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BBAA EEDDTTRR - EDITOR IN CHIEF
Dr. N. Krad TOKEL, Trkiye
BBAA EEDDTTRR YYAARRDDIIMMCCIISSII - ASSOCIATE EDITOR IN
CHIEF
Dr. Nazmi NARN, Trkiye
EEDDTTRR YYAARRDDIIMMCCIILLAARRII - ASSOCIATE EDITORS
Dr. Osman BAPINAR, Trkiye
Dr. Ali BAYKAN, Trkiye
Dr. Ersin EREK, Trkiye
Dr. Yakup ERGL, Trkiye
Dr. Frat KARDELEN, Trkiye
Dr. Sedef TUNAOLU, Trkiye
DDLL EEDDTTRR -- LLAANNGGUUAAGGEE EEDDIITTOORR
Dr. Ayhan KILI, Trkiye
YYAAYYIINN KKUURRUULLUU - EDITORIAL BOARD
Dr. Riyadh M. ABU -SULAIMAN, Saudi Arabia
Dr. Hakan AKINTRK, Germany
Dr. Dursun ALEHAN, Trkiye
Dr. Zahid AMIN, USA
Dr. Semra ATALAY, Trkiye
Dr. hsan BAKIR, Trkiye
Dr. Emre BELL, France
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Dr. Birgl VARAN, Trkiye
Bilimsel olaylar ve yazm kurallarkimlik sayfa saysna eklenmeli
!!!
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Dr. Koray AK, Trkiye
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DDAANNIIMMAA KKUURRUULLUU - ADVISORY BOARD
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TRK PEDATRK KARDYOLOJ VE KALP CERRAHS DERNEADINA SAHBDr. Nazmi
NARN
SORUMLU YAZI LER MDRDr. Nazmi NARN
YNETM YERTrk Pediatrik Kardiyoloji ve Kalp Cerrahisi Dernei
Hodere Caddesi No: 180/4 ankaya, ANKARA
Tel : (0312) 212 02 00
Faks : (0312) 212 02 00
web : www.turkpedkar.org.tr
e-posta : [email protected]
YAYIN PERYODU VE TRPediatric Heart Journal 3 ayda bir olmak zere
ylda 4 say yaynlanr (Mart-
Haziran-Eyll-Aralk).
Yerel sreli yayn.
ADRES DEKLKLERDerginin yaynland tarihlerden en az 15 gn nce
dernek yazma adresine
bildirilmelidir. Zamannda yaplmayan bildirimler nedeniyle
derginin aboneye ula-
mamasndan yaync sorumlu tutulamaz.
YAYIN HAKKIPediatric Heart Journalde yaynlanan yazlar, resim,
ekil ve tablolar yayncnn yazl
izni olmadan ksmen veya tamamen herhangi bir vasta ile baslamaz,
oaltlamaz.
Bilimsel amala kaynak gstermek kaydyla zetleme ve alnt
yaplabilir.
BASILDII YER-BASIMCI-YAYIMCIOrtadou Reklam Tantm Yaynclk Turizm
Eitim naat Sanayi ve TicaretA.. (Trkiye Klinikleri)Trkoca Cad.
No:30 06520 Balgat/Ankara/Trkiye
Tel : 0 312 286 56 56
Faks : 0 312 220 04 70
e-posta : [email protected]
web : www.turkiyeklinikleri.com
Basma verili tarihi: 06.04.2016
ISSN: 2148-4910
Online ISSN: 2458-7591
THE OWNER ON BEHALF OF TURKISH PEDIATRIC CARDIOLOGY AND CARDIAC
SURGERY SOCIETYDr. Nazmi NARN
MANAGING CLERICAL DIRECTORDr. Nazmi NARN
ADDRESS FOR MANAGEMENTTurkish Pediatric Cardiology and Cardiac
Surgery Society
Hodere Caddesi No: 180/4 ankaya, ANKARA
Phone : (0312) 212 02 00
Fax : (0312) 212 02 00
web : www.turkpedkar.org.tr
e-mail : [email protected]
PUBLICATION TYPE AND PERIODSPediatric Heart Journal is published
4 times a year (March-June-September-
December).
Local periodic publication.
CHANGE OF ADDRESSNotify the subscription office for the change
of address at least 15 days before thedate of issue. The publisher
will not be responsible from any lost resulting from anaddress
change if not informed.
COPYRIGHTAll articles, images, figures and tables published in
this journal are protected byCopyright. No material published in
this journal may be reproduced or duplicatedpartially or totally
without the written permission from the copyright holder.
Permis-sion is not required to cite or summarize the publications
for scientific purposes inthe condition that a full reference to
the source is shown.
PUBLISHING HOUSE-PUBLISHEROrtadou Advertisement Presentation
Publication TourismEducation Construction Industry and Trade Co.
(Trkiye Klinikleri)Trkoca Cad. No:30 06520 Balgat/Ankara/Turkey
Phone : +90 312 286 56 56
Fax : +90 312 220 04 70
e-mail : [email protected]
web : www.turkiyeklinikleri.com
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ORJNAL ARATIRMALAR
169 EEffffeeccttss ooff aa CCoommbbiinnaattiioonn ooff
SSppiirroonnoollaaccttoonnee,, FFuurroosseemmiiddee aanndd
CCaappttoopprriill oonn BBlloooodd EElleeccttrroollyyttee
LLeevveellss ooff IInnffaannttss wwiitthh CCoonnggeessttiivvee
HHeeaarrtt FFaaiilluurree DDuuee ttoo CCoonnggeenniittaall
HHeeaarrtt DDiisseeaassee Doutan Kalp Hastal Nedeniyle Konjestif
Kalp Yetersizlii Olan Bebeklerde Spironolakton, Furosemid,
Kaptopril Kombinasyonunun Kan Elektrolit Dzeylerine EtkileriAlev
ARSLAN, Nazan ZBARLAS, Sevcan ERDEM, Osman KKOSMANOLU
174 EEvvaalluuaattiioonn ooff CCaarrddiiaacc FFuunnccttiioonnss
iinn CChhiillddrreenn wwiitthh FFaammiilliiaall
MMeeddiitteerrrraanneeaann FFeevveerrAilevi Akdeniz Atei Olan
ocuklarda Kardiyak Fonksiyonlarn DeerlendirilmesiPelin AYYILDIZ
HACIMEROLU, Yonca AIKGZ, Taner KASAR, Erkut ZTRK,
brahim Cansaran TANIDIR, smail LEK
182 YYeenniiddooaannddaa KKaarrddiiyyoolloojjii
KKoonnssllttaassyyoonnuu:: KKaarrddiiyyaakk AAnnoommaallii SSkkll
vvee EEttiiyyoolloojjiikk FFaakkttrrlleerrCardiology Consultation
in Neonates: The Incidence of Cardiac Anomaly and the Ethiological
FactorsSaadet ELK CENGZ, Ali Rahmi BAKLER, Ula KARADA, Kay ELAIK,
ule DEMR,
Buket DORUSZ, Esra ARUN ZER
189 ffrrmmll ooccuukkllaarrnn
DDeeeerrlleennddiirriillmmeessiiEvaluation of Children with Cardiac
MurmurNurdan EROL, Yusuf zzet AYHAN, Fatma Tuba ALTIN COKUN, Merve
KOLU
OLGU SUNUMLARI
194 LLeefftt VVeennttrriiccuullaarr OOuuttflflooww TTrraacctt
OObbssttrruuccttiioonn aanndd AAoorrttiicc
IInnssuufffificciieennccyy CCaauusseedd bbyy AAcccceessssoorryy
MMiittrraall VVaallvvee:: CCaassee RReeppoorrttAksesuar Mitral
Kapan Neden Olduu Sol Ventrikl k Yolu Obstrksiyonu ve Aort
YetersizliiTaner KASAR, sa ZYILMAZ, Mehmet Bedir AKYOL, Serta
HAYDN, Alper GZELTA
NDEKLER
CCiilltt 22 SSaayy 44 YYll 22001155
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197 CCoonnttrraallaatteerraall SSeeqquueessttrraattiioonn iinn
RRiigghhtt--SSiiddeedd SScciimmiittaarr SSyynnddrroommee
PPrreesseennttiinngg wwiitthh IInnflflaammmmaattoorryy
MMyyoofifibbrroobbllaassttiicc TTuummoorr:: CCaassee RReeppoorrtt
Kar Taraf Sekestrasyondan Kaynakl nflamatuar Miyofibroblastik Tmr
ile Birlikte Grlen Sa Taraf Scimitar Sendromusa ZYILMAZ, Taner
KASAR, brahim Cansaran TANIDIR, Serglen DERVOLU, Alper GZELTA,
Ender DEM
200 PPrroommiinneenntt LLeefftt VVeennttrriiccuullaarr
NNoonnccoommppaaccttiioonn iinn aa PPaattiieenntt wwiitthh
PPuullmmoonnaarryy AAttrreessiiaa--IInnttaacctt
VVeennttrriiccuullaarr SSeeppttuumm:: CCaassee RReeppoorrtt
Pulmoner Atrezi ve ntakt Ventrikler Septumu Olan Bir Hastada
Belirgin Sol Ventrikl NonkompaksiyonuEyp ASLAN, Pelin AYYILDIZ,
brahim Cansaran TANIDIR, Erkut ZTRK, Yakup ERGL, Alper GZELTA
203 AA RRaarree CCoommpplliiccaattiioonn AAfftteerr
PPuullmmoonnaarryy AArrtteerryy BBaannddiinngg::
PPsseeuuddooaanneeuurryyssmm ooff tthhee PPuullmmoonnaarryy
AArrtteerryy:: CCaassee RReeppoorrtt Pulmoner Artere Bant Uygulamas
Sonras Oluan Nadir Bir Komplikasyon: Pulmoner Arter
PsdoanevrizmasFatma Sevin ENGL, Ahmet RDEM, Aysel TRKVATAN, Ersin
EREK, Alper GZELTA
207 DDoouubbllee OOrriifificcee MMiittrraall VVaallvvee iinn aa
CChhiilldd wwiitthh VVeennttrriiccuullaarr SSeeppttaall
DDeeffeecctt;; CCaassee RReeppoorrtt aanndd RReevviieeww ooff
tthhee LLiitteerraattuurreeVentrikler Septal Defektli Bir ocuk
Olguda ift Orifisli Mitral Kapak: Olgu Sunumu ve Literatrn Gzden
GeirilmesiFiliz EKC, Zehra Diyar TAMBURACI USLU, Salih ZOBANOLU,
Mehmet Halil ERTU,
Frat KARDELEN, Gayaz AKURN
211 RReeppaaiirr ooff aann IIaattrrooggeenniicc AAoorrttiicc
VVaallvvee LLeeaaflfleett IInnjjuurryy:: CCaassee RReeppoorrtt
yatrojenik Aort Kapak Yaralanmasnn OnarmMurat KO, mer Faruk EK,
Sercan TAK, Onur IIK, Vehbi DOAN, Hakan AYDIN, Ali KUTSAL
214 MMoonnoozzyyggoottiicc TTwwiinnss wwiitthh WWiilllliiaammss
SSyynnddrroommee:: CCaassee RReeppoorrttMonozigotik kizlerde
Williams SendromuSelcen YAROLU KAZANCI, Mehmet Bedir AKYOL
218 UUzzuunn QQTT SSeennddrroommuu vvee iittmmee KKaayybb::
JJeerrvveellll vvee LLaannggee--NNiieellsseenn SSeennddrroommlluu
kkii KKzz KKaarrddee Long QT Syndrome and Hearing Loss: Two Sisters
with Jervell and Lange-Nielsen SyndromeFunda AKPINAR, Dolunay
GRSES, zlem GL, Oya UYGUNER, Fsun DZCAN
BLMSEL TOPLANTILAR
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Pediatr Heart J 2015;2(4) 169
Effects of a Combination of Spironolactone,Furosemide and
Captopril on Blood ElectrolyteLevels of Infants with Congestive
Heart Failure
Due to Congenital Heart Disease
AABBSS TTRRAACCTT OObbjjeeccttiivvee:: To investigate blood
electrolyte levels when spironolactone is administered
concomi-tantly with furosemide and captopril to infants with
moderate to severe heart failure due to congenital heart dis-ease.
