Pulmonary Venous Anomalies (M/A)

Pulmonary Vein Anomalies (MA37)

Anatomy:

-anomalous connections, anomalous drainage w Nl connectn, stenotic connections, or abNl # of pulm vns

Anomalous Connections & Drainage

-Pulm vn(s) connect to systemic vein(s)

-Total Anomalous Pulmonary Venous Return = all the vns are anomalous

-Partial Anomalous Pulmonary Venous Return = only some vns are anomalous

-Partial/Total Anomalous Pulm Venous Drainage-may have Nl connection w anom drainage (PVns to LA but atrial septum is off)

Stenotic Connections

-vary fr individual stenosis to cor triatriatum --> obstruct Q to LA

Anomalous Number of Pulmonary Veins

-might have a single pulm vn on either side (24% prevalence)

-rarely see a common pulm vn to LA

-rarely see a third pulm vn on either side

Embryology:

-lungs, larynx, tracheobronchial tree are derived fr division of the foregut.

-early on, the lungs get enmeshed in the foreguts vascular plexus (splanchnic plexus), and form the pulm vasc bed later on; without direct connection to the heart. They instead connect to the splanchnic plexus (umbilicovitelline & cardinal systems of vns).

-Then, the intraparenchymal pulm vns connect w LA to -- connection w a common pulm vn

-Then it invaginates fr the LA (as LA grows, it incorps the common pulm vn... ...??)

-the common pulm vn may evag fr LA or come fr the 4pulm vns, ? known

-after connection of pulm vns to heart, the connections to the splanch circ are lost

Early Atresia of the Common Pulm Vein while the Pulm-Syst Venous Connections are still present

-If comm pulm vn fails to dvp or gets atretic early, --> collat channels for pulm vn drainage form fr primitive connections bn splanch plexus and cardinal or umbilicovitilline system, then one of these can persist/enlarge--> TAPVC. If only the R or L part of common pulm vn is atretic, then --> PAPVC

Late Atresia of the Common Pulm Vein after Pulm-Syst Connections are Obliterated

-If the comm pulm vn gets atretic after the pulm-syst connections are lost, then --> atresia of comm pulm vns, and the Qp vn darinage goes into blind cul-de-sac w/o connection to LA or systemic vns.

Stenosis of the Common Pulm Vein

-Cor Triatriatum - bc of comm pulm vn stenosis. usually occurs late, after collat vns connections are lost, but sometimes is assoc w anom pulm vn connection

Anomalous Incorporation of the Common Pulm Vn into the LA

--> abNl # of pulm vns to LA (too many/few)

Normal Absorption of the Common Pulm Vn w Partially or Totally AbNl Pulm Vn Drainage & Nl Connection of the Pulm Vns

-Sinus Venosus Defects- --> atrial level shunt, but they're not ASDs

-always assoc w drainage of some/all R pulm vns into R SVC or RA

-the issue is due to defic in wall bn R SVC and the RUPV or wall bn RA and RUPV and RLPV

--> unrof the RUPV and branches into the R SVC, or unroof RUPV and RLPV into RA

-the interatrial commun'n isn't a defect (bc it uses the os of the pulm vn)

-see diagrams...

-Malposition of Septum Primum

-sept secundum (often w heterotaxy pt) is displaced twd the morphologic LA--> incorp pulm vn(s) into RA, so the connection is Nl but septum is moved over...

PARTIAL ANOMALOUS PULMONARY VENOUS CONNECTION

Anatomy:

-L sided pulm vns usually connect to derivatives of L cardinal system- CS, L innom vn

-R sided pulm vns usually connect to derivatives of R cardinal system- SVC, IVC

-splanchnic plexus is midline so allows for cross drainage of L PVns to R sided system and vice versa

-Excluding PAPVC to RSVC/RA (now not c/s APVR but instead called sinus venosus defect or malposition of septum primum)

-Most common type of PAPVC is the L pulm vn to L innom vn

-2nd most common type is R pulm vns to IVC

R Pulm Veins to R SVC

-may drain to R SVC or RA without abNl connection (bc of sinus venosus defects)

-R upper lb pulm vn opens to SVC w one or more smaller vssls, multiple orifices, enters below azygous, while the R lower lb usually sends pulm vn to LA.

