Bilateral Pulmonary Artery Banding for Resuscitation in High-Risk Single

Ventricle Neonates & Infants: Single Center Experience

KJ Guleserian, MD1, GM Barker, MD2, MS Sharma, MD1, J Macaluso, RN1, AW Nugent, MBBS2, JM Forbess MD1

1Division of Pediatric Cardiothoracic Surgery, 2Division of Pediatric Cardiology

Children’s Medical Center/UT Southwestern Medical Center, Dallas, TX

No Relationships to DiscloseNo Relationships to Disclose

BACKGROUND

• Operative mortality for the Norwood procedure

continues to improve for patients with HLHS or

other complex SV lesions with systemic outflow

tract obstruction.tract obstruction.

• As low as 10-20% in some centers.• Tweddell et al, Circulation 2002

• Karamichalis et al, Ann Thorac Surg 2010

BACKGROUND

• Mortality of 20-70% in “high-risk” patients with intact/restrictive atrial septum, pre-op shock, renal failure, small ascending aorta, prematurity, low birth-weight, ventricular dysfunction, significant AVVR, and/or extracardiac syndrome.and/or extracardiac syndrome.

• Gaynor et al, Eur J Cardiothorac Surg 2002

• Stasik et al, J Thorac Cardiovasc Surg 2006

• Lim et al, Pediatr Cardiol 2006

• Not only for first-stage palliation but also for those awaiting transplantation.

BACKGROUND

• Reduction in pulmonary blood flow by bPAB first reported for HLHS as an alternative to Norwood procedure in 1993.

• Gibbs et al, Br Heart J 1993

• Early success with hybrid palliation has led to its application for “high risk” HLHS and other SV lesions to optimize Qp:Qs imbalance and improve surgical outcomes.

• Galantowicz & Cheatham, Pediatr Cardiol 2005

• Pizzaro et al, Eur J Cardiothorac Surg 2008

PURPOSE

• To determine outcomes with bPAB and DS or PGE1

infusion in our “high-risk” neonates and infants

with HLHS or other complex SV lesions as a means

of resuscitation and stabilization to conventional of resuscitation and stabilization to conventional

Norwood palliation or primary transplantation.

• To identify risk factors for hospital death.

METHODS

• Retrospective review

• Children’s Medical Center Dallas

• January 2007-October 2011

• All patients with single ventricle (SV) < 3 months • All patients with single ventricle (SV) < 3 months

• bPAB & ductal stenting (DS) or PGE1 infusion

METHODS

• Echocardiographic, angiographic, operative and clinical data were reviewed.

• Hemodynamic, fluid balance and laboratory values were assessed immediately pre-bPAB and at 24 were assessed immediately pre-bPAB and at 24 hours and 4 days post-bPAB.

• End points included conventional Norwood, primary transplantation or hospital death.

Sebastian et al, J Card Surg 2010;25:596-600.

“High-Risk”

SV Patients

N=24

HLHS/ Unbalanced Tri. Atresia HLHS/

variant HLHS

N=18

Unbalanced

AVC

N=4

Tri. Atresia

w/ LVOTO

N=2

HLHS N=14

MS/AS 9

MS/AA 3

MA/AA 2

HLHS/variant HLHS (N=18, 75%)

Variant HLHS N=4

MA/AS/VSD w/ type B IAA 1

Heterotaxy w/ mesocardia,

primitive single ventricle, TAPVC,

RAA/vascular ring, interrupted

IVC/azyg. cont.