MMaatteerriiaall aanndd MMeetthhooddss:: Thirty infants with
congenital heart defects and moderate to severe heart failure,which
could not be controlled by the use of captopril and furosemide for
at least one month, were enrolled in thisstudy. Serum potassium,
serum sodium, blood urea nitrogen (BUN) and serum creatinine levels
were determinedat the initiation of the treatment and first week
controls. Data from patients in Group A (2mg/kg/day spirono-lactone
+ furosemide + captopril; n=16) and Group B (>2 mg/kg/day
spironolactone + furosemide + captopril;n=14) were analyzed for
differences. RReessuullttss:: Hyponatremia was detected in 15
patients (50%) on the first weekcontrols after initiation of
spironolactone treatment. There were 10 patients with mild and 5
patients with mod-erate hyponatremia. Three of them had moderate
dehydration and were hospitalized, hydrated intravenouslyand
spironolactone was discontinued. Eight of them had mild
dehydration; spironolactone dosage was reducedand hydrated orally.
Spironolactone dosage was reduced in only 4 cases who were in
better clinical status and notdehydrated. Two days later, hydration
status and electrolyte levels were improved in all patients. None
of the pa-tients experienced hyperkalemia. According to the 1st
week control results, there was a statistically
significantdifference between blood sodium levels in all patients
(p=0,005) and this difference mainly resulted from GroupB patients.
When groups were compared for electrolyte levels before and after
spironolactone treatment, a sta-tistically significant decrease in
blood sodium levels and an increase in blood potassium levels was
noted inGroup B (p=0.009, p=0.016; respectively). There were no
significant differences between the groups in termsof hyponatremia
frequency or severity at the 1st week controls. Serum creatinine
levels at the first week con-trol were higher in Group B patients;
this was statistically significant when compared with Group A
(p=0.002).CCoonncclluussiioonn:: Occurrence of hyponatremia,
hyperkalemia and dehydration in patients receiving spironolactone
isinfluenced by its dosage when used concomitantly with captopril
and furosemide. Even at recommended doses,it is essential to always
monitor serum sodium and potassium levels.
KKeeyy WWoorrddss:: Heart failure; congenital heart disease;
spironolactone; hyperkalemia; hyponatremia
ZZEETT AAmmaa:: Doutan kalp hastalna bal orta-ar iddette kalp
yetersizlii olan, furosemid ve kaptopril te-davisi alan bebeklerde
spironolakton eklendiinde kan elektrolit dzeylerinin
deerlendirilmesi amaland. GGeerreevvee YYnntteemmlleerr:: En az 1
ay sre ile kaptopril ve furosemid kullanmna ramen kontrol altna
alnamayan orta-ariddette kalp yetersizlii olan 30 bebek almaya
alnd. Her hastann tedavi balanmadan nce ve tedavinin bi-rinci
haftasnda serum potasyum, serum sodyum, kan re azotu (BUN), serum
kreatinin deerlerine bakld. GrupA (16 hasta) (2 mg/kg/gn
spironolakton+furosemid+kaptopril) ve Grup B (14 hasta) (>2
mg/kg/gn spirono-lakton+furosemid+kaptopril) olarak rastgele
belirlendi. BBuullgguullaarr:: Spironolakton sonras 15 hastada
(%50) birincihafta kontrollerinde hiponatremi saptand. On hastada
hafif, be hastada orta dzeyde hiponatremi mevcuttu. Buhastalarn nde
orta derecede dehidratasyon gelitii iin spironolakton kesildi,
intravenz hidrasyon saland.Sekiz hastada ise hafif dehidratasyon
mevcuttu,; spironolakton dozu azaltld ve oral hidrasyon saland.
Hafif hi-ponatremisi olan, dehidratasyonu olmayan drt hastada ise
sadece spironolakton dozu azaltld. Onbe hastannda iki gn sonraki
sodyum dzeyi ve hidrasyon durumu normaldi. Hastalarn hibirinde
hiperkalemi gelimedi.Tm hastalarda spironolakton ncesi ve sonras
hiponatremi geliimi karlatrldnda istatistiksel olarak anlamlfark
saptand (p=0,005), bu fark asl olarak Grup B hastalarndan
kaynaklanmaktayd. Gruplar aras birinci haftasonundaki elektrolit
dzeyleri karlatrldnda, sodyum dzeylerindeki d (p=0,009), potasyum
dzeyle-rindeki art (p=0,016) Grup Bde istatistiksel olarak
anlamlyd. Birinci hafta kontrolnde gruplar arasnda hipo-natremi skl
veya ciddiyeti karlatrldnda istatistiksel olarak anlaml fark
saptanmad. Birinci hafta kontrolserum kreatinin dzeyleri Grup B
hastalarda daha yksekti, Grup A hasta sonularyla karlatrldnda
ista-tistiksel olarak anlaml fark saptand (p=0,002). SSoonnuu::
Kaptopril ve furosemid ile e zamanl spironolakton kul-lanan
hastalarda hiponatremi, hiperkalemi, dehidratasyon gelimesi doza
baldr. nerilen dozlardakullanldnda bile serum sodyum ve potasyum
dzeylerinin takibi gerekmektedir.
AAnnaahh ttaarr KKee llii mmee lleerr:: Kalp yetersizlii; doutan
kalp hastal; spironolakton; hiperkalemi; hiponatremi
PPeeddiiaattrr HHeeaarrtt JJ
22001155;;22((44))::116699--7733
Alev ARSLAN,a
Nazan ZBARLAS,a
Sevcan ERDEM,a
Osman KKOSMANOLUa
aDivison of Pediatric Cardiology,ukurova University Faculty of
Medicine,Adana
Ge li Ta ri hi/Re ce i ved: 06.07.2015Ka bul Ta ri hi/Ac cep
ted: 15.12.2015
Ya z ma Ad re si/Cor res pon den ce:Alev ARSLANBakent University
Faculty of Medicine,Adana Teaching and Medical Research
Center,Division of Pediatric Cardiology,
Adana,TRKYE/[email protected]
Copyright 2015 by Trk Pediatrik Kardiyoloji veKalp Cerrahisi
Dernei
ORJNAL ARATIRMA
-
ongestive heart failure is the state of sys-temic and pulmonary
congestion resultingfrom inability of the heart to pump as much
blood as required for the adequate metabolism ofthe body.. The
most common reason of congestiveheart failure (CHF) in early
infancy is congenitalheart defects with large left-to right shunts
(VSD,PDA, AVSD).1 The goals of medical treatment forheart failure
are to reduce the preload, enhancecardiac contractility and reduce
the afterload.Heart failure results from excessive fluid
retentionin large left-to-right shunts and diuretics are
usedprimarily to control symptoms of pulmonary andperipheral
congestion. Treatment paradigm in chil-dren has been adapted from
reports acquired inadults with heart failure. Afterload reduction
is themainstream of heart failure therapy; its efficacy inheart
failure is related to disruption of activation ofthe
renin-angiotensin axis and to reduction of car-diac adrenergic
drive. Aldosterone-blocking agentsare beneficial not only due to
their diuretic effect,but rather due to blockade of aldosterone.2
In pa-tients with severe heart failure, which cant betaken under
control, and particularly if there isneed for higher doses of
diuretics, aldosterone an-tagonists can be added to the
combination.3 The al-dosterone antagonists (most commonly used one
isspironolactone) act as potassium-sparing agents andalso have
antifibrotic and antiremodelling effectson the myocardium.4,5 The
side effects of drugs aremore intense in infancy due to specific
reasonssuch as the premature structure of the kidneys,
im-plementation of inappropriate medication times,dosage
adjustments and dilution of the tablet formsof the drugs. The
hydration status, kidney func-tions and blood electrolyte levels
need to be care-fully controlled particularly when spironolactoneis
used with angiotensin converting enzyme in-hibitors (ACEi). The aim
of this study is to evaluatethe effect of spironolactone,
furosemide and capto-pril combination on blood electrolyte levels
of in-fants with congenital heart disease and congestiveheart
failure, which cant be taken under control.
MATERIAL AND METHODS
Thirty infants aged less than 1 year who had con-genital heart
defects with CHF and could not be
controlled by the use of captopril and furosemide atleast one
month were included in the study (all pa-tients were discussed for
early corrective surgery).The heart failure score was made
according to themodified Ross scoring system (Table 1).6
Infantsscored as moderate and severe congestive heart fail-ure were
included in the study. Furosemide mainte-nance dosage was 1
mg/kg/day given as a single doseand captopril dosage was 0.5
mg/kg/dose every 12hours for all patients. They were given
spironolac-tone with 2 doses of 1-3 mg/kg/day. The bloodsodium,
potassium and creatinine levels, and hydra-tion status were
evaluated before and at the firstweek of the combination treatment
(summarized inTable 2). Dosage of spironolactone was 2 mg/kg/dayin
Group A and >2mg/kg/day in Group B infants. Attheir 1st week
control, the spironolactone dosageswere arranged according to their
electrolyte levelsand hydration statuses. Mild hyponatremia is
definedas a serum sodium concentration between 130 and135 meq/L,
moderate hyponatremia as a concentra-tion between 125 and 129
mEq/L, and severe hy-ponatremia as a concentration less than 125
mEq/L.
Written informed consent was obtained fromall families and the
study was conducted in accor-
Alev ARSLAN et al. EFFECTS OF A COMBINATION OF SPIRONOLACTONE,
FUROSEMIDE AND CAPTOPRIL...
Pediatr Heart J 2015;2(4)170
0 +1 +2
History
Diaphoresis Head only Head and body, Head and body,
at exertion at rest
Tachypnea Rare Several times Frequent
Physical examination
Breathing Normal Retractions Dyspnea
Respiratory rate (breaths/min) (years)
0-1 year 60
1-6 years 45
7-10 years 35
11-14 years 28
Heart rate (beats/min) (years)
0-1 year 170
1-6 year 115
7-10 year 100
11-14 year 90
Hepatomegaly size (cm) 3
TABLE 1: Modified Ross classification of heart failure.
