Recurrent cardiac amyloidosis has been reported in the literature. We present two cases, one at 41 months after heart transplant and autologous stem cell transplant, and the other, at 83 months following heart transplantation. The former is the first case ever reported of a patient with amyloid light chain amyloidosis with systemic recurrence following these two treatment modalities, and the latter is a patient with hereditary amyloidosis, whose interval to disease recurrence is the longest ever reported for this type.

Cardiac transplantation is indicated for patients with end-stage cardiomyopathy secondary to cardiac sarcoidosis. Although rare, recurrent disease has been reported in two cases. The current report presents a case of recurrent cardiac sarcoidosis in a patient 45 months postorthotopic heart transplantation and 40 months following reactivation of latent Mycobacterium tuberculosis infection. The patient was the first to have recurrent disease following an infection that has been proposed to be involved in its pathogenesis. The patient's interval between transplant and recurrence is the longest reported to date.

Aims Heart valve repair today includes several preclinical and clinical trans-catheter techniques, including the Evalve MitraClip, for mitral valve (MV) regurgitation by approximating the leaflets, creating a double orifice valve. This is undergoing clinical evaluation in the United States and is commercially available in Europe. We report the pathological findings of explanted MitraClip devices following chronic implantation in the porcine model. Methods Twenty-one explanted porcine valves with MitraClip devices (implanted for 4, 12, 17, 24 and 52 weeks) were examined with light (16) and electron microscopy (5). All were examined, and submitted for routine histology and methyl methacrylate embedding. Haematoxylin and eosin, Gram and connective tissue stains were used. Results Tissue in-growth and endothelialisation, proportional to duration of implantation, were found on the flow (atrial) and non-flow (ventricular) surfaces of the device. This tissue ‘encapsulated’ the device and bridged the gap between the mitral leaflets. Adjacent chordae tendinae were progressively incorporated into the fibrous tissue on the device, as early as 4 weeks, and in 100% of clips by 52 weeks. Conclusions (1) The MitraClip device provides functional tissue apposition;(2) host tissue encases the device, but the valve continues to function well;(3) no device thrombosis was seen. Heart valve repair today includes several preclinical and clinical trans-catheter techniques, including the Evalve MitraClip, for mitral valve (MV) regurgitation by approximating the leaflets, creating a double orifice valve. This is undergoing clinical evaluation in the United States and is commercially available in Europe. We report the pathological findings of explanted MitraClip devices following chronic implantation in the porcine model. Twenty-one explanted porcine valves with MitraClip devices (implanted for 4, 12, 17, 24 and 52 weeks) were examined with light (16) and electron microscopy (5). All were examined, and submitted for routine histology and methyl methacrylate embedding. Haematoxylin and eosin, Gram and connective tissue stains were used. Tissue in-growth and endothelialisation, proportional to duration of implantation, were found on the flow (atrial) and non-flow (ventricular) surfaces of the device. This tissue ‘encapsulated’ the device and bridged the gap between the mitral leaflets. Adjacent chordae tendinae were progressively incorporated into the fibrous tissue on the device, as early as 4 weeks, and in 100% of clips by 52 weeks. (1) The MitraClip device provides functional tissue apposition;(2) host tissue encases the device, but the valve continues to function well;(3) no device thrombosis was seen.

A 59-year-old male with severe biventricular heart failure presented with worsening dyspnea and angina. Following left ventricular assist device insertion, an Amplatzer Septal Occluder (ASO) was required as the patient's patent foramen ovale reopened. Seven months later, the patient underwent heart transplantation. The excised ASO was covered with a thick layer of host tissue on both right and left atrial sides, and organized thrombus was found between the metal wires. The foreign materials, the Nitinol wire and the synthetic (Dacron) fabric, showed a reactive infiltrate of macrophages, multinucleate giant cells, and scattered mononuclear cells.

