RESUMEN
Background/Objectives: To evaluate how the surgical technique and type of implanted intraocular lens influence the postoperative visual acuity and complications in ectopia lentis associated to Marfan syndrome patients. Materials and Methods: The medical records and videos of ectopia lentis surgeries in patients (children and adults) with Marfan syndrome, were retrospectively reviewed and compared. The study included 33 eyes that underwent four different intraocular lens implantation (IOL) techniques: IOL in conjunction with a simple capsular tension ring, IOL in conjunction with a Cionni modified capsular tension ring (m-CTR), two-point scleral IOL fixation and IOL with one haptic in the bag and one haptic sutured to the sclera. Results: Vision significantly improved from a mean preoperative visual acuity of 0.1122 to a mean postoperative visual acuity of 0.4539 in both age groups (p < 0.0001), with no difference in the primary outcome between children and adults. The most common surgical technique used in both age groups was IOL in conjunction with an m-CTR. There was only one major postoperative complication requiring additional surgery. Conclusions: Zonular weakness mainly influenced by age was the most important selection criterion for the surgical approach. Regardless of the technique employed, the postoperative visual acuity was improved in both adults and children.
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Desplazamiento del Cristalino , Implantación de Lentes Intraoculares , Síndrome de Marfan , Agudeza Visual , Humanos , Síndrome de Marfan/complicaciones , Síndrome de Marfan/cirugía , Síndrome de Marfan/fisiopatología , Desplazamiento del Cristalino/cirugía , Desplazamiento del Cristalino/etiología , Adulto , Niño , Femenino , Masculino , Implantación de Lentes Intraoculares/métodos , Implantación de Lentes Intraoculares/efectos adversos , Adolescente , Estudios Retrospectivos , Persona de Mediana Edad , Resultado del Tratamiento , Preescolar , Adulto JovenRESUMEN
BACKGROUND: Marfan Syndrome is an autosomal dominant disease caused by pathogenetic variants in the FBN1 gene. The progressive dilatation of the aorta and the potential risk of acute aortic syndromes influence the prognosis of these patients. We aim to describe population characteristics, long-term survival, and re-intervention patterns in patients who underwent aortic surgery with a previously confirmed clinical diagnosis of Marfan Syndrome in a middle-income country. METHODS: A retrospective single-center case series study was conducted. All Marfan Syndrome patients who underwent aortic procedures from 2004 until 2021 were included. Qualitative variables were frequency-presented, while quantitative ones adopted mean ± standard deviation. A subgroup analysis between elective and emergent procedures was conducted. Kaplan-Meier plots depicted cumulative survival and re-intervention-free. Control appointments and government data tracked out-of-hospital mortality. RESULTS: Fifty patients were identified. The mean age was 38.79 ± 14.41 years, with a male-to-female ratio of 2:1. Common comorbidities included aortic valve regurgitation (66%) and hypertension (50%). Aortic aneurysms were observed in 64% without dissection and 36% with dissection. Surgical procedures comprised elective (52%) and emergent cases (48%). The most common surgery performed was the David procedure (64%), and the Bentall procedure (14%). The in-hospital mortality rate was 4%. Complications included stroke (10%), and acute kidney injury (6%). The average follow-up was 8.88 ± 5.78 years. Survival rates at 5, 10, and 15 years were 89%, 73%, and 68%, respectively. Reintervention rates at 1, 2.5, and 5 years were 10%, 14%, and 17%, respectively. The emergent subgroup was younger (37.58 ± 14.49 years), had the largest number of Stanford A aortic dissections, presented hemodynamic instability (41.67%), and had a higher requirement of reinterventions in the first 5 years of follow-up (p = 0.030). CONCLUSION: In our study, surveillance programs played a pivotal role in sustaining high survival rates and identifying re-intervention requirements. However, challenges persist, as 48% of the patients required emergent surgery. Despite not affecting survival rates, a greater requirement for reinterventions was observed, emphasizing the necessity of timely diagnosis. Enhanced educational initiatives for healthcare providers and increased patient involvement in follow-up programs are imperative to address these concerns.
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Síndrome de Marfan , Humanos , Síndrome de Marfan/complicaciones , Síndrome de Marfan/cirugía , Masculino , Femenino , Estudios Retrospectivos , Adulto , Persona de Mediana Edad , Disección Aórtica/cirugía , Adulto Joven , Aneurisma de la Aorta/cirugíaRESUMEN
Single-stage total aortic replacement represents a comprehensive approach for patients at high risk of aorta-related complications between procedures. It not only avoids staged surgical treatment but also facilitates quicker rehabilitation. Opting for a radical surgery in such cases can yield superior outcomes compared with a staged approach, making it particularly suitable for young patients with aorta-related risk factors. Moreover, a single-stage aorta repair reduces the likelihood of subsequent aortic interventions.
