Thoracic aortic dissection and rupture in conotruncal cardiac defects: A population-based study

Area of Science:

• Cardiovascular Medicine
• Congenital Heart Disease Research
• Aortic Pathology

Background:

• Thoracic aortic dissection and rupture (TAD) is a known risk in bicuspid aortic valve (BAV).
• The risk of TAD in other congenital heart diseases (CHD), especially conotruncal lesions, is not well-established.
• Conotruncal lesions include tetralogy of Fallot (TOF), truncus arteriosus, D-transposition of the great arteries (D-TGA), and double outlet right ventricle.

Purpose of the Study:

• To describe the occurrence of TAD in patients with conotruncal CHD.
• To investigate any potential association between conotruncal CHD and an increased risk of TAD.

Main Methods:

• Analysis of the Texas Inpatient Public Use Data File (>37.9 million hospitalizations, Jan 1999-June 2012).
• Identification of 12,016 TAD cases and 214 TAD cases in patients with CHD.
• An exploratory case-control study using multilevel logistic regression for patients >1 year old, controlling for known TAD risk factors.

Main Results:

• The most common CHDs associated with TAD were BAV (42%), atrial septal defect (21%), aortic coarctation (7%), ventricular septal defect (6%), and patent ductus arteriosus (4%).
• Three cases of TAD occurred in patients with TOF, 2 with D-TGA, and 1 with truncus arteriosus.
• A significant association was found between TAD and BAV (OR 10), but not with aortic coarctation or any conotruncal lesion.

Conclusions:

• Thoracic aortic dissection and rupture (TAD) is exceptionally rare in conotruncal congenital heart disease (CHD).
• No increased occurrence of TAD was observed in the hospitalized population with conotruncal CHD.
• The findings suggest that conotruncal CHD does not confer an elevated risk for TAD.