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between 2015. 2001 and 20 and Research Center, Iran University of University Iran Center, Research and operable operable due to structural problems or high hypoplastic hypoplastic left them. Inadequate oxygenation in results cya of organs is due to an obstruction or the of circulation deviation the blood flow in the right circulation. pulmonary the from improvement in , 20 , 25 The goal of palliative surgery is to increase

who under hemoglobin levels, pulmonary arterial shunt pulmonary arterial , - April these

to Rafie Khorgami alliative surgery,alliative male. male. Nearly half of the study (23 population patients)

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ABSTRACT we evaluated patients older than 15 years old with , all the patients showed dramatic improvement

functional functional class, , Accepted:

(Iranian Heart Journal 2020; 21(4):

and Glenn shunting was 12.5% and 8.69%, respectively. 8.69%, and 12.5% was shunting Glenn and

study systemic and and palliative.

Rajaie Cardiovascular Medical Medical Cardiovascular Rajaie

via

c c heart diseases ic heart disease, MD; MD;

series , - isease icant drop in the hemoglobin concentration, and a significant increase in increase and a significant concentration, in drop icant the hemoglobin good by comparison by with the good 2 types 2 of types surgical operations series series study manifests early with with early manifests

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yanot

C year; year; however, these improvement congenital cyanotic heart disease

cyanoti , MD; Mohammad

Heart D Heart ar Medical and Research Center Center Medical ar Research and 1 ventricle ventricle anomalies (eg, case

- Taussig shunting Taussig

Zahra Khajali Zahra

– , 2019 ,

eases: eases: corrective two (46.8%) patients two were patients (46.8%) seven seven patients at a mean age of 24.74 ± 5.67 years were enrolled in this study. yanotic yanotic 29 - In In this case

- Generally, Generally,

: latelet count. Five (10.6%) patients expired during the study period. The mortality rate rate mortality The period. study the during expired patients (10.6%) Five count. latelet Patients With Cyanotic Congenital Heart D Heart Congenital Cyanotic With Patients partial partial pressure of carbon dioxide In In this

adults adults with Blalock

Forty KEYWORDS:

yanotic yanotic congenital heart diseases are defined as any malformation of the that birth at heart September risk of anesthesia or surgery. anesthesia risk of postoperative postoperative following 10 years saw a decline in were lab data and In should be considered in patients who are the p the of New York Heart Association the Twenty had central 23 shunting, Glenn patients shunting, and 1 patient Blalock Following surgery, there was a significant rise in the mean partial pressure of O a signif saturation, heart heart dis blood flow pulmonary documented Cardiovascul

usions

[email protected]

Zahra HosseiniZahra Comparisons of Arterial and Venous Palliative and Venous Arterial of Comparisons Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, IR Iran. IR Tehran, Sciences, Medical of University Iran Center, Research and Medical Cardiovascular Rajaie

. cyanosis. The , tricuspid the atresia, of transposition the great vessels, and various single Email: Email: Received: C 1 Author: *Corresponding IR Iran. Concl Results: Methods: Background: Original Article Complications Diabetes 2 andType Irisin Palliative Shunts in C in Shunts Palliative

 Iranian Heart Journal; 2020; 21 (4) 60  Iranian Heart Journal; 2020; 21 (4) 61

3 1 to

the the

For ing, ing, half was 5.67 5.67 tests

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SPSS ) ) had

±

ing. ing. In

directly directly

test nt Hosseini et al 2 χ evaluated evaluated Almost 22 22 (46.8%) was the the hospital.

patients the d d more than 2 Glenn Glenn shunt F F > 55%), 26 was

an (23 (23 creation surgery, surgery, creation less than 0.05

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test, . - of t this study. this their first shu ing

and correlation

, , including

0. The survival analysis , mean mean age of 24.74 , the previous shunt was

had Meier tests were used 23 23 patients value RESULTS –

- hospital hospital mortality at at a P operation -

were were analyzed using the nalysis ing, n ’ ’ cardiac status A s the the operation, version 2 data data 1 month after shunt after month 1 seven seven patients atient patient BT shunt BT patient were enrolled in enrolled were

