Venous Abnormalities Flashcards
(149 cards)
1
Q
What does Scimitar syndrome describe?
A
A specific subset of partial anomalous pulmonary venous return (PAPVR)
In Scimitar syndrome, anomalous right sided pulmonary vein(s) drain into the inferior vena cava.
2
Q
What is the significance of the term ‘Scimitar’ in Scimitar syndrome?
A
It refers to the classic chest x-ray appearance resembling a curved middle eastern sword
The anomalous vein curves downward toward the diaphragm.
3
Q
What are common associations with Scimitar syndrome?
A
- Hypoplasia of the right lung
- Dextroposition of the heart
- Hypoplasia of the right pulmonary artery
- Abnormalities of the bronchial tree
These associations can lead to various complications.
4
Q
What are aortopulmonary collaterals?
A
Collateral vessels from the descending aorta to the right lung
These collaterals are often present in patients with Scimitar syndrome.
5
Q
What are sequestered lung segments?
A
Portions of the lung which do not directly communicate with the bronchial tree
Sequestered lung segments are common in Scimitar syndrome.
6
Q
At what age do patients with Scimitar syndrome typically present clinically?
A
Often in infancy
Presentation can also occur in school-age years or adulthood.
7
Q
What symptoms do infants with Scimitar syndrome commonly experience?
A
Pulmonary overcirculation due to large aortopulmonary collaterals
This can lead to respiratory issues in infancy.
8
Q
What complications may children with Scimitar syndrome face as they grow?
A
Complications related to pulmonary and bronchial hypoplasia
These complications can affect their respiratory function.
9
Q
What late complications can occur in adults with Scimitar syndrome?
A
- Right heart dilation
- Early pulmonary hypertension from the left-to-right shunt
These complications may arise even if the disease was previously subclinical.
10
Q
What is the estimated prevalence of Scimitar syndrome?
A
~2/100,000
11
Q
What percentage of all PAPVR does Scimitar syndrome account for?
A
3-6%
12
Q
What percentage of patients with Scimitar syndrome will have an associated ASD?
A
Approximately 40%
13
Q
What percentage of patients with Scimitar syndrome will have other associated variants of congenital heart disease?
A
25%
14
Q
Scimitar syndrome arises due to an embryological problem with which lung bud?
A
Right lung bud
15
Q
What fails to grow due to hypoplasia and abnormal development in Scimitar syndrome?
A
Endodermal tissue from the lung bud
16
Q
What does the endodermal tissue from the lung bud form?
A
Pulmonary venous plexus
17
Q
What is the outcome of the abnormal development in Scimitar syndrome?
A
Formation of the scimitar vein
18
Q
Fill in the blank: The venous blood from the right lung forms the _______.
A
Scimitar vein
19
Q
True or False: Scimitar syndrome is a common condition.
A
False
20
Q
What causes early symptoms and presentation in hemodynamics?
A
Overcirculation from AP collaterals from the descending aorta to the right lung
21
Q
What condition can result from high pressure left-to-right shunting if not addressed early in life?
A
Congestive heart failure and pulmonary hypertension
22
Q
What is the physiology of patients without significant AP collateral flow?
A
Similar to an ASD or other types of PAPVR with low-pressure left-to-right shunting
23
Q
What long-term effects can low-pressure left-to-right shunting cause?
A
Right heart dilation and pulmonary hypertension
24
Q
Why might many patients with right lung hypoplasia never require intervention?
A
The amount of shunting is often small
25
What is the primary goal of an echocardiography exam in this context? (Scimitar Syndrome)
Determine cardiac position
26
What should be assessed regarding pulmonary venous connections?
Location and any obstruction
27
What should be evaluated regarding AP collaterals during echocardiography?
Presence and location
28
What associated conditions should be assessed during echocardiography? (Scimitar Syndrome)
ASD or other CHD lesions
29
What feature of the heart should be assessed for dilation? (Scimitar Syndrome)
Right heart
30
What is assessed regarding the right pulmonary artery? (Scimitar Syndrome)
Hypoplasia
31
What evidence of pulmonary hypertension should be looked for?
