Thoracic Aortic, Aortic Valve, and Mitral Valve Surgery in Pediatric and Young Adult Patients With Marfan Syndrome: Characteristics and Outcomes

Knadler, Joseph J.; LeMaire, Scott A.; McKenzie, E. Dean; Moffett, Brady S.; Morris, Shaine A.

doi:10.1053/j.semtcvs.2019.06.005

Cited by 12 publications

(11 citation statements)

References 29 publications

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“…To date, most large studies of patients with connective tissue disorders undergoing cardiothoracic surgery describe outcomes of greatest relevance to surgeons (ie, aortic complications such as rupture or dissection, need for urgent re‐operation, injury to surrounding structures, and time to valve replacement for valve‐sparing procedures). Only some outcomes overlap with those related to anesthesia care (ie, bleeding, arrhythmias, respiratory failure, and death) 6,9–12,20 . Many of the surgical studies do have the benefit of decades of experience with robust long‐term follow‐up.…”

Section: Discussionmentioning

confidence: 99%

“…In particular, patients with LDS type I, as compared to type II, have a median age of first surgery in adolescence versus adulthood 3 . Other studies show that patients in both populations may have equally aggressive disease that requires surgery at a young age to prevent fatal complications, though death from disease complications tends to occur earlier and with greater frequency in the LDS population 4–6 . A review of the Pediatric Health Information System registry, which includes pediatric patients at 40 participating institutions, showed that 294 patients with Marfan syndrome under the age of 25 were hospitalized over an 11‐year period 6 .…”

Section: Introductionmentioning

confidence: 99%

“…Other studies show that patients in both populations may have equally aggressive disease that requires surgery at a young age to prevent fatal complications, though death from disease complications tends to occur earlier and with greater frequency in the LDS population 4–6 . A review of the Pediatric Health Information System registry, which includes pediatric patients at 40 participating institutions, showed that 294 patients with Marfan syndrome under the age of 25 were hospitalized over an 11‐year period 6 . Of these 294 patients, 213 (72%) required at least one aortic root and/or valve surgery, 51 (17%) required at least one mitral valve surgery, and 43 (15%) underwent operations on both valves during a single hospitalization 6 .…”

Section: Introductionmentioning

confidence: 99%

“…A review of the Pediatric Health Information System registry, which includes pediatric patients at 40 participating institutions, showed that 294 patients with Marfan syndrome under the age of 25 were hospitalized over an 11‐year period 6 . Of these 294 patients, 213 (72%) required at least one aortic root and/or valve surgery, 51 (17%) required at least one mitral valve surgery, and 43 (15%) underwent operations on both valves during a single hospitalization 6 . Some evidence suggests that patients with LDS have a similar risk of aneurysmal disease but that aortic dissections may occur at smaller diameters than they do in Marfan syndrome patients with comparably sized aortic vasculature and may be affected more distally than the aortic root 3,7,8 …”

Section: Introductionmentioning

confidence: 99%

“…Much of the perioperative literature characterizing Marfan syndrome and LDS is surgical in nature, primarily describing novel surgical techniques and related outcomes 6,9–12 . However, the body of literature describing patient care under general anesthesia is notably limited 1 and largely restricted to orthopedic and obstetric procedures in adult patients 13–15 .…”

Section: Introductionmentioning

confidence: 99%

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Perioperative management and considerations in pediatric patients with connective tissue disorders undergoing cardiac surgery

