Right Ventricular Failure After Tetralogy of Fallot Repair Robert Jeenchen Chen, MD, MPH (rjchen@post.harvard.edu) Cardiovascular Surgery National Taiwan Univ Hosp, Taipei

Case Presentation 1y2m/o female baby (DOB:06/23/2000) Chief Complaint: Acrocyanosis, poor oral intake, hypoactivity for days Past History: Down syndrome, growth<3rd % TOF s/p modified B-T shunt at 2 m/o

Past History--Echo Before B-T Shunt Severe RVOT narrowing, scant antegrade flow, PG=78 Hypoplastic MPA (annulus=2 mm) PDA 2.9 mm McGoon index=1.37 VSD, 6.2 mm, perimembranous ASD, 2.9 mm at fossa ovalis

Past History- Cardiac Cath Before B-T Shunt IVC: ///(43%) RA: //0.5(40.3%) SVC: ///(62.5%) RV: 60/5/(60.6%) Ao: 90/40/65(71.9%) Extreme TOF Hypoplastic PA McGoon index =(5.65+3.84)/6.96 =1.3 Large PDA from LSCA Right aortic arch

Op (2 m/o): Palliative Aorto-Pulmonary Shunting Right modified Blalock-Taussig shunt PA-SCA 3.5 mm GoreTex graft

Past History--Cardiac Echo One Month After B-T Shunt RVOT narrowing, scant antegrade. PG=55 Patent B-T shunt McGoon=1.6 PDA closed ASD, slit VSD, 7.8 mm TR, PG=39 RPA juxta-shunt 2.6/6.1 mm LPA 3 mm, juxta-ductal stenosis

Past History--Cardiac Echo Four Months After B-T Shunt RVOTO Patent B-T shunt McGoon=2.0 ASD, slit VSD, 7 mm TR, PG=40 RPA 5.9 mm, mild juxta-shunt stenosis LPA 6.9 mm

Present Illness (1 Y/O) Followed up at OPD Acrocyanosis, poor oral intake, hypo-activity for days Generalized cyanosis (SpO2=45%) at NTUH Echo: B-T shunt occlusion! Emergent op!

Op (1 Y/O): Total Correction Infundibulectomy ASD fenestration VSD patch repair Trans-annular patch (TAP) augmentation of MPA till LPA orifice Occluded B-T shunt

Course and Treatment Postop low CO High inotrope support Anuria Hepatomegaly Inhaled NO improved condition

Course and Treatment Echo (POD-1): dilated RV, good LV contractility, TR with PG=23.8 mmHg, CVP= 17 mm Hg, no AR Impression: RV failure Op (POD-2): ASD fenestration and VSD fenestration

Course and Treatment– Echo on POD-23 RA, RV chamber enlargement ASD s/p repair and fenestration, 5 mm VSD s/p repair and fenestration, PG=21 mmHg s/p infundubulectomy, TAP of MPA No PG over RVOT or PV Severe PR, PG=5 mmHg Severe TR, PG=40 mmHg

Course and Treatment Lung edema, on diuretics Pleural effusion, frequently tapped Hepatomegaly (2-4 fb below RCM) Respiratory distress with skin mottling MRSE sepsis in 7/2001! Cath (7/6): RVOTO, large Qp/Qs CPR on 7/7/2001

Course and Treatment—Cath One Month After TC IVC 54.7% (55.1%) RA //19 (68.6%) LA //19 (87.5%) LV 90/19 (88.8%) SVC (55.4%) RPA 75/13/40 (79.1%) MPA 75/15/40 (80.5%) RV 75/16 (72.7%) LPA 31/16/24 (81.8%) MPA 75/16/37, distal RPA 40/15/26 Qp/Qs=3.8 VSD Juxta-ductal LPA stenosis (PG=44) Shunt-related RPA stenosis (PG=35) Severe PR Pulmonary HTN RVSP/LVSP=0.83

Re-Op-- 33 Days After TC VSD fenestration (post. inf) repair 1. LPA stenosis, 3 mm 2. MPA s/p TAP augmentation 3. RPA juxta-shunt stenosis, 3.5 mm VSD fenestration (post. inf) repair ASD fenestration downsize from 8 to 5 mm MPA to LPA patch augmentation RPA patch augmentation

