Right-sided infective endocarditis combined with mitral involvement in a patient with ventricular septal defect

An autopsy case of right-sided infective endocarditis combined with mitral valvular involvement in a 20-year-old male Japanese with ventricular septal defect (VSD) was reported. The vegetations were found on the endocardium bordering VSD, tricuspid valve, mural endocardium of the right ventricular outflow tract, and even the pulmonic valve, resulting in forming infective aneurysm of the pulmonary trunk. Streptococcus was morphologically identified in the vegetations obtained at autopsy. On the other hand, smaller vegetations were also noted on the mitral valve. The mechanisms of the mitral extending were discussed when right-sided infective endocarditis associated with VSD preceded that on the mitral valve. The authors think that mitral regurgitation in relation to VSD and right to left shunt through VSD which occur even temporarily may be the most important mechanism responsible for the mitral valvular involvement. Several differences between right-sided and left-sided infective endocarditis were also reviewed.     

Indwelling cardiac catheters. An autopsy study of associated endocardial lesions

An autopsy study was carried out to assess the relationship between indwelling intracardiac catheters and hemorrhagic, thrombotic, and infective lesions of the right heart endocardium and valve. Intracardiac catheters cause such lesions frequently, with a spectrum from uncomplicated valvular hemorrhage through nonbacterial thrombotic endocarditis to infective endocarditis. Pulmonary emboli were associated with the thrombotic, infective lesions. Endocardial lesions are more commonly seen with Swan-Ganz (SG) catheters; with these, lesions are more common and severe, with longer periods of catheterization. Pulmonic-valve lesions were only seen with SG catheters. However, not all hemorrhagic lesions in the right heart endocardium were associated with catheters, because a few inpatients without catheters had small subendocardial valvular hemorrhages; the cause of these hemorrhages is obscure because they were not seen in the hearts of patients who died outside the hospital.     

Double valve disease in systemic lupus erythematosus. Case report and literature review

A young, nonhypertensive female with advanced systemic lupus erythematosus (SLE) presented in congestive cardiac failure due to aortic and mitral regurgitation. The valvular lesions resulted from organization of valvular pocket Libman-Sacks vegetations. Her clinical course mimicked infective endocarditis. She is only the third recorded patient with SLE valvular disease warranting double valve replacement. This patient, who had her valvular disease at presentation (prior to initiation of steroid therapy), illustrates that untreated SLE per se may produce severe organic valvular disease.     

Infective endocarditis of bicuspid pulmonary valve

Infective endocarditis of the cardiac valves mainly involves the aortic mitral valves and less frequently the tricuspid valve. Isolated pulmonary valve involvement is extremely rare with few cases reported in literature. We present a case of a 34-year-old female who presented with sudden onset breathlessness and cardiac failure. A complete autopsy showed an uncommon congenital bicuspid pulmonary valve, which was also affected by infective endocarditis.     

Right-sided infective endocarditis as a consequence of flow-directed pulmonary-artery catheterization. A clinicopathological study of 55 autopsied patients

We studied 142 consecutively autopsied patients prospectively to determine the frequency and clinical importance of right-sided endocardial lesions in patients who had undergone flow-directed pulmonary-artery catheterization within one month of death. Of the 55 catheterized patients, 29 (53 per cent) had one or more right-sided endocardial lesions: 12 (22 per cent) had subendocardial hemorrhage, 11 (20 per cent) sterile thrombus, 2 (4 per cent) hemorrhage and thrombus, and 4 (7 per cent) infective endocarditis. Of 41 lesions seen in the 29 patients, 23 (56 per cent) were located on the pulmonic valve, 6 (15 per cent) on the tricuspid valve, 6 (15 per cent) in the right atrium, 4 (10 per cent) in the right ventricle, and 2 (5 per cent) in the main pulmonary artery. All four patients with infective endocarditis had had positive antemortem blood cultures while the catheter was in place, but in only one had the diagnosis of endocarditis been suspected clinically. The unusual locations of the infected vegetations (on the pulmonic valve in three and in the right atrium in one) and the similar location of the uninfected lesions suggest that the infective endocarditis was a consequence of catheter-induced endocardial damage with concurrent or subsequent bacteremia. Among the 87 non-catheterized patients, there were two subendocardial hemorrhages and one resolving right atrial thrombus. We conclude that endocardial damage from flow-directed pulmonary-artery catheterization is common and that right-sided infective endocarditis should be suspected in bacteremic catheterized patients.     

