CARDIACCARDIACPhysiologyPhysiology

Disease ProcessDisease ProcessPractice IssuesPractice Issues

ByBy

Nadine Bowers CNNPNadine Bowers CNNP

Presbyterian Healthcare ServicesPresbyterian Healthcare Services

SV

ObjectivesObjectives

Identify maternal factors which may Identify maternal factors which may predispose the infant to CHDpredispose the infant to CHD

Identify three ways in which CHD can present.Identify three ways in which CHD can present. Identify three diagnostic study to identify CHDIdentify three diagnostic study to identify CHD Is able to identify one left ventricular outflow Is able to identify one left ventricular outflow

defectdefect Is able to verbalize the dangers of central line Is able to verbalize the dangers of central line

monitoringmonitoring Is able to identify one cause for sinus Is able to identify one cause for sinus

bradycardiabradycardia

ObjectivesObjectives Is able to identify one (L->R) defectIs able to identify one (L->R) defect Is able to identify one (R->L) defectIs able to identify one (R->L) defect Is able to identify one right ventricular Is able to identify one right ventricular

outflow defectoutflow defect Able to identify the drug utilized for Ductal Able to identify the drug utilized for Ductal

dependent lesionsdependent lesions Can verbalize signs and symptoms of PDACan verbalize signs and symptoms of PDA Is able to identify a common arrhythmiaIs able to identify a common arrhythmia Able to describe the correct placement of Able to describe the correct placement of

leads for monitoring of neonatesleads for monitoring of neonates

EMBRINOLOGYEMBRINOLOGY

The cardiovascular system is the first The cardiovascular system is the first system to function in the embryosystem to function in the embryo

Virtually the entire cardiac Virtually the entire cardiac development occurs between the development occurs between the third and seventh weekthird and seventh week

INCIDENCE OF CHDINCIDENCE OF CHD

Occurrence is 8-10/1000 live birthsOccurrence is 8-10/1000 live births This represents 10% of all congenital This represents 10% of all congenital

malformationsmalformations Occurrence increases with decreased Occurrence increases with decreased

birth weightsbirth weights 16% in < 2500 gram infants16% in < 2500 gram infants 75% in < 1200 gram infants75% in < 1200 gram infants

ETIOLOGYETIOLOGY 85%85% unknown unknown Cause most likely inherited predisposition Cause most likely inherited predisposition

and environmental predisposition at a and environmental predisposition at a critical period of fetal developmentcritical period of fetal development

Multifactorial inheritance. Multifactorial inheritance. 40% are associated with chromosomal 40% are associated with chromosomal

abnormalitiesabnormalities Teratogenic causes- maternal ingestionTeratogenic causes- maternal ingestion Viral causesViral causes Maternal conditionsMaternal conditions

CHROMOSOMALCHROMOSOMALABNORMALITIES with CHDABNORMALITIES with CHD

Trisomy 13,18Trisomy 13,18 Trisomy 21Trisomy 21 Turners SyndromeTurners Syndrome Marfans SyndromeMarfans Syndrome

DiGeorgeDiGeorge’’s s syndromesyndrome

VSD,PDA,ASD,TGAVSD,PDA,ASD,TGA ECD,VSD,PDAECD,VSD,PDA COACOA AS, MVS, aortic AS, MVS, aortic

aneurysmsaneurysms Interrupted aortic Interrupted aortic

archarch

MORTALITYMORTALITY

Dependent on the cardiac lesionDependent on the cardiac lesion 39% die in the first year of life when no 39% die in the first year of life when no

other associated anomalies are presentother associated anomalies are present 48% die with severe extra cardiac 48% die with severe extra cardiac

anomaliesanomalies Risk for occurrence in future siblings is 2-Risk for occurrence in future siblings is 2-

6%6% 1-10% CHD occurs in off spring born with 1-10% CHD occurs in off spring born with

