Perioperative Management of TAPVC

Transcription

1 Perioperative Management of TAPVC Professor Andrew Wolf Rush University Medical Center,Chicago USA Bristol Royal Children s Hospital UK I have no financial disclosures relevant to this presentation

2 TAPVC /PAPVC Incidence 1.5% of cardiac lesions Hugely heterogenic - single vein abnormality intact septum PAPVC (often not picked up) -Obstructed Complex TAPVC Evidence based guidance limited Understanding individual pathophysiology key

3 PCCC review of 2191 cases ( ) Overall surgical mortality 13% Era effect: 8% Obstructive features in 29% of cases Obstruction 26% mortality St Louis Ann Thorac Surg 2012: 94:

4 Mortality, Risk Factors and Trends Era effect Age and outcome Postoperative epinephrine Obstruction/reoperation Low flow vs DHCA Specific anesthesia drugs: -milrinone, epinephrine - nitroprusside - nitric oxide Karamlou T Circulation 2007;115:

5 45% 25% 25%

6 TAPVD without Obstruction PA Ao No obstruction in pulmonary veins No obstruction at atrial level

7 Unobstructed TAPVD Can be unsymptomatic Tachypnoea, feeding issues Recurrent chest infections May only be mild desaturation value of neonatal SP02 checks Presentation may be late Physiologically right heart overload Longer term risks to the pulmonary circuit

8 TAPVD with Atrial Obstruction PA Ao shock Large pulmonary flow but no venous obstruction

9 TAPVD with Other Obstruction PA Ao Pulmonary venous obstruction high pulmonary pressure

10 Newborn Obstructed TAPVD Presentation Ventilatory failure Reduced left ventricular output - poor LA preload - Septal deviation from RV - External constraint (IPPV) - PDA vs PFO Often Diagnosed initially as PPHN Need for early Diagnostic Echo

11 Newborn Obstructed TAPVD Presentations Ventilatory failure: primary and secondary Right heart failure Cardiogenic shock

12 Obstructed TAPVD

13 Lung Pathology Interstitial oedema Lymphatic dilatation Engorged parenchymal veins High pressures and resistance Erythrocytes in alveoli Pulmonary hypertensive responses: - medial arterial wall constriction - increase airway resistance - medial wall hypertrophy

14 Obstructed TAPVD and Anesthesia Preoperative condition and on to bypass Management in the OR Early postoperative Care

15 Obstructed patient in PICU May be on prostaglandin ( from outside) May be on Nitric oxide May be on multiple inotropes Systemic Pressure may be poor Ventilation/ Oxygenation may be poor Lactate high, NIRS low Exact anatomy may not be known

16 Potential Therapeutic Preoperative Manoevres Ventilatory strategies: the dilema of - Pulmonary venous hypertension -Hypoxia and pulmonary hypertension -Pulmonary oedema and compliance Right ventricular support Fluids and maintenance of systemic output

17 First point of safety is bypass Little chance of dramatically improving the situation Change anything from PICU?? Septostomy or OR stat Echocardiography does not cure a patient Catheter clip

18 In the OR Do not delay, stay on whatever is working If not on inotropes:? Start milrinone when possible Intensive Care standard ventilator at matched settings Low flow vs no flow bypass Ultrafiltration on bypass and afterwards Take the PICU into the OR, take anesthesia into the PICU

19 Myocardial Development

20 Evidence of Reduction of Energy Substrates with Adrenaline Neonatal lamb vs mature sheep Myocardial O2 consumption ( coronary sinus) Adrenaline infusion to increase heart work Stable ATP/ADP ratios in adult Falling ATP/ ADP in the neonate Portman M. J Clin Investigation. 1989;83:

21 Myocardial Damage Loss of Energy Substrates Caspi J Pediatric Research 1994: 36: 49-54

22 Anesthesia Analgesia 2001;92:

23 Cellular Preservation with PDE Inhibitors Inhibition of Phosphodiesterases Leads to Prevention of the Mitochondrial Permeability Transition Pore Opening and Reperfusion Injury in Cardiac H9c2 Cells. Cardiovasc Drugs Ther 2011; 25: 299 The Role of Mitochondria and Sarcoplasmic Reticulum Calcium Handling Upon Reoxygenation of Hypoxic Myocardium. Circulation Research 1990; 66: 696 Milrinone and Low Cardiac Output Following Cardiac Surgery in Infants: Is There a Direct Myocardial Effect? Paediatr Cardiol. 2005; 26: 642

24 Coming off Bypass Right sided issues Pulmonary trauma/oedema. Pulmonary hypertension No coronary perfusion no chance Beware low blood pressure with a good gas Anesthesia technique and transfer to PICU Leaving connecting vein or PFO as a pop off?

25 Postoperative management Continue PDE blockade Minimal catecholamine Alternate meds: vasopressin ECMO options Calcium correction Protective/therapeutic lung strategies

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