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Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder characterized by fibrillin-1 protein abnormalities, predisposing individuals to cardiovascular complications such as aortic root dilation and mitral valve prolapse (MVP). Management often requires surgical intervention, with the modified Bentall procedure being a standard approach for aortic root replacement. However, managing concomitant mitral valve pathology poses additional challenges due to the complex anatomical relationship between the two valves and the prolonged nature of the procedure. We present a case report of a 35-year-old male with Marfan syndrome who underwent a successful simultaneous modified Bentall procedure and mitral valve replacement for concurrent aortic root aneurysm and mitral valve prolapse. Surgical interventions involved meticulous attention to detail to mitigate risks associated with malpositioned coronary ostial sutures, debridement of the severely calcified mitral valve, and achieving hemostasis. Following surgery, the patient exhibited favorable postoperative outcomes, with evidence of optimal valve function and improved systolic and diastolic parameters, indicative of a successful recovery.
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RESUMEN Background: La cirugía de reemplazo de la raíz aórtica con preservación valvar tipo reimplante (RRAoPV) está indicada en pacientes con aneurisma de la raíz aórtica. Objetivos: Evaluar los resultados a mediano plazo de esta técnica quirúrgica en niños y adolescentes de nuestra institución. Material y métodos: Estudio retrospectivo. Se incluyó a 21 pacientes a quienes se realizó cirugía de RRAoPV entre julio de 2006 y julio de 2022. Se evaluó la relación entre las variables basales y la progresión de insuficiencia aórtica (IAo). Resultados: La mediana de edad fue de 13 años. Diecisiete pacientes tenían enfermedad del tejido conectivo, (3 de ellos además con aorta bicúspide), 2 cardiopatías congénitas operadas aisladas, 1 válvula aórtica bicúspide aislada y 1 síndrome de Turner. Trece pacientes tenían IAo leve y 5 moderada en el preoperatorio. Tres pacientes requirieron reemplazo aórtico precoz, 2 por endocarditis y uno por IAo aguda grave. La mediana de seguimiento fue de 4,1 años. De 17 pacientes en seguimiento, 2 evolucionaron con IAo leve-moderada, 2 moderada y 3 grave. Uno de ellos requirió cirugía de Bentall. No se encontró asociación del grado de IAo preoperatoria, ni de la presencia de aorta bicúspide, con la progresión de la IAo postoperatoria. No hubo asociación entre el tipo de coaptación valvular postoperatorio y el desarrollo de la IAo mayor que leve en el seguimiento. Conclusiones: El RRAoPV tipo reimplante es una técnica quirúrgica viable en pacientes pediátricos con buenos resultados a mediano plazo y baja tasa de reoperación.
ABSTRACT Background: Valve-sparing aortic root replacement (VSARR) with reimplantation technique is indicated in patients with aortic root aneurysm. Objective: To evaluate the mid-term outcomes of this surgical technique in children and adolescents in our institution. Methods: Retrospective study. Twenty-one patients who underwent VSARR surgery were included between July 2006 and July 2022. The relationship between baseline variables and progression of aortic regurgitation (AR) was assessed. Results: The median age was 13 years. Seventeen patients had connective tissue disorders (3 with bicuspid aortic valve), 2 operated congenital heart disease, 1 isolated bicuspid aortic valve and 1 Turner syndrome. Thirteen patients had mild AR and 5 had moderate AR preoperatively. Three patients underwent early aortic replacement, 2 due to endocarditis and 1 due to severe acute AR. The median follow-up was 4.1 years. Of 17 patients at follow-up, 2 developed mild-moderate AR, 2 moderate AR and 3 severe AR. One of them underwent Bentall surgery. No association was found between the degree of preoperative AR or the presence of bicuspid aortic valve and the progression of postoperative AR. There was no association between the type of postoperative valve coaptation and the development of AR greater than mild at follow-up. Conclusions: VSARR with reimplantation is a feasible surgical technique with favorable mid-term outcomes and low reoperation rate in pediatric patients.
