CHD - Adult Flashcards

1
Q

What is situs solitus?

A

refers to the normal position of the thoracic and abdominal organs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

normal orientation of heart called:

A

levocardia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Anomalies of Position Mirror Image Dextrocardia

Type 1

A

sistus inversus totalis

a condition in which the arrangement of the internal organs is a mirror image of normal anatomy.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Anomalies of Position Mirror Image Dextrocardia

Type 2

A

AV (atrial-ventricular) concordance

&

VA (ventricular-arterial) discordance

TGA (transposition of the great arteries) or Dextro-TGA/ d-TGA (dextro-loop occurs)

  • rare
  • creates 2 independent parallel circuits
  • patients show cyanosis
  • systemic flow: RA-RV-AO-body-RA
  • venous flow: LA-LV-PA-lungs-LA
  • rely on PDA, PFO, VSD to get oxygenated blood
  • prostaglandin keeps shunt open
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Anomalies of Position Mirror Image Dextrocardia

Type 3

A

AV (atrial-ventricular) discordance

&

VA (ventricular-arterial) discordance

Atrioventricular and ventriculoarterial discordance, also known as congenitally corrected transposition of the great arteries: Levo-TGA/l TGA/Corrected TGA

  • ventricles are transposed (RV in LV position becoming arterial ventricle & LV in RV position becoming venous ventricle)
  • If no other defects present, heart function normally for 15-20 years
  • eventually systemic ventricle (RV in LV position) will fail
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Anomalies of Position Mirror Image Dextrocardia

Type 4

A

AV (atrial-ventricular) discordance

&

VA (ventricular-arterial) concordance

  • rare
  • inverted heart
  • normally related great vessels
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Most common congenital anomaly involving the _________

A

systemic veins

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Anomalies of Venous Return:

Most common form is the _____drains into the RA via the _______

*Appears as a dilated coronary sinus on PLAX and A4C with inferior angulation

A

left SVC

coronary sinus

*Left SVC draining into LA occurs less often

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

SVC systemic venous return

Contrast injection into the _____will opacify the coronary sinus before the RA and RV. If the same injection leads to left atrial opacification, abnormal drainage of the vena cava is present.

A

left arm

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Anomalies of Venous Return:

_______ occurs when interruption of the IVC;

  • Absence of hepatic segment with azygous vein continuation
  • Absence of the abdominal segment with hemiazygous continuation
  • The IVC draining into the LA

occur

A

Aanomalies of IVC systemic venous return

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

_______ is a condition in which all four of the pulmonary veins are abnormally connected to the right atrium instead of the left atrium. This allows blood high in oxygen to enter the right side of the heart. Blood high in oxygen flows across an ASD, to the left ventricle, allowing some blood with oxygen to reach the body. Without the ASD, blood high in oxygen would not be able to reach the body.

occurs when absence of the right SVC & absence of hepatic segment of IVC

  • blood flows from the IVC to the azygous vein to the left AVC to LA
  • hepatic veins drains into the LA
  • common atrium
A

Total anomalous pulmonary venous return (TAPVR)

or

Total anomalous pulmonary venous drainage (TAPVD)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is TAPVR?

A

Total anomalous pulmonary venous return (TAPVR) is a birth defect of the heart.

abnormal pulmonary veins connection which brings oxygenated blood back to RA instead of LA.

Normally, oxygen-rich blood goes from the lungs to LA and then flows through the body. In TAPVR, an abnormal connection of veins sends blood through the RA instead, where it mixes with oxygen-poor blood. As a result, blood flowing to the body doesn’t have enough oxygen.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

types of TAPVR

A
  • supracardiac - PV drains into SVC
  • intracardiac - PV drains into CS
  • infracardiac - PV drains into SVC

*interrogate and look for confluence of all PV for echo

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Anomalies of the Atria:

PFO found in approximately ____ % of population located in the _______

A

20-25

fossa ovalis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Anomalies of the Atria:

major types of ASD

A
  • ostium primum - fuse with endocardial cushions
  • ostium secundum - most common
  • sinus venosus (inferiro/superior) - least common *also called: SVC (superior vena caval) septal defect/IVC (inferior vena caval) septal defect
  • coronary sinus - rare
  • common atrium
  • atrial septal defect in the septum intermidium
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What type of ASD?

