congenital heart lesions- right to left shunts

Congenital Heart Congenital Heart Lesions-Lesions-

Right to Left ShuntsRight to Left Shunts

Zeev Perles MDZeev Perles MD

Pediatric CardiologyPediatric Cardiology

Hadassah Jerusalem, 11/2013Hadassah Jerusalem, 11/2013

Congenital Heart Congenital Heart Lesions-Lesions-

Right to Left ShuntsRight to Left Shunts

R-L shunting = R-L shunting = blue bloodblue blood contaminating contaminating systemic cyclesystemic cycle

R-L shunting R-L shunting Cyanotic Cyanotic CHDCHD

??????

CyanosiCyanosiss

CyanosiCyanosiss

Kuanosis (Greek) “blueness”Kuanosis (Greek) “blueness” Deoxygenated capillary bloodDeoxygenated capillary blood Lundsgaard & Van Slyke (1923)Lundsgaard & Van Slyke (1923)

≥≥3-5 gram% of reduced Hgb3-5 gram% of reduced Hgb Central or PeripheralCentral or Peripheral Look at skin, mucosa, nailbedsLook at skin, mucosa, nailbeds

Cyanotic CHDCyanotic CHD

TTetralogy of Fallot (TOF)etralogy of Fallot (TOF)

TTransposition of the Great Arteries (TGA)ransposition of the Great Arteries (TGA)

TTricuspid valve atresia + ricuspid valve atresia +

Pulmonary aPulmonary aTTresia (with VSD or IVS)resia (with VSD or IVS)

TTotal anomalous pulmonary venous return otal anomalous pulmonary venous return

(TAPVR)(TAPVR)

TTruncus arteriosusruncus arteriosus

EbsEbsTTeinein’’s Anomaly of the s Anomaly of the TTricuspid Valvericuspid Valve

R-L shunting = R-L shunting = Cyanotic CHD !!!Cyanotic CHD !!!

Qp/QS ?Qp/QS ?

100 100 10060 60 60

90 80 70

♥ Qp/Qs << 1Qp/Qs << 1♥ Severe desaturation/cyanosis (RA Severe desaturation/cyanosis (RA

SiO2 60-75%)SiO2 60-75%)♥ Oligemic CXROligemic CXR♥ Normal respiratory statusNormal respiratory status♥ Normal growthNormal growth♥ Need: Need: BT ShuntBT Shunt RV-PA conduit + RV-PA conduit +

VSDVSD

♥ Qp/Qs >> 1Qp/Qs >> 1♥ Mild/No desaturation (RA SiO2 85-Mild/No desaturation (RA SiO2 85-

99%)99%)♥ CXR CXR –– increased PVM increased PVM’’ss♥ Tachypneic and dyspneicTachypneic and dyspneic♥ FTT !!!FTT !!!♥ Need: Need: RV-PA conduit + VSDRV-PA conduit + VSD

Cyanotic CHDCyanotic CHD

Take-home messageTake-home message::Some Some CYANOTICCYANOTIC CHDs are not CHDs are not necessarily bluenecessarily blue

The degree of “blueness” The degree of “blueness” depends on Qp/Qs ratiodepends on Qp/Qs ratio

ונעבור לנציגנו במיון ילדים...ונעבור לנציגנו במיון ילדים...

Can You Guess Can You Guess –– O O22SS? ?

OO22SS= = OO22SS= =


OO22S = 80%S = 80%OO22S = S = 80%80%!!! !!!


OO22S = 80%S = 80%OO22S = 80%S = 80%!!! !!!

Total Hb- 10g% Total Hb- 10g% Deox Hb=10*20%=Deox Hb=10*20%=2 g2 g%%

Total Hb- 20g% Total Hb- 20g% Deox Hb=20*20%=Deox Hb=20*20%=4 g4 g%%

Can You Guess Can You Guess –– Hgb g Hgb g? %? %


All- All- 75%75%!!! !!!


All- All- 75%75%!!! !!!

HgbHgb??? ???


All- All- 75%75%!!! !!!

HgbHgb??? ???

