Download Ann Mortell Memorial Lecture

Peter Lang, MD
Ann Mortell Lecture
9/29/2009
How Pediatric Cardiologists Think
(and Think Again)
Peter Lang, M.D.
Children’s Hospital Boston
September 30, 2009
Johnson City, Tennessee
Peter Lang, MD
Ann Mortell Lecture
9/29/2009
Peter Lang, MD
Ann Mortell Lecture
9/29/2009
Peter Lang, MD
Ann Mortell Lecture
9/29/2009
Peter Lang, MD
Ann Mortell Lecture
9/29/2009
Peter Lang, MD
Ann Mortell Lecture
9/29/2009
Peter Lang, MD
Ann Mortell Lecture
9/29/2009
Peter Lang, MD
Ann Mortell Lecture
9/29/2009
Peter Lang, MD
Ann Mortell Lecture
9/29/2009
Peter Lang, MD
Ann Mortell Lecture
9/29/2009
Peter Lang, MD
Ann Mortell Lecture
9/29/2009
Peter Lang, MD
Ann Mortell Lecture
9/29/2009
Peter Lang, MD
Ann Mortell Lecture
9/29/2009
Peter Lang, MD
Ann Mortell Lecture
9/29/2009
Peter Lang, MD
Ann Mortell Lecture
9/29/2009
Update on Diagnosis and
Treatment for Complex
Congenital Heart Disease
Late Complications of the Arterial Switch
Procedure and Their Management
Peter Lang, M.D.
Children’s Hospital Boston
May 23, 2007
Shanghai, China
Concerns in 1984
• Mortality
• Status of the brain
• Status of the coronary arteries
• Status of the pulmonary artery and aortic
anastamoses
• Status of the neo-aortic valve and aortic root
Peter Lang, MD
Ann Mortell Lecture
9/29/2009
Mortality
• Western Canada (1996-2004)
- Operative mortality
• Paris, France (1982-2000)
- Operative mortality
• Munich, Germany (1983-1997)
- Operative mortality
1/88 (1.1%)
106/1265 (8.4%)
6/285 (3.5%)
• European Congenital Heart
Surgeons Association (1998-2000)
- Operative mortality
37/613
(6%)
Peter Lang, MD
Ann Mortell Lecture
9/29/2009
Causes of Late Death
• 10
After reoperation (AI 3, caval
thrombosis 3, shunt 2, MR 1, DS 1)
• 9 Coronary related (MI 6, sudden 3)
• 5 Pulmonary
hypertension (despite
early surgery)
• 3 Ventricular dysfunction
• 5 Sepsis, LVOTO, heart block
Losay et al., Circ 2001
Risk of Late Death by
Multivariate Analysis
• Major ICU event
OR = 5.6
• Post ASO surgery
OR = 7.0
Losay et al., Circ 2001
Peter Lang, MD
Ann Mortell Lecture
9/29/2009
Causes of Reoperation
•
•
•
•
•
•
•
•
•
Pulmonary stenosis
Pacemaker
Residual shunt
Coarctation
Aortic insufficiency
Caval thrombosis
LVOT stenosis
Mitral insufficiency
Coronary stenosis
43
16
16
15
13
11
9
7
6
Losay et al., Circ 2001
Peter Lang, MD
Ann Mortell Lecture
9/29/2009
Last Follow-Up
• RV-PA pressure gradient
≥ 50mmHg
• AI, all grades
• AI, more than trivial
• Coronary obstruction
(278 angiograms)
• Normal LV function
Sinus rhythm
SVT
AV block
Pacemaker
41
165
39
(3.9%)
(15.5%)
(3.8%)
23
98.1%
98.1%
6
15
13
Losay et al., Circ 2001
Status of the Brain
General Health Status of Children With
D-Transposition of the Great Arteries
After the Arterial Switch Operation
Dunbar-Masterson et al., Circ 2001
“Physical and psychosocial health status in
children with D-TGA repaired by the arterial
switch operation in infancy is similar to that of
a normative sample.”
Peter Lang, MD
Ann Mortell Lecture
9/29/2009
Status of the Brain
• Mental delay
• Motor delay
• Mental and/or motor delay
14/82 (17.1%)
5/82
(6.1%)
15/82 (18.3%)
“Mental delay was 7.5 times and motor delay
was 2.7 times more frequent than expected
based on population norms.”
Freed et al., JTCVS 2006.
Status of the
Coronary Arteries
• 1/3 of late deaths coronary related
• 1-2% of patients have poor LV function
• 5-10% of patients had coronary
abnormalities during the “learning
curve”
• Now, only selected patients have
coronary angiography
Peter Lang, MD
Ann Mortell Lecture
9/29/2009
Status of the
Coronary Arteries
Intracoronary Ultrasound Assessment
Late After the Arterial Switch Operation
for Transposition of the Great Arteries
Pedra et al., JACC 2005
22 children, unselected, median age 8.6
years, underwent coronary angiography and
IVUS.
