Percutaneous oxygenated right ventricular assist device for pulmonary embolism: A case series.

Acute right ventricular (RV) failure following massive pulmonary embolism (PE) can have significant hemodynamic consequences and is the mode of death. Temporary mechanical circulatory support can provide tissue perfusion required while thrombectomy or lysis-aimed therapies act to relieve the thrombotic obstruction. Veno-arterial extracorporeal membrane oxygenation (V-A ECMO) has conventionally been the first line MCS. A more selective approach to RV support has been advocated in the form of an extracorporeal right ventricular assist device (RVAD) as it mitigates some of the shortcomings of V-A ECMO. We present the first case series of four patients who received fully percutaneous RVAD, with an integrated oxygenator forming an Oxy-RVAD, for selective right heart support following massive PE, including the application of single-access dual-lumen right atrium to pulmonary artery cannula. All patients achieved RV recovery and were successfully weaned from oxy-RVAD support within 5-10 days demonstrating the feasibility of selective percutaneous right heart support in managing these challenging patients.

Impact of third-generation left ventricular assist devices on quality of life: Scoping review and meta-analysis.

OBJECTIVES: Development in device technology and the scarcity of donor's hearts have increased the number of patients with advanced heart failure receiving durable left ventricular assist devices (LVADs) as a bridge to transplantation and destination therapy, with improved prognosis compared with guideline-directed medical therapy. We sought to examine the impact of modern durable LVADs on the quality of life (QoL) of the recipients. METHODS: We carried out a systematic review of articles on QoL following the implantation of third-generation LVADs published between January 2010 and February 2021. Included studies were critically analyzed and evidence synthesis was carried out into a meta-analysis. RESULTS: The systematic search yielded 269 articles, 11 of which met the search predefined criteria. Three of them reported results of randomized trials and eight were retrospective and registry studies. Statistically significant QoL improvement from baseline was observed in all published reports. When using the EuroQol 5L questionnaire (scale 0-100) as a QoL tool 6 months post-LVAD implantation, a meta-analysis of four included studies demonstrated a mean difference increase of 28.9 points (95% confidence interval: 26.71-31.14). CONCLUSIONS: Third-generation LVADs confer a significant improvement in QoL and their use can be supported not only for prognosis but also for symptom control. Although methodological limitations should be considered, the available QoL outcomes can be a useful tool in patient selection and the decision-making process.

Complete resection of pulmonary artery hemangioma on the beating heart.

Cardiac hemangiomas are rare entities and very few cases have been reported where they originate from the main arteries of the heart. We present a case of hemangioma arising from the main pulmonary artery, which was completely resected with clear margins on the beating heart.

Patient outcomes from time of listing for lung transplantation in the UK: are there disease-specific differences?

BACKGROUND: The demand for lung transplantation vastly exceeds the availability of donor organs. This translates into long waiting times and high waiting list mortality. We set out to examine factors influencing patient outcomes from the time of listing for lung transplantation in the UK, examining for differences by patient characteristics, lung disease category and transplant centre. METHODS: Data were obtained from the UK Transplant Registry held by NHS Blood and Transplant for adult lung-only registrations between 1January 2004 and 31 March 2014. Pretransplant and post-transplant outcomes were evaluated against lung disease category, blood group and height. RESULTS: Of the 2213 patient registrations, COPD comprised 28.4%, pulmonary fibrosis (PF) 26.2%, cystic fibrosis (CF) 25.4% and other lung pathologies 20.1%. The chance of transplantation after listing differed by the combined effect of disease category and centre (p<0.001). At 3 years postregistration, 78% of patients with COPD were transplanted followed by 61% of patients with CF, 59% of other lung pathology patients and 48% of patients with PF, who also had the highest waiting list mortality (37%). The chance of transplantation also differed by height with taller patients having a greater chance of transplant (HR: 1.03, 95% CI: 1.02 to 1.04, p<0.001). Patients with blood group O had the highest waiting mortality at 3 years postregistration compared with all other blood groups (27% vs 20%, p<0.001). CONCLUSIONS: The way donor lungs were allocated in the UK resulted in discrepancies between the risk profile and probability of lung transplantation. A new donor lung allocation scheme was introduced in 2017 to try to address these shortcomings.

Paravertebral tumours of the cervicothoracic junction extending into the mediastinum: surgical strategies in a no man's land.

