Risk of pulmonary artery dissection in adults with congenital heart disease
Alexander C Egbe1, William R Miranda1, C Charles Jain1
1Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN 55905, USA.
Insights
The risk of pulmonary artery dissection is very low in adults with congenital heart disease, even with severe aneurysms or during pregnancy. This suggests a generally benign condition, supporting conservative management strategies.
Area of Science:
- Cardiology
- Vascular Medicine
- Congenital Heart Disease
Background:
- Limited data exists on pulmonary artery (PA) dissection risk in adults with congenital heart disease (CHD).
- Pulmonary artery aneurysms are a known complication in this population.
Purpose of the Study:
- To estimate the incidence of PA dissection in adults with CHD.
- To evaluate PA dissection risk in patients with PA aneurysms, including those who become pregnant.
Main Methods:
- Retrospective cohort study of adults with CHD (2003-2020).
- Defined PA aneurysm as >40mm (main) or >30mm (branch) diameter.
- Defined severe PA aneurysm as >50mm diameter.
Main Results:
- One PA dissection occurred in 1,673 patients (incidence 14 per 100,000 patient-years).
- No PA dissections were observed during pregnancy in 163 women with PA aneurysms.
- PA dimensions remained stable before and after pregnancy.
Conclusions:
- PA dissection risk is extremely low in adults with CHD, even with severe PA aneurysms or during pregnancy.
- Data suggests a benign natural history for PA aneurysms in CHD patients without severe pulmonary hypertension.
- A conservative management approach is recommended for PA aneurysms in this population.
Background:
There are limited data about the risk of pulmonary artery (PA) dissection in adults with congenital heart disease (CHD), and the purpose of this study was to estimate the incidence of PA dissection in this population.
Methods:
Retrospective cohort study of adults with CHD that underwent cross-sectional imaging (2003-2020). PA aneurysm was defined as main or branch PA diameter > 40 mm or > 30 mm respectively, and severe PA aneurysm was defined as main or branch PA diameter > 50 mm.
Results:
Of 1, 673 patients (41 ± 10 years; male 58%), 493 (24%), 286 (19%), and 306 (20%) had aneurysms of the main, right, and left PA respectively, while 66 (4%) had severe PA aneurysm. During a median follow-up of 8.2 (interquartile range 3.7-10.3) years, there was one PA dissection in a patient with Eisenmenger syndrome, thus the incidence of PA dissection was 14 per 100,000 patient-years. Of 779 females, 163 had one or more pregnancies during follow-up, and 41 (25%) of these patients had known PA aneurysm at the time of conception. There was no PA dissection during pregnancy. Of the 163 patients, 91 (56%) had cross-sectional imaging before and after pregnancy, there was no significant difference in PA dimension before versus after pregnancy (main PA 35 ± 5 versus 36 ± 4 mm, p = 0.6; right PA 21 ± 3 versus 33 ± 4 mm, p = 0.1; and left PA 23 ± 4 versus 22 ± 4 mm, p = 0.4).
Conclusions:
The risk of PA dissection was extremely low even in patients with severe PA aneurysm, or in patients with PA aneurysm that became pregnant. Collectively, these data suggest a benign natural history for patients without severe pulmonary hypertension and encourage and a conservative approach in managing patients with PA aneurysm.
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