Septal flattening and tricuspid regurgitation to assess RV pressures
32
What is total anomalous pulmonary venous return (TAPVR)?
A rare congenital malformation where all four pulmonary veins do not connect normally to the left atrium
33
How do the pulmonary veins drain in TAPVR?
They drain abnormally to the right atrium via an anomalous venous connection
34
What are the four main types of TAPVR?
* Supracardiac
* Intracardiac
* Infracardiac
* Mixed venous drainage
35
What characterizes infracardiac TAPVR?
Pulmonary veins connect via the umbilicovitelline system
36
Where do the pulmonary veins drain in infracardiac TAPVR?
To a confluence that drains inferiorly, often through a descending vertical vein
37
What is the most common drainage path for infracardiac TAPVR?
Through a descending vertical vein that descends anterior to the esophagus into the portal venous system
38
What complications can arise from infracardiac TAPVR?
Pulmonary venous obstruction
39
What causes pulmonary venous obstruction in infracardiac TAPVR?
* Intrinsic narrowing of the connecting vessel
* Interposition of hepatic sinusoids
* Constriction of the ductus venosus
40
What is necessary for pulmonary venous return to reach the left heart in TAPVR?
An atrial septal defect
41
Fill in the blank: In infracardiac TAPVR, the pulmonary veins drain to a confluence which then drains _______.
inferiorly
42
What is early atresia of the common pulmonary vein associated with?
Pulmonary-systemic venous connections still present
## Footnote
This condition can lead to significant hemodynamic consequences.
43
How does the hemodynamics vary in cases of unobstructed versus obstructed pulmonary veins?
Unobstructed behaves like a large ASD (left to right shunt); obstructed may cause tachypnea and feeding difficulties
## Footnote
Severe obstruction results in decreased pulmonary blood flow and worsening cyanosis.
44
What are the EKG findings associated with early atresia of the common pulmonary vein?
Tall, peaked P waves; RAD; RVH; iRBBB
## Footnote
These findings indicate right heart strain and possible enlargement.
45
What does CXR reveal in cases of early atresia of the common pulmonary vein?
Increased pulmonary vascular markings; increased right heart structures; 'Snowman' sign
## Footnote
The 'Snowman' sign is a characteristic radiological finding in this condition.
46
Fill in the blank: Severe obstruction of pulmonary veins causes _______.
Decreased pulmonary blood flow with worsening cyanosis
47
True or False: Tachypnea is a potential symptom in patients with obstructed pulmonary veins.
True
48
What is the primary goal of an echocardiography exam regarding pulmonary veins?
Determine size and location of each individual pulmonary vein
## Footnote
This goal helps in assessing the anatomy and function of the pulmonary veins.
49
What aspect of pulmonary anatomy does echocardiography aim to locate?
Determine location of pulmonary confluence
## Footnote
The pulmonary confluence is where the pulmonary veins join before entering the left atrium.
50
What does echocardiography assess about the pulmonary venous channel?
Determine course of the pulmonary venous channel and its site of termination
## Footnote
Understanding the course and termination site is crucial for evaluating potential obstructions.
51
What levels of pathology does echocardiography evaluate in relation to pulmonary veins?
Determine level(s) and severity of obstruction
## Footnote
Identifying obstructions is important for diagnosing conditions like pulmonary vein stenosis.
52
What type of communication does echocardiography evaluate? (TAPVR)
Evaluate interatrial communication
## Footnote
This involves checking for conditions like atrial septal defects.
53
What additional abnormalities does echocardiography assess? (TAPVR)
Evaluate other anatomic and functional abnormalities
## Footnote
This includes assessing the structure and function of the heart beyond just the pulmonary veins.
54
What condition related to the right heart does echocardiography assess? (TAPVR)
Assess for right heart dilation
## Footnote
Right heart dilation can indicate underlying heart conditions or increased pressure.
55
What is total anomalous pulmonary venous connection (TAPVC)?
A condition where all pulmonary veins return from the lungs and drain anomalously to the right side of the heart instead of the left atrium.