BACKGROUND:Heart transplantation remains the last treatment option for patients with end-stage cardiac disease. Such diseases include ischemic cardiomyopathy, nonischemic cardiomyopathy and other conditions such as arrhythmogenic right ventricular dysplasia, cardiac sarcoidosis and cardiac amyloidosis.OBJECTIVE:To review the changes that have occurred over time in the etiology of heart disease in patients requiring heart transplantation, and to compare the clinical and histological diagnoses of explanted hearts from patients with progressive cardiac disease.METHODS:The pathological findings of 296 surgically excised hearts over a 20-year period (January 1987 to July 2006) at one institution were examined. Patients were separated into groups based on year of heart transplantation. The tissue was examined to determine the underlying cardiac pathology leading to congestive heart failure. Patient records were reviewed for preoperative clinical diagnoses and other relevant data, including pretransplant endomyocardial biopsy (EMB) results, information regarding left ventricular assist devices and, finally, evidence of disease recurrence in the grafted heart.RESULTS:A shift in the underlying etiology was found in patients who underwent heart transplantation from 1992 to 1996, and 1997 to 2001. Between 1987 and 1997, the majority of transplant cases consisted of ischemic cardiomyopathies. From 1997 to 2001, the majority of patients had nonischemic cardiomyopathies, and this trend continued to 2006. A majority of patients with ischemic and hypertrophic cardiomyopathy were diagnosed correctly (96.5% and 82%, respectively) before transplantation. Most patients diagnosed post-transplant with lymphocytic (viral, 15%), hypersensitive/ eosinophilic (25%) and giant cell (100%) myocarditis, arrhythmogenic right ventricle dysplasia (100%), cardiac sarcoidosis (83%) and iron overload toxicity- associated cardiomyopathy (100%) had been misdiagnosed in pretransplantation investigations. Investigations before transplantation did not include an EMB. Of all 296 patients, 51 patients (17%) were misdiagnosed. Excluding the patients with ischemic cardiomyopathy, 46 of 152 patients (30%) were misdiagnosed before transplantation.CONCLUSIONS:Although cardiac transplantation is a viable treatment option for patients with a variety of cardiac diseases, accurate diagnosis of patients before transplantation remains a priority. Accurate diagnosis of particular diseases (sarcoidosis, myocarditis, iron toxicity-associated cardiomyopathy and others) allows for proper treatment before transplantation, which may slow down disease progression and improve patient outcomes. Furthermore, it is important to accurately diagnose patients with diseases such as sarcoidosis, amyloidosis and particular types of myocarditis because these can readily recur in the grafted heart. The risk for recurrence must be known to practitioners and, most importantly, to the patient. We strongly recommend the use of EMB if a nonischemic cardiomyopathy is suspected, because the results may alter the diagnosis and modify the treatment strategy.

A 31-year-old woman with partial atrioventricular septal defect underwent left atrioventricular valve (LAVV) replacement. Her initial repair was at 8 years of age. At 23 years of age, she underwent reoperation due to a combination of severe left ventricular outlet obstruction and moderate LAVV regurgitation. At that reoperation, she had a Dacron patch enlargement of the infundibular septum and repair of her LAVV with a xenograft (bovine) pericardial patch sutured into the superior bridging leaflet. LAVV replacement was required 8 years later because of valve insufficiency. There was a perforation in the patch with fibrosis, thickening due to pannus, and calcification of the pericardial tissue and the leaflet tissue, leading to stiffening of the tissue.

Systemic vasculitis is often not considered as a possible diagnosis by clinicians because of its low prevalence compared with other more common diseases. Vasculitis can affect any end organ, and it is therefore often missed early on in disease progression. Gastrointestinal (GI) manifestations of vasculitis are considered rare and the presentation is often nonspecific. However, if there is significant involvement of the major vessels of the gastrointestinal system, life-threatening sequelae, including perforation and bowel ischemia, may occur. This makes early and immediate management crucial to improve long-term morbidity and mortality. Diagnosis of various GI vasculitides often relies on correlation of clinical manifestations with pathology and additional investigations. This paper reviews the various vasculitides that affect the GI tract, including systemic lupus erythematosus, mixed connective tissue disease, Henoch Schönlein purpura, polyarteritis nodosa, Churg-Strauss syndrome, Wegener's granulomatosis, microscopic polyangiitis, enterocolic lymphocytic phlebitis, and Behcet's disease. Segmental arterial mediolysis, mistakenly believed to be a vasculitis, is also discussed.

