Download The Dilated Pulmonary Artery: Is there a risk of Dissection?

The Dilated Pulmonary Artery:
Is there a risk of Dissection?
Pastora Gallego, MD, PhD
Inter-center Adult Congenital Heart Disease Unit
Area del Corazón
Seville, Spain
No conflict of interest to disclose
Background
• Pulmonary Artery Aneurysm (PAAn)
uncommon finding, normal reference range for diagnostic radiology
and cut-off values vary between studies
• Spontaneous Pulmonary Artery Dissection (PAD)
extremely rare, nonspecific clinical presentation, typically diagnosed at
postmortem examination rather than during life. Low incidence or
infrequently diagnosed?
• No guidelines for best approach
heterogeneous population, descriptive data, small series limit external
validity and generalizability of the published studies
Flowchart of the study population
Database: 1587 adult patients with CHD
Simple 29%
Moderate 50%
Complex 22%
Excluded
395 patients
No chest X-ray available
1192 adult patients with CHD and chest X-ray
Flowchart of the study population
Database: 1587 adult patients with CHD
Excluded
395 patients
No chest X-ray available
Simple 29%
Moderate 50%
Complex 22%
1192 adult patients with CHD and chest X-ray
No PA Dilatation
Chest radiograph
944 patients
PA Dilatation
Chest radiograph
248 patients
None n = 397 (42%)
Echo n = 105 (11%)
CT or MRI n = 442 (46%)
None n= 0
Echo n = 92 (37%)
CT or MRI n = 156 (63%)
PA Measurement
Prevalence of PA Aneurysms in CHD
PAAn is defined as PA diameter > 40 mm by CT or MRI
• 248 PA dilatation (21%)
– Median PA diameter 35 mm
• 66 (6%) PA Aneurysms
– Median PA diameter 44 mm (IQ 40-50)
– 21 (<2%) PA diameter > 50 mm
Underlying Lesion
N (% PAA)
PAH (n)
SHUNTS
36 (55%)
30 (83%)
ASD
14 (21%)
12 (85%)
VSD
11 (17%)
11 (100%)
ASVD
5 (8%)
4 (80%)
PDA
2 (3%)
2 (100%)
PAPVC
1 (1.5%)
1 (100%)
Coronary Fistulae
DORV + PDA
1 (1.5%)
14 (21%)
T. FALLOT
12 (18%)
T. FALLOT
9 (14%)
ABSENT PV
3 (4.5%)
43 mm
(IQ 40-50.25)
2 (3.0%)
2 (100%)
1 (1.5%)
1 (100%)
IDPA
2 (3%)
1 (50%)
COARCTATION + BAV
1 (1.5%)
1 (100%)
TGA+ VSD
Distribution by diagnosis
1 (1.5%)
PV STENOSIS
TGA
PA Dimensions in CHD
PA Aneurysms
47 mm
(IQ 40-48)
41 mm
42.5 mm
(IQ 40-50)
47.5 mm
(IQ 40-54.75)
Prevalence of PA Aneurysms in CHD
PA diameter > 40 mm
BAV
TGA
AVSD
T. FALLOT
VSD
ASD
SHUNTS
PDA
Pulmonary Valve Stenosis
Overall
0%
20%
40%
60%
80%
100%
Pulmonary Artery Hypertension and Shunts
28/66 (42.5%) Pulmonary Artery Aneurysms were associated with PAH
PH etiologies
n (%)
Eisenmenger
13 (46%)
Left to right ASD
7 (25%)
Left to right AVSD
1 (3.5%)
Postop PAH VSD
1 (3.5%)
Postop PAH PDA
1 (3.5%)
TGA
2 (7%)
Segmental PH
1 (3.5%)
IPA
1 (3.5%)
Coarctation
1 (3.5%)
21/28 (75%)
non-repaired septal defects
SHUNTS
p < 0.0001
34 mm
(32-36)
40 mm
(36.75-45)
max TR Velocity < 3.5 m/s max TR Velocity > 3.5 m/s
High Flow PA Dilatation
Large PA diameters but low incidence of complications:
50% dissections, PDA related1
1. Cong Heart Dis 2016;11:102
48 mm
(IQ 40-53.5)
p < 0.03
Non Hypertensive PAA
38 (58%)
41 mm
(IQ 40-46)
Hypertensive PAA
28 (42%)
Correlation PA diameter and
hemodynamic parameters
r= -0.196
p = 0.218
r= 0.137
p = 0.487
r= -0.203
p = 0.468
r= 0.071
p = 0.725
Histologic evidence of arteriopathy in the context of CHD
The “TISSUE” factor
The issue:
Dilatation of Pulmonary Artery
“..... Out of proportion to hemodynamic or
morphogenetic expectations?”
