Objective To evaluate the early and midterm results of surgical repair of thoracoabdominal aortic aneurysm(TAAA)in patients with Marfan syndrome(MFS). Methods The clinical data of patients with MFS undergoing TAAA repair in Fuwai Hospital between January 2009 and December 2017 were retrospectively analyzed.These patients were divided into two groups:MFS group(=58)and non-MFS group(=98).The baseline data,early postoperative results,and midterm follow-up outcomes were compared between these two groups. Results MFS patients were significantly younger(32 years old 45 years old,=9.603,=0.000)and more frequently had a history of aortic aneurysm or dissection(19% 0,=19.996,=0.000)than non-MFS patients.However,the proportions of males and smokers were significantly lower when compared with non-MFS patients(55.2% 80.6%,=11.489,=0.001;13.8% 46.9%,=17.686,=0.001).There was no significant difference in proportion of emergency operation,prophylactic cerebrospinal fluid drainage,operation time,intra-operative circulation management,and intra-operative blood transfusion(all >0.05).The 30-day mortality rate was significantly lower in MFS group than in non-MFS group(0 9.2%, [Formula: see text]=5.034,=0.025). Conclusions For patients with MFS,TAAA repair provides lower 30-day mortality and comparative middle-term survival.However,the re-intervention rate is higher among MFS patients,highlighting the importance of close follow-up.
Adult ; Aneurysm, Dissecting ; Aortic Aneurysm, Thoracic ; complications ; surgery ; Blood Vessel Prosthesis Implantation ; Female ; Humans ; Male ; Marfan Syndrome ; complications ; Middle Aged ; Retrospective Studies ; Treatment Outcome

PURPOSE: In this study, we evaluated the long term beneficial effect of Renin-Angiotensin-Aldosterone System (RAAS) blockade therapy in treatment of Marfan aortopathy. MATERIALS AND METHODS: We reviewed Marfan syndrome (MFS) patients who underwent aortic root replacement (ARR) between January 1996 and January 2011. All patients were prescribed beta-blockers indefinitely. We compared major aortic events including mortality, aortic dissection, and reoperation in patients without RAAS blockade (group 1, n=27) to those with (group 2, n=63). The aortic growth rate was calculated by dividing the diameter change on CT scans taken immediately post-operatively and the latest scan available. RESULTS: There were no differences in clinical parameters except for age which was higher in patients with RAAS blockade. In group 1, 2 (7%) deaths, 5 (19%) aortic dissections, and 7 (26%) reoperations occurred. In group 2, 3 (5%) deaths, 2 (3%) aortic dissections, and 3 (5%) reoperations occurred. A Kaplan-Meier plot demonstrated improved survival free from major aortic events in group 2. On multivariate Cox, RAAS blockade was an independent negative predictor of major aortic events (hazard ratio 0.38, 95% confidence interval 0.30-0.43, p=0.002). Mean diameter change in descending thoracic and supra-renal abdominal aorta was significantly higher in patients without RAAS blockade (p<0.05). CONCLUSION: In MFS patients who underwent ARR, the addition of RAAS blockade to beta-blocker was associated with reduction of aortic dilatation and clinical events.
Adrenergic beta-Antagonists/pharmacology ; Aged ; Aneurysm, Dissecting/complications/mortality/surgery ; *Angiotensin Receptor Antagonists ; Angiotensin-Converting Enzyme Inhibitors/*therapeutic use ; Aorta/pathology/*surgery ; Aortic Aneurysm/complications/mortality/surgery ; Aortic Valve ; Female ; Humans ; Male ; Marfan Syndrome/mortality/*surgery ; Middle Aged ; Renin-Angiotensin System/*drug effects

OBJECTIVETo review the current evidence about the prevalence of obstructive sleep apnea in patients with Marfan's syndrome, and discuss some proposed potential mechanisms for this relationship.

DATA SOURCESThe data in this review were mainly from Medline and PubMed articles published in English from 1990 to 2013. The search term was "Marfan's syndrome and sleep apnea".

STUDY SELECTIONClinical evidence about the epidemiology of obstructive sleep apnea in patients with Marfan's syndrome; the mechanism that causes obstructive sleep apnea; interventional therapy for patients with Marfan's syndrome, and coexisting obstructive sleep apnea.

RESULTSA high prevalence of obstructive sleep apnea exists in patients with Marfan's syndrome. The potential reasons are craniofacial abnormalities and lax upper airway muscles, which lead to high nasal airway resistance and upper airway collapse. Obstructive sleep apnea mechanically deteriorates aortic dilatation and accelerates progression of aortic aneurysms. The condition is reversible and rapid maxillary expansion and adequate continuous positive airway pressure therapy are possible effective therapies to delay the expansion of aortic diameter in patients with Marfan's syndrome.

