Verification of Heart Disease: Implications for a New Heart Transplantation Allocation System.

OBJECTIVES: This study sought to determine the accuracy of the pre-transplantation clinical diagnosis of heart disease in the United Network for Organ Sharing (UNOS) database. BACKGROUND: Because survival on the heart transplantation waitlist depends on underlying heart disease, a new allocation system will include the type of heart disease. Accuracy of the pre-transplantation clinical diagnosis and the effect of misclassification are unknown. METHODS: We included all adults who received transplants at our center between January 2009 to December 2015. We compared the pre-transplantation clinical diagnosis at listing with pathology of the explanted heart and determined the potential effect of misclassification with the proposed allocation system. RESULTS: A total of 334 patients had the following clinical cardiac diagnoses at listing: 148 had dilated cardiomyopathy, 19 had restrictive cardiomyopathy, 103 had ischemic cardiomyopathy, 24 had hypertrophic cardiomyopathy, 11 had valvular disease, 16 had congenital heart disease (CHD), and 13 patients had a diagnosis of "other." Pathology of the explanted hearts revealed 82% concordance and 18% discordance (10% coding errors and 8% incorrect diagnosis). The most common incorrect diagnoses were sarcoidosis (66%), arrhythmogenic right ventricular dysplasia (60%), and other causes of predominately right-sided heart failure (33%). Among the misclassified diagnoses, 40% were listed as UNOS status 2, 8% remained at status 2 at transplantation, and only sarcoidosis and CHD were potentially at a disadvantage with the new allocation. CONCLUSIONS: There is high concordance between clinical and pathologic diagnosis, except for sarcoidosis and genetic diseases. Few misclassifications result in disadvantages to patients based on the new allocation system, but rare diseases like sarcoidosis remain problematic. To improve the UNOS database and enhance outcome research, pathology of the explanted hearts should be required post-transplantation.

Multimodality Imaging and Pathologic Assessment in an Adult with Endocardial Fibroelastosis.

•The authors report a patient with biopsy-proven adult endocardial fibroelastosis.•Transthoracic echocardiography revealed diffuse coarse endocardial calcifications.•Native CT of the chest revealed LV endocardial calcifications.

Incidence, management, and outcomes of the arteriovenous fistula complicating transvenous lead extraction.

BACKGROUND: An arteriovenous (AV) fistula is an infrequently reported complication of transvenous lead extraction (TLE), but may be under-recognized. OBJECTIVE: To determine the incidence, management, and outcomes of the AV fistula complicating TLE. METHODS: All TLE procedures from July 2001 to December 2012 were reviewed. RESULTS: Of 2471 patients who underwent TLE, the AV fistula occurred in 8 (0.3%; 6 pacemaker and 2 implantable cardioverter-defibrillator) procedures. Patients who developed an AV fistula had longer lead implant duration (11.8 [interquartile range 7.7] vs 5.2 [interquartile range 7.0] years) and were more likely to have required a powered sheath (8 of 8 [100%] vs 1392 of 2463 [56.5%]) compared to those who did not. Other procedural and demographic details did not discriminate. All patients had a continuous bruit along the anterior chest wall. Catheter angiography was more sensitive (100%) than computed tomography (75%) or ultrasound (25%) for confirming the diagnosis and identifying the vessels involved. One fistula closed spontaneously, while others were closed with covered stents (4) or surgical glue (1). One attempt at closure failed, and one was diagnosed postmortem. Mortality related to the AV fistula was 3 of 8 (37.5%). Patients who died all had structural heart disease. In the 5 patients with normal hearts, the AV fistula was well tolerated for up to 2 years. CONCLUSIONS: An AV fistula is an infrequent, though possibly under-recognized, complication of TLE. It requires a high index of suspicion for early diagnosis, may present late, and may be associated with significant morbidity and mortality in patients with structural heart disease.