1

Dextrocardia w/ HLV, LVOT

hypoplasia & straddling TV

1

DORV/MA w/ AS 1

Other Complex SV

Unbalanced AVC N=4

RV dominant w/ RAA/vascular ring,

bilateral SVC, heterotaxy

1

RV dominant w/ CoA, TAPVC 1

RV dominant w/ Ao atresia,

hypoplastic Ao arch, ALCAPA, Scimitar

1

hypoplastic Ao arch, ALCAPA, Scimitar

syndrome, heterotaxy

LV dominant w/ CoA, hypoplastic Ao

arch, Trisomy 21

1

Tricuspid atresia/LVOTO N=2

Heterotaxy w/ mesocardia, D-TGA,

aortic atresia, RAA

1

D-TGA, CoA, hypoplastic Ao arch 1

RESULTS: DemographicsN=24

Gender (% male) 13 (54.2%)

Gestational age (wk) 38 (27-41)

Birth-weight (kg) 3.02 (0.9-4.1)

Age at bPAB (days) 8 (2-44)Age at bPAB (days) 8 (2-44)

Weight at bPAB (kg) 3.01 (1.5-4.4)

Prenatal diagnosis 11 (45.8%)

PDA stent 14 (58.3%)

PGE1 infusion 10 (41.7%)

RESULTS: High-risk CriteriaExtracardiac syndrome 7 (29.2%)

Heterotaxy 4 (16.7%)

Birth-weight < 2.5 kg 4 (16.7%)

Gestational age ≤ 36 wk 6 (25%)

IAS/RAS in HLHS/variant HLHS 9/18 (50%)

Metabolic acidosis (pH < 7.2) 7 (29.2%)

pH < 7.0 5 (20.1%)

AVVR (≥ moderate) 7 (29.2%)

Renal insufficiency/failure 6 (25%)

Poor ventricular fxn 5 (20.1%)

Cardiac arrest 3 (12.5%)

ECMO support 2 (8.3%)

CVA/seizures 2 (8.3%)

“High Risk” bPAB

N=24

HLHS/

variant HLHS

Unbalanced

AVC

Tri. Atresia

w/ LVOTOvariant HLHS

N=18

AVC

N=4

IAS

N=3*

w/ LVOTO

N=2

RAS

N=6

*Open atrial septostomy (N=1)

Transcather atrial septostomy (N=8)

Atrial stent placement (N=4)

“High Risk” bPAB

N=24

PDA Stent

N=14

PGE1

N=10N=14 N=10

N=1

Pre-bPAB

N=6

Post-bPAB

N=7

Simultaneous

RESULTS: Hemodynamic, Fluid Balance, Lab Data

* P < 0.05

** p < 0.01

*** p < 0.001

⌘Patients on pre/post ECMO support excluded

N=24

“High Risk”

N=1

Attempted 2V

N=7 N=9N=1

N=7

Hospital

Death

N=7

Conventional

Norwood

N=9

Listed for

TXPLT

N=1

Comprehensive

Stage 2

N=7

TXPLT

N=2

WL Death

RESULTS:Time Banded to Surgical Palliation or Transplant

Procedure Time Banded (days)

Norwood (N=7) 8 (4-78*)

CS2 (N=1) 118

Transplant (N=7) 89 (21-201)

*Trisomy 21, unbalanced AV canal/CoA, HAA � planned 2V

RESULTS: Hospital Deaths

• 9/24 (37.5%)

– Post bPAB arrest (N=3)

• Ascending Ao < 1.5mm (N=2, incl. ex 33 wk, 2.3 kg)

• Respiratory acidosis (N=1, ex 33 wk, 1.8 kg)

• 2/3 ECMO support• 2/3 ECMO support

– Absolute contraindication to transplant (N=3)

• Chronic renal failure (N=1)

• Scimitar/hypoplastic right lung (N=1)

• Uncontrolled sepsis (N=1, ex 27 wk, 900 gm)

RESULTS: Hospital Deaths

– Developed contraindication to transplant (N=2)

• UNOS status 7 = waitlist death = hospital death

• Recurrent sepsis w/ renal failure (N=1)

• Chronic lung disease (N=1, ex 35 wk, 1.6 kg)• Chronic lung disease (N=1, ex 35 wk, 1.6 kg)

