Chronic paravalvular abscess after aortic valve replacement.
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Biomedical subjects
Publications and source records attributed to Jagdish W Butany.
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Endothelial cell dysfunction plays an important role in the development and progression of cardiovascular and other disease. The purpose of this review is to discuss some of the genetic diseases known to adversely affect endothelial function. Although the list is exhaustive, we focus our discussion on primary pulmonary hypertension, diabetes mellitus, Alzheimer's disease, Crohn's disease, Von-Hippel-Lindau disease, familial Mediterranean fever, thrombotic microangiopathy, and key vascular malformations. Endothelial dysfunction results from a complex interplay between genetic and environmental factors. This review highlights some of the growing body of evidence implicating endothelial dysfunction as an important mediator of diverse diseases.
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Primary pulmonary artery neoplasms are rare and lethal. Those involving the pulmonary valve are even rarer with only a few reported cases in the literature. The poor prognosis of these neoplasms, despite attempted multimodal therapy, is largely related to delay and difficulty in diagnosis. The case of a 53-year-old woman is described who within one month of suddenly developing shortness of breath was found to have a mass arising from the pulmonary valve and extending through the pulmonary vasculature, requiring extensive surgical resection. She died two weeks postoperatively. The morphological findings of this primary pulmonary artery sarcoma are presented. Diagnosis, treatment, prognosis and literature are reviewed, and consideration of this tumour in the differential diagnosis of other pulmonary diseases is emphasized.
Quadricuspid semilunar valves are uncommon congenital anomalies sometimes associated with severe valvular dysfunction. Two cases of isolated quadricuspid semilunar valve were identified. The first, a heavily calcified quadricuspid aortic valve with four equal-sized cusps, was identified in a 56-year-old man at the time of valve replacement surgery for severe aortic stenosis and regurgitation. The second valve was a quadricuspid pulmonary valve with three equal-sized cusps and one smaller cusp, identified incidentally at autopsy in a 79-year-old woman with a ruptured abdominal aortic aneurysm. Review of the literature found that while quadricuspid pulmonary valves are infrequently associated with serious clinical complications, the majority of quadricuspid aortic valves are associated with clinically significant dysfunction, most commonly aortic insufficiency. Further, contrary to previous thinking, the frequency of aortic valvular dysfunction does not appear to be related to valve morphology.
Contemporary prosthetic heart valves (PHV) are a good mode of treatment of valvular heart disease. They last for an average of 5 to 10 years and bioprostheses fail due to the tissue degeneration. Infective endocarditis of the prosthetic valve is a relatively rare complication occurring in 0.5% to 1% of cases per year, and its late occurrence is even less common. Patients with PHV who undergo interventional or surgical procedures under adequate antibiotic coverage, infective endocarditis should not occur. We present a case of a 54-year-old woman who developed infective endocarditis on a porcine bioprosthesis, implanted 15 years earlier. The microorganism was Streptococcus viridans and the vegetations were surprisingly large and led to prosthesis obstruction.
Valvular heart disease has numerous etiologies. These range from congenital malformations to infectious and degenerative diseases. Clinically, these result in significant problems, the management of which can necessitate valve replacement with prosthetic heart valves. A rare cause is the deposition of foreign material in the valvular tissues, and these include inborn errors of metabolism of the essential amino acids. Alkaptonuria, an autosomal recessive trait, can result in the accumulation of excess homogentisic acid. This can manifest as pigmentation in the skin and other tissues, including heart valves. Accumulation of this pigment can lead to an inflammatory reaction and to progressive valve dysfunction.
As the population ages, bioprosthetic heart valves are increasingly being used to replace diseased native valves. Bioprosthetic valve durability depends on patient age and other factors, but rarely exceeds 15 years. Explanted bioprosthetic valves commonly show tissue degeneration, tears, and calcification. Host tissue overgrowth (pannus), to the extent of interfering with their function, is another finding in bioprostheses that have been in place for long periods. We present a case in which a bovine pericardial valve was explanted after more than 20 years of implantation. The longevity of this pericardial valve may have been related to excessive pannus growth, which most likely protected the valve from earlier failure.