9g9g!!! %!!! %

21g21g!!! %!!! %

18g18g!!! %!!! %


Take-home messageTake-home message::A child with A child with CYANOTICCYANOTIC CHD is CHD is not necessarily bluenot necessarily blue

The degree of “blueness” The degree of “blueness” depends on hemoglobin leveldepends on hemoglobin level

Breathing PatternsBreathing Patterns

Breathing PatternsBreathing Patterns

Large VSD+PDA

Tricuspid Atresia (Qp/QS

1.3)


Truncus Arteriosus

Tricuspid Atresia

Medical HistoryMedical History

Large VSD

Tetralogy of

Fallot

Cyanotic CHD- Cyanotic CHD- Pulmonary flow obstruction Pulmonary flow obstruction

with R-L Shuntingwith R-L Shunting

TTetralogy of Fallot (TOF)etralogy of Fallot (TOF)

TTricuspid valve atresia (with PS)ricuspid valve atresia (with PS)

Pulmonary aPulmonary aTTresia (with VSD or resia (with VSD or

IVS)IVS)

Classic Cyanotic Heart Classic Cyanotic Heart lesionslesions



After birth- closure of arterial After birth- closure of arterial

duct:duct:

Baby dies!!!!Baby dies!!!!



After birth- closure of arterial After birth- closure of arterial

duct:duct:

Baby dies!!!!Baby dies!!!!

iv PGEiv PGE

Blue Baby SyndromeBlue Baby Syndrome

Helen B. Taussig 1898-Helen B. Taussig 1898-19861986

♥Orphan- age 11Orphan- age 11

♥XXXX

♥Severe DyslexiaSevere Dyslexia

♥DeafnessDeafness

Founder of Pediatric Founder of Pediatric CardiologyCardiology

TOF TOF Natural Natural historyhistory

ACQUIRED ACQUIRED CONDITIONSCONDITIONS

Brain abscessesBrain abscesses– most commonly with TOFmost commonly with TOF– rarely before 2 years of agerarely before 2 years of age– headaches, fever, seizures, or headaches, fever, seizures, or

neurologic defecitsneurologic defecitsCerebrovascular accidentsCerebrovascular accidentsInfectious endocarditisInfectious endocarditisMay be pre-disposed to otitis mediaMay be pre-disposed to otitis media

Blue Baby SyndromeBlue Baby Syndrome

Physical Exam in Tetralogy of Physical Exam in Tetralogy of FallotFallot

S2 single S2 single

S1S1

ECEC

Systolic Systolic murmur of murmur of PSPS

1)1) Cyanosis - degree depends on Cyanosis - degree depends on PSPS

2)2) Clubbing (>6m)Clubbing (>6m)

3)3) Normal pulsesNormal pulses

4)4) Increased RV impulse at RSBIncreased RV impulse at RSB

5)5) SS2SS2

6)6) Murmur along LSBMurmur along LSB

CXRCXR Heart size enlargedHeart size enlarged Small RVOT Small RVOT

segment+ large RV-segment+ large RV- Coeur en sabot Coeur en sabot or or boot shaped heartboot shaped heart

May see right sided May see right sided aortic arch aortic arch

Pulmonary Pulmonary vasculature vasculature decreased (oligemic)decreased (oligemic)

Anatomy/Anatomy/PhysiologyPhysiology

Edwards, JE: Congenital Heart Disease. WB Saunders and Co. Edwards, JE: Congenital Heart Disease. WB Saunders and Co. 19651965

Anatomy/Anatomy/PhysiologyPhysiology

TOF managementTOF management

SquattingSquatting

SoothingSoothing

SedationSedation

SelineSeline

Slowing the rateSlowing the rate

SurgerySurgery

BLALOCK-TAUSSIG BLALOCK-TAUSSIG SHUNTSHUNT

11/07/200311/07/2003המלאך השחור של הילדים הכחולים, הארץ המלאך השחור של הילדים הכחולים, הארץ

((קרונזון יצחקקרונזון יצחק) )

נעשה בבית חולים בבולטימור ניתוח ניסיוני בתינוקת שסבלה ממומים מולדים בלבה. 1944בנובמבר הד"ר בליילוק, מומחה בעל שם עולמי, נעצר פתאום. "צריך לקרוא לויויאן תומאס", אמר לאחות.

