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RevBrasAnestesiol.2017;67(1):85---88

REVISTA

BRASILEIRA

DE

ANESTESIOLOGIA

PublicaçãoOficialdaSociedadeBrasileiradeAnestesiologia www.sba.com.br

CLINICAL

INFORMATION

Anesthesia

for

cesarean

section

in

a

patient

with

isolated

unilateral

absence

of

a

pulmonary

artery

Tomonori

Furuya,

Ryoji

Iida

,

Jyumpei

Konishi,

Jitsu

Kato,

Takahiro

Suzuki

NihonUniversitySchoolofMedicine,DepartmentofAnesthesiology,Tokyo,Japan

Received1June2014;accepted3July2014 Availableonline28October2014

KEYWORDS

Anesthesia;

Cesareansection;

Pregnancy;

Unilateralabsenceof

apulmonaryartery

Abstract

Backgroundandobjectives: Congenitalunilateralabsence ofapulmonaryartery(UAPA) isa rareanomaly.Althoughthereareseveralreportsregardingpregnancyinpatientswithunilateral absenceofapulmonaryartery,therearenocasereportsdescribinganesthesiafor Cesarean sectioninapatientwithunilateralabsenceofapulmonaryartery.

Casereport: Wepresentapatientwithunilateralabsenceofapulmonaryarterywho under-wentCesareansectionstwiceattheagesof24and26yearsunderspinalanesthesiaforsurgery andepiduralanalgesiaforpostoperativepainrelief.Bothtimes,spinalanesthesiaand epidu-ral analgesiaenabledsuccessfulanesthesia managementwithoutthedevelopmentofeither pulmonaryhypertensionorrightheartfailure.

Conclusion: Spinalanesthesiacombinedwithepiduralanalgesiaisausefulanestheticmethod foraCesareansectioninpatientswithunilateralabsenceofapulmonaryartery.

©2014SociedadeBrasileiradeAnestesiologia.Publishedby ElsevierEditoraLtda.Thisisan openaccessarticleundertheCCBY-NC-NDlicense( http://creativecommons.org/licenses/by-nc-nd/4.0/).

PALAVRAS-CHAVE

Anestesia; Cesariana; Gravidez;

Ausênciaunilateral

deumaartéria

pulmonar

Anestesiaparacesarianaempacientecomausênciaunilateralisoladadeartéria pulmonar

Resumo

Justificativaeobjetivos: A ausênciacongênitaunilateraldeumaartériapulmonar(ACAP)é umaanomaliarara.EmboraexistamváriosrelatossobrepacientesgrávidascomACAP,nãohá relatosdecasosdescrevendoanestesiaparacesarianaempacientescomACAP.

Relatodecaso: ApresentamosumapacientecomACAPquefoisubmetidaaduascesarianas, nasidadesde24e26anos,sobraquianestesiaparaacirurgiaeanalgesiaepiduralparaador nopós-operatório.Nasduascesarianas,araquianestesia eanalgesiaepiduralpossibilitaram

Correspondingauthor.

E-mail:ryoiida03@gmail.com(R.Iida).

http://dx.doi.org/10.1016/j.bjane.2014.07.002

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86 T.Furuyaetal.

omanejobem-sucedidodaanestesia,semaocorrênciadequalquerhipertensãopulmonarou insuficiênciacardíacadireita.

Conclusão:Raquianestesiacombinadacomanalgesiaepiduraléummétodoanestésicoútilpara cesarianasempacientescomACAP.

©2014SociedadeBrasileiradeAnestesiologia.PublicadoporElsevierEditoraLtda.Este ´eum artigoOpen Accesssobumalicenc¸aCCBY-NC-ND( http://creativecommons.org/licenses/by-nc-nd/4.0/).

Introduction

Congenitalunilateralabsenceofapulmonaryartery(UAPA)

isarareanomalywithanestimatedprevalenceof

approx-imately1 in 200,000young adults.1 Patientswhohave no

cardiacanomaliesotherthanUAPAcanremain asymptom-aticevenintolateadulthood.Themostcommonsymptoms arerecurrentpulmonaryinfections,decreasedexercise tol-erance or mild dyspnea on exertion.2 The symptoms of

isolatedUAPAcanbeprovokedbypredisposingfactors,such as pregnancy3---8 or high altitude.2 Pregnancy is known to

increasecardiacoutput.Furthermore,unilateral lung per-fusionwiththeentirecardiacoutputisariskfactorforthe developmentofpulmonaryarterialhypertension.The prog-nosisofisolatedUAPAdependsonthepresenceorabsence ofpulmonaryarterialhypertension.2Althoughthereare

sev-eralreportsregardingpregnancyin patientswithUAPA,3---9

therearenocasereportsdescribinganesthesiafora Cesar-eansectioninapatientwithUAPA.Thus,thebestapproach toanesthesiaforCesareansectioninthesepatientsremains unclear. We present a patient who underwent Cesarean sectionsunder spinal anesthesia for surgery and epidural analgesiaforpostoperativepainrelieftwiceattheagesof 24and26years.