CHDCHD

MANIFESTATIONS OF CHDMANIFESTATIONS OF CHD Central cyanosis- R->L shuntsCentral cyanosis- R->L shunts CHF- L->R shuntsCHF- L->R shunts Dysrhythmias-not common in the newbornDysrhythmias-not common in the newborn MurmursMurmurs Signs of poor cardiac output (poor pulses, Signs of poor cardiac output (poor pulses,

mottling, hypotension, metabolic acidosis, mottling, hypotension, metabolic acidosis, circulatory compromise) circulatory compromise)

Abnormal heart rhythm (tachycardia, Abnormal heart rhythm (tachycardia, bradycardia, heart block)bradycardia, heart block)

Abnormal heart shape, size, locationAbnormal heart shape, size, location

CARDIAC LESIONSCARDIAC LESIONS

ACYANOTIC- LACYANOTIC- L→R SHUNTING→R SHUNTING CYANOTIC- R→L SHUNTINGCYANOTIC- R→L SHUNTING RIGHT VENTRICULAR OUTFLOW RIGHT VENTRICULAR OUTFLOW

TRACT DEFECTSTRACT DEFECTS LEFT VENTRICULAR OUTFLOW TRACT LEFT VENTRICULAR OUTFLOW TRACT

DEFECTSDEFECTS

ACYNOTIC HEART LESIONSACYNOTIC HEART LESIONS

PATENT DUCTUS ARTERIOSUS (PDA)PATENT DUCTUS ARTERIOSUS (PDA) ATRIAL SEPTAL DEFECT (ASD)ATRIAL SEPTAL DEFECT (ASD) VENTRICULAR SEPTAL DEFECT (VSD)VENTRICULAR SEPTAL DEFECT (VSD)

PATENT DUCTUS ARTERIOSUSPATENT DUCTUS ARTERIOSUSPDAPDA

This shows the shunt This shows the shunt from the left side from the left side (systemic) to the (systemic) to the right side (pulmonary right side (pulmonary bed) through the bed) through the Ductus Arteriosus. Ductus Arteriosus. Failure of Failure of spontaneous closure.spontaneous closure.

IncidenceIncidence– 1:10,000 live births1:10,000 live births– 20-40% in 1000-1500 20-40% in 1000-1500

gram infantsgram infants– 5-10% of all CHD5-10% of all CHD

PDA-CLINICAL PDA-CLINICAL MANISFESTATIONSMANISFESTATIONS

+/- murmur+/- murmur CHFCHF Bounding peripheral pulsesBounding peripheral pulses Hyperactive precordiumHyperactive precordium Hypercarbia with Hypercarbia with ↑O2 ↑O2

demands, increased vent demands, increased vent support and pulmonary support and pulmonary deteriorationdeterioration

Widened pulse pressure, low Widened pulse pressure, low diastolic pressure, suggesting a diastolic pressure, suggesting a large runoff from the aorta large runoff from the aorta

PDA- DIAGNOSISPDA- DIAGNOSIS

CLINICAL EXAMCLINICAL EXAM X-RAYX-RAY ECHO- Will show the Ductal flow, the ECHO- Will show the Ductal flow, the

size of the left atrium and ventricle, size of the left atrium and ventricle, and can quantitate the stress of and can quantitate the stress of these structures.these structures.

PDA-TREATMENTPDA-TREATMENT

Prostaglandins are circulating vasoactive Prostaglandins are circulating vasoactive substances known to maintain Ductal substances known to maintain Ductal patency. patency.

Indomethacin Indomethacin is a drug that blocks the is a drug that blocks the synthesis of prostaglandins and will synthesis of prostaglandins and will reduce or close the duct. If indocin fails reduce or close the duct. If indocin fails surgical ligation may be indicated.surgical ligation may be indicated.

Evaluation of platelets, creatinine and Evaluation of platelets, creatinine and urine output prior to treatment is urine output prior to treatment is essential.essential.

Fluid restriction/diureticsFluid restriction/diuretics

PDA-NURSING PDA-NURSING CONSIDERATIONSCONSIDERATIONS

Indocin can cause a transient drop in UO. Indocin can cause a transient drop in UO. Monitoring of accurate I&O, report urine Monitoring of accurate I&O, report urine

< 1.0cc/kg/hr< 1.0cc/kg/hr L->R shunting causes decreased blood L->R shunting causes decreased blood

flow to the mesenteric artery and can flow to the mesenteric artery and can cause bowel ischemia. Infant should be cause bowel ischemia. Infant should be NPO, observe for NEC.NPO, observe for NEC.