  • Second most common type of ASD
  • Failure of septum primum to fuse with the endocardial cushions
  • Located in the inferior atrial septum
  • associated anomalies include malformation of MV
A

ostium primum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What type of ASD?

  • Most common type of ASD (70%)
  • Located in the midportion of the atrial septum.*
  • Perforations in the interatrial septum
  • Excessive resorption of septum primum
A

ostium scundum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What type of ASD?

  • Least common form (10%)
  • Also known as superior vena caval & superior vena caval
  • not properly incorporated into the RA.
  • Located in the superior and posterior portion of the atrial septum near the junction of SVC/IVC
  • Frequently associated with _partial_ anomalous pulmonary venous return
A

sinus venosus ASD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What type of ASD?

  • Rare
  • Results from a lack of formation of the atriosinus venosus fold
  • Original large communication between atria and sinus venosus is preserved
  • Located between the inferior limbic septum, pars atrioventricularis of membranous septum and IVC orifice close to coronary sinus
A

coronary sinus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What type of ASD?

absence or near absence of the IAS resulting in ine common atrial chamber (rare)

A

common atrium

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What type of ASD?

  • rare
  • Defect occurs as a true persistence of the embryonic foramen primum
  • Located between the limbic septum, pars atrioventricularis of the membranous septum and ccoronaray sinus
A

ASD in the septum intermedium

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Initially left to right shunting due to right ventricular compliance being less that left ventricular compliance. Prolonged shunting can result in the ____shunting with elevated right sided pressures and _____physiology (rare with ASDs)

A
  • right to left*
  • Eisenmenger’s*
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Signs and symptoms (clinical presentation) of ASD

A
  • asymptomatic for many years
  • SOB on exertion
  • recurrent respiratory infections
  • Afib
  • diastolic murmur via TV for a large shunt
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

ASD echo indication

A
  • enlarged RA/RV
  • D shape LV
  • elevated RV/RA pressure
  • PHTN
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

ASD Doppler Assessment

From a low parasternal 4ch, apical 4ch, subcostal 4ch view, and subcostal short-axis view, place color-flow map on ____ gate in atrial septum to look for ______ turbulence

A
  • PW*
  • late systolic early diastolic*
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Qp:Qs ratio

normal ration should be

A

1:1

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Qp:Qs ratio

ratio > 1.0 indicates

A

left to right shunt

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Qp:Qs ratio

ratio < 1.0 indicates

A

right to left

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

PA pressure estimate from TR Doppler signal

peak <30 mmHg indicates

A

normal

*note: Normal resting values are usually defined as a peak TR gradient of 2.8 to 2.9 m/s or a peak systolic pressure of 35 to 36 mmHg, assuming an RA pressure of 3 to 5 mmHg.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

PA pressure estimate from TR Doppler signal

peak 40-70 mmHg indicates

A

moderate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

PA pressure estimate from TR Doppler signal

mean ≥40 mmHg indicates

A

severe

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

osteum Secundum defects are best visualized in the _______

A

subcostal 4ch

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Anomalies of the Atria: Technical Considerations and Additional Views

If there is a ____% difference in saturation, then the patient is thought to have a shunt

A

7

34
Q

Atrial-Ventricular Valve Anomalies:

  • involves a combination of congenital heart anomalies that creates a butterfly appearance
  • common valve in place of the MV and TV
A

atrioventricular canal defect

atrioventricular septal defect

endocardial cushion defect

*all used interchangeably

35
Q

atrioventricular canal (septal) defect/AVCD/AVSD

etiology

A
  • high association with Trisomy 21 (Down syndrome)
  • most cases: idiopathic
  • Abnormal development of endocardial cushions resulting in a primum ASD and membranous VSD
  • Complete AV** canal is the absence of the **inferior portion of the atrial septum** and **posterior ventricular septum** with **a common AV valve
  • Incomplete type** consists of **primum ASD, common atrium, cleft mitral valve
36
Q

atrioventricular canal (septal) defect/AVCD/AVSD

symptoms

A

completer

  • Asymptomatic
  • Heart failure
  • Dyspnea
  • Thin, underweight
  • Recurrent pulmonary infections
  • Tachypnea
  • Anemia
  • Fatigue
  • Excessive sweating
  • Pulmonary edema

incomplete

  • Asymptomatic
  • Fatigue
  • Dyspnea
  • Heart failure
  • Recurrent pulmonary infections
37
Q

atrioventricular canal (septal) defect/AVCD/AVSD

echo assessment

A
38
Q

atrioventricular canal (septal) defect/AVCD/AVSD

  • -*Primum defects
  • -Atrioventricular valve insufficiency*

can be viewed from

A

subcostal 4ch

39
Q

Atrial-Ventricular Valve Anomalies:

What is this?

