Left Ventricular Outflow Abnormalities Flashcards
(134 cards)
1
Q
What is the most common congenital heart disease?
A
Bicuspid aortic valve (BAV)
2
Q
What is the estimated prevalence of BAV?
A
0.5-2%
3
Q
In which gender is BAV more commonly observed?
A
Males
4
Q
What is the approximate male to female predominance ratio for BAV?
A
3:1
5
Q
What causes a bicuspid aortic valve?
A
Partial or complete fusion of two of the aortic valve coronary cusps
6
Q
What are the possible abnormal openings of a bicuspid aortic valve?
A
Horizontal or vertical raphe
7
Q
What conditions may result from a bicuspid aortic valve?
A
Aortic stenosis or aortic regurgitation
8
Q
What complications can develop in the adjacent aorta due to BAV?
A
Dilation and aneurysms
9
Q
Which parts of the aorta are commonly affected by dilation and aneurysms in BAV?
A
- Aortic root
- Sinotubular junction
- Ascending aorta
10
Q
At what stages of life are dilation and aneurysms due to BAV most commonly observed?
A
Adolescence and adulthood
11
Q
What is a fixed subaortic membrane?
A
A collar or ridge of membranous and/or fibromuscular tissue extending from the ventricular septum into the left ventricular outflow tract
12
Q
What condition does a fixed subaortic membrane result in?
A
Subaortic stenosis
13
Q
How is the fibromuscular ridge of a fixed subaortic membrane often characterized?
A
Discrete, circumferential with attachments to the mitral valve, or diffuse and tunnel-like
14
Q
Is a fixed subaortic membrane commonly seen in the neonatal period?
A
No, it is rarely seen in the neonatal period
15
Q
When does a fixed subaortic membrane typically develop?
A
Over time due to an abnormal proliferation of fibrous tissue
16
Q
What percentage of fixed subaortic membrane cases are associated with congenital heart defects?
A
More than 50%
17
Q
List some common congenital heart defects associated with fixed subaortic membrane.
A
- Ventricular septal defect
- Coarctation of the aorta
- Atrioventricular septal defect
- Valvular aortic stenosis
- Congenital anomalies of the mitral valve
18
Q
What is aortic valve stenosis in children primarily caused by?
A
A congenital anomaly of the aortic valve
Congenital anomalies include commissural underdevelopment, myomatous thickening of the valve leaflets, annular hypoplasia, or a combination of these elements.
19
Q
What are the types of aortic valve configurations due to commissural underdevelopment?
A
- Unicuspid
- Bicuspid
- Trileaflet
- Quadricuspid
Unicuspid has one well-formed commissure, bicuspid has one underdeveloped commissure, trileaflet has all three well-developed, and quadricuspid has an extra commissure.
20
Q
What important interrogation should be conducted when evaluating aortic stenosis?
A
An interrogation for aortic regurgitation
Aortic regurgitation is a common association with aortic stenosis.
21
Q
What should be included in a full assessment of aortic stenosis?
A
All left heart structures
This is important due to known associations with other left-sided obstructive lesions such as mitral stenosis, subaortic stenosis, and coarctation.
22
Q
What is a known association with aortic stenosis concerning the aorta?
A
Post-stenotic dilation of the proximal ascending aorta
This dilation occurs as a result of the pressure overload from the stenosis.
23
Q
What is supravalvar aortic stenosis?
A
Obstruction above the aortic valve, most commonly at the sino-tubular junction.
It is characterized by a discreet narrowing of the aortic lumen.
24
Q
What are the potential additional areas of narrowing in supravalvar aortic stenosis?
A
Narrowing of the ascending aorta and/or aortic arch.
These additional narrowings can complicate the condition.
25
How does supravalvar aortic stenosis progress over time?
It is a progressive disease that becomes more severe with age and requires continual cardiology follow-up.
## Footnote
Regular monitoring is essential for managing the condition.
26
In which patient population is supravalvar aortic stenosis commonly seen?
Patients with Williams Syndrome.
## Footnote
Williams Syndrome is an autosomal dominant genetic disorder.
