“The history, future treatment and

care of children born with single

ventricle hearts”

W.J. Brawn Cardiac Services

“However beautiful the strategy you should occasionally look at the results”

“Success is the ability to go from one failure to another with no loss of enthusiasm”

• 1947 early closed valvotomy series – 6 consecutive deaths

• Harken very depressed, went to bed.

• Cardiology friend Lawrence Brewster Ellis came to see him

and persuaded him to continue:

“He should not waste the experience of the 6 early deaths”

• Success followed

Dwight Harken

Interview with Gerald Rainer 2004

Transplantation• December 1967 first human

heart transplant – Dr. Christian Barnard

• 1971 146 of 170 transplants had died

• Dr. Norman Shumway of Stanford persisted with the introduction of cyclosporine (soil from Norway’s Hardangerfjord) opened a new era of heart transplantation

How has practice changed over the years?

0

50

100

150

200

250

1988 1989 1990 1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011*

Nu

mb

er

of

case

s

Specific Catheter Interventions

PDA occlusion ASD closure Stenting VSD closure Embolisation

0

20

40

60

80

100

120

140

160

1988 1989 1990 1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011*

Nu

mb

er o

f cas

es (p

er y

ear)

Univentricular Surgical Activity

Norwood Glenn Fontan/TCPC

Surgery for uni-ventricular hearts • Advent of prostaglandin, ICU resuscitation and support, the dominant

lesion has become Hypoplastic Left Heart Syndrome (HLHS) and it’s

variants

• These are systemic right ventricular circulation

Surgery for uni-ventricular hearts

Modified Blalock-Taussig shunt

Surgery for uni-ventricular hearts

The Fontan procedure

Surgery for uni-ventricular hearts

Evolution of the Norwood procedure

a) ‘Classical’ Norwood b) Left RV-PA Conduit c) Right RV-PA Conduit

Surgery for uni-ventricular hearts

The ‘Hybrid’ approach

Surgery for uni-ventricular hearts

Second stage palliation – the Glenn or CP shunt

Surgery for uni-ventricular hearts

Third stage palliation – the Fontan or TCPC

Surgery for uni-ventricular hearts

0

10

20

30

40

50

60

1988 1989 1990 1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011*

Nu

mb

er o

f cas

es (p

er y

ear)

Fontan / TCPC activity

>600 over 22 years

The BCH experience

• The uni-ventricular program began in 1992, since then:

• There have been 728 stage I procedures (the

Norwood operation)

• There have been 744 stage III operations (the

Fontan/TCPC operation)

Survival (days)

Pro

po

rtio

n a

live

0 30 60 90 120 180 240 300 360

0.0

0.2

0.4

0.6

0.8

1.0

206 183 180 174 166 163 158 155 146 143 136 134 132

Survival post Norwood

All Norwood procedures from 2008-2013

NHS Safe and sustainable programme

• Bristol - Kennedy Review 2001

• Monro - NHS Review 2003

• National Review of Experts 2006

• Medical Director NHS Sir Bruce

Keogh instigated Safe and

Sustainable review.

Working environment• There is a delicate balance between the courage

to perform complex risky surgery and the

humility to question one’s own ability on a day

to day basis. This is particularly so for the young

doctor.

• We work under the constant glare of public

scrutiny

Mentorship

• Powerful personal development and

empowerment tool

• Requires a close working partnership

• Requires mutual trust and respect

• Aim to challenge, motivate and inspire

Ott H.C. et al, (2008) Nat. Med.14: 213-21

LV assist devices

Finally

• First do no harm (Hippocrates)

• “We could do this procedure, but should we?” (Waterston)