Surgical strategy and clinical outcomes of reoperative aortic root replacement after prior aortic valve replacement / 中国胸心血管外科临床杂志

@#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.

Modified Yacoub technique with aortic annuloplasty in patients with aortic root aneurysm / 中国胸心血管外科临床杂志

@#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.

Application of valve-sparing aortic root reimplantation for aortic root disorders in patients with Marfan syndrome / 第二军医大学学报

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.

Study on the FBN1 gene mutation spectrum and association between genotype and clinical phenotype in 300 Marfan syndrome patients and their relatives / 中华胸心血管外科杂志

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 .

Surgical treatment of aortic root aneurysm in adolescent patients with Marfan syndrome / 中国胸心血管外科临床杂志

@#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

The study of surgical treatment of the bicuspid aortic valve with aortic root aneurysm / 医学研究生学报

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.

Aortic Valve Sparing Operations: A Review

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.

Evaluation of aortic root reconstruction: (actors influencing surgical results / 中华胸心血管外科杂志

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.

Two Successful Proximal Reoperation Cases after Acute Type A Dissection Repair / 日本心臓血管外科学会雑誌

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.

Aortic Root Replacement with a Freestyle Porcine Valve in a Young Woman with Systemic Lupus Erythematosus and Antiphospholipid Antibodies / 日本心臓血管外科学会雑誌

Aortic root replacement with a Freestyle^TM 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^TM stentless porcine valve offers an acceptable alternative to mechanical prostheses, especially for cases with contraindication for anticoagulant therapy, associated with antiphospholipid antibodies.