27
What type of genetic disorder is Williams Syndrome?
Autosomal dominant genetic disorder.
## Footnote
This means that only one copy of the mutated gene from an affected parent can cause the disorder.
28
What vascular abnormalities are associated with Williams Syndrome?
Thickening of the vascular media layer in medium and large sized arteries.
## Footnote
This thickening can contribute to various cardiovascular complications.
29
Fill in the blank: Supravalvar aortic stenosis typically involves obstruction above the _______.
aortic valve
30
True or False: Supravalvar aortic stenosis is a stable condition that does not require follow-up.
False
31
What is Shone’s complex?
A rare congenital heart defect consisting of multiple, left-sided obstructive heart lesions including: supra-valvar mitral ring or membrane, parachute mitral valve, sub-aortic membrane, and coarctation of the aorta.
## Footnote
True Shone’s complex is rare and involves all four defects.
32
What characterizes a supra-valvar mitral ring?
A membrane of connective tissue superior to the mitral valve orifice, causing restriction of blood flow through the valve.
## Footnote
This membrane can lead to significant hemodynamic consequences.
33
What is a parachute mitral valve?
Characterized by chordal attachments to a single papillary muscle, most commonly the posteromedial papillary muscle, resulting in a narrowed mitral valve annulus.
## Footnote
This condition often leads to mitral valve dysfunction.
34
What features are associated with mitral valve leaflets in Shone's syndrome?
Thickened and dysplastic with limited mobility and restriction of flow.
## Footnote
These changes can contribute to mitral regurgitation.
35
What is sub-aortic stenosis?
May be membranous or muscular, causing restriction of blood flow below the aortic valve and across the left ventricular outflow tract.
## Footnote
This condition can lead to left ventricular outflow tract obstruction.
36
What is a bicommissural aortic valve?
A common finding in patients with Shone’s syndrome, typically resulting in valvar aortic stenosis.
## Footnote
This anatomical variant can complicate surgical interventions.
37
What describes coarctation of the aorta?
Narrowing of the aortic arch, typically at the level of the aortic isthmus.
## Footnote
This condition can lead to hypertension in the upper body.
38
What happens to the transverse arch in coarctation of the aorta?
It may be elongated and hypoplastic.
## Footnote
These changes can affect blood flow dynamics.
39
What is Shone’s variant or incomplete Shone’s syndrome?
More common form typically diagnosed when only two or three left-sided obstructions are present in the setting of left heart hypoplasia.
## Footnote
This variant indicates a less severe form of the complex.
40
True or False: True Shone's complex includes all four defects.
True.
## Footnote
The presence of all four defects is considered rare.
41
Fill in the blank: Shone's like complex refers to multiple sites of left-sided obstruction and _______.
hypoplasia.
42
What is Aortico–left ventricular tunnel (ALVT)?
A rare congenital paravalvar communication between the aorta and the left ventricle (LV)
43
What does ALVT represent?
An extracardiac channel connecting the ascending aorta above the sino-tubular junction to the cavity of the left ventricle
44
What is a significant consequence of ALVT?
Chronic preload due to regurgitation
45
What condition does ALVT lead to in the left ventricle?
Left ventricular (LV) dilatation
46
What is the recommended treatment for ALVT?
Early surgical repair
47
What is coarctation of the aorta?
A narrowing of the aorta most commonly in the region distal to the head and neck vessels in the aortic isthmus.
48
Where is the coarctation of the aorta typically located?
In the region distal to the head and neck vessels, called the aortic isthmus.
49
What is the term used for the portion of the aorta near the ductus arteriosus?
Juxtaductal aorta.
50
What is the ductus arteriosus?
A blood vessel present in the fetus which typically closes postnatally within the first few hours to days of life.
51
What can happen once the patent ductus arteriosus (PDA) closes?
It can cause aortic narrowing.
52
What are the possible presentations of coarctation of the aorta?
Discrete coarctation, long segment coarctation, or diffuse hypoplasia of the transverse arch.
53
What percentage of patients with congenital heart disease have coarctation of the aorta?
6% to 8%.
