Abstract： Objective To evaluate surgical outcomes of patients with Marfan syndrome （MFS） complicated by type A aortic dissection （AAD） during follow-up. Methods We retrospectively reviewed clinical data of 44 patients with MFS complicated by AAD who were admitted to Wuhan Asia Heart Hospital from January 2006 to January 2012. There were 31 male patients and 13 female patients with their age of 12-54 （33.0±9.8） years. Twenty-three patients underwent Bentall procedure at different time after the onset of AAD， while the other 21 patients received conservative treatment in stead of surgery because of economical or other reasons. COX regression with time-varying covariates was performed to analyze related factors， using primary end point， primary end point＋secondary end point as the outcome variables respectively， to compare postoperative outcomes and quality of life between the surgical treatment patients and conservative treatment patients. Results Postoperatively 1 patient died of multiple organ failure， and the other 22 patients survived the surgery. All the 43 patients were followed up from 1 to 75 months. The 3-year survival rate of the 22 surgical treatment patients was 95.7%， and they all had a good quality of life during follow-up. The survival condition and quality of life of the 21 conservative treatment patients was poor， and 13 patients （61.9%） died with the 3-year survival rate of only 31.7%. The main causes of their death included acute cardiac tamponade， aortic dissection rupture， acute myocardial infarction andcardiogenic shock. COX regression with time-varying covariates showed that the treatment outcomes of the surgical treatment patients were statistically different from those of the conservative treatment patients after modifying the influence caused by different operating time （OR of T_COV_ =0.088， P=0.028） . The risk of death of surgical treatment was only 8.8% of that of conservative treatment. Conclusion The prognosis of patients with MFS complicated by AAD is very poor. Therefore， all these patient， both in acute stage and chronic stage， should undergo surgical treatment as early as possible. The short-term and follow-up outcomes of surgical treatment are satisfactory.
Abstract： Marfan syndrome （MFS） is a congenital and heritable autosomal dominant disorder of the connective tissue which is often passed down through families. Its clinical presentation typically involves the skeletal， cardiovascular and ocular systems with a high natural mortality. Aortic root aneurysm and consecutive acute aortic dissection represent the main cardiovascular manifestations and main causes of morbidity and mortality in MFS. At present， the predominant therapeutic method is surgery， but surgical outcomes are quite unsatisfactory. Recent studies demonstrate that losartan， a common antihypertensive agent， is useful to treat MFS， the mechanism of which may results from inhibiting overactivation of transforming growth factor β （TGF-β） signaling. This discovery will definitely promote the transition of traditional surgical treatment of MFS into pharmacotherapy. In this review， we focus on the molecular biological pathogenesis， traditional and new therapeutic strategies for MFS patients.
ObjectiveTo evaluate the clinical effect of modified mini-root operation on aortic root diseases with the short and middle term follow-up results. MethodsWe retrospectively analyzed the data of thirty-one patients of modified mini-root operations between March 2008 to September 2012. There were 22 male and 9 female patients with mean age of 47.2±21.3 years(ranged from 28 to 71 years). Fifteen patients were diagnosed with acute aortic dissection(Standford A). Thirteen patients were of Marfan syndrome including 8 patients with aortic dissection and 3 patients of bi-leaflet aortic valve malformation with aortic dissection. The patients were followed up for 6 months to 50 months. Thirteen patients of mini-root operation without other procedure(mini-root operation group) were selected to compare with 8 patients of Bentall operation(Bentall operation group). Some clinical indexes were compared between the two groups. ResultsThree patients died in hospital, in which 1 died from low cardiac out-put syndrome and multiple organ failure, 1 from descending aortic aneurysm rupture, and 1 from acute cerebral infarction. Three patients suffered with acute renal insufficiency and received hemodialysis. During the follow-up, 1 patient received continuous kidney dialysis treatment, and 3 patients performed reoperation. Compared with the Bentall group, the cardiopulmonary bypass time was shorter (108.5±20.8 min vs. 138.5±19.0 min), postoperative blood transfusion volume in the first 24 h was less(661.6±135.0 ml vs. 1 381.2±517.5 ml) than those in the mini-root group. ConclusionModified mini-root technique can significantly shorten the operation time and reduce the amount of blood transfusion in the treatment of aortic root diseases. The modified mini-root technique has obvious effect on selected aortic root diseases during perioperative period.
