CARDIAC Section 5: Congenital Heart Flashcards
Describe and identify the congenital anomaly
Egg on String: TOTGA
Describe and identify the congenital anomaly
Snow Man: TAPVR (Supracardiac)
Describe and identify the congenital anomaly
Boot-Shaped: TOF
Describe and identify the congenital anomaly
Box-shaped: Ebstein Anomaly
Describe and identify the congenital anomaly
Figure 3: Coarctation
What diseases are associated with the right arch?
Truncus (More closely related)
TOF (More common overall)
With regard to the big box hear is non-cardiac causes of hight output Failure seen in these conditions
Infantile Hemangioendothelioma and Vein of Galen Malformation
Left Side Arch + Massive heart size =
- Ebsteins or
- Pulmonary atresia without VSD
Left Side Arch + Normal heart size + Increased Pulmonary Blood flow
TAPVR (Especially Type 3)
D-Transposision
Truncus (Loof for Rarch)
“Tingle Ventricle”
Left Side Arch + Normal heart size + Decreased or Normal Blood flow
TOF
Ebsteins
Tricuspid Atresia
Causes of CHF in Newbord
TAPVR (Infracardiac Type III)
Congenital Aortic or Mitral Stenosis
Left Sided Hypoplastic Heart
Cor Triatriatum
Survival dependent on admixture - Cvanotics
TAPVR (Has patent forament ovale)
TOTGA
TOF (Has VSD)
Tricuspid Atreasia (Has VSD)
Hypoplastic left
Small Heart DDx
Adrenal Insufficiency (Addisons)
Cachectic State Constrictive Pericarditis
What is the most common type of congenital heart disease, and which subtype is the most prevalent?
Ventricular Septal Defect (VSD) is the most common congenital heart disease.
Within VSD, the membranous subtype, located just below the aortic valve, is the most common, accounting for about 70% of cases.
Why are Outlet subtype VSDs significant, and what complication can they lead to?
Outlet subtype VSDs, located in the infundibulum, are significant because they must be repaired to prevent complications. One such complication is the prolapse of the right coronary cusp into the defect.
What are the common radiological findings of a VSD on a Chest X-Ray (CXR)?
enlarged heart, increased vasculature, and a small aortic knob. In some cases, there might be splaying of the carina due to an enlarged left atrium.
What percentage of small VSDs close spontaneously?
About 70% of small VSDs close spontaneously without the need for medical intervention.
When does the Patent Ductus Arteriosus (PDA) normally close after birth?
The PDA normally closes functionally around 24 hours after birth and anatomically around one month.
What are three key associations to recall when considering a PDA?
(1) Prematurity,
(2) Maternal Rubella, and
(3) Cyanotic Heart Disease.
What are the typical radiological findings of a PDA on a CXR?
The typical radiological findings of a PDA on a CXR include a large heart, increased pulmonary vasculature, and a large aortic arch, often referred to as a “ductus bump.”
What are the medical management options for a PDA?
A PDA can be medically managed either by closing it through intervention or keeping it open with medications, depending on the clinical scenario and patient needs.
What is the most common types of Atrial Septal Defect?
Secundum (50-70%)
The larger subtype of ASD?
Pimum
What ASD subtype results from an endocardial cushion defect?
Primum, is more likely to be symptomatic
What ASD subtype may close withouth treatment?
Secundums
Why is primum not amenable to device closure?
Because of its proximity to AV valve tissue
ASD is common in what gender?
Female
Hand/Thub defects + ASD =
Holt Oram
When I say Ostium primum ASD (endocardial cushion), you say?
Downs Syndrome
When I say Venosus ASD, you say?
PAPVR
What is the most common cardiac manifestation of Holt Oram’s?
ASD
Adults with AV septal defects have how many % risk of recurrence o f heart disease in their kids
10%
Also referred to as an endocardial cushion defect
AV Canal
An endocaridal cushion defect belons to the specrtum of what defect?
Ostium Primum
Described the AV canal
deficient development of a portion of the atrial septum, a portion of the inter-ventricular septum, and the AV valves.
AV canal has strong association with what condition?
Down’s Syndrome
O f all the congenital heart stuff with Downs patients, what is the most common?
AV Canal
What is the best view to see AV canal Defects?
Four chameber horizontal view
This is a rare ASD which occurs secondary to a fenestrated (as in the cartoon) or totally unroofed coronary sinus.
Unfroofed Coronary Sinus (Coronar Sinus ASD)
What is the most important clinical manifestation of Unroofed Coronary Sinus (Coronary Sinus ASD)?
Paradoxical Emboli
Chronic Right heart Volume overload
Unfroofed Coronary SInus has STRONG association with?
A persistent left SVC
What is the most common ASD subtype?
Ostium Secundum
A true ASD defect -
Ostium Secundum
ASD Subtype
Ostium Secndum
ASD subtype associated with PULMONARY HYPERTENSION?
Ostium Secundum
also known as AV canal defects or endocardial cushion defects
Ostium Primum
Spectrum of the atrioventricular (AV) septal defects
Ostium Primum
the complete form of which also includes a large ventricular septal defect and a common AV valve
Ostium Primum
ASD Subtype
Ostium Pimum
associated with anomalies of the atrioventricular (AV) valves, ventricular septal defect (VSD
Ostium Pimum
What are relatively large ASD and lead to right hear dilatation?