-see dilated lower SVC (bn azygous vn and RA)

-usually see interatrial communication (LA orifice of unroofed pulm vns)

-+/- secundum ASD or (rarely) primum ASD

-if RUPV orifice is atretic, then see an intact atrial septum (???why??)

-+/- LSVC

-some times see just a single pulm vn connected to RSVC +/- sinus venosus defect (fr R upper lb to RSVC above the azygous vn)

-all cases have mild-mod dilated and hypertrophy of RA and Rv and dilated PA; Nl L heart

R Pulm Vns to IVC

= Scimitar Syndrome = assoc w other anomalies- R lung hypoplasia, bronchial system xx, horseshoe lung, secondary dextrocardia (2y to the lung hypoplasia...), RPA hypoplasia, anomalous arterial connection fr Ao to R lung, pulmonary sequestration

-can enter above or below diaphragm

--> change respiratory pattern of pulm vns in the R lung = "fir tree" configuration

-usually no ASD

L Pulm Vns to IVC

-rare

-+/- ASD

L Pulm Vns to L Innominate Vn

-usual place for anom LPVn to connect to

-connect via a persistent early embryologic pathway, the vertical vein aka persistent LSVC but this is INCORRECT embryologically and anatomically!, bc the vertical vn is actually the connection bn the splanch plexus in the lung buds and the cardinal vns, , and anatomically the vertical vn is more post than LSVC, which is just behind the LAA, and an LSVC connects w either the CS or LA (if CS is unroofed). The L pulm vns may connect to the true LSVC, but then the LSVC would still drain into CS or LA (not the inomm vn). You must verify pre intervention if it is a vertical vein (going to inomm vn) or an LSVC (going to CS/LA) to Tx correctly.

-usually +ASD

Other

-Rarely L PVns can go to CS, IVC, RSVC, L subclavian vn, azygous

Physiology:

-similar to an ASD: --> incr Qp bc you recirculate Q sent to lungs in the first place

-Sx d/p # vns that are anomalous, where they connect, and if there's an ASD/ASD size

PAPVC w Intact Atrial Septum:

-Amt of Q thru the anomalous vns d/o # of vns, and relative resistance of the vasc beds of the Nly vs anomalously connected vns, and compliance of each atria relative to each other, and if there is obstruction to Qp flow at PAs

-if just a single anom PVn, then there's about 20-25% anomalous Q fr lungs, so rarely symptomatic, with no dilation/hypertrophy of R hrt

-if 3 PVns are anomalous, then then about 80% of pulm Q, and Sx are like TAPVC

-if only one vn has anomalous PVn connection, then the relative pulm resistance and the relative atrial compliance (of the receiving chamber) relative to the other side, determine the relative Q.

-e.g. w Scimitar syndrome (R pulm vns to IVC) there is also pulm parench xx w incr pulm art pressures, so less Q to the R lung, so less impact of the anomalous pulm vns.

-but if the only xx is the anomalous veins fr one lung, then much more Q will go to the anom vns, w Q more likely to go to RA than LA bc RA more compliant

-SO, if PAPVR is the only xx (...assuming PVR in ea lung is =), then bc RA is more compliant than LA, blood flow will be greater to the lung w anomalous PVns

-The lobe that the anom PVn drains also affects amt of Q thru it, and thus amt of L-->R

-When upright position, Qp prefers the middle/lower lobes; in supine/exercise it spreads to upper

--> amt of L-->R also d/o both which PVn and pt posture & activity level

PAPVC w ASD

-small ASD--> similar to as if no ASD

-big ASD--> major impact: with an ASD alone (without PAPVC) the Right lung tends to shunt Q thru ASD more than the L lung bc they are close to the ASD. If there is also PAPVC, Sx can be just like w an ASD. L-->R might be large bc of both the PAPVC shunt and the ASD shunt (at least half of the bld draining into the LA is going to shunt to RA thru the ASD).

-Usually also have some small R-->L if sinus venosus defect present, fr SVC bld- made of both systemic vn bld and PAPVC bld; and fr IVC (as expected for an ASD).