Niwa, Circulation 2001;103:393
Grade 3, large areas of complete loss of elastic fibers
and smooth muscle, large areas of ground substance
accumulation
Niwa, Circulation 2001;103:393
Prognostic Relevance of PAAn in CHD
• 4 no complicated pregnancies
• Progression: none
• 9 Complications in 66 PAA patients (14%)
4% in 248 PAD patients
0.5% in 1792 patients
LMCA compression (3)
percutaneous intervention (2)
Ortner’s Syndrome (2)
Death
(6)
Heart Failure
(4)
Perioperative (1)
Sudden Death (1)
Attributed to PA Dissection in
1 Eisenmenger patient
Absent Pulmonary Valve. Massive dilatation of the Pulmonary
Artery in association with bronchial and left main coronary artery
compression
PA
Ao
PA
LMCA
Left
Bronchus
Ao
PA
PA
PA
LMCA
PA
LMCA
Factors associated with mechanical complications
Sudden Cardiac Death or LMCA compression were significantly
associated with more severe PH in high flow/high pressure PAAn
(120 vs. 55 mmHg; p = 0.002)
MPA ranged from 40 to 65 mm
Complications
(n = 10)
No complications
(n = 66)
p
Age (years)
68 (IQ 41-68)
47 (IQ 32-62.5)
ns.
Females (%)
67%
33%
ns.
51
(IQ 43.5-51)
45
( IQ 40-50)
ns.
40-70
40-75
25 (18-57.50)
37 (20-92.5)
ns.
3 (2-4)
2 (1-3)
ns.
10 (7.5-42)
12 (5-30)
ns.
Median PA diameter
(mm)
Range PA diameter
(mm)
sPAP (mmHg)
Pulmonary Regurg
Peak gradient
Risk factors for dissection
Identification of high-risk patients
• A systematic literature review in Aneurysm of Pulmonary Artery
• Odds ratio for CHD-PH 2.8 (range 1.26-6.16; p=0.0012)
American Journal of Forensic Medicine and Pathology
2007
Cong Heart Dis 2016;11:102
Risk factors for dissection
Identification of high-risk patients
PA Diameter per se cannot explain Dissections
Cong Heart Dis 2016;11:102
Absolute Pressure Limit > 50 mmHg
Risk factors for dissection
Identification of high-risk patients
Absolute PA Diameter Limit > 75 mm
Cong Heart Dis 2016;11:102
Summary
• PA Dilatation in Adults with CHD is a common
finding but only a small proportion have PAAn
• Prevalence of complications is very low
• Interaction between high flow rate and high
pressure increases the risk for dilatation, dissection
and/or sudden death
Recommendations
THRESHOLD FOR SURGERY in asymptomatic
– Low Pressure Pulmonary Artery Aneurysms
• Treatment is based more on RV function and PV lesion severity.
• No indication for surgery based on PA size.
– High Pressure Pulmonary Artery Aneurysms
• Surgery could be considered in patients with progressive changes in PA
diameter in high flow/high pressure aneurysms
• Balance high surgical risk and low incidence of complications in PAH
• Heart-Lung Transplantation might be considered in Eisenmenger
syndrome
– Individualizing indications
Thank you for the attention
Pastora Gallego, MD, PhD
Inter-center Adult Congenital Heart Disease Unit
Area del Corazón
Seville, Spain
High Pressure
Pulmonary
Artery Dilatation
Cardiology 2012;76