Wild-Type Transthyretin Cardiac Amyloidosis in a Transplanted Heart.

Wild-type transthyretin amyloid cardiomyopathy (ATTRwt-CM) is more prevalent than appreciated in the elderly. We present the case of an 88-year-old woman who underwent heart transplantation for ischemic cardiomyopathy and then presented 21 years later with new onset atrial flutter, found on endomyocardial biopsy to have new ATTRwt-CM. (Level of Difficulty: Advanced.).

Initiation of noninvasive surveillance for allograft rejection in heart transplant patients > 1 year after transplant.

BACKGROUND: Gene expression profiling (GEP) and donor-derived, cell-free DNA (dd-cfDNA) measurement are alternative methods to endomyocardial biopsy (EMB) to monitor for rejection following heart transplantation. We aim to describe our use of GEP and dd-cfDNA in heart transplant recipients > 1-year post-transplantation. METHODS: This is a single-center, retrospective study in post-transplant recipients. For patients who were > 1-year post-transplantation and deemed to be at elevated clinical risk for rejection, we collected both GEP and dd-cfDNA every 3 months. Baseline characteristics including GEP, dd-cfDNA levels, rejection episodes, and number of biopsies were obtained. RESULTS: Since July 2019, there were 18 patients being followed with GEP and dd-cfDNA who were > 1-year post-transplantation. Nine EMBs had been performed in seven patients due to as follows; three due to elevated GEP ({greater than or equal to} 34), one due to elevated dd-cfDNA ({greater than or equal to} .20%), two due to elevations of both GEP and dd-cfDNA, two due to clinical rejection and one to follow up a post rejection episode. One of the two biopsies due to elevations of both GEP and dd-cfDNA showed acute cellular rejection grade 2R. None of the biopsies due to either an elevation in the GEP or dd-cfDNA revealed any significant rejection. CONCLUSION: In this study, the use of both GEP and dd-cfDNA led to an increased number of EMB in patients > 1-year post-transplantation. Further studies are needed to validate these findings and evaluate long-term consequences of these diagnostic tests in this population.

Uncommon obstacle: management of end-stage heart failure in thrombocytopenia with absent radii (TAR) syndrome.

Thrombocytopenia with absent radii (TAR) syndrome is a rare genetic condition causing absent radial bones and thrombocytopenia. Management is generally supportive although there may be a role for platelet-stimulating agents such as romiplostim. In this case, we highlight the obstacles in managing end-stage heart failure in a patient with TAR syndrome.

A Case Report of a Left Atrial Mass: The Importance of a Detailed Physical Exam.

Cardiac myxomas are rare clinical findings. They are frequently found in the left atrium and more commonly affect women. Clinical presentation can vary widely and symptoms can be vague and non-specific. We present a case of a 67-year-old woman presenting with 3 weeks of progressive heart failure symptoms that failed to respond to oral diuretic therapy. On physical exam, she was found to have a diastolic murmur, rumble and an early diastolic plop. Transthoracic echocardiogram revealed a 5.6 cm × 2.5 cm × 4.3 cm left atrial mass attached to the mitral valve causing left atrial outflow obstruction. The patient subsequently underwent surgical resection of the mass with resolution of symptoms immediately thereafter. Lack of recognition of this pathologic process as a cause of heart failure symptoms and lack of a quality physical exam can lead to a delay in diagnosis and treatment.

HIV and coronary artery calcium score: comparison of the Hawaii Aging with HIV Cardiovascular Study and Multi-Ethnic Study of Atherosclerosis (MESA) cohorts.

OBJECTIVES: To determine the association of HIV, immunologic, and inflammatory factors on coronary artery calcium (CAC), a marker of subclinical atherosclerosis. METHODS: Cross-sectional study comparing baseline data of males from Hawaii Aging with HIV - Cardiovascular Study (HAHCS) with the Multi-Ethnic Study of Atherosclerosis (MESA) cohort. The cohorts were pooled to determine effects of HIV on CAC and explore immunologic and inflammatory factors that may explain development of CAC in HIV. Multivariable regression models compared CAC prevalence in HAHCS with MESA adjusting for coronary heart disease (CHD) risk profiles. RESULTS: We studied 100 men from HAHCS and 2733 men from MESA. Positive CAC was seen in 58% HAHCS participants and 57% MESA participants. Mean CAC was 260.8 in HAHCS and 306.5 in MESA. Using relative risk (RR) regression, HAHCS participants had a greater risk (RR = 1.20, P < 0.05) of having positive CAC than MESA when adjusting for age, smoking status, diabetes, antihypertensive therapy, BMI, systolic blood pressure, total cholesterol, and HDL cholesterol. Among participants with positive CAC, HIV infection was not associated with larger amounts of CAC. Among HAHCS participants, current HIV viral load, CD4, length of HIV, interleukin 6 (IL-6), fibrinogen, C-reactive protein (CRP), and D-dimer were not associated with the presence or amount of CAC. DISCUSSION: HIV was independently associated with a positive CAC in men with increased likelihood occurring between 45 and 50 years of age. Current HIV viral load, CD4 count, length of HIV, and inflammatory markers were unrelated to either presence or amount of CAC.