Curs Cardiopatii ale Final 2011 2012

8/3/2019 Curs Cardiopatii ale Final 2011 2012

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CONGENITAL HEART DISEASECONGENITAL HEART DISEASE

CUPRINS:CUPRINS:

EmbriologieEmbriologie

ClasificareClasificareTablou clinicTablou clinic

DiagnosticDiagnostic

TratamentTratament

Prof univ dr Ion C.Tintoiu FESCProf univ dr Ion C.Tintoiu FESC


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INCIDENCEINCIDENCE

7 to 10 per 1000 live births7 to 10 per 1000 live births

Premature infants 2Premature infants 2--3X higher incidence3X higher incidence

Most common form of congenital diseaseMost common form of congenital disease:ASD,VSD:ASD,VSD

Accounts for 30% of total incidence of allAccounts for 30% of total incidence of allcongenital diseasescongenital diseases

10%10% --15% have associated congenital15% have associated congenitalanomalies of skeletal, RT, GUT or GITanomalies of skeletal, RT, GUT or GIT

Only 15% survive to adulthood withoutOnly 15% survive to adulthood withouttreatmenttreatment


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Embryologic DevelopmentEmbryologic Development

Braunwauld 6th ed


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ETIOLOGYETIOLOGY

10% associated with chromosomal abnormalities10% associated with chromosomal abnormalities

Two thirds of these occur with Trisomy 21Two thirds of these occur with Trisomy 21

One third occur with karyotypic abnormalities suchOne third occur with karyotypic abnormalities suchas Trisomy 13, Trisomy 18 & Turner Syndromeas Trisomy 13, Trisomy 18 & Turner Syndrome

Remaining 90% are multifactorial in originRemaining 90% are multifactorial in origin

Interaction of several genes with or without externalInteraction of several genes with or without externalfactors such as rubella, ethanol abuse, lithium andfactors such as rubella, ethanol abuse, lithium andmaternal diabetes mellitusmaternal diabetes mellitus


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FETAL CIRCULATIONFETAL CIRCULATION

There are 4 shunts inThere are 4 shunts infetal circulation: placenta,fetal circulation: placenta,ductus venosus, foramenductus venosus, foramen

ovale, and ductusovale, and ductusarteriosusarteriosus

In adult, gas exchangeIn adult, gas exchangeoccurs in lungs. In fetus,occurs in lungs. In fetus,the placenta provides thethe placenta provides theexchange of gases andexchange of gases andnutrientsnutrients


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NORMAL HEMODYNAMICNORMAL HEMODYNAMIC


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RelativeF

requency of LesionsRelativeF

requency of Lesions Ventricular septal defect 25-30

Atrial septal defect (secundum) 6-8

Patent ductus arteriosus 6-8

Coarctation of aorta 5-7

Tetralogy of Fallot 5-7 Pulmonary valve stenosis 5-7

Aortic valve stenosis 4-7

Transposition of great arteries 3-5

Hypoplastic left ventricle 1-3

Hypoplastic right ventricle 1-3

Truncus arteriosus 1-2

Total anomalous pulm venous return 1-2

Tricuspid atresia 1-2

Double-outlet right ventricle 1-2

Others 5-10


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CHDCHD CLASIFICARECLASIFICARE

--SHUNT LSHUNT L--RR

--SHUNT RSHUNT R--LL --NON SHUNTNON SHUNT

--COMPLEXCOMPLEX


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CLASSIFICATION OF CHDCLASSIFICATION OF CHD


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LL -- R SHUNTS INCLUDE :R SHUNTS INCLUDE :

ASDASD 7.5% of CHD7.5% of CHD

VSDVSD COMMONEST CHDCOMMONEST CHD 25%25% PDAPDA 7.5% of CHD7.5% of CHD

Common in premature infantsCommon in premature infants

ENDOCARDIAL CUSHION DEFECTENDOCARDIAL CUSHION DEFECT -- 3%3%

Often seen with trisomy 21Often seen with trisomy 21

AORTOPULMONARY WINDOWAORTOPULMONARY WINDOW


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Noncyanotic CHD (LNoncyanotic CHD (Lpp

R)R) Atrial septal defects (ASD)

Ventricular septal defects (VSD)

Patent ductus arteriosus (PDA)

Obstruction to blood flow

Pulmonic stenosis (PS)

Aortic stenosis (AS)

Aortic coarctation


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ASDASD -- ClinicalClinical

Majority repaired in childhood, but may presentMajority repaired in childhood, but may present

in adolescence/adulthoodin adolescence/adulthood

AsymptomaticAsymptomatic murmur, abnl ECG/CXRmurmur, abnl ECG/CXR

SymptomaticSymptomatic

dyspnea/CHFdyspnea/CHF

CVA/emboliCVA/emboli

Atrial FibrillationAtrial Fibrillation


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Auscultation in ASDAuscultation in ASD

Increased flow across the pulmonary

valve produces a systolic ejection murmur

and fixed splitting of the second heart sound.

Fixed splitting ofS2 may in part be due to

delayed right bundle conduction.

Increased flow across the TV produces a

diastolic rumble at the mid to lower rightsternal border.

Older pt loses pulm ejection

murmur as shunt becomes

bidirectional signs of pulm HTN/CHF may

predominate


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Atrial Septal DefectAtrial Septal Defect

Enlargement of the rightEnlargement of the rightventricleventricle

Enlargement of atriumEnlargement of atrium Large pulmonary arteryLarge pulmonary artery

increased pulmonaryincreased pulmonaryvascularity is.vascularity is.


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CORTRIATRIATUMCORTRIATRIATUM


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COR TRIATIATUMCOR TRIATIATUM


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VENTRICULAR SEPTAL DEFECTVENTRICULAR SEPTAL DEFECT


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VSDVSD

Defined:Defined:A congenital abnormality in which blood flows from highA congenital abnormality in which blood flows from highpressure LVpressure LV low pressure RV through a holelow pressure RV through a hole

TT-- holosystolicholosystolic

LL-- 33rdrd, 4, 4thth, 5, 5thth left interspacesleft interspaces CC-- harsh, loudharsh, loud

AA-- alone or with other abnormalitiesalone or with other abnormalities

BB-- infant: late cyanosis, holo/pancystolicinfant: late cyanosis, holo/pancystolic

adult: progressive pulmonary HTN, Eisengengersadult: progressive pulmonary HTN, Eisengengerssyndromesyndrome


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VENTRICULAR SEPTAL DEFECTVENTRICULAR SEPTAL DEFECT


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VSDVSD


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Ventricular Septal Defect (VSD)Ventricular Septal Defect (VSD)

Large VSD:The presence of right ventricular hypertrophy, olegeimic lung fields

(pulmonary hypertension or an associated pulmonic stenosis), gross

cardiomegaly with prominence of both ventricles, the left atrium.

Small VSDs, the chest radiograph is usually normal


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ATRIOVENTRICULAR CANALATRIOVENTRICULAR CANAL

DEFECTSDEFECTS


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ATRIOVENTRICULAR CANALATRIOVENTRICULAR CANAL

DEFECTDEFECT


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PATENT DUCTUS ARTERIOSUSPATENT DUCTUS ARTERIOSUS


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Patent Ductus ArteriosusPatent Ductus Arteriosus

Defined:Defined: Failure of the duct between pulmonary arteryFailure of the duct between pulmonary arteryand aorta to closeand aorta to close

TT-- ContinuousContinuous

LL-- upper left sternal borderupper left sternal border

CC-- machinemachine--likelike

AA-- leftleft right shunt, cyanosisright shunt, cyanosis

BB-- infants, machineinfants, machine--like,like, continuouscontinuousmurmurmurmur


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Small defect noSmall defect nosymptoms.symptoms.

Large defect:Large defect:

Wide pulse pressureWide pulse pressure Enlarged heartEnlarged heart

Thrill in L second ISThrill in L second IS

Continuous murmurContinuous murmur

XX--ray: prominentray: prominentpulmonary artery withpulmonary artery withincreased vascularincreased vascularmarkings.markings.