Report oozing of blood, delayed clotting. Report oozing of blood, delayed clotting. Indocin interferes with platelet function.Indocin interferes with platelet function.

Anticipate lab drawsAnticipate lab draws

VENTRICULAR SEPTAL DEFECT VENTRICULAR SEPTAL DEFECT (VSD)(VSD)

This shows the classic This shows the classic hemodynamics after hemodynamics after PVRPVR↓↓

Demonstrates blood Demonstrates blood shunting from the left shunting from the left side through the side through the defect to the right defect to the right side of the heartside of the heart

Incidence 1:3000 live Incidence 1:3000 live birthsbirths

Defect is between the Defect is between the ventriclesventricles

VSD-CLINICAL VSD-CLINICAL MANISFESTATIONSMANISFESTATIONS

Two types. Small Two types. Small muscular VSDmuscular VSD’’s which s which spontaneously close in spontaneously close in 30% of cases30% of cases

Moderate-large VSDModerate-large VSD’’ss PresentationPresentation

– Pansystolic murmurPansystolic murmur– Increased pulmonary blood Increased pulmonary blood

flow producing signs of flow producing signs of CHFCHF

– Failure to thriveFailure to thrive

VSD-DIAGNOSISVSD-DIAGNOSIS

Physical examPhysical examTachypnea, and Tachypnea, and

tachycardictachycardic

Liver will be easily Liver will be easily palpatedpalpated

Murmur at the 4Murmur at the 4thth ICS,LSBICS,LSB

Infant will tire with Infant will tire with nipplingnippling

Chest x-ray- Chest x-ray- enlarged heart with enlarged heart with increased vascular increased vascular markingsmarkings

ECHO-diagnosticECHO-diagnostic EKG- Left EKG- Left

ventricular ventricular hypertrophyhypertrophy

VSD-TREATMENTVSD-TREATMENT

MedicalMedical

Treatment of CHF Treatment of CHF utilizing diuretics, utilizing diuretics, oxygen, and good oxygen, and good nutritionnutrition

Some defects can be Some defects can be fixed in the cath lab fixed in the cath lab

SurgicalSurgical

Large defects should Large defects should be closed by two be closed by two years of ageyears of age

Outcome depends on Outcome depends on myocardial myocardial dysfunction form CHF dysfunction form CHF and post op and post op pulmonary pulmonary hypertensionhypertension

Pump casePump case

ATRIAL SEPTAL DEFECT ATRIAL SEPTAL DEFECT (ASD)(ASD)

The classic The classic hemodynamics hemodynamics demonstrates demonstrates shunting from the Lshunting from the L→R →R after pulmonary after pulmonary resistance decreases.resistance decreases.

Incidence- 1:5000 live Incidence- 1:5000 live birthsbirths

Defect is between the Defect is between the atriaatria

ASD CLINICAL ASD CLINICAL MANIFESTATIONS & THERAPYMANIFESTATIONS & THERAPY

Many are generally asymptomatic and go Many are generally asymptomatic and go unrecognized, if diagnosed in young unrecognized, if diagnosed in young adulthood, prognosis is much worse.adulthood, prognosis is much worse.

If diagnosed in infancy they are picked up If diagnosed in infancy they are picked up by a murmur at the 2by a murmur at the 2ndnd ICS, LSB. ICS, LSB.

Treated medically, CHF, if intractable CHF Treated medically, CHF, if intractable CHF surgical repair. Mortality is <1 if done in surgical repair. Mortality is <1 if done in the first two years of age. 5-10% atrial the first two years of age. 5-10% atrial arrhythmias.arrhythmias.

If undetected until 3If undetected until 3rdrd or 4 or 4thth decade decade ↑risk ↑risk of pulm vascular disease and LV of pulm vascular disease and LV dysfunction.dysfunction.