54
What associated anomalies are commonly found with coarctation of the aorta?
* Bicuspid aortic valve
* Ventricular septal defect
* Small left sided structures (Shone’s variant/complex)
* Variants of single ventricle anatomy.
55
True or False: Coarctation of the aorta accounts for a large percentage of children diagnosed later in life with systemic hypertension.
False.
56
Fill in the blank: Coarctation of the aorta is associated with _____ aortic valve.
bicuspid.
57
When do the aortic arch and head and neck vessels develop?
Between 6 to 8 weeks of gestation
58
What does the embryologic 3rd aortic arch persist as?
Common carotid arteries
59
What does the left 4th aortic arch form?
Thoracic aortic arch and aortic isthmus
60
What normally happens to the right 4th aortic arch?
It involutes
61
What does the left 6th aortic arch develop into?
PDA
62
What is coarctation a result of?
Abnormal embryologic development of the left 4th and 6th aortic arches
63
What is the Ductal Tissue Theory?
Coarctation is a result of migration of ductal tissue into the region of the aortic isthmus with subsequent constriction and narrowing of the aortic lumen
64
What is the Hemodynamic Theory?
Coarctation develops secondary to hemodynamic abnormalities with decreased blood flow during fetal life through the aortic arch
65
Which congenital heart defects can contribute to the development of coarctation?
* VSD
* Aortic stenosis
* Arch hypoplasia
66
In which genetic syndromes is there an increased prevalence of coarctation?
* Turners syndrome (XO)
* Williams Syndrome
* Noonan Syndrome
* Rubenstein-Taybi syndrome
* Alagille Syndrome
* Neurofibromatosis
* Kabuki Syndrome
* PHACES syndrome
67
Is there a gender predominance for coarctation?
Yes, there is a slight male predominance
68
What is the risk of coarctation for offspring and first-degree relatives diagnosed with an obstructive left-sided cardiac lesion?
Ten times the risk
69
What percentage of combined ventricular output travels through the aortic isthmus in fetal life?
Small percentage
## Footnote
In fetal life, hemodynamics are rarely disturbed.
70
What happens to cardiac output after birth?
All cardiac output must cross the aortic isthmus
## Footnote
This occurs following the closure of the PFO and PDA.
71
What can be the variable presentations of coarctation severity?
Mild hypertension to LV systolic dysfunction with congestive heart failure and cardiogenic shock
## Footnote
Presentation varies based on the severity of coarctation.
72
What are the hemodynamic effects of severe postnatal coarctation following ductal closure?
Decreased stroke volume, increased left ventricular end diastolic pressure, elevated left atrial pressure
## Footnote
These changes can lead to pulmonary venous congestion and pulmonary hypertension.
73
What complications can arise from decreased cardiac output in severe coarctation?
Diminished myocardial and systemic perfusion, development of lactic acidosis, end organ injury
## Footnote
Affected organs may include kidney, liver, and bowel.
74
True or False: In fetal life, hemodynamics are often disturbed.
False
## Footnote
Hemodynamics are rarely disturbed in fetal life.
75
Fill in the blank: Following birth, all cardiac output must cross the _______.
aortic isthmus
## Footnote
This occurs after the closure of the PFO and PDA.
76
Best view to visualize coarctation of the aorta
Suprasternal notch view with 2D, color and spectral Doppler imaging
## Footnote
This view allows for comprehensive assessment of the aortic arch and coarctation.
77
What is obtained to assess coarctation?
Peak velocity with a mean pressure gradient across transverse arch and isthmus
## Footnote
This helps evaluate the severity of coarctation.
78
What is often observed in flow patterns with coarctation?
High velocity with persistence of flow into diastole (diastolic runoff)
## Footnote
This pattern indicates abnormal hemodynamics.
79
What should be identified from the suprasternal notch view?
Arch sidedness and branching pattern of the head and neck vessels
## Footnote
This assessment includes checking for distal displacement of the left subclavian artery.
80
What measurements are obtained during the echocardiographic exam?
2D aortic arch measurements along with z-scores
## Footnote
Key measurements include ascending aorta, proximal transverse arch, distal transverse arch, and aortic isthmus.