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Disección Aórtica , Implantación de Prótesis Vascular , Síndrome de Marfan , Humanos , Disección Aórtica/cirugía , Disección Aórtica/diagnóstico , Síndrome de Marfan/complicaciones , Síndrome de Marfan/cirugía , Implantación de Prótesis Vascular/métodos , Aneurisma de la Aorta Torácica/cirugía , Aneurisma de la Aorta Torácica/diagnóstico , Masculino , AdultoRESUMEN
A 52-year-old woman with Marfan syndrome developed Stanford type B aortic dissection and was treated with thoracic endovascular aortic repair. However, 29 months later, she presented with retrograde Stanford type A aortic dissection. We successfully performed aortic arch replacement with the frozen elephant trunk technique and valve-sparing aortic root replacement. The advantages of the frozen elephant trunk technique are that the distal anastomosis can be created without stent-graft resection and the cardiac arrest time is shortened. Therefore, the frozen elephant trunk technique was considered valuable and safe in this potentially lethal situation.
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Aneurisma de la Aorta Torácica , Disección Aórtica , Procedimientos Endovasculares , Síndrome de Marfan , Humanos , Femenino , Síndrome de Marfan/complicaciones , Síndrome de Marfan/cirugía , Persona de Mediana Edad , Disección Aórtica/cirugía , Disección Aórtica/etiología , Disección Aórtica/diagnóstico por imagen , Aneurisma de la Aorta Torácica/cirugía , Aneurisma de la Aorta Torácica/diagnóstico por imagen , Aneurisma de la Aorta Torácica/etiología , Implantación de Prótesis Vascular , Aorta Torácica/cirugía , Aorta Torácica/diagnóstico por imagen , Reparación Endovascular de AneurismasRESUMEN
PURPOSE: To report the long-term clinical and endothelial cell count (ECC) results of lensectomy with primary anterior chamber iris claw lens implantation in the eyes of patients ≤18-year-old with ectopia lentis due to Marfan syndrome. METHODS: The medical records of Marfan patients operated on at a single institution from September 2007 to August 2020, with minimum follow-up of 2 years, were reviewed retrospectively. The following data were analyzed: sex, age at surgery, indication for surgery, the position of the lens in relation to the undilated and dilated pupil, corneal endothelial cell counts (ECC), peri- and postoperative complications, pre- and postoperative best-corrected visual acuity. RESULTS: A total of forty-two eyes of 23 patients (12 girls and 11 boys) were included. At least two or more postoperative ECCs were collected from 33 eyes (17 patients). Median age at IOL implantation was 6.1 years (range, 1.8-18). Median overall follow-up time was 6.2 years (range, 2-13.5). Median ECC follow-up time was 6.2 years (range, 2-10). Mean best-corrected visual acuity was 0.71 ± 0.38 logMAR before surgery and 0.02 ± 0.25 logMAR at final follow-up. The mean annual ECC decline was 0.71% ± 2.24. Total cell loss from first to last postoperative measurement was 150 cells ± 394 cells/mm2 (4.81%). Pre- and first postoperative data were available for 17 eyes of 10 patients, with a mean cell loss before and directly after surgery of 269 ± 268 cells (7.94%). Surgery related complications were iris bombé due to blockage of peripheral iridectomy in 3 eyes and claw dislocation due to direct impact trauma in 3 eyes. CONCLUSIONS: In our large, pediatric study cohort, anterior chamber iris claw IOL implantation resulted in an excellent visual outcome and normal endothelial cell loss compared with normative data. Safety measures are recommended to avoid traumatic dislocation of IOLs.
