Cardiovascular surgery in Turner syndrome - early outcome and long-term follow-up.

Published on Mar 26, 2020in World Journal of Cardiology
· DOI :10.4330/WJC.V12.I3.97
Margaret M. Fuchs2
Estimated H-index: 2
(Mayo Clinic),
Christine H. Attenhofer Jost11
Estimated H-index: 11
(Mayo Clinic)
+ 4 AuthorsAlexander C. Egbe20
Estimated H-index: 20
(Mayo Clinic)
Sources
Abstract
BACKGROUND: Cardiovascular disease is the leading cause of death in patients with Turner syndrome (TS), and cardiovascular surgery is frequently required for management of these patients. TS is associated with medical comorbidities than can complicate the care of this patient population. AIM: To describe the cardiovascular surgical outcomes of patients with TS. METHODS: A retrospective case series was compiled of 51 consecutive TS patients who had at least one cardiovascular surgery at Mayo Clinic Rochester from 1977-2017. The baseline clinical data of these patients were reviewed including demographics, medical comorbidities, congenital heart disease history, and medications. Echocardiographic reports were analyzed in detail. Operative reports and surgical hospital courses were reviewed. Long-term mortality was determined using medical records and the Social Security Death Index. Survival analysis was performed with the Kaplan Meier method. RESULTS: The cohort comprised 51 TS patients, average age at the time of surgery at Mayo Clinic was 28 (8-41) years, and 23 (45%) patients were under the age of 18. At the time of first Mayo Clinic surgery, 18 (35%) patients had previously undergone cardiac surgery at another institution. The most common procedures were repair of aortic coarctation in 14 (28%) patients, aortic valve replacement in 6 (12%) patients, and composite aortic root/ascending aorta replacement in 7 (14%) patients, with 7 patients undergoing repair of more than one lesion. Aortic dissection required operative intervention in 5 patients. After initial Mayo Clinic surgery, subsequent operations were required in 6 (13%) patients. Average hospital length of stay was 6 ± 2 d. There were 4 (8%) early surgical deaths. Freedom from death was 97% and 89% at 10 and 20 years, and the freedom from reoperation was 93% and 81% at 10 and 20 years. CONCLUSION: Cardiovascular surgery is associated with 8% early mortality given the medical complexity of TS patients. Those who survive to dismissal have good survival. Later cardiovascular reoperations are not rare.
References20
Newest
#1Joshua D. Chew (VUMC: Vanderbilt University Medical Center)H-Index: 2
#2Kevin D. Hill (Duke University)H-Index: 32
Last. Jonathan H. Soslow (VUMC: Vanderbilt University Medical Center)H-Index: 10
view all 11 authors...
Abstract Background Turner syndrome (TS) is a genetic syndrome characterized by monosomy X (45XO) in phenotypic females and is commonly associated with congenital heart disease. We sought to describe the distribution, mortality, and morbidity of congenital heart surgery in TS and compare outcomes to subjects without genetic syndromes. Methods The Society of Thoracic Surgeons Congenital Heart Surgery Database was used to evaluate index cardiovascular operations performed from 2000-2017 in pediatr...
4 CitationsSource
#2Niels H. AndersenH-Index: 30
Last. Philippe Backeljauw (Cincinnati Children's Hospital Medical Center)H-Index: 23
view all 18 authors...
Turner syndrome affects 25-50 per 100,000 females and can involve multiple organs through all stages of life, necessitating multidisciplinary approach to care. Previous guidelines have highlighted this, but numerous important advances have been noted recently. These advances cover all specialty fields involved in the care of girls and women with TS. This paper is based on an international effort that started with exploratory meetings in 2014 in both Europe and the USA, and culminated with a Cons...
385 CitationsSource
#1Emilio Quezada (OHSU: Oregon Health & Science University)H-Index: 7
#2Jodi Lapidus (OHSU: Oregon Health & Science University)H-Index: 36
Last. Michael Silberbach (OHSU: Oregon Health & Science University)H-Index: 26
view all 5 authors...
In Turner syndrome, linear growth is less than the general population. Consequently, to assess stature in Turner syndrome, condition-specific comparators have been employed. Similar reference curves for cardiac structures in Turner syndrome are currently unavailable. Accurate assessment of the aorta is particularly critical in Turner syndrome because aortic dissection and rupture occur more frequently than in the general population. Furthermore, comparisons to references calculated from the tall...
28 CitationsSource
#1Lynne L. Levitsky (Harvard University)H-Index: 30
Last. Angela E. LinH-Index: 59
view all 4 authors...
Purpose of reviewWe review recent understanding of the pathophysiology, molecular biology, and management of Turner syndrome.Recent findingsSophisticated genetic techniques are able to detect mosaicism in one-third of individuals previously thought to have monosomy X. Prenatal detection using matern
29 CitationsSource
#1Sarah A. LawsonH-Index: 3
#2Elaine M. Urbina (Cincinnati Children's Hospital Medical Center)H-Index: 55
Last. Philippe BackeljauwH-Index: 23
view all 6 authors...