81
Proximal transverse arch definition
Diameter of aortic arch between innominate artery and left carotid artery
## Footnote
This measurement is crucial for assessing the aortic arch.
82
Distal transverse arch definition
Diameter of aortic arch between left carotid artery and left subclavian artery
## Footnote
Accurate measurement can indicate arch anomalies.
83
Aortic isthmus definition
Diameter of aortic arch distal to the left subclavian artery
## Footnote
This dimension is important for diagnosing coarctation.
84
Normal newborn arch measurement estimation for proximal arch
Weight in kg + 1
## Footnote
This formula provides a rough estimation for assessing arch size.
85
Normal newborn arch measurement estimation for distal arch
Weight in kg
## Footnote
This helps determine if the arch size is appropriate.
86
What may be observed in abdominal aortic Doppler in coarctation?
Dampened/blunted flow patterns (reduced velocity)
## Footnote
This indicates altered hemodynamics due to coarctation.
87
Pulse-wave Doppler assessment should be performed at what angle?
Most parallel and least perpendicular to the angle of the abdominal aorta
## Footnote
This ensures accurate velocity measurements.
88
What should be assessed in left-sided structures?
Hypoplasia of aortic valve, mitral valve, left ventricle
## Footnote
Z-scores are used for precise evaluation of these structures.
89
What defects should be assessed in the ventricular septum?
Ventricular septal defects (VSD)
## Footnote
Detailed sweeps from various views are necessary to identify VSD.
90
How should ductus arteriosus be evaluated?
By 2D, color and spectral Doppler from suprasternal notch and parasternal views
## Footnote
Assessing flow patterns is crucial, especially in coarctation.
91
What flow pattern should be expected in ductus arteriosus in coarctation?
Right to left or bidirectional
## Footnote
This indicates the hemodynamic impact of coarctation.
92
True or False: A coarctation of the aorta can be ruled out in the presence of a PDA.
False
## Footnote
Often, the PDA must constrict before coarctation becomes evident.
93
What should be assessed for right/left heart disproportion?
Right ventricular hypertrophy (RVH) and right ventricular dilation
## Footnote
These findings are critical in evaluating heart function in coarctation.
94
What needs to be evaluated regarding atrial communication?
Presence, size, direction and mean pressure gradient
## Footnote
This assessment is vital for understanding the hemodynamics.
95
What is assessed to evaluate pulmonary hypertension?
Right heart size, tricuspid and pulmonary regurgitation Doppler patterns, septal configuration
## Footnote
This assessment is done from parasternal short axis views.
96
What methods are used to assess left ventricular systolic function?
M-mode, bullet and Simpson’s biplane
## Footnote
Critical coarctation can lead to depressed ventricular function.
97
What is hypoplastic left heart syndrome (HLHS)?
A variant of congenital heart disease characterized by underdevelopment of left sided cardiac structures
98
Which cardiac structures are underdeveloped in HLHS?
* Mitral valve
* Left ventricle
* Aortic valve
* Aortic arch
99
What is the consequence of the left side of the heart being unable to support systemic circulation in HLHS?
Blood must flow through an atrial septal defect
100
How does blood reach the systemic circulation in HLHS?
Through a patent ductus arteriosus
101
What is the role of the right ventricle in HLHS?
It pumps blood into the pulmonary artery
102
What is a key feature of the left ventricle in HLHS?
Severe hypoplasia of the left ventricular cavity
103
What percentage of all congenital heart disease does HLHS comprise?
1.4-3.8%
104
Is there a known specific genetic abnormality linked to HLHS?
No specific genetic abnormality has been linked
105
What is the risk of recurrence of other congenital heart disease in the same family with one affected member?
2.2-13.5%
106
What is the spectrum of HLHS?
HLHS occurs in a spectrum ranging from:
* Aortic valve atresia with mitral atresia (AA/MA)
* Aortic valve atresia with a patent mitral valve with stenosis (AA/MS)
* Aortic stenosis with a patent mitral valve with stenosis (AS/MS)
107
What congenital abnormality is associated with aortic atresia with a patent mitral valve?