PURPOSE: Cervicothoracic paravertebral neoplasms extending into the mediastinum pose a surgical challenge due the complex regional anatomy, their biological nature, rarity and surgeon's unfamiliarity with the region. We aim to define a surgical access framework addressing the aforementioned complexities whilst achieving oncological clearance. METHODS: We carried out a retrospective review of 28 consecutive patients operated in two tertiary referral centres between 1998 and 2015. Pathology was located paravertebrally from C6 to T4 with superior mediastinum invasion. Patients were operated jointly by a spinal and a thoracic surgeon. RESULTS: Tumours were classified according to subclavian fossa involvement as anteromedial, anterolateral and posterior and according to histology in benign nerve sheath tumour group (n = 10) and malignant bone or soft tissue tumours (n = 18). Three surgical routes were utilised: (1) median sternotomy (n = 11), (2) anterior cervical transsternal approach (n = 7) and (3) high posterolateral thoracotomy (n = 10). Resection was en bloc with wide margins in 22 cases, marginally complete in 3 and incomplete in 3. Complications included Horner's syndrome (n = 3), infection (n = 2) and transient neurological deficit (n = 4). In the nerve sheath tumour group, no recurrence or reoperation took place with a median follow-up of 4.5 years. In the malignant bone and soft tissue group, 96% of the patients were alive at 1 year, 67% at 2 years and 33% at 5 years. No vascular injuries or operative related deaths were observed. CONCLUSIONS: Classification of cervicothoracic paravertebral neoplasms with mediastinal extension according to the relationship with the subclavicular fossa and dual speciality involvement allows for a structured surgical approach and provides minimal morbidity/maximum resection and satisfactory oncological outcomes. These slides can be retrieved under Electronic Supplementary Material.

Glycoproteomics Reveals Decorin Peptides With Anti-Myostatin Activity in Human Atrial Fibrillation.

BACKGROUND: Myocardial fibrosis is a feature of many cardiac diseases. We used proteomics to profile glycoproteins in the human cardiac extracellular matrix (ECM). METHODS: Atrial specimens were analyzed by mass spectrometry after extraction of ECM proteins and enrichment for glycoproteins or glycopeptides. RESULTS: ECM-related glycoproteins were identified in left and right atrial appendages from the same patients. Several known glycosylation sites were confirmed. In addition, putative and novel glycosylation sites were detected. On enrichment for glycoproteins, peptides of the small leucine-rich proteoglycan decorin were identified consistently in the flowthrough. Of all ECM proteins identified, decorin was found to be the most fragmented. Within its protein core, 18 different cleavage sites were identified. In contrast, less cleavage was observed for biglycan, the most closely related proteoglycan. Decorin processing differed between human ventricles and atria and was altered in disease. The C-terminus of decorin, important for the interaction with connective tissue growth factor, was detected predominantly in ventricles in comparison with atria. In contrast, atrial appendages from patients in persistent atrial fibrillation had greater levels of full-length decorin but also harbored a cleavage site that was not found in atrial appendages from patients in sinus rhythm. This cleavage site preceded the N-terminal domain of decorin that controls muscle growth by altering the binding capacity for myostatin. Myostatin expression was decreased in atrial appendages of patients with persistent atrial fibrillation and hearts of decorin null mice. A synthetic peptide corresponding to this decorin region dose-dependently inhibited the response to myostatin in cardiomyocytes and in perfused mouse hearts. CONCLUSIONS: This proteomics study is the first to analyze the human cardiac ECM. Novel processed forms of decorin protein core, uncovered in human atrial appendages, can regulate the local bioavailability of antihypertrophic and profibrotic growth factors.

Statin pretreatment and risk of in-hospital atrial fibrillation among patients undergoing cardiac surgery: a collaborative meta-analysis of 11 randomized controlled trials.

AIMS: Statin pretreatment in patients undergoing cardiac surgery is understood to prevent postoperative atrial fibrillation (AF). However, this is based on observational and limited randomized trial evidence, resulting in uncertainty about any genuine anti-arrhythmic benefits of these agents in this setting. We therefore aimed to quantify precisely the association between statin pretreatment and postoperative AF among patients undergoing cardiac surgery. METHODS AND RESULTS: A detailed search of MEDLINE and PubMed databases (1st January 1996 to 31st July 2012) was conducted, followed by a review of the reference lists of published studies and correspondence with trial investigators to obtain individual-participant data for meta-analysis. Evidence was combined across prospective, randomized clinical trials that compared the risk of postoperative AF among individuals randomized to statin pretreatment or placebo/control medication before elective cardiac surgery. Postoperative AF was defined as episodes of AF lasting ≥5 min. Overall, 1105 participants from 11 trials were included; of them, 552 received statin therapy preoperatively. Postoperative AF occurred in 19% of these participants when compared with 36% of those not treated with statins (odds ratio 0.41, 95% confidence interval 0.31-0.54, P < 0.00001, using a random-effects model). Atrial fibrillation prevention by statin pretreatment was consistent across different subgroups. CONCLUSION: Short-term statin pretreatment may reduce the risk of postoperative AF among patients undergoing cardiac surgery.

