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A 12-years-old girl with hypertension associated with aortic

coarctation, congenital valvular aortic stenosis and bicuspid


aortic valve
Authors: Carmen Ginghina, Mirela Marinescu, Dinu Dragomir, Olguta Iliescu*, Roxana
Hagioglu*, Dan Deleanu, V. Iliescu, V. Ene**
Institute ! Car"io#ascular Disease
* stu"ent $M% &Carol Da#ila'
** Hos(ital &).G.*u(u'
Abstract
+e are (resenting the case re(ort o, a -. !ears ol" (atient /ith se#ere aortic coarctation,
secon"ar! h!(ertension, aortic stenosis an" icus(i" aortic #al#e. 0he /as acci"ental!
,oun" /ith ele#ation o, loo" (ressure 1-234-33 mmHg5 in the u((er extremities an"
un"etectale loo" (ressure in the lo/er extremities. 6he "iagnosis ecame certain a,ter
the ra"iological exam, the t/o7"imensional an" Do((ler echocar"iogra(h!, MRI an"
angiogra(h!. 6he treatment /as surgical.
Introduction
Coarctation o, the aorta is a narro/ing o, the lumen o, the "escen"ing aorta usuall!
"istal to the origin o, the le,t sucla#ian arter! near the insertion o, the ligamentum
arteriosum.
Ma! e isolate" or associate" /ith other anomalies, occuring in aout 8 (ercent o,
(atients /ith congenital heart "isease. In chil"ren, it can e asociate" /ith (atent "uctus
arteriosus, icus(i" aortic #al#e, aortic stenosis, h!(o(lasia o, aortic arch, #entricular
se(tal "e,ect, trans(osition o, the great arteries. 6he most im(ortant extracar"iac anomal!
is aneur!sm o, the circle o, +illis.
6his anomal! is characteri9e" ! arterial h!(ertension in the u((er (art o, the o"!,
asence or mar:e" "iminution (ulsations in the ,emoral arteries, collateral com(ensator!
circulation, le,t #entricular h!(ertro(h!, "ilatation o, the le,t sucla#ian arter!, "ilatation
o, ascen"ing aorta an" (re7 an" (oststenotic "ilatation. O,ten (atients are as!m(tomatic,
ut occasionall!, some (atients (resent /ith recurrent hea"ache, noselee"s, or lo/er
lim clau"ication an" /ea:ness.
6he (athogenesis o, h!(ertension in the coarctation o, the aorta ma! e much more
com(lex than the mechanical ostructution. 6he renin7angitensin s!stem an" increase"
catecholamine secretion coul" e also in#ol#e".
A s!stolic murmur ma! e (resent o#er the anterior le,t (recor"ium, ra"iating to the
ac:, /ith continuous murmurs ,rom collateral #essels in some (atients o#er the su(erior
le,t an" right chest as /ell as the ac:. 6he t/o7"imensional echocar"iogra(h! i"enti,ies
the site an" length o, the coarctation, ut the "iagnosis ecome certain a,ter coronar!
arterogra(h! 1in#asi#e (roce"ure5 or Do((ler, C6, MRI 1nonin#asi#e (roce"ure5.
6he corection is etter to e ma"e in chil"hoo" ! inter#entional treatment ; alloon
angio(last! ; or ! surger!, in or"er to "escrease the ris: o, heart ,ailure, en"ocar"itis or
stro:e. <ara"oxical h!(ertension o, short "uration o,ten is note" in the imme"iate
(osto(erati#e (erio", a (henomenon much less common a,ter alloon angio(last!. A
resetting o, caroti" arorece(tors an" increase" catecholamine secretion a((ears to e
res(onsile ,or the initial (hase o, (osto(erati#e s!stemic h!(ertension /ith a later,
secon" (hase o, (rolonge" ele#ation o, s!stolic an" (articularl! "iastolic loo" (ressure
relate" to acti#ation o, the renin7angiotensin s!stem. A = to -3 (er cent ris: o, recurrent
narro/ing exists a,ter re(air o, coarctation in in,anc!. 6his (rolem is treate" most
e,,ecti#el! ! transcutaneous alloon angio(last!.
Clinical History
A month ago, the (atient has een "iagnosticate" acci"ental! /hen she came to the
"octor ,or sim(toms o, acute tonsilitis. 6hen she ha" an ele#ation o, loo" (ressure
1-234-33 mmHg5 /ithout ha#in, an! case o, h!(ertension in her ,amil!.
0he /as a"mite" to the hos(ital /ith a histor! o, hea"ache, col" extremities an"
clau"ications occurre" /ith exercise.