Paecilomyces javanicus endocarditis of native and prosthetic aortic valve

A 41-year-old diabetic woman developed endocarditis of the aortic valve caused by Paecilomyces javanicus six years after insertion of a porcine mitral valve heterograft. The patient died shortly after aortic valve replacement. Autopsy revealed vegetations of the aortic heterograft, valve ring abscess and ascending aortitis due to Paecilomyces. There was no involvement of the mitral valve heterograft. Lesions due to mycotic emboli were found in the kidneys, spleen, and brain. Cultures of the surgically removed aortic valve and of the kidney at autopsy produced rapid growth of P. javanicus. The gross and microscopic pathologic and cultural characteristics of this organism are described with a review of the literature. Previously reported cases of Paecilomyces endocarditis occurred only in prosthetic heart valves. This is the first known report of P. javanicus endocarditis of a native valve and its prosthetic heart valve heterograft.     

Isolated infective endocarditis of the pulmonary valve: an autopsy analysis of nine cases

IntroductionInfective endocarditis (IE) of the pulmonary valve is uncommon and usually occurs in conjunction with tricuspid and/or left-sided valvular endocarditis. There have been only sporadic reports of isolated pulmonary valvular infective endocarditis (PVIE). This report documents the pathological features of nine such cases at autopsy.MethodsAmong 155 cases of IE encountered in a 14-year period, we selected nine cases that had isolated PVIE for analysis. The clinical records were reviewed for the patient demographics, presence or absence of underlying cardiac disease or other predisposing factors, and modes of presentation; these were correlated with the relevant investigations. A detailed study of the heart was done in all with special attention to the pulmonary valve morphology.ResultsThe nine cases of isolated PVIE formed 5.8% of the IE cases, seen in six males and three females, largely adults. Seven patients (77.8%) had admissions for about 24 h. Hospital admission was sought for mainly progressive shortness of breath (66.7%) and fever (44.4%). Congenital heart disease was seen in seven (77.8%); two (an infant and an adult) had normal hearts. A single blood sample for blood culture in two patients was negative. Two-dimensional echocardiography, performed in eight, revealed vegetations on the pulmonary valve in four. The pulmonary valve was tricuspid in six cases, bicuspid in two, and unicuspid in one. The vegetations (active in three, active and healing in two, healed in four) were accompanied by concomitant thickening, shortening, perforations, or complete destruction of the cusps. Involvement of the right ventricular outflow tract or the main pulmonary artery was identified in five hearts. Five patients (55.6%) developed pulmonary complications, related to the endocarditis.ConclusionsDetection of PVIE, especially the isolated type, may be underdiagnosed. This condition should be kept in mind during evaluation of patients especially with cardiac anomalies, who present with fever, prominent respiratory symptoms, and negative blood cultures.     

Renal infarction with fatal bleeding — an unusual complication ofCandida albicans endocarditis

Summary A patient with mitral value endocarditis due toCandida albicans infection is presented. Because of persistent fever and one episode of transient right-sided hemiparesis, the patient underwent mitral value replacement. Two weeks after successful valve replacement, the patient suddenly developed acute abdominal pain and died before a surgical intervention could be initiated. Autopsy examination revealed a renal infarction in the lower part of the right kidney with a large rupture of the capsule and retroperitoneal bleeding. A myocotic aneurysm could not be detected. The clinical significance of renal infarction in infective endocarditis is discussed.Abbreviations LV left ventricle - LA left atrium - RV right ventricle     

Unusual course of infective endocarditis: acute renal failure progressing to chronic renal failure

Infective endocarditis is an infection of the endocardium that usually involves the valves and adjacent structures. The classical fever of unknown origin presentation represents a minority of infective endocarditis. The presented case was a 21-yearold young lady presenting with acute renal failure and fever to the emergency room. Cardiac auscultation revealed a soft S1 and 4/6 apical holosystolic murmur extended to axilla. Echocardiography showed mobile fresh vegetation under the mitral posterior leaflet. She was diagnosed as having infective endocarditis. Hemodialysis was started with antimicrobial therapy. However, because of the presence of severe mitral regurgitation with left ventricle dilatation and large mobile vegetation, mitral prosthetic mechanical valve replacement was performed. Although treated with antibiotics combined with surgery, renal functions were deteriorated and progressed to chronic renal failure.     

