Presentation on theme: "Pulmonary Atresia and Intact Ventricular Septum"— Presentation transcript:
1 Pulmonary Atresia and Intact Ventricular Septum Seoul National University HospitalDepartment of Thoracic & Cardiovascular Surgery
2 Pulmonary Atresia and Intact Ventricular Septum 1. DefinitionCongenital malformation in which pulmonary valve is atretic,coexisting with variable degrees of RV & TV hypoplasia2. Historical NoteHunter : 1st case report in 1783Peacock : Collected 7 patients report in 1839Grant : Coronary sinusoid & fistula recognized in 1926Davignon : Suggest systemic-pulmonary artery shunt in 1961Bowman : Shunt and RV outflow operation in 1971
3 PA+IVS Pathophysiology Atretic pulmonary valve combined with varying degrees of tricuspid valve and right ventricular hypoplasia, leading to inadequate pulmonary blood flow(supported by PDA) and hypoxia.Right ventricular-to-coronary artery fistulae are seen in 50~60% of patients, resulting in a right ventricular-dependent coronary circulation in 10~20%
4 Surgical Morphology of PA+IVS 1. Pulmonary valve : remnant fibrous component2. Pulmonary artery : usually normal in size3. Right ventricleVariable, enlarged in 5%, severely reduced capacity in 60 %Ebstein malformation & severe TR in enlarged thin RV.Diffuse fibrosis of hypertrophied small RV & fibroelastosis4. RV-coronary fistulaCoronary sinusoid in 50%, dependent circulation in 10%5. Tricuspid valve : always abnormal6. Right atriumIntraatrial communication in all cases, & enlarged7. Left-sided chambersLV compliances is depressed in many cases.LA enlarged & hypertrophy8. Coexisting cardiac anomalies ; uncommon
6 Clinical Features & Diagnosis 1. Symptoms & signsCyanosis after birth & becomes rapidly more severeas the ductus closes, respiratory distress, metabolic acidosis,Absence of RV impulse, no typical murmur2. Chest radiographyDiminished (or normal) vascular marking3. ElectrocardiographyProminent right atrial P-wave ( RA enlargement )4. Echocardiography5. Cardiac catheterization & cineangiography
7 Natural History of PA+IVS 1. IncidenceUncommon malformation1~1.5% of CHD3% of critically ill infants with CHD2. Survival50% survival in 2weeks of life15% survival in 6months of life3. Modes of deathSevere hypoxia & metabolic acidosis(Coincides with spontaneous closure of PDA)Rarely survival into young adult life
8 Technique of Operation for PA+IVS 1. Concomitant placement of patch & shunt2. Isolated procedureValvotomy onlyPlacement of transannular patchSystemic-pulmonary artery shunt only3. Definitive proceduresSeparated two-ventricle repairSingle-ventricle repair ( Fontan )*One & a half ventricle repairOverhauling RV cavity
9 Operation for PA+IVS Concomitant placement of patch & shunt
13 Surgical Results of PA+IVS 1. SurvivalEarly deathTime-related survival2. Modes of death3. Incremental risk factors for death1) Dimension of TV2) RV dependent coronary circulation3) Systemic-pulmonary shunt4) Birth weight4. Interim Intervention after the initial procedureTransannular patch is better than valvotomy5. Definitive procedures ; two ventricle repair
14 Surgical Indications of PA+IVS The presence of the malformation is generally anindication for operation because of highly lethal natureof the condition. As soon as diagnosis is suspected,neonate is intubated, and an infusion of PGE1 is begun.Selection of the initial intervention is based in large parton estimated probability( Z value, -3 ) that a separatedtwo-ventricle system will ultimately be possibleThe initial goals of correction are to improve pulmonaryblood flow with a systemic-to- pulmonary shunt & topromote RV development by relieving RVOTO
15 Special Situation & Controversies 1. Percutaneous valvotomyLaser valvotomy followed by balloon dilatation2. Formalin infiltration of ductus arteriosus3. Right ventricle to aorta conduit4. Tricuspid valve closure in RV-coronary fistula5. Tricuspid valve growth- TV and RV cavity tend to increase in size as thepatients grow with forward flow across valve.- With growth of RV, fistulae may disappear.- Abnormality of RV compliance usually remains.
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