Heart & vascular pathology

• ICD-9: 996.83 – complications of transplanted heart
• ICD-10: T86.290 – cardiac allograft vasculopathy

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• Coronary angiography: diffuse, longitudinal and concentric luminal narrowing of primary or branch vessels (J Heart Lung Transplant 2010;29:717)
• Intravascular ultrasound: increased maximal intimal thickness of the coronary arteries, predominantly left anterior descending (J Am Coll Cardiol 2005;45:1532)

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• Thickened intramural arteries on the ventricular cut surface (Arch Pathol Lab Med 2007;131:1169)
• Gross luminal narrowing of the epicardial coronary arteries
• Focal areas of myocardial scarring (J Heart Lung Transplant 2011;30:1044)

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Contributed by Carolyn Glass, M.D., Ph.D.

• CD3 (Leu4) and CD68 may be positive particularly in subendothelium (Catheter Cardiovasc Interv 2018;92:E527)

• Given the histological heterogeneity of chronic allograft vasculopathy, this is a diagnosis made clinically rather than microscopically. The ISHLT grading guidelines rely upon coronary angiography (demonstrating luminal narrowing of primary vessels) and assessment of allograft function (i.e. ultrasound or echo). While this entity does have distinct microscopic features, it is usually not included in a pathology report line diagnosis.

A 34 year old Hispanic man underwent an orthotopic heart transplantation six years ago following intractable idiopathic restrictive cardiomyopathy where he received a heart from a 25 year old white woman. Routine posttransplant endomyocardial biopsies showed no histological evidence of rejection but surveillance coronary angiography and intravascular ultrasound recently revealed diffuse, circumferential thickening of the left anterior descending artery with significant intimal thickening and 70% luminal stenosis. The patient has no history of dyslipidemia, diabetes or CMV infection. Which of the following is a significant risk factor in the development of this patient's disease?

1. Absence of histological evidence of rejection
2. Donor female sex
3. Donor white race
4. Donor young age
5. Recipient CMV IgG/IgM seronegativity

C. This patient has chronic allograft vasculopathy as indicated by diffuse concentric intimal hyperplasia and 70% stenosis of a single primary vessel (left anterior descending) on angiography. White race of the donor is associated with an increased incidence of cardiac allograft vasculopathy in the recipient. Recipient CMV IgG/IgM seropositivity, not seronegativity, is also associated with an increased risk of disease. Absence of histological evidence of rejection has no effect on incidence and may be reflective of sampling error (i.e., biopsies did not capture diseased microvasculature). Donor old age and male sex, not young age and female sex, are risk factors for the development of cardiac allograft vasculopathy.

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Reference: Chronic allograft vasculopathy

A 51 year old woman received an orthotopic heart transplant four years ago due to end stage dilated cardiomyopathy. One year ago, she began experiencing mild exertional dyspnea. At that time, invasive angiography demonstrated 60% stenosis and diffuse narrowing of the left circumflex artery. Over time, the patient developed progressive heart failure and required a repeat transplantation. A representative section of her explanted heart is shown above.

Which of the following statements about this disease is true?

1. Although donor vasculature is affected, recipient vasculature is spared
2. Characteristic mononuclear inflammatory infiltrate is limited to the adventitia
3. Concentric intimal thickening and luminal stenosis is found in epicardial arteries but not epicardial veins
4. Development of the disease is closely associated with EBV seroconversion or reactivation of anti-EBV IgM
5. Positive immunofluorescence for IgG, IgM or IgA is diagnostic of the disease

A. This patient has chronic allograft vasculopathy as indicated by diffuse concentric intimal hyperplasia and 60% stenosis of a single primary vessel (left main coronary artery) on angiography. The patient's biopsy also shows severe intimal hyperplasia with marked luminal stenosis. In this disease, the donor coronary vasculature is affected while the recipient vasculature is spared. EBV seroconversion or reactivation is more associated with posttransplant lymphoproliferative disease. While acute antibody mediated rejection events (indicated by immunofluorescence in choice B) may occur in the context of cardiac allograft vasculopathy, it is not diagnostic. The inflammatory infiltrate seen in the disease may occur in any layer of the vessel and concentric intimal thickening may occur in both epicardial arteries and veins.

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Reference: Chronic allograft vasculopathy