Mean pulmonary artery pressure estimated by echocardiogram rapidly exceeds 20 mmHg from the normal range in patients with connective tissue disease.

Earlier intervention for pulmonary hypertension (PH) has been reported to improve the prognosis of patients with connective tissue disease (CTD). However, it is not fully elucidated how rapidly PH develops in patients showing normal mean pulmonary arterial pressure (mPAP) at the index investigation. We evaluated 191 CTD patients with normal mPAP retrospectively. The mPAP was estimated by the formerly defined method employing echocardiography (mPAPecho). We investigated predictive factors that predict increasing mPAPecho at follow-up transthoracic echocardiography (TTE) using uni- and multi variable analysis. The mean age was 61.5 years old, and 160 patients were female. The percentage of patients in whom mPAPecho exceeded 20 mmHg at follow-up TTE was 38%. Multivariable analysis revealed that acceleration time/ejection time (AcT/ET) measured at the right ventricular outflow tract at initial TTE was independently associated with the consequent increase of mPAPecho at the follow-up TTE. When using 0.43 of best cutoff value in AcT/ET calculated by receiver operating characteristic analysis, the change in mPAPecho in patients with low AcT/ET was significantly higher than in those with high AcT/ET (3.05 mmHg in patients with AcT/ET < 0.43 and 1.00 mmHg in patients with AcT/ET ≥ 0.43, p < 0.001). Thirty-eight percent of CTD patients who show the normal estimated mPAP by TTE develop gradual elevation of mPAP to the level to consider early intervention within 2 years. AcT/ET at initial TTE can predict increasing mPAP at follow-up TTE.

Clinical utility of reticulocyte hemoglobin equivalent in patients with heart failure.

Anemia and iron deficiency (ID) are common in patients with heart failure (HF) and intravenous (IV) administration of iron to patients hospitalized for decompensated HF with ID improves outcome. The diagnosis of ID in routine practice is based on serum ferritin and transferrin saturation (TSAT) but both have limitations; alternatives should be considered. Reticulocyte hemoglobin equivalent (Ret-He) reflects iron content in reticulocytes but its clinical utility in patients with HF remains uncertain. We prospectively enrolled 142 patients hospitalized for decompensated HF. Sixty five percent had ID as defined in current international guidelines. Ret-He was directly correlated with serum iron and ferritin concentrations and with TSAT. There was a poor relationship between quartile of Ret-He and HF hospitalization or death but increases or decreases in Ret-He between admission and discharge were associated with a worse outcome. The clinical utility of Ret-He for identifying ID and predicting response to IV iron and prognosis for patients with HF requires further investigation.

Severe acute heart failure during or following cytokine release syndrome after CAR T-cell therapy.

Although cardiac dysfunction after chimeric antigen receptor (CAR) T-cell therapy has been increasingly reported, the underlying dynamics and pathogenesis are not well documented. Herein, we describe the clinical presentation and treatment for two patients who developed severe acute heart failure after CAR T-cell therapy. Both cases shared several common characteristics, including the bone marrow involvement at the time of CAR T-cell therapy and early onset of cytokine release syndrome (CRS) with fever developing on the day of CAR T-cell infusion. Patients with early onset and/or severe CRS should be carefully monitored for the possibility of heart failure.

Urine albumin-to-creatinine ratio on admission predicts early rehospitalization in patients with acute decompensated heart failure.