- way way ANOVA the 1 -

formed formed consent form on the understanding normal normal LV dysfunction function systolic mild had patients (LVE (55.3%) (45% < LVEF < 55%), 13 (27.7%) patients had moderate systolic dysfunction (30% < LVEF < 30%). < 45%), (LVEF and dysfunction systolic severe 1 (2.1%) patient had The The explained to comprehensively the patients or purpose their legal of custodians, who signed a written in the highly treated be would information their that study confidential. was of Rajaie Committee by Ethics the confirmed The study Center. Research and Medical Cardiovascular protocol was Forty male patients, years of the study population central shunt and Thirteen patients the other patients and in 30 cases in 4 cases. patent occluded based 4 groups into divided were patients The on their left preoperative ejection ventricular fraction (LVEF): 7 (14.9%) patients had a The The rate of i extracted from medical files in The p times: year after the after years A Statistical All software, and the Kaplan compare survival between the patients. further analyses, the one were used. significant. as defined Ethical Approval

, - s e

in 6 to the the are list

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right right adult shunt -

, and direct

( for 12 to

- s who had 11,

underwent

. unts, central central unts,

abolished to record th enrolled

between including including age,

who , inadequate inadequate

isease side connection pulmonary pulmonary artery) s

-

D s or patients an an

to -

the eart eart ment of cyanotic heart

and and palliative retrospectively retrospectively extracted

between 2001 and 2015.

series study treat -

r ascending ascending aorta and the METHODS

the ’ information by the researcher case s

2 types of surgical operations

the

corrective corrective data, data, was

esign Taussig (BT) (arterial) sh (arterial) (BT) Taussig Rajaie Rajaie Cardiovascular Medical and

up

side connection – - - atient es: their medical documents. A check

to ending ending aorta and ative techniques serve to “buy time” until until time” “buy to serve techniques ative - occluded occluded shunt or ollow information for each patient. each for information F Data Gathering Data The p sex, lab data, echocardiographic data surgical from was devised an an flow in Research Cente These patients were not ideal cases procedure. Fontan The The present patients with cyanotic heart documented palliative disease surgery at a minimum age congenital of years 18 for the first time first first introduced in 1946 complications. to serious 1967 due and Study D Other Other famous shunts shunts include (direct Waterston between side pulmonary artery) and Potts shunt side desc body size for subsequent definitive repairs. repairs. definitive subsequent for size body of the palliation for shunts common most The patients with cyanotic Blalock heart diseases shunts. venous) (central/ are Glenn and shunts, the the pulmonary blood flow via the promote to or shunting systemic arterial pulmonary mixing of the For shunting. some blood with patients other cases, through left palli the patient attains a more desirable age and Generally, available for diseas The goal of palliative surgery is to augment Palliative Shunts in Cyanotic H Cyanotic in Shunts Palliative

) 2 . ± P 2

the up. in PaO - before (

2 Hosseini et al and and

2 compares significantly follow PaO

partial partial pressure of

xygen saturation xygen the the ups between the o -

erative operation . Figure 2 shunt type 2groups shunt type surgery, surgery, and 18.60

0.01)

postop 1 compares hanges hanges in .

hanges in hanges =

also also increased P before before the (

year year after gnificantly gnificantly after the operation (

longer groups partial pressure of oxygen

in terms of c at at 1 the ups between the ups between

in terms of c in of terms

ean

- saturation saturation saturation

1.42 ter surgery 2 2

groups groups

± 1.38 in The m increased si = 0.01). Figure surgery and in the follow shunt type O af O follow type type type

shunt , , shunt

2 2 15

of the in .3%), .3%), 4

patients in 2. , and IV,

17

between between the level between the between

before before surgery, I, I, II, III

isease nd severe nd severe es , moderate

3.05 3 1, a

and 3 (6.4%) patients , systemic ventricular

±

Heart D Heart class ,

in s are illustrated sillustrated are month month after surgery, 18.58 al

. yanotic yanotic creation creation surgery, 2 (4 at at 1 - severe in

operation (80.9%) - ) after surgery. ) after

2 to

omparison omparison function surgery the New York Heart Association -

C 1.43 C moderate moderate )