Coronary cameral fistulas
108
What percentage of neonates with HLHS have an intact or restrictive atrial septum?
1%
109
What is the occurrence rate of persistent left superior vena cava in HLHS?
15%
110
What is the occurrence rate of anomalous pulmonary venous drainage in HLHS?
5-10%
111
How is HLHS usually diagnosed?
Via prenatal ultrasound due to advancements in fetal echocardiography
## Footnote
Fetal echocardiography allows for early detection of congenital heart defects like HLHS.
112
What treatment is initiated postnatally for HLHS patients?
PGE to maintain ductal patency until stage I palliation
## Footnote
PGE stands for prostaglandin E1, which helps keep the ductus arteriosus open.
113
What procedure may be performed on fetuses with critical aortic stenosis?
Fetal aortic valvuloplasty
## Footnote
This procedure aims to relieve obstruction and improve blood flow to the heart.
114
What is the indication for placement of an intra-atrial stent during fetal life?
Restrictive/intact atrial septum
## Footnote
An intra-atrial stent can help improve blood flow in fetuses with certain cardiac conditions.
115
What are the early clinical manifestations for children born with restrictive/intact atrial septum?
Cyanosis and respiratory distress
## Footnote
These symptoms indicate inadequate oxygenation and blood flow.
116
What initial appearance might children with non-restrictive atrial communication have?
Acyanotic
## Footnote
Acyanotic children may not show immediate symptoms until PDA closure occurs.
117
What signs of poor systemic perfusion might be observed in children with HLHS?
Respiratory distress, pale, cool extremities, lethargy
## Footnote
These signs indicate inadequate blood flow and oxygen delivery to the body.
118
What is the primary goal of echocardiography in patients with HLHS?
To provide all the anatomic details needed for surgical planning
119
What characteristic feature is observed in the left ventricle during the parasternal long axis view?
A small muscle bound, non-apex forming left ventricle
120
In echocardiography, what does an echo-bright endocardial surface of the left ventricle indicate?
Endocardial fibroelastosis
121
How does the left atrium typically present in patients with HLHS?
Generally small, but can be dilated with a restrictive atrial septum
122
What is the condition of the ascending aorta in HLHS?
Hypoplastic with/without a patent aortic valve
123
What is a common finding regarding the mitral valve in echocardiography of HLHS?
Often imperforate; if patent, the leaflets are thick with short or absent papillary muscle chordal attachments
124
What may interrogation of the ventricular septum reveal in cases of aortic atresia?
Ventriculo-coronary arterial connections
125
What is the purpose of the parasternal short axis view in echocardiography?
To assess left ventricular size, systolic function, and mitral valve papillary muscles
126
What anatomical structures can be visualized from the parasternal short axis view?
* Aortic valve morphology
* Left ventricle to coronary artery connections
* Pulmonary valve
* PDA continuing into the descending aorta
* Branch pulmonary arteries
127
What additional information can be obtained from the apical four chamber view?
Size and systolic function of the ventricles, mitral and tricuspid valve anatomy, and annulus measurements
128
What is the best view for atrial septal interrogation?
Subcostal view
129
What should be assessed in the subcostal view for atrial septal interrogation?
* Presence and size of an atrial communication
* Mean and peak Doppler gradients for left atrial hypertension
130
Why is estimating restrictive atrial level shunting critically important postnatally?
Patients may require urgent balloon atrial septostomy
131
What can be assessed regarding pulmonary venous drainage from the subcostal view?
Presence of a levoatrial cardinal vein draining pulmonary venous blood to a systemic vein
132
What is visualized from the suprasternal notch view?
Aortic arch anatomy including coarctation/arch interruption
133
What can Doppler interrogation reveal in the aortic arch from the suprasternal notch view?
Retrograde systolic flow from a patent ductus arteriosus
134
What additional structures can be visualized from the suprasternal notch view?
* Pulmonary venous drainage
* Left superior vena cava
* Levo-atrial cardinal vein
* Ductus arteriosus
* Proximal branch pulmonary arteries