Case

report

Firstcesareansection

A 24-year-old woman, pregnant patient, with UAPA was admittedtoourhospitalat 35weeks’gestationfor deliv-eryandperinatalcare.ShewasdiagnosedwithUAPAatthe ageof15yearswhensheincurred right-sidedpneumonia. AchestX-rayrevealedtheabsenceoftherightpulmonary arterytrunk.Computedtomography revealedthe absence oftherightpulmonaryarteryandthepresenceofthree col-lateralvesselsfromtheascending aortatotheright lung. An echocardiogram did not image any additional cardiac anomalies. Right cardiac catheterization revealed normal pulmonary arterialpressure (PAP). At thetime of diagno-sis,shewasasymptomaticforUAPAandsurgicalcorrection wasnotindicated. She had nopast medicalhistory other thanUAPAandtheright-sidedpneumonia.

On admission, the patient wasasymptomatic. She had nopregnancy-relatedcomplications.Herheightwas154cm andherweightwas63kg.AchestX-rayshowedtheabsence of the right pulmonary artery trunk, mediastinal shift to the right and an expanded left pulmonary artery trunk

Figure1 Chestroentgenographyshowingabsenceoftheright pulmonary artery trunk,decreased pulmonary vasculaturein therightlung,shiftofthemediastinalstructurestotheright andanexpandedleftpulmonaryarterytrunk.

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Anesthesiaforapatientwithunilateralabsenceofapulmonaryartery 87

via afacemask, an epiduralcatheterwasinserted at the Th12/L1 interspace. Next, spinal anesthesiawas adminis-tered at the L3/L4 interspace with 10mg of hyperbaric bupivacainehydrochloridehydrate.Fiveminutesafterthe injection, SAPwas 108/52mmHg, PAP was 6/2mmHg, HR was82beats/minandSpO2was99%.Fifteenminutesafter theinjection,thesensoryblocklevelwasbetweenTh1and S5. Atthis time,300mLof crystalloidfluidand 400mLof colloidal fluid had been administered intravenously. SAP, PAP andSpO2 did not change significantly. No cardiovas-cular agents were administered. Intraoperatively aswell, SAP,PAPandSpO2didnotchangesignificantly.Atdelivery, the neonate hadApgar scores of 8 and9 at 1 and 5min, respectively.Duringthedelivery,thePAPincreasedslightly from 10/7mmHg to 15/6mmHg while the SAP increased slightlyfrom100/50mmHgto108/65mmHg.Afterthe pla-centawasdelivered,10unitsofoxytocinwereadministered intravenously,which is routinepracticeatour institution. This administrationcaused a slight decrease in SAP from 108/65mmHgto100/55mmHgandnochangesinPAP. Mida-zolam wasadministered at a dose of 2mg asa sedative, whichresultedinnochangesinSAPorPAP.The administra-tionofasedativewasbasedonpatientdemand.Totalblood lossexcludingdilutionwithamnioticfluidwasapproximately 180mL.TotalfluidsadministeredunderPAPmonitoringand centralvenous pressure(CVP) monitoringincluded 400mL ofcrystalloidfluidand900mLofcolloidalfluid.Thepatient was pain-free throughout the operation. No cardiovascu-laragents wereadministered.The surgerywascompleted uneventfully.Postoperativepainwasadequatelycontrolled by epidural analgesia with 0.2% ropivacaine at a rate of 5mL/h, without supplementary analgesics. The high-est pulmonary systolicpressure reached was 20mmHg on thesecond postoperativeday. Postoperatively,theclinical coursesofthepatientandherinfantwereuneventful.