Malformation of the tricuspid valve with inferior displacement (toward apex) of the posterior and septal leaflets; the anterior leaflet is usually large with abnormal attachments to the RV wall. (Often associated with an ASD. Shunt across ASD may be right to left; therefore, patient may present cyanotic)

A

Ebstein anomaly

40
Q

Atrial-Ventricular Valve Anomalies: Ebstein anomaly

echo assessment

  • Inferior displacement of tricuspid valve leaflet(s)
  • Increased size of RA
  • TV insufficiency
  • M-mode: May demonstrate delay in TV closure from MV closure > ____sec
A

0.05

41
Q

Atrial-Ventricular Valve Anomalies:

What is this condition?

  • During embryology, the right sided venous valves fail to regress resulting in the absence of tricuspid valve and thus, no division between the right atrium and a Hypoplastic right ventricle
  • may be isolated anomaly or can occur with normally related great arteries, transposed great arteries or pulmonary atresia
  • absence of normal RA/RV
  • hypoplastic RV
  • ventricular volume overloading due to both inlets being committed to one ventricle
A

Tricuspid atresia

42
Q

Atrial-Ventricular Valve Anomalies: Tricuspid Atresia

common treatment

A
  • Surgical repair-modified Fontan (direct the systemic venous blood flow to PA without going through a ventricle
  • Bicaval-connects** the SVC to right pulmonary artery and the IVC is connected to underside of heart or RA. This procedure separates systemic and pulmonary venous return, and is designed to prevent ventricular volume overload **** **Aids in reducing degree of cyanosis
43
Q

_________ is uncommon, complex anomaly; critical underdevelopment of the left ventricle due to restriction of the LV inflow/outflow as a result of :

  • mitral valve atresia- inflow reduction; hypoplasia or complete closure (atresia)
  • aortic valve atresia - outflow obstruction, AV hypoplasia/severe stenosis, or coarctation of aorta
  • hypoplasia of aorta - outflow obstruction
  • Small atrial septal defect typically present
A

HLHS (hypoplastic left heart syndrome)

44
Q

HLHS is diagnosed is _____, _______, ______ are present

A

mitral atresia

aortic atresia

hypolasia of aorta

45
Q

HLHS

Small nonfunctional left ventricle due to left sided valve anomaly; Results in _____requiring early intervention to maintain _____and _______.

A
  • cyanosis*
  • PFO*
  • PDA*
46
Q

HLHS patients receive _______ to maintain the shunts.

A

prostaglandin E1

47
Q

HLHS

________ may be performed to create a more pronounced communication between the atria; Multistage surgery is required in order for survival (survival rate still low)

A

Atrial septostomy

48
Q

possible surgical procedures performed on HLHS patients are:

A

Norwood procedure

BI-directional Glenn Shunt procedure

Fontan procedure

49
Q

HLHS echo features

A
  1. Small left ventricle inner chamber with lack of contractility
  2. Valve(s) appearance dependent on etiology of left ventricle Hypoplastic syndrome.
  3. Necessary to evaluate and document the following for surgical planning:
  • ASD –size is recommended to be more that 1⁄2 the length of the atrial septum; if restricted ASD then pulmonary venous hypertension may result; ASD can become progressively smaller usually due to regrowth or superior limbic atrial septum and can obstruct the right upper pulmonary vein).
  • Arch obstruction can be seen at the proximal suture line, distal suture line, or distal to the reconstructive surgery.
  • PA branch distortion
  • Other problems associated with surgical repair of Hypoplastic left heart syndrome: Neoaorta aneurysms; Neoaorta insufficiency; Poor RV function; TV insufficiency; Intracardiac thrombi; Poor systemic flow; Dilated RA and/or RV.
50
Q