-Phtn is not common but some pts get pulm vasc dz at older age.

Sx/Si:

-single anom PVn--> ASx

-3 anom PVn--> similar to TAPVC

-2 anom PVn fr same lung--> usually ASx, sometimes DOE

-usually no cyanosis as child, even w the small R-->L, d/o PAPVC location, but in 20s and onward can get phtn fr incr Qp-->more R-->L so incr number pts w cyanosis

-Scimitar syndrome pts have varied Sx

-in one series, most infants had sev Sx w phtn bc of Qp fr Desc Ao, stenosis of the anom PVns, pulm parench xx, L heart/Ao obstruction xx.

-common to see R lung hypoplasia w dextrocardia

-some present later w few Sx, and ~Nl pulm pressure,

-if ASD, then Sx just like uncomplicated ASD

-if no ASD, S2 isn't very split, and has Nl variation, but do often hear pulm outflow murmur and +/- diastolic rumble fr tricuspid

Assoc xx/Syndromes

-assoc w visceral heterotaxy, polysplenia (sx 2y to malposition of septum primum), pts w asplenia have more TAPVR.

-?assoc w Turner and Noonan syndrome

Dx:

ECG- like w uncomplicated ASD - rR' and rsR' but may be Nl

-peaked P waves and RVH bc of systolic OD pattern if phtn

CXR- incr Qp w RV dilation

-Scimitar syndr (RPVns to IVC)--> crescentlike shadow at R lower lung field, along w hypoplastic R lung and chest, mesocardia or dextrocardia

-If vns drain to SVC, then see dilated lower SVC, just above RA contour

-if connect via azygous vn, then see it enlarged as a round bulge in the R superior mediast at R hrt border

-If vns drain to L innom vn--> see prominent supracardiac shadow of vertical vn on the L, the innominate vn above it, and teh SVC at the right. (this is what--> "snowman" look of TAPVC)

Echo-

-must get comprehensive echo to dx it!

-ensure u check for each pulm vn on each pt by 2D and doppler

-ensure you see RUPV, RLPV, and LLPV connecting to LA on 2D and color

-check on subcostal, apical, parasternal, suprasternal notch windows

-check closely if you have a pt w RV vol OD but no ASD!!

-Dx by seeing pulm vn connect w a systemic vn

-see syst vn gets dilated distal to where pulm vn connects.

-to SVC or innom: check at suprasternal or high PSLA

-to CS: check by scanning posterior L AV groove in subs/AP4C/parasternal (c/s if dilated CS vs LSVC)

-to IVC: subcostals, check for meso/dextrocardia if scimitar syndrome (70%+ dextro/meso)

MRI - good for Dx...

Cath - usually not needed except to DDx fr ASD and to estimage amt of L00>R

Tx:

-Rx: for heart failure if needed for incr Qp

-Surgery when pt shows pulm overcirc Sx

PAPCC Draining to RA

-2y to Malposed Septum Primum:

-if AV concordance to get biventric repair, then excise atrial septum and make a new one to incorp PVns into LA

-if AV discordance but still to get biventric repair, then baffle pulm vns to the systemic ventricle and systemic vns to pulm ventricle

-if planning single ventric repair, then the septal position isn't relavent, bc pt will get Glenn/Fontan

-2y to Sinus Venosus Defect

-Close defect bn SVC and RPVn with a suture or patch

-Add 2nd patch to enlarge the SVC

-Warden procedure- divide SVC and azygous vn above entrance of pulm vns, then sew the cranial end of the SVC to the RAA, then oversew the cardiac end of the SVC (containing the pulm vn connection), and use an atrial septal patch to include the SVC (w pulm vns) drainage into the LA thru the ASD

PAPVC to IVC

-divide the anom PVn at where it connects to IVC and anastomose it to RA, then make an ASD if not already there and use a patch to direct the Q to LA. xx-obstructing the vn Q

PAPVC to CS

-repair as you would for TAPVC to CS (see below)

PAPVC of LPulm Vns to Innominate Vn

-Kirklin-->anastomose common LPVn to base of the amputated LAA

Px:

-Untreated- limited info, good Px if only 1 vn, remainder like an ASD

-Treated- d/o pulm vasc bed at time of surgery- if high Qp, then much improved Sx, and ~Nl survival, but can't be so sure of outcome if pt had pulm vasc dz

-must follow for pulm vn obstruction and arrhythmias postoperatively

-obstruction fr occlusion of APV and can --> no Q to the affected lung, see on V/Q testing, echo, MRI, angio.

