A retrospective analysis of treatment outcomes in 45 patients with cardiac light-chain amyloidosis: a single-center experience in Japan.

The prognosis of cardiac light-chain (AL) amyloidosis is considered to be very poor. We studied the treatment efficacy and outcomes by retrospectively analyzing the clinical results of 45 patients with cardiac AL amyloidosis treated at our hospital between September 2008 and March 2016. The group of patients analyzed included 29 males and 16 females with a median age of 68 years. Their baseline median NT-proBNP, cTnT, and dFLC were 3167 pg/ml, 0.080 ng/ml, and 286.17 mg/l, respectively. Twenty-eight patients were in Cardiac Stage (CS) III and 17 patients were in Revised Prognostic Stage (RPS) IV. At the median follow-up of 10 months, the median overall survival (OS) was 16 months and 3-year OS was 35.9%. The patients in CS III showed significantly poorer survival rate than those in CS I or II (3-year OS: 12.2% vs. 65.8%, p = 0.0115) and the patients in RPS IV showed significantly poorer survival rate than those in RPS I, II, or III (3-year OS: 11.0% vs. 53.3%, p = 0.000914). Regardless of the therapeutic approaches, patients who achieved hematological CR or cardiac organ response demonstrated significantly improved prognosis. Therefore, achievement of hematological and organ responses is important in the treatment of cardiac AL amyloidosis.

Impact of Left Ventricular Diastolic Property on Left Atrial Function from Simultaneous Left Atrial and Ventricular Three-Dimensional Echocardiographic Volume Measurement.

Simultaneous left atrial (LA) and left ventricular (LV) 3-dimensional (3D) echocardiographic volume measurements enable the quantifying of precise LA function, by virtue of their independence on any geometric assumption and capability of measurement for net LA conduit volume. We sought to elucidate the impact of conventional Doppler LV diastolic property on LA volume and function derived from this method. Our study subjects consisted of 381 patients who underwent 3D echocardiography. From LA time-volume curve, maximum and minimum volume index (VI) and VI before atrial contraction (LAVIpre-c) were determined; subsequently, active emptying volume was calculated as LAVIpre-c - minimum LAVI. From LA and LV volume measurement, conduit volume was calculated as LV stroke VI - (maximum LAVI - minimum LAVI). LA volume increased depending on the severity of diastolic dysfunction. Compared with patients with normal diastolic function, LA booster pump function, as the contribution of active emptying volume to LV filling, was higher in those with mild diastolic dysfunction. Additionally, it gradually decreased as diastolic dysfunction was advanced from mild to moderate and moderate to severe degree (23.2 ± 15.5%, 29.5 ± 15.1%, 25.1 ± 16.2%, 14.9 ± 14.1%, respectively; p <0.001). Contrarily, conduit contribution was significantly higher in patients with severe diastolic dysfunction than in those with mild diastolic dysfunction; furthermore, conduit function tended to increase, reciprocally to booster pump function, as diastolic dysfunction grade was advanced (39.1 ± 28.8%, 36.8 ± 26.2%, 42.7 ± 25.6%, 52.9 ± 26.2%, respectively; p = 0.034). In conclusion, simultaneous LA and LV volumetric analyses through 3D echocardiography clearly demonstrate the characteristic LA functional alterations following LA dilation caused by LV diastolic dysfunction.

[Hypereosinophilic syndrome accompanied with digital gangrene: efficacy of therapeutic angiogenesis by autologous bone marrow mononuclear cells transplantaion].

A 62-year-old-man presented to our hospital with complaints of coldness, numbness, pain, weakness and cyanosis on the fingers and toes in March 2010. Laboratory findings revealed marked eosinophilia (46.6%; WBC 20600/µl), an elevation of serum creatine kinase, proteinuria and hematuria. He was diagnosed as hypereosinophilic syndrome (HES) without evidence to support a diagnosis of underlying diseases causing eosinophilia. After the initiation of corticosteroid therapy, peripheral eosinophil count was dramatically decreased, and both serum CK value and urinary findings became normalized. However, his symptoms persisted and digital necrotic changes developed. Angiography of the bilateral upper and lower extremities showed multiple arterial occlusions with poor collaterals. The digital gangrenes were unresponsive to peripheral circulation ameliorators and gradually progressed. In July 2010, autologous transplantation of bone marrow mononuclear cells was performed for achievement of therapeutic angiogenesis. His digital skin color was ameliorated by the angiogenic therapy in two weeks, and digital gangrenes did not progress after that. After amputation of his fingers and toe, cut surfaces healed with favorable epithelization, and the symptoms were subsequently eliminated. Moreover, during three years after the therapy, as well as the effect on the skin lesion, the significant improvement in peripheral circulation was observed. Therefore, we proposed that therapeutic angiogenesis by autologous bone marrow mononuclear cells transplantation was a novel and effective treatment for intractable digital gangrene associated with HES.

Primary percutaneous coronary intervention and intravascular ultrasound imaging for coronary thrombosis after cisplatin-based chemotherapy.

Although cisplatin is indispensable for the chemotherapy treatment of many malignancies, cisplatin-associated thrombosis is attracting increasing attention. However, experience of primary percutaneous coronary intervention (PCI) and intravascular ultrasound imaging (IVUS) for coronary thrombosis, possibly due to cisplatin-based chemotherapy, has been limited. Case 1 with postoperative gastric cancer developed acute myocardial infarction (AMI) on the sixth day of the second chemotherapy course with conventional doses of cisplatin and tegafur gimeracil oteracil potassium. Emergency coronary angiography (CAG) showed a filling defect in the proximal left anterior descending coronary artery (LAD) concomitant with no reflow in the distal LAD. Case 2 with advanced lung cancer and brain metastasis suffered AMI on the fifth day of the first chemotherapy course with conventional doses of cisplatin and gemcitabine. Emergency CAG delineated a total occlusion in the proximal right coronary artery. In both cases, thrombectomy using aspiration catheter alone obtained optimal angiographic results and subsequent IVUS revealed no definite atherosclerotic plaque, while slow flow still remained even after selective intra-coronary infusion of vasodilator in the case 1. These cases suggest that primary PCI using thrombus-aspiration catheter might be safe and effective for coronary thrombosis due to cisplatin-based chemotherapy.

Efficacy and safety of continuous hemodiafiltration for acute decompensated heart failure.

The mortality of heart failure patients with renal insufficiency is high, and these patients tend to develop diuretic resistance. Under these conditions, continuous hemodiafiltration (CHDF) is a possible alternative volume reduction therapy to diuretics. However, its efficacy and safety are not clear. Between April 2005 and March 2008, 248 patients with acute decompensated heart failure were admitted to the CCU of Kyoto City Hospital. Of those patients, 31 (20 volume overloaded heart failure, 11 cardiogenic shock) received CHDF therapy, and their weight loss, acute hemodynamic changes, and clinical outcome were assessed to evaluate the efficacy and safety of CHDF therapy. CHDF was performed for 6.5 +/- 6.5 days. There was no significant change in acute hemodynamics after CHDF initiation. In the volume overloaded heart failure (VH) group, significant weight loss was observed at 24 hours and 48 hours after CHDF initiation (P < 0.001). In-hospital mortality of the VH group and cardiogenic shock (CS) group were 10.0% and 54.5%, respectively. CHDF for acute decompensated heart failure (ADHF) is a safe, effective, and reliable volume reduction therapy for volume overloaded heart failure. Further investigation is required to assess the effectiveness of CHDF for cardiogenic shock.