Phisycal examination sho/e":
7 ele#ation o, oo" (ressure in the u((er extremities1-234-33 mmHg5
- "iminishe" (ulsations in the le,t rachial, ulnar an" ra"ial arteries
- asent (ulsations in the oth ,emoral, (o(liteal (ulsations
- un"etectale arterial (ressure in the lo/er extremities
- a mi"s!stolic murmur o#er the anterior chest, ac: an" s(inous (rocesses
- a lou", narsh s!stolic murmur est hear" at the ase o, the heart
- a""itional s!stolic an" continuous murmur o#er the lateral thoracic /all
Electrocardiography 1,ig.-5 re#eals le,t #entricular h!(ertro(h!, the sum o, the &s' /a#e
in V. an" &R' /a#e in V. is greater than >= mm. In lea" V=1V?5 there is &06'
"e(ression com(atile /ith &*V stain'
Chest X ray 1,ig..5 re#eals ri notching est seen along the un"ersur,ace o, the thir" an"
,ourth ris 1/hich has een cause" ! collateral #essels, secon"ar! to ostruction lesion
o, aorta5
At the fundus of the eye 1,ig.>5 there are retinal changes, "emonstrating the t!(ical
,eatures o, se#ere h!(ertension : the arterio7#enous ni((ing, cotton /ool s(ots, har"
exu"ates, a ,e/ ,lame7sha(e" hemorrahge.
The two-dimensional and oppler echocardiography sho/s the (ro,ile o, ,lo/ #elocit!
at aortic #al#e: the (ea: 1instantaneous5 an" the mean (ressure gra"ient@ ?24A? mmHg,
the #elocit! (ro,ile otaine" in area o, turulence at the site o, coarctation: high s!stolic
gra"ient@ ?. mmHg an" "iastolic (ersistance, iscu(i" aortic #al#e, #al#ular thic:ening,
the o(ening ori,ice is -A mm, aortic rgurgitation.
6he !ascular oppler echography re#eals:
A"ominal aortic: Bmm "iameter
Crachial arter!: ..=mm
0u(erior mesenteric arter! is "ilatate": Bmm "iameter
Right ,emoral arter!: =.=mm "iameter
0!stolic #elocit!: >3mm
Diastolic #eloct!: .3mm
*e,t ,emoral arter!:= mm "iameter
"#I $can 1,ig.=5 re#eals: concetric h!(ertro(h! o, the le,t #entricle "ue to high loo"
(ressure, aortic #al#e stenosis, coarctation o, the aorta. 6his exam /as (articularl!
hel(,ul ,or nonin#asi#e localisation o, the site o, the ostruction an" ,or the in,ormation
o, the /hole aorta:
7"ilatation o, ascen"ing aorta: maximum "iameter@Bmm
7"escen"ing thoracical aorta has less than - cm "iameter, onl! su(rah!atal has -.. cm
7,irst main narro/ing o, the aortic lumen 1-.-cm5is (osterior o, the origin o, the le,t
caroti" arter!
7the secon" narro/ing is at the aortic isthmus 13.B cm5, (osterior o, the origin o, the le,t
sucla#ian arter!
7im(ortant anormalities o, the su(ra#al#ular ,lo/ an" o, the ascen"ing aorta
7"ilatation an" sinous rachioce(halic trun: 1..> cm5 in the right hal, o, the anterior
su(erior me"iastinum, in the lel, (art there is the th!mus
7the ascen"ing aorta is continue" /ith the rachioce(halic trun:
7h!(o(lasia o, the le,t #enous rachioce(halic trun:
Arteriography 1,ig.?,8,25 sho/e" the same mo"i,ications as those (resente" at the t/o7
"imensional an" Do((ler echocar"iogra(h! an" MRI exams.
6he ,inal "iagnosis /as aortic coarctation, secon"ar! h!(ertension, congenital #al#ular
aortis stenosis an" icus(i" aortic #al#e. 6he treatment o, the aortic coarctation /as
surgical /ith a (atch o, autologous (ericar"ial. 6he as!m(tomatic congenital aortic
stenosis nee"s close su(er#ision an" serial assesment ,or (rogression o, the "isease. In
,uture it coul" e nessesar! surgical treatment /ith aortic #al#ulotom! or #al#ulo(last!.
Discussions
+e ha#e (resente" the case re(ort o, a -.7!ears7ol" girl, /ith se#ere aortic coarctation,
secon"ar! h!(ertension, aortic stenosis an" icus(i" aortic #al#e, /ho /as acci"ental!
"iagnosticate" /hen she came to the hos(ital ,or sim(toms o, acute tonsilitis.
