Aims Temporal conversions among ejection fraction (EF) classes can occur across the heart failure (HF) spectrum reflecting amended structural and functional outcomes unaccounted for by current taxonomy. This retrospective study aims to investigate the differences in serum laboratory values, guideline-directed medical therapy (GDMT), and co-morbidity burden across EF conversion groups. Methods and resultsHeart failure patients at least 18-year-old who obtained at least two echocardiograms between January 2018 and January 2020 were identified using ICD-10 codes. Analysis of variance, chi-square tests, and analysis of means for proportions were used as appropriate to identify associations with class conversion groups. A total of 874 patients who underwent 1748 echocardiograms on unique visits were categorized according to initial EF as HF with preserved EF (HFpEF) (n = 531, 61%), HF with mildly reduced or midrange EF (HFmrEF) (n = 132, 15%), or HF with reduced EF (HFrEF) (n = 211, 24%). In accordance with follow-up EF, class conversions were categorized into HF with improved EF (HFiEF) (n = 143, 16%), HF with worsened EF (HFwEF) (n = 171, 20%), or HF with stable EF (HFsEF) (n = 560, 64%). The average age was 75 ± 13 years old; 54% were male, 85% were Caucasian, 11% were African American, and 4% other. The mean time between EF assessments was 208.6 ± 170.2 days. Serum sodium levels were greater in HFwEF (139 ± 3 mmol/L) when compared with HFsEF (138 ± 4 mmol/L) (P = 0.05). Pro-BNP levels were higher in HFiEF (12 150 ± 19 554 pg/mL) versus HFsEF (6671 ± 10 525 pg/ mL) (P = 0.007). Angiotensin receptor-neprilysin inhibitors (ARNI) were more frequently ordered on index visit in HFiEF (P = 0.03), but no other significant differences in GDMT were identified. Despite similar Elixhauser Co-morbidity Measure (ECM) scores, ECM categorical analysis revealed that HFwEF was more likely to have an established diagnosis of depression (P = 0.03) and a spectrum of psychiatric illnesses (P = 0.03) on preliminary visit. HFsEF was less likely to have an established diagnosis of blood loss anaemia (P = 0.04). Metastatic cancer was more likely to have been diagnosed in HFiEF and less likely in HFsEF (P = 0.002). Conclusions Despite similar ECM scores, EF class conversion groups demonstrated salient differences in average serum sodium and pro-BNP levels. Inpatient ARNI orders, psychiatric, hematologic, and oncologic co-morbidity patterns were also significantly different. Findings demonstrate blood-based biomarker patterns and targetable co-morbid conditions which may play a role in future EF class conversion. Dedicated studies evaluating measurements related to GDMT dose-titration, quality of life, and functionality are the next steps in this field of HF.

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B-cell prolymphocytic leukemia (B-PLL) is a rare leukemia characterized by rapidly increasing leukocytosis with splenomegaly and lymphadenopathy. Treatment strategies are largely based on studies of chronic lymphocytic leukemia (CLL). Antibodies against the cell surface protein CD20 are considered to be first-line therapy. A 76-year-old male with known CLL presented 2 weeks after starting chemoimmunotherapy for newly refractory CLL after failing ibrutinib therapy. White blood cell count was elevated at 226.7 × 103/µL. Fluorescent in situ hybridization analysis of a bone marrow specimen showed new development of complex cytogenetics. Flow cytometry revealed B cells appearing slightly dimmer on CD45 and brighter on CD20 compared with typical B-CLL suggestive of less mature lymphocyte forms. The patient was diagnosed with B-PLL and started on obinutuzumab and venetoclax with rapid normalization of white blood cells. This case recapitulates the challenges in diagnosing and treating B-PLL. Ibrutinib resistance is a growing area of study with several proposed mechanisms of acquired resistance. The pathogenesis of B-PLL is not completely understood, although mutations in MYC are presumed to play a role.

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Idiopathic granulomatous mastitis (IGM) is a rare, benign inflammatory disorder of the breast. Clinical features may include painful breasts, erythema, subcutaneous nodules, and ulcerative lesions. It can mimic various other breast pathologies, and it is a diagnosis of exclusion after infection, malignancy, and other inflammatory conditions have been ruled out. In this article, we present a case of IGM developing in a 40-year-old female 3 months after hospitalization for myxedema coma. A contrast-enhanced magnetic resonance imaging of the breasts showed bilateral edema, and a biopsy was negative for malignancy or infection. She was started on prednisone and had noticeable improvement of ulcerations within several weeks. IGM is a rare condition that requires a multimodal treatment approach. Often recalcitrant disease is encountered and requires surgical intervention, immunosuppression, and antimicrobial therapy. The diagnosis should be entertained in patients with bilateral breast inflammation to avoid unnecessary surgical resection early on.