Ostium primum
(Optimus PRime is Large)
Defect in the SVC-right atrial junction
Or less commonly the IVC - Right Atrial
Junction
Sinus Venosus
ASD Subtype
Sinus VEnosus
ASD Subtype associated with PAPVR
SInus Venosus
Defect (partial or
complete) in the Coronary Sinus
Unroofed Coronary Sinus
Least common ASD Subtype
Unroofed Coronary Sinus
Allows a left-to- right shunt to occur
Unroofed Coronary Sinus
Associated with a
persistent left SVC
Unroofed Coronary Sinus
one (or more) of the four pulmonary veins draining into the right atrium.
PAPVR
PAPVR is associated with these types of ASDs
Secondum and sinus venosus
When I say Right Sided PAPVR,
you say Sinus Venosus ASD
When I say Right Sided PAPVR + Pulmonary Hypoplasia
Scimitar Syndrome
Identify
Scimitar Syndrome
a cyanotic heart disease characterized by all of the pulmonary venous system draining to the right side of the heart
Total Anomalous Pulmonary Venous Retrun
- essentially a total eclipse of the heart. ;P
What are the essential anatomical features for survival in TAPVR?
A large Patent Foramen Ovale (PFO) or, less commonly, an Atrial Septal Defect (ASD) is essential for survival in TAPVR.
These openings allow for some oxygenated blood to mix and circulate in the systemic circulation.
What the three types of TAPVR?
- Supracardiac
- Cardiac
- Infracardiac
Most commont type of TAPVR?
Describe
Supracardiac.
Veins drain above the heart producing a snowman sign.
Second most common TAPVR?
Cardiac
Type of TAPVR causing obstruction and pulmonary edema appearance in newborns.
Infracardiac.
The most common cause of cyanosis during the first 24 hours?
Transposition of the Great Arteries
Seen most commonly in infants of diabetic mothrs.
TGA
Describr the TGA
The basic idea is that the aorta arises from the right ventricle and the pulmonary trunk from the left ventricle {ventricularterial discordance).
What do you have to find to determine if its right or left ventricle
Find the Moderator band (That defines the RV)
What does TGA need in order to survive?
ASD
VSD (Most common)
PDA
What are the two (2) TGA flavors?
D type and L type.
What type of TOGA is lucky enough to be compatible with life?
L-Transposition (LUCKY)
What is the classic x ray appearance of d-TGA
Egg on a string
What procedure is performed to fix the d-TGA
Intra-atrial baffle (Mustard or Senning procedure)
This occurs from a “double discordance” where the atrium hooks up with the wrong ventricle and the ventricle hooks up with the wrong vessel.
l-TGA
Describe the d-TGA
In D-Transposition, the ductus may be the only connection between the two systems, which would othenwise be separate (and not compatible with life)
What can be the only connection between the two systems in d-TGA
PDA
Describe the l-TGA
In L-Transposition of the great vessels - there is an inversion of the ventricles, leading to a “congenital correction. ” No PDA is needed.
Identify and described
Corrected d-TGA
What is the procedure of d-TGA correction
“LeCompte Maneuver”
Jatene Arterial Switch
What is the most common cyanotic hear disease?
Tetralogy of Fallot (TOF)
Describe the 4 major findings of TOF
(1) VSD, (2) RVOT Obstruction - often from valvular obstruction, (3) Overriding Aorta, (4) RV hypertrophy (develops after birth).
The degree of seerity in symtpoms in TOF depends on?
How bad the RVOT obstruction is.
Whats is the pentalogy of Fallot?
(1) VSD,
(2) RVOT Obstruction - often from valvular obstruction,
(3) Overriding Aorta,
(4) RV hypertrophy (develops after birth).
(5) ASD
What is the shunt procedure for TOF?
Blalock-Taussig shunt
What is the most common complication following TOF repair?
Pulmonary regurgitation.
Describe Truncus Arteriosus
Cyanotic anomaly - a single trunk supplying both the pulmonary and systemic circulation, not a separate aorta and pulmonary trunk.
What CHD is associated with DiGeorge Syndrome (CATCH 22 gene ;P)
Truncus arteriosus.
What is the most common associated defect in CoA?
Bicuspid Aortic Valve (80%)
CoA is associated with what condition?
Turners Syndrome (15-20%)
What CHD have more berry aneurysms?
CoA
Rib notching invovles what rib level?
4th-8th ribs
Differentiate the Infantile vs Adult CoA
Infantile:
1. Presents with heart failure within the first week of life.
2. Pre-Ductal (Before the left Subclavian A.)
3. Aortic Arch = Hypoplastic
Adult:
1. Leg Claudication
2. BP differences between arms and legs.
3. Post-Ductal (Distal to left Subclavian A.)
4. Aortic Arch = Normal Diameter
5. Collateral Formation is More Likely
What is hypoplastic left heart?
- Left ventricle and aorta are hypoplastic.
- They present with PULMONARY EDEMA.
- Must have an ASD or large PFO. They also typically have a large PDA to put blood in their arch.
- Strongly associated with aortic coarctation and endocardial fibroelastosis.
What is this? Descibe
Cor Triatriatum Sinsitrum
Abnormal pulmonary vein draining into the left atrium with an unnecessary fibromuscular membrane that causes a subdivision of the left atrium.
creating the appearance of TRI-TRIUM heart.
What CHD can cause an unexplained hypertension in pediatritcs?
Cor Triatriatum
Why does Cor triatriatum sinistrum cause pulmonary edema?
It acts like mitral stenosis.
What’s the prognosis of CTS?
bad (fatal within 2 years)