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OBSTRUCTIVE LESIONSOBSTRUCTIVE LESIONS


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COARCTATION OF AORTACOARCTATION OF AORTA


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Coarctation of Aorta: ClinicalCoarctation of Aorta: Clinical

Most repaired, but adult presentation may be:Most repaired, but adult presentation may be:

HTNHTN

murmur (continuous or systolic murmur heard in back ormurmur (continuous or systolic murmur heard in back or

SEM/ejection click of bicuspid AV)SEM/ejection click of bicuspid AV)

weak/delayed LE pulsesweak/delayed LE pulses

Rib notching on CXR pathognomonicRib notching on CXR pathognomonic


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Rib notchingRib notching


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Coarctation of AortaCoarctation of Aorta

Narrowing in proximalNarrowing in proximaldescending aortadescending aorta

May be long/tubular butMay be long/tubular but

most commonly discretemost commonly discreteridgeridge

Natural hx: poor prognosisNatural hx: poor prognosisif unrepairedif unrepaired

Aortic Aneurysm/dissectionAortic Aneurysm/dissection CHFCHF

Premature CADzPremature CADz


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Coarcation: TreatmentCoarcation: Treatment

Despite surgery, patients still have significantDespite surgery, patients still have significantmorbidity/mortality with average age 38morbidity/mortality with average age 38

Up to 70% of repaired patients still go on to developUp to 70% of repaired patients still go on to develop

HTN, pathology not well understoodHTN, pathology not well understood Recurrence in 8Recurrence in 8--54% of repairs, can undergo repeat54% of repairs, can undergo repeat

surgery or balloon angioplastysurgery or balloon angioplasty

Aortic Aneurysm/ruputure may occur despiteAortic Aneurysm/ruputure may occur despite

successful repair and correction of HTN (freq aroundsuccessful repair and correction of HTN (freq aroundanastomosis site on patch repairanastomosis site on patch repair 30% in one study)30% in one study)

C i R iC i R i


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Coarctation RepairCoarctation Repair

Edmunds Cardiac Surgery in the Adult, Ch 47

Surgical correction1) Patch aortoplasty with removal of segment

and end to end anastomosis or

subclavian flap repair

2) bypass tube grafting around segment


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Coarctation: FollowupCoarctation: Followup

Every 1Every 1--2 years2 years

Document arm/leg BPDocument arm/leg BP

Screen/treat CAD risk factorsScreen/treat CAD risk factors

HTN: rest, provoked by exercise or seen onHTN: rest, provoked by exercise or seen onambulatory monitoringambulatory monitoring

ECHO/doppler to eval recurrentECHO/doppler to eval recurrent

MRI for aneurysmMRI for aneurysm


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AORTIC STENOSIS AORTIC STENOSIS


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Aortic StenosisAortic Stenosis

Defined:Defined: Narrowing of the aortic outflow tract causingNarrowing of the aortic outflow tract causingobstruction of flow from the left ventricle into the ascendingobstruction of flow from the left ventricle into the ascendingaortaaorta

TT-- midmid--systolicsystolic

LL-- apexapex-- R 2R 2ndnd intercostal space, radiates to carotidsintercostal space, radiates to carotids

CC-- harsh, loud, may have associated thrill, ejectionharsh, loud, may have associated thrill, ejectionclickclick

AA-- older age, bicuspid aortic valve, rheumatic feverolder age, bicuspid aortic valve, rheumatic fever BB-- age of patient, pulsus parvus et tardus, angina,age of patient, pulsus parvus et tardus, angina,

syncope, heart failuresyncope, heart failure


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CYANOTIC CONGENITAL HDCYANOTIC CONGENITAL HD


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TETRALOGY OFFALLOTTETRALOGY OFFALLOT


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Tetralogy ofFallotTetralogy ofFallot


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4 features4 features

Malalignment VSDMalalignment VSD

Overriding AortaOverriding Aorta

Pulmonic StenosisPulmonic Stenosis

RVHRVH

Variability correlates withVariability correlates with

degree of RVOTdegree of RVOTobstruction andobstruction andsize/anatomy of PAsize/anatomy of PA


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Tetralogy: Treatment/complicationsTetralogy: Treatment/complications

SystemicSystemic--Pulm shuntPulm shunt

leads to high flow through PA, elevated PVR andleads to high flow through PA, elevated PVR andbranch PA distortionbranch PA distortion

survival after repair worse in pt with prior Waterstonsurvival after repair worse in pt with prior Waterstonor Potts shunt (?higher flow); some pt with Blalockor Potts shunt (?higher flow); some pt with Blalock--Taussig shunts may survive unrepaired intoTaussig shunts may survive unrepaired intoadulthoodadulthood

these pt should be evaluated for pulm artery stenosisthese pt should be evaluated for pulm artery stenosisand Pulm HTNand Pulm HTN


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Tetralogy: Surgical TreatmentTetralogy: Surgical Treatment

SystemicSystemic Pulmonary ShuntPulmonary Shunt

BlalockBlalock--TaussigTaussig

Waterston (RPA)Waterston (RPA)

Potts (LPA)Potts (LPA) Complete RepairComplete Repair

takedown of prior shunttakedown of prior shunt

patch VSDpatch VSD

resection of subpulmonic obstructionresection of subpulmonic obstruction

transannular patch around pulm valve annulus (usually leadstransannular patch around pulm valve annulus (usually leadsto severe PI)to severe PI)


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Tetralogy: Treatment/complicationsTetralogy: Treatment/complications

Prior pulmonary valve atresia or anomalousPrior pulmonary valve atresia or anomalous

LAD may have had prosthetic or homograftLAD may have had prosthetic or homograft

conduitconduit valve placed between RV and PAvalve placed between RV and PA

Conduits can undergo endothelial overgrowthConduits can undergo endothelial overgrowthand obstruction of pseudo RVOTand obstruction of pseudo RVOT can Rxcan Rx

with balloon angioplasty or operative conduitwith balloon angioplasty or operative conduit

replacementreplacement


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Tetralogy: Risk/followupTetralogy: Risk/followup

SCD 25SCD 25--100 fold100 fold risk can occur 2 decades after correctionrisk can occur 2 decades after correction

related to QRS duration> 180msecrelated to QRS duration> 180msec

? Due to PI/RV conduction defect? Due to PI/RV conduction defect

atrial arrhythmias also commonatrial arrhythmias also common Hemodynamic effects of PIHemodynamic effects of PI

Chronic RV volume overload, RV dysfunction and exerciseChronic RV volume overload, RV dysfunction and exerciseintoleranceintolerance

Pulmonic Valve Replacement can decrease QRS durationPulmonic Valve Replacement can decrease QRS durationand stabilize RV fxn; timing unclear but earlier better thanand stabilize RV fxn; timing unclear but earlier better thanlaterlater

RV fxn: ECHO or MRIRV fxn: ECHO or MRI

TRANSPOSITION OF GREATTRANSPOSITION OF GREAT


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TRANSPOSITION OF GREATTRANSPOSITION OF GREAT

ARTERYARTERY


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Transposition DTransposition D--typetype

PA arises from LV, Aorta from RV and

anterior/right ofPA

cyanosis

corrected initially with prostaglandin to keep

ductus open and balloon atrial septostomy toimprove systemic saturation

repair via atrial switch Mustard procedure

SVC/IVC baffled to LA/LV/PA

Pulm Veins baffled to RA/RV/Ao

Symptom free survival until 2nd-3rd

decade of life repair via arterial switch

long term data ?

pulmonic valve (neo-aortic valve)

competence?, reimplanted coronaries

may develop ostial stenoses


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DD--Transposition complicationsTransposition complications

ComplicationsComplications arrhythmias/SCDarrhythmias/SCD

Only 18% maintain SR; most go on to SSS/Afib/ Aflutter; pacemaker oftenOnly 18% maintain SR; most go on to SSS/Afib/ Aflutter; pacemaker oftenneededneeded

systemic (tricuspid) atrioventricular valve regurgitationsystemic (tricuspid) atrioventricular valve regurgitation

? TVR? TVR systemic (RV) ventricular failuresystemic (RV) ventricular failure

15% have CHF sxs by 215% have CHF sxs by 2ndnd--33rdrd decadedecade

Rx transplant or staged Arterial switch (pulm banding to train LV)Rx transplant or staged Arterial switch (pulm banding to train LV)

baffle obstructionbaffle obstruction Rare (5%) but serious complication; venous more commonRare (5%) but serious complication; venous more common

Suspect if new upper extremity edema (venous) or new CHF sxs (pulmSuspect if new upper extremity edema (venous) or new CHF sxs (pulmvenous)venous)

ECHO or Cath to eval, pulm venous obstruction Rx with surgery, systemicECHO or Cath to eval, pulm venous obstruction Rx with surgery, systemicvenous with angioplasty/stentsvenous with angioplasty/stents


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LL--type Transpositiontype Transposition

AtrialAtrial--ventricular ANDventricular ANDventricularventricular--arterial discordancearterial discordance

Physiologically correct,Physiologically correct,anatomically incorrectanatomically incorrect

congenitally correctedcongenitally correctedtranspositiontransposition

RV is systemic ventricle, TV isRV is systemic ventricle, TV issystemic AV valvesystemic AV valve

Asymptomatic for many years,Asymptomatic for many years,often into adulthoodoften into adulthood


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LL--type transposition: complicationstype transposition: complications

Although seemingly benign, survival is reduced withAlthough seemingly benign, survival is reduced withone study showing 25% of patients died by mean ageone study showing 25% of patients died by mean age3838