CYANOTIC HEART DEFECTSCYANOTIC HEART DEFECTSRR→L SHUNTS→L SHUNTS

These defects have blood returning These defects have blood returning form the body, entering the right side form the body, entering the right side of the heart and then shunting across of the heart and then shunting across the defect to the left side of the the defect to the left side of the heart, bypassing the lungs and heart, bypassing the lungs and producing cyanosisproducing cyanosis– TRANSPOSITION OF THE GREAT VESSELSTRANSPOSITION OF THE GREAT VESSELS– TETROLOGY OF FALLOTTETROLOGY OF FALLOT

TRANSPOSITION OF THE GREAT TRANSPOSITION OF THE GREAT VESSELSVESSELS

This demonstrates two This demonstrates two completely separate completely separate circuits without mixing circuits without mixing of blood at any levelof blood at any level

Aorta arises from the Aorta arises from the right ventricle and the right ventricle and the pulmonary valve from pulmonary valve from the left ventriclethe left ventricle

Incidence- 1:5000 live Incidence- 1:5000 live births, 5-10% of CHD, births, 5-10% of CHD, 3:1 males3:1 males

Most common cyanotic Most common cyanotic defect in first wk of lifedefect in first wk of life

TRANSPOSITION OF THE GREAT TRANSPOSITION OF THE GREAT VESSELSVESSELS

Cyanosis is usually severeCyanosis is usually severe Systolic murmur with a Systolic murmur with a

single second heart sound is single second heart sound is heardheard

Metabolic acidosis is usually Metabolic acidosis is usually presentpresent

Tachypneic but comfortableTachypneic but comfortable If there is a sizable VSD, the If there is a sizable VSD, the

infant may have mild infant may have mild cyanosis with crying. CHF, cyanosis with crying. CHF, with a loud murmur will with a loud murmur will develop 2-6 weeks of lifedevelop 2-6 weeks of life

TRANSPOSITION OF THE GREAT TRANSPOSITION OF THE GREAT VESSELSVESSELS

Diagnosis Diagnosis

X-ray-X-ray- enlarged heart enlarged heart with a narrow base with a narrow base because the aorta is because the aorta is over the pulmonary over the pulmonary artery, artery, ““egg on a stringegg on a string””

EKGEKG- RVH- RVH

ECHOECHO- abnormal origin - abnormal origin of the great vesselsof the great vessels

TRANSPOSITION OF THE GREAT TRANSPOSITION OF THE GREAT VESSELSVESSELS

Medical treatmentMedical treatment

--Correction of metabolic Correction of metabolic derangementsderangements

-PGE1 to maintain ductal patency -PGE1 to maintain ductal patency until palliative surgery can be until palliative surgery can be performedperformed

-management of CHF-management of CHF

Palliation of TGAPalliation of TGA includes creating an ASD includes creating an ASD through a Balloon Atrial Septostomy( Rashkind). through a Balloon Atrial Septostomy( Rashkind).

This improves ad mixing of systemic and This improves ad mixing of systemic and pulmonary bloodpulmonary blood..

Surgical Correction- TGASurgical Correction- TGA

Definitive surgical correction Definitive surgical correction involves switching the right involves switching the right and left sided structures.and left sided structures.

Without surgical correction Without surgical correction 90% mortality.90% mortality.

Post op complications Post op complications include arrhythmias, include arrhythmias, obstruction to systemic or obstruction to systemic or pulmonary venous return pulmonary venous return and right ventricular and right ventricular dysfunction. Damage to the dysfunction. Damage to the coronary arteries, MI.coronary arteries, MI.

TETROLOGY OF FALLOT (TOF)TETROLOGY OF FALLOT (TOF)

The hemodynamics of TOF The hemodynamics of TOF demonstrate demonstrate ↓blood flow to ↓blood flow to the lungs and ↑blood flow to the lungs and ↑blood flow to body.body.

Incidence- 1:5000 live births Incidence- 1:5000 live births and accounts for 10% of CHD. and accounts for 10% of CHD. Most common CHD beyond Most common CHD beyond neonatal period.neonatal period.

