Familial aortic dissection in absence of ascending aortic aneurysms: a lethal syndrome associated with precocious systemic hypertension.

The interaction of elevated blood pressure and aortic metabolism in the genesis of aortic dissection is uncharacterized. A kindred with fatal familial aortic dissection in association with precocious systemic hypertension and in absence of a definable connective tissue syndrome has undergone genealogical, clinical, pathological, and biochemical evaluation. Six family members spanning three generations have died of acute dissection. Five men died at a mean age of 28 years (range 22-34), while the proband's paternal grandmother died at 62 years of age. All were hypertensive. A constellation of subtle clinical features points toward deficient integrity of connective tissues; however, major hallmarks of known connective tissue syndromes including aortic root ectasia or aneurysms are absent. Studies of cultured dermal and aortae fibroblasts of two of the proband's brothers mitigate against Ehlers-Danlos IV syndrome. This family's susceptibility to aortic dissection reflects the synergistic liability of coexistent elevated blood pressure and metabolic abnormalities in the genesis of aortic degeneration.

Immunohistochemical characterization of lymphocytes in uninflamed ventricular myocardium. Implications for myocarditis.

Evaluation of endomyocardial biopsy specimens for lymphocytic myocarditis requires accurate identification of lymphocytes, a task at times difficult considering that other myocardial interstitial cells mimic lymphocyte morphology. To wit, the number of mononuclear cells present in normal (uninflamed) myocardium has remained in doubt. We studied the myocardium from hearts that were obtained at autopsy and transvenous endomyocardial biopsy specimens with monoclonal antibodies and immunohistochemical stains to determine the normal numbers and distribution of lymphocytes in uninflamed hearts. In the ventricular myocardium of hearts obtained at autopsy, total immunohistochemically marked lymphocytes averaged 3.6/sq mm, with most being T-cell marker-positive. The ratio of T-helper to T-suppressor-cytotoxic (OKT-4:OKT-8) cells was 1.44. The number of myocardial lymphocytes demonstrated by immunohistochemical staining correlated well with, but was consistently less than, the number obtained by quantitative light microscopic studies on unmarked samples. Thus, the immunohistochemical technique allows for objective enumeration of cells and provides avenues for quantitation of lymphocyte subpopulations in inflamed hearts.

Quantitative evaluation of inflammation in biopsy specimens from idiopathically failing or irritable hearts: experience in 80 pediatric and adult patients.

The histologic criteria for the diagnosis of myocarditis on an endomyocardial biopsy specimen are troubled by varying institutional criteria and interobserver differences. A comprehensive approach to tissue evaluation including quantitative assessment of mononuclear cell populations enhances the accuracy and the specificity of the morphologic diagnosis. Future efforts to characterize the infiltrating cell lines in inflammatory conditions of the heart will aid in the ultimate refinement of therapeutic efficacy.

Fatal vasculitis (periarteritis nodosa) of the coronary arteries: angiographic ambiguities and absence of aneurysms at autopsy.

A 30 year old man died during coronary artery bypass grafting for presumed premature atherosclerotic coronary disease. Autopsy revealed vasculitis with characteristics of periarteritis nodosa of the coronary arteries with severe luminal narrowing, but without aneurysms. This is the first report of a patient with these findings studied angiographically and at autopsy.