Progressive Heart FailureProgressive Heart Failure

ArrhythmiasArrhythmias SCDSCD

AV blockAV block

Atrial arrhythmiasAtrial arrhythmias

Severe AV (tricuspid) regurgitationSevere AV (tricuspid) regurgitation TVRTVR difficult to image using conventional ECHOdifficult to image using conventional ECHO

MRI becoming test of choice for RV functionMRI becoming test of choice for RV function


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TRUNCUS ARTERIOSUSTRUNCUS ARTERIOSUS


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EBSTEIN ANOMALYEBSTEIN ANOMALY


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Ebsteins AnomalyEbsteins Anomaly

Atrialization of RV, sailAtrialization of RV, sail--like TV,like TV,TRTR

50% ASD/PFO50% ASD/PFO

50% ECG evidence of WPW50% ECG evidence of WPW

Age at presentation varies fromAge at presentation varies from

childhoodchildhoodadulthood and dependsadulthood and dependson factors such as severity of TR,on factors such as severity of TR,Pulm Vascular resistance inPulm Vascular resistance in

newborn, and associatednewborn, and associatedabnormalities such as ASDabnormalities such as ASD

www.ucch.org


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Ebsteins: Clinical PresentationEbsteins: Clinical Presentation

PediatricPediatric

murmurmurmur

Adult (unrepaired with ASD)Adult (unrepaired with ASD)

atrial arrhythmiasatrial arrhythmias murmurmurmur

cyanosiscyanosis

RRL shunt NOT due to PulmHTN but TR jet directed across ASDL shunt NOT due to PulmHTN but TR jet directed across ASD

exercise intoleranceexercise intolerance

Surgery in pts with significant TR/sxsSurgery in pts with significant TR/sxs


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Massive cardiomegaly,

mainly due to RAE


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PULMONARY HYPERTENSIONPULMONARY HYPERTENSION

EISENMENGER SYNDROMEISENMENGER SYNDROM


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Eisenmenger SyndromEisenmenger Syndrom PulmonaryPulmonary


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Eisenmenger SyndromEisenmenger Syndrom-- PulmonaryPulmonaryHypertensionHypertension

Prominent pulmonaryProminent pulmonaryartery.artery.

Prominent rightProminent rightventricleventricle

Prominent vascularity inProminent vascularity inthe hilar areasthe hilar areas

Decreased vascualrDecreased vascualrmarking in themarking in the

periphery.periphery. No treatmentNo treatment

RxRx


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Eisenmengers SyndromeEisenmengers Syndrome

Final common pathway for all significant LFinal common pathway for all significant LRRshunting in which unrestricted pulmonary bloodshunting in which unrestricted pulmonary bloodflow leads to pulmonary vasoflow leads to pulmonary vaso--occlusive diseaseocclusive disease

(PVOD); R(PVOD); RL shunting/cyanosis devleopsL shunting/cyanosis devleops

Generally need Qp:Qs >2:1Generally need Qp:Qs >2:1


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Eisenmenger: TreatmentEisenmenger: Treatment

Sxs +polycythemiaSxs +polycythemia phlebotomyphlebotomy Careful if microcytosis, strongest predictor of cerebrovascularCareful if microcytosis, strongest predictor of cerebrovascular

eventsevents

RULE OUT CORRECTABLE DISEASERULE OUT CORRECTABLE DISEASE

Once diagnosis established, avoid aggressive testing asOnce diagnosis established, avoid aggressive testing asmany patients die during cardiovascular proceduresmany patients die during cardiovascular procedures

Diuretics prn, oxygenDiuretics prn, oxygen

Definitive: Heart Lung transplantDefinitive: Heart Lung transplant Prostacyclin therapy may delay, expensiveProstacyclin therapy may delay, expensive


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Eisenmenger ComplicationsEisenmenger Complications

Coagulopathy/platelet consumptionCoagulopathy/platelet consumption

Brain abcessesBrain abcesses

Cerebral microemboliCerebral microemboliAirway hemorrhageAirway hemorrhage

especially moving from lowerespecially moving from lowerhigher altitudes (airhigher altitudes (airtravel, mountains)travel, mountains)


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COARTATION af the AORTACOARTATION af the AORTA


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Coarcation: TreatmentCoarcation: Treatment

Despite surgery, patients still have significantDespite surgery, patients still have significantmorbidity/mortality with average age 38morbidity/mortality with average age 38

Up to 70% of repaired patients still go on to developUp to 70% of repaired patients still go on to developHTN, pathology not well understoodHTN, pathology not well understood

Recurrence in 8Recurrence in 8--54% of repairs, can undergo repeat54% of repairs, can undergo repeatsurgery or balloon angioplastysurgery or balloon angioplasty

Aortic Aneurysm/ruputure may occur despiteAortic Aneurysm/ruputure may occur despitesuccessful repair and correction of HTN (freq aroundsuccessful repair and correction of HTN (freq aroundanastomosis site on patch repairanastomosis site on patch repair 30% in one study)30% in one study)


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Coarctation: FollowupCoarctation: Followup

Every 1Every 1--2 years2 years

Document arm/leg BPDocument arm/leg BP

Screen/treat CAD risk factorsScreen/treat CAD risk factors

HTN: rest, provoked by exercise or seen onHTN: rest, provoked by exercise or seen onambulatory monitoringambulatory monitoring

ECHO/doppler to eval recurrentECHO/doppler to eval recurrent

MRI for aneurysmMRI for aneurysm


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PULMONARYVALVE STENOSISPULMONARYVALVE STENOSIS


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Surgery for CongenitalSurgery for CongenitalHeart DiseasesHeart Diseases


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Surgical IndicationsSurgical Indications

andandOptimal Timing of OperationOptimal Timing of Operation

Palliative SurgeryPalliative Surgery


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Palliative SurgeryPalliative Surgery

SystemicSystemic pulmonary artery shuntpulmonary artery shunt

BlalockBlalock--Taussig shuntTaussig shunt

Unifocalization and shuntUnifocalization and shunt

Cavopulmonary shunt (BCPS)Cavopulmonary shunt (BCPS) RVOT reconstructionRVOT reconstruction

ValvotomyValvotomy

Patch wideningPatch widening

Valved conduitValved conduit

Pulmonary artery bandingPulmonary artery banding

Atrial septectomyAtrial septectomy


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Pulmonary Artery BandingPulmonary Artery Banding


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Pulmonary Artery BandingPulmonary Artery Banding

Pulmonary artery banding is indicated to decreasePulmonary artery banding is indicated to decreasepulmonary blood flow & protect vascular disease when:pulmonary blood flow & protect vascular disease when:

Control of congestive heart failureControl of congestive heart failure

Complex or multiple VSD (+/Complex or multiple VSD (+/-- coarctation)coarctation)

Single ventricle, Tricuspid atresia without PSSingle ventricle, Tricuspid atresia without PS

CPB medically contraindicatedCPB medically contraindicated

Protection of pulmonary vascular bedProtection of pulmonary vascular bed

Single ventricleSingle ventricle Fontan operationFontan operation Preparation of LV for arterial switch operationPreparation of LV for arterial switch operation

TGA with IVS / restrictive VSDTGA with IVS / restrictive VSD

Atrial SeptectomyAtrial Septectomy


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Atrial SeptectomyAtrial Septectomy

For the increasing of effective pulmonaryFor the increasing of effective pulmonary

flow and systemic oxygen saturationflow and systemic oxygen saturation

Indication of atrial septectomy :Indication of atrial septectomy :TGATGA

Tricuspid atresiaTricuspid atresia

Pulmonary atresia + IVSPulmonary atresia + IVS

MV and LV hypoplasiaMV and LV hypoplasia

Decreasing tendency of indication due to earlyDecreasing tendency of indication due to earlytotal correction or interventiontotal correction or intervention


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Reparative SurgeryReparative Surgery

NonNon--open heart surgeryopen heart surgery

Open heart surgeryOpen heart surgery

Palliative procedurePalliative procedure

Corrective procedureCorrective procedure

Anatomic correctionAnatomic correction

Physiologic correctionPhysiologic correction

NN H SH S


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NonNon--open Heart Surgeryopen Heart Surgery

Palliative procedurePalliative procedure

Corrective procedureCorrective procedure

PDAPDA

COACOA

Vascular ring and slingVascular ring and sling

Coronary artery anomaliesCoronary artery anomalies Stenotic valvular diseasesStenotic valvular diseases

Inflow occlusion techniqueInflow occlusion technique

Instrumental dilatationInstrumental dilatation

P t t D t A t iP t t D t A t i


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Open communication usually between upperOpen communication usually between upperdescending Ao and proximal portion of LPAdescending Ao and proximal portion of LPA

Significant PDA : indicated after 1Significant PDA : indicated after 1stst monthmonth

