LifeLife withwith aa SingleSingle VentricleVentricle

StephenStephen Crumb,Crumb, APNAPN CoordinatorCoordinator CColumbusolumbus OOhiohio AAdultdult CCongenitalongenital HHearteart DiseaseDisease ProgramProgram Biology 101

Q. Your heart has ______chambers?

A. ___3____

Wrong! You need to study!! But you asked how many chambers MY heart has!

My heart has only 3 chambers because I have a single ventricle !! ObjectivesObjectives

„ DescribeDescribe thethe congenitalcongenital heartheart defectsdefects thatthat leadlead toto singlesingle ventricleventricle „ DiscussDiscuss longlong--termterm issuesissues thatthat maymay arisearise inin peoplepeople withwith singlesingle ventricleventricle andand howhow toto treattreat themthem „ DiscussDiscuss waysways toto staystay healthyhealthy „ LearnLearn fromfrom eacheach otherother TheThe NormalNormal HeartHeart WhatWhat isis SingleSingle Ventricle?Ventricle?

„ AbnormalAbnormal heartheart structurestructure presentpresent atat birthbirth

„ OnlyOnly oneone functionalfunctional pumpingpumping chamberchamber (ventricle)(ventricle)

„ SeveralSeveral congenitalcongenital heartheart defectsdefects leadlead toto singlesingle ventricle,ventricle, aa featurefeature ofof allall isis cyanosiscyanosis CyanosisCyanosis

„ BlueBlue colorcolor duedue toto lowlow bloodblood oxygenoxygen „ CausedCaused byby mixingmixing ofof oxygenoxygen--poorpoor (blue)(blue) bloodblood andand oxygenoxygen--richrich (red)(red) bloodblood „ MixingMixing isis necessarynecessary forfor initialinitial survivalsurvival HypoplasticHypoplastic LeftLeft HeartHeart SyndromeSyndrome DoubleDouble InletInlet LeftLeft VentricleVentricle DoubleDouble OutletOutlet RightRight VentricleVentricle TricuspidTricuspid AtresiaAtresia TheThe RoadRoad toto SingleSingle VentricleVentricle

„ First,First, assureassure appropriateappropriate bloodblood flowflow toto thethe lungs,lungs, soso thethe bodybody getsgets enoughenough oxygenoxygen

„ BloodBlood isis rere--routedrouted throughthrough surgicalsurgical shuntsshunts toto getget moremore bloodblood toto thethe lungslungs ShuntsShunts TheThe RoadRoad toto SingleSingle VentricleVentricle

„ AfterAfter somesome timetime toto grow,grow, itit isis timetime toto beginbegin separatingseparating thethe blueblue bloodblood andand redred blood.blood.

„ ShownShown herehere isis thethe BidirectionalBidirectional GlennGlenn Shunt:Shunt: SVCSVC blueblue bloodblood goesgoes toto rightright andand leftleft lungslungs ArrivalArrival atat SingleSingle VentricleVentricle

„ CompleteComplete separationseparation ofof blueblue bloodblood andand redred bloodblood isis achievedachieved throughthrough anan operationoperation calledcalled thethe FontanFontan „ FirstFirst describeddescribed inin 19711971 „ PerformedPerformed afterafter moremore growthgrowth „ 33 variationsvariations FontanFontan

Classic Lateral Tunnel Extra-cardiac TheThe RoadRoad Ahead:Ahead: LifeLife beyondbeyond thethe FontanFontan

„ YouYou getget reallyreally wellwell acquaintedacquainted withwith youryour cardiologistcardiologist andand nursenurse practitionerspractitioners „ YouYou cancan expectexpect aa nearlynearly normalnormal lifelife –– EducationEducation –– CareerCareer developmentdevelopment –– ActivityActivity –– RelationshipsRelationships –– FamilyFamily PregnancyPregnancy

„ WomenWomen withwith singlesingle ventricleventricle maymay bebe ableable toto safelysafely andand successfullysuccessfully carrycarry aa pregnancypregnancy andand deliverdeliver theirtheir childchild „ WomenWomen withwith singlesingle ventricleventricle maymay faceface increasedincreased riskrisk ofof impairedimpaired fertilityfertility „ GreaterGreater raterate ofof obstetricobstetric complications,complications, prepre--termterm laborlabor andand miscarriagemiscarriage PregnancyPregnancy

„ SuccessSuccess ofof pregnancypregnancy isis stronglystrongly influencedinfluenced byby mothersmothers healthhealth beforebefore conceptionconception „ DiscussDiscuss plansplans forfor pregnancypregnancy withwith usus –– WeWe willwill discussdiscuss youryour overalloverall conditioncondition –– IdentifyIdentify risksrisks ofof pregnancypregnancy –– EvaluateEvaluate youryour cardiovascularcardiovascular healthhealth –– ReviewReview youryour medicationsmedications——somesome areare toxictoxic toto thethe fetusfetus TheThe RoadRoad maymay havehave bumpsbumps