The authors describe five children with nasolacrimal duct obstruction who developed anisometropic amblyopia in the same eye. Because three children had no indication of vision loss at their initial presentation, cycloplegic refraction and periodic visual acuity screenings for such patients is recommended.

Pericardial tumors are rare. A majority of the reported cases are metastatic in nature and indicate a poor prognosis. Primary tumors of the pericardium are extremely rare and occur in a broad age range. This review describes several of the more common lesions: germ cell tumors, solitary fibroma, pericardial mesothelioma, and metastatic disease.

INTRODUCTION:Percutaneous mitral repair with the MitraClip device ("clip") is currently being evaluated in a Phase II clinical trial (EVEREST II). This device was evaluated in an animal model prior to use in humans. MATERIALS AND METHODS:Twenty-one excised clips with accompanying leaflet tissue from pigs were examined at 4, 12, 17, 24, and 52 weeks. Sixteen specimens were available for hematoxylin and eosin and Movat pentachrome staining, and five were sent for scanning electron microscopy. The devices were examined grossly for tissue growth on flow and nonflow surfaces, thrombus, and vegetations. Microscopic evaluation focused on the presence of tissue growth around the device, the inflammatory response, and the presence of thrombus, infective endocarditis, and hematoma. RESULTS:Tissue growth on both flow and nonflow surfaces was seen in all specimens with variation of tissue thickness proportional to the duration of device implantation. Evidence of endothelialization, fibrous encapsulation, and organization of tissue between the aortic and mitral leaflets was observed. Adjacent chordae tendinae were incorporated into the healing tissue growth around the device as early as 4 weeks, in 33% of clips implanted for that time period, increasing to 67% of clips at 12 weeks, and 100% of clips at 17, 24, and 52 weeks. Two animals were diagnosed with infective endocarditis during life. CONCLUSIONS:Mechanical coaptation of the mitral leaflets in an animal model demonstrates adequate tissue response and healing with complete encapsulation of the device by 12 weeks and ongoing healing response proportional to duration of implantation. Infective endocarditis remains a potential complication in the animal model and for all implanted prosthetic devices.

PURPOSE:Many have observed what appears to be declining interest on the part of ophthalmology graduates in pediatric ophthalmology and strabismus (PO&S) as a career. Four questions might address this concern: (1) Has there been a decline in the number of fellowship positions filled in the period 2000 to 2005? (2) Why do graduates choose other career paths? (3) Assuming there has been a decrease in interest in PO&S, does it reflect dissatisfaction on the part of pediatric ophthalmologists in their field? (4) What can be done to enhance the appeal of the subspecialty?METHODS:Data from the San Francisco Matching Programs covering the years 2000 through 2005 included the numbers of ophthalmology graduates, their subspecialty choices, the number of applicants to PO&S and other subspecialty fellowships, and the number of fellowship positions offered. Supplemental surveys assessed positions filled outside the match and international fellows. Factors influencing residents' career choices and the job satisfaction of pediatric ophthalmologists were analyzed in separate surveys.RESULTS:The number of fellowship positions in PO&S increased from 41 to 50 between 2000 and 2004. The number of graduates participating in the match has varied but remained the same (38) in 2005 as in 2000. Graduates were discouraged from PO&S by inadequate mentoring, by aversion to children, and by higher compensation in other fields. Pediatric ophthalmologists generally are highly satisfied in their careers, although financial compensation is a concern for many.CONCLUSIONS:Pediatric ophthalmologists should remain optimistic about recruitment but could enhance the appeal of PO&S by teaching more effectively and by promoting the field.