Update from Columbus on the Hybrid Approach for HLHS and Complex Single Ventricles

Transcription

1 Update from Columbus on the Hybrid Approach for HLHS and Complex Single Ventricles Tanya Maria Kempton MSN RN CPN CNL SPCN Educational Meeting November 3, 2012 Los Angeles, CA

2 Background Treatment of HLHS neonates remains challenging and controversial Two conventional, comprehensive surgical options require CPB: Norwood (modified BT shunt) (64 %) Sano (RV-PA shunt) (74 %) Ohye, et al. (Pediatric Heart Network) reported the 12 month transplant free survival rate for each approach Surgical results are variable by institution for morbidity and mortality

3 Hybrid History The Giessen Germany Pediatric Heart Center Stenting of the Arterial Duct and Banding of the Pulmonary Arteries: Basis for Combined Norwood Stage I and II Repair in Hypoplastic Left Heart Hakan Akintuerk.Dietmar Schranz Circulation 2002; 105: Journal of the American Heart Association

4 Why Do It? Drs. John P. Cheatham and Mark Galantowicz came together in Orlando in 1999 Results for Norwood were good but looking at published neurodevelopmental outcomes they asked themselves: is there a better way? HLHS is the WORST! What about the high risk patients? e.g. Extreme low birth weight, extremis presentation, poor RV fx, bad TR, etc What about the borderline, small LV patients? What about the high risk 2 ventricle patients? What about a bridge to heart transplant?

5 Drs. John P. Cheatham and Mark Galantowicz

6 Hybrid Concept of HLHS Repair Initial palliation with less invasive procedure (Hybrid Stage I) in neonatal period to stabilize to an age and weight appropriate for the big operation Control & protect pulmonary blood flow (LPA/RPA bands) Provide reliable systemic cardiac output (PDA stent) Create unobstructed flow from LA (BAS/stent IAS) One comprehensive open heart procedure (combine stage I, II and part of III), flanked by two less invasive procedures Develop a way to less invasively complete the Fontan circuit (Covered Stent Availability)

7 HLHS Hybrid Staged Palliation Hybrid Stage I Comprehensive Stage II Fontan

8 Goals of Hybrid Stage I Palliation Reduce morbidity & mortality Reduce cumulative impact of multiple interventions Deliver more efficient & cost effective care Improve neurodevelopmental outcome and quality of life

9 Cardiac Catheterization Suite

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11 Case Scenario (Part I and II) B.B. 6 day old male Wt. 3.4kg Ht. 51.5cm DX: HLHS variant mitral stenosis & severe Aortic stenosis Procedure: Bilateral PA bands & PDA stent Admitted per protocol to CTICU B.B. 13 day old male DX: HLHS variant s/p Hybrid stage I palliation Procedures: 12mm Stent Retrograde Aortic arch Balloon Atrial Septostomy (BAS) Admitted per protocol to CTICU

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13 Comprehensive stage II Remove RPA & LPA Bands Remove PDA stent Cavo-pulmonary anastomosis Repair pulmonary arteries if necessary D-K-S anastomosis Aortic Arch reconstruction Atrial Septectomy

14 Exit Angiography 2009 Described the importance of Completion Exit Angiography in the Hybrid Operative Suite In 32 cardiac procedures over 11 months 13/32 after Comprehensive Stage II Unexpected pathology identified 18/32 (56.3%) 9/32 (28.1%) therapy changed Complications 1/32 staining of LPA Holzer, R. J., et al. "Completion Angiography After Cardiac Surgery for Congenital Heart Disease: Complementing the Intraoperative Imaging Modalities." Pediatric Cardiology 30.8 (2009)

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17 What Have We Done? 2003 Described the early strategies for treating HLHS using a new Hybrid approach combining transcatheter & surgical techniques though all 3 stages, including PA Flow Restrictors & transcatheter Fontan Emerging Strategies in the Treatment of HLHS: Combined Transcatheter and Surgical Techniques Sharon L. Hill, ACNP, Mark Galantowicz, John P. Cheatham Pediatric Cardiology Today, 2003,1(3), Described the early results of the Hybrid Stage I approach to HLHS (Aug 2001-Dec 2004) in the first 29 pts. ( kg). Covered stent transcatheter Fontan completion is described. Interstage monitoring initiated after first 10 patients. Lessons Learned from the Development of a New Hybrid Strategy for the Management of Hypoplastic Left Heart Syndrome M. Galantowicz, J.P. Cheatham Pediatr Cardiol 2005, 26:

18 Columbus Updates 2007 A case report of the successful Hybrid Stage I palliation in a complex 1.4 kg premature neonate. Palliation via Hybrid Procedure of a 1.4-kg Patient with a Hypoplastic Left Heart Clifford L. Cua, MD, et al. Congenit Heart Dis., 2007;2: The landmark manuscript describing the intermediate results of the Hybrid approach to HLHS after the learning curve. In 40 patients with standard risks, the actuarial survival was 87% after Stage I, Interstage, and Comprehensive Stage II Hybrid Approach for Hypoplastic Left Heart Syndrome: Intermediate Results After the Learning Curve M. Galantowicz, MD, John P. Cheatham, Alistair Phillips, MD, Clifford L. Cua, MD, Timothy M. Hoffman, MD, Sharon L. Hill, ACNP, and Roberta Rodeman, RN. Ann Thorac Surg 2008;85;

19 Columbus Updates 2008 Described the challenges and results of atrial septal interventions after the Hybrid approach to HLHS 56 (July Sept 2007) patients major AE occurred in 8.9 % with 10.7 % have an intact atrial septum Hybrid Approach for Hypoplastic Left Heart Syndrome: Intermediate Results After the Learning Curve Mark Galantowicz, MD, John P. Cheatham, Alistair Phillips, MD, Clifford L. Cua, MD, Timothy M. Hoffman, MD, Sharon L. Hill, ACNP, and Roberta Rodeman, RN. Ann Thorac Surg 2008;85; Described the echo features during the interstage period after Hybrid Stage I palliation significant changes include: LPA & RPA band gradients increased PDA & RAA gradients increased-improved after intervention Mean ASD gradients increased-improved with intervention Pre- Comprehensive Stage II RV fx tended to predict death Interstage Echocardiographic Changes in Patients Undergoing Hybrid Stage I Palliation for Hypoplastic Left Heart Syndrome Bernadette Fenstermaker, RDCS et al. J Am Soc Echocardiogr 2008; 21,

20 Columbus Updates 2009 Described incidence of RAA obstructions in 66 consecutive patients after Hybrid Stage I palliation: 24% developed RAAO: Rx coronary stent or Reverse BT shunt (3) After 2 years, survival reduced in pts with RAAO (56.3%) vs no RAAO (88%) If RAAO at birth or early interstage, Norwood favored The Retrograde Aortic Arch in the Hybrid Approach for Hypoplastic Left Heart Syndrome Serban C. Stoica, MD, Alistair B. Philips, MD, Matthew Egan, MD, Roberta Rodeman, RN, Joanne Chisolm, RN, Clifford L. Cua, MD, Sharon Hill, ACNP, John P. Cheatham, MD and Mark Galantowicz, MD. Ann Thorac Surg 2009;88; Described predictors of RAAO by reviewing echos and angiographies in 96 patients who underwent Hybrid Stage I between July 2002 and July 2009; 68 patients met inclusion criteria for standard HLHS: 29 % developed RAAO Mean Ao root size smaller Angle of Ao isthmus arising from PDA larger Predictors of Retrograde Aortic Arch Obstruction after Hybrid Palliation of Hypoplastic Left Heart Syndrome Matthew Egan, MD, Sharon L. Hill, ACNP, Bethany L. Boettner, Ralf J. Holzer, MD, Mark Galantowicz, MD, John P. Cheatham, MD and John P. Kovalchin, MD. Pediatr Cardiol 2011, 32;

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22 Hybrid Stage I Retrograde AAO

23 Columbus Updates 2009 A journal article highlighting and detailing the development of the technique in over 100 children. The Hybrid Approach to Hypoplastic Left Heart Mark Galantowicz, MD, in Operative Techniques in Thoracic and Cardiovascular Surgery: A Comparative Atlas. 2009; Described the anesthetic management during Hybrid Stage I in 77 patients: After PAB- increase SBP 11mmHg, decrease O2 sats 7% 64.9% extubated within 24 hours 73% received no blood transfusion Fentanyl used for analgesia Anesthetic Management of the Hybrid Stage 1 procedure for Hypoplastic Left Heart (HLHS) Aymen N. Naguib, MD, Peter Winch, MD, Lawrence Schwartz, MD, Janet Isaacs, CRNA, Roberta Rodeman, RN, John P. Cheatham, MD, and Mark Galantowicz, MD. Pediatric Anesthesia, 2010, 20;