Four anatomical conditionsFour anatomical conditionsVSDVSDPulmonary StenosisPulmonary StenosisOverriding aortaOverriding aortaRVHRVH

TOF CLINICAL PRESENTATION TOF CLINICAL PRESENTATION AND DIAGNOSISAND DIAGNOSIS

Clinical PresentationClinical PresentationTiming of presentation Timing of presentation depends on the degree depends on the degree of PS, will have a systolic of PS, will have a systolic murmurmurmur

DiagnosisDiagnosisx-ray- boot shapedx-ray- boot shapedEKG- RVHEKG- RVHECHO- diagnosticECHO- diagnostic

TREATMENT OF TETROLOGY TREATMENT OF TETROLOGY OF FALLOTOF FALLOT

MEDICALMEDICAL

Treatment is aimed Treatment is aimed correcting correcting hypoxemia, hypoxemia, polycythemia and polycythemia and infection.infection.

PGE1PGE1

SURGICALSURGICAL

Palliative-Palliative-improve improve pulmonary blood flow pulmonary blood flow (Blalock-Taussig)(Blalock-Taussig)

SurgicalSurgical correction is correction is best at 3-4 years. best at 3-4 years. Close the VSD, Close the VSD, eliminating PS by eliminating PS by resection and resection and replacement of the replacement of the valve. valve.

LEFT VENTRICULAR LEFT VENTRICULAR OUTFLOW DEFECTSOUTFLOW DEFECTS

Impedes blood flow leaving the LV to Impedes blood flow leaving the LV to the body. They can be cyanotic or the body. They can be cyanotic or

acyanotic depending upon the acyanotic depending upon the severity of the defect and associated severity of the defect and associated

featuresfeatures

AORTIC STENOSISAORTIC STENOSISCOARCTATION OF THE AortaCOARCTATION OF THE Aorta

Hypoplastic Left Heart SyndromeHypoplastic Left Heart Syndrome

AORTIC STENOSISAORTIC STENOSIS

Demonstrates blood Demonstrates blood trying to squeeze through trying to squeeze through a stenotic aortic valve to a stenotic aortic valve to the aorta.the aorta.

Incidence- 3-6% of CHD, Incidence- 3-6% of CHD, males 4:1males 4:1

Stenosis of the aortic Stenosis of the aortic valve with thickened valve with thickened deformed cusps, deformed cusps, biventricular biventricular hypertrophy, hypoplastic hypertrophy, hypoplastic LV, MV may be affected.LV, MV may be affected.

AORTIC STENOSISAORTIC STENOSISPRESENTATION AND PRESENTATION AND

DIAGNOSISDIAGNOSIS Presentation is dependent Presentation is dependent

on the degree or stenosis. on the degree or stenosis. Some are normal at birth Some are normal at birth but progress rapidly to but progress rapidly to failurefailure

DiagnosisDiagnosis– X-ray-may be normal with X-ray-may be normal with

↑ PVM↑ PVM– EKG- Biventricular EKG- Biventricular

hypertrophyhypertrophy– ECHO- diagnosticECHO- diagnostic

AORTIC STENOSIS- treatmentAORTIC STENOSIS- treatment

MedicalMedical

Conservative Conservative approach unless approach unless infant de-infant de-compensates with compensates with CHFCHF

Valvuloplasty- Valvuloplasty- surgical team on surgical team on standbystandby

SurgicalSurgical

Valve replacement Valve replacement if progressive if progressive symptoms such as symptoms such as increased LVH, or increased LVH, or EKG changesEKG changes

COARCTATION OF THE AORTACOARCTATION OF THE AORTA(COA)(COA)

Usually juxtaductal Usually juxtaductal (opposite the entry (opposite the entry point of the DA) this point of the DA) this type usually presents type usually presents in Infancy.in Infancy.