Secondcesareansection

Thepatientachievedasecondpregnancyatageof26years and was admitted toour hospital at 36 weeks’ gestation with nopregnancy-related complications. Her height was 154cmandherweightwas61kg. HerPAP waswithin nor-mallimits.ShewasscheduledtoundergoCesareansection at 37 weeks’ gestation under the same anesthetic plan as that of her first Cesarean section. On arrival to the OR, SAP was 115/76mmHg, PAP was 8/4mmHg, HR was 90beats/min and SpO2 was 97%. After application of all monitors asbefore and administration of 5L/min of oxy-gen viaafacemask, an epiduralcatheterwasinsertedat theTh12/L1interspace.Next,spinalanesthesiawas admin-istered at the L3/L4 interspace with 9mg of hyperbaric bupivacainehydrochloridehydrate.Fiveminutesafterthe injection,SAPwas115/52mmHg,PAPwas10/5mmHg,HR was92beats/minandSpO2was100%.Fifteenminutesafter theinjection,thesensoryblocklevelwasbetweenTh2and S5. Atthis time,300mLof crystalloidfluidand 300mLof colloidalfluidwereadministeredintravenously.SAP,PAPand SpO2didnotchangesignificantly.Therefore,no cardiovas-cular agents were administered. At delivery, the neonate had Apgar scores of 8and 9 at 1and 5min, respectively. During the delivery, the patient’s PAP increased slightly

from 11/4mmHg to 15/7mmHg. After the placenta was delivered,intravenous oxytocinat a dose of 10 units and intravenousmidazolamatadoseof2mgdidnotcauseany significantchangesinSAPorPAP.Totalbloodlossexcluding dilutionwithamnioticfluidwas190mL.Totalfluids admin-isteredunderPAPmonitoringandCVPmonitoringincluded 800mLofcrystalloidfluidand500mLofcolloidalfluid.No cardiovascularagents wereadministered.The patientwas pain-freethroughout the operation,which wasperformed uneventfully.Postoperativepainwasadequatelycontrolled withepiduralanalgesiawith0.2%ropivacaineat arateof 5mL/h,withoutsupplementaryanalgesics.Thehighest pul-monarysystolicpressurereachedwas22mmHg2hafterthe conclusionofsurgery.Thepostoperativeclinicalcoursesof thepatientandherinfantwereuneventful.

Discussion

Therehavebeennopreviouscasereportsdescribing anes-thesia for Cesarean section in patients with UAPA. We administered spinal anesthesia for surgery and epidural analgesiaforpostoperativepainrelieftoourpatientwith UAPA.Theadvantageofthisprotocolisthatitblocks intra-operativeandpostoperativenociceptivestimulicausedby surgery,thuspreventingthecardiovascularchangesinduced by these nociceptive stimuli. The disadvantage is that patients might need vasopressordrug administration or a significantlylargefluidinfusiontomaintainarterialpressure ifthelevelofspinalblockrises.Duringbothsurgeriesinour patient,spinalanesthesiacaused nosignificant cardiovas-cularorrespiratorychanges.Moreover,thepatientdidnot developanyseriouscomplicationsduringtheperioperative period.

SpinalanesthesiacausedsensoryblockadeuptoTh1for thefirstsurgeryandTh2forthesecondsurgery.The possi-blereasonswhythepatientdidnotrequireanyvasopressors includedproperfluidadministrationandthepatient’srobust circulation. A slow incremental epidural anesthesia com-bined with the lower-dose spinal anesthesiawas another anestheticoptionforthiscase.Thisoptioncouldhave reg-ulatedtheregionofsensoryblockade.

The PAP increased slightly on the second postopera-tive day for the first surgery and 2h postoperatively for the second surgery. It was probably due toongoing uter-inecontractionsafterdelivery.Uterinecontractionsinduce hypervolemia especially during the first few postpartum days.CloseobservationofthePAPisimportantduringthis period.Therefore,postoperativeanalgesiaisalsoimportant becausenociceptive sympathetic stimulation might cause rightheartoverload.

Administration of 10 units of oxytocin did not cause anysignificant cardiovascular changesduring both surger-ies; however, this dose is large for an elective Cesarean section.10 Adequate uterine contractions can usually be

achievedwithoxytocin at adose of <5units; this dosage can also minimize cardiovascular side effects.10---12

Oxy-tocinisproblematicparticularlyinpatientswithpulmonary hypertension becauseit raises the PAP.13 In addition, the

dropinsystemicvascularresistanceduetooxytocin.11 can

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88 T.Furuyaetal.

obstetriciansandusedtheminimumdosenecessaryfor ade-quateuterinecontractionswhileconsideringtheamountof bloodloss.