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Cámara Anterior , Desplazamiento del Cristalino , Iris , Implantación de Lentes Intraoculares , Síndrome de Marfan , Agudeza Visual , Humanos , Desplazamiento del Cristalino/cirugía , Síndrome de Marfan/complicaciones , Síndrome de Marfan/cirugía , Femenino , Masculino , Niño , Implantación de Lentes Intraoculares/métodos , Estudios Retrospectivos , Agudeza Visual/fisiología , Preescolar , Adolescente , Iris/cirugía , Cámara Anterior/patología , Estudios de Seguimiento , Lactante , Lentes Intraoculares , Complicaciones Posoperatorias , Endotelio Corneal/patología , Recuento de CélulasRESUMEN
OBJECTIVES: The aim of this study was to report on mid-term outcomes after endovascular aortic repair (EVAR) in patients with Marfan (MFS) or Loeys-Dietz (LDS) syndrome. METHODS: We analysed data from 2 European centres of patients with MFS and LDS undergoing EVAR. Patients were analysed based on (i) timing of the procedure (planned versus emergency procedure) and (ii) the nature of the landing zone (safe versus non-safe). The primary end-point was freedom from reintervention. Secondary end-points were freedom from stroke, bleeding and death. RESULTS: A population of 419 patients with MFS (n = 352) or LDS (n = 67) was analysed for the purpose of this study. Thirty-nine patients (9%) underwent EVAR. Indications for thoracic endovascular aortic repair or EVAR were aortic dissection in 13 (33%) patients, aortic aneurysm in 22 (57%) patients and others (intercostal patch aneurysm, penetrating atherosclerotic ulcer, pseudoaneurysm, kinking of frozen elephant trunk (FET)) in 4 (10%) patients. Thoracic endovascular repair was performed in 34 patients, and abdominal endovascular aortic repair was performed in 5 patients. Mean age at 1st thoracic endovascular aortic repair/EVAR was 48.5 ± 15.4 years. Mean follow-up after 1st thoracic endovascular aortic repair/EVAR was 5.9 ± 4.4 years. There was no statistically significant difference in the rate of reinterventions between patients with non-safe landing zone and the patients with safe proximal landing zone (P = 0.609). Furthermore, there was no increased probability for reintervention after planned endovascular intervention compared to emergency procedures (P = 0.916). Mean time to reintervention, either open surgical or endovascular, after planned endovascular intervention was in median 3.9 years (95% confidence interval 2.0-5.9 years) and 2.0 years (95% confidence interval -1.1 to 5.1 years) (P = 0.23) after emergency procedures. CONCLUSIONS: EVAR in patients with MFS and LDS and a safe landing zone is feasible and safe. Endovascular treatment is a viable option when employed by a multi-disciplinary aortic team even if the landing zone is in native tissue.
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Aneurisma de la Aorta Torácica , Implantación de Prótesis Vascular , Procedimientos Endovasculares , Síndrome de Loeys-Dietz , Síndrome de Marfan , Humanos , Adulto , Persona de Mediana Edad , Síndrome de Loeys-Dietz/cirugía , Síndrome de Loeys-Dietz/complicaciones , Reparación Endovascular de Aneurismas , Síndrome de Marfan/complicaciones , Síndrome de Marfan/cirugía , Implantación de Prótesis Vascular/efectos adversos , Implantación de Prótesis Vascular/métodos , Procedimientos Endovasculares/efectos adversos , Procedimientos Endovasculares/métodos , Resultado del Tratamiento , Estudios Retrospectivos , Aneurisma de la Aorta Torácica/cirugía , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/cirugíaRESUMEN
PURPOSE: To evaluate the safety and efficacy of capsular tension ring and capsular hook (CTR-CH) implantation in Marfan syndrome (MFS) patients with ectopia lentis (EL). SETTING: Eye and ENT Hospital of Fudan University, Shanghai, China. DESIGN: Retrospective propensity score-matched cohort study. METHODS: This study included patients with MFS who had in-the-bag intraocular lens (IOL) implantation assisted by CTR-CH or modified CTR (MCTR). The safety analysis focused on the resurgery rate. The efficacy analysis compared the corrected distance visual acuity (CDVA) and the incidence of laser capsulotomy after propensity score matching (PSM). RESULTS: This study encompassed 148 eyes that had the CTR-CH procedure and 162 eyes that received MCTR implantation. In the CTR-CH group, the median age at the time of surgery was 5 years, with a mean follow-up duration of 1.81 ± 0.4 years. 5 eyes (3.38%) required a second surgery because of retinal detachment (2, 1.35%), IOL decentration (2, 1.35%), and CH dislocation (1, 0.68%). The resurgery rate was comparable with that of the MCTR group ( P = .486). After PSM, a total of 108 eyes were recruited in each group. Postoperative CDVA was significantly improved in both groups (both P < .001), but comparable between the groups ( P = .057). The posterior capsular opacification took place earlier ( P = .046) while the anterior capsular opacification required laser capsulotomy at a later stage ( P = .037) compared with the MCTR group. CONCLUSIONS: The CTR-CH procedure was a feasible, safe, and efficient approach for managing EL in patients with MFS.
