Systemic and Pulmonary Venous Connections Flashcards
TAPVR, PAPVR, Pulmonary vein stenosis, Left SVC
What is Partial anomalous pulmonary venous return, PAPVR
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
What atrial septal defect can occur in conjunction with PAPVR?
Sinus venosus ASD
Clinical signs of PAPVR
in many cases there are no significant symptoms
similar to a small ASD
PAPVR, the right upper PV is commonly found draining indirectly into the RA via
abnormal connection where the RUPV drains into the SVC and then into the RA
what are the characteristics of the RV due to PAPVR is
RV is often dilated and can become hypertrophic due to the volume overload
PAPVR surgical treatment
what to look for in postop
- 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
IS TAPVR a cyanotic defect? What makes TAPVR more severe in some cases vs others?
**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
What are the types of TAPVR?
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.
What must be present in order for mixing of the blood in TAPVR?
ASD or PFO
Must Have right to left shunting, for partially oxgenated blood to get to the left side
The vertical vein in TAPVR is a embryonic remnant of the?
Cardinal Vein
what normally drains into the coronary sinus?
Coronary veins of the myocardium empty into the sinus and into the RA
During Supracardiac TAPVR where does the mixing of oxygenated and deoxygenated blood take place?
SVC and RA
During Infracardiac TAPVR where does the mixing of oxygenated and deoxygenated blood take place?
IVC and RA
During Intracardiac TAPVR where does the mixing of oxygenated and deoxygenated blood take place?
coronary sinus and RA
Two-dimensional Echo findings from TAPVR
- 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
Which type of TAPVR is mostly associated with pulmonary vein obstruction?
infracardiac
Newborn with acute respiratory distress, large right heart, small LA, and right-left shunting at the atrial level most likely has?
TAPVR
What other congenital defects are associated with right side enlargement other than TAPVR?
- HLHS
- Single Ventricle
Pulmonary vein stenosis definition
when one or more of the pulmonary veins are narrowed or fibrosed
Pulmonary vein stenosis can cause
- RVH
- RVE
- PHTN
determine RVSP by TR jet to access PHTN
How is pulmonary vein stenosis treated?
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
How does the left superior vena cava normally drain into the RA?
via the coronary sinus
hence dilated coronary sinus
Persistent LSVC can impact what surgical treatments
pacemaker insertion
anesthesia
What is a unroofed coronary sinus?
Where the coronary sinus normal opening extends across the interatrial septum and drains into both LA/RA
Coronary sinus ASD
venous flow velocity usually measures __ m/s
IVC/SVC etc
0.5m/sec or less
with PLSVC venouse flow can be even lower at 0.3m/s, turn color scale down.
How is the SVC formed?
union of the right and left innominate veins
What two pulmonary veins can you usually see in AP 4ch view?
Right upper and left lower pulmonary veins
What pulmonary veins can you usually see in Subcx sagittal view?
Right upper pulmonary vein
Coronary artery fistula
abnormal communication between CA and a chamber of the heart or pulmonary circulation
What is the most common coronary artery fistula?
What is it associated with?
right coronary artery to the right heart
associated with pulmonary atresia/intact septum
What type of doppler does a Coronary artery fistula have?
high-velocity continuous flow
What is the leading cause of acquired heart disease in the US?
Kawasaki
What’s another name for Kawasaki
Mucocutaneous lymph node syndrome
Kawasaki disease is greater in ___
males or females
Males
Japanese
Clinical signs of Kawasaki
- 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)
*
Left Main and LAD CA can be seen in
PSAX at base
PLAX tilted superiorly to PA
Left circumflex CA can be seen in what views?
PSAX at base
AP4ch tilted anteriorly
Subcux coronal
right main CA can be seen in what views?
PSAX-base
Subcx coronal
Subcx sagittal at level of AV groove
Infants with ____ defects are at risk of PHTN
What causes excessive blood into the PA
Truncus arteriosus
VSD
ASD
AP window
PDA