CONCLUSIONSObstructive sleep apnea is strongly associated with Marfan's syndrome. Craniofacial abnormalities and lax upper airway are the main mechanisms. Untreated obstructive sleep apnea accelerates progression of aortic dissection and rupture. Effective therapies for obstructive sleep apnea could postpone the aortic dilatation in patients with Marfan's syndrome.

Humans ; Marfan Syndrome ; epidemiology ; etiology ; Prevalence ; Sleep Apnea, Obstructive ; complications ; epidemiology

Humans ; Infant, Newborn ; Male ; Marfan Syndrome ; complications ; Urinary Tract ; abnormalities

BACKGROUND: We reviewed the long-term outcomes of aortic root replacement at Asan Medical Center and investigated the predictors affecting mortality. MATERIALS AND METHODS: A retrospective analysis was performed on 225 consecutive adult patients undergoing aortic root replacement with mechanical conduits (n=169), porcine aortic root prosthesis (n=23), or aortic homografts (n=33) from January 1992 to September 2009. The median follow-up duration was 6.1 years (range, 0 to 18.0 years). RESULTS: The porcine root group was older than the other groups (freestyle 55.9+/-14.3 years vs. mechanical 46.3+/-14.6 years, homograft 48.1+/-14.7 years; p=0.02). The mechanical group had the highest incidence of the Marfan syndrome (mechanical 22%, freestyle 4%, homograft 3%; p=0.01). Surgery performed for infective endocarditis was more frequent in the homograft group (mechanical 10%, freestyle 10%, homograft 40%; p<0.001). The overall 30-day mortality was 5.3% (12/225). Actuarial survival rates in the mechanical, porcine root, and homograft groups were 79.4%, 81.5%, and 83.5% at 5 years and 67%, 61.9%, and 61.1% at 10 years, respectively (p=0.73). By multivariate analysis, preoperative diabetes mellitus, older age, and longer cardiopulmonary bypass time were independent predictors of mortality. Incidence of postoperative complications, including infective endocarditis and thromboembolism were comparable in all of the groups. CONCLUSION: Aortic root replacement can be safely performed with different types of prostheses as the outcome was not affected by the choice of prosthesis. Further studies are required to assess the long-term durability of biological prostheses.
Adult ; Cardiopulmonary Bypass ; Diabetes Mellitus ; Endocarditis ; Follow-Up Studies ; Heart Valve Prosthesis ; Humans ; Incidence ; Marfan Syndrome ; Multivariate Analysis ; Postoperative Complications ; Prostheses and Implants ; Retrospective Studies ; Survival Rate ; Thromboembolism ; Transplantation, Homologous

Thoracoabdominal aortic aneurysm (TAAA) involving the roots of the celiac, superior mesenteric and both renal arteries is a rare, but potentially lethal disease. The overall postoperative mortality rate is high even when the intact TAAA is electively repaired. Furthermore, the postoperative complications are often serious and they include acute renal failure, paraplegia, respiratory distress and intestinal ischemia. The inclusion technique using a visceral-aortic patch (VAP) is considered the gold standard method for visceral artery revascularization for the treatment of TAAA. However, the inclusion technique is not feasible for patients with Marfan syndrome or for those patients with inappropriate anatomy for VAP. In such cases, separate visceral revascularization is a useful alternative and this may decrease the visceral ischemic time. Herein we report on 3 cases of TAAA, and the patients all underwent successful separate visceral revascularization, including one patient with Marfan syndrome.
Acute Kidney Injury ; Aortic Aneurysm, Thoracic ; Arteries ; Humans ; Ischemia ; Marfan Syndrome ; Paraplegia ; Postoperative Complications ; Renal Artery

OBJECTIVETo investigate the impairment pattern and the influencing factors of pulmonary function in patients with Marfan and Marfanoid syndrome associated scoliosis (MS).

METHODSIn this retrospective study, totally 25 MS patients (aged 11 - 20 years, 11 boys and 14 girls) who received posterior instrumentation and fusion (Group A) and 38 adolescent idiopathic scoliosis (AIS) patients (Group B) (aged 10 - 19 years, 11 boys and 27 girls) were included from February 1998 to September 2007. The curve pattern was matched in both groups. The preoperative pulmonary function test (PFTs) were compared in two groups. And the parameters influencing the preoperative pulmonary function were analyzed in group A.

RESULTSIn Group A, the Cobb angle of thoracic curve was negatively correlated with the percentage of predicted pulmonary volumes (VC%, FVC% and FEV1%) (r = -0.514, -0.503, -0.464, P < 0.05). And the reduction of lung function parameters (VC%, FVC%, FEV1% and MMEF%) was more severe in Group A than in Group B with compared magnitude of thoracic curve (P < 0.05). In Group A, the extent of impairment of pulmonary function in patients with the number of vertebrae involved ≥ 8 were more severe than those involved < 8 vertebrae (P < 0.05). However, there was no significant difference of deterioration of lung function between the higher apex (T₄₋₈) subgroup and lower apex (T₉₋₁₂) subgroup. And no correlation was found between thoracic kyphosis and the degrees of impairment of respiration function.