- ean ean hemoglobin (Hb) PaO ( ± 1: 2:

to

- ) after 2 ygen NYHA SO Figure ( Figure ox patients patients was 18.55 18.08 respectively. Before the regurgitation (SVR) was mild in mild moderate The m Before Before shunt (8.5%), 38 were in ( Palliative Shunts in C in Shunts Palliative

 Iranian Heart Journal; 2020; 21 (4) 62  Iranian Heart Journal; 2020; 21 (4) 63

. s

on ing

total total 13

ted ted a

the nd 75%

Hosseini et al a et et al

on on surgical exhibi

, and diac diac death,

, mortality mortality rate

creati deaths deaths occurred 14

- period period since the

Further, Further, the mean ing. ing. According to Petrucci

the

.

s the ferritin level after surgery. after level ferritin the report on patients with

year - 2009, the decades. decades. after after surgery

them, 3 them, shunt had central

62

6 undergoing BT shunt tetralogy tetralogy of Fallot was the and

DISCUSSION 33% 33% of s the the hours and and annual mortality

Among

center center study by 2002 over the the over over over a

at at home (sudden car 3% 3% and the morbidity rate was 13.1% had Glenn shunt Glenn had

2 most common indication (72%) indication common most mortality rate was 14%. annual number of BT shunt operations decrease probably). probably). and 12.5% was shunts central of rate mortality The of rate mortality the while of 24 out (3 cases), cases). 23 of out (2 8.69% was shunts Glenn Our study is the first cyanotic heart diseases who for the first time underwent palliative shunting in adulthood. In a multi 1273 neonates with heart cyanotic disease congenital between was 7. Additionally, within 24 within 30 days. occurred Another study evaluated all 2016 BT shunts created introduction of BT shunt the results, while

1 . for for

due due up.

- and and 1 month month central central

2 2 years after after 1 1 stay stay due

compares at at after after the ) s

the the

and 95% and significantly significantly , , 1 after after in in and and 86% for

ICU

(

s

term follow term , the patency rate patency the , after surgery after - rate rose

isease shunts shunts up Figure Figure 3 ient ignificantly ignificantly after - D

expired expired during the . 2 shunt type 2groups shunt type shunts

shunts shunts as opposed to pat years years

increased increased significantly

eart eart e long e s s rate of shunt patency

. = 0.01), but this decline decline this but 0.01), =

s immediately immediately 3 atency .

P due due to heart failure,

before before surgery and in the .

= 0.02) 1 level 1 at

patients The p The

P long follow long

up tests some months after the (

. 0.05) - = 0.01)=

s. s. (Three

ron levels decreased decreased s = partial pressure of carbon dioxide

P

100% for Glenn Glenn for 100% (

low P failure, failure,

In the In ( Comparisons are illustrated between the 2 shunt type groups in terms of changes in changes of terms in groups type shunt 2 the between illustrated are Comparisons

) period:

ups between the ups between shunts the intensive the unit intensive care 2 ) ean ean -

. erum erum ferritin level ery ery early succes

the the operation ation ation due to refractory heart failure, ferritin CO the fol shunt a

P to to valve and endocarditis sepsis after 75% for central shunt central for 75% Five (10.6%) study oper during central was 100% for Glenn central shunt shunt group had occluded month shunt was 100% for Glenn follow The serum i in operation The v 97%: was after the operation ( operation the after th in observed not was The s after surgery the The m ( surgery significantly decreased The Hb concentration Figure 3: Figure Palliative Shunts in Cyanotic H Cyanotic in Shunts Palliative

- 2 2 d 1

the ince ince CO after 14%

a S

in in the due todue t t after and and 1 a

third of 2 years in and and O - ptable in ptable

mortality Hosseini et al making making a

BT BT shunts

2

hours s es improved improved es use use of iron

the

on adults on with adults , 1 at .