CHF: Congestive heart failureTotal score: 0-2 (no CHF), 3-6
(mild CHF), 7-9 (moderate CHF), 10-12 (severe CHF).
-
dance with the Helsinki Declaration. Parents wereinformed about
the drugs, dosages, administrationtimes, amount of feeding, daily
diaper consump-tion, urine output and absolute clinical signs of
de-hydration and electrolyte imbalance.
STATISTICAL ANALYSIS
Statistical analyses were performed using SPSS soft-ware version
17. The variables were investigatedusing analytical methods
(Kolmogorov-Smirnovand Shapiro-Wilks tests) to determine whether
ornot they were normally distributed. Descriptiveanalyses were
presented using mean SD, me-dian (minmax value), and percentages
were ex-pressed where appropriate. The Wilcoxon testwas used to
compare the change in blood sodiumand potassium levels before and
after spironolac-tone treatment. The Mann-Whitney U test wasused to
compare blood sodium levels afterspironolactone treatment between
groups. A p-value of less than 0.05 was considered to be
sta-tistically significant.
RESULTS
Thirty patients were included in the study. Sixteenof the
patients were indicated as Group A and 14were indicated as Group B.
There were 11 patientsdiagnosed as complete atrioventricular septal
de-fect (AVSD) (7 of them were Down Syndrome), 1as partial AVSD
(large primum atrial septal defect,mitral cleft and severe mitral
insufficiency), 5 aslarge perimembranous outlet ventricular septal
de-fect (VSD), 8 as perimembranous inlet VSD, 3 asmoderate VSD +
patent ductus arteriosus and 2 aslarge aortopulmonary window. The
average age ofthe patients was 4.20.3 months (range 2-10months),
average weight was 4.81.5 kg (range 3-8.4 kg). Their average sodium
level was 1372.6mEq/L (range 131-142 mEq/L) and average potas-sium
level 4.60.4 mEq/L (range 3.5-5.4 mEq/L) be-fore the
spironolactone. The mean age of Group Apatients was 4.92.1 months
(range 1-10 months)and Group B was 4.52.1 months (range 2-9months).
The mean weight of the Group A patientswas 5.21.5 kg (range 3-7.9
kg) and Group B was4.81.2 kg (range 3-8.4 kg). There was no
statisti-cally difference between the groups with regard toweight
and age. The mean spironolactone dose was1.70.3 mg/kg (range 1.1-2
mg/kg) in Group A and2.30.2 mg/kg (range 2.1-2.8 mg/kg) in Group
B.The pre-treatment and 1st week control values ofelectrolyte
levels, BUN and creatinine results ofthe groups have been
summarized in Table 2 andFigure 1.
EFFECTS OF A COMBINATION OF SPIRONOLACTONE, FUROSEMIDE AND
CAPTOPRIL... Alev ARSLAN et al.
Pediatr Heart J 2015;2(4) 171
GroupA (n:16) Group B (n:14)mean SD mean SD(min-max) (min-max) P
values
Pretreatment Na+ (mEq/L) 1362.6 1382.4 0.120
(131-141) (134-142)
First week control (mEq/L) 1354.2 1333.7* 0.060
blood Na+ (126-141) (128-141)
Pretreatment K+ (mEq/L) 4.60.6 4.60.2 0.700
(3.5-5.4) (4.3-5)
First week control blood 4.50.5 4.80.3** 0.250
K+ (mEq/L) (3.4-5.2) (4.3-5.4)
Pretreatment BUN (mg/dL) 10.12.6 10.13.7 >0.05
(6.4-14.7) (4.8-20)
First week control BUN 11.53.9 12.37.5 >0.05
(mg/dL) (6.4-20) (4.3-29)
Pretreatment Cr (mg/dL) 0.290.17 0.30.12 >0.05
(0.2-0.9) (0.2-0.6)
First week control Cr (mg/dL) 0.240.1 0.35 0.002
(0.2-0.5) (0.2-2.7)
TABLE 2: Blood Na+ and K+ values before and after spironolactone
treatment.
* Significant decrease in blood Na levels after spironolactone
in Group B (p=0.009)** Significant increase in blood potassium
levels after spironolactone in Group B(p=0.016) descriptive
analysis was presented using median (min-max) due to non-normal
distribution)
FIGURE 1: Blood Na+ values before and after spironolactone
treatment.
-
In the 1st week of the treatment, hyponatremiawas detected in 15
of all patients (50%), which wasstatistically significant
(p=0.005). There were 10patients with mild and 5 with moderate
hypona-tremia (Table 3). In the first week control, 3 ofthem were
hospitalized for moderate dehydra-tion, hydrated intravenously and
spironolactonewas discontinued. Eight cases with mild dehy-dration
were hydrated orally and spironolactonedosage was reduced. In 4
cases without dehydra-tion and better clinical statuses, only
spironolac-tone dosage was reduced. Two days later,
hydrationstatuses and electrolyte levels were improved in
allpatients.
According to the first week control results,there was a
statistically significant difference be-tween blood sodium levels
in all patients (p135 mEq/L 11 (68.8%) 4 (28.6%) 15 (50%)
130-135 mEq/L (mild hyponatremia) 3 (18.8%) 7 (50%) 10
(33.3%)
125-129 mEq/L (moderate hyponatremia) 2 (12.5%) 3 (21.4%) 5
(16.7%)
TABLE 3: Blood sodium levels and hyponatremiaseverity per
groups.
-
EFFECTS OF A COMBINATION OF SPIRONOLACTONE, FUROSEMIDE AND
CAPTOPRIL... Alev ARSLAN et al.
standard deviations and narrow data ranges, mini-mal changes
could make the difference statisticallysignificant.
The hormone aldosterone affects glomerulardistal tubules and
increases reabsorption of sodiumand water. Spironolactone is
generally a weak na-triuretic agent with diuretic activity related
to thelevel of aldosterone; however, when it is combinedwith loop
diuretics, its diuretic effect becomes potentiated. The combination
of aldosterone bloc-king agents, ACEi and a loop diuretic can cause
ex-cessive diuresis and natriuresis. Development ofhyperkalemia may
be prevented with the effects ofloop diuretics.9
In the current study, hyponatremia was ob-served in both patient
groups at different spirono-lactone dosages; however, their
clinical statusrecovered quickly after reduction or cessation
ofspironolactone. Therefore, if this triple combina-tion is going
to be used, even at the spironolactonedose of 1 mg/kg/day, the
serum sodium and potas-sium levels should be monitored carefully.
Therecommended spironolactone dose when com-
bined with loop diuretics should not exceed 2 mg/kg/day unless
mandatory.
CONCLUSION
Close clinical and laboratory monitoring is neces-sary in the
combination treatment of heart failure.Excessive diuresis,
hyponatremia and hyperkalemiamay worsen the patients clinical
condition andheart failure. It has been determined that, when
aspironolactone range of 1-3 mg/kg/dose is to beadded to a
combination of furosemide and ACEi,the potassium levels are not
affected but hypona-tremia can develop in short-term electrolyte
mon-itoring. Therefore, close electrolyte monitoring isnecessary.
Parents should be counselled about theamount of feeding, daily
diaper consumption, urineoutput and absolutely clinical signs of
dehydrationand electrolyte imbalance.
AAcckknnoowwlleeddggeemmeennttssThe authors gratefully
acknowledge to ala Sartrk forreviewing the statistical analyses and
zgr Sandal whochecked for language.
Pediatr Heart J 2015;2(4) 173
1. Scott DJ, Rigby ML, Miller GA, ShinebourneEA. The
presentation of symptomatic heartdisease in infancy based on 10
years experi-ence (1973-82). Implications for the provisionof
services. Br Heart J 1984;52(3):248-57.
2. Shaddy RE, Tani LY. Chronic heart failure inchildren. In:
Allen HD, Driscoll DJ, Shaddy RE,Feltes TF, eds. Moss and Adams'
Heart Disease in Infants, Children, and Adolescents.8th ed. Vol 2.
Philadelphia: Lippincott Williams& Wilkins; 2013.
p.1565-78.
3. Blume ED, Freed MD, Colan SD. Congestiveheart failure. In:
Keane JF, Lock JE, Fyler DC,eds. NADAS Pediatric Cardiology. 2nd
ed.Philadelphia: Saunder Elsevier; 2006. p 83-97.
4. Pitt B, Zannad F, Remme WJ, Cody R, Castaigne A, Perez A, et
al. The effect ofspironolactone on morbidity and mortality in
patients with severe heart failure. Ran-domized Aldactone
Evaluation Study Inves-tigators. N Engl J Med
1999;341(10):709-17.
5. Ezekowitz JA, McAlister FA. Aldosteron block-ade and left
ventricular dysfunction: a sys-temic review of randomized clinical
trials. EurHeart J 2009;30(4):469-77.
6. Ross RD. The Ross classification for heart fail-ure in
children after 25 years: a review and anage-stratified revision.
Pediatr Cardiol 2012;33(8):1295-300.
7. Randomized Aldactone Evaluation Study In-vestigators. ACE
inhibitor co-therapy in pa-tients with heart failure: Rationale for
theRandomized Aldactone Evaluation Study(RALES). Am J Cardiol
1996;78(8):902-7.
8. Saito M, Takada M, Hirooka K, Isobes F, Ya-sumura Y. Serum
concentration of potassiumin chronic heart failure patients
administeredspironolactone plus furosemide and eitherenalapril
maleate, losartan potassium or can-desartan cilexetil. J Clin Pharm
Ther 2005;30(6):603-10.
9. Dedieu N, Burch M. Understanding and treat-ing heart failure
in children. Paediatrics andChild Health 2013;23(2):47-52.
REFERENCES
-
Pediatr Heart J 2015;2(4)174
Evaluation of Cardiac Functions inChildren with Familial
Mediterranean Fever
AABBSS TTRRAACCTT OObbjjeeccttiivvee:: Familial Mediterranean
Fever (FMF) is a chronic inflammatory disease characterized
byrecurrent attacks of serositis. Colchicine treatment is known to
prevent clinical attacks. This study aimed to eval-uate cardiac
functions of children with FMF and compare with those of healthy
controls, and secondly, to revealthe difference in cardiac indices
between patients on regular colchicine treatment versus those
recently startedon colchicine treatment. MMaatteerriiaall aanndd
MMeetthhooddss:: 45 patients and 24 healthy controls were
evaluated. Patientswere divided into two subgroups. Group I
consisted of patients who were on regular colchicine treatment
andGroup II consisted of patients who just started using
colchicine. Demographic features, two dimensional (2D), M-mode,
pulsed wave (PW) and tissue Doppler imaging (TDI) echocardiography
findings of the patients and the con-trols were evaluated.