-obstruction fr SVC/IVC stenosis which can be seen on angio and Tx w stent

TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION/DRAINAGE

Anatomy:

-Type I = Supracardiac

-Type II = Cardiac (e.g. to CS)

-Type III = Infracardiac

-Type IV = Mixed (2 or more of the above)

-can also classify as supracardiac w/o pulm vn obst, and infradiaphragmatic w pulm vn obst

-TAPV drainage to RA 2y to malposed septum primum - usually assoc w visceral heterotaxy and other cardiac xx

- >1/3 pts have TAPVC to L innom vn

-Must have ASD/PFO to stustain life (!)

-most usually have a PDA

-+RA/RV dilation & hypertrophy, and dilated PA

-Nl LV size; LA is small

Connection to R SVC or R Azygos Vein

-PVns--> confluence behind LA, then vssl comes off R side and goes ant to R lung hilum to enter back of the R SVC (rarely it connects to R azygos instead)

Connection to L Cardinal System

-Connection to L Innominate Vn

-PVns--> confluence post to LA, then vssl fr L side passes in front of LPA and LMSB and then goes to superior mediastinum, going ant to Ao Arch, and joins the L innom vn, proximal to where it <-- fr L jugular and L subclavian vns.

-the Ascending vn might instead pass bn LPA and LMSB which --> obstruct Q in pulm vn

-the vssl connecting the PVn to L Innom vn is an anomalous vertical vn that was the channel bn the embryologic splanchnic plexus and the cardinal venous system (thus it is NOT a persistent LSVC as some would call it; a persistent LSVC would connect the CS or LA if CS was unroofed w the systemic vns)

-Connection to CS

-PVns join a common vssl--> connect to CS in the AV groove--> CS then goes to RA as NL

-see bigger CS orifice in Nl place (bn orifice of IVC and TV) or displaced posterior to IVC os

-Cardinal vns drain as Nl to CS

-rare to have stenosis at jct bn PVns and CS

-Connection to Umbilicovitelline System

-PVns--> confluence just behind LA--> vssl going down just in front of esoph, then thru diaph at the esoph hiatus, and then joins portal vn at confluence of splenic and sup mesenteric vns

-might instead join ductus venosus, to a hepatic vn, or to IVC

-PVn obstruction is common here

Sites of Obstruction to Pulm Vn Drainage:

-Obst at Atrial Septum- ASD size predicts Px- if large--> survive longer

-Ostr at Anomalous Vns Channel-

-bc of narrowing in the walls of the vessel or bc of extrensic pressure onto the vssl

-e.g. vertical vn bn PVns and Innom goes bn LPA and LMSB--> obst the vertical vn

-e.g. annom pn bn PVns and SVC can be obstructed by RPA and trachea

-ductus venosus constriction postnatally can --> obstruction if PVns connect to it

-if connect to portal vn/vns draining to portal vn, then get obst fr the hepatic sinusoids (which lay bn the PVns and the IVC...)

-Length of the vertical vn can also affect resistance to PVn Q

Associated Cardiac Anomalies

-May have TGA, TOF, single ventricle, TrArt, TriAtresia, HLHS, pulm atresia, etc.

-TAPVD to RA occurs in pts w visceral heterotaxy and polysplenia (as a rule)

Microscopic Anatomy

-Anomalous vssls- much fibrosis at adventitia +/- media, wall atrophy, intimal atrophy or media-adventitia atropy. If obstructed vn, then see media-adventitial thick w intimal prolif.

-LA- atrophic LA

-Lungs-

-if no obstruction, see prominent medial hypertrophy of the arteriesarterioles (incr Qp), and then intimal xx as child ages

-if + obstruction, see medial hypertrophy within the anomalous vns, extrapulmonary vns and in the smaller lung vns. +pulm edema, RBC extravasation into alveoli, dilated subpleural & interlobular lymphatics, and much medial hypertrophy of pulm arts/arterioles.