Other positions Other positions present later in lifepresent later in life

8-10% of all CHD. 8-10% of all CHD. Males 2:1, Males 2:1,

30% associated with 30% associated with Turners syndromeTurners syndrome

COARCTATION OF THE AORTACOARCTATION OF THE AORTACLINICAL MANIFESTATIONSCLINICAL MANIFESTATIONS

MildMild

Feeding intoleranceFeeding intolerance

Failure to thriveFailure to thrive

MottledMottled

Poor urinary outputPoor urinary output

Unequal pulsesUnequal pulses

SevereSevere

Respiratory distressRespiratory distress

CyanosisCyanosis

HypertensionHypertension

Unequal pulsesUnequal pulses

MurmurMurmur

CHFCHF

NECNEC

ATNATN

CORACTATION OF THE AORTACORACTATION OF THE AORTA

DiagnosisDiagnosis– X-ray- X-ray-

Cardiomegaly with Cardiomegaly with increased PVMincreased PVM

– ECHO- diagnosticECHO- diagnostic

COARCTATION OF THE AORTACOARCTATION OF THE AORTATREATMENTTREATMENT

MedicalMedicalPGE1 to improve blood PGE1 to improve blood

flow to extremities, flow to extremities, mesentery, and mesentery, and kidneyskidneys

Correction of metabolic Correction of metabolic acidosisacidosis

Consider diureticsConsider diuretics

Maintain normal HCTMaintain normal HCT

Support blood pressureSupport blood pressure

SurgicalSurgical- end to end - end to end anastomosis is anastomosis is preferred, some need preferred, some need patches, or BT shuntpatches, or BT shunt

-post op hypertension is -post op hypertension is more common in older more common in older children than neonateschildren than neonates

-Clamp time important -Clamp time important

-Small percentage of re--Small percentage of re-coarctation needing coarctation needing repairrepair

RIGHT VENTRICULARRIGHT VENTRICULAROUTFLOW DEFECTSOUTFLOW DEFECTS

Impedes blood flow leaving from or Impedes blood flow leaving from or out of the Right Ventricle.out of the Right Ventricle.

Can be cyanotic or acyanotic Can be cyanotic or acyanotic depending on the severity of the depending on the severity of the defect and associated features.defect and associated features.

Pulmonary StenosisPulmonary Stenosis Tricuspid AtresiaTricuspid Atresia

PULMONARY STENOSISPULMONARY STENOSISPSPS

This demonstrates blood This demonstrates blood trying to squeeze through a trying to squeeze through a stenotic valve.stenotic valve.

Incidence- 1:14,000Incidence- 1:14,000 There is a narrowed There is a narrowed

opening in the pulmonary opening in the pulmonary valve as a consequence of valve as a consequence of cusp valve fusions; this cusp valve fusions; this causes causes ↑↑pressure load to ↑↑pressure load to the RV = RVHthe RV = RVH

Pulmonary StenosisPulmonary StenosisDiagnosisDiagnosis

X-rayX-ray- normal sized - normal sized heart with decreased heart with decreased pulmonary markingspulmonary markings

EKGEKG- RAH and RVH- RAH and RVH ECHOECHO- visualization - visualization

of the valve and its of the valve and its movement, velocity movement, velocity across the valve and across the valve and the amount of RVH the amount of RVH presentpresent

Pulmonary StenosisPulmonary StenosisTreatmentTreatment

Treatment varies according to Treatment varies according to presentation and degree of stenosis.presentation and degree of stenosis.

Severe stenosis will require PGE1Severe stenosis will require PGE1 Medical treatment will be to do a Medical treatment will be to do a

pulmonary valvuloplasty by balloon pulmonary valvuloplasty by balloon dilation. The surgical team must be on dilation. The surgical team must be on standby.standby.

Surgical repair will be delayed and a BT Surgical repair will be delayed and a BT shunt may be required before resection of shunt may be required before resection of the valve can be performed.the valve can be performed.

TRICUSPID ATRESIATRICUSPID ATRESIA(TA)(TA)

85% Hypoplastic RV, RV 85% Hypoplastic RV, RV muscle hypertrophied, muscle hypertrophied, 2/3rds have PS2/3rds have PS

Tricuspid valve fails to formTricuspid valve fails to formA VSD is usually presentA VSD is usually presentAt birth the only flow to the PA At birth the only flow to the PA

is via the PFOis via the PFOIncidence- 1-3% of all CHD, Incidence- 1-3% of all CHD,

some are associated with some are associated with TGA, if so males are more TGA, if so males are more affectedaffected