Prophylactic closure : 6Prophylactic closure : 6--12 mo12 mo

Sx of heart failure or failure to thrive : indicatedSx of heart failure or failure to thrive : indicatedat any timeat any time

Severe pulmonary vascular disease : contraindicatedSevere pulmonary vascular disease : contraindicated

Coarctation of the AortaCoarctation of the Aorta


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Coarctation of the AortaCoarctation of the Aorta Congenital narrowing of upper thoracic aorta adjacentCongenital narrowing of upper thoracic aorta adjacent

to the ductus arteriosusto the ductus arteriosus

Operation is indicated when :Operation is indicated when :

Reduction of luminal diameter > 50%Reduction of luminal diameter > 50%Upper body HT > 150mmHg in young infantUpper body HT > 150mmHg in young infant

With CHF at any ageWith CHF at any age

COA with VSDCOA with VSD

Two stage repairTwo stage repairOne stage repairOne stage repair

COA with other important intracardiac defectsCOA with other important intracardiac defects

One stage repairOne stage repair


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Open Heart SurgeryOpen Heart Surgery

Total Anomalous PulmonaryTotal Anomalous Pulmonary


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Total Anomalous PulmonaryTotal Anomalous Pulmonary

Venous ConnectionVenous Connection

These are no direct connection between anyThese are no direct connection between any

pulmonary vein and the LA. But rather, all thepulmonary vein and the LA. But rather, all the

pulmonary veins connect to the RA or one of itspulmonary veins connect to the RA or one of its

tributariestributaries

Dx is an indication of operationDx is an indication of operation

Immediate repair with Dx in any ill neonate :Immediate repair with Dx in any ill neonate :Preop preparation is not neededPreop preparation is not needed

Repair should be done nearly always before 6 moRepair should be done nearly always before 6 mo

Dx at 6Dx at 6--12 mo : prompt repair is indicated12 mo : prompt repair is indicated

Ventric lar Septal DefectVentric lar Septal Defect


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Ventricular Septal DefectVentricular Septal Defect

A hole (or multiple holes) between Lt & Rt ventricleA hole (or multiple holes) between Lt & Rt ventricle

Symptomatic large VSD : an indication of operationSymptomatic large VSD : an indication of operation

Before 3 mo : indicated in large VSDs with CHF orBefore 3 mo : indicated in large VSDs with CHF orrespiratory symptomsrespiratory symptoms

Moderate sized VSDs (Qp/Qs < 3.0) with fewModerate sized VSDs (Qp/Qs < 3.0) with fewsymptoms : observation during infancysymptoms : observation during infancy

Small VSDs (Qp/Qs < 1.5) :Small VSDs (Qp/Qs < 1.5) :

not indicated, risk of bacterial endocarditisnot indicated, risk of bacterial endocarditis

Subarterial type : early repair is indicated beforeSubarterial type : early repair is indicated beforechildhoodchildhood

Atrioventricular Septal DefectAtrioventricular Septal Defect


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Atrioventricular Septal DefectAtrioventricular Septal Defect

Abnormalities of atrioventricular valve form &Abnormalities of atrioventricular valve form &function and interatrial & interventricularfunction and interatrial & interventricularcommunication from maldevelopment of thecommunication from maldevelopment of theendocardial cushionsendocardial cushions

Presence of AVSD : indicated with DxPresence of AVSD : indicated with Dx Partial AVSD : 1Partial AVSD : 1--2 years of age except CHF or2 years of age except CHF or

growth failuregrowth failure

Complete AVSD with good condition : 3Complete AVSD with good condition : 3--6 mo6 mo Complete AVSD with refractory CHF or respiratoryComplete AVSD with refractory CHF or respiratory

Sx : indicated promptlySx : indicated promptly

Development of pulmonary vascular obstructiveDevelopment of pulmonary vascular obstructivedisease : not indicateddisease : not indicated

C i l A i S iC i l A i S i


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Congenital Aortic StenosisCongenital Aortic Stenosis

TThe various forms of LVOTO occur in combinationhe various forms of LVOTO occur in combinationwith other cardiac lesions (IAA, COA, MVanomalies,with other cardiac lesions (IAA, COA, MVanomalies,LVhypoplasia) and obstructive types areLVhypoplasia) and obstructive types aresupravalvular, valvular, subvalvular, intraventricularsupravalvular, valvular, subvalvular, intraventricular

Critical AS in neonates : urgent (severe CHF, LVCritical AS in neonates : urgent (severe CHF, LV

dilatation, hypertrophy)dilatation, hypertrophy)

Infants and childrenInfants and children

Pressure gradient > 75mmHgPressure gradient > 75mmHgSx of angina, syncope, exercise intolerance, LVH,Sx of angina, syncope, exercise intolerance, LVH,

pressure gradient > 50mmHgpressure gradient > 50mmHg

Pressure gradient over 40mmHg in subvalvular lesionPressure gradient over 40mmHg in subvalvular lesion

to prevent progressionto prevent progression

Aortopulmonary WindowAortopulmonary Window


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Aortopulmonary WindowAortopulmonary Window

Abnormal development of aortopulmonary septumAbnormal development of aortopulmonary septumcaused by incomplete formation of the right and leftcaused by incomplete formation of the right and leftconotruncal ridges.conotruncal ridges.

Symptomatic AP window is an indicationSymptomatic AP window is an indication

Symptomatic infants : indicated with DxSymptomatic infants : indicated with Dx

Repair is advised before 3 mo of age (size, increasedRepair is advised before 3 mo of age (size, increased

shunt, pulmonary hypertension)shunt, pulmonary hypertension) Old children should be operated on unless PVR renderOld children should be operated on unless PVR render

them inoperablethem inoperable


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Aneurysm of Sinus ofValsalvaAneurysm of Sinus ofValsalva Thin walled, tubular outpouchings, nearly always RtThin walled, tubular outpouchings, nearly always Rt

sinus or adjacent half of the noncoronary sinus and withsinus or adjacent half of the noncoronary sinus and withan entirely intracardiac course, that may rupture into thean entirely intracardiac course, that may rupture into theright (rarely left) heart chamber to form a fistularight (rarely left) heart chamber to form a fistula

Ruptured sinus ofValsalva : indicated promptly becauseRuptured sinus ofValsalva : indicated promptly becauseof abrupt development of CHFof abrupt development of CHF

With VSD or VSD+AI : prompt repair is indicatedWith VSD or VSD+AI : prompt repair is indicated

Large aneurysms producing hemodynamic derangement :Large aneurysms producing hemodynamic derangement :indicatedindicated

Small and moderate sized aneurysm without symptom :Small and moderate sized aneurysm without symptom :not indicatednot indicated

Cor TriatriatumCor Triatriatum


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Cor TriatriatumCor Triatriatum

A rare congenital cardiac anomaly in which pulmonaryA rare congenital cardiac anomaly in which pulmonaryveins typically enter a proximal left atrial chamberveins typically enter a proximal left atrial chamberseparated from the distal left atrial chamber by aseparated from the distal left atrial chamber by adiaphragm in thich there are one or more restrictive ostiadiaphragm in thich there are one or more restrictive ostia

(sinister, dexter)(sinister, dexter)

Indication for operationIndication for operation

Restrictive aperture in the partition is an urgentRestrictive aperture in the partition is an urgentindicationindication

Symptoms usually develop early, and operation isSymptoms usually develop early, and operation isnecessary in the 1necessary in the 1ststyear of lifeyear of life

Life expectancy after repair in infancy is excellentLife expectancy after repair in infancy is excellent

Ebsteins anomalyEbsteins anomaly


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Ebsteins anomalyEbsteins anomaly

A congenital defect of tricuspid valve in which the origin ofA congenital defect of tricuspid valve in which the origin of

septal and posterior leaflets or both are displaced downwardseptal and posterior leaflets or both are displaced downward

into the right ventricle and the leaflets are variably deformedinto the right ventricle and the leaflets are variably deformed

Symptomatic Ebsteins anomaly is an indicationSymptomatic Ebsteins anomaly is an indication Neonates presenting in extremes :Neonates presenting in extremes :

Starnes procedure in first weekStarnes procedure in first week

Valve repair and ASD closure :Valve repair and ASD closure :

with important TRwith important TRmoderate and severe cyanosismoderate and severe cyanosis

WPW syndromes :WPW syndromes :

ablation of accessory conduction pathwayablation of accessory conduction pathway

Pulmonary StenosisPulmonary Stenosis


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Pulmonary StenosisPulmonary Stenosis

A form of RVoutflow obstruction in which stenosis isA form of RVoutflow obstruction in which stenosis isusually valvar or both valvar & infundibular or onlyusually valvar or both valvar & infundibular or onlyinfundibularinfundibular

Critical PS in neonate : indicated with DxCritical PS in neonate : indicated with Dx