Physiology:

-all PVn Q returns to RA, so must have interatrial communication to survive

-as fetus, only slight incr in Q to RA bc most of the blood didn't go to lungs/PVns anyway

--> no stimulus to dvp large ASD

-70-80% have some restriction at PFO; this can --> decr Qs

-get massive pulm overcirc as PVR drops and as systemic demands for Qs increase

-the incr P in RA --> both congestion at systemic vns w HSM etc, and PVns w pulm edema etc

-if the ASD/PFO is wide, then --> better mixing, w amt of mixing d/o relative compliance of the atria and ventricles, and the relative PVR:SVR

-the PVR is the major variable to Q

TAPVC without Pulm Vn Obstruction

-birth- PVR=SVR so Qp=Qs, as PVR drops then Qp incr w Qp 3-5x Qs often; Qs is usually Nl

-SO2 of RA can be >90% bc the blood in is is 3-5x more fr pulm vns than fr systemic circ..., and blood mixes well in RA, so the RV and PA, and LA, LV and Ao have = SO2 as RA

-systemic SO2 varies much, d/o type of TAPVC bc of streaming of the mixed bld

-may see progressive RVH and dilation, along w PA too

-PAP can be slightly high to systemic levels

-the ASD/PFO in pts w TAPVC and no pul vn obst has huge impact on amt of Qp, PAP, and PVR (bc big ASD--> shunt to L side much at atrium so less Qp, better situation...)

TAPVC with Pulm Vn Obstruction

-the high P at PVn gets transmitted back to capillary bed--> pulm edema when P hydrostatic > P osmotic at capillaries. Pt tries to prevent this w incr pulm lymphatic Q, dvping alternate pulm vn channels, changes to cap wall permeability, and reflex PA contriction (which -- decr Qp, but also phtn w RVH fr RV htn --> R heart failure!!)

-RV vol and P OD--> shift IVS leftward--> decr LV volume even more (even more than just dec Q to LA)--> decr systemic CO

Sx/Si:

TAPVC without Pulm Vn Obstruction

-Sx

-ASx initially- of 74 pts in a study, only 56% had Sx at <1mo, all had Sx by 1yo

-incr RR, poor feeding, usually in first few weeks of life (as PVR drops, less R-->L at PFO)

-FTT, recurrent resp infection, and then cardioresp failure by 6mo usually

-Cyanosis- can be mild/inapparent, espec in pt w cardiac failure and in pt who survives long enough to get pulm vasc dz

-if pulm vsc dz, then 75-85% die by 1yo (most by 3mo)

--> -thin, dusky w crying/exertion, dyspnea, tachycardia...

-Prominent RV heave

-multiple cardiac sounds- loud S1 w systolic ejection click often; S2 widely split and w/o resp variation, S3 (loudest at apex) is ~always there. S4 common in older pts.

-might not have a murmur, but usually has 2/6 soft blowing pulmonic SEM fr turbulance oat pulm outflow tract or fr TR; +tricuspid diastolic flow murmur at LLSB common,

-+venous hum if anom PVn is connected to L innom vn- hear at L or R base of hrt, but this isn't louder during diastole like w a benign venous hum, and isn't changed w change in position/neck pressure

-Heart failure by 6mo- hepatomegaly in all pts, periph edema in 1/2, clubbing if >1yo

-ECG- tall P wave in II & R precordials bc RAE; often RAD; always see RVH w high R precordial ld voltages

-CXR- incr pulm markings, big RA and RV, big PA segment. No enlargement of LA/LV.

-figure 8 or snowman look- if TAPVC to L innom vn (top part of snowman is the anom vert vn to L and L innom vn superiorly, and SVC on R.