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Desplazamiento del Cristalino , Cápsula del Cristalino , Implantación de Lentes Intraoculares , Síndrome de Marfan , Agudeza Visual , Humanos , Desplazamiento del Cristalino/cirugía , Desplazamiento del Cristalino/fisiopatología , Estudios Retrospectivos , Síndrome de Marfan/complicaciones , Síndrome de Marfan/cirugía , Agudeza Visual/fisiología , Masculino , Femenino , Cápsula del Cristalino/cirugía , Niño , Preescolar , Facoemulsificación , Prótesis e Implantes , Resultado del Tratamiento , Adolescente , Implantación de Prótesis , AdultoRESUMEN
BACKGROUND Micropulse transscleral cyclophotocoagulation is a non-invasive, widely employed procedure that uses diode laser to target the ciliary body to lower the intraocular pressure. Despite its acknowledged efficacy, certain complications are recognized, with neurotrophic keratopathy being a rare yet serious consequence. This report seeks to shed light on a potential high-risk subgroup susceptible to neurotrophic keratopathy, exemplified by our patient with Marfan syndrome, a condition characterized by thin sclera. CASE REPORT Our patient, who was confirmed to have Marfan syndrome with pseudophakic glaucoma, underwent micropulse transscleral cyclophotocoagulation due to high intraocular pressure bilaterally and subsequently quickly manifested neurotrophic keratopathy in both eyes postoperatively. Swift initiation of management involved a comprehensive approach, including topical antibiotics, preservative-free lubrication, medroxyprogesterone acetate 1%, serum balanced salt solution (BSS) 50%, and the application of bandage contact lenses to expedite healing. Fortunately, the left eye demonstrated resolution within 10 days, while the right eye exhibited delayed healing, leading to subsequent scarring. CONCLUSIONS This report highlights the critical importance of recognizing populations predisposed to neurotrophic keratopathy before subjecting them to micropulse transscleral cyclophotocoagulation. Such awareness allows for the fine-tuning of procedural parameters, offering a strategic approach to mitigate the risk of neurotrophic keratopathy development. By further exploring and recognizing potential risk factors, clinicians can enhance patient outcomes and refine the safety profile of micropulse transscleral cyclophotocoagulation.
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Glaucoma , Síndrome de Marfan , Humanos , Cuerpo Ciliar/cirugía , Coagulación con Láser/efectos adversos , Coagulación con Láser/métodos , Síndrome de Marfan/complicaciones , Síndrome de Marfan/cirugía , Glaucoma/etiología , Glaucoma/cirugía , Medición de Riesgo , Resultado del TratamientoRESUMEN
Aortic root aneurysms are one of the most common aortic root diseases, involving the aortic valve, aortic sinus, bilateral coronary arteries, and part of the ascending aorta. It is a life-threatening aortic disease with a high mortality rate of approximately 90%, due to aortic aneurysm rupture. Aortic valve insufficiency is one of the most common complications of aortic root aneurysms that can lead to acute left heart failure. The etiology of aortic root aneurysms is not yet completely clear and is mainly related to genetic diseases, such as Marfan syndrome and atherosclerosis. It can also occur secondary to aortic valve stenosis or a bivalve deformity. Surgery is the primary treatment for aortic root aneurysms, and aortic root replacement is a classic surgical method. However, the incidences of perioperative complications and mortality are relatively high, particularly in high-risk patients. In recent years, the anatomical structure of the aortic root has been gradually refined, and an in-depth understanding of root aneurysms has led to individualized treatment methods. Conservative drug therapy (ß-receptor blockers, angiotensin-converting enzyme inhibitors, or angiotensin receptor blockers), Bentall and modified Bentall surgeries (Button technology, Cabrol surgery, and modified Cabrol surgery), valve-sparing aortic root replacement (David and Yacoub), personalized external aortic root support, and endovascular intervention therapy have significantly improved the perioperative and long-term survival rates of patients with aortic root aneurysms. However, different treatment methods have their own advantages and disadvantages. This review aimed to summarize the current research progress and treatment of aortic root aneurysms.
