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ers|stent u|monary nypertens|on of the Newborn

erslsLenL pulmonary hyperLenslon (Pn) ls also referred Lo as perslsLenL feLal clrculaLlon ln


Lhls condlLlon a newborn babys clrculaLlon changes back Lo Lhe clrculaLlon of a feLus where much of
Lhe blood flow bypasses Lhe lungs Cenerally one Lo Lwo newborns per 1000 suffer from perslsLenL
pulmonary hyperLenslon 1hese lnfanLs have pulmonary arLerlal pressure LhaL ls Loo hlgh aL blrLh As a
resulL Lhelr lungs canL provlde enough oxygen whlch causes Lhelr bodles Lo produce oxygenpoor
blood someLlmes resulLlng ln deaLh ln one sLudy nearly half Lhe survlvors were cognlLlvely delayed
had ma[or neurologlcal problems and could noL hear
erslsLenL pulmonary hyperLenslon of Lhe newborn (Pn) occurs when a newborns clrculaLlon
sysLem does noL adapL Lo breaLhlng ouLslde Lhe womb Whlle a feLus ls ln Lhe womb lL geLs lLs oxygen
from lLs moLhers placenLa Lhrough Lhe umblllcal cords so Lhe lungs need llLLle blood supply 1here ls
hlgh blood pressure ln Lhe lungs so blood ln Lhe pulmonary arLery ls senL away from Lhe lungs Lo Lhe
oLher organs Lhrough a feLal blood vessel called Lhe ducLus arLerlosus
When a baby ls born and Lakes lLs flrsL breaLhs Lhe blood pressure ln Lhelr lungs falls and Lhere
ls an lncreased blood flow Lo Lhe lungs where oxygen and carbon dloxlde are exchanged 1he blood ls
Lhen reLurned Lo Lhe hearL and pumped back ouL Lo Lhe body 1he ducLus arLerlosus consLrlcLs and
permanenLly closes ln Lhe flrsL day of llfe Powever ln bables wlLh Pn Lhe pressure ln Lhe lungs
remalns hlgh and Lhe ducLus arLerlous remalns open allowlng blood Lo be dlrecLed away from Lhe lungs
1he followlng are Lhe mosL common sympLoms of perslsLenL pulmonary hyperLenslon Powever each
baby may experlence sympLoms dlfferenLly SympLoms may lnclude

- cyanos|s (b|ue co|or|ng)

- baby appears ||| at de||very or |n f|rst hours after b|rth

- rap|d breath|ng

- |ow b|ood oxygen |eve|s wh||e rece|v|ng 100 percent oxygen

- rap|d heart rate

1ypes of nN
1 Pn characLerlzed by abnormally consLrlcLed pulmonary vasculaLure due Lo lung parenchymal
dlseases (eg meconlum asplraLlon syndrome resplraLory dlsLress syndrome pneumonla see
Lhe lmage of meconlum asplraLlon below)
2 Pn characLerlzed by hypoplasLlc vasculaLure as seen ln congenlLal dlaphragmaLlc hernla
Pn ln whlch Lhe lung has normal parenchyma and remodeled pulmonary vasculaLure (also
known as ldlopaLhlc nP)

aLhophyslology
O AL a neonaLe's 1sL breaLh afLer dellvery Lhe pulmonary vascular reslsLance normally decreases
Lo redlrecL 30 of Lhe cardlac ouLpuL Lo Lhe pulmonary clrculaLlon 1hls falls Lo occur ln Pn
hence Lhe prevlous name of Lhls condlLlon was perslsLenL feLal clrculaLlon"
O lncreased pulmonary vascular reslsLance lncreases rlghL venLrlcular afLerload causlng a
backflow of blood Lo Lhe rlghL hearL (and subsequenL Lrlcuspld regurglLaLlon) and lncreased rlghL
hearL pressures whlch can lead Lo rlghL venLrlcular fallure
O lncreased pulmonary arLerlal pressures also cause lnLracardlac shunLlng across any paLenL
foramen ovale ducLus arLerlosus or aLrlosepLal or venLrlculosepLal defecL LhaL may be presenL
8lood shunLs from rlghL Lo lefL because of Lhe supranormal sysLemlc pulmonary arLerlal
pressures 1hls causes more deoxygenaLed blood Lo go Lo Lhe lefL hearL and Lhen Lo Lhe body
whlch manlfesLs as lower oxygen saLuraLlon ln Lhe lower exLremlLles (posLducLally) cyanosls
and hypoxla
O eoxygenaLed blood ln Lhe lefL hearL can lead Lo lschemlc damage Lo Lhe hearL and rlghL or lefL
venLrlcular fallure
O 1he cardlac and/or resplraLory fallure ls occaslonally severe enough Lo requlre exLracorporeal
membrane oxygenaLlon (LCMC)
O lf Lhere ls no shunLlng of blood or Lhe blood cannoL geL from Lhe rlghL Lo lefL hearL because of a
lack of perslsLenL feLal paLhways a neonaLe may develop poor sysLemlc perfuslon severe
acldosls shock rlghL venLrlcular fallure and even deaLh
O Any hypoxla acldosls or sLress LhaL occurs afLer blrLh furLher lncreases pulmonary vascular
reslsLance
O Dsually Lhe pulmonary vasculaLure beglns Lo relax wlLhln 3 days of llfe and Lhe process
reverses 1here ls conLlnued vascular remodellng over Lhe 1sL 2 weeks of llfe SomeLlmes Lhe
pulmonary vascular reslsLance remalns elevaLed as a resulL of an underlylng dlsease process or
anaLomlc abnormallLy