.תומאס, עובד מעבדה שחור, שלא הורשה עד אז לעטות חלוק לבן, שינה את עולם הרפואה

CLASSIC BLALOCK-TAUSSIG CLASSIC BLALOCK-TAUSSIG SHUNTSHUNT

BLALOCK-TAUSSIG SHUNTBLALOCK-TAUSSIG SHUNT


Denton Cooley

MODIFIED BLALOCK-MODIFIED BLALOCK-TAUSSIG SHUNTTAUSSIG SHUNT

TOF - HISTORYTOF - HISTORY

Stenson 1671Stenson 1671 First descriptionFirst description

Fallot 1888Fallot 1888 Description of featuresDescription of features

Blalock and Taussig 1945Blalock and Taussig 1945 First systemic-to-pulmonary First systemic-to-pulmonary

anastamosisanastamosisLillehei 1955Lillehei 1955

First definitive surgical repairFirst definitive surgical repair

INCIDENCEINCIDENCE

Most common cyanotic cardiac Most common cyanotic cardiac

lesionlesion

Third most common cardiac lesionThird most common cardiac lesion

Occurs 3-5 times per 10,000 birthsOccurs 3-5 times per 10,000 births

5-10% of all cardiac defects5-10% of all cardiac defects

Anatomy/Anatomy/PathologyPathology

Ventricular septal defectVentricular septal defect

Pulmonary stenosisPulmonary stenosis

Aortic overrideAortic override

Right ventricular hypertrophyRight ventricular hypertrophy

Anatomy/Anatomy/PathologyPathology

Gross example of Tetrology of Fallot. The interior of the right ventricle is exposed showing a large VSD (D) and an aorta (A)straddling (ie over-riding) the VSD. A probe (arrow) is positioned inside of the stenotic subpulmonary infundibular channel.

Edwards, JE: Congenital Heart Disease. WB Saunders and Co. 1965

This is an example of tetralogy of Fallot opened sagittally. The aorta and its valve (AV) clearly straddle the VSD (D) such that the aorta appears to arise equally from each ventricle. The right ventricular (RV) is clearly hypertrophic as it is nearly as thick as left ventricle (LV).

Edwards, JE: Congenital Heart Disease. WB Saunders and Co. 1965

ASSOCIATED ANOMALIESASSOCIATED ANOMALIES

Pulmonary arteries:Pulmonary arteries:– Valvar, main, branch PS Valvar, main, branch PS – Discontinuous PA’sDiscontinuous PA’s

Atrial septal defect (up to 80%)Atrial septal defect (up to 80%)Additional VSD’sAdditional VSD’sConotruncal defects-Conotruncal defects-

– Right sided aortic arch (25%)Right sided aortic arch (25%)– Aortic branch anomaliesAortic branch anomalies– LSVCLSVC

PDAPDA

CORONARY ANOMALIESCORONARY ANOMALIES

Present in 2-9% of patientsPresent in 2-9% of patientsSingle right or left coronary arterySingle right or left coronary arteryLAD from right coronary arteryLAD from right coronary arteryAccessory LAD from right coronary Accessory LAD from right coronary arteryartery

Pre-operative identification Pre-operative identification importantimportant

CYANOSISCYANOSIS

Two-thirds acyanotic at birthTwo-thirds acyanotic at birth75-90% have cyanosis within 6 75-90% have cyanosis within 6 monthsmonths

CYANOSISCYANOSIS

Two-thirds acyanotic at birthTwo-thirds acyanotic at birth75-90% have cyanosis within 6 months75-90% have cyanosis within 6 months

Progressive Progressive disease!!!disease!!!