TRICUSPID ATRESIATRICUSPID ATRESIAClinical ManifestationsClinical Manifestations

Cyanosis soon after birth Cyanosis soon after birth Increasing cyanosis when the PDA Increasing cyanosis when the PDA

closescloses Murmur is associated with Murmur is associated with

pulmonary stenosis or VSDpulmonary stenosis or VSD

TRICUSPID ATRESIATRICUSPID ATRESIADiagnosisDiagnosis

X-rayX-ray- normal heart - normal heart size with decreased size with decreased pulmonary pulmonary vascularityvascularity

EKGEKG- Left axis - Left axis deviation, increased deviation, increased LV forces, LV forces, decreased RV forcesdecreased RV forces

ECHOECHO- diagnostic- diagnostic

TRICUSPID ATRESIATRICUSPID ATRESIAManagementManagement

PGE1PGE1 Rashkind procedure if inadequate RRashkind procedure if inadequate R→L shunting →L shunting

(usually not needed)(usually not needed) If ↑PBF →→PA bandingIf ↑PBF →→PA banding If ↓ PBF →shunt will be required between the If ↓ PBF →shunt will be required between the

subclavian and PA-(BTshunt)subclavian and PA-(BTshunt) Surgical correction involves closure of the VSD Surgical correction involves closure of the VSD

and eliminating the pulmonary Stenosis by and eliminating the pulmonary Stenosis by resectionresection

In children a Fontan procedure is used to reroute In children a Fontan procedure is used to reroute blood by anastomosis of the right atrium to the blood by anastomosis of the right atrium to the PA.PA.

HYPOPLASTIC LEFT HEART HYPOPLASTIC LEFT HEART SYNDROME- (HLHS)SYNDROME- (HLHS)

Describes a group of Describes a group of malformations in malformations in which there is which there is obstruction to the obstruction to the left side and left side and underdevelopment underdevelopment of the systemic of the systemic pumping chamberpumping chamber

HYPOPLASTIC LEFT HEART HYPOPLASTIC LEFT HEART SYNDROMESYNDROME

1% of all CHD1% of all CHD Leading cause of Leading cause of

death in the first death in the first month of lifemonth of life

Hypoplasia of LV, Hypoplasia of LV, severe mitral valve severe mitral valve stenosis or Atresia, stenosis or Atresia, hypoplastic ascending hypoplastic ascending aortic archaortic arch

Blood supply to the Blood supply to the descending aorta, descending aorta, aortic arch and aortic arch and coronary arteries are coronary arteries are all depending on the all depending on the PDAPDA

HYPOPLASTIC LEFT HEART HYPOPLASTIC LEFT HEART SYNDROME- Clinical SYNDROME- Clinical

ManifestationsManifestations Usually normal at birthUsually normal at birth Tachypnea and dyspnea develop as the PA Tachypnea and dyspnea develop as the PA

flow increases, murmur, single S2flow increases, murmur, single S2 Development of CHF by 24-48hrs of lifeDevelopment of CHF by 24-48hrs of life Low output signs develop as cardiac Low output signs develop as cardiac

output decreasesoutput decreases Severe mottling, gray pallor of the skin, Severe mottling, gray pallor of the skin, ↓↓

pulses, pulses, ↓↓ output, output, ↑ CFT, possible ↑ CFT, possible hepatomegalyhepatomegaly

HYPOPLASTIC LEFT HEART HYPOPLASTIC LEFT HEART SYNDROME- DIAGNOSISSYNDROME- DIAGNOSIS

X-rayX-ray generalized generalized cardiomegaly and cardiomegaly and increased increased pulmonary pulmonary markingsmarkings

EKGEKG- decreased - decreased left ventricular left ventricular voltagevoltage

ECHOECHO- diagnostic- diagnostic

HYPOPLASTIC HEART HYPOPLASTIC HEART SYNDROME- ManagementSYNDROME- Management

PGE1 to maintain systemic perfusion PGE1 to maintain systemic perfusion to vital organs and management of to vital organs and management of CHFCHF

Three therapies:Three therapies:

1. Compassion care1. Compassion care

2. Staged Norwood2. Staged Norwood

3. Cardiac Transplant3. Cardiac Transplant