RReessuullttss:: Although the demographic features and conventional
(2D, M-mode and PW)echocardiographic parameters of FMF patients
were not significantly different, the TDI parameters
significantlydiffered between the patients and the controls. The Am
values were significantly higher in Group I (0.090.02cm/s), and
Group II (0.080.01 cm/s) versus the control group (0.070.02 cm/s),
(p=0.001, p=0.026; respectively).Em/Am values of only Group I was
significantly lower than the control group (1.930.37, 2.20.36,
respectively;p=0.023). Although lower in Group II, the difference
of Em/Am values did not reach significance in betweenGroup II and
controls (p=0.130). IVRT was significantly shorter in Group II than
Group I and the control group(Group I, II and control groups
43.697.71 ms, 33.2513.65 ms and 41.9611.54 ms, respectively;
p=0.01). CCoonn--cclluussiioonn:: Although conventional systolic
and diastolic echocardiographic parameters were in normal ranges,
someof the diastolic functions demonstrated by TDI echocardiography
were impaired in FMF patients with or with-out colchicine
treatment. While only late diastolic filling was found to be
affected in children with an initial di-agnosis, the lower Em/Am
values also reached a statistical significance in FMF patients on
regular colchicinetreatment when compared to the control group. We
suggest that effective amounts of colchicine treatment whichare
sufficient for prevention of overt inflammatory attacks and
amyloidosis do not prevent deterioration of dias-tolic indices.
KKeeyy WWoorrddss:: Familial Mediterranean Fever; cardiac
diastolic functions; colchicine
ZZEETT AAmmaa:: Ailevi Akdeniz Atei (AAA) tekrarlayan serozit
ataklar ile karakterize kronik inflamatuar bir has-talktr. Kolisin
tedavisinin klinik ataklar nledii bilinmektedir. Bu almada AAA tans
ile izlenmekte olanhastalarn kardiyak fonksiyonlarnn
deerlendirilmesi ve salkl kontrollerle karlatrlmas ve ikinci olarak
d-zenli kolisin tedavisi alan ve kolisin tedavisi yeni balanan
hastalarn kardiyak fonksiyonlarnn karlatrl-mas planland. GGeerree
vvee YYnntteemmlleerr:: Krkbe hasta ve 24 salkl kontrol
deerlendirildi. Hastalara iki grubaayrld. Grup I dzenli kolisin
tedavisi kullanan hastalardan ve Grup II kolisin tedavisi yeni
balanan hastalar-dan oluturuldu. Hastalarn ve salkl kontrollerin
demografik zellikleri, iki boyutlu (2D), M-mod, pulsed Dopp-ler
(PW) ve doku Doppler (DD) ekokardiyografi bulgular deerlendirildi.
BBuullgguullaarr:: AAA hastalar ve kontrollerinarasnda demografik
bulgular ve konvansiyonel ekokardiyografi bulgular (2D, M mode ve
PW), asndan anlamlfark saptanmamakla beraber DD parametreleri
kontrollerden anlaml derecede farkl idi. Grup I ve II Am
deerleri(srasyla 0,090,02 cm/s, 0,080,01 cm/s) kontrol grubundan
(0,070,02 cm/s) anlaml derecede yksek idi(srasyla p=0,001,
p=0,026). Sadece Grup Iin Em/Am deerleri kontrol grubundan anlaml
derecede dk idi(1,930,37, 2,20,36; p=0,023). Grup IIde daha dk
olmakla beraber Grup II ve kontrol grubu Em/Am deer-leri arasnda
istatistiksel olarak anlaml fark saptanmad (p=0,130). IVRT Grup II
de Grup I ve kontrol grubundananlaml derecede ksayd (Grup I, II ve
kontrol grubu srasyla 43,697,71 ms, 33,2513,65 ms, 41,9611,54
ms;p=0,01). SSoonnuu:: Kolisin tedavisi kullanan ya da kullanmayan
FMF hastalarnn konvansiyonel sistolik ve diasto-lik fonksiyonlar
normal olmakla beraber, baz DD diastolik ekokardiyografi
bulgularnda farkllk saptanmtr.Yeni tan alan hastalarda sadece ge
diastolik dolum etkilenmi grlmekle beraber, dzenli kolisin tedavisi
alanhastalarn Em/Am deerlerinde de kontrollere gre anlaml dklk
saptanmtr. Klinik inflamatuar ataklarve amiloidozu engellemek iin
kullanlan kolisin dozunun, diastolik kardiyak fonksiyonlarn
bozulmasn en-gellemediini dnyoruz.
AAnnaahh ttaarr KKee llii mmee lleerr:: Ailevi Akdeniz Atei;
kardiyak diastolik fonksiyonlar; kolisin
PPeeddiiaattrr HHeeaarrtt JJ
22001155;;22((44))::117744--8811
Pelin AYYILDIZ HACIMEROLU,a
Yonca AKIKGZ,b
Taner KASAR,a
Erkut ZTRK,a
brahim Cansaran TANIDIR,a
smail LEKc
aDepartment of Pediatric Cardiology,Mehmet Akif Ersoy Thoracic
and Cardiovascular Surgery Center and Research Hospital,
stanbulbDepartment of Pediatric Nephrology,Samsun Education and
Research Hospital,SamsuncDepartment of Pediatric Rheumatology,
mraniye Education and Research Hospital, stanbul
Ge li Ta ri hi/Re ce i ved: 13.10.2015Ka bul Ta ri hi/Ac cep
ted: 12.12.2015
This study was presented as a poster in 12th National Pediatric
Cardiology and Cardiovascular Surgery Congress, 1-5 May 2013,
Fethiye-Mula, Turkey.
Ya z ma Ad re si/Cor res pon den ce:Pelin AYYILDIZ
HACIMEROLUMehmet Akif Ersoy Thoracic and Cardiovascular Surgery
Center and Research Hospital, Department of Pediatric
Cardiology,stanbul,TRKYE/[email protected]
Copyright 2015 by Trk Pediatrik Kardiyoloji veKalp Cerrahisi
Dernei
ORJNAL ARATIRMA
-
EVALUATION OF CARDIAC FUNCTIONS IN CHILDREN WITH FAMILIAL
MEDITERRANEAN FEVER Pelin AYYILDIZ HACIMEROLU et al.
Pediatr Heart J 2015;2(4) 175
amilial Mediterranean Fever (FMF), alsocalled recurrent
polyserositis, is an autosomalrecessive disease characterized by
recurrent
attacks of serositis with accompanying fever, whichmostly
affects people of Mediterranean descent.Nonsense or missense
mutations in the MEFV genewere shown to be the cause in most
cases.1 In pa-tients with FMF, there is a lack of inhibiting
theactivity of chemotactic factors which leads toepisodes of
inflammation of serosal tissues.2 Apartfrom renal involvement,
clinical or subclinical car-diac involvement has also been reported
in patientswith FMF.3-5 Colchicine treatment is known to pre-vent
clinical attacks and amyloid development inmost patients; hoever,
it has been stated that sub-clinical inflammation continues during
the attack-free period.6
Cardiac functions of FMF patients were stud-ied in detail with
conventional echocardiograpy,which was a partially subjective
method as it isload-dependent. Tissue Doppler imaging is re-ported
to provide more accurate means of quanti-fying both ventricular
functions than conventionalechocardiography and to be more
sensitive forevaluating subclinical cardiac abnormalities.7
Thereare few studies in the pediatric population evalu-ating
cardiac functions, but to our knowledge,there is no study
evaluating the cardiac functions atthe time of initial diagnosis
and after a regulartreatment of colchicine.
This study aimed to evaluate cardiac functionsof children with
FMF in comparison with cardiacfunctions of normal children and
secondly to com-pare cardiac functions of two groups of
childrenwith FMF: patients who were on regular colchicinetreatment
and those who were only recently diag-nosed and put on colchicine
treatment.
MATERIALS AND METHODS
Data of 45 FMF patients and 24 healthy controlswere evaluated
retrospectively from patient files.All the patients fullfilled the
clinical criteria forFMF.8 Patients were divided into two
subgroupsaccording to the duration of colchicine treatment.Group I
consisted of patients who were oncolchicine treatment for more than
6 months with
normal renal function tests and Group II consistedof patients
who were just diagnosed and put oncolchicine. Group III (Control
group) consisted ofchildren who were referred to cardiology for
eval-uation for sports or innocent murmur.
Evaluation of the study population was per-formed during an
attack-free period. Patients withacute infection, acute attack
related to disease, pa-tients with other chronic diseases, valve
abnor-malities and those unresponsive to colchicinetreatment were
excluded. Body mass index was cal-culated as the ratio of weight in
kilograms to thesquare root of height in meters. Blood pressure
wasmeasured after 10 minutes of rest with a mercurysphygmomanometer
and an appropriate sized cuff.
The study was approved by local ethics com-mittee, written
informed consent was taken fromparents of all patients and healthy
controls.
Echocardiographic studies were performed bya commercially
available ultrasound system with3S and 6S sector transducers (VIVID
7 pro, GE,Milwaukee, WI, USA). An electrocardiogram
wassimultaneously recorded in all examinations. Allexaminations
were performed by the same experi-enced pediatric cardiologist
using standard viewsand techniques according to the guidelines of
theAmerican Society of Echocardiography, subjectslying in supine
position without any sedation.9
The measurements were calculated by averaging3 consecutive
cardiac cycles. The corrected andweighted kappa statistics for
intra-observer vari-ability demonstrated good intra-observer
agree-ment (kappa=0.72).
The standardized examinations included 2-di-mensionally guided
M-mode echocardiograms andselected 2-dimensional and Doppler
recordings.The tricuspid and mitral inflow velocities for
dias-tolic functions were recorded from the apical 4chamber view,
with the sample volume placed atthe tip of the leaflets of the
valves during diastole.10
Measured parameters were: peak velocity of earlydiastolic
filling wave (E), measured as the height ofmaximal deflection
during the first half of diastole;peak velocity of the atrial
filling wave (A), meas-ured as the height of maximal deflection
after the
-
Pelin AYYILDIZ HACIMEROLU et al. EVALUATION OF CARDIAC FUNCTIONS
IN CHILDREN WITH FAMILIAL MEDITERRANEAN FEVER
Pediatr Heart J 2015;2(4)176
plateau phase of the early filling period; the earlymitral flow
deceleration time (DTE), measured asthe time taken from the maximum
E point to base-line. Ratio of early to atrial peak velocity (E/A),
de-fined as peak velocity of the early diastolic fillingwave
divided by peak velocity of atrial filling wave,were also recorded.
Myocardial performance index(MPI) was calculated as the sum of
isovolumic con-traction time (IVCT) and isovolumic relaxationtime
(IVRT) divided by left ventricular ejectiontime (LVET).11 Diastolic
functions were also evalu-ated by tissue Doppler for left and right
ventricleswhile the sample volume was at the lateral annulusof
mitral valve and tricuspid valve, respectively, inthe apical
4-chamber view.
Statistical analyses were performed using theNumber Cruncher
Statistical System (NCSS 2007Statistical Software, Utah, USA)
statistical packprogramme. Data were expressed as the means
andstandard deviations. Groups were compared withone-way analysis
of variance (One way ANOVA),subgroup comparisons were done with
Tukey mul-tiple comparison test. Chi-square and Fischer real-ity
tests were used to compare qualitative data.Values for p less than
0.05 were considered sig-nificant.