Valve Type and Operative Risks in Surgical Explantation of Transcatheter Aortic Valves: A Systematic Review and Meta-Analysis.

Indication to perform surgical explantation of TAVR is becoming increasingly more frequent, due to the higher number of transcatheter procedures performed in patients with longer life expectancy. We proposed to perform a systematic review and meta-analysis with metaregression to identify potential factors that can determine an increase in the high mortality and morbidity that characterize these surgical procedures. MEDLINE and Embase were searched for relevant studies. Twelve studies were eligible according to our inclusion criteria. TAVR explantation was confirmed as a procedure with high 30-day mortality (0.17; 95% CI, 0.14-0.21) and morbidity (stroke incidence 5%; 95% CI, 0.04-0.07; kidney injury incidence 16%; 95% CI, 0.11-0.24). The type of transcatheter valve implanted during the index procedure did not influence the outcomes after surgical explantation. The role of these high-risk operations is growing, and it will likely expand in the coming years. Specific tools for risk stratification are required.

The Surgical Treatment of Infective Endocarditis: A Comprehensive Review.

Infective endocarditis (IE) is a severe cardiac complication with high mortality rates, especially when surgical intervention is delayed or absent. This review addresses the expanding role of surgery in managing IE, focusing on the variation in surgical treatment rates, the impact of patient demographics, and the effectiveness of different surgical approaches. Despite varying global data, a notable increase in surgical interventions for IE is evident, with over 50% of patients undergoing surgery in tertiary centres. This review synthesizes information from focused literature searches up to July 2023, covering preoperative to postoperative considerations and surgical strategies for IE. Key preoperative concerns include accurate diagnosis, appropriate antimicrobial treatment, and the timing of surgery, which is particularly crucial for patients with heart failure or at risk of embolism. Surgical approaches vary based on valve involvement, with mitral valve repair showing promising outcomes compared to replacement. Aortic valve surgery, traditionally favouring replacement, now includes repair as a viable option. Emerging techniques such as sutureless valves and aortic homografts are explored, highlighting their potential advantages in specific IE cases. The review also delves into high-risk groups like intravenous drug users and the elderly, emphasizing the need for tailored surgical strategies. With an increasing number of patients presenting with prosthetic valve endocarditis and device-related IE, the review underscores the importance of comprehensive management strategies encompassing surgical and medical interventions. Overall, this review provides a comprehensive overview of current evidence in the surgical management of IE, highlighting the necessity of a multidisciplinary approach and ongoing research to optimize patient outcomes.

Sarcopenia Adversely Affects Outcomes following Cardiac Surgery: A Systematic Review and Meta-Analysis.

BACKGROUND: Sarcopenia is a degenerative condition characterised by the loss of skeletal muscle mass and strength. Its impact on cardiac surgery outcomes remains poorly investigated. This meta-analysis aims to provide a comprehensive synthesis of the available evidence to determine the effect of sarcopenia on cardiac surgery outcomes. METHODS: A systematic review and meta-analysis followed PRISMA guidelines from inception to April 2023 in EMBASE, MEDLINE, Cochrane database, and Google Scholar. Twelve studies involving 2717 patients undergoing cardiac surgery were included. Primary outcomes were early and late mortality; secondary outcomes included surgical time, infection rates, and functional outcomes. Statistical analyses were performed using appropriate methods. RESULTS: Sarcopenic patients (906 patients) had a significantly higher risk of early mortality (OR: 2.40, 95% CI: 1.44 to 3.99, p = 0.0007) and late mortality (OR: 2.65, 95% CI: 1.57 to 4.48, p = 0.0003) compared to non-sarcopenic patients (1811 patients). There were no significant differences in overall surgical time or infection rates. However, sarcopenic patients had longer ICU stays, higher rates of renal dialysis, care home discharge, and longer intubation times. CONCLUSION: Sarcopenia significantly increases the risk of early and late mortality following cardiac surgery, and sarcopenic patients also experience poorer functional outcomes.