24 Columbus Updates 2010 Described adverse events and procedural characteristics from the multicenter C3PO registry between Feb 2007 and Dec 2008 in 7 institutions: 128 Hybrid procedures (1-89) / 7019 caths 60 % SV circulation 15/128 (12%) had 16 AE Trivial (9), Moderate (5), Major (1), Catastrophic (1) 56% not preventable, 38% possibly preventable, and 6% preventable PDA stent by Hybrid 10% AE vs percutaneous (80%) LIMITATION: capture of hybrid procedures was incomplete because of differences in definitions-in the future categorize hybrid procedures into procedural groups Hybrid Procedures: Adverse Events and Procedural Characteristics---Results of a Multi-Institutional Registry Ralf Holzer, MD et al. Congenit Heart Dis 5;2010:

25 Columbus Updates 2010 Described echo parameters that may predict outcome in 34 patients with aortic atresia and HLHS undergoing Comprehensive Stage II: The Echocardiographic Parameters that Predict Outcome in Aortic Atresia Patients Undergoing Comprehensive Stage II Procedure Brian Birnbaum, MD et al. Congenit Heart Dis, 2010, 5; Described the incidence of NEC in 73 patients after Hybrid Stage I palliation: 8/73 developed NEC 3/8 died, 2 patients required abdominal surgery Earlier gestational age (<37 weeks), lower max PGE1dose, and unexpected readmission to CTICU were statistically associated with NEC Demographic, perinatal, periop, clinical, or procedural variables were not associated with NEC (enteral feeding, UAC lines, inotropes, avg O2 sats, diastolic pressures) Incidence of NEC post-hybrid Stage I similar to Norwood Necrotizing Colitis in Neonates Undergoing the Hybrid Approach to Complex Congenital Heart Disease Wendy E. Luce et al.,pediatr Crit Care Med, 2011,12(1);

26 Columbus Updates Pre-publication: Describes the weight gain of HLHS patients, who underwent the Hybrid Stage I procedure, during the interstage (IS) period. Goals: 1. To determine if weight parameters were associated with mortality 2. To determine if home monitoring improved weight gain Conclusions: Home monitoring improved IS weight gain in babies undergoing Hybrid Stage I Decreased weight gain associated with IS mortality Interstage Weight Gain for Patients with Hypoplastic Left Heart Syndrome Undergoing the Hybrid Procedure Holly Miller-Tate, RN et al.

27 Post-Operative Care after Hybrid Stage I Medications: Inotrope (epi, milrinone) rare ASA, lasix + digoxin + enalapril if valve regurgitation or hypertension Nutrition consult: Inpatient Followed concomitantly in cardiology clinic OT consult for feeding problems PT consult for development

28 Post-Operative Care after Hybrid Stage NCH Columbus July Jan 2012 N=133 (HLHS; complex single ventricle) After the learning curve Excluded deaths and patients transferred Data available for analysis n=82 Median Hybrid 7 days; median weight 3.0kg 15/82 (18%) intubated prior to Hybrid Stage I 46/82 (56%) extubated in Hybrid suite Median ICU LOS 5 days (1-55 days) Median hospital LOS 14 days (4-133 days) Median enteral feed initiated POD #2 I

29 Post-Operative Care after Hybrid Stage I Home Monitoring: Crucial to overall success Clinic visit every 1-2 weeks with: Physical Exam-VS and O2 sat Echocardiogram* ECG Neurodevelopmental follow-up: TIMP (Test of Infant Motor 2 and 4 months of age Assesses motor performance Bayley Scales 5-6 months of age prior to Comprehensive Stage II gross & fine motor skills receptive & expressive language cognitive

30 Columbus Updates Abstracts 2012 Home monitoring program and risk factors during Interstage follow-up after Hybrid Stage I palliation: The Impact of Interstage Monitoring after Hybrid Palliation of Hypoplastic Left Heart Syndrome Nazia Husain, MD et al. Risk Factors for Interstage Mortality in HLHS Nazia Husain, MD et al.