RESULTS
There were no statistically significant differencesfor age, sex,
weight, height, body mass index, sys-tolic and diastolic blood
pressures between the
groups (p>0.05). The acute phase reactants [C reac-tive
protein (CRP), erythrocyte sedimentation rate,fibrinogen] were
within normal ranges in allgroups. There were no significant
differences foracute phase reactants between groups
(p>0.05),except CRP, which was significantly higher inGroups I
and II than that of Group III (p=0.019)(Table 1).
All patients were started recently or alreadyreceiving
colchicine at a dosage of 0.05-0.075mg/kg/day. The duration of
treatment was 3.172.86 (0.6-13) years in Group I.
All measured cardiac and left ventricle M-mode indices (left
atrium/aort ratio, interventricu-lar septum thickness, left
ventricular end-diastolicand end-systolic dimensions, left
ventricular pos-terior wall thickness), left ventricular ejection
frac-tion and fractional shortening and heart rate werein normal
ranges and did not differ between groups(p>0.05) (Table 2). Peak
mitral and tricuspid earlyand late diastolic velocities measured by
standardpulse wave Doppler, mitral valve early diastolicflow
deceleration time, E/A values, MPI valueswere similar in all groups
(p>0.05) (Table 3).
The tissue Doppler imaging data are given inTables 4 and 5.
There were statistically significantdifferences in Am and Em/Am
ratio betweengroups (p
-
EVALUATION OF CARDIAC FUNCTIONS IN CHILDREN WITH FAMILIAL
MEDITERRANEAN FEVER Pelin AYYILDIZ HACIMEROLU et al.
Group I Group II Group III pHeart rate/min 89.9615.05 91.0610.86
9215.01 0.874
LA diameter (cm) 2.370.47 2.230.36 2.30.34 0.549
Aort diameter (cm) 2.330.39 2.130.4 2.290.38 0.285
LA/Ao 1.020.14 1.060.16 1.020.16 0.708
IVSd (cm) 0.820.18 0.760.22 0.840.2 0.490
IVSs (cm) 1.020.24 1.060.2 1.010.22 0.808
LVEDd (cm) 3.680.51 3.730.62 3.680.52 0.943
LVIDs (cm) 2.280.28 2.280.42 2.340.38 0.807
LVPWd (cm) 0.80.18 0.730.16 0.770.23 0.522
LVPWs (cm) 1.080.3 10.25 10.19 0.478
EF (%) 68.656.17 69.884.9 67.225.76 0.359
FS (%) 37.815.08 38.883.84 36.744.5 0.363
TABLE 2: M-mode echocardiographic data.
Data were expressed as meanSD; LA: Left atrium; LA/Ao: Left
atrium/aort; IVS: Interventricular septum; LVEDD: Left ventricular
end diastolic dimension; LVESD: Left ventricular endsistolic
dimension; LVPW: Left ventricular posterior wall; EF: Ejection
fraction of the left ventricle; FS: Fractional shortening of the
left ventricle.
Group I Group II Group III pMitral inflow
E (m/s) 1.040.14 0.970.22 1.050.15 0.275
A (m/s) 0.580.11 0.550.09 0.580.09 0.584
E/A 1.850.3 1.790.41 1.850.38 0.867
DT Em (ms) 148.450.84 141.1428.67 149.139.28 0.813
MPI (%) 0.40.07 0.420.09 0.410.06 0.462
Tricuspid inflow
E (m/s) 0.750.11 0.730.12 0.750.1 0.827
A (m/s) 0.420.07 0.40.06 0.430.07 0.471
E/A 1.80.3 1.830.32 1.790.34 0.912
TABLE 3: Standard Pulsed Wave Doppler echocardiographic
data.
Data were expressed as meanSD; E: Peak early diastolic velocity;
A: Peak late diastolic flow velocity; DT Em: Early diastolic flow
deceleration time; MPI: Myocardial performance index.
Group I Group II Group III pMitral annulus
Em (cm/s) 0.160.03 0.160.02 0.150.03 0.249
Am (cm/s) 0.090.02 0.080.01 0.070.02 0.001
Em/Am 1.930.37 1.970.33 2.20.36 0.023
DT Em (ms) 88.7718.81 87.839.08 84.0116.39 0.553
IVRT (ms) 43.697.71 33.2513.65 41.9611.54 0.01
Tricuspid annulus
Et (cm/s) 0.180.02 0.170.03 0.180.02 0.633
At (cm/s) 0.110.02 0.110.02 0.110.02 0.629
Et/ At 1.590.27 1.620.41 1.650.36 0.775
TABLE 4: Pulsed Wave Tissue Doppler Echocardiographic data. Data
were expressed as meanstandard deviation.
Em peak vel: Early diastolic myocardial peak velocity of mitral
annulus; Am peak vel: Late diastolic myocardial peak velocity of
mitral annulus; DT Em: Early diastolic myocardial peakvelocity
deceleration time; IVRT: Isovolumic relaxation time; Et peak vel:
Early diastolic myocardial peak velocity of tricuspid annulus; At
peak vel: Late diastolic myocardial peak ve-locity of tricuspid
annulus.
Pediatr Heart J 2015;2(4) 177
-
Pelin AYYILDIZ HACIMEROLU et al. EVALUATION OF CARDIAC FUNCTIONS
IN CHILDREN WITH FAMILIAL MEDITERRANEAN FEVER
Pediatr Heart J 2015;2(4)178
Groups I and II (p=0.838). Em/Am values of thecontrol group were
significantly higher than GroupI (p=0.023). Although Em/ Am values
of the con-trol group were higher than Group II, differencewas not
statistically significant (p=0.130). Therewas a statistically
significant difference of IVRT pa-rameters between groups (p=0.01)
where IVRT val-ues of Group II were significantly shorter thanthose
of Group I and the control group (p=0.009,p=0.039, respectively).
There was no statisticallysignificant difference in IVRT values
betweenGroup I and the control group (p=0.838).
DISCUSSION
In this study, cardiac functions of patients with fa-milial
Mediterranean fever (FMF) were evaluatedwith conventional and
tissue Doppler echocardio-graphy and compared with healthy
controls. Wedemonstrated that while systolic and
conventionaldiastolic functions were in normal ranges, some ofthe
tissue Doppler parameters were impaired inFMF patients. Secondly,
the cardiac functions ofpatients who were on regular colchicine
treatmentand who were only recently diagnosed and startedcolchicine
treatment were evaluated in two sub-groups. We found impairment in
some of the dias-tolic parameters in recently diagnosed
patientswithout colchicine treatment (significantly higherpeak A
velocity, lower but not statistically signifi-cant Em/Am values)
and subclinical diastolic dys-function in the patient group on
regular colchicinetreatment (significantly lower Em/Am values)when
compared with controls.
Echocardiographic assessment of ventriculardiastolic functions
relied on Doppler patterns of
mitral and tricuspid inflow. Reflecting the pressuregradient
between the atrium and ventricle, trans-valvular velocities are
directly related to atrial pres-sure (preload) and inversely
related to ventricularrelaxation. The principle of detection of the
shiftin frequency of ultrasound signals reflected frommoving blood
cells in conventional Doppler tech-niques, are used to quantify the
higher amplitude,lower-velocity signals of myocardial tissue
motionin TDI. The annulus initially moves away from theapex during
diastole and then back towards theapex during atrial contraction
and these values arecomparable with those of transmitral
flow.12
The E peak arises due to early diastolic filling.Approximately
70-75% of the ventricular filling isduring this phase. The A peak
arises due to atrialcontraction, pumping 20-25% of stroke
volumeinto the ventricle. The A wave includes flow oc-curring
during atrial systole.13 Matsuda et al. statedthat a wave of left
atrial systolic pressure is not asimple wave produced by left
atrial systole, it in-cludes a component of reflection associated
withincreased left ventricular end diastolic pressure.14
The E/A ratio is a marker of the function ofthe ventricle of the
heart. The abnormalities in theE/A ratio suggest that the ventricle
cannot fill prop-erly in the period between contractions.13
There are few previous studies demonstratingthe subclinical
changes in diastolic cardiac func-tions in children with FMF.
zdemir et al evalu-ated both right and left ventricular functions
andfound right ventricular diastolic dysfunction inFMF patients.
They suggested that right ventriclemight be affected first due to
the difference in mor-phology of the right ventricle when compared
toleft ventricle.15 On the other hand, Sari et al eval-uated 44
adult FMF patients (median age: 30, range:19-47 years) who were on
regular daily colchicinetreatment and measurements of the study
wereperformed during the attack-free period and sug-gested that
subclinical myocardial involvement ispresent in relatively young
FMF patients who werealso free of classical cardiovascular risk
factors.16
Tavil et al reported impaired left ventricular dias-tolic
indices with tissue Doppler imaging in adultFMF patients.17
Likewise Baysal et al studied car-
Group 1- Group 1- Group 2-p values Group 2 Group 3 Group 3
Am (cm/s) 0.838 0.001 0.026
Em/ Am 0.924 0.023 0.130
IVRT (ms) 0.009 0.838 0.039
TABLE 5: p values in between groups.
Em peak vel: Early diastolic myocardial peak velocity of mitral
annulus; Am peak vel: Late diastolic myocardial peak velocity of
mitral annulus; IVRT: Isovolumic relaxation time.
-
EVALUATION OF CARDIAC FUNCTIONS IN CHILDREN WITH FAMILIAL
MEDITERRANEAN FEVER Pelin AYYILDIZ HACIMEROLU et al.
diac functions in children with FMF with bothconventional and
tissue Doppler echocardiographyand reported significant changes in
left ventriclediastolic indices of FMF patients when comparedwith
controls while systolic functions were simi-lar.4 Nearly all of the
studies mentioned abovefound, significant or not, a lower peak E
velocity, ahigher peak A velocity and lower Em/Am param-eters in
patients with FMF.4,16,17 Baysal et al.claimed that the increase of
peak A velocity in chil-dren with FMF was probably due to increased
leftventricular chamber stiffness caused by FMF.4 Sup-porting these
studies, the present study foundhigher peak A velocity values in
patients with FMFboth with and without colchicine treatment,
alsolower but not significant Em/Am ratios in initiallydiagnosed
patients and significantly lower Em/Amvalues in FMF patients on
regular colchicine treat-ment.
Isovolumic relaxation time is an interval inthe cardiac cycle,
from the end of the aortic ejec-tion to the onset of filling by
opening of the mitralvalve. Two different opposing processes of
IVRTcan be defined with left ventricular disease. Pro-longation by
the disease process as in left ventric-ular hypertrophy, diabetes,
or coronary arterydisease or shortening as left atrial pressure
rises.18 Avery short IVRT is a sign of a raised left atrial
pres-sure. If it is taken into consideration how a patientmay
improve clinically while at the same time di-astolic measurements
become more abnormal, itshould be remembered that the most common
wayfor diastolic measurements to normalize is for leftatrial
pressure to rise.19 In the present study, theIVRT values of FMF
patients without colchicinetreatment were significantly shorter
than FMF pa-tients on regular colchicine treatment and the con-trol
group. The shortening of IVRT might beexplained by the attempt to
normalize diastolicmeasurements in untreated FMF patients by
in-creasing left atrial pressure.