6he ele#ation o, the loo" (ressure at the u((er lims, the "iminushe" (ulsations o, the
,emoral arteries, the mi"s!stolic murmur o#er the anterior chest, ac: an" s(inous
(rocesses, a""itional s!stolic an" continuous murmur o#er the lateral thoracic /all
sus(ecte" the "iagnosis. A lou", narsh s!stolic murmur est hear" at the ase o, the heart
in"icate" the (ossiilit! o, existing more mal,ormations associate": aortic stenosis.

6he "iagnosis ecame certain a,ter the t/o7"imensional an" Do((ler echocar"iogra(h!
/hich ga#e us ,unctional "atas 1C+D5: the gra"ient across the coarctation an" the
icus(i"e aortic #al#e, an" mor(hological "atas 1.D5: a com(lex mal,ormation /hich
assocites: aortic coarctation, aortic stenosis, icus(i"e #al#e /ith conseDuences o#er the
le,t #entricle 7 concetric h!(ertro(h!. +ith the other nonin#asi#e in#estigations /e ha#e
otaine" in,ormation aout the conseDuences o, the congenital anormalitis an"
h!(ertension on the car"iac chamers 1 le,t #entricular h!(ertro(h! on ECG, Echo,
MRI5, on the e!e 1retinal changes5, on the ris 1ri notching along the in,erior margin o,
the thir" to the ,ourth ris "ue to the (ulsations o, the "ilate" intercostal arteries5.6he
MRI exam sho/s t/o stenosis, gi#ing #er! exactel! in,ormation aout the localisation o,
the ostruction an" the aortic mor(holog! (re an" (oststenosis.
Although the "iagnosis /as ma"e /ith nonin#asi#e in#estigations, the surgeon as:e" ,or
an aortogra(h! as the mal,ormation /as #er! com(lex.
6he (atient has een succes,ul! treatete" surgical /ithout s!stemic h!(ertension
(osto(erati#el!.
Conclusions
+e ha#e chosen this case re(ort to (resent ecause /e /ant to re#eal that unex(laine"
h!(ertension in the u((er extremities an" asence o, the (ulsations in the lo/er lims,
must sus(ect the "iagnosis o, aortic coarctation. 6he "iagnosis can e ma"e /ith
nonin#asi#e in#estigations 1Echo .D, Do((ler, MRI5, ut the in#asi#e technics
1aortogra(h!5 (ut the "iagnosis o, certain, es(ecial! /hen the mal,ormation is com(lex.
6he corection is etter to e ma"e in chil"hoo" ! inter#entional treatment ; alloon
angio(last! ; or ! surger!, in or"er to "escrease the ris: o, heart ,ailure, en"ocar"itis or
stro:e. <ara"oxical h!(ertension o, short "uration o,ten is note" in the imme"iate
(osto(erati#e (erio", a (henomenon much less common a,ter alloon angio(last!. 6his
can e a#oi" ! using o, the eta loc:ers.
%igures
%ig.- ECG le,t #entricular h!(ertro(h!, the sum o, the &s' /a#e in V. an" &R' /a#e in
V. is greater than >= mm. In lea" V=1V?5 there is &06' "e(ression com(atile /ith &*V
stain'
%ig.. Chest E ra! :ri notching est seen along the un"ersur,ace o, the thir" an" ,ourth
ris
%ig> %un"us o, the e!e: retinal changes, the arterio7#enous ni((ing, cotton /ool s(ots,
har" exu"ates, a ,e/ ,lame7sha(e" hemorrahge.
%ig.A 6/o "imensional an" Do((ler echocar"iogra(h!: the (ea: 1instantaneous5 an" the
mean (ressure gra"ient@ ?24A? mmHg, the #elocit! (ro,ile otaine" in area o, turulence
at the site o, coarctation: high s!stolic gra"ient@ ?. mmHg an" "iastolic (ersistance,
iscu(i" aortic #al#e, #al#ular thic:ening, the o(ening ori,ice is -A mm, aortic
regurgitation.
%ig.= MRI exam: concetric h!(ertro(h! o, the le,t #entricle "ue to high loo" (ressure,
aortic #al#e stenosis, coarctation o, the aorta an" localisation o, the site o, the
ostruction
%ig.? Aortogra(h!: Cicus(i"e aorti #al#e, "ilatation o, ascen"ing aorta, aortic
regurgitation
%ig.8 Aortogra(h!: the "ilatation o, ascen"ing aorta an" rachioce(halic trun:, increase"
,lo/ through "ilatate" an" tortuous collateral #essels
%ig.2 Aortogra(h!: coarctation o, aorta locate" at the (roximal region o, the "escen"ing
aorta, Fust "istal o, the origin o, the le,t sucla#ian arter!

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