Context: Turner syndrome (TS) carries an increased risk for vascular disease, or vasculopathy. Objective: Vasculopathy can be detected in young TS patients. Design and Patients: Vasculopathy was prospectively assessed by measuring vascular function and structure in TS patients (n = 49) and lean (L) (n = 76) and obese (O) controls (n = 52) through noninvasive techniques. Controls were drawn from previously known adolescents who were age-matched and disease-free. Data collected: Pulse wave velocit...
24 CitationsSource
#1Morgan L. Brown (Mayo Clinic)H-Index: 24
#2Harold M. Burkhart (Mayo Clinic)H-Index: 53
Last. Hartzell V. Schaff (Mayo Clinic)H-Index: 131
view all 9 authors...
Objectives The objective of our study was to review the long-term outcomes of patients undergoing surgical repair of aortic coarctation. Background Surgical repair of aortic coarctation has been performed at the Mayo Clinic, Rochester, Minnesota, for over 60 years. Methods Between 1946 and 2005, 819 patients with isolated coarctation of the aorta underwent primary operative repair. Medical records were reviewed and questionnaires mailed to the patients. Results Mean age at repair was 17.2 ± 13.6...
174 CitationsSource
#1Erin J. Madriago (OHSU: Oregon Health & Science University)H-Index: 6
#2Thuan Nguyen (OHSU: Oregon Health & Science University)H-Index: 17
Last. Michael Silberbach (OHSU: Oregon Health & Science University)H-Index: 26
view all 7 authors...
Cardiac malformations occur commonly in Turner syndrome (TS), but the outcomes of cardiac operations and catheter-based procedures are unknown. The Pediatric Cardiac Care Consortium database was queried for individuals with TS and other female subjects without genetic abnormalities or syndromes (non-TS [NTS]). Procedures for left-sided heart lesions represented most TS procedures (95.2%). Three hundred ninety-eight patients with TS who underwent 637 of these procedures of interest were compared ...
23 CitationsSource
#1Kristian H. Mortensen (University of Cambridge)H-Index: 18
#2Niels H. Andersen (Aarhus University Hospital)H-Index: 30
Last. Claus Højbjerg Gravholt (Aarhus University Hospital)H-Index: 67
view all 3 authors...
Cardiovascular disease is emerging as a cardinal trait of Turner syndrome, being responsible for half of the 3-fold excess mortality. Turner syndrome has been proposed as an independent risk marker for cardiovascular disease that manifests as congenital heart disease, aortic dilation and dissection, valvular heart disease, hypertension, thromboembolism, myocardial infarction, and stroke. Risk stratification is unfortunately not straightforward because risk markers derived from the general popula...
132 CitationsSource
#1Kristian H. Mortensen (Aarhus University Hospital)H-Index: 18
#2Britta E Hjerrild (Aarhus University Hospital)H-Index: 15
Last. Claus Højbjerg Gravholt (Aarhus University Hospital)H-Index: 67
view all 9 authors...
Background: Ectatic aortopathy and arterial abnormalities cause excess morbidity and mortality in Turner syndrome, where a state of vasculopathy seemingly extends into the major head and neck branch arteries. Objective: We investigated the prevalence of abnormalities of the major intrathoracic arteries, their interaction with arterial dimensions, and their association with karyotype. Design: Magnetic resonance imaging scans determined the arterial abnormalities as well as head and neck branch ar...
49 CitationsSource
#2Anthony J. SwerdlowH-Index: 111
Last. Patricia A. Jacobs (Salisbury District Hospital)H-Index: 54
view all 5 authors...
Context: Turner syndrome is characterized by complete or partial X chromosome monosomy. It is associated with substantial morbidity, but mortality risks and causes of death are not well described. Objectives: Our objective was to investigate mortality and causes of death in women with Turner syndrome. Design and Setting: We constructed a cohort of women diagnosed with Turner syndrome at almost all cytogenetic centers in Great Britain and followed them for mortality. Patients: A total of 3439 wom...
191 CitationsSource
Cited By1
Newest
#1Isani Singh (Harvard University)
#3David P. Kao (CU: University of Colorado Boulder)H-Index: 20
Last. Amber Khanna (CU: University of Colorado Boulder)H-Index: 8
view all 5 authors...
OBJECTIVE To evaluate outcomes in patients with Turner Syndrome, especially those with cardiac conditions, compared to those without Turner syndrome. DESIGN Retrospective cohort study utilising hospitalisation data from 2006 to 2012. Conditional logistic regression models are used to analyse outcomes of interest: all-cause mortality, increased length of stay, and discharge to home. PARTICIPANTS We identified 2978 women with Turner syndrome, matched to 11,912 controls by primary diagnosis. RESULT...
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