醫(yī)學(xué)課件冠心病

1、Congenital Heart DiseasesIISamer Abbas, MDCardiology UIC/Christ02/27/2008ClassificationCyanosis with low pulm flow: - TOF - Ebstien Anamoly - Hypoplastic of RV - Tricuspid atresia - Pulm atresiaCyanosis with high pulm flow:TGADouble outlet V.Double inlet VTAPVD.Eisenmenger SyndromeIn 1897 Victor Eis

2、enmenger published a paper entitled “Congenital Defects of the Ventricular Septum.”In 1958, Paul Wood summarized Eisenmengers accounts:“The patient was a powerfully built man of 32 who gave a history of cyanosis and moderate breathlessness since infancy. He managed well until January of 1894 when dy

3、spnea increased and edema set in. Seven months later he was admitted to the hospital in a state of heart failureHe improved with rest and digitalis, but collapsed and died suddenly on November 13 following a large hemoptysis”Eisenmenger SyndromeLarge left to right shunt:severe pulmonary vascular dis

4、ease leading to shunt reversalInitial reversible changes:Medial hypertrophy of the pulmonary vasculatureIntimal proliferation Eisenmenger SyndromeProgressive irreversible changes:plexiform lesionsnecrotizing arteritisAs the increased PVR approaches or exceeds the SVR the shunt is reversed.As R to L

5、shunting develops cyanosis appears.Most patients will develop exertional dyspnea and impaired exercise tolerance.Eisenmenger SyndromePalpitations occur in 50% of patients A. fib/flutter in 40% VT in 10%)Hemoptysis in 20%PE, angina, syncope, endocarditis 10%Signs of PHTNRV heave, palpable P2, and rig

6、ht sided S4Pulmonary ejection click and a soft scratchy SEM d/t dilated pulmonary trunkHigh pitched decrescendo diastolic murmur (Graham-Steele) audible in most patients Usually no peripheral edema until R HF ensuesEisenmenger SyndromeECG shows RVH, RAE and RADAtrial arrhythmias may be presentEisenm

7、enger SyndromeCXR reveals prominent central pulmonary arteries and decreased vascular markings (pruning) of the peripheral vesselsEisenmenger SyndromeLarge variation in life expectancy in adults with Eisenmenger syndromeRate of survival among patients with Eisenmenger syndrome is:80% at 10 years77%

8、at 15 years42% at 25 yearsRecent study of 109 adults revealed following as independent predictors of mortality:Age at presentationSupraventricular arrhythmiasPoor NYHA functional class (III or IV)Eisenmenger Syndrome Pregnancy is discouraged due to high maternal (50%) and fetal (60%) mortality.CVA m

9、ay occur secondary to paradoxical emboliCerebral abscessesAvoid intravascular volume depletion, heavy exertion, high altitudes, and use of vasodilatorsIV epoprostenol may be beneficial in decreasing PVRPhlebotomy with isovolumic replacement is recommended for patients with moderate to severe symptom

10、s of hyperviscosity and an elevated hematocrit 65%Prevention of iron deficiency is important Supplemental oxygen reduces episodes of dyspnea Lung transplantation (with repair of cardiac defect) or heart/lung transplantation is an optionTetralogy of FallotEtienne-Louis Arthur Fallot made the first pu

11、blished bedside diagnosis that was proven at post-mortem in 1888 and called the condition of maladie bleue.EpidemiologyExact incidence of TOF with PS versus pulmonary atresia or absent pulmonary valve is not known as most studies lump them together.Most recent study is the BWIS (Baltimore Washington

12、 Infant Study) - -Fifth most common defect overall (6.8% of all CHD) -Most common cyanotic CHD -TOF with PS 0.26/1000 live births (5.4% of all CHD) -Suggestion of male predominance ( p=NS)GeneticsSyndromes associated with TOF:DiGeorges VelocardiofacialAlagillesTrisomy 21/18/13Cat EyeSan Luis Valley

13、Mutations of Nkx 2.5 may be associated with TOF. MorphologyVentricular Septal Defect80% of cases fibrous continuation between the mitral, tricuspid, and aortic valves20% cases there is a muscular rim around the defect.Rarely subarterial defect due to hypoplasia of the outlet septum. The pulmonary va

14、lve annulus tends to be hypoplastic or atretic.MorphologyOverriding AortaDegree of override can vary from an exclusive connection of the right ventricle to an exclusive connection to the left ventricle.Pulmonary Stenosis:InfundibularvalvularMorphologyConcentric right ventricular hypertrophy: Seconda