Berger

Huh

Lee

et al. 2021

Pediatric Anesthesia

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Background Marfan syndrome and Loeys‐Dietz syndrome are connective tissue disorders associated with cardiac and vascular disease. Patients often require surgical repair, but limited data exist to describe their perioperative management. Aims Our goals were to review the perioperative features of patients with Marfan and Loeys‐Dietz syndrome that may affect anesthesia care and to describe the differences in preoperative clinical characteristics and intra‐operative anesthetic management. Methods We conducted a retrospective cohort study of patients with Marfan and Loeys‐Dietz syndrome who underwent cardiac surgery at a single institution. We collected demographic and perioperative data from the electronic medical record and performed descriptive statistics to characterize the patient populations and describe their anesthetic management. Results In 71 patients (40 Marfan, 31 Loeys‐Dietz), we found significant differences between the Marfan and Loeys‐Dietz patients in airway difficulty, preoperative weight, blood utilization, valvular disease, and age at first cardiac surgery. Patients with Loeys‐Dietz syndrome had higher preoperative rates of severe noncardiac comorbidities, including gastroesophageal reflux and asthma that required chronic medical therapy. Conclusions Despite undergoing similar surgical procedures, patients with Marfan and Loeys‐Dietz syndrome have different intrinsic patient characteristics and comorbidities that may affect their perioperative care. This retrospective cohort study identified some factors, but additional collection and reporting of patient data based on multicenter experience are essential for the ongoing optimization of perioperative care in these patient populations.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Perioperative management and considerations in pediatric patients with connective tissue disorders undergoing cardiac surgery

Berger

Huh

Lee

et al. 2021

Pediatric Anesthesia

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Extracardiac Manifestations Fail to Predict the Severity of Cardiac Phenotype in Children and Young Adults with Marfan Syndrome

John,

Young,

Lacro

et al. 2024

Pediatr Cardiol

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We performed a secondary analysis of the Pediatric Heart Network Marfan Trial public-use database to evaluate associations between extracardiac features and cardiac and aortic phenotypes in study participants. Aortic aneurysm phenotype was de ned as aortic root Z-score ≥ 4.5, aortic root growth rate ≥ 75th percentile, aortic dissection, and aortic surgery. Severe cardiac phenotype was de ned as aortic dissection, aortic Z-score ≥4.5, aortic valve surgery, at least moderate mitral regurgitation, mitral valve surgery, left ventricular dysfunction, or death. Extracardiac manifestations were characterized by speci c organ system involvement and by a novel aggregate extracardiac score that was created for this study based on the original Ghent nosology. Logistic regression analysis compared aggregate extracardiac score and systems involvement to outcomes. Of 608 participants (60% male), the median age at enrollment was 10.8 years (interquartile range: 6, 15.4). Aortic aneurysm phenotype was observed in 71% of participants and 64% had severe cardiac phenotype. On univariate analysis, skeletal (OR: 1.95, 95% CI: 1.01, 3.72; p = 0.05), skin manifestation (OR: 1.62, 95% CI: 1.13, 2.34; p = 0.01) and aggregate extracardiac score (OR: 1.17, 95% CI: 1.02, 1.34; p = 0.02) were associated with aortic aneurysm phenotype but were not signi cant in multivariate analysis. There was no association between extracardiac manifestations and severe cardiac phenotype. Thus, the severity of cardiac manifestations in Marfan syndrome was independent of extracardiac phenotype and aggregate extracardiac score. Severity of extracardiac involvement did not appear to be a useful clinical marker for cardiovascular riskstrati cation in this cohort of children and young adults with Marfan syndrome. HighlightsWe performed a secondary analysis of the Pediatric Heart Network Marfan Trial public-use database to evaluate associations between extracardiac features and cardiac and aortic phenotypes.Although skeletal and skin manifestations and aggregate extracardiac score were associated with aortic aneurysm phenotype in univariate analysis, they were not signi cant in multivariate models.Severity of cardiovascular disease in MFS was independent of extracardiac manifestations or aggregate extracardiac score.

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Marfan Syndrome

Pyeritz

2025

Emery and Rimoin’s Principles and Practice of Medical Genetics and Genomics

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Thoracic Aortic, Aortic Valve, and Mitral Valve Surgery in Pediatric and Young Adult Patients With Marfan Syndrome: Characteristics and Outcomes

Cited by 12 publications

References 29 publications

Perioperative management and considerations in pediatric patients with connective tissue disorders undergoing cardiac surgery

Perioperative management and considerations in pediatric patients with connective tissue disorders undergoing cardiac surgery

Extracardiac Manifestations Fail to Predict the Severity of Cardiac Phenotype in Children and Young Adults with Marfan Syndrome

Marfan Syndrome

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