Sizing the Frozen Elephant Trunk Based on Aortic Pathology and the Importance of Pre-Operative Imaging.

The frozen elephant trunk is a formidable tool for the aortovascular surgeon. An appreciation of how to size the graft in different pathologies is key in achieving optimal results. Herein, we demonstrate worked examples of how imaging can be used to plan for a frozen elephant trunk and discuss the nuisances and uncertainties of sizing using three index cases: Type A aortic dissection, distal thoracic aortic aneurysm and chronic dissection.

Transcriptomic Analysis of Tight Junction Proteins Demonstrates the Aberrant Expression and Function of Zona Occludens 2 (ZO-2) Protein in Stanford Type A Aortic Dissection.

OBJECTIVE: Thoracic aortic aneurysm dissection (TAAD) represents a cardiac surgery emergency characterized by the disrupted integrity of the aortic wall and is associated with poor prognosis. In this context, the identification of biomarkers implicated in the pathobiology of TAAD is crucial. Our aim in the present original in silico study is to assess the differential gene expression profile of the tight junction proteins (TJPs) in patients with TAAD and to propose novel biomarkers for the diagnosis and prognosis of this disease. METHODS: We implemented bioinformatics methodology in order to construct the gene network of the TJPs family, identify the differentially expressed genes (DEGs) in pathologic aortic tissue excised from patients with TAAD as compared to healthy aortic tissue, and assess the related biological functions and the associated miRNA families. RESULTS: Data regarding the transcriptomic profile of selected genes were retrieved and incorporated from three microarray datasets, including 23 TAAD and 20 healthy control samples. A total of 32 TJPs were assessed. The zona occludens 2 (ZO-2) protein encoded by the gene TJP2 was significantly under-expressed in patients with TAAD compared to the control group (p = 0.009). ZO-2 was associated with fair discrimination and calibration traits in predicting the TAAD presentation. CpG islands of ZO-2 were demonstrated. No important difference was found regarding ZO-2 expression between aneurysmal non-dissected and healthy control aortic tissue. Finally, we performed gene set enrichment analysis (GSEA) and uncovered the major biological functions and miRNA families (hsa-miR-155-5p, hsa-miR-1-3p, hsa-miR-2118-5p, hsa-miR-4691-3p, and hsa-miR-1229-3p) relevant to ZO-2. CONCLUSIONS: These outcomes demonstrated the important role of ZO-2 in the pathobiology of TAAD.

Proximal versus extensive repair in acute type A aortic dissection: an updated systematic review and meta-analysis.

OBJECTIVES: Our aim was to compare the safety and efficacy of proximal repair (PR) versus extensive repair (ER) for acute type A aortic dissection (ATAAD). METHODS: A literature search in three databases was performed according to the PRISMA statement. Studies comparing PR versus ER for ATAAD were included. Random-effects meta-analyses were performed. RESULTS: A total of 27 studies incorporating 7113 patients (PR: 5080; ER: 2033) were included. Patients undergoing PR presented decreased in-hospital mortality (odds ratio [OR]: 0.67 [95% Confidence Interval (95% CI) 0.53-0.85]; p < 0.01) and post-operative bleeding (OR 0.75 [95% CI 0.60-0.95]; p = 0.02) compared to ER. Meta-regression analysis revealed that in-hospital mortality was not influenced by differences regarding the extent of dissection (p = 0.43). Cardiopulmonary bypass time (SMD:-0.93 [95% CI - 1.22, - 0.66]; p < 0.01) and length of hospital stay (SMD:-0.19 [95% CI - 0.34, - 0.05]; p = 0.01) were also lower in the PR group, while there was no difference in terms of renal failure and permanent neurological deficit. The ER approach demonstrated a lower post-discharge mortality compared to PR (OR 1.46 [95% CI 1.09, 1.97]; p = 0.01), while the post-discharge reoperation rate was comparable between the two groups. 1 and 3-year overall survival (OS) were comparable between PR and ER (OR 1.05, [95% CI 0.77-1.44]; p = 0.76) and (OR 1.27 [95% CI 0.86-1.86]; p = 0.23), respectively. The 5-year OS (OR 1.67 [95% CI 1.16-2.41]; p = 0.01) was in favor of the PR arm. CONCLUSIONS: In patients with ATAAD, PR was associated with lower odds of in-hospital mortality but higher odds of late mortality. ER and PR demonstrated similar post-operative complication and reoperation rates.

Upstream therapies for management of atrial fibrillation: review of clinical evidence and implications for European Society of Cardiology guidelines. Part I: primary prevention.

