Adult Surgical Experience With Loeys-Dietz Syndrome

Williams, Jason A.; Hanna, Jennifer M.; Shah, Asad Ali; Andersen, Nicholas D.; McDonald, Marie; Jiang, Yong‐Hui; Wechsler, Stephanie Burns; Zomorodi, Ali R.; McCann, Richard L.; Hughes, G. Chad

doi:10.1016/j.athoracsur.2014.11.021

Cited by 28 publications

(18 citation statements)

References 19 publications

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“…Mortality and morbidity were low in this group, likely because patients with connective tissue disease tend to be younger with few comorbidities. This result is consistent with previous series in which patients with connective tissue disease had low mortality and morbidity following multiple reinterventions . In the series of patients with Loeys‐Dietz syndrome reported by Williams et al, those who experienced acute type A dissection underwent multiple surgical interventions with a mean of 3.4 operations performed.…”

Section: Discussionsupporting

confidence: 90%

“…This result is consistent with previous series in which patients with connective tissue disease had low mortality and morbidity following multiple reinterventions. 20,21 In the series of patients with Loeys-Dietz syndrome reported by Williams et al, 20 those who experienced acute type A dissection underwent multiple surgical interventions with a mean of 3.4 operations performed. Despite these multiple operations, there were no aortic-specific deaths in the cohort.…”

Section: Discussionmentioning

confidence: 99%

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Outcomes of Reoperation After Acute Type A Aortic Dissection: Implications for Index Repair Strategy

Wang

Wagner

Benrashid

et al. 2017

JAHA

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BackgroundThe optimal surgical approach for management of acute type A aortic dissection remains controversial. This study aimed to assess outcomes of reoperation after acute type A dissection repair to help guide decision making around index operative strategy.Methods and ResultsAll aortic reoperations (n=129) at a single referral institution from August 2005 to April 2016 after prior acute type A dissection repair were reviewed. The primary outcome was 30‐day or in‐hospital mortality. Secondary outcomes included organ‐specific morbidity and 1‐ and 5‐year outcomes as estimated using the Kaplan–Meier method. The majority of initial reoperations were proximal aortic (aortic valve, aortic root, or ascending) or aortic arch procedures (62.5%, n=55); most initial reoperations were performed in the elective setting (83.1%, n=74). Additional nonstaged second or more reoperations were required in 21 patients (23.6%) after the initial reoperation, during a median follow‐up of 2.5 years after the initial reoperation. Thirty‐day or in‐hospital mortality for all reoperations was 7.0% (elective: 6.3%; nonelective: 11.1%) with acceptable rates of organ‐specific morbidity, given the procedural complexity. One‐ and 5‐year overall survival after initial reoperation was 85.9% and 64.9%, respectively, with aorta‐specific survival of 88% at 5 years.ConclusionsReoperation after acute type A aortic dissection repair is associated with low rates of mortality and morbidity. These data support more limited index repair for acute type A dissection, especially for patients undergoing index repair in lower volume centers without expertise in extensive repair, because reoperations, if needed, can be performed safely in referral aortic centers.

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Section: Discussionsupporting

confidence: 90%

Section: Discussionmentioning

confidence: 99%

Outcomes of Reoperation After Acute Type A Aortic Dissection: Implications for Index Repair Strategy

Wang

Wagner

Benrashid

et al. 2017

JAHA

Self Cite

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“…The results of aortic surgery in this group of patients appear to be similar to those observed in patients with MFS, i.e. a decreased risk for dissection and a sustained benefit over time 13,14 . The fact that the benefit is at least in part the result of the care provided to these patients is also suggested by the divergence of the vascular event-free survival curve and the crude survival curve: the vascular event-free survival curve (i.e.…”