Detecting high-risk patients for early rehospitalization is crucial in heart failure patient care. An association of albuminuria with cardiovascular events is well known. However, its predictive impact on rehospitalization for acute decompensated heart failure (ADHF) remains unknown. In this study, 190 consecutive patients admitted due to ADHF between 2017 and April 2019 who underwent urinalysis were enrolled. Among them, 140 patients from whom urine albumin-to-creatinine ratio (UACR) was measured with spot urine samples on admission were further analyzed. The association between UACR and rehospitalization due to HF during 1 year after discharge was evaluated. The mean age of 140 participants was 77.6 years and 55% were men. Only 18% (n = 25) of patients presented with normoalbuminuria (UACR < 30 mg/g∙creatinine), whereas 59% (n = 83) and 23% (n = 32) showed microalbuminuria (UACR 30-300 mg/g·creatinine) and macroalbuminuria (UACR > 300 mg/g·creatinine), respectively. The level of UACR on admission was correlated with the risk of subsequent rehospitalization due to HF (p = 0.017). The receiver operating characteristic analysis indicated that the best cut-off values for the UACR and B-type natriuretic peptide (BNP) levels to predict ADHF rehospitalization were 50 mg/g·creatinine and 824 pg/ml, respectively. When the patients were divided into four groups using both cut-off values, the individual predictive impacts of UACR and BNP on rehospitalization were comparable. Patients with both elevated UACR and BNP levels had a higher rate of HF rehospitalization than those with elevated BNP levels alone (p < 0.05). The combination of both values enabled more accurate prediction of HF rehospitalization than BNP levels alone. In conclusion, UACR could be a new useful biomarker to predict HF rehospitalization in patients with ADHF, especially in combination with the levels of BNP, and should be further evaluated in a prospective study.

Usefulness of electron microscopy in the diagnosis of wild-type transthyretin cardiac amyloidosis.

Wild-type transthyretin cardiac amyloidosis (ATTRwt) has been recognized as an important cause of heart failure with preserved ejection fraction; thus, its accurate diagnosis is crucial. Herein, we describe the case of a 76-year-old man who presented with dyspnea and palpitation. On observing the laboratory evaluations and clinical course, we suspected cardiac amyloidosis. However, optical microscopic analysis by Congo-red and direct fast scarlet staining revealed no amyloid deposits in the biopsy samples. Therefore, a more thorough investigation was pursued by examining the myocardial tissue under electron microscopy. We could recognize amyloid deposits between the myocardial fibers using electron microscopy. We submitted all the pathological specimens to a specialized facility for genetic testing to ensure the accurate diagnosis of the amyloidosis disease type. As a result, a biopsy sample from the minor salivary gland was stained with the Congo red stain. Anti-transthyretin antibody detected using immunohistochemical analysis of amyloidosis supported the presence of transthyretin form of amyloid proteins. Genetic testing revealed the absence of TTR gene mutations. The final diagnosis was ATTRwt. We believe that this case suggests the usefulness of electron microscopy in the diagnosis of ATTRwt and other related disorders. Further study is warranted to validate our findings.

A cavotricuspid isthmus pouch revealed to be a breakout site for gap conduction of recurrent common atrial flutter.

A cavotricuspid isthmus pouch can be a breakout site for gap conduction of cavotricuspid isthmus block line. If the previous block line is electrically silent, high-density 3-D mapping and pouchgraphy are useful to find the pouch and ablate within it.

Pseudoblock of cavotricuspid isthmus via detouring gap conduction.

To detect detouring gap conduction, as demonstrated in this case, 3-D mapping is useful in addition to conventional methods.

Disappearance of an anticoagulation-resistant left atrial appendage thrombus after administration of low-dose pimobendan.

Anticoagulation therapy is essential for preventing thrombus formation in the left atrial appendage (LAA) and subsequent ischemic strokes in patients with atrial fibrillation (AF). The complete disappearance of any existing LAA thrombi is crucial before AF ablation. Currently, warfarin and direct oral anticoagulants are widely used for this purpose. However, treatment strategies for anticoagulation-resistant LAA thrombi are not well established. Here, we present a case of an 85-year-old male who was scheduled to undergo AF ablation. He developed an LAA thrombus that was resistant to 300 mg/day of dabigatran. Low-dose pimobendan was prescribed in addition to dabigatran; three months later, the thrombus was dissolved successfully. This case demonstrates the potential efficacy of a low-dose oral inotrope for treating an anticoagulation-resistant LAA thrombus. .