Pathology and pathogenesis of infective endocarditis in native heart valves.

Infective endocarditis is an endovascular microbial infection of cardiovascular structures, including large intrathoracic vessels and intracardiac foreign bodies. The characteristic lesions consist of vegetations composed of platelets, fibrin, microorganisms, and inflammatory cells, as well as leaflet disruption. The commonly accepted pathogenetic theory is herein reported, from endothelial injury with deposition of noninfective sterile thrombotic vegetations to transient bacteremia with microorganism adhesion (injury-thrombus-infection theory). This review addresses the pathology of native valve endocarditis, including local (valvular and perivalvular destruction) and distal (embolism, metastatic infection, and septicemia) complications. Old and new cardiac conditions and patients at risk, predisposing to the occurrence of infective endocarditis, are then discussed. Particular emphasis is given to hidden bicuspid aortic valve and the need of early carrier identification for prophylaxis.     

Lactobacillus Endocarditis: A Chronic Active Infection

Lactobacilli are gram positive rod-shaped or filamentous bacteria that are a rare cause of endocarditis. We review the literature of less than 50 reported cases, and present a case of a 43-year-old male with a protracted, one-year history consistent with endocarditus. The pathologic findings in the heart at autopsy indicate a long, chronic course of smoldering infection with extensive secondary fibroelastosis of valvular structure and adjacent endocardium, although superimposed more acute vegetation with viable organisms consistent with the premortem blood culture of Lactobacillus acidophilus were abuntly present. Thus, the pathologic and clinical features of this case, and our review of the available literature, suggest that infective endocarditis caused by Lactobacillus species has an indolent nature and results in severe, chronic alterations of valvular structure.     

Nonsurgical Management of Mitral Valve Endocarditis Due to Cardiobacterium valvarum in a Patient with a Ventricular Septal Defect

Cardiobacterium valvarum is a relatively novel agent of infective endocarditis. We describe the first case of infective endocarditis due to this pathogen in the Asian Pacific. This case is unique in its involvement of the mitral valve as well as its clinical resolution exclusively resulting from treatment with antibiotics without resorting to valve replacement/explantation.     

Aneurysm of the mitral valve in a patient with hypertrophic cardiomyopathy

We report a case of mitral valve aneurysm and severe mitral regurgitation complicating infective endocarditis in a patient with hypertrophic cardiomyopathy. 2-dimensional echocardiography revealed a saccular structure in the anterior mitral leaflet that bulged into the left atrium throughout the cardiac cycle. Pathology of the excised valve showed inflammation, early repair and perforation of the aneurysm. Concurrent mitral insufficiency or trauma resulting from septal-anterior mitral leaflet contact may predispose to valvular infection. The repair process of this damaged focus and the loss of the elastic properties of the valve may contribute to aneurysm formation. The aneurysm in this case formed in less than 5 weeks.     

Multi-valvular endocarditis

Objective   Seventy-seven cases of native valve infective endocarditis as determined by the Duke criteria, were reviewed to determine the incidence and clinical features of multi-valvular endocarditis.
Methods   Fourteen of 77 patients (18%) had multi-valvular endocarditis most commonly involving the mitral and aortic valves. Staphylococcus aureus (43%) and viridans streptococci (36%) were the most common organisms causing multi-valvular endocarditis.
Results   Definite or probable vegetations were found in 50% of the patients by two-dimensional transthoracic echocardiograph and/or transesophageal echocardiograph, and possible vegetations were detected in 21%. The overall mortality in our series was 21%; 29% underwent valve replacement and 50% were treated medically. The major complications of multi-valvular endocarditis were congestive heart failure (64%), acute renal failure (50%), embolic events (21%), and splenic abscess/infarcts (21%).
Conclusions   Our data suggests complications of multi-valvular endocarditis, compared with uni-valvular endocarditis are similar except for heart failure. Heart failure is statistically more common in multi-valvular endocarditis ( P 0.002).     