Percutaneous balloon valvotomyPercutaneous balloon valvotomy

Valvotomy with CPBValvotomy with CPB

Transannular RVOT patch wideningTransannular RVOT patch widening

Valvotomy with inflow occlusion techniqueValvotomy with inflow occlusion technique

PS in infants and children : indicated with Sx & PrPS in infants and children : indicated with Sx & Prgradient over 50mmHggradient over 50mmHg

Surgical treatment is not indicated with mild stenosisSurgical treatment is not indicated with mild stenosis

Tetralogy of FallotTetralogy of Fallot


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Characterized by underdevelopment of RVCharacterized by underdevelopment of RVinfundibulum with anterior and left wardinfundibulum with anterior and left warddisplacementdisplacement

Dx is an indication of operationDx is an indication of operation Symptomatic complicated in early life :Symptomatic complicated in early life :

Early total correction orEarly total correction or

Shunt (1Shunt (1--2 mo) and total correction (1 year)2 mo) and total correction (1 year)

Asymptomatic uncomplicated :Asymptomatic uncomplicated :

Total correction at 3Total correction at 3--24 mo24 mo

Multiple VSDs, LAD from RCA :Multiple VSDs, LAD from RCA :

Initial shunt and total correctionInitial shunt and total correction


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Tr n p iti n f Gr t Art riTr n p iti n f Gr t Art ri


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Transposition of Great ArteriesTransposition of Great Arteries

A cardiac anomaly in which the Ao arises entirely or in largeA cardiac anomaly in which the Ao arises entirely or in largepart from the RV, and PA from LV (atrioventricularpart from the RV, and PA from LV (atrioventricularconcordant connection and ventriculoarterial discordantconcordant connection and ventriculoarterial discordantconnection)connection)

Simple TGA in neonate :Simple TGA in neonate :

arterial switch operation within 1 moarterial switch operation within 1 mo

Simple TGA beyond 30 days :Simple TGA beyond 30 days :

rapid tworapid two--stage operationstage operation

atrial switch operation (Mustard, Senning)atrial switch operation (Mustard, Senning) TGA with VSD :TGA with VSD :

arterial switch operation as earlyarterial switch operation as early

TGA with VSD and LVOTOTGA with VSD and LVOTO

Lecompte operation at 6Lecompte operation at 6--18 mo18 mo

--

Tricuspid AtresiaTricuspid Atresia


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Tricuspid AtresiaTricuspid Atresia

A cardiac anomaly in which RV fails to open into aA cardiac anomaly in which RV fails to open into aventricle through a AVvalve. There is thus aventricle through a AVvalve. There is thus a

univentricular AVconnectionuniventricular AVconnection

PVR is an important indicatorPVR is an important indicator

> 4 unit> 4 unit ---- contraindicatoncontraindicaton22--4 unit4 unit ---- BCPSBCPS

< 2 unit< 2 unit ---- Fontan operationFontan operation

Symptomatic in early lifeSymptomatic in early life

early shunt or PABearly shunt or PABBCPS or hemiBCPS or hemi--Fontan at 6Fontan at 6--12 mo12 mo

Fontan at 12Fontan at 12--24 mo24 mo

NonsymptomaticNonsymptomatic

Fontan candidate : 12Fontan candidate : 12--30 mo30 mo

Interrupted Aortic ArchInterrupted Aortic Arch


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Interrupted Aortic ArchInterrupted Aortic Arch

Complete luminal and anatomic discontinuity betweenComplete luminal and anatomic discontinuity betweentwo segments of the aortic arch, and generalizedtwo segments of the aortic arch, and generalizednarrowing of LVOT, posterior malalignment, muscle ofnarrowing of LVOT, posterior malalignment, muscle ofMoulaert, small aortic annulus, aortic hypoplasiaMoulaert, small aortic annulus, aortic hypoplasia

Dx is an indication of operationDx is an indication of operation Coexisting cardiac anomaly : not contraindicationCoexisting cardiac anomaly : not contraindication

OneOne--stage repair : preferredstage repair : preferred

TwoTwo--stage repair : in complicated intracardiacstage repair : in complicated intracardiacanomaliesanomalies

Single ventricle associated : alternative planSingle ventricle associated : alternative plan

Hypoplastic Left Heart SyndromeHypoplastic Left Heart Syndrome


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Hypoplastic Left Heart SyndromeHypoplastic Left Heart Syndrome

A wide spectrum of cardiac anomaly with various degreeA wide spectrum of cardiac anomaly with various degreeof hypoplasia of the structure of the left side of the heartof hypoplasia of the structure of the left side of the heart

Dx is an indication of operationDx is an indication of operation First stage palliation : 1First stage palliation : 1--30days30days

Norwood operationNorwood operation Second stage palliation : 6Second stage palliation : 6--12 mo12 mo

BCPSBCPS

HemiHemi--FontanFontan Third stage correction : 18Third stage correction : 18--24 mo24 mo

Completion FontanCompletion Fontan Cardiac transplantation :Cardiac transplantation :

Aortic diameter < 2.5 mmAortic diameter < 2.5 mm

Truncus ArteriosusTruncus Arteriosus


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A cardiac anomaly in which one great artery, arisingA cardiac anomaly in which one great artery, arisingfrom the base of the heart by way of a truncal valve,from the base of the heart by way of a truncal valve,PAs proximal to the origin of the brachiocephalicPAs proximal to the origin of the brachiocephalicbranches.branches.

Presence of truncus arteriosus : an absolutePresence of truncus arteriosus : an absolute

surgical indication.surgical indication.

Neonatal repair is recommended :Neonatal repair is recommended :

Homograft interpositionHomograft interposition

Prosthetic valve conduitProsthetic valve conduit

Autologous tissue reconstructionAutologous tissue reconstruction

Repair should be done before 6 moRepair should be done before 6 mo

Pulmonary vascular obstructive disease :Pulmonary vascular obstructive disease :

contraindicationcontraindication

Congenitally Corrected TGACongenitally Corrected TGA


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Congenitally Corrected TGACongenitally Corrected TGA

A cardiac anomaly with ventriculoarterial discordantA cardiac anomaly with ventriculoarterial discordantconnection & atrioventricular discordant connection. Theconnection & atrioventricular discordant connection. The

circulatory pathways are therefore in seriescirculatory pathways are therefore in series

The presence of CCTGA per se is not an indication.The presence of CCTGA per se is not an indication. With VSD : indications for VSDWith VSD : indications for VSD

With VSD + PS : indications for TOFWith VSD + PS : indications for TOF

Isolated TR : indication for MRIsolated TR : indication for MR

With complete heart block : pacingWith complete heart block : pacing

Fontan procedure indicated :Fontan procedure indicated :

straddling tricuspid valvestraddling tricuspid valve

complete AVSDcomplete AVSD

left sided tricuspid valve incompetenceleft sided tricuspid valve incompetence

Coronary Arteriovenous FistulaCoronary Arteriovenous Fistula


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yy

Direct communication between coronary artery and lumen ofDirect communication between coronary artery and lumen ofany one of cardiac chamber, or coronary sinus, or itsany one of cardiac chamber, or coronary sinus, or itstributary veins, or SVC, PAs, or Pulmonary veins close to thetributary veins, or SVC, PAs, or Pulmonary veins close to theheartheart

Indicated unless the shunt is small (Qp/Qs


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Eisenmenger: TreatmentEisenmenger: Treatment

Sxs +polycythemiaSxs +polycythemia phlebotomyphlebotomy Careful if microcytosis, strongest predictor of cerebrovascularCareful if microcytosis, strongest predictor of cerebrovascular

eventsevents

RULE OUT CORRECTABLE DISEASERULE OUT CORRECTABLE DISEASE

Once diagnosis established, avoid aggressive testing asOnce diagnosis established, avoid aggressive testing asmany patients die during cardiovascular proceduresmany patients die during cardiovascular procedures

Diuretics prn, oxygenDiuretics prn, oxygen

Definitive: Heart Lung transplantDefinitive: Heart Lung transplant Prostacyclin therapy may delay, expensiveProstacyclin therapy may delay, expensive

Cardiac TransplantationCardiac Transplantation


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pp

IndicationsIndicationsCardiac diseases that has a poor prognosis for shortCardiac diseases that has a poor prognosis for short--termterm

survival(8 Wood Units)

Immunologic status of the recipient panelImmunologic status of the recipient panel--reactive antibodyreactive antibody

(PRA) > 25%(PRA) > 25%

Questions?Questions?


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Questions?Questions?