ACQUIRED ACQUIRED CONDITIONSCONDITIONS

Brain abscessesBrain abscesses– most commonly with TOFmost commonly with TOF– rarely before 2 years of agerarely before 2 years of age– headaches, fever, seizures, or headaches, fever, seizures, or

neurologic defecitsneurologic defecitsCerebrovascular accidentsCerebrovascular accidentsInfectious endocarditisInfectious endocarditisMay be pre-disposed to otitis mediaMay be pre-disposed to otitis media

PHYSICAL EXAMPHYSICAL EXAM

Vital signs normalVital signs normal Normal ventricular impulsesNormal ventricular impulses First heart sound normalFirst heart sound normal Second heart sound usually Second heart sound usually

singlesingle Ejection click may be heardEjection click may be heard Normal pulsesNormal pulses Clubbing after 6 months of ageClubbing after 6 months of age

MURMURSMURMURS

Systolic murmur at upper-left sternal borderSystolic murmur at upper-left sternal border– varies in intensityvaries in intensity– turbulent flow across right-ventricular outflow turbulent flow across right-ventricular outflow

tracttract– typically typically crescendo-decrescendocrescendo-decrescendo– may be difficult to distinguish from VSD murmurmay be difficult to distinguish from VSD murmur

Continuous murmurs uncommonContinuous murmurs uncommon– ductus arteriosusductus arteriosus– pulmonary collateralspulmonary collaterals

Diastolic murmurs rareDiastolic murmurs rare

Physical Exam in Tetralogy of Physical Exam in Tetralogy of FallotFallot

S2 single

S1

EC

Systolic murmur of PS

1) Cyanosis - degree depends on PS

2) Normal pulses

3) Increased RV impulse at RSB

4) Murmur along LSB

CXRCXR Heart size enlargedHeart size enlarged Small RVOT Small RVOT

segment+ large RV-segment+ large RV- Coeur en sabot Coeur en sabot or or boot shaped heartboot shaped heart

May see right sided May see right sided aortic arch aortic arch

Pulmonary Pulmonary vasculature vasculature decreased (oligemic)decreased (oligemic)

ELECTROCARDIOGRAMELECTROCARDIOGRAM

RVHRVH Right axis deviationRight axis deviation Difficult to discern Difficult to discern

in neonatein neonate

Evident by 3 monthsEvident by 3 months RAE rare in childrenRAE rare in children LAD with AV canalLAD with AV canal

ECHOCARDIOGRAPHYECHOCARDIOGRAPHY

CARDIAC CARDIAC CATHETERIZATIONCATHETERIZATION

Usually not Usually not neededneeded

TOF managementTOF managementMedicalMedical::

–Cyanotic spellsCyanotic spells

TET SPELLSTET SPELLS

Increased cyanosis, abnormal Increased cyanosis, abnormal respirationsrespirations

Lethargy or unconsciousnessLethargy or unconsciousnessRapidly developing metabolic acidosisRapidly developing metabolic acidosisMore common in the morningMore common in the morningMay be precipitated by feeding, May be precipitated by feeding, crying, having a bowel movementcrying, having a bowel movement

TET SPELLSTET SPELLS

Incidence peaks between 3 months and 5 yearsIncidence peaks between 3 months and 5 years– uncommon in neonatal perioduncommon in neonatal period

– rare after 5 years of agerare after 5 years of age

Increased right-to-left shuntingIncreased right-to-left shunting Decrease in intensity of systolic pulmonary Decrease in intensity of systolic pulmonary

outflow tract murmuroutflow tract murmur Usually self-limited, but may be life-threateningUsually self-limited, but may be life-threatening

TET SPELLS-TET SPELLS-MECHANISMMECHANISM

Exact cause unknownExact cause unknown May be infundibular spasmMay be infundibular spasm

– may be primary or induced by catecholaminesmay be primary or induced by catecholamines– doesndoesn’’t explain occurrence with pulmonary t explain occurrence with pulmonary

atresiaatresia Tachypnea may be primary causeTachypnea may be primary cause

– increased systemic venous returnincreased systemic venous return– work of breathing increases oxygen work of breathing increases oxygen

consumptionconsumption– doesndoesn’’t explain decrease in murmurt explain decrease in murmur