HLHS common treatment : staged surgical repair

stage 1

A
  • creation of a new aorta using the pulmonary artery,
  • Blalock-Taussig shunt:
  • the creation of an atrial septal defect
51
Q

HLHS common treatment : staged surgical repair

stage 2

A
  • Bi-directional Glenn
  • hemi-Fontan procedure
  • Aorticopulmonary shunt is closed
  • SVC anastomosed to pulmonary artery
  • IVC blood still mixes in the heart causing patient to be cyanotic.
52
Q

HLHS common treatment : staged surgical repair

stage e3

A
  • completion of Fontan also known as Norwood III.
  • I**VC blood is diverted directly to the pulmonary artery by a modified **Fontan procedure
53
Q

Aortic coarctation typically occur at __________

A

AO isthmus (between the left subclavian artery and and the 1st intercostal artery

54
Q

Ventricular Anomalies: Ventricular Septal Defect (VSD)

What type of VSD?

  • MOST COMMON TYPE OF VSD IN ADULTS (80%)
  • bordered by TV, AoV, and muscle
  • high on the septal wall and closer to the valves and the great vessels
  • thinner, more flexible
A

perimembranous/membranous

55
Q

Ventricular Anomalies: Ventricular Septal Defect (VSD)

What type of VSD?

  • VSD located posteriorly
  • bordered by MV, TV, and muscle
  • often associated with atrioventricular septal defect
A

inlet

  • atrioventricular canal type,*
  • endocardial cushion type,*
  • AV septum type,*
  • juxtatricuspid*
56
Q

Ventricular Anomalies: Ventricular Septal Defect (VSD)

What type of VSD?

  • 5-20%
  • located between the bodies of RV and LV
  • usually low on the septal wall
  • completely surrounded by muscle
  • can be singular or multiple (Swiss cheese appearance)
A

Trabecular or muscular VSD

57
Q

Ventricular Anomalies: Ventricular Septal Defect (VSD)

What type of VSD?

  • 3-5%
  • located between RVOT and LVOT
  • bordered by Aov and PV, and muscle
  • Associated with aortic regurgitation secondary to the prolapse of the right aortic cusp
A

outlet VSD

aka

supracristal

  • conal septal*
  • infundibular*
  • subpulmonic*
  • subarterial*
  • subarterial doubly committed*
58
Q

Ventricular Anomalies: Ventricular Septal Defect (VSD)

What type of VSD?

  • occur in several locations
  • Atrial and ventricular septa do not align normally
  • overriding aortic valve/root
  • Can be a posterior or anterior displacement of the membranous septum
  • Posterior displacement associated with muscular aortic stenosis and/or aortic arch anomalies.
  • Anterior displacement may or may not be associated with RVOT obstruction.
A

Malignant VSD

59
Q

Malignant VSD is associated with:

A

Tetralogy of Fallot

Truncus Arteriosus

60
Q

VSD clinical presentation

A
  • Increased flow across the pulmonic valve
  • Decreased systemic CO2 due to Lt to Rt shunt
  • LA enlargement
  • LV volume overload (LVH, Elevated LVEDP: ^LA pressure & ^pulmonary venous pressure; enlarged LV)
61
Q

VSD Clinical Presentation-Signs and Symptoms

small VSD

A
  • May not have any hemodynamic disturbance*
  • High velocity jet with loud murmur*
62
Q

VSD Clinical Presentation-Signs and Symptoms

large VSD

A
  • Cardiac failure
  • Pansystolic murmur at lower left sternal edge
  • Enlarged LV
63
Q

VSD Qp:Qs ratio

Qp:Qs < 1.5 indicates

A

small shunt

64
Q

VSD Qp:Qs ratio

Qp:Qs 1.5-2.0 indicates

A

moderate shunt

65
Q

VSD Qp:Qs ratio

Qp:Qs > 2.0 indicates

A

large shunt

66
Q

___________is defined as the process in which a long-standing Lt > Rt cardiac shunt caused by a congenital heart defect (typically a VSD, ASD or PDA) causes pulmonary hypertension and eventual reversal of the shunt into a cyanotic Rt > Lt shunt.

