ISHLT Working Formulation of a Standardized Nomenclature for Cardiac Allograft Vasculopathy—2010

The development of cardiac allograft vasculopathy (CAV) remains the Achilles heel of cardiac transplantation. This entity, characterized by intimal proliferation, develops early after transplant, is progressive, and accounts for major morbidity and mortality late in the transplant natural history. Initially, the diagnosis of CAV was made pathologically and was discovered in its most aggressive form of a vasculitis in an era of sub-optimal immunosuppression. As immunosuppression improved and post-cardiac transplant survival increased, angiographic diagnosis became the norm. In the mid-1990s, several groups began to use the innovative technique of intravascular ultrasound (IVUS) to define the early development of angiographically silent cardiac allograft vasculopathy, and this led to an era of greater understanding of this disease. In the 21st century, pathologic definitions of the disease began to surface with the advent of immunohistologic biomarkers and circulating biomarkers.

Despite these advances, there are no standards in the nomenclature of CAV. Much confusion abounds. An early attempt at angiographic classification was not widely adopted due to its lack of prognostic direction and was overshadowed by the advent of IVUS technology. The lack of a standard language has led to confusion in the interpretation of various studies and several unanswered questions persist.

This document represents a working formulation for an international nomenclature of cardiac allograft vasculopathy, similar to the development of the system for adjudication of cardiac allograft rejection by histology.