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A 12-years-old girl with hypertension associated with aortic coarctation, congenital valvular stenosis and bicuspid aorta. 'Iagnosis ecame certain a,ter the ra#iological exam, the t / o7"imensional an" angiogra(h!. 6he treatment / as surgical.
A 12-years-old girl with hypertension associated with aortic coarctation, congenital valvular stenosis and bicuspid aorta. 'Iagnosis ecame certain a,ter the ra#iological exam, the t / o7"imensional an" angiogra(h!. 6he treatment / as surgical.
A 12-years-old girl with hypertension associated with aortic coarctation, congenital valvular stenosis and bicuspid aorta. 'Iagnosis ecame certain a,ter the ra#iological exam, the t / o7"imensional an" angiogra(h!. 6he treatment / as surgical.
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!