-usually not seen until pt is a few months old, common as child/adult

-if TAPVC to SVC, see dilated SVC w prominence of upper R heart border

-Echo-

-Goals:

-Dx type of TAPVC,

-check size of ea pulm vn,

-check that all 4 PVns join confl. & that no additional pulm vns drain separately (aside fr the 4 PVns),

-check size of confluence and relation to LA,

-check course of the anom pulm vns channel (vertical vn or horiz vn) and its connectn to systmc vn

and its relation to other structures (trachea, LMSB, PAs etc)

-check for obstruction to pulm vns Q, and mechanism

-check the interatrial communication, r/o obstruction

-complete heart check for fx/other xx

-see RV OD in all types w RA/RV dilatn, atrial septum bows to L, RV compress LV w IVS to L, decr LV vol

-see big PAs

-see that you can't find PVns in the LA --> c/s TAPVC

-see small LA

-see the confluence as an echofree space behind LA, then look for each pulm bn (2D & Doppler each)

-check course/diameter of each - PVn size at Dx --> Px

-check ea vn fr each window- parasternal, subclavicular, suprasternal notch

-check size and orientation (horiz vs vertical) of the confluence and relation to LA (for pre-op plan)

(if they connect to confl at same level, near LA, and horiz vn--> easier repair)

-Then, see venous channel that connects to confluence to the systemic vn, 2D & Doppler

-Supracardiac - best in precordial windows; Infradiaph- best w subs

-check relation of the venous channel to the PAs and bronchi (looking for obstruction)

-check for stenosis where it connects w the systemic vn (common there)

-No obstruction--> low velocity, phasic laminar flow w brief flow reversal at atrial systole

- + obstruction--> incr flow velocity, turbulent Q pattern, loss of phasic variation

-If TAPVC to CS, see CS dilation on subcostal and PSLA and AP4C- dilated CS bulges ant-sup into LA

(but DDx = LSVC to CS)

-If TAPVC Infradiaphragmatic- usually goes to portal system, also hepatic vns

-pulm vns form a common channel that is inf to the LA just above teh diaphragm, and may look like its own chamber

-best seen fr SCSA and SCLA, scanning fr L to R and sup to inf to ID the abdoml conectn

-Transverse plane: desc Ao is L and post, IVC R and ant to the channel

-might miss the channel if compressed by a big liver

-DDx the abd vssls via Doppler:

-Ao--> systolic laminar flow directed away fr heart

-IVC--> continuous phasic flow twd the heart

-PVn channel--> venous flow pattern directed AWAY fr heart, twd abd.

-c/s Mixed TAPVC, so look for each.

-connection to CS and L innom vn is the most common type

-MRI- great if you can't see things well enough on echo

-can tell anatomy and direction of Q and flow velocity

-Cath- not needed usually, but angios can make accurate Dx

-would see RV and PA pressure slightly high to suprasystemic

-hard to interpret atrial pressures, especially if trying to decide if ASD/PFO is big enough bc if they are equal, that's not reliable to say there's enough shunting, but rather they're equal bc compliance of each lung is equal. Though RAP>LAP by >2mmHg is relatively reliable to tell you there's restriction

TAPVC with Pulm Vn Obstruction

-Usual if connected to umbilicovitelline venous system

-Half of supracardiac pts have obst.

-less common w connection to CS

-Sx

-Most (3/4) present at <1mo, the rest by 1yr

-Sx after 12 hrs old, (so DDx's pt fr RDS)

-then rapid dyspnea, poor feeding, cardioresp failure

-die by 2 days to 4.5months w/o Tx

-if infradiaphragmatic connection, --> cyanosis/dyspnea worse w straining/swallowing bc of incr intra-absominal P or esoph impinges on the common PVn as it goes thru esoph hiatus of diaphragm

-can rapidly --> sev resp distress/failure within hours of life

-may have minimal cardiac findings, w a Nl sized heart, no signif RV heave, Nl S2, but accentuated P2.

-Often no murmur, and if + murmur then it is a soft blowing pulmonic murmur

-+rales at bases; always has hepatomegaly and often periph edema

-ECG- RVH, but no RA enlargement (unlike if no obst)

-Echo- as above, check each vn for obstruction, espec before insertion...

-CXR- Nl or near Nl, if abNl then see diffuse stippled densities that --> reticular pattern that fans out fr hilar regions. obscured heart borders. Kerley B lines, prominent superior pulm vns

-Cath- rarely needed

-be careful interpreting oximetry at IVC and SVC- while Qp is decr so the amt of Qp might not allow for high SO2 after mixing PVn Q w systemic vn Q, there is also streaming effects of renal bld mixing w IVC bld can cause higher SO2.