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Aneurisma de la Aorta Torácica , Enfermedades de la Aorta , Aneurisma de la Raíz de la Aorta , Insuficiencia de la Válvula Aórtica , Síndrome de Marfan , Humanos , Aorta/cirugía , Aneurisma de la Aorta Torácica/terapia , Síndrome de Marfan/cirugía , Insuficiencia de la Válvula Aórtica/complicaciones , Válvula Aórtica/cirugía , Resultado del TratamientoRESUMEN
BACKGROUND: Spinal deformities are common in Marfan syndrome (MFS). They usually involve the thoraco-lumbar spine but rarely involves the cervical spine. Kyphosis is the common spine deformity of the cervical spine and mandates surgical correction as they are at risk of neurological deterioration since they are refractory to conservative management. Few studies of surgical correction of spine deformity included cervical deformity. OBJECTIVES: To analyze the challenges faced during surgery, clinical and radiological outcome, and complications following surgical correction for cervical kyphosis in Marfan syndrome. METHODS: We identified that 5 patients with a diagnosis of MFS with cervical kyphosis who underwent fusion surgery between the years 2010 and 2022 were reviewed, retrospectively. We analyzed the demographic details, radiological parameters, operative variables (blood loss and nuances), perioperative complications, length of stay, clinical and radiological outcome, and complications following fusion surgery for cervical kyphosis in MFS. RESULTS: The mean age of patients was 16.6 ± 4.72 years (range, 12-23 years). The average kyphotic vertebra involved is 3 ± 0.7 bodies (range 2-4) with 2 patients with thoracic deformity. All patients underwent surgical deformity correction. All patients improved clinically with Nurick grade (pre vs. post: 3.4 vs. 2.2) and mJOA (pre vs. post: 8.2 vs. 12.6). There was significant deformity correction from 37.48° to 9.1°. Mean blood loss encountered was 900 ± 173.2 ml. Perioperative complications: wound complication with CSF leak (1). Late complications: ventilator dependence (1) and junctional kyphosis (1). Mean length of hospital stay was 103 ± 178.9 days. All patients were doing symptomatically better after mean follow-up of 58 ± 28.32 months. One patient is bedridden and hospitalized. CONCLUSION: Cervical kyphosis is a rare spine deformity in patients with MFS, and they usually present with neurological deterioration mandating surgical correction. Multidisciplinary approach (pediatrics, genetics and cardiology) is required for systematic evaluation of these patients. They should be evaluated with necessary imaging to rule out associated spinal deformity (atlanto-axial subluxation, scoliosis, and intraspinal pathology like ductal ectasia). Our results suggest better surgical outcome in terms of low operative complications with neurologic improvement in MFS patients. These patients require regular follow-up to identify late complications (instrument failure, non-union, and pseudarthrosis).
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Cifosis , Síndrome de Marfan , Fusión Vertebral , Humanos , Niño , Adolescente , Adulto Joven , Adulto , Síndrome de Marfan/complicaciones , Síndrome de Marfan/cirugía , Estudios Retrospectivos , Resultado del Tratamiento , Cifosis/diagnóstico por imagen , Cifosis/etiología , Cifosis/cirugía , Vértebras Cervicales/diagnóstico por imagen , Vértebras Cervicales/cirugía , Fusión Vertebral/métodosRESUMEN
STUDY DESIGN: Retrospective matched case cohort. OBJECTIVE: Compare postoperative opioid utilization and prescribing behaviors between patients with Marfan syndrome (MFS) and adolescent idiopathic scoliosis (AIS) after posterior spinal fusion (PSF). SUMMARY OF BACKGROUND DATA: Opioids are an essential component of pain management after PSF. However, due to the potential for opioid use disorder and dependence, current analgesic strategies aim to minimize their use, especially in younger patients. Limited information exists on opioid utilization after PSF for syndromic scoliosis. PATIENTS AND METHODS: Twenty adolescents undergoing PSF with MFS were matched with patients with AIS (ratio, 1:2) by age, sex, degree of spinal deformity, and the number of vertebral levels fused. Inpatient and outpatient pharmaceutical data were reviewed for the quantity and duration of opioid and adjunct medications. Prescriptions were converted to morphine milligram equivalents (MMEs) using CDC's standard conversion factor. RESULTS: Compared with patients with AIS, patients with MFS had significantly greater total inpatient MME use (4.9 vs . 2.1 mg/kg, P ≤ 0.001) and longer duration of intravenous patient-controlled anesthesia (3.4 vs . 2.5 d, P = 0.001). Within the first 2 postop days, MFS patients had more patient-controlled anesthesia boluses (91 vs . 52 boluses, P = 0.01) despite similar pain scores and greater use of adjunct medications. After accounting for prior opioid use, MFS was the only significant predictor of requesting an opioid prescription after discharge (odds ratio: 4.1, 95% CI: 1.1-14.9, P = 0.03). Patients with MFS were also more likely to be discharged with a more potent prescription (1.0 vs . 0.72 MME per day/kg, P ≤ 0.001) and to receive a longer-duration prescription (13 vs . 8 d, P = 0.005) with a greater MME/kg (11.6 vs . 5.6 mg/kg, P ≤ 0.001) as outpatients. CONCLUSION: Despite a similar intervention, patients with MFS and AIS seem to differ in their postoperative opioid usage after PSF, presenting an opportunity for further research to assist clinicians in better anticipating the analgesic needs of individual patients, particularly in light of the ongoing opioid epidemic.