Congestive Cardiac FailureCongestive Cardiac Failure


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Congestive Cardiac FailureCongestive Cardiac Failure

Enlarged heartEnlarged heart

Plethoric lung fieldsPlethoric lung fieldsspecially at basesspecially at bases



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PediatricPediatric

murmurmurmur

Adult (unrepaired with ASD)Adult (unrepaired with ASD)

atrial arrhythmiasatrial arrhythmias murmurmurmur

cyanosiscyanosis

RRL shunt NOT due to PulmHTN but TR jet directed across ASDL shunt NOT due to PulmHTN but TR jet directed across ASD

exercise intoleranceexercise intolerance Surgery in pts with significant TR/sxsSurgery in pts with significant TR/sxs

Eisenmengers SyndromeEisenmengers Syndrome


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Eisenmenger s SyndromeEisenmenger s Syndrome

Final common pathway for all significant LFinal common pathway for all significant LRRshunting in which unrestricted pulmonary bloodshunting in which unrestricted pulmonary bloodflow leads to pulmonary vasoflow leads to pulmonary vaso--occlusive diseaseocclusive disease

(PVOD); R(PVOD); RL shunting/cyanosis devleopsL shunting/cyanosis devleops Generally need Qp:Qs >2:1Generally need Qp:Qs >2:1



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Defined:Defined: Failure of the duct between pulmonary arteryFailure of the duct between pulmonary artery

and aorta to closeand aorta to close





BB-- infants, machineinfants, machine--like, continuous murmurlike, continuous murmur


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Defined:Defined: Failure of the duct between pulmonary arteryFailure of the duct between pulmonary artery

and aorta to closeand aorta to close







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Vascular RingVascular Ring


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Anomalies of the great arteries that compress theAnomalies of the great arteries that compress thetrachea or esophagustrachea or esophagus

Double aortic archDouble aortic arch

Rt aortic arch with retroesophageal anomalous Lt.Rt aortic arch with retroesophageal anomalous Lt.

SCA and ligamentum arteriosumSCA and ligamentum arteriosum

Rt aortic arch with retroesophageal ligamentumRt aortic arch with retroesophageal ligamentum

arteriosumarteriosum

Operation is indicated when : Obstructive Sx andOperation is indicated when : Obstructive Sx and

radiologic signs of obstructionradiologic signs of obstruction

Division by thoracotomyDivision by thoracotomy

CPB in associated cardiac anomalyCPB in associated cardiac anomaly

Vascular SlingVascular Sling


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Lt. pulmonary artery arises anomalously from RtLt. pulmonary artery arises anomalously from Rtpulmonary artery extrapericardially, form a slingpulmonary artery extrapericardially, form a slingaround tracheaaround trachea

Sx & Signs of obstruction : indicatedSx & Signs of obstruction : indicated

Tracheal anomaly or other airway problems (+)Tracheal anomaly or other airway problems (+)

(ASD, VSD, PDA, Lt SVC)(ASD, VSD, PDA, Lt SVC) Thoracotomy without CPBThoracotomy without CPB

Median sternotomy with CPBMedian sternotomy with CPB

ASD and PAPVRASD and PAPVR


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A hole of variable size in the atrial septum and isA hole of variable size in the atrial septum and is

most common cardiac malformation with variousmost common cardiac malformation with various

location of defect, fossa ovalis, posterior, ostium,location of defect, fossa ovalis, posterior, ostium,

primum, coronary sinus, subcaval (sinus venosus)primum, coronary sinus, subcaval (sinus venosus)

Uncomplicated ASD or of PAPVC with RVvolumeUncomplicated ASD or of PAPVC with RVvolume

overload (Qp/Qs>1.5 or 2.0) : an indicationoverload (Qp/Qs>1.5 or 2.0) : an indication

Scimitar syndromeScimitar syndrome Isolated PAPVCIsolated PAPVC

Optimal age : under 5 years but recently 1Optimal age : under 5 years but recently 1--2 years2 yearsto avoid RVvolume overloadto avoid RVvolume overload

Unroofed Coronary Sinus SyndromeUnroofed Coronary Sinus Syndrome


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A spectrum of cardiac anomalies in which part or all of theA spectrum of cardiac anomalies in which part or all of the

common wall between the coronary sinus and left atrium iscommon wall between the coronary sinus and left atrium is

absentabsent

Operation is advisable with diagnosis whenOperation is advisable with diagnosis when

With persistent Lt SVCWith persistent Lt SVC

Arterial desaturationArterial desaturation

Risk of cerebral embolismRisk of cerebral embolism

Good results of operationGood results of operation

Without persistent Lt SVC(Coronary sinus ASD)Without persistent Lt SVC(Coronary sinus ASD)

Same as for other type ASDSame as for other type ASD

Associated with other major cardiac anomaliesAssociated with other major cardiac anomalies

Clear indication for operationClear indication for operation

Congenital MS and MRCongenital MS and MR


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A developmental malformation of one or more of theA developmental malformation of one or more of thecomponents of MVapparatus, including LA wallcomponents of MVapparatus, including LA walladjacent insufficiency or a combined lesionadjacent insufficiency or a combined lesion

InfancyInfancy Mild and moderate Sx without MVR :Mild and moderate Sx without MVR :

Supravalvular ring, commmissural fusionSupravalvular ring, commmissural fusion

Other circumstances : only for infants with heartOther circumstances : only for infants with heart

failurefailure ChildhoodChildhood

Considerations for operation are similar CHFConsiderations for operation are similar CHF

Severe pulmonary HTSevere pulmonary HT

MVR shouldMVR should be withheld whenever ossiblebe withheld whenever ossible

PA with Intact Ventricular septumPA with Intact Ventricular septum


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A cardiac anomaly in which the pulmonary valve isA cardiac anomaly in which the pulmonary valve isatretic, coexisting with variable degree of right ventricleatretic, coexisting with variable degree of right ventricleand tricuspid valve hypoplasiaand tricuspid valve hypoplasia


Size of the TV : ZSize of the TV : Z--value of the tricuspid valvevalue of the tricuspid valve> --22 ------ RVOT patchRVOT patch

Evaluation after 6Evaluation after 6--12 mo after initial procedure :12 mo after initial procedure :Two ventricle repairTwo ventricle repair

One and half ventricle repairOne and half ventricle repair

F

ontan procedureF

ontan procedure

Double Outlet Right VentricleDouble Outlet Right Ventricle


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A congenital cardiac anomalies which both great arteriesA congenital cardiac anomalies which both great arteriesrise wholly or in large part from the RV. It is, then, a typerise wholly or in large part from the RV. It is, then, a type

of ventriculoarterial connection.of ventriculoarterial connection.


Simple DORVwith subaortic VSD : repair by 6 moSimple DORVwith subaortic VSD : repair by 6 mo

with PSwith PS ------ repair like TOFrepair like TOF

DORVwith subpulmonic VSD (TaussigDORVwith subpulmonic VSD (Taussig--Bing heart) :Bing heart) :

arterial switch operation within 1 moarterial switch operation within 1 mo

with PSwith PS ------ REVor Rastelli operation at 3REVor Rastelli operation at 3--5 years5 years

DORVwith noncommitted VSD :DORVwith noncommitted VSD :

VSD enlargement and intraventricular tunnelVSD enlargement and intraventricular tunnel

Fontan operationFontan operation


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Possible Exam QuestionPossible Exam Question


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A termA term babybabyis sent home with his mother after delivery.is sent home with his mother after delivery.The pregnancy was normal. No anomalies were noted at theThe pregnancy was normal. No anomalies were noted at thetime of birthtime of birth. Five weeks later. Five weeks later, the mother brings the baby, the mother brings the babyto the clinic because the baby has difficulty breathing andto the clinic because the baby has difficulty breathing andoccasionallyoccasionallyturns blueturns blue. You hear a. You hear a pansystolic murmurpansystolic murmur

which is probably due to a(an):which is probably due to a(an):

A. Hypertrophic subaortic stenosisA. Hypertrophic subaortic stenosis

B. Hypoplastic left heart syndromeB. Hypoplastic left heart syndrome

C. Coarctation of the aortaC. Coarctation of the aorta

D. Ventricular septal defectD. Ventricular septal defect

E. Bicuspid aortic valveE. Bicuspid aortic valve

Possible Exam Question


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Atermbaby is sent home with his mother afterdelivery. The pregnancy was normal. No anomalies

were noted at the time of birth. Five weeks later, themother brings the baby to the clinic because the babyhas difficulty breathing and occasionally turns blue.

You hear apansystolic murmurwhich is probably dueto a(an):

A. Hypertrophic subaortic stenosis

B. Hypoplastic left heart syndrome

C. Coarctation of the aortaD. Ventricular septal defect

E. Bicuspid aortic valve



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A 58A 58--yearyear--old man develops deep venous thrombosis during aold man develops deep venous thrombosis during ahospitalization for prostatectomy. He exhibits decreased mental statushospitalization for prostatectomy. He exhibits decreased mental status

with right hemiplegia, and a CT scan of the head suggests an acutewith right hemiplegia, and a CT scan of the head suggests an acutecerebral infarction in the distribution of the left middle cerebral artery.cerebral infarction in the distribution of the left middle cerebral artery.

