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Editor's Choice - Open Thoracic and Thoraco-abdominal Aortic Repair in Patients with Connective Tissue Disease

Paula R Keschenau1, Drosos Kotelis1, Jeroen Bisschop2

  • 1European Vascular Centre Aachen-Maastricht, Department of Vascular Surgery, RWTH University Hospital Aachen, Aachen, Germany.

European Journal of Vascular and Endovascular Surgery : the Official Journal of the European Society for Vascular Surgery
|September 17, 2017
PubMed

Insights

Open complex aortic repair in patients with connective tissue disease (CTD) is a durable therapy, though associated with significant risks. Specialized centers should reserve this procedure, prioritizing staged approaches to reduce operating time and improve outcomes.

Area of Science:

  • Cardiovascular Surgery
  • Thoracic Aortic Aneurysm and Dissection Repair
  • Connective Tissue Diseases

Background:

  • Connective tissue diseases (CTD) predispose patients to complex aortic pathologies.
  • Previous aortic repair is common in CTD patients undergoing further aortic surgery.
  • Open aortic repair in CTD patients presents unique challenges and risks.

Purpose of the Study:

  • To evaluate the outcomes of open complex aortic repair in patients diagnosed with connective tissue disease (CTD).
  • To identify risk factors and complications associated with aortic repair in this patient cohort.
  • To assess the long-term durability and survival rates following open aortic repair in CTD.

Main Methods:

  • Retrospective, cross-border, single-center study analyzing 72 aortic operations on 65 CTD patients (2000-2016).
  • Detailed analysis of open repair procedures, including types of aortic repair and previous interventions.
  • Median follow-up of 42 months to assess outcomes, survival, and re-intervention rates.

Main Results:

  • In-hospital mortality was 14%, with major complications including bleeding, sepsis, and neurological events.
  • Paraplegia/paraparesis occurred in 7% of patients; 11% required temporary dialysis.
  • Operating time exceeding 7 hours was an independent predictor of increased mortality; 1-year survival was 80%.

Conclusions:

  • Open thoracoabdominal aortic (TAA(A)) repair is a durable treatment for CTD patients, often as revision surgery.
  • The procedure carries significant risks and should be performed in specialized centers.
  • Staged procedures are recommended to reduce operating time and potentially mitigate risks.
Abstract

Keywords:
Connective tissue diseasesEhlers–Danlos syndromeLoeys–Dietz syndromeMarfan syndromeOpen aortic repairThoraco-abdominal aortic aneurysm

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