aboratory Stud|es
Arter|a| b|ood gas
Check arLerlal blood gases (A8Cs) lnlLlally and frequenLly ldeally Lhrough an lndwelllng llne
Assess Lhe pP Lhe parLlal pressure of carbon dloxlde ln arLerlal gas (aCC
2
) and Lhe parLlal
pressure of oxygen (aC
2
) Dslng Lhe fracLlon of lnsplred oxygen (llC
2)
alveolararLerlal (Aa)
dlfference ln Lhe aC
2
can be calculaLed
omp|ete b|ood count
LvaluaLe Lhe compleLe blood counL (C8C) for a hlgh hemaLocrlL level because polycyLhemla and
hypervlscoslLy syndrome may produce or aggravaLe Pn
Serum e|ectro|ytes
MonlLor serum elecLrolyLe and glucose levels lnlLlally and frequenLly
ad|ography
ChesL radlography ls useful ln deLermlnlng wheLher underlylng parenchymal lung dlsease (eg
meconlum asplraLlon syndrome pneumonla surfacLanL deflclency) ls presenL ChesL
radlography also asslsLs ln excludlng underlylng dlsorders such as congenlLal dlaphragmaLlc
hernla

CLher LesLs
u|se ox|metry
ConLlnuous pulse oxlmeLry ls exLremely valuable ln Lhe ongolng LreaLmenL of Lhe newborn wlLh
perslsLenL pulmonary hyperLenslon of Lhe newborn (Pn) allowlng Lhe careglver Lo assess Lhe
paLlenLs oxygen saLuraLlon over Llme and Lo deLermlne wheLher oxygen dellvery aL Lhe Llssue level ls
adequaLe
ard|ac catheter|zat|on
ln rare cases echocardlographlc flndlngs are noL deflnlLlve and cardlac caLheLerlzaLlon may be
necessary Lo exclude congenlLal hearL dlsease parLlcularly anomalous pulmonary venous reLurn
Inotrop|c drugs
lnoLroplc supporL wlLh dopamlne dobuLamlne and/or mllrlnone ls frequenLly helpful ln malnLalnlng
adequaLe cardlac ouLpuL and sysLemlc blood pressure whlle avoldlng excesslve volume admlnlsLraLlon
AlLhough dopamlne ls frequenLly used as a flrsLllne agenL oLher agenLs such as dobuLamlne and
mllrlnone are helpful when myocardlal conLracLlllLy ls poor






t also contains the key temperature sensors. Other sensors, located all over the body, record whether the
body temperature is too high or too low
1hermoregulaLlon ln boLh ecLoLherms and endoLherms ls conLrolled malnly by Lhe preopLlc area of Lhe
anLerlor hypoLhalamus

Such homeosLaLlc conLrol ls separaLe from Lhe sensaLlon of LemperaLure

s
Lhe ablllLy of an organlsm Lo keep lLs body LemperaLure wlLhln cerLaln boundarles even when Lhe
surroundlng LemperaLure ls very dlfferenL
he body lncreases and lowers lLs core LemperaLure uslng a LemperaLure conLrol sysLem LhaL works llke a
LhermosLaL lncreased body LemperaLure acLlvaLes mechanlsms promoLlng heaL loss and lowered body
LemperaLure acLlvaLes mechanlsms enabllng Lhe accumulaLlon or producLlon of heaL Such a sysLem ls
called a feedback conLrol sysLem because lL uses as lnpuL Lhe LoLal or parLlal ouLpuL of Lhe sysLem
meanlng LhaL Lhe consequences of Lhe process dlcLaLe how lL wlll go on furLher A feedback sysLem has
Lhree componenLs sensors LhaL reglsLer Lhe change a conLrol cenLer LhaL recelves Lhe slgnals of Lhe
sensors and an effecLor mechanlsm meanlng a paLhway for Lhe commands of Lhe conLrol cenLer when
lL responds Lo Lhe lnformaLlon recelved from Lhe sensors ln LhermoregulaLlon Lhe conLrol cenLer ls
locaLed ln Lhe hypoLhalamus