A chest radiograph reveals cardiacA chest radiograph reveals cardiac enlargement and prominence of theenlargement and prominence of the

main pulmonary arteries that suggestsmain pulmonary arteries that suggests pulmonary hypertensionpulmonary hypertension. His. Hisserum troponin I is


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A58-year-old man develops deep venous thrombosis during ahospitalization for prostatectomy. He exhibits decreased mental

status with right hemiplegia, and a CT scan of the head suggestsan acute cerebral infarction in the distribution of the left middlecerebral artery.A chest radiograph reveals cardiac enlargementand prominence of the main pulmonary arteries that suggests

pulmonary hypertension. His serum troponin I is


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Defined:Defined:A bulging of one or both mitral valve leaflets into the leftA bulging of one or both mitral valve leaflets into the leftatrium during systoleatrium during systole

TT-- late systoliclate systolic

LL-- apexapex CC-- midsystolic clickmidsystolic click

AA-- ~5% normal population, asymptomatic, sudden~5% normal population, asymptomatic, suddendeathdeath

BB-- midsystolic click, most common valvular lesion,midsystolic click, most common valvular lesion,balloning/floppy valve, Marfans syndromeballoning/floppy valve, Marfans syndrome



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A 19A 19--yearyear--old woman is found to have a cardiac murmurold woman is found to have a cardiac murmurcharacterized by acharacterized by a mid systolic clickmid systolic click. An echocardiogram. An echocardiogramdemonstratesdemonstrates mitral insufficiencymitral insufficiencywith upward displacement of onewith upward displacement of oneleaflet. There isleaflet. There is aortic root dilationaortic root dilation to 4 cm. She has a dislocated rightto 4 cm. She has a dislocated rightocular crystalline lens. She dies suddenly and unexpectedly. Theocular crystalline lens. She dies suddenly and unexpectedly. The

medical examiner finds amedical examiner finds a prolapsed mitral valveprolapsed mitral valvewithwith elongation,elongation,thinning, and rupture of chordae tendineaethinning, and rupture of chordae tendineae. A mutation involving. A mutation involvingwhich of the following genes is most likely to be present in thiswhich of the following genes is most likely to be present in thispatient:patient:

A.A. BetaBeta--myosinmyosin

B.B.

CFTRCFTRC.C. FGFRFGFR

D.D. FibrillinFibrillin

E.E. SpectrinSpectrin



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A19-year-old woman is found to have a cardiac murmurcharacterized by a mid systolic click.An echocardiogram

demonstrates mitral insufficiency with upward displacementof one leaflet. There is aortic root dilation to 4 cm. She has adislocated right ocular crystalline lens. She dies suddenly andunexpectedly. The medical examiner finds aprolapsed mitral

valve with elongation, thinning, and rupture of chordaetendineae.Amutation involving which of the following genesis most likely to be present in this patient:

A. Beta-myosin

B. CFTR

C. FGFR

D. Fibrillin

E. Spectrin

Aortic RegurgitationAortic Regurgitation


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Defined:Defined: Retrograde flow from the aorta into the leftRetrograde flow from the aorta into the leftventricle through incompetent aortic cuspsventricle through incompetent aortic cusps

TT-- DiastolicDiastolic

LL-- 22ndnd

--44thth

left interspacesleft interspaces CC-- highhigh--pitched, blowingpitched, blowing

AA-- aortic root degeneration, rheumatic heartaortic root degeneration, rheumatic heartdisease, VSD w/aortic valve prolapse (kids)disease, VSD w/aortic valve prolapse (kids)

BB-- high pulse pressure = bounding pulseshigh pulse pressure = bounding pulses



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A 67A 67--yearyear--old man presents to his doctors office for anold man presents to his doctors office for aninsurance physical. During blood pressure measurement, theinsurance physical. During blood pressure measurement, thenurse notes that systolic sounds are heard with the cuffnurse notes that systolic sounds are heard with the cuffcompletely deflated. Thecompletely deflated. The blood pressure is 180/60 mmblood pressure is 180/60 mm Hg.Hg.Physical examination reveals bounding pulses and aPhysical examination reveals bounding pulses and a highhigh--pitched,pitched,

blowing diastolic murmurblowing diastolic murmur, heard best along the, heard best along the left sternalleft sternalborderborder. Which of the following are the most likely diagnosis?. Which of the following are the most likely diagnosis?

A. Aortic regurgitationA. Aortic regurgitation

B.B. Aortic valve obstructionAortic valve obstruction

C.C. Cardiac TamponadeCardiac TamponadeD.D. Heart failureHeart failure

E.E. HypovolemiaHypovolemia



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A67-year-old man presents to his doctors office for aninsurance physical. During blood pressure measurement,

the nurse notes that systolic sounds are heard with the cuffcompletely deflated. Theblood pressure is 180/60 mm Hg.Physical examination reveals bounding pulses and a high-

pitched, blowing diastolic murmur, heard best along theleft sternal border. Which of the following are the mostlikely diagnosis?

A.Aortic regurgitation

B. Aortic valve obstruction

C. Cardiac TamponadeD. Heart failure

E. Hypovolemia

Mitral StenosisMitral Stenosis


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Defined:Defined: Obstruction of flow from left atrium to left ventricleObstruction of flow from left atrium to left ventriclebecause of a narrowed mitral orificebecause of a narrowed mitral orifice

TT-- DiastolicDiastolic

LL--ApexApex CC-- opening snap, low pitchedopening snap, low pitched

AA-- Rheumatic feverRheumatic fever

BB-- hx of childhood rheumatic fever; presents withhx of childhood rheumatic fever; presents with

progressive dyspnea, pulmonary edema, hempotysisprogressive dyspnea, pulmonary edema, hempotysis



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A 30 yearA 30 year--old Hispanic woman reports to her primary care physicianold Hispanic woman reports to her primary care physiciancomplaining ofcomplaining ofprogressive dyspneaprogressive dyspnea. She reports she has a two. She reports she has a two--year historyyear historyof exertional shortness of breath that has now worsened and affects her evenof exertional shortness of breath that has now worsened and affects her evenwith modest amounts of activity. Occasionally, a dry cough accompanies herwith modest amounts of activity. Occasionally, a dry cough accompanies herdyspnea. Today, she had a mild episode ofdyspnea. Today, she had a mild episode ofhemoptysishemoptysis, which prompted her, which prompted hervisit. She denies fever, chills, or sputum production. Her medical history isvisit. She denies fever, chills, or sputum production. Her medical history is

significant for asignificant for a febrile illness with sore throat and joint pain at the age of 15febrile illness with sore throat and joint pain at the age of 15in Mexicoin Mexico. On examination, she is a well. On examination, she is a well--developed female in no acutedeveloped female in no acutedistress. Adistress. A crescendo diastolic rumble is present at the apex of the heartcrescendo diastolic rumble is present at the apex of the heart andandfinefine crackles are auscultated at the lung basescrackles are auscultated at the lung bases. Which of the following is the. Which of the following is themost likely diagnosis?most likely diagnosis?

A.A. Mitral stenosisMitral stenosis

B.B. Myocardial infarcionMyocardial infarcion

C.C. PneumoniaPneumonia

D.D. Pulmonary effusionPulmonary effusion

E.E. Pulmonary embolusPulmonary embolus



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A30 year-old Hispanic woman reports to her primary care physiciancomplaining ofprogressive dyspnea. She reports she has a two-year

history of exertional shortness of breath that has now worsened andaffects her even with modest amounts of activity. Occasionally, a drycough accompanies her dyspnea. Today, she had a mild episode ofhemoptysis, which prompted her visit. She denies fever, chills, or

sputum production. Her medical history is significant for a febrileillness with sore throat and joint pain at the age of 15 in Mexico. Onexamination, she is a well-developed female in no acute distress. Acrescendo diastolic rumble is present at the apex of the heart and finecrackles are auscultated at the lung bases. Which of the following isthe most likely diagnosis?

A. Mitral stenosis

B. Myocardial infarcion

C. Pneumonia

D. Pulmonary effusion

E. Pulmonary embolus



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Defined:Defined: Failure of the duct between pulmonary arteryFailure of the duct between pulmonary arteryand aorta to closeand aorta to close


LL-- upper left sternal borderupper left sternal border CC-- machinemachine--likelike





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An xAn x--ray performed on a newborn infant shows enlargementray performed on a newborn infant shows enlargementof the left ventricle and left atrium as well as dilation of theof the left ventricle and left atrium as well as dilation of theaorta. Echocardiographic studies demonstrate volumeaorta. Echocardiographic studies demonstrate volume--overloading of the left ventricle. Cardiac auscultation revealsoverloading of the left ventricle. Cardiac auscultation revealsthe presence of a continuous, machinethe presence of a continuous, machine--like murmur. Which oflike murmur. Which of

the following is the most likely diagnosis?the following is the most likely diagnosis?