31 Columbus Home Monitoring Daily weight and oxygen saturation, monitoring of enteral intake volume and calories and weekly telephone calls from nursing with defined minimum criteria for breach Breach criteria included: Poor weight gain (< 0.01kg/day) or weight loss over 3 days Poor oral intake (<100ml/kg/d) or vomiting > 2 times over 24 hours Oxygen saturation < 75% or breathing faster/harder Inconsolable fussiness or other concerns June 2008 Enrolled first patient (incl. shunt dependent patients) Patients followed outside our institution: North Carolina, Texas, Toledo, West Virginia, Wisconsin, Florida, California

32 Clinical Management Team member of the JCCHD NPC-QIC initiative to establish Good Clinical Practice Guidelines Anticoagulation protocol after Comprehensive Stage II with suspected/documented stenosis/thrombus Angiojet in Hybrid cath/o.r. Suite IV Heparin & Discharge Lovenox x 6 weeks Now with feeding protocol after BAS before discharge NPO for 24 hours in CTICU, then slow increase in volume and concentration Organizing a Single Ventricle Clinic

33 Other Columbus Initiatives Single Ventricle Work-Groups -Following protocols to improve outcomes Imaging Nutrition Communication CTICU Feeding Guidelines-procedure specific Rooming-In Neurodevelopmental Outcomes Study

34 Hybrid Stage I: Impact on Cerebral Blood Flow Advantages avoid DHCA as neonate avoid CPB (ACP or full flow) as neonate Diminish the systemic inflammatory response from CPB Diminish risk of reperfusion injury Unknown contributing factors Pre-procedure brain imaging: PVL, white matter injury Cerebral ischemic or embolic event Questions to be answered Is there adequate cerebral oxygenation for brain growth & development? Do outcomes differ in HLHS with aortic atresia? When is it necessary to treat retrograde aortic arch gradient? Is there adequate cerebral perfusion during interstage period?

35 Neurodevelopmental Outcomes Study Designed to examine neurodevelopment and test the overall working hypothesis that there is a correlation between cerebral blood flow and neurodevelopmental outcome in infants after Hybrid Stage I palliation for HLHS. Assessment of neurodevelopment outcomes and Transcranial Doppler studies in patients undergoing Hybrid Stage I palliation vs normal controls Baseline 2 month & 4 month post palliation, and pre- Comprehensive Stage II repair

36 How Do You Assess A New Technique? If there is a suboptimal outcome with a new procedure, performed on a high risk patient; is it because of: Poor patient selection High risk patients at greater risk A learning curve phenomena Or is it a bad technique or bad operators? Remember going from Mustard/Senning back to the Jatene Arterial Switch Remember the TAVI beginning Remember the Norwood early years

37 Hybrid Approach to HLHS CONCLUSIONS 2 independent centers have published similar results all within a short learning curve What were the results of the Norwood in the 1 st 10 years? The procedure can be applied to the high risk neonate with complex 1 and 2 ventricle repair, regardless of size or sickness Also applied to the borderline LV, transplant candidate The main unanswered question and potential drawback: What happens to the brain with an additional 6 months of RAA flow?

38 Hybrid Approach to HLHS CONCLUSIONS A comprehensive multi-institutional trial comparing Norwood, Sano, and Hybrid palliations & outcomes should be performed Congratulations to Toronto Sick Kids for leading the way Offering the Hybrid Stage I palliation as an alternative to conventional surgical repair is justified and will allow improvement in the techniques required by the surgeon, interventionalist, and all of the team members required to care for these complicated and fragile patients

39 Are We Satisfied? THE QUESTIONS: 1. Should we be satisfied with the recent results from the PHN SVRT reported by Rick Ohye representing a modern era of traditional surgery for HLHS? ---Transplant-free survival after 14 mos: N- 63%, S- 67% Should we be satisfied with the neuro-cognitive outcomes in babies with HLHS? 3. Should we continue to keep an open mind to other therapies and not make biased and judgmental decisions of therapy? THE FUTURE: All patients undergoing successful Hybrid Stage I palliation, Comprehensive Stage II repair, and Fontan completion to return for brain and heart MRI, neurodevelopmental testing, and TCD scheduled for

40 International Symposiums on the Hybrid Approach to Congenital Heart Disease

41 Thank you! Questions?

42 Nationwide Children s Hospital At a glance Non-profit free-standing Children s Hospital Founded 1892 Serves all children regardless of families ability to pay America s 5th largest Children s Hospital Top 10 Research Center 1 million patient visits/admissions annually Referrals from all 50 states and 35 countries 7,500 employees Dept. of Pediatrics for The Ohio State University