Status of the
Coronary Arteries
22 patients, all asymptomatic, no risk factors
for CAD, normal LV function by ECHO.
7 with RVOTO > 50mmHg
13 with LVEDP > 12mmHg
1 with RCA ostial narrowing
Pedra et al., JACC 2005
Peter Lang, MD
Ann Mortell Lecture
9/29/2009
Status of the
Coronary Arteries
IVUS:
• Performed in 20 of 22
• Proximal eccentric intimal thickening seen
in 89% of studied arteries
• 30% of vessels with significant (>0.3mm)
proliferation
Pedra et al., JACC 2005
Status of the
Coronary Arteries
Speculation:
1. “Reimplantation of the coronary arteries result in
proximal flow disturbance which may alter shear
stress and induce lateral thickening.”
Pedra et al., JACC 2005
2. Denervation may alter vascular function.
3. Hypertrophy due to residual lesion has adverse
effects on coronary flow reserve.
Peter Lang, MD
Ann Mortell Lecture
9/29/2009
Status of the
Coronary Arteries
Detection of Coronary Complications After the
Arterial Switch Operation for Transposition
of the Great Arteries: First Experience with
Multislice Computer Tomography in Children
Ou et al., JTCVS 2006
• Comparison of 49 patients using angiography and
MSCT.
• All patients clinically well undergoing “routine.”
Status of the
Coronary Arteries
• 4/49 patients (8.1%) had significant stenoses
(1 ostial, 3 proximal)
• Angiography and MSCT had perfect agreement
• MSCT may be superior to conventional
angiography demonstrating the mechanism of
stenosis such as compression
• But: radiation, heart rate, and sedation
problems
Ou et al., JTCVS 2006
Peter Lang, MD
Ann Mortell Lecture
9/29/2009
Status of the Pulmonary
and Aortic Arteries
1. The aortic anastomosis virtually never causes
a problem with obstruction, but may contribute
to aortic valve insufficiency.
2. The pulmonary artery anastomosis remains
a. the most common cause for reoperation
b. unchanged in frequency of construction
since the introduction of the Lecompte
maneuver.
Status of the Pulmonary
and Aortic Arteries
Technique for pulmonary artery reconstruction
1. Direct pulmonary artery anastomosis
• Little early obstruction
• 5% late obstruction
2. Two free periocardiac patches
• 5% early obstruction
• 5% late obstruction
3. “Pantaloon-shaped” periocardial patch
• 5% early obstruction
• 5% late obstruction
Hass et al., Ann Thorac Surg, 1999
Peter Lang, MD
Ann Mortell Lecture
9/29/2009
Status of the
Conduction System
• The promise of superiority to the Mustard
and Senning procedures has been
fulfilled.
• Expected incidence of pacemakers
required for complete heart block and
sinus node dysfunction.
Status of the Neo-Aortic
Valve and Aortic Root
1156 patients followed for a median of 75
months
•172
• 104
• 43
• 19
• 6
(14.9%)
(9.0%)
(3.7%)
(1.6%)
(0.5%)
All aortic regurgitation
Trivial
Mild
Moderate
Severe
Losay et al., JACC 2006
Peter Lang, MD
Ann Mortell Lecture
9/29/2009
Status of the Neo-Aortic
Valve and Aortic Root
Predictive of Aortic Regurgitation
Univariate
• Complex TGA
• Prior PA band
• CoA or IAA
• Pre-ASO Ao >> PA
• Older age at ASO
• AR at hospital discharge
Multivariate
• VSD
• AR at hospital discharge
Losay et al., JACC 2006
Peter Lang, MD
Ann Mortell Lecture
9/29/2009
Status of the Neo-Aortic
Valve and Aortic Root
Reoperation
• 16/1156 patients (1.4%)
- 3 Aortic vaculoplasty
- 9 Aortic valve replacement
- 4 Bentall procedures
Late Complications of the
Arterial Switch Procedure
Summary
1. Mortality, early and late, is low.
2. Neurologic and developmental problems remain
a problem, but results are improving.
3. The coronary artery story has not yet been told.
4. Pulmonary artery stenosis remains a real
problem which places patients at risk secondary
to reoperations.
5. The conducting system is not an independent
problem.
6. The aortic valve and aortic root are likely to be a
persistent but uncommon problem, requiring
surgical reintervention.
Peter Lang, MD
Ann Mortell Lecture
9/29/2009
Peter Lang, MD
Ann Mortell Lecture
9/29/2009