13 PaO increase increase s

2000 , 16 the

decreased decreased P due due to refractory

indicat patients, patients,

a et al

to to the tissues an in in decreased need for them, 3 had central ion, ion, Hb, and NYHA ferritin ferritin and serum iron about was 7.3%. One our our

2

after the operation

Still, Still, the s end organs. end diseases diseases who underwent O d d flow and adequate

during ICU stay due to shunt Petrucci the the saturat

5 (10.6%) patients expired: 14

years years in our study our in its introduction reported

1 after surgery certain

had Glenn shunt Glenn had

up. up. All the Glenn shunts, and 2

Among - 3

2 O nd 75% occurred within 30 days. a

since at at home (sudden cardiac death, , and and is is difficult.

s to to supply s , , indicates improvement ignificant ignificant increase in deaths deaths occurred within 24

the study by e created created rate. mortality In the study by et Berdjis al congenital heart or corrective 3 surgeries, palliative out of 15 oxygenation, oxygenation, resulting Hb consequently decreased need for iron. we are not supplements by all judgment as regards level count platelet of oxygenation In this study, immediately heart failure, 1 at failure, valve endocarditis and sepsis after surgery due to while heart failure, probably). shunt In rate from BT shunt th surgery Another study evaluating all clinical status and lab acce data were status clinical surgery). (before baseline the with comparison Our the study underscored of results benefits both central and Glenn shunts immediately after surgery, but of better preservation these improvements in the central shunt group in terms of functional in improvements class. nonsignificant the systemic ventricular Our EF in long aggregated which year, postoperative patients and SVR term had follow in the 75% of the central shunts first were paten years. 10 almost A s saturation, together with level pulmonary bloo

, , ; - e

2

.

an an 17 at at a who who ce. ing thos

. Fifteen , , PaCO s 2 and long

-

technique technique and heart heart diseases

of adult patients isease

the o evaluate adult the short t , we conducted the

success success rate appears

t palliative t surgery in palliative .

lso lso benefit from these

decreased hand for hand decreased a

Since no study has

Heart D Heart saturation, PaO saturation, in

2 local the surgeon; thus, cyanotic evaluated evaluated 200 adults diagnosed diagnosed with congenital failure evaluated 80 adult patients at at patients adult 80 evaluated O

incidentally, incidentally, or those

prognosis prognosis compare yanotic yanotic the success rate in every type 16 15

adulthood the ysrhythmias ysrhythmias (20%). Clinical palliative palliative surgeries for the first s

to depends on edly improved survivors. edly the in the shunt

with

l l class, and their et et al

s

who who are show show their first symptoms in adulthood

These improvements were more remarkable in in remarkable more were improvements These arterial shunts. In the following years, these improvements declined; nevertheless, the shunts shunts or had their first shunt due to the late the patients All of symptoms. commencement NYHA in the improvements dramatic showed functiona in Hb, the and ferritin surgery. first after year term results of arterial and shunt of arterial venous results term In this case series, we evaluated adolescents and adults with congenital diseases who underwen cyanotic heart their adulthood. Except for palliative 4 occluded had previously cases other patients, the who undergo time in current investigation patients with disease congenital cyanotic heart It It is clear that of surgery experience of evaluation of to be examined necessary. the the Fontan operation include adults Such operations. surgical who those heart disease experience procedures. procedures. Three died due to low output cardiac or status mark d in Most of are shunts infancy these created for cases eligible not are who adults however, extremity and result result and extremity Berdjis mean age of surgery for congenital heart disease 38 years patients who underwent underwent 11 corrective and 4 shunt Le Logan et al al et Logan Le BT a age of who mean had previous 36 years shunts and showed that BT shunting could decrease the blood flow to the right upper Palliative Shunts in C in Shunts Palliative

 Iranian Heart Journal; 2020; 21 (4) 64  Iranian Heart Journal; 2020; 21 (4) 65

9. -

8. or their - 128:189

were were in

Hosseini et al ments or y, y, which procedures shunts shunts are

,

stud up and physical high high risk of - authors authors declare

96. ese the -

163(5):492

the NW. Congenital heart

participants Follow . Th All 7.

- All Informed Informed consent was lformations of the heart in

27, vii.