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@#Objective To summarize the surgical strategy of reoperative aortic root replacement after prior aortic valve replacement (AVR), and analyze the early and mid-term outcomes. Methods From April 2013 to January 2020, 75 patients with prior AVR underwent reoperative aortic root replacement in Fuwai Hospital. There were 54 males and 21 females with a mean age of 56.4±12.7 years. An emergent operation was performed in 14 patients and an elective operation in 61 patients. The indications were aortic root aneurysm in 38 patients, aortic dissection involving aortic root in 30 patients, root false aneurysm in 2 patients, prosthesis valve endocarditis with root abscess in 2 patients, and Behçet's disease with root destruction in 3 patients. The survival and freedom from aortic events during the follow-up were evaluated with the Kaplan-Meier survival curve and the log-rank test. Results The operative procedures included prosthesis-sparing root replacement in 45 patients, Bentall procedure in 26 patients, and Cabrol procedure in 4 patients. Operative mortality was 1.3% (1/75). A composite of adverse events occurred in 5 patients, including operative death (n=1), stroke (n=1), and acute renal injury necessitating hemodialysis (n=3). The follow-up was available for all 74 survivors, with the mean follow-up time of 0.5-92.0 (30.3±25.0) months. Four late deaths occurred during the follow-up. The survival rate at 1 year, 3 years and 6 years was 97.2%, 91.4% and 84.4%, respectively. Aortic events developed in 2 patients. The rate of freedom from aortic events at 1 year, 3 years, and 6 years was 98.7%, 95.0% and 87.7%, respectively. There was no difference in rate of survival or freedom from aortic events between the elective patients and the emergent patients. Conclusion Reoperative aortic root replacement after prior AVR can be performed to treat the root pathologies after AVR, with acceptable early and mid-term outcomes.
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@#Objective To evaluate the feasibility and safety of modified Yacoub technique with aortic annuloplasty in the patients with aortic root aneurysm and dilatation of aortic annular base. Methods We performed a retrospective review of 6 patients with aortic root aneurysm undergoing modified Yacoub technique with aortic annuloplasty from November 2017 to January 2019. There were 5 males and 1 female, with a mean age of 54.1±12.3 years. The preoperative cardiac function of 3 patients was in New York Heart Association (NYHA) classⅡand the other 3 patients were in class Ⅲ. There were two patients with bicuspid aortic valve, and no Marfan syndrome. There was aortic regurgitation in the patients measured by the echocardiogram, 1 in mild aortic regurgitation, 1 in moderate aortic regurgitation, and 4 in severe aortic regurgitation. The diameter of aortic annular base was 27.8±1.9 mm, and the largest diameter of aortic root was 49.8±3.7 mm. Six patients underwent modified Yacoub technique with aortic annuloplasty, including 5 patients who underwent aortic cusp repair at the same time. Results All 6 identified patients survived. There was no severe complication (bleeding, stroke, or acute renal failure). The cardiopulmonary bypass time was 204.6±13.5 min, aortic cross-clamping time 168.0±17.1 min, mechanical ventilation time 21.3±19.5 h, ICU stay time 67.8±62.2 h. The follow-up time ranged from 4 to 18 months with an average time of 12.8±4.7 months. Patients' cardiac function improved postoperatively with four patients in NYHA classⅠand two patients with classⅡ. Two patients had no aortic valve regurgitation, four patients had mild regurgitation. Left ventricular end diastolic volume decreased significantly (118.6±20.4 mL vs. 169.1±58.4 mL, P<0.05). Conclusion The modified Yacoub technique with aortic annuloplasty is effective and safe for the patients with aortic root aneurysm and dilatation of aortic annular base, and the early- and mid-term outcomes are satisfactory.
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ObjectiveTo evaluate the appropriate indications, key points of surgical techniques, and early to midterm outcomes of valve-sparing aortic root reimplantation in patients with Marfan syndrome. MethodsThe clinical data of 52 patients with Marfan syndrome who underwent valve-sparing aortic root reimplantation in our department from June 2006 to July 2017 were retrospectively analyzed. Preoperative patient characteristics, surgical techniques, and follow-up outcomes were summarized. ResultsThe patients had a mean age of (36.6±14.5) years, with 35 males and 17 females. Severe aortic valve insufficiency (AI) was found in 37 cases (71.2%) preoperatively. Echocardiography indicated that the average diameter of aortic annulus, aortic sinus, and sinotubular junction of this cohort were 22-30 mm, 40-56 mm, and 34-51 mm, respectively. Pure valve-sparing aortic root reimplantation was performed in 40 patients presenting aortic root aneurysm, with a cross-clamp time of 61-108 min and a mean of (81.3±11.6) min, and with a cardiopulmonary bypass time of 88-129 min and a mean of (97.3±10.8) min. The other 12 patients with Stanford type A aortic dissection underwent concomitant total arch replacement and elephant trunk implantation in the descending aorta, with a cross-clamp time of 93- 126 min and a mean of (107.4±11.2) min, and with a cardiopulmonary bypass time of 127-180 min and a mean of (143.5±17.1) min. Vascular prostheses with a diameter of ≤30 mm were adopted in all patients. Aortic root bleeding was drained to the right atriums in 9 patients. Transesophageal echocardiography was performed in 42 cases before extracorporeal circulation removal, with 16 cases presenting no AI, 18 presenting trace AI, and 8 presenting mild AI. There was one in-hospital death (1.9%) and no secondary thoracotomy for bleeding or other major complications. The mean follow-up was (3.2±2.1) years, with 43 cases completed the follow-up and no death during follow-up. Echocardiography in the latest follow-up indicated that 6 cases presented no AI, 24 presented trace AI, 12 presented mild AI, and only one case presented moderate AI, with 97.7% (42/43) patients free from moderate AI. No case underwent aortic valve replacement for severe AI. There were 39 (90.7%) patients in New York Heart Association classIand 4 (9.3%) patients in classII . ConclusionApplication of valve-sparing aortic root reimplantation using vascular prostheses with appropriate diameter is safe and effective for suitable patients with Marfan syndrome, and can obtain satisfactory outcomes.