Course and Treatment—Echo Two Month After TC Mild RA, RV chamber enlargement VSD, PG=31 mmHg Mild PS, PG=10 mmHg Moderate PR, PG=4 mmHg TR, PG=33 mmHg ASD, 1 mm Pleural and pericardial effusion

Course and Treatment Febrile pleural effusion Elevated CRP=7.83 Uncontrolled by specific antibiotics CT (8/2): loculated pleural effusion with atelectasis (L't 50 cc, R't 20 cc) Op (8/6): lung empyema debridement and adhesiolysis

Course and Treatment—Echo Nine Weeks After TC Enlarged LA, RA, and RV chambers PFO 2.7 mm VSD, repaired, PG=27 Moderate TR, PG=53 mmHg RVOT without significant stenosis Moderate PR, PG=9 mmHg Mild LPA stenosis, PG=12 mmHg Right pleural effusion, massive ascites

WBC

Worsening LVEF

McGoon Index

Case Summary: TOF Birth: cyanosis 1.5 m/o: Mod B-T shunt 3 m/o: PDA occluded 5.5 m/o: Shunt-dependent pulmonary flow 11.5 m/o: Emergent total correction 11.5 m/o: ASD and VSD fenestration for postop RV failure 1 y/o: VSD Qp/Qs=3.8, pulmonary HTN 1 y/o: Downsizing of ASD and VSD fenestration, re-augmentation of PA 1.2 y/o: RV failure (pleural effusion, ascites)

RV Failure After TOF Repair Discussion RV Failure After TOF Repair

Controversies Palliation or primary total correction? PFO and VSD sizes? A 2nd palliation rather than TC? Myocardium injury during resuscitation? Trans-annular patch and RV dysfunction?

Early TC Advantages Reduce hypoxia changes VSD closure: reduce late Cx (CHF, cerebral emboli/abscess, IE) Decrease RVP: reduce RVH Adequate PA flow: enhance growth Abolish progressive RVOT stenosis Separate MAPCA: reduce risk of PVRD Rudolph AM: Congenital Diseases of the Heart 2001: 544-5

Early TC Advantages: Avoid Complications of Palliation Reduce risk for high PVR due to shunt PA stenosis/distortion not problem Prevent shunt-related LV failure Rudolph AM: Congenital Diseases of the Heart 2001: 544-5

Early TC Disadvantages RVOT stenosis relief: inadequate PA size, large VSD L-R shunt Coronary artery crossing RVOT Trans-annular patch causes severe PR Palliation allows growth of pulmonary artery and annulus Rudolph AM: Congenital Diseases of the Heart 2001: 545-6

ASD and VSD Fenestration in TOF/PA Open VSD: markedly increases pulmonary blood flow -> sudden CHF and lung edema. PFO (ASD) in TOF: allows mild right-to-left shunt-> maintains systemic C.O. and not lead to RV failure. Mavroudis & Backer. Pediatric Cardiac Surgery, 2nd ed. Ch.21: 279-81

VSD Fenestration in TOF/PA RV Failure (RVP>LVP): VSD has to be fenestrated; closed later when PA growth suffices Residual VSD: poorly tolerated; must be closed if Qp/Qs>1.5. Mavroudis & Backer. Pediatric Cardiac Surgery, 2nd ed. Ch.21: 279-81, 287-9

RV Failure Cause: Trans-annular Patch (TAP)? Direct closure: less late adverse events, less PR, smaller RVEDD/LVEDD TAP & RV patch: long-term PR & RV dilatation Circulation.  102 (19 Suppl 3):III116-22, 2000

RV Failure Cause: Myocardial Injury Acute RV restriction after TOF repair: low CO, long ICU stay N=11, serial prospective, doppler echo Cardiac troponin T, NO-x, iron metabolism and antioxidants Restrictive (N=4) vs non-restrictive: longer ICU stay, higher trop-T, higher NO-x, higher iron loading, and less iron-binding antioxidant activity Circulation.  100(14):1540-7, 1999

What to Change Next Time? TOF/PA, McGoon index=1.3 Earlier total correction before B-T shunt occlusion ASD and VSD fenestrations at the total correction VSD closed later when PA larger or Qp/Qs>1.5

What Next for This Case? Trisomy 21 Clear consciousness Recurrent Infections Refractory RV failure Prolonged intubation Inotrope dependency Infection eradication Nutrition support Diuretics Ventilator weaning Re-operation

Thank You!