Truffle's revenge: a pig-eating fungus.

A 77-year-old female initially presented with symptomatic mitral valve stenosis involving a bioprosthesis that had been implanted 8 months earlier for myxomatous mitral valve disease and severe valvular regurgitation. The patient was taken for a second mitral valve replacement due to stenosis. Intraoperatively, the bioprosthetic mitral valve was noted to have an unusual clot-like mass on the atrial side. Initial fungal smears were positive for yeast stains, and pathology revealed extensive colonization by thick filamentous fungus with apparent true hyphae, pseudohyphae, and yeast forms. The fungus was identified as Hormographiella aspergillata, the asexual form of Coprinus cinereus, a common inky cap mushroom that grows in the lawn. She was treated with 6 weeks of liposomal amphotericin B and then switched to voriconazole for long-term (lifelong) suppressive therapy in the setting of a new mechanical mitral valve. The only other reported case of infective endocarditis caused by a Coprinus species occurred in a 53-year-old man who had developed native aortic valve fungal endocarditis and died [J Med Microbiol (1971);4(3):370-4]. The valve isolate was identified as probable C. cinereus.     

Fatal Vibrio fetus endocarditis: Report of one case and review of the literature

Summary Clinical, bacteriologic, and autopsy findings in a case of a 67-year-old man confirmed the diagnosis of Vibrio fetus endocarditis of the aortic, mitral, and tricuspid valve. To our knowledge, this is the first case to involve three valves. The bacterial endocarditis was superimposed on an old, probably rheumatic, endocarditis of the aortic valve, with minimal involvement of the mitral and tricuspid valves. A review of the literature indicates that the aortic valve is the most frequent site of V. fetus endocarditis.     

Discrete subvalvular aortic stenosis

Discrete Subaortic Stenosis is one of the many lesions responsible for left ventricular outflow tract (LVOT) obstruction. It may present as in an isolated from as membranous or fibromuscular ring below the aortic valve or in association with other congenital anamolies such as VSD, PDA, coarctation of aorta, hypoplastic aortic annulus, double chamber right ventricle among others. The condition is rarely diagnosed antenataly or in infancy but often manifests in the first decade of life with features of progressive LVOT obstruction, LV hypertrophy and dysfunction aortic regurgitation due to damage to the aortic cusps because of the jet from the subaortic narrowing which may also render the aortic valve prone to infective endocarditis. Interaction of genetic predisposition and morphologically deformed long and narrow LVOT cause rheological abnormalities and increased shear stress in the region of subaortic stenosis and seem to be the main etiological factor alongwith poorly defined role of more extensive but subtle changes in the LV endocardium. Condition can be easily diagnosed by cross-sectional and Doppler echocardiography and confirmed by demonstrating a pressure gradient below aortic valve on cardiac catheterisation and LV angiography. Surgical membranectomy alongwith myotomy or myomectomy remain the mainstay of treatment but long term results are not satisfactory as there is a high rate of recurrences requiring reoperations. A close follow up with serial echocardiographic examinations is very helpful in early detection of subaortic obstruction in patients who have so called functional murmurs in the childhood.     

Biopsy of the heart valves. 872 cases

In this study, 872 heart valves surgically excised from 810 patients during a period of 5 years (1994 through 1998) were examined pathologically. There was a predominance of aortic (506 patients) versus mitral valves (246 pts.). While aortic valves came more often from men (364) than from women (142), in mitral valves the M:F ratio is 82/164. Isolated calcific aortic stenosis appeared as the most frequent valvular disease (418 pts.), with predominance of its sclerotic-senile type (238 pts.). Mitral stenosis (185 pts.) remains the classical post-rheumatic disease. The relative frequency of a subvalvular stenosing mitral lesion is stressed. The "pure" incompetence of both aortic (70 pts.) and mitral (56 pts.) valve was usually based on valvular myxoid degeneration. An aorto-mitral disease requiring replacement of both valves (51 pts.) presented typically as a post-rheumatic lesion, however, a combination of a post-rheumatic mitral with a degenerative-sclerotic aortic valve disease may be possible. In 30 patients, the valvular replacement was performed for infective endocarditis or a post-IE lesion, mostly of the aortic valve. With the almost non-existence of acute rheumatic fever and with the increasing average age of population in this country, we may expect a long-term decline in mitral valve disease and an increase in aortic valve disease, particularly in the sclerotic type of aortic stenosis.     