Previous studies reported the potential forchronic inflammatory
diseases like systemic lupuserythematosus or rheumatoid arthritis
to impairvascular and cardiac functions.20-22 The main
patho-physiology claimed for developing cardiac impair-
ment in those disorders is the inflammation, whichmay accelerate
the development of atherogenesis,thrombosis and congestive heart
disease. Systemicinflammation is also claimed to be an
importantfactor for development of amyloidosis in whichdeposition
of these amyloid fibrils in the cardiac in-terstitium and
conducting system leads to restric-tive cardiomyopathy,
arrhythmias, eventuallybiventricular heart failure and death.23
Supportingthese studies, presence of subclinical athereoscle-rosis
was reported in FMF patients demonstratedwith increased carotid
intima media thickness.3,24
Likewise, it was reported that subclinical inflam-mation occurs
over prolonged periods in patientswith FMF and infrequent
clinically overt attacksrepresent the tip of the iceberg in FMF
patients.23
Yalnkaya et al evaluated 36 FMF patients andtheir 39 healthy
parents (obligate heterozygotes)and reported that acute phase
reactants in FMF pa-tients during attack-free periods and their
rela-tives were significantly higher than healthycontrols,
indicating the presence of continuingsubclinical inflammation
during the attack-freeperiods in patients with FMF receiving
regularcolchicine therapy.25 Similar observations havebeen made by
other authors like Dzova andLanchmann et al.23,26 Similarly, Baysal
et al. foundhigher CRP and serum amyloid A levels in FMF pa-tients
when compared to controls which supportedan ongoing subclinical
inflammation.4 In anotherstudy, coronary flow reserve were found
impairedin patients with FMF and they found positive cor-relation
between the severity of the impairmentand CRP levels, which also
supports the findingthat cytokine levels remain elevated even in
the at-tack-free period.27,28 Our results also
demonstratedsignificantly higher, albeit within normal limits,
ofacute phase reactant levels in FMF patients receiv-ing regular
colchicine therapy supporting ongoingsubclinical inflammation in
the attack-free periodas in the previous reports.
Colchicine is an alkaloid drug that is effec-tively used in
several inflammatory diseases likegout, Behets disease and it is in
use since 1970s toprevent the acute attacks of FMF.29 Regular
pro-phylactic treatment with colchicine at a dose of 1
Pediatr Heart J 2015;2(4) 179
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Pelin AYYILDIZ HACIMEROLU et al. EVALUATION OF CARDIAC FUNCTIONS
IN CHILDREN WITH FAMILIAL MEDITERRANEAN FEVER
Pediatr Heart J 2015;2(4)180
2 mg daily was reported to prevent or substantiallyreduce the
clinical manifestations of FMF in at least90% of cases.6 There are
many reports demon-strating the efficacy of colchicine in
prevention ofinflammatory attacks and amyloid developmentalong with
studies reporting ongoing subclinical in-flammation in FMF patients
despite colchicinetreatment.6,29-31
Our results demonstrated diastolic dysfunctionin patients with
FMF despite colchicine treatment.To the best of our knowledge, none
of the reportsabout the cardiac functions of FMF patients
evalu-ated the cardiac functions at the beginning of thediagnosis
before a regular colchicine treatment.Furthermore, some of the
authors noted as a limi-tation of their study that all of their
study patientswere on regular colchicine treatment and theycould
not compare the results of colchicine re-sponders with
nonresponders and claimed thatcolchicine probably protects patients
from theharmful effects of inflammation and its inverse ef-fects on
the heart.4,15 We suggest that effectiveamounts of colchicine
treatment that is reported tobe sufficient for prevention of overt
inflammatoryattacks and amyloidosis does not prevent deterio-ration
of diastolic indices, thus, impairment insome of the diastolic
parameters is present from thebeginning of the symptoms or probably
much ear-
lier and maintained despite regular colchicinetreatment.
Although total number of patients was com-parable to some of the
previous studies evaluatingcardiac functions of children and adults
withFMF, the small number of subgroups and short-ness of duration
of the disease were the limita-tions in this study. The results of
this studysuggested that larger scale prospective longitudi-nal
studies are needed before definitive conclu-sions can be made and
recent study might be aprelude for this purpose.
In conclusion, although conventional systolicand diastolic
echocardiographic parameters werein normal ranges, some of the
diastolic functionsdemonstrated by TDI were impaired in FMF
pa-tients with or without colchicine treatment. Theseresults and
previous studies support that the longerthe duration of the
disease, the worse the diastoliccardiac indices, probably due to
ongoing subclini-cal inflammation which is claimed to accelerate
thedevelopment of atherogenesis, thrombosis inchronic inflammatory
diseases like FMF despiteregular colchicine treatment. Therefore,
life-longfollow-up by noninvasive screening methods canbe
recommended for these patients for early un-masking of cardiac
deterioration as in other chronicinflammatory diseases.
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Pediatr Heart J 2015;2(4) 181
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Pediatr Heart J 2015;2(4)182
Yenidoanda Kardiyoloji Konsltasyonu:Kardiyak Anomali Skl ve
Etiyolojik Faktrler
ZZEETT AAmmaa:: Bu alma, hastanemiz yenidoan nitesine yatan
hastalar arasnda ocuk kardiyolojisi kon-sltasyonu istenenlerde
konjenital kalp hastalklarnn (KKH) skl ve dalm ile etyolojik
faktrlerinideerlendirmek amacyla yapld. GGeerree vvee
YYnntteemmlleerr:: almamz, 4553 hasta arasndan ocuk kardiyo-lojisi
konsltasyonu istenen 573 hasta prospektif olarak incelenerek
gerekletirildi. Olgularn antenataltan ve bulgular, antenatal risk
faktrleri (diyabet, preeklampsi, erken membran rptr, enfeksiyon,
oli-gohidramnios, polihidramnios, uyuturucu madde ve ila kullanm),
ekokardiyografi yaplma nedenleriile elde edilen tanlar arasndaki
ilikiye bakld. BBuullgguullaarr:: almaya alnan 573 hastann130unda
(%22,6)KKH tespit edildi. Konjenital kalp hastal tespit edilen 130
hastann %8,4 siyanotik, %91,6s asiyano-tik KKHye sahipti.
Asiyanotik KKHler arasnda en sk ventrikler septal defekt (%42,3,),
atriyal septal de-fekt (%35,3), patent duktus arteriyozus (%9,2),
pulmoner stenoz (%7) grlrken, siyanotik KKHler iindeFallot
tetralojisi (%3) ile byk arter transpozisyonunun (%1,5) en sk olduu
tespit edildi. Konjenitalkalp hastal tans alan olgularn %96,1inin
kardiyovaskler sistem d nedenlerle hastaneye yatrldgrld. ocuk
kardiyolojisi konsltasyonu isteme sebeplerinin banda en sk takipne
(n=183), saturas-yon dkl (n=100) ve frm (n=73) vard. Konjenital
kalp hastal grlme sklnn zellikle e-itli anomalilere sahip ve
sendromlu olgularda artt ortaya konarak genetik sendrom tanl ya
dasendromik zellikleri olan bu olgularn %50sinde KKH olduu tespit
edildi. Down sendromlu olgularn%71inde KKH tespit edilmi olup bu
KKHlerin %57sinin atriyoventrikler septal defekt olduu
grld.SSoonnuu:: Konjenital kalp hastalklarnn yenidoan dneminde
asemptomatik olabilecei gibi, konjestif kalpyetersizlii bulgular,
siyanoz ile ortaya kabilecei zellikle eitli anomalilere sahip ve
sendromlu ol-gularda sklnn artt bir kez daha ortaya kondu.
AAnnaahh ttaarr KKee llii mmee lleerr:: Yenidoan; kardiyoloji
konsltasyonu; doumsal kalp hastal
AABBSS TTRRAACCTT OObbjjeeccttiivvee:: This study is performed
in order to detect congenital heart disease incidence andthe
etiological factors that contributed in patients who were
hospitalized in neonatal care unit in our hos-pital and consulted
to pediatric cardiology. MMaatteerriiaall aanndd MMeetthhooddss::
Our study was performed with 573 of4553 patients who were consulted
to pediatric cardiology prospectively. The antenatal diagnosis and
find-ings of the patients, the antenatal risk factors (diabetes,
preeclampsia, early membrane rupture, infection,oligohydramnios,
polyhydramnios, narcotic and drug usage), the reason of
echocardiographic evaluationand the disease that were found were
evaluated and the relationship between the findings were
evaluated.RReessuullttss:: Congenital heart failure was found in
130 (%22,6) of 573 patients who were enrolled to the study.%8,4
were cyanotic and %91,6 were non-cyanotic in these 130 patients.
While ventricular septal defect(%42,3), atrial septal defect
(%35,3), patent ductus arteriosus (%9,2) and pulmonary stenosis
(%7) weremost frequently seen in non-cyanotic patients, tetralogy
of Fallot (%3) and big artery transposition (%1,5)were most
frequently in cyanotic patients. The %96.1 of patients with
congenital heart disease were hos-pitalized because of other
reasons rather than cardiac diseases. Tachypnea (n: 183),
desaturation (n: 100),and murmur (n: 73) were the most frequent
consultation to cardiology reasons. The incidence of con-genital
heart disease is again found to be increased in patients with
several congenital anomalies and syn-dromes and %50 of these
patients had congenital heart disease. Congenital heart disease was
found to be%71 of the patients with Down syndrome and %57 was
atrioventriculer septal defect. CCoonnlluussiioonn:: In neona-tal
period, it may be either asymptomatic or congestive heart failure
and cyanosis may be seen. The inci-dence of congenital heart
disease is again found to be increased in patients with several
congenitalanomalies and syndromes.
KKeeyy WWoorrddss:: Newborn; consultation to cardiology;
congenital heart disease
PPeeddiiaattrr HHeeaarrtt JJ 22001155;;22((44))::118822--88
Saadet ELK CENGZ,a
Ali Rahmi BAKLER,b
Ula KARADA,b
Kay ELAIK,a
ule DEMR,a
Buket DORUSZ,b
Esra ARUN ZERc
aocuk Sal ve Hastalklar Klinii,bocuk Kardiyolojisi
Klinii,cYenidoan Klinii,zmir Tepecik Eitim ve Aratrma Hastanesi,
zmir
Ge li Ta ri hi/Re ce i ved: 22.10.2015Ka bul Ta ri hi/Ac cep
ted: 19.01.2016
Ya z ma Ad re si/Cor res pon den ce:Ula KARADAzmir Tepecik Eitim
ve Aratrma Hastanesi,ocuk Kardiyolojisi Klinii,
zmir,TRKYE/[email protected]
Copyright 2015 by Trk Pediatrik Kardiyoloji veKalp Cerrahisi
Dernei
ORJNAL ARATIRMA
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YENDOANDA KARDYOLOJ KONSLTASYONU: KARDYAK ANOMAL SIKLII...
