Lai Echo Pulmonary Veins

Pulmonary Venous Anomalies Echocardiography (Lai9)

Check for:

-AbNl # of PVns

-AbNl drainage of PVns

-Stenotic connections

-Anomalous connections

-At 1mo GA, the pulm vns connect to the common PVn, which is then incorp'd into LA

-?Comm PVn dvps fr evagination of sinoatrial region of heart, or fr PVn plexus confluence, or fr a confluence of capillaries growing into the mesocardium bn lung buds and heart

NORMAL

ABNORMAL DRAINAGE

Sinus Venosus Defect

-Anomalous drainage but Nl connections, bc the sinus venosus tissue is unroofed/absent bn the R PVns and SVC or RA (so vn goes to LA, but Q goes cross the defect to RA...

STENOTIC CONNECTION

-in PVns or in LA as cor triatriatum sinister

Cor Triatriatum Sinister

-bc of incomplete Comm PVn incorp to LA

-AS usually intact, and LAA is usually distal to the membrane

-some times it can be "subtotal" in that some vns go to the true LA

Common PVn Atresia

-no communication bn PVn confluence and heart/systemic vns, just see a blind ending pouch

PARTIAL ANOMALOUS PULMONARY VENOUS CONNECTION

-L PVns might go to L innom vn or CS, R PVns might go to cardinal system (SVC/IVC) or to spanch vns

-Vertical Vein- a persistent embryologic vn- connects the PVns to the L innom vn

-or LPVns may go to LSVC or CS or R sided vns like SVC, IVC, Azygous

-RPns might go to IVC (Scimitar syndrome) w assoc R lung hypoplasia

Goals

[ ] PVn #

-subcostal in in infant/young pts

-PS/Subclavicular/Suprasternal in older pts; R parasternal and subs better than L PS for RUPV

-subcostal LA & SA views for RUPV entering LA superiorly, just post to RSVC

-suprasternal notch for crab view to see all 4 PVns in infants

[ ] PVn Connections

-Stenosis- best fr high PS, suprasternal/subs in younger pts

-use PW and CW to check for obstruction

-Nl = laminar, low v, phasic, w short Q reversal during a-systole (retro A wave)

-Obstruction--> no retro A wave, incr v and lose phasicity - hard bc the vn is often parallel...

-can't see PVns distally, but can check fr phtn on R side...

[ ] PVn Drainage

-best at subxiphoid, apical, high PS SA views to see PVn connects to post LA and establish AS plane

-older pts- low PSSA good if subs and Suprasternal not good enough to show septum primum/ASD

-use Doppler for Q...

[ ] Cor Triatriatum Assessment

-see well fr AP4C - curvilinear membrane at mid LA, separting PVns fr true LA

-check at PSSA and Subs to show relation of membrane to LAA and septum primum

-DDx fr malposition of the septum primum, but w Cor Tri the LAA is distal to membrane

-supramitral stenosing ring will bee just above the annulus and less mobile

[ ] Atrial Septum Position/ASDs

[ ] Systemic Vn Connections

[ ] Hemodynamics

[ ] Direction of Flow

[ ] PVn Obstruction?

[ ] Atrial Septal Restriction (by Doppler)?

[ ] Hemodynamic Load (RA/RV dilation, diastolic septal flattening fr RV vol OD, incr Qp)?

[ ] RV or Phtn at TR jet?

[ ] RV dysfx/RV failure?

PAPVC

-the systemic vn distal to the PVn insertion is often dilated

-check for connection to sVC or L innom fr high PSSA/LA, and suprasternal; subs in infants

-must DDx fr Nl syst vns (azygous, superior intercostal vn)

-you won't see Nl connected PVn to the LA in true PAPVC, and the SVC/Innom dilated, and RV vol OD w diastolic septal flattening

-PAPVC to CS best seen fr subs (infants/children) and PS/AP4C w dilated CS but must r/o LSVC too

-PAPVC to IVC- best seen fr subs both SA and LA, check Doppler to show connection to IVC, usually connected just prox to IVC-RA jct

TAPVC

-RA dilation, w atrial septum bowing into LA

-RV dilation w RV htn in addition to vol OD, so IVS bows to LV

-mild LV hypoplasia common

-can't image PVns into LA

-can be hard to see each vn, and check size, course, and drainage

-Supracardiac- vertical vn often to L of midline, Q goes up to jn L aspect of Innom vn usually

-see dilated SVC and L innom vn

-often see well fr subs in infants

-Vertical vn to systemic vn connection seen well fr high PSSA and suprasternal

-usually goes up ant to LPA and LMSB, but check if it goes in bn (+/- obstruction)

-Vert vn might go instead to RSVC or Azygous vn, and vert vn passes bn RPA and trachea/RMSB

-Cardiac- see sev dilated CS - seen best fr subcostal and PS views, but must r/o LSVC and PAPVC

-Infradiaphragmatic- confl usually behind LA, drains to vertical vn down thru esoph hiatus to portal vn system usually, ductus venosus, hep vn, or IVC

-see the confl fr high parasternal and suprasternal notch, and subs for location, size, course

-see obstruction on PW/CW w loss of phasicity and incr velocities

-see phtn on TR jet

-must check the ASD bc it is life sustaining in TAPVC...