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Objective To investigate the correlations between the FBN1 gene mutation types and the clinical phenotype . Methods 87 probands with Marfan or Marfan-like syndromes and their family members were enrolled in this study ( total 300 cases).The clinical manifestations of each patients involving the ocular, cardiovascular system, skeletal system and other im-plicated systems were collected and evaluated .According to the clinical manifestations , these patients were divided into two groups, namely aortic dissection group and aortic root aneurysm group.Blood samples were taken from patients and DNA se-quencing was performed on each patient by the genetic aortic disease gene Panel .The detected single nucleotide variants ( SNVs)/indel were interpreted according to the ACMG guidelines, and the pathogenic variation was confirmed through Sanger sequencing.The aortic wall tissue was obtained from MFS patients who underwent surgery .The correlations between genotypes and clinical phenotypes were further explored by comparing the aortic wall tissue histological specimens of each genotype pa-tient.Results A total of 92 FBN1 mutations(31%) were detected in 300 people with Marfan syndromes or Marfan-like syn-dromes, 18 of which were undiscovered mutations.There were 49 missense mutations(53.26%), 13 splicing mutations (14.13%), 17 frameshift mutations(18.48%), and 13 nonsense mutations(14.13%).In this cohort, 24 cases had aortic dissection and 25 cases were aortic root aneurysm.Statistical analysis revealed that patients with aortic dissection mostly ap-peared in frameshift mutations(29.17% vs.4.00%, P =0.017).However, patients with aortic root aneurysm mostly ap-peared in missense mutations(72.00% vs.37.50%, P =0.015), and accompanied with ectopia lentis(41.67% vs. 8.33%, P=0.008).Pathological specimens staining found that elastic fibers in the aortic wall of patients with frameshift mu-tations are sparser, and the smooth muscle cells are more deficient and more disorganized than patients with missense muta-tions.Conclusion FBN1 gene frameshift mutations result a lack of elastic fibers and disorganized smooth muscle cells in aor-tic wall and are presented more in patients with aortic dissection than aortic root aneurysm .
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@#Objective To study the mid-term and long-term postoperative results of Bentall procedure in patients with Marfan syndrome. Methods From February 2009 to July 2016, ten adolescent patients (mean age of 12.30±2.31 years ranged 9-16 years, mean height of 172.50±12.55 mm, mean weight of 48.60±17.08 kg) underwent Bentall procedure in our hospital. All these teenage patients were with Marfan syndrome, 6 boys and 4 girls. Five of them underwent mitral valve replacement procedure at the same time while 2 of them with tricuspid valvuloplasty. Results No mortality was found in hospitalization. One patient suffered respiratory failure after surgery. No renal failure, mediastinal infection or re-operation caused by bleeding were observed. All were well followed up for 62 months. One died of malignant arrhythmia, the other were in good condition with the latest interview. Conclusion Teenage patients with Marfan syndrome who underwent Bentall procedure have favorable outcomes in mid-term and long-term follow-up, they probably will not suffer anti-coagulation complications and restriction of growth
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Bicuspid aortic valve (BAV) is one of the most common congenital valvular diseases, which is often associated with aortic stenosis or insufficiency.Aortic disease is an important component of BAV, aortic root aneurysm is the most finding when it comes to the dilatation of any or all segments of the proximal aorta from the aortic root to the aortic arch.The choice of operation has a great influence on the prognosis of patients.This article discusses the anatomic basis, pathophysiology, surgical treatment and treatment effects of the bicuspid aortic valve with aortic root aneurysm.