15、ry to RV outflow obstructionAssociated LesionsPulmonary valvar stenosisPulmonary atresiaAbsence of pulmonary valve leafletspulmonary insufficiency in utero and marked dilatation of the pulmonary trunk.Right aortic archPatent foramen ovaleAtrial Septal Defect ( Pentalogy of Fallot)Anomalous origin of

16、 the LAD from the RCA.On ExamCyanosis of varying degreesClubbingAccentuated RV impulseSecond heart sound usually single and loud. If mild PS with normal valve apparatus S2 normally split.Pulse pressure may be wide in the presence of aortopulmonary collaterals or PDA.On ExamSystolic Murmur: Upper lef

17、t sternal border, crescendo-decrescendo or plateauIntensity inversely proportional to severity of the right to left shuntIn PS with intact septum murmur directly proportional to the severity of stenosisMurmur decreases in intensity during hypercyanotic spellsDiastolic Murmur:If AI concomitantly hear

18、d in TOF with APV syndromeTetralogy of FallotTetralogy of FallotCatheterization Surgical repair Closure of VSDRelieving the RVOTOPulmonary valvotomyResection of infundibular muscleRVOT or subannular patchTransannular patch only when PV annulus is restrictivePulmonary valve implantation usually in ad

19、olescents and adultsExtracardiac conduit between the RV and PAAngioplasty or patch augmentation of the central pulmonary arteriesClosure of ASD or PFOComplications After RepairMechanical problemsSignificant Pulmonary regurgitationRV DilatationRestrictive RVResidual RVOTOAneurysmal dilatation of the

20、RVOT Residual VSDAR with or without root dilatationLV dysfunctionInfective endocarditisComplications after repairElectrical ProblemsHeart block: RBBB, heart block is rare.Supraventricular arrythmia () Relatively common. A.flutter-SCD,1:1.NSVT: Significance uncertain, ? SCDVTSCDRisks for SCD/VTOlder

21、age at repairMale genderWorse heart failure NYHA ClassChronic RV volume overloadPresence of PRQRS duration 180 msecTransient complete heart block lasting more than 3 days after repair. Management of the complicationsSignificant PR and RV dilatation Pulmonic valve replacement with homograft or porcin

22、e bioprosthesisTiming of surgery is debated. Selection of asymptomatic patient ?criteria.Might TV annuloplasty if concomitant moderate to severe TR.Restrictive RVDiuretics in the post-operative period. Residual RVOTOIndicated if RVSP2/3 systemic pressure.Surgery or balloon angioplasty with stenting.

23、Management of complicationsAneurysmal dilatation of the RVOTResidual VSD:Surgical correction if Qp/Qs2:1 or 1.5-2:1 with LV dilatation or dysfunctionor H/O paradoxical emboliDilatation of aortic root:Aortic root replacement when ascending aorta55 mm and/or in the presence severe AI.Management of the

24、 ComplicationsHeart block:Patients with bifascicular block with prolonged PR (0.20 msec) should have holter or EPS. If true trifascicular block is documented then pacemaker.SVTCorrection of target residual hemodynamic lesions.RF ablation for or reentrant tachycardia either percutaneously or during s

25、urgery.Biatrial maze for .Anti-arrhythmic meds /antitachycardia pacemakers can be used as adjunctivetools.Management of ComplicationsVTCorrection of the hemodynamic lesionsRF ablation of VT focusRole of AICD (?)Antiarrhythmic meds for symptomatic patients.SCDResuscitated SCD-AICDRole of prophylactic

26、 AICD (?)Fontan procedure-requiring patientTricuspid atresiaHypoplastic left ventricleDouble inlet ventricleIsomerismTricuspid atresiaTricuspid AtresiaOn Echo Hypoplastic Left HeartDouble Inlet VentricleFontan procedure10 year survival is 60-70%Poor prognosis5 year survival 46-59%Fontan PatientLong

27、term complications includeAtrial flutter or fibrillationIncidence increases with timeRight atrial thrombus formationObstruction of the Fontan circuitShould be r/o in all pts presenting with atrial arrhythmiasVentricular dysfunctionProtein losing enteropathyEtiology unclear: pleural effusions, ascite

28、s, edema and chronic diarrhea. Low , high -1 antitrypsin stool clearance.Management of ComplicationIn patients with atrial fibrillation and flutterPrompt TEE followed by cardioversionMaintenance of sinus with drugs ( Amiodarone) 50% success rate.Transcatheter atrial ablation 50% success rateRecalcit

29、rant cases surgical revision with antiarrhythmic surgeryManagement of ComplicationsIn patients with established thrombusThrombolytic therapySurgical thrombectomyConversion of Fontan circuitLong term anticoagulationMany centers prophylactically anticoagulate all patients with Fontan.Management of com