## Footnote
TAPVC is a serious congenital heart defect that can lead to significant clinical consequences.
56
What is the most common type of anomalous pulmonary venous connection?
Supracardiac TAPVC.
## Footnote
Supracardiac TAPVC accounts for a significant proportion of total anomalous pulmonary venous connections.
57
In supracardiac TAPVC, where does the common pulmonary vein drain?
Superiorly via a vertical vein into the left innominate vein, the right superior vena cava, or the right azygos vein.
## Footnote
This drainage occurs through an ascending vertical vein.
58
What is the incidence of TAPVC in the population?
Described as 9 in 100,000 of the population.
## Footnote
This statistic highlights the rarity of the condition.
59
What percentage of TAPVC cases are supracardiac TAPVC according to the largest published series?
47%.
## Footnote
This indicates that supracardiac TAPVC is the predominant form of TAPVC.
60
What is the most common site of connection for supracardiac TAPVC?
Left innominate vein (36% of all cases).
## Footnote
This connection site is critical for understanding the anatomy involved in supracardiac TAPVC.
61
From which part of the embryo are the lungs, tracheobronchial tree, and larynx derived?
Foregut
## Footnote
The primordia of these structures originate from the foregut during embryonic development.
62
What is the origin of the pulmonary vascular bed?
Vascular plexus of the foregut (splanchnic plexus)
## Footnote
The pulmonary vascular bed develops from the splanchnic plexus associated with the foregut.
63
In early embryonic development, how does the pulmonary vascular plexus connect to the heart?
No connection
## Footnote
Initially, the pulmonary vascular plexus shares drainage routes with the splanchnic plexus but has no direct connection to the heart.
64
What connects the pulmonary venous plexus to the heart by the end of the first month of gestation?
Common pulmonary vein
## Footnote
The common pulmonary vein connects the pulmonary venous plexus to the sinoatrial part of the heart.
65
What happens to the connection between the pulmonary venous plexus and the splanchnic plexus after the first month of gestation?
It involutes
## Footnote
The connection between these two plexuses reduces and the common pulmonary vein incorporates into the left atrium.
66
Where do the individual pulmonary veins connect after the common pulmonary vein incorporates into the left atrium?
Directly to the left atrium
## Footnote
After incorporation, each pulmonary vein connects separately to the left atrium.
67
What condition results from aberrancies during embryologic development of pulmonary veins?
Anomalous pulmonary venous connection
## Footnote
This condition can arise from various developmental issues during embryogenesis.
68
What is TAPVC?
Total Anomalous Pulmonary Venous Connection
## Footnote
TAPVC occurs when the common pulmonary vein fails to develop or becomes atretic, leading to drainage via collaterals.
69
What are the drainage routes for pulmonary venous blood in TAPVC?
Via collaterals from primitive connections
## Footnote
In TAPVC, blood drains through alternative connections between the splanchnic plexus and other venous systems.
70
Name a genetic syndrome associated with TAPVC.
Cat eye syndrome
## Footnote
Other syndromic associations include Heterotaxy syndrome and Holt-Oram syndrome.
71
What is the relationship between Heterotaxy syndrome and TAPVC?
TAPVC is most common with right atrial isomerism
## Footnote
This syndrome is characterized by abnormal arrangement of internal organs and is associated with TAPVC.
72
What is a suggested pattern of inheritance for TAPVC?
Monogenic pattern
## Footnote
Evidence for a monogenic pattern of inheritance has been suggested in affected families.
73
Where was the gene for TAPVC mapped in a large Utah kindred?
4p13-q12
## Footnote
Genetic mapping efforts in Utah have localized the TAPVC gene to this chromosomal region.
74
What is Type I TAPVC?
Anomalous connections at the supracardiac level
## Footnote
TAPVC stands for Total Anomalous Pulmonary Venous Connection
75
What characterizes Type II TAPVC?
Anomalous connection to the coronary sinus
## Footnote
TAPVC refers to a congenital heart defect.
76
What defines Type III TAPVC?
