Exaggerated elastin turnover in young individuals with Marfan syndrome: new insights from the AIMS trial.

Aims: The fragmentation and loss of elastic fibre in the tunica media of the aorta are pathological hallmarks of Marfan syndrome (MFS) but the dynamics of elastin degradation and its relationship to aortic size and physiological growth remain poorly understood.

Methods And Results: In this analysis of the AIMS randomized controlled trial, the association of plasma desmosine (pDES)-a specific biomarker of mature elastin degradation-with age and aortic size was analysed in 113 patients with MFS and compared to 109 healthy controls. There was a strong association between age and pDES in both groups, with higher pDES levels in the lower age groups compared to adults.

Angiotensin receptor blockers and β blockers in Marfan syndrome: an individual patient data meta-analysis of randomised trials.

Background: Angiotensin receptor blockers (ARBs) and β blockers are widely used in the treatment of Marfan syndrome to try to reduce the rate of progressive aortic root enlargement characteristic of this condition, but their separate and joint effects are uncertain. We aimed to determine these effects in a collaborative individual patient data meta-analysis of randomised trials of these treatments.

Methods: In this meta-analysis, we identified relevant trials of patients with Marfan syndrome by systematically searching MEDLINE, Embase, and CENTRAL from database inception to Nov 2, 2021.

Challenges and opportunities in improving left ventricular remodelling and clinical outcome following surgical and trans-catheter aortic valve replacement.

Over the last half century, surgical aortic valve replacement (SAVR) has evolved to offer a durable and efficient valve haemodynamically, with low procedural complications that allows favourable remodelling of left ventricular (LV) structure and function. The latter has become more challenging among elderly patients, particularly following trans-catheter aortic valve implantation (TAVI). Precise understanding of myocardial adaptation to pressure and volume overloading and its responses to valve surgery requires comprehensive assessments from aortic valve energy loss, valvular-vascular impedance to myocardial activation, force-velocity relationship, and myocardial strain.

Mitigating cardiac dysfunction by TOE-guided cardioplegia and mitral valve repair.

Based on current guidelines, 15% to 20% of patients undergoing mitral valve repair for regurgitation develop left ventricular dysfunction (ejection fraction < 50%-55%) despite a normal baseline. Two schools of thought have been debated: preexisting myocardial disease or suboptimal intraoperative myocardial protection. In our view, they could be reconciled.

Surgical Complexity and Outcome of Patients Undergoing Re-do Aortic Valve Surgery.

Objectives: Re-do aortic valve surgery carries a higher mortality and morbidity compared with first time aortic valve replacement (AVR) and often requires concomitant complex procedures. Transcatheter aortic valve replacement (TAVR) is an option for selective patients. The aim of this study is to present our experience with re-do aortic valve procedures and give an insight into the characteristics of these patients and their outcomes.

Correction to: Timing of thoracic radiotherapy is more important than dose intensification in patients with limited-stage small cell lung cancer: a parallel comparison of two prospective studies.

Correction to: Strahlenther Onkol 2019 https://doi.org/10.1007/s00066-019-01539-1 The original version of this article unfortunately contained a mistake.

Irbesartan in Marfan syndrome (AIMS): a double-blind, placebo-controlled randomised trial.

Background: Irbesartan, a long acting selective angiotensin-1 receptor inhibitor, in Marfan syndrome might reduce aortic dilatation, which is associated with dissection and rupture. We aimed to determine the effects of irbesartan on the rate of aortic dilatation in children and adults with Marfan syndrome.

Methods: We did a placebo-controlled, double-blind randomised trial at 22 centres in the UK.

Aortic valve repair techniques: an early UK experience.

Objectives: Retrospective review of early results with aortic valve repair (AVr) techniques.

Methods: 61 consecutive patients underwent AVr surgery at our institutions between 2008 and 2018. 14 patients had a bicuspid aortic valve and 16 had a connective tissue disorder.