– Palliative care per family (N=1)

RESULTS: Risk Factors for Hospital Death

Gestational age P=NS

Gestational age ≤ 36 wk P=NS

Birth-weight P=0.055

Birth-weight < 2.5 kg P< 0.0001

IAS/RAS P=NS

Age at bPAB P=NS

Age at Norwood, CS2 or transplant P=NSAge at Norwood, CS2 or transplant P=NS

Metabolic acidosis P=NS

≥ moderate AVVR P=NS

Poor ventricular function P=NS

Renal insufficiency/failure P=NS

Cardiac arrest P=NS

Neurologic deficit (CVA/seizures) P=NS

ECMO P=NS

Extracardiac syndrome P=NS

RESULTS: Risk Factors for Hospital Death

Gestational age P=NS

Gestational age ≤ 36 wk P=NS

Birth-weight P=0.055

Birth-weight < 2.5 kg P< 0.0001

IAS/RAS P=NS

Age at bPAB P=NS

Age at Norwood, CS2 or transplant P=NSAge at Norwood, CS2 or transplant P=NS

Metabolic acidosis P=NS

≥ moderate AVVR P=NS

Poor ventricular function P=NS

Renal insufficiency/failure P=NS

Cardiac arrest P=NS

Neurologic deficit (CVA/seizures) P=NS

ECMO P=NS

Extracardiac syndrome P=NS

RESULTS: Follow-up

• N=15 (62.5% survival)

• Median of 1.58 yrs (0.34-4.36)

• 6/7 (85.7%) of Norwood pts � BDG

– LPA plasty (N=2)– LPA plasty (N=2)

• 6/7 (85.7%) of transplanted pts are alive at median

follow-up 33.6 mos

– B PA plasty (N=3), RPA plasty (N=1) at transplant

– LPA plasty (N=1)

RESULTS: Late Deaths

• N=4 (4/15, 26.6%)

– Respiratory failure 3 mos post transplant (N=1)

• Right-sided PV stenosis, LPA stenosis

– Influenza B/PA thrombosis 1 mos post CS2 (N=1)– Influenza B/PA thrombosis 1 mos post CS2 (N=1)

– RSV bronchiolitis 2.5 yrs post BDG (N=1, Trisomy 21)

– Aspiration at home 2 mos post Norwood (N=1, mosaic

Turner’s) = inter-stage death

LIMITATIONS

• Retrospective

• Non-randomized

• Small number of patients

• Heterogeneous population• Heterogeneous population

• Limited follow-up

CONCLUSIONS

• bPAB and maintenance of ductal patency with

either DS or PGE1 is an effective means of

resuscitation for “high-risk” single ventricle

neonates and infants. neonates and infants.

• This strategy allows for reasonable survival to

either conventional Norwood palliation or primary

transplantation when appropriate.

CONCLUSIONS

• DS in the presence of a diminutive ascending aorta may compromise coronary perfusion and should be avoided.

• Branch PA intervention is more frequent in patients banded for longer periods of time.banded for longer periods of time.

• Early conventional Norwood is preferred when possible and thorough branch PA assessment made at transplantation.

CONCLUSIONS

• Birth-weight < 2.5 kg continues to be a significant

risk factor for hospital death.

N=24

“High Risk”

N=1

Attempted 2V

N=7 N=9N=1

N=9

Hospital

Death

N=7

Norwood/

DKS

N=9

Listed for

TXPLT

N=1

Comprehensive

Stage 2

N=7

TXPLT

N=2

WL Death

N=24

“High Risk”

N=1

Attempted 2V

N=7 N=9N=1

N=7

Hospital

Death

Figure 1

N=7

Norwood

N=9

Listed for

TXPLT

N=1

Comprehensive

Stage 2

N=7

TXPLT

N=2

WL Death

N=6

BDG

N=1

Inter-stage

Death

Sebastian et al, J Card Surg 2010;25:596-600.