Atrial fibrillation (AF) is associated with significant morbidity and mortality. It is also a progressive disease secondary to continuous structural remodelling of the atria due to AF itself, to changes associated with ageing, and to deterioration of underlying heart disease. Current management aims at preventing the recurrence of AF and its consequences (secondary prevention) and includes risk assessment and prevention of stroke, ventricular rate control, and rhythm control therapies including antiarrhythmic drugs and catheter or surgical ablation. The concept of primary prevention of AF with interventions targeting the development of substrate and modifying risk factors for AF has emerged as a result of recent experiments that suggested novel targets for mechanism-based therapies. Upstream therapy refers to the use of non-antiarrhythmic drugs that modify the atrial substrate- or target-specific mechanisms of AF to prevent the occurrence or recurrence of the arrhythmia. Such agents include angiotensin-converting enzyme inhibitors (ACEIs), angiotensin receptor blockers (ARBs), statins, n-3 (ω-3) polyunsaturated fatty acids, and possibly corticosteroids. Animal experiments have compellingly demonstrated the protective effect of these agents against electrical and structural atrial remodelling in association with AF. The key targets of upstream therapy are structural changes in the atria, such as fibrosis, hypertrophy, inflammation, and oxidative stress, but direct and indirect effects on atrial ion channels, gap junctions, and calcium handling are also applied. Although there have been no formal randomized controlled studies (RCTs) in the primary prevention setting, retrospective analyses and reports from the studies in which AF was a pre-specified secondary endpoint have shown a sustained reduction in new-onset AF with ACEIs and ARBs in patients with significant underlying heart disease (e.g. left ventricular dysfunction and hypertrophy), and in the incidence of AF after cardiac surgery in patients treated with statins. In the secondary prevention setting, the results with upstream therapies are significantly less encouraging. Although the results of hypothesis-generating small clinical studies or retrospective analyses in selected patient categories have been positive, larger prospective RCTs have yielded controversial, mostly negative, results. Notably, the controversy exists on whether upstream therapy may impact mortality and major non-fatal cardiovascular events in patients with AF. This has been addressed in retrospective analyses and large prospective RCTs, but the results remain inconclusive pending further reports. This review provides a contemporary evidence-based insight into the role of upstream therapies in primary (Part I) and secondary (Part II) prevention of AF.

Upstream therapies for management of atrial fibrillation: review of clinical evidence and implications for European Society of Cardiology guidelines. Part II: secondary prevention.

Fundamental research into molecular mechanisms of atrial fibrillation (AF) and improved understanding of processes involved in the initiation and maintenance of AF have transformed the traditional approach to its management by targeting only the electrical aspects, usually with antiarrhythmic drugs and, recently, by ablation. The antiarrhythmic potential of upstream therapies, such as angiotensin-converting enzyme inhibitors, angiotensin receptor blockers (ARBs), statins, and n-3 (ω-3) polyunsaturated fatty acids, extends beyond the benefit of treating underlying heart disease to modifying the atrial substrate and intervening in specific mechanisms of AF. The key target is structural remodelling of the atria, particularly inflammation and fibrosis, although there is evidence to suggest the direct involvement at the ion channel level. Positive clinical reports supported by robust experimental data have suggested that upstream therapies can be valuable strategies for primary prevention of AF in selected patients and have resulted in several class IIA recommendations in the new European guidelines on AF. However, these results have not been consistently replicated in the secondary prevention setting, and several recent randomized controlled studies failed to demonstrate any effect of upstream therapies on AF burden or on major cardiovascular outcomes. Part II of the review summarizes the evidence base for the use of upstream therapies for secondary prevention of AF.

Practice considerations of early aspirin administration following coronary artery bypass surgery.

Thrombotic occlusion of saphenous vein grafts (SVG), the conduits most commonly used in coronary artery bypass grafting (CABG) surgery, causes significant morbidity and mortality. There is class 1A evidence that early aspirin administration following CABG reduces thrombotic SVG occlusion, as well as overall morbidity and mortality. The American Heart Association/American College of Cardiology and the European Association of Cardiothoracic Surgeons have issued guidelines recommending that 150 to 325 mg aspirin be administered within 6 hours following CABG. We carried out a clinical audit of our practice to identify any reasons for deviation from these standards of care and to implement any corrective measures. We prospectively collected data on 200 consecutive patients who underwent CABG to assess both the compliance in prescribing and administering aspirin and the effect on blood loss and transfusion requirements. Sixty-nine percent of patients received an aspirin loading dose 6 hours postoperatively. The reasons for nonadministration of aspirin were postoperative bleeding (10%), lack of a prescription despite aspirin being clinically indicated (13%), and a prescription for aspirin but no administration (9%). Reasons included inadequate handover between clinical teams (4%), aspirin loading ≤24 hours preoperatively (2%), and administration after the first 6 hours (3%). Our audit showed that early aspirin administration did not cause further bleeding or increase blood or blood product transfusion. We followed the recommendations in the majority of cases, but there is scope for improvement in this practice and a need to address "gray areas" not covered by the guidelines.