Mycobacterium fortuitum prosthetic valve endocarditis: a case for the pathogenetic role of biofilms

BackgroundProsthetic valve endocarditis presents unique challenges for both diagnosis and treatment. A potential role of biofilm has been hypothesized in the pathogenesis of these infections.MethodsA patient with infective endocarditis involving a stentless (Freestyle) porcine prosthetic aortic valve with annular abscess and paravalvular leak 8 months after implantation is reported.ResultsThe infected valve did not show vegetations or perforations, but histiocytic inflammation was seen along the endocardial surfaces of the valve. Auramine–rhodamine staining revealed many acid-fast organisms associated with the inflammation. There was also an acellular matrix material with ultrastructural features of biofilm. Blood cultures grew Mycobacterium fortuitum, a biofilm-associated microbe.ConclusionsThe role of biofilm in prosthetic valve endocarditis is discussed. The importance of microscopy for prosthetic valves, even when no vegetations are present, is highlighted along with correlation of pathologic findings with culture results.     

Pathology and pathogenesis of rheumatic heart disease

Cardiovascular disease is on the rise. In India and other developing countries, rheumatic heart disease (RHD) continues to be a major public health problem and contributes to significant cardiac morbidity and mortality. RHD in the juvenile age group namely juvenile mitral stenosis is a variant which is unique to the Indian subcontinent. Severe valve deformities lead to high morbidity and mortality. Despite various measures no appreciable decline in prevalence of RHD has been documented. At autopsy, mitral valve was most commonly affected either alone or in combination with aortic and tricuspid valves. Both functional and organic involvement of tricuspid valve was documented. It has been convincingly demonstrated that molecular mimicry between Streptococcus pyogenes antigen and human proteins lead to autoimmune reactions both humoral and cell mediated causing RF/RHD. Heart tissues namely the valves, left atrial appendage (LAA) and myocardium reveal variable amounts of infiltration by lymphocytes. Significant endocarditis and valvulitis is observed in these cases. CD4+ T cells are most likely the ultimate effectors of chronic valve lesions in RHD. They can recognize Streptococcal M5 protein peptides and produce various inflammatory cytokines such as TNF-alpha, IFN-gamma, IL-10, IL-4 which could be responsible for progressive fibrotic valvular lesions. Cardiac myosin has been defined as a putative autoantigen recognized by autoantibodies of RF patients. Cross reactivity between cardiac myosin and group A beta hemolytic Streptococcal M protein has been adequately demonstrated. Cardiac myosin has been shown to produce myocarditis in rats and mice. Valvulitis/ endocarditis has been observed in excised LAA, cardiac valves and in hearts at autopsy from cases of RHD. The disease predominantly affects the valvular endocardium culminating in crippling valve deformities. Endocardial infiltrate and their migration into the valve substance has been elegantly demonstrated in rats and mice. Immune responses against cardiac myosin lead to valvular heart disease and infiltration of the heart by Streptococcal M protein reactive T lymphocytes. Mitral valves showed various degrees of calcification. An interesting observation is the nature of calcification in diseased/distorted valves in RHD. Recent studies indicate that calcification is not merely an inactive, "dystrophic" process but involves a regulated inflammatory process associated with expression of osteoblast markers and neoangiogenesis. Increased plasma osteopontin levels correlated with severity of mitral valve calcification. Further evidence of inflammation is supported by high levels of advanced oxidation protein products and high sensitive C-reactive protein in plasma detected in patients with RHD. Presence of inflammatory cells and increased expression of several cytokines in cases of "end stage" RHD reflects a possible subclinical, ongoing insult/injury to some unrecognized antigenic stimulus by beta hemolytic Streptococcal antigens that have sensitized/primed the various target tissues and which further culminate in permanent valve deformities.