Anomalous connections below the diaphragm (infracardiac)
## Footnote
This type involves connections that occur below the heart.
77
What is Type IV TAPVC?
Mixed type of connections
## Footnote
This type encompasses various anomalous connection patterns.
78
What are the two subtypes of supracardiac TAPVC?
1. Connection to the Right SVC or Right Azygous vein
2. Connection to the Left Innominate Vein (LIV)
## Footnote
Supracardiac TAPVC can be classified based on the connection of the pulmonary veins.
79
Describe the connection to the Right SVC or Right Azygous vein subtype of supracardiac TAPVC.
The pulmonary veins from each lung join to form a confluence posterior to the LA, with an anomalous vessel ascending to enter the posterior aspects of the right SVC.
## Footnote
In rare cases, this vessel may connect to the azygous vein.
80
What is the most common type of supracardiac TAPVC?
Connection to the Left Innominate Vein (LIV)
## Footnote
This subtype is characterized by the pulmonary veins forming a confluence immediately posterior to the LA.
81
In the connection to the Left Innominate Vein subtype, where does the venous channel originate?
From the confluence immediately posterior to the LA
## Footnote
This channel traverses anterior to the left pulmonary artery, mainstem bronchus, and aortic arch.
82
How does the ascending vein in the LIV subtype typically connect to the right SVC?
The LIV joins the right SVC in a normal fashion
## Footnote
The connection occurs proximal to its origin from the left jugular and subclavian veins.
83
What can happen if the ascending vein in the LIV subtype passes between the left pulmonary artery and left main bronchus?
It can lead to extrinsic compression to pulmonary venous flow causing obstruction
## Footnote
This anatomical positioning may obstruct venous flow.
84
What do hemodynamics depend on in relation to TAPVC?
Presence or absence of pulmonary venous obstruction and size of the interatrial defect
## Footnote
Hemodynamics refer to the dynamics of blood flow and its interaction with the cardiovascular system.
85
What condition does supracardiac TAPVC cause?
Right-sided volume overload and pulmonary over-circulation
## Footnote
This can lead to right ventricular dilation, hypertrophy, and various degrees of pulmonary hypertension.
86
What effect does restriction of interatrial communication have? (TAPVC)
Increases pulmonary over-circulation and diminishes systemic output
## Footnote
This can exacerbate heart failure symptoms.
87
What percentage of patients with unobstructed pulmonary veins are asymptomatic at birth? (TAPVC)
About 50%
## Footnote
Asymptomatic patients may still develop symptoms later.
88
When do most patients with TAPVC present with heart failure?
By the first year of life, most have heart failure by 6 months
## Footnote
Presentation varies, with about 50% presenting in the first month.
89
What is the mortality rate of untreated patients with TAPVC by the first year of life?
75-85%
## Footnote
Early intervention is crucial to improve survival rates.
90
What is the first goal of the echocardiographic exam? (TAPVC)
Identify each pulmonary vein and its connection site by 2D and color Doppler
## Footnote
Do not assume two veins from each lung; the number of individual pulmonary veins varies.
91
What should be excluded when identifying pulmonary veins? (TAPVC)
Additional vein(s) with separate connection(s) to a systemic vein
## Footnote
Examples include left innominate vein, azygous vein, IVC, SVC.
92
In supracardiac TAPVC, where is the connection between the vertical vein and the systemic venous system frequently easily imaged?
From high parasternal short axis and suprasternal notch views
## Footnote
This aids in visualizing the anomalous connections.
93
What is the process for following each anomalously connecting pulmonary vein? (TAPVC)
Follow from its origin to its connection to the confluence or vertical vein from multiple views by 2D and color Doppler sweeps.
94
What should be interrogated using spectral Doppler during the exam? (TAPVC)
Each pulmonary vein and record mean gradients
## Footnote
Record the mean gradient in every site where flow velocity accelerates and the lumen narrows.
95
What should be measured and reported regarding the vertical vein? (TAPVC)
Maximum mean gradient and site of highest gradient
## Footnote
This should be done with good sweeps, paying close attention to potential obstruction sites.