Is the quality-of-life improvement after transcatheter aortic valve implantation equivalent to that achieved by surgical aortic valve replacement?

A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was 'is the quality-of-life (QoL) improvement after transcatheter aortic valve implantation (TAVI) equivalent to that achieved by surgical aortic valve replacement (sAVR)?' Literature search revealed 189 papers with reference to QoL after TAVI, of which 7 represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these papers were tabulated. QoL plays a crucial role in the decision-making process for procedures such as TAVI and sAVR. Current evidence included and analysed in this review have shown a clear improvement in QoL after both TAVI and sAVR. TAVI offers a rapid improvement of QoL, evident within the first 30 days. There is no difference in QoL at 2- and 5-year follow-up between TAVI and sAVR. There are currently paucity of data on long-term QoL and the potential impact of structural valve degeneration following TAVI.

Aberrant left coronary artery, modified unroofing technique, and patch angioplasty.

BACKGROUND: We present a case of surgical correction of an aberrant left coronary artery arising from the right sinus of Valsalva. METHOD: In order to prevent stenosis and kinking of the neo-ostium, modified unroofing technique was combined with patch angioplasty. RESULTS: The clinical outcome was excellent with complete preservation of ventricular function, resolution of angina, and absence of aortic regurgitation. CONCLUSION: Addition of patch angioplasty to the unroofing technique improves outcome.

Current concepts in the pathogenesis of atrial fibrillation.

Current evidence suggests that the pathogenesis of atrial fibrillation (AF) is multifactorial. The observation that AF, once present, alters the electrophysiologic properties of the atrial myocardium causing self-perpetuation of the arrhythmia raised the importance of electrical remodeling in its pathogenesis. Although these changes are potentially reversible, maintenance of AF continues even after electrical remodeling has occurred. Clinical and experimental studies have highlighted the role of a susceptible atrial anatomical substrate with features of myocyte degeneration and interstitial fibrosis in the initiation and maintenance of AF. Finally, the association of increased inflammatory burden with the presence and future development of AF has implicated inflammation in the pathogenesis of the arrhythmia. The purpose of this review is to provide current evidence on the dominant theories on AF pathogenesis, namely, electrical remodeling, structural remodeling, and inflammation; describe the various experimental models and methods used; and identify a cause-effect association, when present. In addition, the interrelation between different mechanisms responsible for AF will be demonstrated, providing further insight into the complex pathophysiology.

Assessment of carotid compliance using real time vascular ultrasound image analysis in Marfan syndrome.

BACKGROUND: Fibrillin-1 deficiency, dysregulated cytokine transforming growth factor-beta, and increased collagen deposition related to fibrillin-1 gene mutations could predispose to impaired carotid compliance (CC) in Marfan syndrome (MFS). We sought to detect any alterations in CC using the vascular image analysis system (VIA). METHODS AND RESULTS: Thirty-two MFS patients, 20 men and 12 women (mean age 34.2 +/- 12.05 years), and 29 controls matched for age, sex, and body surface area (BSA) were recruited. The entire length of each carotid system was initially scanned longitudinally using a 14 MHz linear transducer. Then, a stereotactic clamp held the transducer in contact with the carotid artery. Arterial diameter changes during the cardiac cycle were recorded for 1 minute from both right (RCCA) and left common carotid arteries (LCCA) separately using the VIA system. RCCA and LCCA compliance and distensibility measurements were significantly reduced in MFS patients when compared to controls, P < 0.05. RCCA and LCCA intima-media thickness did not differ between patients and controls, P > 0.05. MFS diagnosis and age were associated with reduced CC in both carotid arteries after adjusting for variables such as, sex, BSA, heart rate, beta-blockade, intima-media thickness, and aortic root size. CONCLUSIONS: Our findings showed a reduction in CC in adult patients with MFS. This could be attributed to fibrillin-1 deficiency resulting in structural abnormalities in the carotid arterial wall.