Corey Bregman, M.D. Children’s Memorial Hospital Chicago, IL Pulmonary Atresia with Intact Ventricular Septum.

Corey Bregman, M.D.Corey Bregman, M.D.

Children’s Memorial HospitalChildren’s Memorial Hospital

Chicago, ILChicago, IL

Pulmonary Atresia with Pulmonary Atresia with Intact Ventricular Intact Ventricular SeptumSeptum

OverviewOverview

Accounts for fewer than 1% of all Accounts for fewer than 1% of all congenital heart defectscongenital heart defects

However is one of the more common However is one of the more common etiologies of cyanotic congenital heart etiologies of cyanotic congenital heart diseasedisease

Cause is unknownCause is unknown No predilection by sex, and many No predilection by sex, and many

sources believe no genetic basissources believe no genetic basis

OverviewOverview

Defined by an imperforate pulmonary Defined by an imperforate pulmonary valve combined with an intact ventricular valve combined with an intact ventricular septumseptum

Both an interatrial defect and a systemic Both an interatrial defect and a systemic to pulmonary shunt are required for to pulmonary shunt are required for survivalsurvival

Though conceptually simple, there is vast Though conceptually simple, there is vast morphologic variation of the right heart morphologic variation of the right heart and coronary arteriesand coronary arteries

Treatment is tailored to this variationTreatment is tailored to this variation

Anatomy of the DefectAnatomy of the Defect

SpectrumSpectrum

Well formed infundibulum, and the Well formed infundibulum, and the imperforate pulmonary valve will have imperforate pulmonary valve will have three cusps with commissure fusionthree cusps with commissure fusion

Diminutive right ventricle, a severely Diminutive right ventricle, a severely narrowed or atretic infundibulum, and a narrowed or atretic infundibulum, and a primitive valveprimitive valve

Spectrum and TimingSpectrum and Timing

Variation in timing of insult Variation in timing of insult relates to the morphologic relates to the morphologic variationsvariations

Insult probably occurs later than Insult probably occurs later than that of pulmonary atresia with that of pulmonary atresia with VSDVSD

Note that the pulmonary trunk is Note that the pulmonary trunk is almost always present almost always present

Path of Blood FlowPath of Blood Flow

Typically Typically systemic blood systemic blood travels through a travels through a true secundum true secundum ASD or a PFOASD or a PFO

Rarely there are Rarely there are reports of a reports of a coronary sinus to coronary sinus to left atrium left atrium fenestrationfenestration

Systemic to Systemic to pulmonary pulmonary arterial circulation arterial circulation through left sided through left sided PDAPDA

Rarely there are Rarely there are reports of reports of descending aorta descending aorta branches to the branches to the PA and of bilateral PA and of bilateral PDAPDA

Associated AnomaliesAssociated Anomalies

Hypoplastic but hypertrophied Hypoplastic but hypertrophied right ventricleright ventricle

Right atrial dilatationRight atrial dilatation Tricuspid valve is rarely normalTricuspid valve is rarely normal Coronary artery anomaliesCoronary artery anomalies Often see aortic valve stenosisOften see aortic valve stenosis

Tricuspid ValveTricuspid Valve

Can see extreme stenosis to profound Can see extreme stenosis to profound regurgitationregurgitation

Severe in 25% of cases and florid Severe in 25% of cases and florid regurgitation may result in fetal demiseregurgitation may result in fetal demise

Profound displacement, dysplastic leaflets, Profound displacement, dysplastic leaflets, shortened chordaeshortened chordae

Patients with the largest right ventricle Patients with the largest right ventricle usually have severe tricuspid regurgitationusually have severe tricuspid regurgitation

Patients with most underdeveloped right Patients with most underdeveloped right ventricle usually have severe tricuspid ventricle usually have severe tricuspid stenosisstenosis

Some patients have features of or Some patients have features of or concomitant Ebstein’s anomalyconcomitant Ebstein’s anomaly

Dilated Dilated tricuspid tricuspid annulusannulus

Downward Downward displacement displacement of the septal of the septal and posterior and posterior leafletsleaflets

Coronary Artery Anomalies Coronary Artery Anomalies

Only seen when right ventricular is thick Only seen when right ventricular is thick walled high pressure chamberwalled high pressure chamber

Anomalies specific to this condition Anomalies specific to this condition include:include:– Coronary artery stenosisCoronary artery stenosis– Coronary artery interruptionCoronary artery interruption– ““Coronary-cameral fistula” which represents Coronary-cameral fistula” which represents

direct communication between the right direct communication between the right ventricle and a coronary artery through ventricle and a coronary artery through endothelium lined sinusoidendothelium lined sinusoid

Coronary-Cameral FistulaCoronary-Cameral Fistula

Sinusoidal channels provide blood to Sinusoidal channels provide blood to myocardium prior to development of myocardium prior to development of the coronary arteries during cardiac the coronary arteries during cardiac developmentdevelopment

Thought to persist with elevated Thought to persist with elevated right ventricle pressure and may right ventricle pressure and may communicate with one or both communicate with one or both coronary arteriescoronary arteries