Section: Discussionsupporting

confidence: 68%

International Registry of Patients Carrying TGFBR1 or TGFBR2 Mutations

Jondeau

Ropers²,

Regalado

et al. 2016

Circ Cardiovasc Genet

169

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Background The natural history of aortic diseases in patients with TGFBR1 or TGFBR2 mutations reported by different investigators has varied greatly. In particular, the current recommendations for the timing of surgical repair of the aortic root aneurysms may be overly aggressive. Methods and Results The Montalcino Aortic Consortium, which includes 15 centers worldwide that specialize in heritable thoracic aortic diseases, was used to gather data on 441 patients from 228 families, with 176 cases harboring a mutation in TGBR1 and 265 in TGFBR2. Patients harboring a TGFBR1 mutation have similar survival rates (80% survival at 60 years), aortic risk (23% aortic dissection, 18% preventive aortic surgery), and prevalence of extra-aortic features (29% hypertelorism, 53% cervical arterial tortuosity, 27% wide scars) when compared to patients harboring a TGFBR2 mutation. However, TGFBR1 males had a greater aortic risk than females, whereas TGFBR2 males and females had a similar aortic risk. Additionally, aortic root diameter prior to or at the time of type A aortic dissection tended to be smaller in patients carrying a TGFBR2 mutation and was ≤45 mm in 6 women with TGFBR2 mutations, presenting with marked systemic features and low body surface area. Aortic dissection was observed in 1.6% of pregnancies. Conclusions Patients with TGFBR1 or TGFBR2 mutations show the same prevalence of systemic features, and the same global survival. Preventive aortic surgery at a diameter of 45 mm, lowered toward 40 in females with low body surface area, TGFBR2 mutation and severe extra-aortic features may be considered.

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“…Additionally, because of the repeatedly demonstrated greater incidence of reintervention for treatment failure, not to mention the not infrequent development of metachronous aortic disease in this population, further strength is given to the previous notion that routine aortic surveillance with cross-sectional imaging by an experienced aortic center is an absolute necessity. 8,31 …”

Section: Discussionmentioning

confidence: 99%

Evolving practice pattern changes and outcomes in the era of hybrid aortic arch repair

Benrashid

Wang

Keenan

et al. 2016

Journal of Vascular Surgery

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Objective The role of hybrid repair in the management of aortic arch pathology, and long-term outcomes with these techniques, remains uncertain. We report a decade of experience with hybrid arch repair (HAR) and assess institutional practice patterns with regard to the use of hybrid and open techniques. Methods Hybrid and open total and distal arch procedures performed between July 2005 and January 2015 were identified from a prospectively maintained, institutional aortic surgery database. Perioperative morbidity and mortality, freedom from reintervention, and long-term survival were calculated. Hybrid and open procedural volumes over the study period were assessed to evaluate for potential practice pattern changes. Results During the study period 148 consecutive procedures were performed for repair of transverse and distal aortic arch pathology, including 101 hybrid repairs and 47 open total or distal arch repairs. Patients in the hybrid repair group were significantly older with a greater incidence of chronic kidney disease, peripheral vascular disease, and chronic lung disease. Perioperative mortality and outcomes were not significantly different between the hybrid and open groups, aside from decreased median length of stay after hybrid repair. Need for subsequent reintervention was significantly greater after hybrid repair. Unadjusted long-term survival was superior after open repair (70% 5-year survival open vs 47% hybrid; P = .03), although aorta-specific survival was similar (98% 5-year aorta-specific survival open vs 93% hybrid; P = .59). Institutional use of HAR decreased over the final 3 years of the study, with an associated increased use of open total or distal arch repairs. This was primarily the result of decreased use of native zone 0 hybrid procedures. Concurrent with this apparent increased stringency around patient selection for HAR, perioperative morbidity and mortality was reduced, including avoidance of retrograde type A dissection. Conclusions HAR remains a viable option for higher-risk patients with transverse arch pathology with perioperative outcomes and long-term aorta-specific survival similar to open repair, albeit at a cost of increased reintervention. This observational single-institution study would suggest decreased use in more recent years in favor of open repair due to avoidance of native zone 0 hybrid procedures. This decline in the institutional use of native zone 0 hybrid repairs was associated with improved perioperative outcomes.

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Adult Surgical Experience With Loeys-Dietz Syndrome

Cited by 28 publications

References 19 publications

Outcomes of Reoperation After Acute Type A Aortic Dissection: Implications for Index Repair Strategy

Outcomes of Reoperation After Acute Type A Aortic Dissection: Implications for Index Repair Strategy

International Registry of Patients Carrying TGFBR1 or TGFBR2 Mutations

Evolving practice pattern changes and outcomes in the era of hybrid aortic arch repair

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