Objective To retrospectively reviewed our experience of the surgical and perioperative treatment of patients suffering from critical Marfan syndrome with severe left ventricular dysfunction and to evaluate its therapeutic effect and prognosis. Methods Between January 2012 and October 2016, 15 patients diagnosed with Marfan syndrome combined with severe left ventricular dysfunction (left ventricular ejection fraction≤40% or left ventricular end diastolic diameter≥75 mm) underwent operations for aortic root aneurysm in Zhujiang Hospital and Guangdong General Hospital. Among them, 11 were males and 4 were females with a mean age of 32.9±8.7 years ranging from 19 to 55 years. Five patients with aortic dissection underwent Bentall procedure and total arch reconstruction with stent graft implantation. Two patients underwent Bentall procedure and hemi-arch replacement, seven patients underwent Bentall procedure and one patient underwent Cabrol procedure. Concomitant procedures included mitral valve repair in 12 patients, mitral valve replacement in 3 patients and tricuspid valve repair in 12 patients. Results There were 11 patients (73.3%) receiving intra-aortic balloon pumping implantation. One (6.7%) in-hospital death occurred. The left ventricular end diastolic diameter decreased from 80.5±7.4 mm to 58.3±6.0 mm (P<0.05) and the left ventricular ejection fraction improved from 37.3%±5.2% to 46.3%±4.4% 3 months postoperatively (P<0.05). The left ventricular end diastolic diameter decreased from 80.5±7.4 mm to 53.7±3.6 mm (P<0.05) and the left ventricular ejection fraction improved from 37.3%±5.2% to 57.7%±4.2% after one year (P<0.05). No death and reoperation occurred in the follow-up. Conclusion Although the patients with Marfan syndrome and severe left ventricular dysfunction usually have a high surgical mortality, the key to satisfactory outcomes of severe Marfan syndrome is adequate preoperative preparation, complete correction of all vascular lesions during the operation, application of circulatory auxiliary device and perioperative strict and long-term ICU monitoring.
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.
Aortic dissection during pregnancy is rare in clinics. Because the symptoms are lack of specificity, early diagnosis is difficult. However, the progression of aortic dissection is fast, therefore, the mortality of pregnant women and fetuses is high, and half of the death in pregnant women is due to aortic dissection. Although the development of medical condition is rapid, aortic dissection of pregnancy is still a great challenge for patients and clinicians, and is one of the most important diseases in obstetric medical disputes. In this paper, combined with the literatures published in recent years, we summarized the epidemiological characteristics and related treatment suggestions of the aortic dissection in pregnancy.
ObjectiveTo summarize the surgical treatment plan and experience of patients with Marfan syndrome complicated with Stanford type B aortic dissection, and to explore the treatment strategy selection.MethodsA retrospective analysis was conducted on 27 patients with Marfan syndrome complicated with thoracoabdominal aortic diseases who were treated in the department of cardiovascular surgery of our hospital from January 2013 to June 2019, including 13 males and 14 females, with an average age of 32.2±8.6 years. According to the patients' conditions, 19 of them received single pump-assisted blood transfusion combined with total thoracoabdominal aortic replacement (TAAAR), and 8 received thoracic endovascular aortic repair (TEVAR) in critically ill and pregnant patients. The patients were followed up in the outpatient clinic, and the thoracoabdominal aortic CT angiography was reexamined at 3 months, 6 months, 12 months and annually. The outcome of surgery, the incidence of intermediate cardiovascular adverse events, defined as the reoperation due to aortic or cardiac diseases, and intermediate survival rate were studied.ResultsAll 27 patients successfully completed the operation, the operation time was 60-852 (395.10±222.60) min, the spinal cord ischemia time was 14-26 (19.33±3.44) min, and the abdominal viscera ischemia time was 16-23 (19.83±1.94) min. Eight patients of TEVAR were all operated in acute phase and 19 patients of TAAAR in chronic phase. Two early postoperative deaths occurred in TEVAR patients. One died of puerperal infection and multiple organ dysfunction after cesarean section at the same time. After TEVAR, type A dissection re-ocurred in one patient. The family member gave up the treatment, and the patient died of the dissection ruptured after cesarean section. During the average follow-up of 47.6±36.7 months, 1 patient died of cerebrovascular accident and 9 patients were reoperated for adverse cardiovascular events, including 4 in TEVAR and 5 in TAAAR.ConclusionTAAAR is the first choice for the treatment of Marfan syndrome combined with thoracoabdominal aortic diseases. TEVAR is easy to operate, with a low incidence of early mortality and complications, but has the risk of internal leakage and avulsion, and a high reoperation rate in the middle stage, so it can be used for high-risk elderly patients not suitable for open surgery, or as a bridge therapy for emergency patients before open surgery.