Timing of thoracic radiotherapy is more important than dose intensification in patients with limited-stage small cell lung cancer: a parallel comparison of two prospective studies.

Purpose: The optimal radiotherapy dose/fraction for limited-stage small cell lung cancer (SCLC) is undefined. Our objectives were to compare efficacy between hyperfractionated thoracic radiotherapy (TRT; 1.5 Gy 2 times per day [bid] in 30 fractions) and hypofractionated TRT (2.

Comparison Of Long Term Results Between Matched Chemoradiotherapy And Surgery For Limited Stage Small Cell Lung Cancer.

Purpose: To compare the efficacy of chemoradiotherapy or surgery for limited-stage small cell lung cancer (SCLC).

Patients And Methods: A retrospective analysis was performed on 138 patients with limited-stage SCLC who received surgery (69 patients) or chemoradiotherapy (69 patients) between January 2000 and September 2016 in Zhejiang Cancer Hospital. Patients of the chemoradiotherapy group were selected by using "pair-matched case-control" methodology from a cohort of 503 patients who received chemoradiotherapy.

Final report of a prospective randomized study on thoracic radiotherapy target volume for limited-stage small cell lung cancer with radiation dosimetric analyses.

Background: The thoracic radiotherapy (TRT) target volume for limited-stage small-cell lung cancer (SCLC) has been controversial for decades. In this report, the final results of a prospective randomized trial on the TRT target volume before and after induction chemotherapy are presented.

Methods: After 2 cycles of etoposide and cisplatin, patients arm were randomized to receive TRT to the postchemotherapy or prechemotherapy tumor volume in a study arm and a control arm.

Whole-tumor perfusion CT using texture analysis in unresectable stage IIIA/B non-small cell lung cancer treated with recombinant human endostatin.

Background: To observe the dynamic changes of blood perfusion with whole-tumor computed tomography (CT) perfusion imaging using texture analysis in patients with unresectable stage IIIA/B non-small cell lung cancer (NSCLC) treated with recombinant human endostatin (Endostar).

Methods: This phase II clinical trial recruited 11 patients diagnosed with stage IIIA/B NSCLC. Histological examination prior to treatment revealed squamous cell carcinoma in 4 cases and adenocarcinoma in 7 cases.

Coronary Injury in Mitral and Aortic Valve Surgery: A Case Series Using Optical Coherence Tomography.

Coronary occlusion is a rare but well-described complication of heart valve surgery. The left circumflex coronary artery, especially when it is dominant, is particularly at risk owing to the proximity of the mitral valve annulus. We report three cases of acute coronary complications of different valvular surgical procedures diagnosed and treated in the cardiac catheterization laboratory.

The impact of prophylactic cranial irradiation for post-operative patients with limited stage small cell lung cancer.

To evaluate the impact of prophylactic cranial irradiation (PCI) on the prognosis of patients who received definitive surgery for surgically resected small cell lung cancer (SCLC).A retrospective analysis was performed on post-operative SCLC patients treated in Zhejiang Cancer Hospital from January 2003 to December 2015. According to the treatment modality, patients were allocated to PCI group and non-PCI group.

An Option for Concomitant Management of Moderate Marfan Root Aneurysm at the Time of Mitral Valve Repair: A Role for Personalized External Aortic Root Support.

Two patients had mitral valve repair for severe regurgitation in the presence of a Marfan aortic root aneurysm. Concomitant personalized external aortic root support was used at the same operation to halt aneurysm progression and to correct mild aortic regurgitation.

Incremental value of contrast echocardiography in the diagnosis of left ventricular noncompaction.

Contrast echocardiography with left ventricular opacification (LVO) improves the definition of endocardium in two-dimensional echocardiography (2DE). This study was aimed to determine whether LVO offered added diagnostic value in noncompaction of left ventricular myocardium (NCVM). A total of 85 patients (40 ± 20 years, 54 males) with suspected NCVM were subjected to transthoracic 2DE and LVO, and 40 healthy volunteers were examined with 2DE and assigned as control subjects.