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Aortic valve sparing operations were developed to preserve the native aortic valve during surgery for aortic root aneurysm as well as surgery for ascending aortic aneurysms with associated aortic insufficiency. There are basically two types of aortic valve sparing oprations: remodeling of the aortic root and reimplantation of the aortic valve. These operations have been performed for over two decades and the clinical outcomes have been excellent in experienced hands. Although remodeling of the aortic root is physiologically superior to reimplantation of the aortic valve, long-term follow-up suggests that the latter is associated with lower risk of developing aortic insufficiency. Failure of remodeling of the aortic root is often due to dilatation of the aortic annulus. Thus, this type of aortic valve sparing should be reserved for older patients with ascending aortic aneurysm and normal aortic annulus whereas reimplantation of the aortic valve is more appropriate for young patients with inherited disorders that cause aortic root aneurysms. This article summarizes the published experience with these two operations. They are no longer experimental procedures and should be part of the surgical armamentarium to treat patients with aortic root aneurysm and ascending aortic aneurysms with associated aortic insufficiency.
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Humans , Aneurysm , Aortic Aneurysm , Aortic Valve , Dilatation , Hand , ReplantationABSTRACT
Objective Evaluate the outcome of aortic root reconstruction on the analysis of the risk factors influencing surgical results. Methods Between August 1996 and November 2009, 92 patients(56 men, 36 women) aged from 14 to 77years [mean (44.8 ±1.4) years] with aortic root aneurysm underwent aortic root reconstruction. 72 patients had over moderate aortic valve insufficiency. 47 patients suffered from Marfan syndrome. The aortic pathology was aortic dissection in 45. Bentall technique was used in 59 patients, the button technique in 13, the David I with the Valsalva graft in 6 patients and the aortic valve resuspension in 14 patients. Results The hospital mortality rate was 8.7%. The major complications 31. 7%. 18patients died during the period of follow-up. Late complications among 55 survivors were 12. Univariate predictors of the morbidity were the presence of male, non-Marfan, concomitant procedure, deep hypothermia cardiac arrest, aortic cross clamp time and blood infusion. Risk facts for mortality were emergent or urgent operation, aortic dissection, concomitant procedure, aortic cross clamp time and blood infusion. Multivariate analysis revealed risk factors of concomitant procedure and blood infusion were responsible for both morbidity and mortality. The overall long-term survival rate is (97.1 ±2.0)% at 1-year, (88.1 ±4.7)% at 5-year, (54.0 ±9.2)% at 10-year. The mean for survival time is (9.9 ±0.59) years, 95% confidence interval 8.70 -11.01. Conclusion The aortic root restitution procedures are safe and effective in general. The short and long-term outcome is satisfactory. The button technique is the first choice for reimplantation coronary patch. Valve-sparring aortic root reconstructions show promise in safety and applicability.
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We report 2 cases of successful proximal reoperations after acute type A dissection. Case 1 : A 53-year-old man underwent ascending aorta and aortic arch replacement and aortic valve re-suspension for acute type A dissection with aortic valve regurgitation in 1992. Thirteen years after the first operation, computed tomography demonstrated a Valsalva aneurysm (74 mm) and Doppler echocardiography showed moderate aortic valve regurgitation. Therefore, we performed an operation. We could not locate the dissection in the Valsalva sinus, and the aortic valve cusps had organic change. A David procedure was performed. The postoperative course was uneventful and he was discharged on the 19th postoperative day. Case 2 : A 65-year-old woman underwent ascending aorta replacement and aortic valve resuspension for acute type A dissection with aortic valve regurgitation in 1997, but 11 years after the first operation, computed tomography demonstrated a Valsalva aneurysm (55 mm) and arch aneurysm (65 mm) with stenosis of the innominate vein and she had facial and left arm edema. Doppler echocardiography showed moderate aortic valve regurgitation. We could not find the location of dissection in the Valsalva sinus or aortic arch, and aortic valve cusps had no organic change. A Bentall procedure and total arch replacement were performed and her postoperative course was uneventful.
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Aortic root replacement with a Freestyle<sup>TM</sup> stentless porcine valve (Medtronic Inc.) was performed on a 32-year-old woman for aortic root aneurysm. The patient had been given a diagnosis of systemic lupus erythematosus and had been maintained on steroid therapy for 15 years. Lupus anticoagulant was present and the anticardiolipin antibody titer was abnormal as follows: IgG, 2.0IU/ml (normal<1.0IU/ml). For the patient requiring aortic root reconstruction, many options are available. The use of a biological valved conduit should be considered for patients in whom anticoagulation is not desirable. The Freestyle<sup>TM</sup> stentless porcine valve offers an acceptable alternative to mechanical prostheses, especially for cases with contraindication for anticoagulant therapy, associated with antiphospholipid antibodies.