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

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1 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

2 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

3 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

4 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 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%) PA Measurement None n= 0 Echo n = 92 (37%) CT or MRI n = 156 (63%)

5 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

6 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 1 (1.5%) DORV + PDA 1 (1.5%) PV STENOSIS 14 (21%) T. FALLOT 12 (18%) T. FALLOT 9 (14%) ABSENT PV 3 (4.5%) TGA 2 (3.0%) 2 (100%) TGA+ VSD 1 (1.5%) 1 (100%) IDPA 2 (3%) 1 (50%) COARCTATION + BAV 1 (1.5%) 1 (100%) PA Dimensions in CHD 42.5 mm (IQ 40-50) 43 mm (IQ ) PA Aneurysms Distribution by diagnosis 47.5 mm (IQ ) 41 mm 47 mm (IQ 40-48)

7 Prevalence of PA Aneurysms in CHD BAV TGA AVSD T. FALLOT VSD SHUNTS ASD PDA Pulmonary Valve Stenosis Overall PA diameter > 40 mm 0% 20% 40% 60% 80% 100%

8 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 34 mm (32-36) p < max TR Velocity < 3.5 m/s SHUNTS 40 mm ( ) max TR Velocity > 3.5 m/s

9 High Flow PA Dilatation Large PA diameters but low incidence of complications: 50% dissections, PDA related 1 1. Cong Heart Dis 2016;11: mm (IQ ) p < mm (IQ 40-46) Non Hypertensive PAA 38 (58%) Hypertensive PAA 28 (42%)

10 Correlation PA diameter and hemodynamic parameters r= p = r= p = r= p = r= p = 0.725

11 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

12 Grade 3, large areas of complete loss of elastic fibers and smooth muscle, large areas of ground substance accumulation Niwa, Circulation 2001;103:393

13 Prognostic Relevance of PAAn in CHD 4 no complicated pregnancies Progression: none 9 Complications in 66 PAA patients (14%) LMCA compression (3) percutaneous intervention (2) Ortner s Syndrome (2) Death (6) 4% in 248 PAD patients 0.5% in 1792 patients Heart Failure (4) Perioperative (1) Sudden Death (1) Attributed to PA Dissection in 1 Eisenmenger patient

14 Absent Pulmonary Valve. Massive dilatation of the Pulmonary Artery in association with bronchial and left main coronary artery compression PA LMCA PA Ao LMCA PA Left Bronchus PA PA Ao PA LMCA

15 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) Age (years) 68 (IQ 41-68) 47 (IQ ) ns. Females (%) 67% 33% ns. Median PA diameter (mm) Range PA diameter (mm) 51 (IQ ) 45 ( IQ 40-50) spap (mmhg) 25 ( ) 37 ( ) ns. Pulmonary Regurg 3 (2-4) 2 (1-3) ns. Peak gradient 10 (7.5-42) 12 (5-30) ns. p ns.

16 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 ; p=0.0012) American Journal of Forensic Medicine and Pathology 2007 Cong Heart Dis 2016;11:102

17 Risk factors for dissection Identification of high-risk patients PA Diameter per se cannot explain Dissections Cong Heart Dis 2016;11:102

18 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

19 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

20 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

21 Thank you for the attention Pastora Gallego, MD, PhD Inter-center Adult Congenital Heart Disease Unit Area del Corazón Seville, Spain

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23 High Pressure Pulmonary Artery Dilatation Cardiology 2012;76