CONCLUSIONSPatients with MS have mixed ventilation dysfunction, which is more severe than AIS patients with matched age and Cobb angle. The pulmonary dysfunction in MS patients can be influenced by the severity of thoracic curve and the number of involved vertebrae.

Adolescent ; Child ; Female ; Humans ; Lung ; physiopathology ; Male ; Marfan Syndrome ; complications ; physiopathology ; Respiratory Function Tests ; Retrospective Studies ; Scoliosis ; complications ; physiopathology ; Young Adult

OBJECTIVETo explore the experiences of aortic valve preservation and root reconstruction in patients with Marfan syndrome.

METHODSFrom July 2003 to Dec 2007, 22 patients with Marfan syndrome were treated by aortic valve preservation and root reconstruction. There were 12 male and 10 female, the age ranged from 10 to 57 years old with a mean of (28 +/- 10) years. The operation procedures included reimplantation technique in 9 patients, remodeling technique in 8 patients, and patch technique in 2 patients. In addition, reimplantation technique + total aorta replacement in 1 patient, remodeling technique + "aortic arch replacement + stent-elephant trunk" in 1 patient, patch technique + "aortic arch replacement + stent-elephant trunk" in 1 patient. The patients were followed-up by 17 to 64 months with a mean of (46 +/- 16) months.

RESULTSNo in-hospital and follow-up period death occurred. There was one reexploration for bleeding 1 d postoperative. No valve-related complication occurred during the follow-up. At the end of follow-up, no aortic regurgitation was demonstrate in 16 patients, but mild regurgitation in 4 patients, moderate regurgitation in 1 patient and severe regurgitation in 1 patient. Two patients with moderate and severe aortic regurgitation need reoperation 1 year postoperative.

CONCLUSIONThe early and mid-term results of aortic valve preservation and root reconstruction operations in Marfan syndrome were favorable.

Adolescent ; Adult ; Aorta ; surgery ; Aortic Aneurysm ; etiology ; surgery ; Aortic Valve ; surgery ; Child ; Female ; Follow-Up Studies ; Humans ; Male ; Marfan Syndrome ; complications ; Middle Aged ; Retrospective Studies ; Treatment Outcome ; Young Adult

PURPOSE: Aortic dissection (AoD) is one of the most common catastrophes involving the aorta. Nevertheless, early diagnosis remains to be a challenge in the Emergency Department (ED), particularly in young individuals. In this study, we attempted to identify the characteristics of acute AoD among young individuals, particular in patients with Marfan syndrome. MATERIALS AND METHODS: This was an retrospective chart-review study conducted in a tertiary referring hospital. The hospital database was queried for the combination of AoD and patients under age of 40 years. The medical charts were reviewed to obtain demographic data, clinical data and laboratory characteristics by using a standardized data collection sheet. A comparison between Marfan syndrome and non-Marfan syndrome patients was performed. RESULTS: During the 10-years period, 18 of 344 patients with acute AoD were younger than 40 years-old. Patients with Marfan syndrome developed acute AoD at a younger age than patients without Marfan syndrome. The mean diastolic blood pressure was significantly lower in patients with Marfan syndrome upon presenting to the ED than those without. Patients with Marfan syndrome had trends toward higher risk of development of type A AoD, increased recurrence rate and higher mortality rate than those without. However, statistical significance was not present. CONCLUSION: ED physicians should have high alert to acute AoD in young patients presenting with severe unexplained chest and back pain, particularly in those patients with a history of heart diseases, hypertension, and Marfan syndrome or featuring Marfanoid habitus. Acute coronary syndrome, unexplained abdominal symptoms, and sudden cardiac arrest could be the initial manifestation of AoD in young patients. A low threshold to perform enhanced computed tomography may facilitate early diagnosis and timely treatment in this patient population.
Adult ; Aneurysm, Dissecting/*complications/epidemiology/*pathology ; Aortic Aneurysm/complications/epidemiology/*pathology ; Asian Continental Ancestry Group ; Female ; Heart Defects, Congenital/complications/physiopathology ; Humans ; Hypertension/complications/physiopathology ; Male ; Marfan Syndrome/*complications/*physiopathology ; Middle Aged ; Retrospective Studies ; Young Adult

Aortic Aneurysm, Thoracic ; surgery ; C-Reactive Protein ; analysis ; Humans ; Male ; Marfan Syndrome ; complications ; Middle Aged ; Salmonella Infections ; etiology ; Stents ; adverse effects