Presentation on theme: "Pulmonary Atresia with Intact Ventricular Septum"— Presentation transcript:
1 Pulmonary Atresia with Intact Ventricular Septum Corey Bregman, M.D.Children’s Memorial HospitalChicago, IL
2 Overview Accounts for fewer than 1% of all congenital heart defects However is one of the more common etiologies of cyanotic congenital heart diseaseCause is unknownNo predilection by sex, and many sources believe no genetic basis
3 OverviewDefined by an imperforate pulmonary valve combined with an intact ventricular septumBoth an interatrial defect and a systemic to pulmonary shunt are required for survivalThough conceptually simple, there is vast morphologic variation of the right heart and coronary arteriesTreatment is tailored to this variation
5 SpectrumWell formed infundibulum, and the imperforate pulmonary valve will have three cusps with commissure fusionDiminutive right ventricle, a severely narrowed or atretic infundibulum, and a primitive valve
6 Spectrum and TimingVariation in timing of insult relates to the morphologic variationsInsult probably occurs later than that of pulmonary atresia with VSDNote that the pulmonary trunk is almost always present
7 Path of Blood FlowTypically systemic blood travels through a true secundum ASD or a PFORarely there are reports of a coronary sinus to left atrium fenestrationSystemic to pulmonary arterial circulation through left sided PDARarely there are reports of descending aorta branches to the PA and of bilateral PDA
8 Associated AnomaliesHypoplastic but hypertrophied right ventricleRight atrial dilatationTricuspid valve is rarely normalCoronary artery anomaliesOften see aortic valve stenosis
9 Tricuspid Valve Can see extreme stenosis to profound regurgitation Severe in 25% of cases and florid regurgitation may result in fetal demiseProfound displacement, dysplastic leaflets, shortened chordaePatients with the largest right ventricle usually have severe tricuspid regurgitationPatients with most underdeveloped right ventricle usually have severe tricuspid stenosis
10 Some patients have features of or concomitant Ebstein’s anomaly Dilated tricuspid annulusDownward displacement of the septal and posterior leaflets
11 Coronary Artery Anomalies Only seen when right ventricular is thick walled high pressure chamberAnomalies specific to this condition include:Coronary artery stenosisCoronary artery interruption“Coronary-cameral fistula” which represents direct communication between the right ventricle and a coronary artery through endothelium lined sinusoid
12 Coronary-Cameral Fistula Sinusoidal channels provide blood to myocardium prior to development of the coronary arteries during cardiac developmentThought to persist with elevated right ventricle pressure and may communicate with one or both coronary arteries
13 Coronary AnomaliesRight ventricular pressure transferred to the coronary arteriesMyointimal hyperplasiaDistortion of architecture and endotheliumResultant stenosis and interruptionThese lesions are often close to the fistulas
14 Coronary Anomalies Diminutive right ventricle Communication to LAD coronary arteryArrow points to interruption with no filling of aortic rootThis artery is right ventricle dependent
16 Presentation and Management Infants deteriorate within hours after birth becoming cyanotic and hypoxemicHave a single S2, tricuspid and PDA murmersProstaglandin E to maintain ductusMetabolic acidosis is ominous and usually indicates hypoxic cell damageLong term management more complexWill typically evaluate with both echocardiography and angiography
17 RadiographyHeart size ranges from only mildly enlarged to filling the entire chest cavityIf pulmonary parenchyma is visible may see reduced vascular markings
18 Further ImagingRole for both Echocardiography and AngiographyEcho is weak in identifying the ventriculocoronary connectionsAngio is warranted to evaluate for these and the status of the coronaries
20 Angiography Thickened tricuspid vlve and regurgitation has caused severe right atrialenlargementVentriculocoronarycommunication withmultiple stenoses
21 Surgical Considerations Is the patient a candidate for biventricular or univentricular repair?Are there ventriculocoronary connections? How much of the coronary circulation is right ventricle dependent?Is there an infundibulum? Is there a main pulmonary trunk in continuity with the imperforate valve?Is the left ventricular function preserved?
22 Right Ventricular Outflow Reconstruction Will give biventricular circulationSmall hypertrophic right ventricle may enlarge and adapt after reconstructionMethods includePulmonary valvotomyOutflow tract patchCatheter perforation of pulmonary valve
23 Univentricular Repair Algorithm Majority or entirety of coronary circulation is right ventricle dependentSevere tricuspid regurgitation or Ebstein’s anomaly
24 SummaryConceptually simple – but varied morphologic presentationPay attention to the status of the right ventricle, tricuspid valve, and coronary arteriesManagement is specifically tailored to morphologyPatients will require frequent follow-up
25 SourcesAllen, Hugh D. Moss & Adams’ Heart Disease in Infants, Children & Adolescents: Including the Fetus and Young Adults, 6th Edition. Lippincott Williams & Wilkins. (2001).Mavroudis, C.; Backer, C. Pediatric Cardiac Surgery, 3rd Edition. Mosby. (2003).Park, Myung K. Pediatric Cardiology for Practitioners, 4th Edition. Mosby. (2002).
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