Cardiothoracic surgery and peripheral endovascular intervention in cardiovascular damage from a cohort of orphan rheumatological diseases-epidemiological and survival analysis

Maria Elena Soto; Huitzilihuitl Saucedo-Orozco; Eric Ochoa-Hein; Guering Eid-Lidt; Javier E Anaya-Ayala; Israel Pérez-Torres; Solange Gabriela Koretzky; Pedro A Reyes; Edison Ricardo Espinoza-Saquicela; Ivan Hernandez; Humberto Martinez-Hernandez

doi:10.21037/jtd-21-1523

Cardiothoracic surgery and peripheral endovascular intervention in cardiovascular damage from a cohort of orphan rheumatological diseases-epidemiological and survival analysis

J Thorac Dis. 2022 Jun;14(6):1815-1829. doi: 10.21037/jtd-21-1523.

Affiliations

¹ Immunology Department, Instituto Nacional de Cardiología Ignacio Chávez, and Research Department, Cardiovascular Line, American British Cowdray Hospital Center, Mexico City, Mexico.
² Cardioneumology Department, Instituto Nacional de Cardiología Ignacio Chávez and Cardioneumology Department, Specialty Hospital, National Medical Center "La Raza", Mexican Social Security Institute, Mexico City, Mexico.
³ Hospital Epidemiology Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
⁴ Hemodynamics Department, Instituto Nacional de Cardiología Ignacio Chávez, Mexico City, Mexico.
⁵ Vascular Surgery and Endovascular Therapy, Surgery Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
⁶ Cardiovascular Biomedicine Department, Instituto Nacional de Cardiología Ignacio Chávez, Mexico City, Mexico.
⁷ Publication Department, Instituto Nacional de Cardiología Ignacio Chávez, Mexico City, Mexico.
⁸ Research Directorate and Ethics Committee Instituto Nacional de Cardiología Ignacio Chávez, Mexico City, Mexico.
⁹ Cardiothoracic Surgery Department, Instituto Nacional de Cardiología Ignacio Chávez, Mexico City, Mexico.
¹⁰ Head of Cardiothoracic Surgery Department, Instituto Nacional de Cardiología Ignacio Chávez, Mexico City, Mexico.

Abstract

Background: Aortic diseases in some orphan rheumatological diseases require medical, surgical or peripheral endovascular intervention because they can be catastrophic. Objectives: to analyze the main clinical and epidemiological characteristics of patients with Takayasu arteritis (TA), Marfan syndrome (MS) and similar conditions that were treated with cardiothoracic surgery and peripheral endovascular intervention.

Methods: Retrospective and descriptive cohort study that included patients of any age and gender with TA (as per the criteria of the American College of Rheumatology and EULAR/PRINTO), MS (according to Ghent criteria), and similar conditions who underwent cardiothoracic surgery or peripheral endovascular intervention. Data were collected from electronic charts.

Results: A total of 77 patients with TA and 135 patients with MS and similar conditions were included. The frequency of surgical or interventional requirements in patients with TA and MS/similar conditions was 77/364 (21.2%) and 135/300 (45%), respectively; such patients were followed for a median of 6 [2-12] and 3.29 (0.42-6.62) years, with (maximum follow-up range of 47 and 21.37 years, respectively). Aneurysms were present in 11 (14.3%) and 66 (48.9%) in patients with TA and MS/similar conditions, respectively. Aortic, mitral and tricuspid valve damage occurred in 8 (10.4%) patients, 4 (5.2%) patients and 1 (1.3%) patient with TA, respectively; corresponding frequencies in patients with MS/similar conditions were 98 (72.6%), 50 (37.0%) and 20 (14.8%). We identified that 20% of patients with TA died after 5.08 years (95% CI: 0.23-25.42 years) and 20 % of the patients with MS and other similar conditions died after 7.52 years (95% CI: 1.10-9.02 years).

Conclusions: The frequency of surgical intervention was low in this study. Long-term prognosis is good if surgery is performed in a timely manner. Epidemiological studies provide relevant information for public health decisions related to the management of orphan rheumatological diseases.

Keywords: Marfan syndrome (MS); Orphan disease; Takayasu arteritis (TA); cardiovascular surgical procedure; peripheral intervention.