-RV P systemic to suprasystemic while RA P usually NL; LA P Nl but much lower than the elevated pulm art wedge pressure!!

-Angios- check site of obst...

DDx:

-TAPVC without PVn Obst:

-large VSD, PDA, TrArt, AVSD, single ventricle w/o pulm stenosis

-but each of these would have CXR and ECG showing LA and LVH, would not hear multiple heart sounds, and usually have a harsh cardiac murmur w thrill

-older kids w TAPVC DDx: ASD, common atrium, PAPVC

-TAPVC with PVn Obst

-pulm vn obstruction, persistence of the fetal circulation

Tx:

-correct ASAP

-support w mech vent, inotropes, diuretics, etc

-may need to do a BAS, but really you should just go to the OR

TAPVC to CS

-excise wall bn CS and LA to allow PVns to drain into LA via CS

-then close CA orifice in RA w suture/patch, and close ASD

-if needed (rarely) correct any stenosis bn PVn and CS

TAPVC to L Innom Vn

-Connect the LA to the pulm vn confluence w anast. then close ASD and ligate the site of the anomalous connection to the L Innom Vn.

-connect LA to confl w side-to-side anast bc an end-to-side anast often kinks

-don't connect it to the LAA bc the LAA diameter is too small, smaller than the comm pulm vn

Px:

-Untreatedd/o ASD/PFO size

-1/2 dead at 2mo, 80% by 1yo; worse Px if restricted intraatrial communication

-bad Px if obstructed PVns, w death in first fwe weeks of life

-Postop-

-long term Px d/o state of pulm vasc bed at time of operation, and adequacy of the PVn-LA anast.

-of 30 infants operated on in 1980s, 2 died late bc of phtn, 2 reoperations for PVn obst

-13% hospital mortality in 1980s, fr phtn in all four deaths

-operative mortality not effected by TAPVC type, pt age/wt, CPV duration etc

-it was affected by preop status and post op PAP

-4pts had late PVn obst

-residual stenosis in 8/68 pts in another series

-about 10% got PVn obst later in a diff study (Sano)

-a pt w SVC obst had SSS and needed a pacemaker

-Some pts had late arrhythmias- usually atrial w sinus brady, a-flutter, SVT

ATRESIA OF THE COMMON PULMONARY VEIN AFTER THE PULMONARY-SYSTEMIC CONNECTIONS ARE OBLITERATED

-no connection bn the PVns and the LA, and there is no anomalous connection bn the PVns and the systemic vn circulation either ==> severe obstruction to pulmonary vn Q

-rare

Anatomy:

-absent fx'l connection bn PVns and LA or other cardiac chamber/systemic vns

-the 4 PVns converge into a confluence just behind LA--> cul-de-sac, w no outlet for PVn Q

-lungs are firm and congested, and the pleural surfaces of the lung lobules have edematous interlobular tissue and dilated lymphatic channels

-pulm vns are thick walled w medial hypertrophy, and PAs also have medial hypertrophy

-subpleural/interlobular lymphatics are very dilated/edematous

-alveoli have many RBCs and Fe containing macrophages

Physiology:

-sev obst to PVns--> much decr Qp w R-->L at PFO and DA

-there must be some exit to the lungs bc pts do live days/weeks postnatally - ?bronchiopulmonary veins bring Q to systemic circ...

Sx/Si:

-dyspnea, cyanosis just after birth

-death by 1mo

-no thrill; +/- 1-2/6 soft syst murmur at LSB

-ECG Nl or RH

-CXR- sev pulm vn obstruction w incr pulm vasc markings w diffuse reticular pattern

-no cardiomegaly

-Echo- see common pulm vn atresia

-RA, RV, PA dilation, small LA

-PVns don't return to LA; confluence seen w/o exit...; can't get good PVn Q on doppler

-R-->L at PFO/PDA bc of phtn

-Cath- check severity of phtn, see marked systemic desaturation, see contrast into RV doesn't go much to the lungs

Tx:

-can connect the confl to LA if anatomically do-able

Px:

-Sx in first days of life, then death by 1mo if no surgical Tx. ? outcome of surgery bc too few have been done.

