Israel Medical Association Journal

Background: Magnetic resonance imaging (MRI), which has recently become the leading imaging modality in the study of ankylosing spondylitis (AS), has not been evaluated in the assessment of disease-specific changes at the craniocervical junction (CCJ) in patients with AS.

Objectives: To describe the spectrum of active inflammatory lesions at the CCJ using MRI in a cohort of patients with AS and neck pain.

Methods: The study included 18 patients with AS presenting with neck pain and a control group of 9 fibromyalgia patients matched for age and levels of neck pain. All patients underwent a focused rheumatologic examination, X-ray of the cervical spine, and a 3T MRI study, which included STIR, CUBE T2, FSE and FSE FAT SAT sequences before and after administration of gadolinium.

Results: The median age of AS patients was 43 years with a median disease duration of 7 years. Fifteen of 18 patients were under biologic treatment. Seven of 18 AS patients had evidence of cervical syndesmophytes on X-ray films. Active inflammatory lesions of atlanto-occipital joints and apical and alar ligaments were detected in MRIs in 2 out of the 18 patients with AS and in none of the patients with fibromyalgia. Both AS patients with active inflammation of CCJ detected on MRI received treatment with biological agents prior to and during the study.

Conclusions: Active inflammation of both entheses and joints of the CCJ can be demonstrated by MRI in patients with AS.

Background: The rate of mitral bioprosthesis implantation in clinical practice is increasing. Transcatheter valve-in-valve implantation has been described for high risk patients requiring redo valve surgery. 

Objectives: To report our experience with transapical valve-in-valve implantation for failed mitral bioprosthesis.

Methods: Since 2010, 10 patients have undergone transapical valve-in-valve implantation for failed bioprosthesis in our center. Aortic valve-in-valve implantation was performed in one of them and mitral valve-in-valve implantation in nine. Mean age was 82 ± 4 years and 6 were female (67%). Mean time from original mitral valve (MV) replacement to valve-in-valve procedure was 10.5 ± 3.7 years. Follow-up was completed by all patients with a mean duration of 13 ± 12 months. 

Results: Preoperatively, all patients presented with significant mitral regurgitation; two with mitral stenosis due to structural valve failure. All nine patients underwent successful transapical valve-in-valve implantation with an Edwards Sapien™ balloon expandable valve. There was no in-hospital mortality. Mean and median hospital duration was 15 ± 18 and 7 days respectively. Valve implantation was successful in all patients and there were no major complications, except for major femoral access bleeding in one patient. At last follow-up, all patients were alive and in NYHA functional class I or II. Echocardiography follow-up demonstrated that mitral regurgitation was absent or trivial in seven patients and mild in two. At follow-up, peak and mean gradients changed from 26 ± 4 and 8 ± 2 at baseline to 16.7 ± 3 and 7.3 ± 1.5, respectively.

Conclusions: Transcatheter transapical mitral valve-in-valve implantation for failed bioprosthesis is feasible in selected high risk patients. Our early experience with this strategy is encouraging. Larger randomized trials with long-term clinical and echocardiographic follow-up are recommended.

 

Background: Long-term follow-up in apical hypertrophic cardiomyopathy is rare.

Objective: To study the natural history of the disease.

Methods: We followed 11 patients, 5 women and 6 men, for 5-20 years.

Results: At presentation all 11 patients had typical features of apical hypertrophic cardiomyopathy, with dyspnea in 3 and chest pains in 8, of whom 5 were typical of angina and 3 had myocardial infarction. R-wave voltage and T-wave negativity progressively decreased in magnitude at serial electrocardiograms in four patients. Perfusion defects were detected on thallium myocardial scintigraphy in three, increased apical uptake in two, and normal in one patient. Apical aneurysm with normal coronary arteries developed in a patient who had sustained ventricular tachycardia. All of the 10 catheterized patients had normal coronaries except for one with significant left anterior descending artery stenosis and another with a minor lesion. Symptomatic sustained ventricular tachycardia was found in two patients, one of whom required the implantation of an internal cardioverter-defibrillator.

Conclusions: Apical hypertrophic cardiomyopathy may develop morphologic and electrocardiographic changes with life-threatening arrhythmias necessitating close follow-up and treatment.