96
What should be measured regarding the pulmonary veins? (TAPVC)
Diameters of each pulmonary vein and the smallest diameter of the pulmonary venous confluence.
97
What anatomical relationship should be imaged during the exam? (TAPVC)
The relationship between the pulmonary venous confluence and the left atrium.
98
What should be ruled out during the echocardiographic exam? (TAPVC)
Mixed drainage and anomalous systemic venous connections.
99
What aspects of the atrial septum should be assessed? (TAPVC)
Direction of atrial shunt and evaluate for restriction to flow.
100
What indicators of right ventricular volume load should be assessed? (TAPVC)
TV annulus diameter, diastolic septal flattening, qualitative assessment of RV size, RV volume by 3D, if feasible.
101
How can pulmonary hypertension be assessed?
With RV pressure by TR and PR jet velocities and by systolic septal configuration.
102
What is total anomalous pulmonary venous return (TAPVR)?
A rare congenital malformation where all four pulmonary veins do not connect normally to the left atrium.
103
How do the pulmonary veins drain in TAPVR?
They drain abnormally to the right atrium through an anomalous venous connection.
104
What are the four main types of TAPVR?
* Supracardiac
* Intracardiac
* Infracardiac
* Mixed venous drainage
105
What characterizes TAPVR to the coronary sinus (TAPVR to CS)?
It is a form of intracardiac TAPVR where all four pulmonary veins return to a pulmonary venous confluence that connects to the coronary sinus.
106
Where does the coronary sinus drain in TAPVR to CS?
Into the right atrium.
107
Fill in the blank: TAPVR is a rare _______ malformation.
[congenital]
108
True or False: In TAPVR, the pulmonary veins connect normally to the left atrium.
False
109
What type of drainage occurs in TAPVR to CS?
Abnormal pulmonary venous return.
110
Echo Goals Total Anomalous Pulmonary Venous Return to the Coronary Sinus (TAPVR to CS)
Goals of echocardiography exam
Determine size and location of each individual pulmonary vein
Determine location of pulmonary confluence
Determine course of the pulmonary venous channel and it’s site of termination
Determine level(s) and severity of obstruction
Evaluate interatrial communication
Evaluate of other anatomic and function abnormalities
Assess for right heart dilation
111
What is Cor Triatriatum Sinister?
A rare congenital condition in which the left atrium is subdivided by a fibromuscular membrane into two distinct chambers
112
What are the two chambers created in Cor Triatriatum Sinister?
* Posterosuperior chamber (accessory LA chamber) * Anteroinferior chamber (true LA)
113
Where do the pulmonary veins enter in Cor Triatriatum Sinister?
The posterosuperior chamber
114
What does the anteroinferior chamber communicate with?
The mitral valve and gives rise to the LA appendage
115
How do the two chambers in Cor Triatriatum Sinister communicate?
Through a membrane
116
What variations can the membrane that divides the atrium exhibit?
* Size and shape may vary * Similar to a diaphragm * Funnel-shaped * Bandlike * Entirely intact (imperforate) * Contains openings (fenestrations)
117
What is the most common form of cor triatriatum?
All four pulmonary veins drain to an accessory atrial chamber
118
What is a rarer variant of cor triatriatum?
Partial or total anomalous pulmonary venous connection
119
What is the most widely accepted theory for the cause of cor triatriatum?
Secondary to an incomplete incorporation of the common pulmonary vein into the posterior aspect of the left atrium
120
What other cardiac abnormalities are frequently associated with cor triatriatum?
* PFO (Patent Foramen Ovale) * Secundum ASD (Atrial Septal Defect) * Left SVC to coronary sinus * Less commonly, anomalous pulmonary venous connection
121
What causes symptoms in cor triatriatum?
The gradient across the dividing septum, secondary to the number and size of the fenestrations in the septum
122
True or False: Cor Triatriatum Sinister is a common congenital heart defect.
False
123
Fill in the blank: The anteroinferior chamber of Cor Triatriatum Sinister is referred to as the _______.
true LA
124
What percentage of all congenital abnormalities does this condition represent?