Saadet ELK CENGZ ve ark.
Pediatr Heart J 2015;2(4) 183
onjenital kalp hastalklar, doutan olananomalilerin iinde en sk
rastlanlandr.Bir yenidoann konjenital kalp hastalkl
doma riski 1000 canl doumda 8-12dir (yaklak%1).1-3
Konjenital kalp hastalklarnn ou olgudanedeni bilinmemekle
birlikte, genetik ve evre-sel faktrlerin birlikte etkileimine bal
olduudnlr. Yenidoan dneminde KKHlerin kli-nik bulgular anatomik
bozuklua gre deikenlikgsterir. Solunum zorluu, siyanoz, ok tablosu
gibiar belirtilerden veya asemptomatik olarak fizikmuayenede tek
bana frm saptanmasndan yada genetik bir sendromun bulgularndan yola
k-larak yaplan ekokardiyografik deerlendirmedetespit
edilebilir.4,5
Tannn erken konularak tedavi planlanmas,konjenital kalp hastalkl
ocuklarn morbidite vemortalitesinin azaltabilmesi asndan
nemlidir.Ancak klinik bulgular erken dnemde ortaya k-mayabilir.
Tandaki gecikmeler nedeniyle erken te-davi ve giriimlerin
uygulanamamas durumundabu hastalklar lmcl seyredebilir. En erken
tanise, prenatal dnemde yaplan fetal ekokardiyografiile
konulabilir.6-8
Bu alma, hastanemiz yenidoan nitesineyatan hastalar arasnda ocuk
kardiyolojisi konsl-tasyonu istenmi olanlarda KKHnin skl ve da-lm
ile etiyolojik faktrlerini deerlendirmekamacyla yapld.
GERE VE YNTEMLER
almamz, 1 Kasm 2012-30 Haziran 2014 tarih-leri arasnda zmir
Tepecik Eitim ve AratrmaHastanesi yenidoan nitesinde yatrlan
4553hasta arasndan ocuk kardiyolojisi konsltasyonuistenen 573 hasta
prospektif olarak incelenerek ger-ekletirildi. alma ncesi
hastanemiz etik kurulizni alnd. Hastanemiz blge hastanesi olup
yeni-doan nitesi 3. dzey ve 50 yatak kapasitelidir.
almaya alnan olgularn hastanede yatlarsrasnda gebelik ya, doum
arl, cinsiyeti,doum ekli, postnatal ya, akraba evlilii varl,anne ve
baba ya, varsa antenatal tan ve bulgular,antenatal risk faktrleri
(diyabet, preeklampsi,
erken membran rptr, enfeksiyon, oligohidram-nios,
polihidramnios, uyuturucu madde ve ilakullanm), yat anndaki tans,
fizik muayenebulgular, ekokardiyografi yaplma nedeni,
ekokar-diyografi yaplma srasnda postnatal ya, ekokar-diyografi
sonular kaydedildi.
Ekokardiyografik inclelemeler tm hastalaraayn pediatrik
kardiyoloji uzman tarafndan, So-nosite M-turbo ekokardiyografi
cihaz ile yapld.Her iki atriyum arasnda renkli Doppler ile
restrik-tif olmayan soldan saa ant oluturan defektler se-kundum tip
atriyal septal defekt (ASD) olarak kabuledildi. Restriktif gei
oluturanlar patent foramenovale (PFO) kabul edilerek bu olgular
almayaalnmad. Prematre bebeklerde periferik vasklerdirencin d hz ve
duktusun oksijene verdiiyanta bal olarak ak kalma sresi
uzamaktadr.9
Bu nedenle patent duktus arteriyozusa (PDA) sahiptm prematr
bebekler ile izlemde PDAs kapananmatr bebekler almaya alnmad.
almada, KKHnin skl ve dalm deer-lendirildi. Olgularn antenatal
tan ve bulgular, an-tenatal risk faktrleri (diyabet, preeklemsi,
erkenmembran rptr, enfeksiyon, oligohidromnioz,polihidromnioz,
uyuturucu madde ve ila kulla-nm), ekokardiyografi yaplma nedenleri
ile eldeedilen tanlar arasndaki ilikiye bakld.
statiksel analizler SPSS 21.0 International Bu-siness Machines
Corporations bilgisayar programile yapld. Sonular ortalama-standart
sapma, or-tanca ve yzdelik deerler eklinde belirtildi.
BULGULAR
alma grubuna dahil edilen 573 hastann 314(%54) erkek, 259u (%46)
kz olup196s (%34)matr, 377si (%66) prematr bebek olup; alma-daki
hastalarn ortalama gebelik sresi 33,55,3hafta, doum arlklar
21461075 gr idi. Hastala-rn kardiyoloji konsltasyonlar ortanca
olarakpostnatal 3 gnde (1-130 gn) gerekletirildi.Hasta bebeklerin
ebeveynlerinin 96snda (%17)akraba evlilii mevcuttu. Ortalama anne
ya286,1, baba ya 316,6 ya idi.
almaya alnan 573 hastann130unda(%22,6) KKH tespit edildi. Bu 130
olgunun 57si
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Saadet ELK CENGZ ve ark. YENDOANDA KARDYOLOJ KONSLTASYONU:
KARDYAK ANOMAL SIKLII...
Pediatr Heart J 2015;2(4)184
(%44) erkek, 73 (%56) kz olup; gebelik sresi35,54,7 hafta, doum
arlklar 2445962 gr idi.Postnatal ortanca tan ya ise 3 (1-120 gn)
gnd.Hasta bebeklerin ebeveynlerinin 23nde (%17,6)akraba evlilii
mevcuttu. Ortalama anne ya27,26,5, baba ya 30,66,6 ya idi.
Konjenital kalp hastal tespit edilen 130 has-tann %8,4
siyanotik, %91,6s asiyanotik KKHyesahipti. Asiyanotik KKHler
arasnda en sk ventri-kler septal defekt (VSD) (%42,3) grlrken,
si-yanotik KKHler iinde Fallot tetralojisininin (%3)en sk olduu
tespit edildi (Tablo 1).
almamzda asiyanotik KKHli hastalarn%61i kz iken siyanotik KKHli
hastalarn%27sinin kz olduu grld. Atriyal septal defekt(ASD) (%54
kz), VSD (%66s kz) ve pulmonerstenoz (PS) (%60 kz) kzlarda daha sk
grlr-ken atriyoventrikler septal defektin (AVSD)(%56s erkek)
erkeklerde daha sk olduu tespitedildi. Patent duktus arteriyozus
ise kz ve erkekolgularda eit orandayd.
Konjenital kalp hastalklar iin antenatal riskfaktr saptanan 139
olgunun annelerinin 50sindediyabet (%36), 60nda (%43) preeklampsi
(8 olgudadiyabet ve preeklapmsi birlikte idi), 14nde (%10)
erken membran rptr, 11inde (%7,9) oligohid-ramnios, 2sinde
(%1,5) polihidramnios, 1inde(%0,75) antikonvlsan ila kullanm,
1inde(%0,75) uyuturucu madde kullanm tespit edildi.Risk faktrlerine
gre KKH saptanma oranlar vetipleri Tablo 2de gsterildi.
Konjenital anomaliler nedeniyle sendrom d-nlen ancak kesin tans
konamam, Down sen-dromu gibi kesin tans olan ve orta hat defekti
ileanorektal malformasyonu olan hastalarda KKHninsaptanma oranlar
ve dalm ise Tablo 3te gste-rildi.
ocuk kardiolojisi konsltasyonu istem sebep-leri ve
ekokardiyografide KKH saptanma sklTablo 4te gsterildi. frm (n=73)
nedeniyle eko-kardiyografi (EKO) yaplarak KKH tespit edilen 41(%56)
olgunun 20sinde (%49) VSD, 10unda(%24,5) ASD, 2sinde (%5) aort
koarktasyonu (AK),2sinde (%5) Fallot tetralojisi ve 7sinde (%17)
PStespit edildi. Desaturasyon (n=100) nedeniyle EKOyaplarak KKH
tespit edilen 23 (%23) olgunun8inde siyanotik KKH (bu hastalarn 2si
Fallot tet-ralojisi, 2si byk arter transpozisyonu (BAT), 2sitrunkus
arteriyozus (TA), 1i hipoplastik sol kalpsendromu ve 1i tek
ventrikl idi) tespit edildi.
Olgu says KKH iinde oran (%)Asiyanotik tan
VSD 55 42,3
ASD 46 35,3
PDA 12 9,2
PS 10 7
AVSD 9 6,9
AK 5 3,8
Kesintili arkus aorta 1 0,7
Siyanotik tan
Fallot tetralojisi 4 3
BAT 2 1,5
Pulmoner atrezi 1 0,76
Hipoplastik sol kalp 1 0,76
Trunkus arteriozus 1 0,76
ift kl sa ventrikl 1 0,76
Tek ventrikl 1 0,76
TABLO 1: Asiyanotik ve siyanotik konjenital kalphastalklarnn
dalm.
ASD: Atriyal septal defekt; AK: Aort koarktasyonu; BAT: Byk
arter transpozisyonu;AVSD: Atriyoventrikler septal defekt; PDA:
Patent duktus arteriyozus; PS: Pulmonerstenoz; VSD: Ventrikler
septal defekt.
Risk faktrleri (n) KKH Olgu says % Diyabet (50) Hipertrofik 2
4
kardiyomiyopati
ASD 2 4
VSD 1 2
PS 1 2
Preeklampsi (60) ASD 3 5
VSD 5 8,3
Bikspit aorta 1 1,6
Fallot tetralojisi 3 5
PA 1 1,6
AVSD 1 1,6
Diyabet ve Hipertrofik 1 12,5
preeklampsi (8) kardiyomiyopati
Erken membran rptr (14) AVSD 1 16,6
Oligohidramnios (11) VSD 1 9
ASD 2 18
Antikonvlsan ila (1) VSD 1 100
Uyuturucu (1) Normal 1 0
TABLO 2: Risk gruplarna gre konjenital kalp hastalklar ve
oranlar.
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YENDOANDA KARDYOLOJ KONSLTASYONU: KARDYAK ANOMAL SIKLII...
Saadet ELK CENGZ ve ark.
Dier hastalarn 8inde (%35) ASD, 6snda (%26)VSD, 1inde (%4)
kesintili arkus aorta olduu g-rld. Hidrops fetalis (n=10) nedeniyle
EKO yap-larak KKH tespit edilen olgularn 1inde musklerVSD, 1inde
muskler VSD ve bikspit aorta bir-likte saptand. Fetal EKOsunda
patolojik bulgu ol-mas (n=7) nedeniyle EKO yaplan olgularn
6snda(%85) KKH saptanm olup bu hastalarn 2sindeVSD, 1inde sekundum
ASD, 1inde trunkus arte-riyozus, 1inde kesintili aortik ark, 1inde
intra-kardiyak kitle tespit edildi. Aritmi nedeniyledeerlendirilen
39 olgunun 7sinde supraventrik-
ler taikardi (SVT), 2sinde erken atrial vuru, 1indeerken
ventrikler vuru, 5inde bradikardi ve24nde sinzal taikardi tespit
edilmi olup SVTliolgulardan 1inde (%14) sekundum ASD, bradikar-disi
olan 1 olguda sekundum ASD ve taikardisiolan 1 olguda AVSD
saptand.