A

Eisenmenger’s syndrome

67
Q

ASD/VSD Qp:Qs equation

*note: Qp/Qs = SVRVOT/SVLVOT

A

Qp/Qs = SVRVOT/SVLVOT

= (0.785)(RVOTdiameter)2(RVOT VTI) divided by

(0.785)(LVOTdiameter)2(LVOT VTI)

*note: RVOT/LVOT VTI: RVOT/LVOT planimeter PW waveform

68
Q

At the end of 3rd wk of development, the common trunk divided into the PA and and the aorta. This division consists of a spiral growth process. If there is a disruption in any these mechanism, _______ can result.

A

TGA (transposition of the great arteries)

69
Q

Transposition of the Great Arteries

_____ TGA

  • ventricles are transposed
  • anatomic RV is displaced in LV position with tricuspid valve
  • anatomic LV is displaced in RV position with mitral valve
  • systemic and venous flow is functionally correct = acyanotic
A

Levo-TGA

l-TGA

congenitally corrected TGA

ventricular inversion TGA

69
Q

_____ TGA

  • ventricles are transposed
  • anatomic RV is displaced in LV position with tricuspid valve
  • anatomic LV is displaced in RV position with mitral valve
  • systemic and venous flow is functionally correct = acyanotic
A

Levo-TGA

l-TGA

congenitally corrected TGA

ventricular inversion TGA

70
Q

Transposition of the Great Arteries

Levo-TGA associated findings:

A
  • Ebstein malformation of left-sided AV valve (Tv placed in LV location)
  • PS
  • VSD
  • arrhtymias
71
Q

Transposition of the Great Arteries

_____ TGA

  • abnormal conotruncal septation
  • Ao connected to RV
  • Pa connected to LV
  • 2 independent parallel circuits
  • cyanotic at birth
  • incompatible with life unless surgically corrected
  • dependent on shunts (PFO, PDA, VSD) Need shunt to survive!
A

dextra-TGA

d-TGA

simple TGA

72
Q

What type of surgical procedure?

  • Native pulmonary artery is used to replace aortic valve*
  • Indications: Severe aortic stenosis with Hypoplastic aortic annulus*
A

Ross

73
Q

What type of surgical procedure?

  • Anastomosis between the descending aorta and the left PA*
  • Indication: to increase pulmonary flow*
A

Potts

74
Q

What type of surgical procedure?

  • Anastomosis of the ascending aorta to the right PA*
  • Indication: To increase pulmonary flow*
A

Waterston

75
Q

What type of surgical procedure?

Percutaneous (Transcatheter) atrial septostomy

  • Performed to increase atrial mixing of blood
  • Indications: - TGA

-Tricuspid atresia

A

Rashkind balloon

76
Q

What type of surgical procedure?

  • Valved conduit*
  • -RV to PA*
  • -LV to aorta via VSD and patch*

Performed to increase pulmonary flow or establish flow from ventricles to PA and aorta respectively

Indications:

  • -TGA, VSD, subvalvular PS -TA*
  • -DORV:* Double outlet right ventricle (DORV) is a heart disease that is present from birth (congenital). The aorta connects to the right ventricle
  • -PA*
A

Rastelli procedure

77
Q

What type of surgical procedure?

Reroutes the systemic venous return to the pulmonary arteries

  • Performed to repair: -Pulmonary atresia with*
  • intact ventricular septum - Tricuspid atresia -Univentricular heart*
A

Fontan

78
Q

What type of procedure?

SVC is connected to the right PA

  • Performed to increase PA flow*
  • Indications: -Tricuspid atresia -Pulmonary atresia -Tetralogy of Fallot*
    • Univentricular heart*
A

Glenn

79
Q

What type of surgical procedure?

PA is anastomosed to the aorta

  • Creates conduit from aorta to MPA*
  • Performed to:*
  • -Increase flow to neoaorta*
  • -Stage procedure leading to Fontan*

procedureIndications:

  • -Aortic valve atresia*
    • Hypoplastic left heart syndrome*
A

Norwood procedure

80
Q

What type of surgical procedure?

  • Closed pulmonary Valvotomy
  • Performed prior to balloon Valvuloplast
A

Brock procedure

81
Q

What type of surgical procedure?

Anastomosis of the subclavian artery to the PA

Performed to increase pulmonary blood flowEchocardiographic views utilized to best

  • demonstrate shunt:*
    1. Suprasternal or high parasternal window*
    1. Utilize spectral Doppler to assess gradients*
A

Blalock Taussig