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Aims: To evaluate the influence of endomyocardial biopsy (EMB)-proven intramyocardial inflammation on mortality in patients with cardiac transthyretin amyloid (ATTR) or amyloid light-chain (AL) amyloidosis., Methods and Results: We included 54 consecutive patients (mean age 68.83 ± 9.59 years; 45 men) with EMB-proven cardiac amyloidosis. We followed up patients from first diagnostic biopsy to as long as 36 months (mean 11.5 ± 12 months) and compared their outcome with information on all-cause mortality with or without proof of inflammation on EMB. Intramyocardial inflammation was assessed by quantitative immunohistology. Patients suffering from amyloidosis revealed a significant poor prognosis with proof of intramyocardial inflammation in contrast to those without inflammation (log-rank P = 0.019). Re-grouping of patients indicated AL amyloidosis to have a significant impact on all-cause mortality (log-rank P = 0.012). The detailed subgroup analysis showed that patients suffering from AL amyloidosis with intramyocardial inflammation have a significantly worse prognosis compared with AL amyloidosis without inflammation and ATTR with or without inflammation, respectively (log-rank P = 0.014, contingency Fisher's exact test, P = 0.008)., Conclusion: Our study reports for the first time a high incidence (48.1%) of intramyocardial inflammation in a series of patients with EMB-proven cardiac amyloidosis and could show that in patients with AL amyloidosis, intramyocardial inflammation correlated significantly with increased mortality. Our data have a direct clinical impact because one can hypothesize that additional immunomodulating/anti-inflammatory treatment regimens in patients with biopsy-proven inflammation of heart muscle tissue could be beneficial for patients suffering from cardiac AL amyloidosis., (© 2017 The Authors. European Journal of Heart Failure © 2017 European Society of Cardiology.)

Animal studies have shown clearly that a peripheral circulatory support system can effectively unload the left ventricle. We report here the first implantation of one such system (Cancion, Orqis Medical, Lake Forest, CA) in a human. The Cancion system consists of a centrifugal pump connected to the circulation via a graft cannula anastomosed to the left axillary artery and a percutaneous cannula placed into the left common femoral artery. Flow is initiated from the femoral to the axillary artery. The system was implanted in a 62-year-old man with ischemic cardiomyopathy suffering from decompensation whose condition had not improved with catecholamine therapy. The Cancion system supported the patient for 3 days, after which it was electively explanted. During the support period, pulmonary capillary wedge pressure dramatically decreased from 28 to 9 mm Hg, left ventricular diastolic dimension decreased from 6.78 to 6.16 cm, creatinine levels decreased from 1.9 to 0.9 mg/dl, cardiac index improved from 1.5 to 2.7 L/min, and ejection fraction improved from 25 to 35%. Together, our data indicate that the Cancion system quickly and effectively improved the patient's hemodynamics. This suggests that the device may one day become a short-term alternative to high dose inotrope therapy and that its application may delay the need for more invasive forms of mechanical circulatory support.

To investigate long-term follow-up and identify prognostic factors in patients with dilated cardiomyopathy (DCM) the authors investigated 167 consecutive patients on an outpatient basis. All patients underwent left- and right-heart catheterization; follow-up comprised clinical and echocardiographic investigations. Results: After a mean follow-up period of ninety-three ± thirty-six months 82 patients (49%; 71 men, 11 women, mean age fifty-five ± eleven years) were alive. 29 of them (27 men, 2 women, mean age fifty-two ± nine) showed normal left ventricular ejection fraction (LVEF) after a mean follow-up period of one hundred four ± forty months. The remaining 53 patients (44 men, 9 women, mean age fifty-six ± eleven) revealed LVEF similar to that of the first examination. Eighty-five patients died (51%; 73 men, 12 women). Causes of death were the following: progressive heart failure, 24; sudden death, 23; stroke, 3; pulmonary embolism, 2; noncardiac death, 4; unknown causes, 29. The median period from the onset of first symptoms until definite diagnosis was two months in patients with stable conditions, three months in those with normalization of LVEF and twenty-four months in those who died, respectively (P < 0.01). At the time of diagnosis, patients with stable outcome had a mean LVEF (LVEF 1) of 37%, those who returned to normal had 40% (ns). Patients who died had a mean LVEF 1 of 32% and therefore differed significantly from both groups of survivors (P < 0.001). Left ventricular end-diastolic pressure (LVEDP) at the time of diagnosis was highest in patients who died (22 mmHg) and therefore differed significantly from both groups of survivors (normalization: 16 mmHg, stable patients: 18 mmHg, P < 0.001). Conclusions: According to their results, time until diagnosis, LVEF, and LVEDP are prognostic indicators. No difference was noted between the groups concerning etiology, medical treatment, or functional classification according to the New York Heart Association. [ABSTRACT FROM AUTHOR]