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Endrín/análogos & derivados , Síndrome de Marfan , Trastornos Relacionados con Opioides , Escoliosis , Fusión Vertebral , Humanos , Adolescente , Analgésicos Opioides/uso terapéutico , Síndrome de Marfan/complicaciones , Síndrome de Marfan/cirugía , Escoliosis/cirugía , Escoliosis/etiología , Estudios Retrospectivos , Dolor Postoperatorio/tratamiento farmacológico , Dolor Postoperatorio/etiología , Trastornos Relacionados con Opioides/etiología , Fusión Vertebral/efectos adversosAsunto(s)
Insuficiencia de la Válvula Aórtica , Síndrome de Marfan , Humanos , Síndrome de Marfan/complicaciones , Síndrome de Marfan/diagnóstico , Síndrome de Marfan/cirugía , Insuficiencia de la Válvula Aórtica/cirugía , Resultado del Tratamiento , Válvula Aórtica/diagnóstico por imagen , Válvula Aórtica/cirugía , Estudios RetrospectivosRESUMEN
INTRODUCTION: Acute aortic dissection Stanford type A (AADA) is a surgical emergency associated with high morbidity and mortality. Although surgical management has improved, the optimal therapy is a matter of debate. Different surgical strategies have been proposed for patients under 60 years old. This paper evaluates the postoperative outcome and the need for secondary aortic operation after a limited surgical approach (proximal arch replacement) vs. extended arch repair. METHODS: Between January 2000 and January 2018, 530 patients received surgical treatment for AADA at our hospital; 182 were under 60 years old and were enrolled in this study - Group A (n=68), limited arch repair (proximal arch replacement), and group B (n=114), extended arch repair (> proximal arch replacement). RESULTS: More pericardial tamponade (P=0.005) and preoperative mechanical resuscitation (P=0.014) were seen in Group A. More need for renal replacement therapy (P=0.047) was seen in the full arch group. Mechanical ventilation time (P=0.022) and intensive care unit stay (P<0.001) were shorter in the limited repair group. Thirty-day mortality was comparable (P=0.117). New onset of postoperative stroke was comparable (Group A four patients [5.9%] vs. Group B 15 patients [13.2%]; P=0.120). Long-term follow-up did not differ significantly for secondary aortic surgery. CONCLUSION: Even though young patients received only limited arch repair, the outcome was comparable. Full-arch replacement was not beneficial in the long-time follow-up. A limited approach is justified in the cohort of young AADA patients. Exemptions, like known Marfan syndrome and the presence of an intimal tear in the arch, should be considered.
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Disección Aórtica , Implantación de Prótesis Vascular , Síndrome de Marfan , Humanos , Persona de Mediana Edad , Implantación de Prótesis Vascular/efectos adversos , Complicaciones Posoperatorias/etiología , Disección Aórtica/cirugía , Síndrome de Marfan/cirugía , Factores de Tiempo , Estudios Retrospectivos , Resultado del Tratamiento , Aorta Torácica/cirugíaRESUMEN
Importance: Endovascular treatment is not recommended for aortic pathologies in patients with connective tissue diseases (CTDs) other than in redo operations and as bridging procedures in emergencies. However, recent developments in endovascular technology may challenge this dogma. Objective: To assess the midterm outcomes of endovascular aortic repair in patients with CTD. Design, Setting, and Participants: For this descriptive retrospective study, data on demographics, interventions, and short-term and midterm outcomes were collected from 18 aortic centers in Europe, Asia, North America, and New Zealand. Patients with CTD who had undergone endovascular aortic repair from 2005 to 2020 were included. Data were analyzed from December 2021 to November 2022. Exposure: All principal endovascular aortic repairs, including redo surgery and complex repairs of the aortic arch and visceral aorta. Main Outcomes and Measures: Short-term and midterm survival, rates of secondary procedures, and conversion to open repair. Results: In total, 171 patients were included: 142 with Marfan syndrome, 17 with Loeys-Dietz syndrome, and 12 with vascular Ehlers-Danlos syndrome (vEDS). Median (IQR) age was 49.9 years (37.9-59.0), and 107 patients (62.6%) were male. One hundred fifty-two (88.9%) were treated for aortic dissections and 19 (11.1%) for degenerative aneurysms. One hundred thirty-six patients (79.5%) had undergone open aortic surgery before the index endovascular repair. In 74 patients (43.3%), arch and/or visceral branches were included in the repair. Primary technical success was achieved in 168 patients (98.2%), and 30-day mortality was 2.9% (5 patients). Survival at 1 and 5 years was 96.2% and 80.6% for Marfan syndrome, 93.8% and 85.2% for Loeys-Dietz syndrome, and 75.0% and 43.8% for vEDS, respectively. After a median (IQR) follow-up of 4.7 years (1.9-9.2), 91 patients (53.2%) had undergone secondary procedures, of which 14 (8.2%) were open conversions. Conclusions and Relevance: This study found that endovascular aortic interventions, including redo procedures and complex repairs of the aortic arch and visceral aorta, in patients with CTD had a high rate of early technical success, low perioperative mortality, and a midterm survival rate comparable with reports of open aortic surgery in patients with CTD. The rate of secondary procedures was high, but few patients required conversion to open repair. Improvements in devices and techniques, as well as ongoing follow-up, may result in endovascular treatment for patients with CTD being included in guideline recommendations.
