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Ann Thorac Surg. 2019 Sep 27. pii: S0003-4975(19)31437-7. doi: 10.1016/j.athoracsur.2019.08.047. [Epub ahead of print]

Open Thoracoabdominal Aortic Repair in Patients with Heritable Aortic Disease in the GenTAC Registry.

Author information

1
Baylor College of Medicine, Houston, TX.
2
Division of Cardiothoracic Surgery, Oregon Health & Science University, Portland, OR.
3
Division of Cardiovascular Surgery, University of Pennsylvania, Philadelphia, PA.
4
Division of Vascular Surgery, University of Washington, Seattle, WA.
5
Biostatistics and Epidemiology Division, RTI International, Research Triangle Park, NC.
6
Division of Cardiology, University of Michigan, Ann Arbor, MI.
7
Division of Cardiology, Weill Cornell Medicine, New York, NY.
8
Department of Medicine, University of Pennsylvania, Philadelphia, PA.
9
Knight Cardiovascular Institute, Oregon Health & Science University, Portland, OR.
10
Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD.
11
Division of Medical Genetics, The University of Texas Health Science Center at Houston, Houston, TX.
12
Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, and Department of Cardiovascular Surgery, Texas Heart Institute, Houston, TX.
13
Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, and Department of Cardiovascular Surgery, Texas Heart Institute, Houston, TX. Electronic address: slemaire@bcm.edu.

Abstract

BACKGROUND:

Although patients with various types of heritable aortopathy often require distal aortic repair, data are limited regarding the most extensive operations-open thoracoabdominal aortic aneurysm (TAAA) repairs. The objective of this multicenter registry study was to characterize TAAA repairs in a large cohort of patients with different heritable aortic diseases.

METHODS:

From the 3,699 patients enrolled at 8 participating centers in the Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) Registry, we identified 155 open TAAA repairs in 142 unique patients. We examined data related to clinical characteristics, surgical techniques, and outcomes.

RESULTS:

The primary diagnoses included Marfan syndrome (n=76; 54%), familial thoracic aortic aneurysm and dissections (FTAAD) (n=31; 22%), and Loeys-Dietz syndrome (n=10; 7%). Most repairs were performed for aneurysms associated with aortic dissection (n=110; 71%). The most common repairs involved the entire descending thoracic aorta with distal extension (21% Crawford extent I and 36% extent II). Adjuncts used during repair varied substantially. The operative mortality rate was 1.3%. Other complications included paraplegia (4%), acute renal failure (5%), and vocal cord paralysis (21%). Reoperation after TAAA repair was required in a subset of cases for early bleeding (n=15; 10%) and late repair failure (n=7; 5%).

CONCLUSIONS:

Open TAAA repairs are necessary in a variety of heritable aortic diseases. These patients often require extensive surgical repair, and a variety of adjunctive techniques are employed. The risk of repair failure and the need for reoperation in a subset of patients supports the need for vigilant long-term surveillance after repair.

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