Presentation on theme: "TOF with Absent Pulmonary Valve"— Presentation transcript:
1 TOF with Absent Pulmonary Valve Seoul National University HospitalDepartment of Thoracic & Cardiovascular Surgery
2 TOF with Absent Pulmonary Valve 1. DefinitionA subset of TOF determined largely by vestigial,severely hypoplasic, nonfunctioning pulmonaryleaflets at the junction of RV & pulmonary trunk2. History1) Royer & Wilson : 1st description in 19082) Kurtz : 2nd report in 1927
3 TOF with Absent Pulmonary Valve Morphology1. Pulmonary valveMyxomatous nubbins of valve tissue, severely hypoplastic,Both nonfunctioning & only minimally stenotic2. RVOTOften dilated and elongated3. Pulmonary trunkCentral portion of RPA & LPA are often aneurysmally dilatedDilation into hilar portion, then tracheobronchial compressionBeyond hilar portion, pulmonary arteries are normal in size
4 TOF with Absent Pulmonary Valve PAPulmonary ValvePulmonary annulus
5 Clinical Features & Diagnosis 1) Severe pulmonary regurgitation and somewhat increasedpulmonary blood flow2) Low pulmonary artery pressure & similar peak pressure in bothventricles due to narrowing annulus & large VSD3) Presentation is dependent on the severity of pulmonary arterialdilatation, Qp increased and tracheobronchial compression2. Diagnostic criteria1) Physical examinationOveractive heart, cardiomegaly, raised venous pressure2) Chest radiographySupracardiac mediastinal widening , atelectasis or overinflation3) Other studies include ECG, echocardiography, aortography
6 Natural History 1. Incidence 5% of TOF born with a large VSD + PS 2. 50% die in the 1st year of life if untreated, and mostin the few months of life, from the respiratory distressSuch patients also have heart failure with large shuntwith decreased systolic function.3. Patients who survive infancy, generally do well fortime being, and ultimately die from intractable rightheart failure.
7 Operative Treatment 1. Indications 2. Techniques 1) Urgently needed for small babies who present withsevere respiratory distress2) If infants responds well this therapy (prone, head-up),operation is deferred selectively to 3 -5 years electively.2. Techniques1) VSD closure and insertion of homograft beyond infancy2) Reduction pulmonary angioplasty with corrective repairis preferred in neonates and infants3) Pulmonary arterioplasty to takes pressure off underlyingtracheobronchial tree.
8 Operative ProcedureVentriculotomy and resection of dilated portions of main &branch pulmonary arteries.Reconstruction of the right and left pulmonary arteries &insertion of a homograft
9 Operative ProcedurePlacement of a homograft with a tube graft extensionfrom the diaphragmatic surface of the right ventricle
10 TOF with Absent Pulmonary Valve General management principles1. Preoperative1) Sternotomy2) Prone position3) Adequate management2. Postoperative1) Prone position in head-up2) Avoid barotrauma3) Avoid hyperinflation by air trapping4) Short inspiratory phase (1:E>1:4)
11 Operative Results 1. Survival ; a high probability of hospital death after repair in young infants, currentlyemphasizing pulmonary arterioplastyEarly deathPoor preoperative conditionsSevere respiratory problemsTime-related survivalSimilar to those with TOF2. Incremental risk factors for deathAllograft valve conduit