Abrupt, transient, and severe hypertension evoked by catecholamine-secreting tumors has the potential to manifest as acute aortic dissection. We report the successful, multidisciplinary management of an insidious, extra-adrenal, functional paraganglioma, suddenly presenting as acute aortic dissection. ( Level of Difficulty: Beginner. )

Crescentic glomerulonephritis, also known as rapidly progressive glomerulonephritis, is a syndrome characterized by progressive and rapid deterioration of renal function over the course of weeks to months. Oliguria, hematuria, azotemia, and hypertension are characteristic features of this condition. Crescentic glomerulonephritis is further classified according to the staining pattern on immunofluorescence. In rare instances, a mixed pattern of injury is encountered as in the case of double antibody-positive rapidly progressive glomerulonephritis (RPGN). This case illustrates the challenge in treatment of double antibody-positive RPGN in an elderly female with no previous renal disease. The patient was found to be positive for anti-GBM antibody and MPO-ANCA. Treatment was initially targeted against MPO-ANCA as the biopsy was most consistent with this process; however, the patient failed to respond to treatment and was subsequently transitioned to oral cyclophosphamide directed against anti-GBM disease. In cases of doubly antibody-positive RPGN with anti-GBM disease and ANCA-associated vasculitis, initial treatment should focus on inducing remission of anti-GBM disease as double antibody-positive disease often presents with the aggressive morbidity and mortality seen in anti-GBM disease, and the chronic risk of relapse seen in ANCA-mediated vasculitis.

Background: Excess thyroid hormone alters the hemodynamics of the heart and vascular system resulting in a hyper-dynamic circulatory state which can lead to thyrotoxic heart disease. Clinical case: A 48-year-old male presented to the hospital with several days of rapid heart rate and weakness. He denied prior medical history, medication or supplement intake. Pertinent exam revealed a very thin, hoarse gentleman with shallow breathing and obvious hand tremors; heart exam with irregular rate and tachycardia, absent peripheral edema and bibasilar crackles bilaterally. EKG was consistent with atrial fibrillation with rapid ventricular response. Lab work revealed a pro-BNP of 4000 and positive D-dimer. CTA-Thorax was consistent with CHF with cardiomegaly, large pleural effusions, pulmonary edema and an incidental finding of thyromegaly. Echocardiogram revealed global LV hypokinesis with Ejection Fraction of 10-15%. TSH was <0.005, free Thyroxine >8.00, and Triiodothyronine was 27.04. Thyroid ultrasound showed heterogeneous enlarged gland with hypervascularity and no focal nodules. Graves’ Disease was confirmed with thyroid stimulating Immunoglobulin of 507. Methimazole and hydrocortisone were initiated along with Metoprolol and digoxin for rate control. Thyroid hormone has significant impact on cardiac function, structure and the vascular system. In the vasculature, T3 works to decrease systemic vascular resistance via direct vasodilatation. The decrease in SVR activates RAAS resulting in retention of sodium and fluid. Thyroid hormone also stimulates erythropoiesis which results in an increase in blood volume and stroke volume. In the heart, T3 enters the myocyte via specific transport proteins and via transcription-and non-transcription-mediated effects, increases contractility and relaxation of the cells. Given the increased metabolism, low SVR and increase in total blood volume, this creates a high cardiac output state. Untreated high-output state leads to ventricular dilatation, persistent tachycardia and eventual heart failure. Thus, prompt diagnosis and treatment of cardiac dysfunction is key. Of most importance is correction of thyroid dysfunction with either anti-thyroid medication, radioactive iodine ablation or thyroidectomy. This can possibly reverse and “cure” patient’s systolic heart failure. Conclusion: We present a case of thyrotoxicosis -induced cardiomyopathy with left ventricular ejection fraction of 10% secondary to undiagnosed Graves disease and emphasize the importance of thyroid disease as a possible cause of systolic heart failure. References: Riaz K, Forker AD, Isley WL, Hamburg MS, McCullough PA. (2007). Hyperthyroidism: A “Curable” Cause of Congestive Heart Failure - Three Case Reports and a Review of the Literature. Congestive Heart Failure. 2007; 9: 40-46