Post-treatment serum lactic dehydrogenase as a predictive indicator for distant metastasis and survival of patients with nasopharyngeal carcinoma.

Purpose: To examine the function of serum lactic dehydrogenase (SLDH) level after intensity-modulated radiotherapy (IMRT) as a predictive factor for and loco-regional relapse free survival (LRFS), distant metastasis-free survival (DMFS), disease free survival (DFS), and overall survival(OS) among patients with in-situ nasopharyngeal carcinoma (NPC).

Results: Compared with the normal pt-SLDH group, elevated pt-SLDH demonstrated significant lower DMFS (46 versus 66 months, hazard ratio (HR) 4.07, 95% CI 2.

Design and rationale of a prospective, collaborative meta-analysis of all randomized controlled trials of angiotensin receptor antagonists in Marfan syndrome, based on individual patient data: A report from the Marfan Treatment Trialists' Collaboration.

Rationale: A number of randomized trials are underway, which will address the effects of angiotensin receptor blockers (ARBs) on aortic root enlargement and a range of other end points in patients with Marfan syndrome. If individual participant data from these trials were to be combined, a meta-analysis of the resulting data, totaling approximately 2,300 patients, would allow estimation across a number of trials of the treatment effects both of ARB therapy and of β-blockade. Such an analysis would also allow estimation of treatment effects in particular subgroups of patients on a range of end points of interest and would allow a more powerful estimate of the effects of these treatments on a composite end point of several clinical outcomes than would be available from any individual trial.

The evolution of surgical and medical treatment of aortic root aneurysm.

Since first report of aortic root replacement in 1968, the surgical risk and long term outcome of patients with aortic root aneurysm have been continuously improving. In the last 30 years, the surgical approach is also evolving towards more valve conservation with prophylactical intervention at an earlier clinical stage. Translational research has also led to emerging surgical innovation and new drug therapy.

PAK1 is a novel cardiac protective signaling molecule.

We review here the novel cardiac protective effects of the multifunctional enzyme, p21-activated kinase 1 (PAK1), a member of a serine/threonine protein kinase family. Despite the large body of evidence from studies in noncardiac tissue indicating that PAK1 activity is key in the regulation of a number of cellular functions, the role of PAK1 in the heart has only been revealed over the past few years. In this review, we assemble an overview of the recent findings on PAK1 signaling in the heart, particularly its cardiac protective effects.

A prospective, randomized, placebo-controlled, double-blind, multicenter study of the effects of irbesartan on aortic dilatation in Marfan syndrome (AIMS trial): study protocol.

Background: Cardiovascular complications are the leading cause of mortality and morbidity in Marfan syndrome (MFS), a dominantly inherited disorder caused by mutations in the gene that encodes fibrillin-1. There are approximately 18,000 patients in the UK with MFS. Current treatment includes careful follow-up, beta blockers, and prophylactic surgical intervention; however, there is no known treatment which effectively prevents the rate of aortic dilatation in MFS.

Local suture annuloplasty for posterior mitral valve repair: 18-year experience.

We retrospectively reviewed 128 consecutive patients who underwent quadrangular resection of a prolapsed posterior mitral leaflet and local suture annuloplasty. The median age was 68.1 ± 10.

[Screening of candidate proteins interacting with Foxp3δ2 by yeast two-hybrid system].

Aim: To screen Foxp3Delta2-interacting proteins by yeast two-hybria.

Methods: pGBKT7-Foxp3Delta2 bait plasmid was constructed. Its toxicity and transcriptional activation in yeast cell AH109 was tested by observation of phenotypes and color.

3f prosthesis aortic cusp replacement: implantation technique and early results.

Stentless aortic bioprostheses have been successfully used for over a decade. The 3f bioprosthesis is a new equine pericardial stentless valve, unique in its tubular design, preserving the native aortic sinuses post-implant. Forty-six consecutive aortic valve replacements with the 3f bioprosthesis were performed between June 2003 and January 2005.