Rarely, precipitated by SVTRarely, precipitated by SVT

TET SPELLS-TREATMENTTET SPELLS-TREATMENT

Calm and comfort childCalm and comfort child OxygenOxygen Knee-chest positionKnee-chest position

– compresses femoral arteriescompresses femoral arteries– increases blood return from the increases blood return from the

legslegs MorphineMorphine

– may directly relax infundibulummay directly relax infundibulum– may act at the level of the CNSmay act at the level of the CNS

TET SPELLS-TREATMENTTET SPELLS-TREATMENT

Knee-chest position- SquattingKnee-chest position- Squatting

TET SPELLS-TET SPELLS-TREATMENTTREATMENT

Intravenous fluidsIntravenous fluidsBicarbonateBicarbonateBeta-blocker (propranalol)Beta-blocker (propranalol)Vasoconstrictor (phenylephrine)Vasoconstrictor (phenylephrine)External compression of External compression of abdominal aortaabdominal aorta

General anesthesiaGeneral anesthesia

SURGICAL OPTIONSSURGICAL OPTIONS

Palliation followed by complete repairPalliation followed by complete repair– pulmonary-to-systemic anastamosis (shunt)pulmonary-to-systemic anastamosis (shunt)– modified Blalock-Taussig most commonly modified Blalock-Taussig most commonly

usedusedPrimary complete repairPrimary complete repair

– may not be possible because may not be possible because size of patientsize of patientsmall caliber of pulmonary arteriessmall caliber of pulmonary arteriescoronary artery anomaliescoronary artery anomalies

TOTAL REPAIRTOTAL REPAIR

Closure of ventricular septal defectClosure of ventricular septal defect– transatrial or transverse ventricular incisiontransatrial or transverse ventricular incision– patch of synthetic material (Dacron or Teflon)patch of synthetic material (Dacron or Teflon)– avoid damaging conduction system avoid damaging conduction system

(perimembranous)(perimembranous)

Relief of right ventricular outflow tract Relief of right ventricular outflow tract obstructionobstruction

– resection of infundibular tissueresection of infundibular tissue– may need a transannular patch or valved conduitmay need a transannular patch or valved conduit

TOF managementTOF management

VSD closureVSD closure

TOF managementTOF managementRVOT reliefRVOT relief

TRANSANNULAR TRANSANNULAR PATCHPATCH

Infundibulum short Infundibulum short and hypoplasticand hypoplastic

Simple resection of Simple resection of tissue does not tissue does not relieve obstructionrelieve obstruction

Can be extended to Can be extended to pulmonary arteriespulmonary arteries

Causes post-Causes post-operative pulmonary operative pulmonary regurgitationregurgitation

TOF TOF –– Postop MRI Postop MRI

VALVED CONDUITVALVED CONDUIT

Used when Used when pulmonary pulmonary regurgitation will be regurgitation will be poorly toleratedpoorly tolerated

– Pulmonary Pulmonary hypertensionhypertension

– Distal PA stenosisDistal PA stenosis Dacron conduitDacron conduit Porcine or human Porcine or human

valvevalve

TOF- Late TOF- Late ComplicationsComplications Lancet. 2000 Sep 16;356(9234):975-81Lancet. 2000 Sep 16;356(9234):975-81 Risk Risk

factors for arrhythmia and sudden cardiac death late factors for arrhythmia and sudden cardiac death late after repair of TOF: a multicentre study.after repair of TOF: a multicentre study.

793 repaired TOF patients793 repaired TOF patients mean age at repair 8.2 years mean time from repair mean age at repair 8.2 years mean time from repair

21.1 years [8.7])21.1 years [8.7]) 33 - sustained monomorphic VT33 - sustained monomorphic VT

16 - died suddenly 16 - died suddenly 29 - had new-onset sustained 29 - had new-onset sustained

atrial atrial flutter or fibrillationflutter or fibrillation

The Beginning………….The Beginning………….

Anatomy of Tetralogy of FallotAnatomy of Tetralogy of Fallot

congenital heart lesions- right to left shunts

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