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Aneurisma de la Aorta Torácica , Enfermedades del Tejido Conjuntivo , Síndrome de Ehlers-Danlos Tipo IV , Procedimientos Endovasculares , Síndrome de Loeys-Dietz , Síndrome de Marfan , Humanos , Masculino , Persona de Mediana Edad , Femenino , Síndrome de Marfan/complicaciones , Síndrome de Marfan/cirugía , Síndrome de Loeys-Dietz/complicaciones , Estudios Retrospectivos , Resultado del Tratamiento , Procedimientos Endovasculares/métodos , Enfermedades del Tejido Conjuntivo/complicaciones , Enfermedades del Tejido Conjuntivo/cirugía , AortaRESUMEN
OBJECTIVES: To compare operative and long-term outcomes between patients with and without Marfan syndrome undergoing thoracoabdominal aortic aneurysm repair. METHODS: We identified all consecutive patients undergoing thoracoabdominal aortic aneurysm repair between 1997 and 2022. Primary outcome was composite of major adverse events. Secondary outcomes were individual complications and long-term survival. Inverse probability of treatment weighting was performed. Weighted Kaplan-Meier curves were used to estimate long-term survival. Multivariable analysis identified factors associated with major adverse events. RESULTS: Six hundred eighty-four patients underwent open thoracoabdominal aortic aneurysm repair. Ninety (13.1%) had Marfan syndrome, whereas 594 (86.9%) did not. Marfan patients were younger (46 years [range, 36-56 years] vs 69 years [range, 61-76 years]; P < .001). Extent II or III aneurysms (57 out of 90 [63.3%] vs 211 out of 594 [35.6%]; P < .001) and type I or III chronic dissection (77 out of 90 [85.3%] vs 242 out of 594 [40.8%]; P < .001) were more common. Cardiovascular risk factors were less frequent in Marfan patients. There was no difference in major adverse events between groups (12 out of 90 [13.3%] vs 100 out of 594 [16.8%]; P = .49). Operative mortality was similar between groups (3 out of 90 [3.3%] vs 28 out of 594 [4.7%]; P = .75). Unweighted survival at 10 years was 78.7% vs 46.8% (P = .001). Weighted Kaplan-Meier curves showed no difference in long-term survival (adjusted hazard ratio, 0.79; 95% CI, 0.32-1.99; P = .62; Log-rank P = .12). At multivariable analysis, renal insufficiency (odds ratio, 2.29; 95% CI, 1.43-3.68; P < .01) and urgent/emergency procedure (odds ratio, 2.17; 95% CI, 1.35-3.48; P < .01) were associated with major adverse events, whereas Marfan syndrome was not (odds ratio, 1.56; 95% CI, 0.69-3.49; P = .28). CONCLUSIONS: Open thoracoabdominal aortic aneurysm repair can be performed with similar operative outcomes in patients with and without Marfan syndrome despite differing risk profiles. Operative/perioperative strategies must be tailored to specific needs of each patient to optimize outcomes.