- 68(3):477 the

acceptable acceptable quality of life

(1):83

the present an 8

Emerg Med Clin North Am. 2011 REFERENCES

29(4):811 pproval:

human participants

This This study was funded by Rajaie A

ee ee and with the 1964 Helsinki Am. 1988Am. Jun; Blalock A, treatment Taussig of ma HB. which The there surgical pulmonary atresia. is JAMA 1945; pulmonary 202. stenosis or Trojnarska O. Adolescents with congenital heart diseases. Cardiol J. 2010;17(1):11 Dolbec K, Mick disease. Nov; Colonna P, Manfrin M, Cecconi M, GP, Perna Picchio FM. activity in postoperative disease. congenital J heart Cardiovasc Med 2007Jan; (Hagerstown). Foster E. Congenital heart disease in adults. West J Med.1995 Nov; Cooley DA. Palliative surgery for cyanotic congenital heart disease. Surg Clin North

...... 6 1 2 3 4 5 claration claration and its later amend comparable ethical standards. ethical comparable Informed Consent: obtained from all custodians. legal to to structural problems anesthesia or or surgery lifesaving with adulthood. in even Funding: Center. Research and Medical Cardiovascular Conflict of Interest: interests. financial no competing Ethical performed in involved accordance with the ethical standards of the institutional and/or committ national De research

2 2 it it in in

, the the the the

were more more temic temic

volume volume

powerful powerful

s, s, palliative there there are operable due due operable

the change the in change surgery in surgery

atrioventricular , nd nd after shunt in in terms of e e of the , which , was which

isease after s involving involving systemic D the the present study that that in adults with eart eart

concerning . d the the systemic ventricle and theory theory ing

good good option and should be

roup. roup. There was, no however, of significantly in the survivors. the in significantly

in in consequenc improvement CONCLUSIONS

function function EF

conclude As As a . . Our results showed an increase

grading grading of regurgitation sidered in patients who are in are who patients in sidered

rstly, rstly, systemic shunting caused volume congenital congenital cyanotic heart disease shunting is a con saturation and patients’ symptoms. patients’ and saturation In light of the results of can be study study is the size branches. of In the our pulmonary opinion, artery it marker is in a the patency and shunt. efficacy of Future the pulmonary the of studies size the between should relationship evaluate the artery branches and improvements in O arterial shunt arterial g significant change in regurgitation shunt severity Glenn after One of the unfortunately we most failed significant to factors consider in that our exacerbation exacerbation overload of the ventricle. The regurgitation changes grading in in negative this study of the regurgitation severity year in first the significant valve valve regurgitation before a surgery. ventricular the possibilities: increased ventricular function. In our study, we found an an found we study, our In function. ventricular especially surgery, in after the LVEF increase after a the few during first Nonetheless, year. decreased. improvement this years, We also evaluated systemic Fi overload as patent ductus effect arteriosus negative to a on leading ventricle, the systemic on EF. the on Secondly, ventricular systemic the other hand, reducing cyanosis via shunt surgery had positive effects on sys (20%) patients died. Clinical status, however, however, status, Clinical died. patients (20%) improved was after function ventricular systemic of Apropos shunt insertion, there are 2 probabilities. Palliative Shunts in Cyanotic H Cyanotic in Shunts Palliative

- - - 51;

- EA.

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bs ML, Jacobs

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5; discussion 2070 - 2. Hay AA, Shinebourne - - 21(5):330

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. . . . . 17 13 14 15 16

- 8. 8. 21. – –

(1973). 3.

– our E, et al. of of tetralogy

Taussig shunt?

-

5. – isease

Heart D Heart

3. 3. – , Budts W, Daenen W, Gewillig 57. yanotic yanotic – time observations on the Blalock -

. Transcatheter closure of a Potts' shunt Daehnert I, Wiener M, Kostelka M. Covered stent treatment of right stenosis and Waterston pulmonary shunt. Ann. arteryThorac. 2005Surg. 79 1754(5): Boshoff D M with subsequent surgical repair of fallot". Catheter Cardiovasc Interv 2005, 64 (1): 121 Kreutzer G, Galindez H, Bono "An operation H, for the correction of ". cardiovascular Journal Surgery PMID 4518787. 66 of (3): 613 Thoracic and Long Taussig operation. operation. I. Johns Hopkins Results 129:243 Med of J first 1971; Fontan F, Baudet tricuspid atresia. Thorax 197126 (3): 240 E. Surgical repair of Brogan TV, Alfieris GM. Has the time come to rename Pediatr CareCrit Med 4:4502003; the Blalock Taussig HB, Crocetti EshaghpA,

. . .

. . . 12 11 10 9 7 8

Palliative Shunts in C in Shunts Palliative

 Iranian Heart Journal; 2020; 21 (4) 66