment:

If no other defects present, no treatment needed!!

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2 types of Coronary Artery Anomalies:

1. Abnormal origins 2. Abnormal connections or fistulas

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2 Abnormal origins resulting in myocardial ischemia:

1. coronary arteries arise from RV where there is deoxygenated blood 2. LCA arising from the pulmonary artery

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Coronary Fistulas occur rarely and involve the: • ___ __% • ___ __% • ___ __%

• RCA 50% • LCA 42% • Both 5%

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Coronary Fistulas are seen and discovered mostly by _______

angiography

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Most Coronary Fistulas are benign without problems, but some can cause these 4 problems:

• myocardial ischemia • ventricular dysfunction • arrhythmias • sudden death

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Venous Malformations – Two common types:

1. Anomalous Pulmonary Veins 2. Persistent Left Superior Vena Cava

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Anomalous Pulmonary Veins – 4 connections:

1. directly to the RA 2. SVC, then drain to RA 3. coronary sinus, then drain to RA 4. Connect posterior to portal venous system, then drain to the RA

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Anomalous Pulmonary Veins can present _____ or _____

partial or complete

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Anomalous Pulmonary Veins – Treatment:

Surgery! • Once born, PFO & PDA must remain open for mixing of blood! This is vital to survival • Once stabilized, surgery is performed

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Persistent Left Superior Vena Cava: 1. During development of sinus venosus, the _____ sinus horn is normally obliterated 2. Rarely, _______ drainage into the _____ sinus horn remains and forms a persistent LSVC

1. left 2. cephalad, left sinus horn

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Persistent Left Superior Vena Cava Echo Findings:

DILATED CORONARY SINUS. Contrast can be injected to see if it is seen in the coronary sinus before the right atrium. This will confirm the presence of this disorder

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__% of children have innocent murmurs sometime during childhood

50%

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Timing of murmurs - 3 types:

o *Systolic o *Diastolic o *Continuous

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3 Systolic Functional Murmurs:

1. “Still’s” (due to false tendon) 2. Tricuspid regurgitation (80% found in normal patients) 3. Subclavian bruit

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5 causes of Pathological Systolic Murmurs:

1. VSD 2. AS 3. PS 4. ASD 5. Coarctation

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Diastolic Murmurs – Functional cause is:

Venous Hum (caused by turbulence in jugular venous flow)

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Diastolic Murmurs – 3 Pathological causes:

1. PDA 2. AV malformation 3. Ruptured Sinus of Valsalva

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Two Types of CHF:

1.) “Backward Failure” or Hypodynamic • Low CO d/t obstruction or myocardial disease • LESS common with newborns 2.) “Forward Failure” or Hyperdynamic • Very high CO d/t shunts, valve regurge, or peripheral vasodilation • MORE common with newborns

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CHF is characterized by 3 things:

1. Tachycardia 2. Tachypnea 3. poor feeding (d/t greatest exertion of newborns being feeding)

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Leading cause of acquired heart disease in children and young adults:

Rheumatic Fever

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Rheumatic Fever Affects 4 structures:

1. AV valves-usually mitral 2. Semilunar valves-usually Aortic 3. Myocardium 4. Pericardium

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Acutely, Rheumatic Fever damage to valves can cause 4 problems:

1. Regurgitation – possible valve replacement 2. Possible myocardial damage 3. Pericardial inflammation with PE 4. CHF

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Chronically, Rheumatic Fever can cause the development of 5 problems:

1. MS 2. AS 3. TS 4. PS 5. could lead to CHF over time (These stenoses are not usually seen until adulthood)

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Cardiomyopathy – 2 Causes:

1. Acute infectious disease affecting otherwise normal healthy heart 2. Abnormal myocardium that may or may not be inherited (HOCM)

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Cardiomyopathy – 2 Types:

1. Hypertrophic 2. Dilated/Congestive

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Most damaging Cardiotoxicity agents are:

Anthracyclines (adriamycin & Cerubidine)

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Cardiotoxicity damage causes ______ ________

ventricular dysfunction

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Systemic Hypertension – cardiovascular congenital cause is:

Coarctation

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Systemic Hypertension – 2 Effects on the heart:

1. Ventricular Hypertrophy (most prominent) 2. Diastolic Dysfunction

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_______ _________is a common pulmonary hypertension finding seen in children and young adults

Cystric Fibrosis

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Pulmonary hypertension Echo Exam – 2 findings:

1. Look for RVH and TR to assess pressure 2. Look for right-to-left shunt if PFO is seen

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CHD can contribute in the formation of thrombus (Examples: MS/AS/DCM). Use 3 techniques to look for thrombus:

1. Increase frequency (if possible) 2. Document in at least 2 views 3. Adjust depth for artifact

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Diagnosis of Fetus/Pediatric pericardial effusion is made when:

heart is surrounded by at least 2 mm of fluid (Confirm PE by 2D & M-mode)

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The effect of End Stage Renal Disease/Failure on the heart can be seen in at least 5 different presentations:

1. PERICARDIAL EFFUSION 2. LVH 3. LVE 4. LVE w/systolic dysfunction 5. AI/MR

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Eisenmenger’s Syndrome – definition:

• Reversal of a congenital shunt from left-to-right, to right-to-left • Secondary to irreversible elevation of pulmonary vascular resistance and severe pulmonary HTN • Must have a congenital shunt of either ASD, VSD, or PDA

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Eisenmenger’s Syndrome Patients commonly present with:

exertional dyspnea

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3 Eisenmenger’s Syndrome Complications:

1. CHF 2. Early Death 3. Sudden Death

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Eisenmenger’s Syndrome Treatment:

• Surgery may be too late if enlargement is excessive • If above finding is indicated, heart-lung transplant is required

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Kawasaki’s Disease defined:

Acute vasculitis that occurs in children <5 years of age

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Kawasaki’s Disease Presentation (3 things):

Children present with: 1. Strawberry tongue 2. Swelling of hands and feet 3. Total body rash

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Vasculitis occurs within the first _______ of Kawasaki’s Disease, affecting the _____ _____

10 days, coronary arteries

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Kawasaki’s Disease – 3 Complications:

1. Acute thrombosis 2. infarction 3. sudden death

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Kawasaki’s Disease Treatment:

Early detection and reducing inflammatory process before irreparable damage occurs.

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