A.A. Atrial septal defectAtrial septal defect

B.B. Patent ductus arteriosusPatent ductus arteriosus

C.C. Pulmonic stenosisPulmonic stenosisD.D. Tetralogy of FallotTetralogy of Fallot

E.E. Ventricular septal defectVentricular septal defect



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An x-ray performed on a newborn infant showsenlargement of the left ventricle and left atrium as well

as dilation of the aorta. Echocardiographic studiesdemonstrate volume-overloading of the left ventricle.Cardiac auscultation reveals the presence of a continuous

murmur. Which of the following is the most likelydiagnosis?

A. Atrial septal defect

B. Patent ductus arteriosus

C. Pulmonic stenosis

D. Tetralogy of Fallot

E. Ventricular septal defect

Bonus QuestionBonus Question


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The infants murmur is most likely related to which of theThe infants murmur is most likely related to which of thefollowing:following:

A.A. Blood flowing across the aortic valveBlood flowing across the aortic valve

B.B. Blood flowing from the aorta to the pulmonary arteryBlood flowing from the aorta to the pulmonary artery

C.C. Blood flowing from the left ventricle to the right ventricleBlood flowing from the left ventricle to the right ventricle

D.D. Blood flowing from the pulmonary artery to the aortaBlood flowing from the pulmonary artery to the aorta

E.E. Blood flowing from the right ventricle to the left ventricleBlood flowing from the right ventricle to the left ventricle

Bonus Question


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The infants murmur is most likely related to which ofthe following:

A. Blood flowing across the aortic valve

B. Blood flowing from the aorta to the pulmonary artery

C. Blood flowing from the left ventricle to the rightventricle

D. Blood flowing from the pulmonary artery to the aorta

E. Blood flowing from the right ventricle to the left

ventricle

Congestive Heart FailureCongestive Heart Failure


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CauseCause EffectEffect LV output does notLV output does not w/exercisew/exercise DOEDOE

End Diastolic VolumeEnd Diastolic Volume Cardiac DilationCardiac Dilation

LV failureLV failure

pulmonary venous pressurepulmonary venous pressure

fluidfluidtransudationtransudation Pulmonary Edema, PNDPulmonary Edema, PND

venous return exacerbates pulmonary vascularvenous return exacerbates pulmonary vascular

congestioncongestion OrthopneaOrthopnea

Congestive Heart FailureCongestive Heart Failure


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central venous pressurecentral venous pressure resistance to portal flowresistance to portal flowHepatomegalyHepatomegaly(nutmeg liver)(nutmeg liver)

RV failureRV failure venous venouspressurepressure fluidfluidtransudationtransudationAnkle, sacralAnkle, sacral

edemaedema

Cardiac TumorsCardiac Tumors


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Most common tumor = MetastasisMost common tumor = MetastasisII

Most common 1Most common 1 adult tumor = Myxomaadult tumor = Myxoma

BallBall--valvevalve obstructionobstruction in Left Atrium (LAin Left Atrium (LA Dodgers)Dodgers)

Most common 1Most common 1 pediatric tumor =pediatric tumor =

rhabdomyomarhabdomyomaAssociated with tuberous sclerosisAssociated with tuberous sclerosis

Rheumatic FeverRheumatic Fever


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FFeverever EErythema marginatumrythema marginatum

VValvular damagealvular damage

EESRSR RReded--hot joints (polyarthritis)hot joints (polyarthritis)

SSubcutaneous nodulesubcutaneous nodules

SSt. Vitus Dance (chorea)t. Vitus Dance (chorea)

SStrep antibody titer (ASOtrep antibody titer (ASO ))

Aschoff BodiesAschoff Bodies Granulomas withGranulomas with

giant cellsgiant cells

Anitschkows cellsAnitschkows cells

ActivatedActivatedhistiocyteshistiocytes

TwoTwo RHRHussiansussians

withwith RHRHumaticumaticheart diseaseheart disease

Rheumatic FeverRheumatic Fever


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John Travolta careerJohn Travolta careerslumpslump

Makes sequel toMakes sequel to SaturdaySaturdayNightFeverNightFevercalledcalled RheumaticRheumaticFeverFever

John getsJohn gets FeverFever

MyocarditisMyocarditis

Joint swellingJoint swelling((PolyarthritisPolyarthritis))

ChoreaChorea (uncontrolled(uncontrolleddancedance--like movements oflike movements ofextremitiesextremities St. Vitus Dance



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A 42A 42--yearyear--old woman has increasing congestive heartold woman has increasing congestive heartfailure. As a child she sufferedfailure. As a child she suffered recurrent bouts ofrecurrent bouts ofpharyngitispharyngitiswithwith group A beta hemolytic streptococcalgroup A beta hemolytic streptococcalinfections. The cardiac valves most likely to beinfections. The cardiac valves most likely to be

affected are:affected are:A.A. Aortic and tricuspidAortic and tricuspid

B.B. Mitral and pulmonicMitral and pulmonic

C.C. Aortic and pulmonicAortic and pulmonic

D.D. Tricuspid and pulmonicTricuspid and pulmonicE.E. Mitral and aorticMitral and aortic


A


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A42-year-old woman has increasing congestiveheart failure.As a child she suffered recurrent

bouts of pharyngitis with groupA beta hemolyticstreptococcal infections. The cardiac valves mostlikely to be affected are:

A. Aortic and tricuspid

B. Mitral and pulmonic

C. Aortic and pulmonic

D. Tricuspid and pulmonicE. Mitral and aortic

PericarditisPericarditis


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SSerouserous SSLE,LE,

Rhuematoid arthritisRhuematoid arthritis

UremiaUremia

Infection (Infection (seriousserious))

FibrinousFibrinous

MI (death of muscleMI (death of musclefibersfibers))

Rhuematic feverRhuematic fever

UremiaUremia

HemorrhagicHemorrhagic (Invasive!)(Invasive!) TB (thinkTB (thinkhemoptysishemoptysis))

Malignancy (aggressive)Malignancy (aggressive)

May resolve withoutMay resolve withoutscarringscarring

May progress to chronicMay progress to chronicadhesive or constrictiveadhesive or constrictivepericarditispericarditis

Syphilitic Heart DiseaseSyphilitic Heart Disease


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33 syphillissyphillis-- long standinglong standing Destruction ofDestruction ofvasa vasorumvasa vasorum

Dilation of aortic rootDilation of aortic rootwithout atherosclerotic lesionwithout atherosclerotic lesion

== syphilitic aneurysmsyphilitic aneurysm Calcification of ascendingCalcification of ascending

arch and aortic rootarch and aortic root

TreeTree--barkingbarking-- postpost--

inflammatory scarring of theinflammatory scarring of theaortaaorta



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Examination of an autopsy specimen from aExamination of an autopsy specimen from a MexicanMexicanimmigrantimmigrant demonstrates a heart with massivedemonstrates a heart with massive dilationdilationof the aortic rootof the aortic root and adjacent aortic arch. Openingand adjacent aortic arch. Openingthe aorta reveals a distinctivethe aorta reveals a distinctivewrinkling of the intimalwrinkling of the intimal

surfacesurface. If a histological section through the aortic. If a histological section through the aorticwall is made, which of the following will be seen?wall is made, which of the following will be seen?

A.A. A heavy eosinophilic infiltrateA heavy eosinophilic infiltrate

B.B. Fibrinoid necrosis with a neutrophilic infiltrationFibrinoid necrosis with a neutrophilic infiltration

C.C. Focal fragmentation of elastic elementsFocal fragmentation of elastic elementsD.D. Obliterative endarteritis of vasa vasorumObliterative endarteritis of vasa vasorum

E.E. RingRing--like calcification of the vessel medialike calcification of the vessel media



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Examination of an autopsy specimen from aMexican immigrant demonstrates a heart withmassive dilation of the aortic root and adjacentaortic arch. Opening the aorta reveals adistinctive wrinkling of the intimal surface. If a

histological section through the aortic wall ismade, which of the following will be seen?

A. A heavy eosinophilic infiltrate

B. Fibrinoid necrosis with a neutrophilic infiltration

C. Focal fragmentation of elastic elements

D. Obliterative endarteritis of vasa vasorum

E. Ring-like calcification of the vessel media

VSDVSD


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Defined:Defined:A congenital abnormality in which blood flows from highA congenital abnormality in which blood flows from highpressure LVpressure LV low pressure RV through a holelow pressure RV through a hole

TT-- holosystolicholosystolic

LL-- 33rdrd, 4, 4thth, 5, 5thth left interspacesleft interspaces

CC-

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Curs Cardiopatii ale Final 2011 2012

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