STENOSIS OF THE COMMON PULMONARY VEIN

Cor Triatriatum:

= PVns enter an accessory chamber that joins LA via narrow opening; or the accessory chamber goes to RA directly/indirectly via a channel.

-Uncommon

-Varied anatomy, hard to ID embrogologic etiology: most say it's bc LA fails to incorp the common PVn, but some variants are inconsistent with this.

-Cor Triatriatum Dexter = persistence of R valve of the sinus venosus (a diff issue not addressed here)

-this is Cor Triatriatum Sinister (LEFT sided issue)

Anatomy:

-Accessory LA Chamber Receives all PVns & Communicates w LA

-No other connections - = classic version

-see a windsock shaped membranous partition separating proximal chamber getting the PVns and the distal LA going to MV. the windsock is directed twd the MV. Orifice diameter 3-10mm

-+/- some small defects in the septum

-may have calcified muscle fibers

-True LA communicates w the LAA, always has the FO lying bn it and the RA

-usually NO PFO or ASD

-see RVH and RV dilatino, and RA hypertrophy/dilation in many

-With Anomalous connections

-may communicate w more distal true LA via a stenotic opening, and have comm'n w RA thru a defect in the shared wall..., or the accessory LA chamber can communicate w RA thru an anom vn

-Accessory ATrial Chamber Receives All PVns & Does NOT Communicate w LA

-stops PVn Q to LA. So there's a defect bn the accessory LA chamber and the RA, and then the PFO sends the Q to the LA to go to MV

-Subtotal Cor Triatriatum

-Accessory atrial chamber gets part of the PVns and Connects to LA...

-Microscopic Anatomy- pulm edema, intra-alveolar hemorrhage, medial hypertrophy of PVns, lymph channel dilation, PA lesions- medial hypertrophy +/- intimal prolif to necrotizing arteriolitis

Physiology:

-like TAPBC if they drain directly/indirectly to RA, but if there's no alternative pathway then the stenotic opening in LA --> incr PVn P--> Sx like pulm vn obstruction

-if subtotal cor triatriatum, then Sx only affect 1 lung--> reflex pulm art constriction affects the one lung--> decr Qp to that lung, but the other lung then gets incr Qp, but is okay ==> neither lung has incr in PAP...

Sx (for classic cor triatriatum):

-Sx at 1 week old usually, but may be ASx until teens or 20s

-h/o SOB, freq resp infections, often thought to have a primary lung dz

-phtn- loud P2, RV heave, Pulm syst ejection click

-soft blowing systolic murmur at LSB, +/- diastolic murmur at mitral area/continuous murmur

-R sided heart failure

-crackles

-Echo

-see it on echo well, in parasternal, subs, apical

-check for assoc xx- TAPVC, CoAo, HLHS, critical AS

-DDx supravalvar stenosing ring of the LA--> PVn obst w membrane in LA

-Cor Triatriatum membrane is more curvilinear w windsock look, and a supramitral stenosing ring is located at atrial surface of base of MV valve leaflets, and is more immobile

-w diastole, see a cor triatriatum membrane go twd MV w Nl MV motion

-LAA and FO are distal to membrane in cor triatriatum, but are proximal to supramitral stenosing ring.

-supramitral stenosing ring adheres to MV usually, and moves away fr the valve w diastole

-ECG- RV systolic overload- RA hypertrophy w tall, peaked P waves; might be broad and notched bc of accessory chamber dilation

-CXR- PVn obst w fine diffuse retricular pulm markings, +/- Kerley B lines, +/- staghorn sign of upper PVn engorgement, big MPA, RVH, LAE w posterior deviation of esoph on barium study

-MRI- shows it well

-Cath- no phtn usually, check for shunting at atrium...

Tx:

-if pulm edema/R heart failure-> medical Tx, but then expect it to get worse so do surgery ASAP to resect the membrane

Px:

-d/o orifice size/amt of obst

-survival to 3mo if <3mm, 16yrs if >3mm; but decreases to a few months if pt gets pulm edema and R hrt failure