30、plicationsIn patients with Fontan obstructionSurgical revisionBalloon angioplastyProtein losing enteropathyCreation of a fenestration in the atrial septumHeparin SQIV Octreotide PrednisoneIn ECHOThe presence or absence of right atrial stasis, thrombus, patency of a fenestration, and Fontan circuit o

31、bstruction should be sought. Superior and inferior vena cavae biphasic and pulmonary artery triphasic flow patterns suggest unobstructed flow in the Fontan circuit.Mean gradient between the Fontan circuit and the pulmonary artery of 2 mm Hg or more may represent significant obstruction. Conduit or L

32、ateral Tunnel FontanFenestration FontanTGAD loopL loopRV follow the LoopD loop-RV on the rightL loop-RV on the leftL TGACongenitally corrected TGA.Ventricular inversion D TGAIncidence 20-30/1000,000 live births.Without treatment 30% die within 1st week, 50% within 1st month, 70% in 6 months and 90%

33、in first year.With current medical and surgical interventions 90% early and midterm survival.In complete Transposition the connection between atria and ventricles are normal. The connections between the ventricles and the great arteries are discordant.D TGASurgeryMost commonly done procedures were a

34、trial switch operations:Blood redirected at atrial level with a baffle made of dacron or pericardium (Mustard) With atrial flaps (Senning) to achieve physiological correctionSurgeryCurrent practice is the arterial switch operation developed in 1980s. Blood is redirected at the level of the great art

35、ery the morphological left ventricle becomes the subaortic ventricle the morphological right ventricle becomes the subpulmonic ventricle. Atrial Switch Surgery (Mustard/Senning Procedure) Mustard ProcedureMustard ProcedureModified Arterial Switch Operation Consequences of MustardMost patients reachi

36、ng adulthood have NYHA I/II symptoms over the next 25 years. 50% develop moderate systolic dysfunction of the RV but only few present with CHF.1/3rd have severe systemic TR.Atrial flutter arises in 20% by age 20.50% patients have sinus node dysfunction by age 20.Baffle leak or obstruction can also o

37、ccur.Baffle LeakConsequences of Arterial SwitchData about long term consequences still unavailable.Concerns include:supra neopulmonary artery stenosisostial coronary artery disease progressive neoaortic valve regurgitationTreatmentUse of ACE inhibitors suggests a benefit in non-randomized studies.ra

38、ndomized study using Ramipril is underway.Failed Mustard or Senning might be a reason for heart transplantation.Alternatively, atrial baffle can be converted to an arterial switch after retraining the LV with a pulmonary band.No data available for outcomesTV replacement is not recommended since it w

39、orsens RV function.Ebsteins anomalyMay be associated with:PFOASDMost commonly secundum rarely primumVSDwith or without pulmonary atresiaPulmonary outflow obstructionPDACoarctation of the aortaLV cardiomyopathyEbsteins AnomalyPrevalence 1:50,000 to 100,000. Occurs with higher frequency in infants of

40、mothers who took Lithium in early pregnancy.Clinical FeaturesDepends on the severity of the pathology and the magnitude of right to left intraatrial shunting. In milder forms of the condition incidental murmur and arrhythmia may be the only features. In severe forms of the disease cyanosis is seen a

41、nd depends on the extent of right to left shunting. Increased incidence of WPW syndrome.EchoIncrease in RV volumeParadoxical septal motionIncrease in TV excursionDelay in TV closure compared to MV closureDecreased TV EF slopeAbnormal anterior position of the TV in diastolePrognosis and ManagementSur

42、vival at 1 year 67% and 10 year 59 %.Survival after Tricuspid bioprosthesis: 10 yr 93% and 15 yr 81%. 94% in NYHA I/II.Management consists of surgical repair of the TV or replacement in older individuals.PregnancyBP decrease early in pregnancy 10 mmHg, usually secondary to decreased SVR This increas

43、es flow across right to left shunts.30-50% increase in intravascular volume. Poorly tolerated by women with valvular lesions and LV/RV dysfunction.Marked fluctuations occur in CO during normal labor and delivery.PregnancyMajor maternal cardiac risk factorsPulmonary HTN -Maternal mortality 30-50% wit

44、h Eisenmenger syndrome.Maternal Cyanosis-Cyanotic worse then acyanotic. -Erythrocytosis worsens outcome. -Goal Hct65Poor maternal functional class -Difficult to assess in cyanotic CHD.H/O Arrhythmia-Antiarrhythmic drugs can be used with accepted risk for debilitating symptoms AnticoagulationSpecific PatientsTOF Uncorrected TOF associated with worsening cyanosis. Susceptible to Infective endocarditis. Corrected TOF usual