enlarged RA/RV D shape LV elevated RV/RA pressure PHTN

CHD - Adult Flashcards by Yoshiko Peterson

What is situs solitus?

refers to the normal position of the thoracic and abdominal organs

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normal orientation of heart called:

levocardia

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Anomalies of Position Mirror Image Dextrocardia

Type 1

sistus inversus totalis

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

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Anomalies of Position Mirror Image Dextrocardia

Type 2

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

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Anomalies of Position Mirror Image Dextrocardia

Type 3

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

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Anomalies of Position Mirror Image Dextrocardia

Type 4

AV (atrial-ventricular) discordance

VA (ventricular-arterial) concordance

rare
inverted heart
normally related great vessels

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Most common congenital anomaly involving the _________

systemic veins

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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

left SVC

coronary sinus

*Left SVC draining into LA occurs less often

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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.

left arm

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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

Aanomalies of IVC systemic venous return

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_______ 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

Total anomalous pulmonary venous return (TAPVR)

Total anomalous pulmonary venous drainage (TAPVD)

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What is TAPVR?

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.

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types of TAPVR

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

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Anomalies of the Atria:

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

20-25

fossa ovalis

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Anomalies of the Atria:

major types of ASD

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

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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

ostium primum

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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

ostium scundum

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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

sinus venosus ASD

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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

coronary sinus

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What type of ASD?

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

common atrium

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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

ASD in the septum intermedium

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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)

right to left*
Eisenmenger’s*

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Signs and symptoms (clinical presentation) of ASD

asymptomatic for many years
SOB on exertion
recurrent respiratory infections
Afib
diastolic murmur via TV for a large shunt

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ASD echo indication

enlarged RA/RV
D shape LV
elevated RV/RA pressure
PHTN

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_ASD Doppler Assessment_ ## Footnote *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*

* PW* * late systolic early diastolic*

_Qp:Qs ratio_ normal ration should be

1:1

_Qp:Qs ratio_ ratio \> 1.0 indicates

left to right shunt

_Qp:Qs ratio_ ratio \< 1.0 indicates

right to left

PA pressure estimate from TR Doppler signal peak \<30 mmHg indicates

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.

PA pressure estimate from TR Doppler signal peak 40-70 mmHg indicates

moderate

PA pressure estimate from TR Doppler signal mean ≥40 mmHg indicates

severe

***osteum Secundum defects are best visualized in the \_\_\_\_\_\_\_***

***subcostal 4ch***

_Anomalies of the Atria: Technical Considerations and Additional Views_ ## Footnote ***If there is a \_\_\_\_% difference in saturation, then the patient is thought to have a shunt***

_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

**atrioventricular canal defect** **atrioventricular septal defect** **endocardial cushion defect** \*all used interchangeably

**atrioventricular canal (septal) defect/AVCD/AVSD** etiology

* 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***

**_atrioventricular canal (septal) defect/AVCD/AVSD_** symptoms

_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***

**_atrioventricular canal (septal) defect/AVCD/AVSD_** echo assessment

**_atrioventricular canal (septal) defect/AVCD/AVSD_** ## Footnote * -****Primum defects*** * **-Atrioventricular valve insufficiency*** can be viewed from

## Footnote subcostal 4ch

_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)*

Ebstein anomaly

_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

0.05

_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*

Tricuspid atresia

_Atrial-Ventricular Valve Anomalies:_ **_Tricuspid Atresia_** common treatment

* *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***

\_\_\_\_\_\_\_\_\_ 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

**HLHS (hypoplastic left heart syndrome)**

HLHS is diagnosed is \_\_\_\_\_, \_\_\_\_\_\_\_, ______ are present

mitral atresia aortic atresia hypolasia of aorta

_HLHS_ ## Footnote *Small nonfunctional left ventricle due to left sided valve anomaly;* *Results in \_\_\_\_\_requiring early intervention to maintain \_\_\_\_\_and \_\_\_\_\_\_\_.*

* cyanosis* * PFO* * PDA*

HLHS patients receive _______ to maintain the shunts.

