ulmonlc SLenosls

SLenoLlc and domed ulmonlc valve w/ posLsLenoLlc dllaLaLlon of Lhe maln pulmonary arLery
w/ Llme see 8vP as Lhe lncreased ressures of Lhe rlghL venLrlcle are requlred Lo malnLaln CC
ctltlcol lolmoooty 5teoosls decreased 8v compllance lncreased 8A pressure openlng of Lhe
foramen ovale small rlghL Lo lefL shunL

Lxam
1 ShorL low plLched systo||c e[ect|on murmer heard best at the 2
nd
3
rd
LLI1 ICS w] rad|at|on to
both |ung f|e|ds
a Pear an e[ecLlon murmer lmmedlaLely afLer S1 ln Lhe lefL upper sLernal boarder
2 W/ worsenlng 8v heave decreased 2 harsher murmer
Cx8
1) Lnlarged hearL w/ decreaslng pulmonary vascularlLy (more black appearlng more alr)
2) osLsLenoLlc dllaLaLlon of Lhe maln pulm aa
LkC
1) 8lghL ALrlal LnlargemenL @oll/peokeJ l woves
2) I lo moJetote to sevete coses

AorLlc SLenosls (lefL venLrlcular ouLlflow LracL)
MosL are due Lo blcuspld aorLlc valve
3) usually noL sympLomaLlc unLll adulLhood






Classlfled lnLo 1P8LL Lypes
1) valvular llke blcuspld valve
2) Subvalvular sLenosls flbromuscular shelf or markedly hyperLrophled sepLum (lPSS)
3) Supravalvular sLenosls rare



resenLaLlon depends on severlLy
Severe AS
4) resenLs early ln lnfancy crlLlcal aorLlc sLenosls
4 efL venLrlcular fallure and decreased CC
4 -o murmur wlll be ausculLaLed
Mlld Lo ModeraLe
3) ulscovered w/ murmer
6) AsympLomaLlc ln chlldhood
7) 8arely Leens w/ syncope and anglna on exerLlon
As severlLy lncreases see decreased pulses lncreased hearL slze v heave


LxamlnaLlon/Mumer
8) arsh Systo||c L[ect|on murmer w] thr|||
4 Peard besL aL Lhe 23 lCS on Lhe 8lCP1 chesL
4 8adlaLes Lo Lhe neck and lefL sLernal boarder
9) Larly L[ecLlon Cllck" heard ln Lhe apex of Lhe hearL Lo Lhe lefL sLernal boarder
10)alpaLe a Lhrlll ln Lhe suprasterna| notch
11)
Coarctation of tbe Aorta:
-arrowlng of Lhe aorLa aL any polnL from Lhe Lransverse arch Lo Lhe lllac blfurcaLlon pre vs posL ducLal

9resentat|on
uepends on severlLy
12)Severe form ls seen ln neonaLe and called reducLal/lnfanLlle" Lubular hypoplasla of Lhe
Lransverse arch of Lhe aorLa
4 CrlLlcal CoarcLaLlon ls uA dependenL musL keep open wlLh CL1 wlll presenL w/
clrculaLory collapse lf Lhe uA ls closlng
13)lnfanLs may presenL w/ lrrlLablllLy dlfflculLy feedlng and l11
4 L cyanosls
4 uL plnk w/ hyperLenslon
14)|der Ch||d 1ype (Iuxtaducta|) 90 of cases (usually males when female conslder 1urners)
usually occurs [usL dlsLal Lo Lhe orlgln of Lhe lefL subclavlan arLery aL Lhe orlgln of Lhe ducLus
arLerlosus
May hear a murmer nonspeclflc e[ecLlon murmer
resenL w/ Loq of lemoro/ pu/se when simu/toneous/y po/potinq brochio/ pu/se
lower ln L
J||| deve|op extens|ve co||atera| c|rcu|at|on over t|me!


1x
-eonaLes
4 CL1 lnfuslon
4 Surgery afLer sLablllzaLlon
Clder
4 1reaL PyperLenslon Lhen surgery
4 uelay ls unwarranLed as v funcLlon decllnes by second decade and surgery ls noL as
successful

runcus Arteriousus
Slngle arLerlal Lrunk arlses and supplles Lhe sysLem pulmonary and coronary clrculaLlon
ISD |s a|ways present
ssoc|ated w] 22q11 de|et|ons D|george and ve|ocard|ofac|a| syndrome

Cllnlcally a nonspeclflc murmer may be presenL aL blrLh ln addlLlon Lo mlolmol cyooosls
CongesLlve PearL lallre develops ln a manner weeks as Lhe ulmonary vascular 8eslsLance falls
and pulmonary blood flow lncreases aL Lhe expense of sysLemlc blood flow
Lxam
uepends on depends on age and v8
1) SysLollc murmer and mlnlmal cyanosls as lnlLlal slgns
2) oud s|ng|e S2 sysLollc e[ecLlon murmer and Lhrlll aL Lhe lefL sLernal base
3) PearL ls enlarged and hyperdynamlc precordlum
Cx8 Lnlarged ouLflow LracL!







llgure 8" xray

\o\astic L sndrome
ALresla of Lhe aorLlc or mlLral orflce and hypoplasla of Lhe ascendlng aorLa w/
1 v ls small or LoLal aLreLlc
2 I Mointoins both pu/monory ond systemic circu/otions /eodinq to deoth from cnu by one
month of oqe

lolmoooty Ieooos 8looJ A5u/llO mlxloq of oxyqeooteJ ooJ JeoxyqeooteJ blooJ
O uescendlng aorLa ls compleLely dependenL on uA
O 8eLrograde aorLlc flow Lo supply upper body
O May presenL as graylsh blue ln color and go lnLo shock when Lhe uA closes



Lxam
See Cardlac LnlargemenL wlLh rlghL parasLernal llfL
Weak or absenL pulses/ w/ cold exLremlLles
Cx8
PearL enlargemenL wlLh w/ lncreased ulmonary blood flow
LkC
I L w] decreased |eft s|ded forces
**AssoclaLed w/ exLracardlac abnormallLles of Lhe kldneys and C-S




ulmonlc SLenosls
8vP
Cverldldng AorLa
vSu

1eLL Spells


1x squaLLlng resulLs ln lncreased afLerload bb o2









Sexua| Deve|opment |n k|ds
C8a AM
(order of developmenL)
C8owLh spurL
reasL uevelopmenL (1helarce)
ubarche (ublc Palr)
Adrenarche (axlllary halr acne eLc)
Menarche