AlthoughUAPAcanremainasymptomaticuntillate adult-hood,itcanbeunmaskedbypregnancy,3---8probablydueto

increasingcardiacpreloadduringpregnancy. Symptomsof UAPAcanalsobetriggeredbyspinalanesthesiaorCesarean section,becausetheseproceduresmightcauserightheart failureorpulmonaryhypertension.Rightheartfailureor pul-monaryhypertensionresultsfromexcessivecardiacpreload orafterloadduetoincreasedvenousreturnafterdelivery, excessivetransfusionorexcessivesympatheticstimulation duetonociceptivestimuli.Koga etal.reportedacase of awomaninwhomPAProseafterCesareansectionbecause ofa sudden increase in venous returnafter labor.3 Inour

patient, close cardiovascular and respiratory monitoring indicatedthatthesefactorswerenotsignificantlyalteredby eitherthespinalanesthesiaortheCesareansection.Spinal anesthesiaforsurgeryandpostoperativeepiduralanalgesia resultedincompletepainrelief.Thus,thisprotocolisa use-fulanestheticoptionforCesareansectioninpatientswith UAPA.

AreviewbyHarkeletal.,2reportedamortalityrateof

isolatedUAPA to be 7 (6.5%) of 108. The cause of death included pulmonary hemorrhage, right heart failure, pul-monaryhypertension,respiratoryfailureandhigh altitude pulmonary edema.2 Pregnancy is known to increase

car-diacoutput,whichcouldinducepulmonaryhypertensionin pregnantpatientswithUAPA,leadingtorightheartfailure orruptureofaffectedpulmonarycapillaries.Theliterature contains only seven previous reports addressing the clini-calcourseofpregnancyinpatientswithUAPA.3---9According

tothesestudies, UAPA wasunmasked by pregnancyin 10 patientswhopresentedwithsymptomssuchasdyspneaand chestpain.3---8Onepatientdevelopedadultrespiratory

dis-tresssyndromeonthesecondpostpartumdayanddied15 daysafterdelivery.6Therefore,patientswithUAPA

under-goingCesareansectionshouldbecloselyand continuously followed throughout the perioperative period, especially whenuterinecontractionsarepresent.

Conclusions

We administered spinal anesthesia for the Cesarean sec-tionandepiduralanalgesiaforpostoperativepainreliefin

a patient withUAPA in two instances. In both instances, neither pulmonary hypertension nor right heart failure developed. Thus, this combination is a useful anesthetic methodforCesareansectioninpatientswithUAPA.

Conflicts

of

interest

Theauthorsdeclarenoconflictsofinterest.

References

1.Bouros D, Pare P, Panagou P, et al. The varied manifes-tation of pulmonary artery agenesis in adulthood. Chest. 1995;108:670---6.

2.HarkelAT,BlomNA,OttenkampJ.Isolatedunilateralabsence ofapulmonaryartery.Chest.2002;122:1471---7.

3.KogaH,HidakaT,MiyakoK,etal.Age-relatedclinical character-isticsofisolatedcongenitalunilateralabsenceofapulmonary artery.PediatrCardiol.2010;31:1186---90.

4.KoT, GatzMG,ReiszGR. Congenitalunilateral absenceof a pulmonaryartery:areportoftwoadultcases.AmRevRespir Dis.1990;141:795---8.

5.StillerRJ,SobermanS,TuretskyA,etal.Agenesisofthe pul-monaryartery:anunusualcauseofdyspneainpregnancy.AmJ ObstetGynecol.1988;158:172---3.

6.FerrariM,KarraziR,LamprontiG,etal.Effectofchanging posi-tiononarterialoxygenationinapatientwithagenesiaofthe leftpulmonaryartery.Respiration.1997;64:371---4.

7.LalC,BarkerJ,StrangeC.Unilateralpulmonaryarteryaplasia inapregnantpatient.CaseRepMed.2011;2011:806723.

8.VohraN,AlvarezM,AbramsonAF,etal.Hypoplasticpulmonary artery:anunusualentitymimickingpulmonaryembolismduring pregnancy.ObstetGynecol.1992;80:483---5.

9.YoshiharaK,KurabayashiT,TsuchiyaM,etal.Pregnancywith congenital unilateral absence of a pulmonary artery. Acta ObstetGynecolScand.2006;85:755---7.

10.ButwickAJ, Coleman L, CohenSE,et al. Minimumeffective bolusdoseofoxytocinduringelectiveCaesareandelivery.BrJ Anaesth.2010;104:338---43.

11.SartainJB, Barry JJ,Howat PW, et al. Intravenousoxytocin bolusof2unitsissuperiorto5unitsduringelectiveCaesarean section.BrJAnaesth.2008;101:822---6.

12.CarvalhoJC,BalkiM,KingdomJ,etal.Oxytocinrequirements at elective Cesarean delivery: a dose-finding study. Obstet Gynecol.2004;104:1005---10.

Imagem

Figure 1 Chest roentgenography showing absence of the right pulmonary artery trunk, decreased pulmonary vasculature in the right lung, shift of the mediastinal structures to the right and an expanded left pulmonary artery trunk.

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