TARTIMA
Konjenital kalp hastalklarnn gerek skln sy-leyebilmek iin bu
hastala sahip olan olgularntmne tan konulmas gerekmektedir. Ancak
bubaz nedenlerden dolay mmkn deildir. Bu ne-denlerin banda,
hastalklarn doru tansn koy-mak iin gerekli tbbi donanm ve
deneyimlipediatrik kardiyoloun henz birok lkede yeterlidzeyde
olmamas gelmektedir. Bunlarn dndaFallot tetralojisi ve geni VSD
gibi hemen klinikbulgu veren ve tannan kardiyak patolojiler ya-nnda
minimal PS, kk VSD ve ASD gibi lez-yonlar ou zaman bulgu
vermedikleri gibi rutinfizik muayenede de gzden kaabilirler. Ayrca
do-umdan hemen sonra oluan yenidoan lmleri-nin bir ksmndan da KKH
sorumlu olup buhastalarda tan konmas iin iin yeterli sre
ola-mamaktadr. Tm bu nedenlerden dolay bildirilensklklarn gerek
sklklardan daha az olduu d-nlmektedir.10,11
almamzda ocuk kardiyolojisi konsltas-yonu istenen 573 olguda KKH
skl %22,6 (130olgu) olarak bulundu. Bulut ve ark.nn alma-snda,
takiplerde PDAs kapanan matr yenido-anlar, izole PDA saptanan
prematreler ve izolePFO saptanan hastalar almaya dahil
edilmedi-inde bizim almamzdaki gibi KKH skl 367yenidoanda %28
olarak bildirilmitir.12 Gven veark.nn 201 yenidoan ile yaptklar
almada iseKKH saptanma sklnnn %76 gibi yksek biroranda olduu
grlmtr.13 almalar arasndakioranlarn bu kadar farkl olmasnn sebebi,
yenido-an hekimlerinin semptomlara duyarllnn farklolmas nedeniyle
ocuk kardiyolojisi konsltas-yonu isteme sklndaki farkllk ve almaya
al-nan hastalarn klinik zellikleri (dismorfik hastalar,kromozom
bozuklular olan hastalar, ekstrakardi-yak anomalileri olan hastalar
vb) gibi baka neden-ler ile aklanabilecei dnlmtr.
Tan Ekokardiyografi sonucu/n %Konjenital anomali (n=18) Normal
(n=16) 89
Sekundum ASD (n=1) 5,5
Muskuler VSD (n=1) 5,5
Down sendromu (n=14) Normal (n=4) 29
AVSD (n=8) 57
VSD (n=1) 7
VSD + ASD (n=1) 7
Tracher-Collins sendromu (n=1) Sekundum ASD (n=1) 100
Pierre-Robin sendromu (n=2) Normal (n=2) 0
Konj. kistik adenomatz mal. (n=1) Normal (n=1) 0
Koolen de Vries (n=1) Sekundum ASD (n=1) 100
Bohring-Opitz sendromu (n=1) Sekundum ASD (n=1) 100
Smith-Lemli-Opitiz sendromu (n=1) Sekundum ASD (n=1) 100
Amniyotik band sendromu (n=1) Normal (n=1) 100
Dandy-Walker sendromu (n=2) Normal (n=2) 0
Ambigious genitale (n=1) Normal (n=1) 0
Anorektal malformasyon (n=1) Sekundum ASD (n=1) 100
TABLO 3: Konjenital anomalili ve sendromlu olgulardakonjenital
kalp hastalklarnn dalm
Endikasyon Olgu says KKH skl n/%frm 73 41/56
Saturasyonda dme 100 23/23
Takipne 183 33/18
Dolam bozukluu 55 9/16
Non-immn hidrops fetalis 10 2/20
Konjenital anomali 83 17/20
Maternal diyabet 50 9/18
Fetal EKOda patoloji 7 6/85
Disritmi 39 3/7,6
TABLO 4: Ekokardiyografi endikasyonlar ve konjenital kalp hastal
skl.
Pediatr Heart J 2015;2(4) 185
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Saadet ELK CENGZ ve ark. YENDOANDA KARDYOLOJ KONSLTASYONU:
KARDYAK ANOMAL SIKLII...
Pediatr Heart J 2015;2(4)186
Siyanotik olmayan KKHler iinde en sk VSD(%25-30) grlrken,
siyanotik KKHler iinde ensk BAT (%5-6) grlmektedir.14 Bulut ve
ark.nnyapt almada VSD (%34,3) en sk asiyanotikKKH iken BATn (%6,6)
en sk siyanotik KKH ol-duu grlmtr.12 Ayn almada PDA %14,3,ASD
%10,5, AK %6,6, AVSD %5,7, Fallot tetralo-jisi %4,8, PS %4,8, PA
%3,8, hipoplastik sol kalpsendromu %2,9, tek ventrikl %1,9 sklkta
bil-dirilmitir. Bizim almamzda da asiyanotikKKHler iinde en sk
(%42,3) VSD tespit edilirken,siyanotik KKHler iinde Fallot
tetralojisi %3, BATise %1,5 orannda grlmtr. Konjenital
kalphastalklarnn dalmna bakldnda literatrleuyumlu olarak VSD
(%42,3), ASD (%35,3), PDA(%9,2) ve PS (%7) en sk tespit edilen
KKHler ikenalmamzda siyanotik KKH orannn dier al-malara gre daha dk
olduu grlmtr. Budurumun hastanemizde henz yenidoan kalp cer-rahisi
yaplamamasna bal olduu dnlm-tr.
almamzda cinsiyet dalmna bakldndaKKHli olgularn %56s kz idi.
Siyanotik KKHyesahip olgularn %73 erkek iken asiyanotikKKHye sahip
olgularn ise %61i kzd. Bulut veark.nn yapt almada da benzer oranlar
bildi-rilmi olup KKHli olgularn %54,3 kz olup siya-notik KKHli
olgularn %64nn erkek olduugrlmtr.13 Literatrde de ciddi KKHlerin
er-keklerde daha sk olduu, ASD ve PDAnn kzlardadaha sk grld
bildirilmektedir.9,15,16 Bizim a-lmamzda da ASDli olgularn %54 kz
olupPDAl olgularda cinsiyet fark saptanmamtr.
Konjenital kalp hastalklarnn geliiminde ge-netik ve evresel
faktrlerin neden olduu multi-faktriyel bir kaltm sz konusudur.17
Annedekidiyabetes mellitus, romatoid artrit ve sistemiklupus
eritematozus gibi kollajenozlar, fenilketo-nri, koaglopati ve
sferositozis gibi hematolojikbozukluklar ile annenin hamilelikte
ila (lityum,talidomid, warfarin, alkol, antikonvlsanlar vb) al-mas,
doumsal rubella gibi durumlarn ocuklardakonjenital kalp hastal
grlme skln artrdbilinmektedir.18,19 almamzda diyabetik anne
be-beklerinde hipertrofik kardiyomiyopati (HKMP)grlme skl %6 tespit
edilmitir. Preeklampsili
anne bebeklerinin %35inde KKH saptanmtr.Ayrca saylar az olmasn
ramen, erken memeb-ran rptr ve oligohidramniosa sahip
annelerinbebeklerinde de KKH sklnn artt grlm-tr. Preeklampsinin,
erken membran rptrnnve oligohidramniosun konjenital kalp hastal
ge-liimini ve skln etkileyip etkilemedii konu-sunda literatrde
yeterli alma bulunmamaktadr.Bu konuda yorum yaplabilmesi iin ok
saydahastay ieren vaka-kontrol almalarnn yapl-mas gerektii
dnlmtr.
Birok genetik sendrom ve dier sistem mal-formasyonlar ile KKH
birliktelii bilinmektedir.9
KKHlerin %5-8i de kromozom anomalileri ile be-raber grlmektedir.
rnein Down sendromu(trizomi 21) ile ilikili konjenital kalp
defektleri iyitanmlanm ve bu sendroma sahip ocuklarn yak-lak %40nda
aikar kalp hastal (zellikleAVSD) gsterilmitir. Atriyoventrikler
septal de-fektli ocuklarn da %75inin Down sendromlu ol-duu tespit
edilmitir.16 almamzda genetiksendrom olabilecei dnlen tans
konmamanomalili ve tans konmu sendromik olan 34 ol-gunun %50sinde
KKH saptanmtr. Down sen-dromlu olgularn %57sinde AVSD tespit
edilmitir.Bulgularmzn literatrle uyumlu olduu d-nlmtr.
Konjenital kalp hastal olan yenidoanlarzellikle yaamn ilk
gnlerinde asemptomatikolabileceinden ounlukla kalp damar
sistemidndaki nedenlerle hastaneye yatrldn d-nmekteyiz. Bulut ve
ark.nn yapt almadaKKH tans alan hastalarn %83,9unda, Gven veark.nn
yapt almada KKH tans alan hastala-rn %59unda hastaneye yat
nedeninin kardiyo-vaskler sistem d nedenlerden
kaynaklandbildirilmitir.12,13 Bizim almamzda ise bu orann%96,1
olduu grlmtr. almamzda bu ora-nn yksek olmas hastanemizde henz
yenidoankalp cerrahisinin yaplamamasna balanmatr.
frm varl genellikle KKH ile ilgili olsa dafrm olan bebeklerin
ounda yapsal lezyonyoktur ve KKH grlen bebeklerin hepsinde f-rm
tespit edilmeyebilir.20 frmn saptanmasayrca muayene eden kiinin
yetenek ve deneyi-
-
YENDOANDA KARDYOLOJ KONSLTASYONU: KARDYAK ANOMAL SIKLII...
Saadet ELK CENGZ ve ark.
mine, zamanlama, frekans ve muayenenin hangikoullar altnda
yapldna baldr.20 Bir almada7204 yenidoan bebein 46snda frm
saptana-rak ekokardiyografi uygulanm, bu olgularn13nde kalp normal
olarak deerlendirilmi, 8 be-bekte frm fizyolojik nedenlere balanm
(fiz-yolojik hafif periferik pulmoner stenoz gibi) olup25 bebekte
(%54) frmn kardiyak malformas-yona bal olduu tespit edilmitir. Bu
hastalarnda tm asemptomatik olup 15inde VSD, 3ndeAK, 3nde Fallot
tetralojisi, 2sinde ASD, 1inde PSve 1inde aort kapak stenozu
saptanmtr.21 al-mamzda takipne ve desaturasyondan sonra f-rm 3. en
sk konsltasyon isteme sebebi olupfrm tespit edilen olgularn %56snda
KKH tes-pit edilmitir.
Siyanozun fizik muayenede farkedilir hale gel-mesi iin