0.1-0.4%
125
Name one associated cardiac lesion related to this condition.
Atrial septal defect
## Footnote
Other associated lesions include anomalous pulmonary venous return, bicuspid aortic valve, and PDA.
126
What is the most commonly accepted theory for the embryological cause of this condition?
Incomplete incorporation of the embryonic common pulmonary vein into the left atrium
127
What is one of the proposed mechanisms for this condition related to the septum?
Abnormal growth of the septum primum
128
What happens to the common pulmonary vein in one of the proposed mechanisms?
Entrapment of the common pulmonary vein by the left horn of the sinus venosus
129
Fill in the blank: Persistence of the left superior _______ impinges on the developing left atrium.
vena cava
130
What happens if there is no alternative pathway for accessory atrial chamber?
Elevated pressure in accessory atrial chamber transmitted to pulmonary veins
## Footnote
This can lead to pulmonary venous obstruction in severe cases.
131
What is the physiology of pulmonary venous obstruction associated with accessory atrial chamber issues?
It leads to secondary pulmonary hypertension
## Footnote
Severe cases can cause significant complications.
132
True or False: Elevated pressure in the accessory atrial chamber does not affect the pulmonary veins.
False
## Footnote
Elevated pressure is transmitted to the pulmonary veins.
133
Fill in the blank: Elevated pressure in the accessory atrial chamber results in _______ to the pulmonary veins.
transmission
134
What is the function of the accessory atrial chamber?
Receives all pulmonary veins and communicates with left atrium
## Footnote
This is characteristic of classic cor triatriatum.
135
What does TAPVC stand for?
Total Anomalous Pulmonary Venous Connection
136
In classic cor triatriatum, how does the accessory atrial chamber connect?
It communicates with the left atrium
137
What is a characteristic of a subtotal cor triatriatum?
Accessory atrial chamber receives part of pulmonary veins and connects to LA
## Footnote
Remaining pulmonary veins can connect normally or anomalously.
138
Fill in the blank: An accessory atrial chamber that receives all pulmonary veins and does not communicate with the left atrium is classified as _______.
classic cor triatriatum
139
What happens in TAPVC concerning the accessory atrial chamber?
It receives all pulmonary veins and connects anomalously to the RA directly
140
What is a potential connection for the remaining pulmonary veins in a subtotal cor triatriatum?
Connect normally or anomalously
141
True or False: In a classic cor triatriatum, there are no other connections besides the left atrium.
True
142
In the case of an accessory atrial chamber that receives part of the pulmonary veins, where can it connect?
To LA or RA
## Footnote
Remaining pulmonary veins may connect normally.
143
Cor membrane is seen as a ____
linear echodensity traversing the left atrial cavity
144
Cor Triatriatum (CT) versus Supravalvar MV ring (SVMR)
CT: Linear echodensity, curvilinear and wind sock appearance moves toward MV in diastole and above the left atrial appendage
SVMR: Near atrial surface at base of mitral valve leaflets and relatively immobile usually adherent to MV and moves away in diastole
145
What do disturbed diastolic profiles mimic?
Mitral stenosis
## Footnote
Disturbed diastolic profiles can present similarly to mitral stenosis in echocardiographic findings.
146
What should be measured to assess trans-membrane pressure gradients?
Mean and maximal trans-membrane pressure gradients and maximal velocity by spectral Doppler
## Footnote
These measurements are critical for understanding the hemodynamics involved.
147
What may a mild pressure gradient across the membrane indicate in the context of a non-restrictive superior ASD?
It may not reflect the high resistance across the circuit
## Footnote
This condition can lead to a misleading assessment of hemodynamics.
148
What does a non-restrictive superior ASD act as in terms of pressure?
A 'pop-off' decompressing the higher pressure pulmonary venous chamber into the lower pressure atrium
## Footnote
This mechanism can affect hemodynamic assessments.
149
Why must patients with a non-restrictive superior ASD be followed closely in infancy?
To ensure no interval restriction of membrane or atrial communication
## Footnote
Close monitoring is essential to prevent complications.