Asunto(s)
Aneurisma de la Aorta Torácica , Aneurisma de la Aorta Toracoabdominal , Disección Aórtica , Implantación de Prótesis Vascular , Procedimientos Endovasculares , Síndrome de Marfan , Humanos , Síndrome de Marfan/cirugía , Aneurisma de la Aorta Torácica/diagnóstico por imagen , Aneurisma de la Aorta Torácica/cirugía , Aneurisma de la Aorta Torácica/etiología , Disección Aórtica/diagnóstico por imagen , Disección Aórtica/cirugía , Resultado del Tratamiento , Estudios Retrospectivos , Complicaciones Posoperatorias , Factores de Riesgo , Procedimientos Endovasculares/efectos adversosRESUMEN
OBJECTIVES: To define and compare growth rates of the distal aorta in Marfan patients with and without aortic root replacement using serial MR angiography (MRA). METHODS: We retrospectively included 136 Marfan patients with a total of 645 MRAs who underwent a median of five MRAs (range: 2-13) at 1.5 T and 3 T in annual intervals. Of these, 41 patients (34.8 ± 12 years) had undergone aortic root replacement. The remaining 95 patients (29.0 ± 17 years) still had a native aorta and served as the control group. Thoracic aortic diameters were independently measured at eleven predefined levels. Estimated growth rates were calculated using a mixed effects model adjusted for sex, age, BMI, and medication. RESULTS: Marfan patients with aortic root replacement revealed the highest mean estimated growth rate in the proximal descending aorta (0.77 mm/year, CI: 0.31-1.21). Mean growth rates at all levels of the distal thoracic aorta were significantly higher in patients with aortic root replacement (0.28-0.77 mm/year) when compared to patients without aortic root replacement (0.03-0.07 mm/year) (all p < 0.001). Antihypertensive medication, gender, and BMI had no significant impact on the distal aortic growth rates. CONCLUSION: Distal thoracic aortic diameters increase at a significantly higher rate in Marfan patients with aortic root replacement compared to Marfan patients without aortic root replacement. Further studies are warranted to investigate if the increased growth rate of the distal thoracic aorta after aortic root replacement is caused by altered hemodynamics due to the rigid aortic root graft or due to the general genetic disposition of post-operative Marfan patients. CLINICAL RELEVANCE STATEMENT: High growth rates of the distal aorta after aortic root replacement underline the need for careful life-long aortic imaging of Marfan patients after aortic root replacement. KEY POINTS: ⢠Aortic growth rates in Marfan patients with aortic root replacement are highest in the mid-aortic arch, the proximal- and mid-descending aorta. ⢠Growth rates of the distal thoracic aorta are significantly higher in Marfan patients with aortic root replacement compared to Marfan patients without aortic root replacement. ⢠Antihypertensive medication, gender, and BMI have no significant impact on distal aortic growth rates in Marfan patients.
Asunto(s)
Aneurisma de la Aorta Torácica , Síndrome de Marfan , Humanos , Síndrome de Marfan/complicaciones , Síndrome de Marfan/cirugía , Estudios de Seguimiento , Estudios Retrospectivos , Válvula Aórtica , Antihipertensivos , Dilatación , Aorta/diagnóstico por imagen , Aorta/cirugía , Aorta Torácica/diagnóstico por imagen , Aorta Torácica/cirugía , Angiografía , Dilatación Patológica , Aneurisma de la Aorta Torácica/cirugíaRESUMEN
OBJECTIVES: The aim of this study was to determine the outcome of Stanford type B aortic dissection in patients with Marfan syndrome (MFS) and to evaluate aortic diameters at time of dissection as well as the impact of previous aortic root replacement. METHODS: Analysis of all patients with MFS fulfilling Ghent criteria seen at this institution since 1995 until 2022. RESULTS: Thirty-six (19%) out of 188 patients with MFS suffered from Stanford type B aortic dissection during the study period. The Mean aortic diameter at the time of dissection was 39.0 mm [95% confidence interval (CI): 35.6-42.3]. The mean pre-dissection diameter (available in 25% of patients) was 32.1 mm (95% CI: 28.0-36.3) and the mean expansion was 19% (95% CI: 11.9-26.2). There was no correlation between age and diameter at the time of dissection (<20, 21-30, 31-40, 41-50, 51-60, <61 years; P = 0.78). Freedom from intervention after dissection was 53%, 44% and 33% at 1, 5 and 10 years. Aortic growth rate in those patients that had to undergo intervention within the 1st year after dissection was 10.2 mm/year (95% CI: 4.4-15.9) compared to 5.8 mm/year (95% CI: 3.3-8.3, P = 0.109) in those thereafter. The mean time between dissection and intervention was 1.8 years (95% CI: 0.6-3.0). While type B dissection seems more frequent after previous elective aortic repair (58% vs 42%), there was no difference between valve-sparing root replacement (VSRR) compared to Bentall procedures [Hazard ratio (HR) for VSRR 0.78, 95% CI: 0.31-2.0, P-value = 0.61]. The mean age of the entire population at the end of follow-up was 42 years (95% CI: 39.2-44.7). The mean follow-up time was 9 years (95% CI: 7.8-10.4). CONCLUSIONS: Stanford type B dissection in patients with MFS occurs far below accepted thresholds for intervention. Risk for type B dissection is present throughout lifetime and two-thirds of patients need an intervention after dissection. There is no difference in freedom from type B dissection between a Bentall procedure and a VSRR.