Systemic and Pulmonary Venous Connections Flashcards

TAPVR, PAPVR, Pulmonary vein stenosis, Left SVC

1
Q

What is Partial anomalous pulmonary venous return, PAPVR

A

when one or more, but not all, of the pulmonary veins drain back to the RA

severity of symptoms will depend upon the number of the veins draining anomalously

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2
Q

What atrial septal defect can occur in conjunction with PAPVR?

A

Sinus venosus ASD

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3
Q

Clinical signs of PAPVR

A

in many cases there are no significant symptoms

similar to a small ASD

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4
Q

PAPVR, the right upper PV is commonly found draining indirectly into the RA via

A

abnormal connection where the RUPV drains into the SVC and then into the RA

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5
Q

what are the characteristics of the RV due to PAPVR is

A

RV is often dilated and can become hypertrophic due to the volume overload

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6
Q

PAPVR surgical treatment

what to look for in postop

A
  • Usually does not require treatment if not symptomatic
  • Correct drainage to LA if needed

Post op confirm there is no PV obtrustion and ASD closed

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7
Q

IS TAPVR a cyanotic defect? What makes TAPVR more severe in some cases vs others?

A

**Yes **

If the pulmonary veins are obstructive and the ASD is restrictive there less oxygenated flow getting to the body. Therefore, the baby is more symptomatic and cyanotic than a baby with unobstructive Pulmonary veins and unrestrictive ASD.

VSD are not a typical characteristic of TAPVR but can occur in conjunction with TAPVR

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8
Q

What are the types of TAPVR?

A

Supracardiac TAPVR: all four veins return to the RA above the diaphragm. From the confluence there is a pulmonary vertical vein that connects to the SVC via a innominate vein.
Intracardiac TAPVR: the veins drain to a dilated coronary sinus and drains into the RA.
Infracardiac TAPVR: the four veins drain into a *confluence *behind the LA then descends to enter the IVC and then RA.

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9
Q

What must be present in order for mixing of the blood in TAPVR?

A

ASD or PFO

Must Have right to left shunting, for partially oxgenated blood to get to the left side

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10
Q

The vertical vein in TAPVR is a embryonic remnant of the?

A

Cardinal Vein

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11
Q

what normally drains into the coronary sinus?

A

Coronary veins of the myocardium empty into the sinus and into the RA

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12
Q

During Supracardiac TAPVR where does the mixing of oxygenated and deoxygenated blood take place?

A

SVC and RA

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13
Q

During Infracardiac TAPVR where does the mixing of oxygenated and deoxygenated blood take place?

A

IVC and RA

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14
Q

During Intracardiac TAPVR where does the mixing of oxygenated and deoxygenated blood take place?

A

coronary sinus and RA

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15
Q

Two-dimensional Echo findings from TAPVR

A
  • ASD, Right to Left atrial level shunting “necessary to survive”
  • RVE
  • smallish LA
  • confluence posterior to LA
  • Dilated coronary sinus whale tale (in apical/subcostal views)
  • RAE

Whale tale is the Rt and Lt pulmonary veins dumping into the confluence

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16
Q

Which type of TAPVR is mostly associated with pulmonary vein obstruction?

A

infracardiac

17
Q

Newborn with acute respiratory distress, large right heart, small LA, and right-left shunting at the atrial level most likely has?

18
Q

What other congenital defects are associated with right side enlargement other than TAPVR?

A
  • HLHS
  • Single Ventricle
19
Q

Pulmonary vein stenosis definition

A

when one or more of the pulmonary veins are narrowed or fibrosed

20
Q

Pulmonary vein stenosis can cause

A
  • RVH
  • RVE
  • PHTN

determine RVSP by TR jet to access PHTN

21
Q

How is pulmonary vein stenosis treated?

A

Cath lab can attempt ballooning affected veins, stenting is unsuccessful
-usually poor outcome, death before age of 5yrs

associated with ipsilateral hypoplastic lungs, oxygen therapy needed

22
Q

How does the left superior vena cava normally drain into the RA?

A

via the coronary sinus

hence dilated coronary sinus

23
Q

Persistent LSVC can impact what surgical treatments

A

pacemaker insertion
anesthesia

24
Q

What is a unroofed coronary sinus?

A

Where the coronary sinus normal opening extends across the interatrial septum and drains into both LA/RA

Coronary sinus ASD

25
Q

venous flow velocity usually measures __ m/s

IVC/SVC etc

A

0.5m/sec or less

with PLSVC venouse flow can be even lower at 0.3m/s, turn color scale down.

26
Q

How is the SVC formed?

A

union of the right and left innominate veins

27
Q

What two pulmonary veins can you usually see in AP 4ch view?

A

Right upper and left lower pulmonary veins

28
Q

What pulmonary veins can you usually see in Subcx sagittal view?

A

Right upper pulmonary vein

29
Q

Coronary artery fistula

A

abnormal communication between CA and a chamber of the heart or pulmonary circulation

30
Q

What is the most common coronary artery fistula?
What is it associated with?

A

right coronary artery to the right heart
associated with pulmonary atresia/intact septum

31
Q

What type of doppler does a Coronary artery fistula have?

A

high-velocity continuous flow

32
Q

What is the leading cause of acquired heart disease in the US?

33
Q

What’s another name for Kawasaki

A

Mucocutaneous lymph node syndrome

34
Q

Kawasaki disease is greater in ___

males or females

A

Males
Japanese

35
Q

Clinical signs of Kawasaki

A
  • Fever for 5 days
  • bright red lips, tongue, eyes
  • red, glossy, “strawberry” tongue
  • swollen hands
  • rash, especially in groin area
  • cervical lymphadenopathy
  • peeling skin (hands and feet)
    *
36
Q

Left Main and LAD CA can be seen in

A

PSAX at base
PLAX tilted superiorly to PA

37
Q

Left circumflex CA can be seen in what views?

A

PSAX at base
AP4ch tilted anteriorly
Subcux coronal

38
Q

right main CA can be seen in what views?

A

PSAX-base
Subcx coronal
Subcx sagittal at level of AV groove

39
Q

Infants with ____ defects are at risk of PHTN

What causes excessive blood into the PA

A

Truncus arteriosus
VSD
ASD
AP window
PDA