***prostaglandin E1***

**_HLHS_** ## Footnote ***\_\_\_\_\_\_\_\_ may be performed to create a more pronounced communication between the atria; Multistage surgery is required in order for survival (survival rate still low)***

***Atrial septostomy***

possible surgical procedures performed on **_HLHS_** patients are:

Norwood procedure BI-directional Glenn Shunt procedure Fontan procedure

HLHS echo features

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.*

_HLHS common treatment :_ staged surgical repair stage 1

* *creation of a new aorta using the pulmonary artery,* * ***Blalock-Taussig shunt:*** * *the creation of an atrial septal defect*

_HLHS common treatment :_ staged surgical repair stage 2

* ***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.*

_HLHS common treatment :_ staged surgical repair stage e3

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

Aortic coarctation typically occur at \_\_\_\_\_\_\_\_\_\_

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

_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

perimembranous/membranous

_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

***inlet*** * atrioventricular canal type,* * endocardial cushion type,* * AV septum type,* * juxtatricuspid*

_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)

Trabecular or muscular VSD

_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***

outlet VSD aka supracristal * conal septal* * infundibular* * subpulmonic* * subarterial* * subarterial doubly committed*

_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.*

Malignant VSD

Malignant VSD is associated with:

Tetralogy of Fallot Truncus Arteriosus

_VSD clinical presentation_

* *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)*

*_VSD Clinical Presentation-Signs and Symptoms_* small VSD

* May not have any hemodynamic disturbance* * High velocity jet with loud murmur*

*_VSD Clinical Presentation-Signs and Symptoms_* large VSD

* *Cardiac failure* * *Pansystolic murmur at lower left sternal edge* * *Enlarged LV*

_VSD Qp:Qs ratio_ *Qp:Qs \< 1.5 indicates*

small shunt

_VSD Qp:Qs ratio_ *Qp:Qs 1.5-2.0 indicates*

moderate shunt

_VSD Qp:Qs ratio_ *Qp:Qs \> 2.0 indicates*

large shunt

***\_\_\_\_\_\_\_\_\_\_\_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.***

***Eisenmenger’s syndrome***

ASD/VSD _Qp:Qs equation_ \*note: Qp/Qs = SV_RVOT/SV_LVOT

Qp/Qs = SV_RVOT/SV_LVOT ₌ (0.785)(RVOT_diameter)²(RVOT VTI) divided by (0.785)(LVOT_diameter)²(LVOT VTI) \*note: RVOT/LVOT VTI: RVOT/LVOT planimeter PW waveform

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.

TGA (transposition of the great arteries)

_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**

**Levo-TGA** l-TGA congenitally corrected TGA ventricular inversion TGA

\_\_\_\_\_ 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**

**Levo-TGA** l-TGA congenitally corrected TGA ventricular inversion TGA

_Transposition of the Great Arteries_ Levo-TGA associated findings:

* Ebstein malformation of left-sided AV valve (Tv placed in LV location) * PS * VSD * arrhtymias

_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!

**dextra-TGA** d-TGA simple TGA

What type of surgical procedure? * Native pulmonary artery is used to replace aortic valve* * Indications: Severe aortic stenosis with Hypoplastic aortic annulus*

Ross

What type of surgical procedure? ## Footnote * Anastomosis between the descending aorta and the left PA* * Indication: to increase pulmonary flow*

Potts

What type of surgical procedure? * Anastomosis of the **ascending aorta to the right PA*** * Indication: To increase pulmonary flow*

Waterston

What type of surgical procedure? ## Footnote *Percutaneous (Transcatheter) atrial septostomy* * ***Performed to increase atrial mixing of blood*** * *Indications: - **TGA*** *-T**ricuspid atresia***

***Rashkind balloon***

What type of surgical procedure? ## Footnote * 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***

***Rastelli procedure***

What type of surgical procedure? ## Footnote ***Reroutes the systemic venous return to the pulmonary arteries*** * Performed to repair: -Pulmonary atresia with* * intact ventricular septum - Tricuspid atresia -Univentricular heart*

Fontan

What type of procedure? ## Footnote ***SVC is connected to the right PA*** * Performed to increase PA flow* * Indications: -Tricuspid atresia -Pulmonary atresia -Tetralogy of Fallot* * - Univentricular heart*

Glenn

What type of surgical procedure? ## Footnote ***PA is anastomosed to the aorta*** * Creates conduit from aorta to MPA* * Performed to:* * -Increase flow to neoaorta* * -Stage procedure leading to Fontan* *procedure* • *Indications:* * -Aortic valve atresia* * - Hypoplastic left heart syndrome*

Norwood procedure

What type of surgical procedure? * *Closed pulmonary Valvotomy* * • *Performed prior to balloon Valvuloplast*

Brock procedure

What type of surgical procedure? ## Footnote ***Anastomosis of the subclavian artery to the PA*** • *Performed to increase pulmonary blood flow* • *Echocardiographic views utilized to best* * demonstrate shunt:* * 1. Suprasternal or high parasternal window* * 2. Utilize spectral Doppler to assess gradients*

Blalock Taussig