„ ArrhythmiasArrhythmias „ CyanosisCyanosis „ LiverLiver abnormalitiesabnormalities „ ProteinProtein--losinglosing enteropathyenteropathy „ HeartHeart failurefailure „ BloodBlood clotsclots ArrhythmiasArrhythmias

„ HeartbeatHeartbeat tootoo fastfast – Arise from upper or lower heart chamber – Heartbeat signal is “short-circuited” by scar tissue from prior surgeries – May be treated with medications, device (defibrillator), or ablation „ HeartbeatHeartbeat tootoo slowslow – Natural pacemaker scarred by surgery – Treat with implantable pacemaker Pacemaker Ablation CyanosisCyanosis

„ MixingMixing ofof blueblue bloodblood andand redred bloodblood –– TooToo muchmuch bloodblood flowflow throughthrough intentionalintentional holehole (fenestration)(fenestration) inin FontanFontan pathwaypathway –– AbnormalAbnormal connectionsconnections betweenbetween arteriesarteries andand veinsveins (AVM)(AVM) –– CollateralCollateral (extra)(extra) bloodblood vesselsvessels thatthat growgrow –– LeakLeak atat thethe suturesuture lineline ofof thethe FontanFontan bafflebaffle CyanosisCyanosis

„ MayMay notnot appearappear blue!blue! „ DetectedDetected byby pulsepulse oximeteroximeter inin officeoffice –– ThisThis isis whywhy wewe likelike toto seesee youyou soso muchmuch –– SoSo wewe cancan detectdetect thesethese thingsthings earlyearly „ CauseCause cancan oftenoften bebe determineddetermined andand treatedtreated byby catheterization.catheterization. Leak closed with Baffle leak Amplatzer device HeartHeart FailureFailure

„ FailureFailure ofof thethe heartheart toto pumppump effectivelyeffectively (doesn(doesn’’tt meanmean itit stops!)stops!) – Weakening of pump – Enlargement of chamber „ TreatedTreated withwith medicationsmedications „ MayMay indicateindicate needneed forfor revisionrevision ofof FontanFontan (classic(classic toto laterallateral tunnel)tunnel) „ EnlargementEnlargement maymay stretchstretch heartheart valvesvalves causingcausing leaking,leaking, needneed valvevalve replacementreplacement HeartHeart FailureFailure FontanFontan revisionrevision ValveValve replacementreplacement LivingLiving LongLong andand WellWell withwith SingleSingle VentricleVentricle

„ RegularRegular followfollow--upup withwith ACHDACHD teamteam –– RoutineRoutine physicalphysical exam,exam, ECG,ECG, oximetryoximetry –– EchocardiogramEchocardiogram toto monitormonitor heartheart functionfunction andand bloodblood flowflow throughthrough FontanFontan pathwaypathway –– PeriodicPeriodic HolterHolter monitormonitor toto looklook forfor arrhythmiasarrhythmias –– WeWe maymay recommendrecommend MRIMRI oror CTCT ScansScans toto getget moremore detaileddetailed informationinformation aboutabout youryour heartheart LivingLiving LongLong andand WellWell withwith SingleSingle VentricleVentricle

–– YouYou maymay needneed cardiaccardiac catheterizationcatheterization toto evaluateevaluate andand treat:treat: „ Leaks in the Fontan pathway „ Narrowing in the Fontan pathway „ Arrhythmias (Ablation)

–– YouYou maymay needneed toto havehave moremore surgerysurgery „ Fontan revision „ Pacemaker / ICD placement „ Valve replacement LivingLiving LongLong andand WellWell withwith SingleSingle VentricleVentricle

„ ListenListen toto youryour bodybody – Call us with any symptoms or concerns „ TakeTake youryour medicationsmedications asas prescribedprescribed „ AvoidAvoid tobaccotobacco productsproducts „ GetGet regularregular exerciseexercise „ EatEat healthyhealthy – Fruits and vegetables – Whole grains – Go easy on fats, processed carbohydrates and salt ConclusionsConclusions

„ SingleSingle ventricleventricle includesincludes severalseveral differentdifferent congenitalcongenital heartheart defectsdefects „ TheThe RoadRoad toto singlesingle ventricleventricle hashas severalseveral stopsstops (operations)(operations) „ TheThe RoadRoad beyondbeyond thethe FontanFontan maymay havehave somesome bumpsbumps „ YouYou cancan helphelp smoothsmooth thethe RoadRoad byby gettinggetting preventivepreventive maintenancemaintenance ConclusionsConclusions

„ MostMost peoplepeople withwith singlesingle ventricleventricle livelive satisfying,satisfying, successfulsuccessful liveslives „ SurvivingSurviving andand ThrivingThriving withwith singlesingle ventricleventricle isis commoncommon „ IfIf youyou havehave singlesingle ventricle,ventricle, youyou areare notnot alone!alone! FinalFinal ThoughtThought

„ MayMay youyou livelive longlong andand wellwell enoughenough toto visitvisit thethe ““GoldenGolden YearsYears ClinicClinic onon CenturyCentury SquareSquare forfor SpleenSpleen ReadjustmentReadjustment andand MufflerMuffler RepairRepair””