Coronary AnomaliesCoronary Anomalies

Right ventricular pressure Right ventricular pressure transferred to the coronary arteriestransferred to the coronary arteries

Myointimal hyperplasiaMyointimal hyperplasia Distortion of architecture and Distortion of architecture and

endotheliumendothelium Resultant stenosis and interruptionResultant stenosis and interruption These lesions are often close to the These lesions are often close to the

fistulasfistulas

Coronary AnomaliesCoronary Anomalies

Diminutive right Diminutive right ventricleventricle

Communication to Communication to LAD coronary LAD coronary arteryartery

Arrow points to Arrow points to interruption with no interruption with no filling of aortic rootfilling of aortic root

This artery is right This artery is right ventricle ventricle dependentdependent

Myointimal HyperplasiaMyointimal Hyperplasia

Presentation and ManagementPresentation and Management

Infants deteriorate within hours after birth Infants deteriorate within hours after birth becoming cyanotic and hypoxemicbecoming cyanotic and hypoxemic

Have a single S2, tricuspid and PDA Have a single S2, tricuspid and PDA murmersmurmers

Prostaglandin E to maintain ductusProstaglandin E to maintain ductus Metabolic acidosis is ominous and usually Metabolic acidosis is ominous and usually

indicates hypoxic cell damageindicates hypoxic cell damage Long term management more complexLong term management more complex Will typically evaluate with both Will typically evaluate with both

echocardiography and angiographyechocardiography and angiography

RadiographyRadiography

Heart size ranges Heart size ranges from only mildly from only mildly enlarged to filling enlarged to filling the entire chest the entire chest cavitycavity

If pulmonary If pulmonary parenchyma is parenchyma is visible may see visible may see reduced vascular reduced vascular markingsmarkings

Further ImagingFurther Imaging

Role for both Echocardiography Role for both Echocardiography and Angiographyand Angiography

Echo is weak in identifying the Echo is weak in identifying the ventriculocoronary connectionsventriculocoronary connections

Angio is warranted to evaluate for Angio is warranted to evaluate for these and the status of the these and the status of the coronaries coronaries

EchocardiographyEchocardiography

Imperforate Pulmonary Valve

AngiographyAngiography

Thickened tricuspid vlveand regurgitation hascaused severe right atrialenlargement

Ventriculocoronarycommunication with multiple stenoses

Surgical ConsiderationsSurgical Considerations

Is the patient a candidate for Is the patient a candidate for biventricular or univentricular repair?biventricular or univentricular repair?

Are there ventriculocoronary Are there ventriculocoronary connections? How much of the coronary connections? How much of the coronary circulation is right ventricle dependent?circulation is right ventricle dependent?

Is there an infundibulum? Is there a Is there an infundibulum? Is there a main pulmonary trunk in continuity with main pulmonary trunk in continuity with the imperforate valve?the imperforate valve?

Is the left ventricular function preserved?Is the left ventricular function preserved?

Right Ventricular Outflow Right Ventricular Outflow ReconstructionReconstruction

Will give biventricular circulationWill give biventricular circulation Small hypertrophic right ventricle Small hypertrophic right ventricle

may enlarge and adapt after may enlarge and adapt after reconstructionreconstruction

Methods includeMethods include– Pulmonary valvotomyPulmonary valvotomy– Outflow tract patchOutflow tract patch– Catheter perforation of pulmonary valveCatheter perforation of pulmonary valve

Univentricular Repair AlgorithmUniventricular Repair Algorithm

Majority or entirety of coronary Majority or entirety of coronary circulation is right ventricle circulation is right ventricle dependentdependent

Severe tricuspid regurgitation or Severe tricuspid regurgitation or Ebstein’s anomalyEbstein’s anomaly

SummarySummary

Conceptually simple – but varied Conceptually simple – but varied morphologic presentationmorphologic presentation

Pay attention to the status of the Pay attention to the status of the right ventricle, tricuspid valve, and right ventricle, tricuspid valve, and coronary arteriescoronary arteries

Management is specifically tailored Management is specifically tailored to morphologyto morphology

Patients will require frequent Patients will require frequent follow-upfollow-up

SourcesSources

1.1. Allen, Hugh D. Moss & Adams’ Heart Allen, Hugh D. Moss & Adams’ Heart Disease in Infants, Children & Adolescents: Disease in Infants, Children & Adolescents: Including the Fetus and Young Adults, 6Including the Fetus and Young Adults, 6thth Edition. Lippincott Williams & Wilkins. Edition. Lippincott Williams & Wilkins. (2001).(2001).

2.2. Mavroudis, C.; Backer, C. Pediatric Cardiac Mavroudis, C.; Backer, C. Pediatric Cardiac Surgery, 3Surgery, 3rdrd Edition. Mosby. (2003). Edition. Mosby. (2003).

3.3. Park, Myung K. Pediatric Cardiology for Park, Myung K. Pediatric Cardiology for Practitioners, 4Practitioners, 4thth Edition. Mosby. (2002). Edition. Mosby. (2002).