Long-term outcome in patients treated with combined heart and liver transplantation for familial amyloidotic cardiomyopathy.

BACKGROUND: The amyloidogenic transthyretin (ATTR) mutation Leu111Met causes a primarily cardiac amyloidosis: Familial amyloidotic cardiomyopathy (FAC). Combined heart-liver transplantation (CHLTx) is the preferred treatment for patients with heart failure due to familial amyloidosis, but information on outcome of patients with Leu111Met mutation is limited. The aim of this study was to evaluate the long-term outcome of CHLTx in patients with FAC. METHODS AND MATERIALS: Between 1998 and 2009, CHLTx was performed in 7 FAC patients (four men). Six patients underwent simultaneous transplantation. All patients suffered from severe cardiomyopathy. RESULTS: Mean recipient age at transplantation was 48.3 ± 4.2 yr. Mean follow-up was 55 months. No peroperative mortality occured. Two patients died within the first year (infection, multi-organ failure) of transplantation. Cumulative survival at 4.5 yr was 71%. No significant liver rejections occurred. One patient experienced an episode of cardiac rejection requiring treatment (H2R). For the surviving five patients, most recent left ventricular ejection fraction was 0.61 ± 0.02, and plasma creatinine was 129 ± 47 µM. None developed significant allograft vasculopathy or neuropathy after transplantation. No recurrence of cardiac amyloid was found. CONCLUSIONS: CHLTx in selected patients with FAC due to Leu111Met mutation offers acceptable long-term survival, almost comparable with isolated cardiac transplantation. Allograft rejection was rare.

Right ventricular failure after implantation of a continuous-flow left ventricular assist device: early haemodynamic predictors.

OBJECTIVES: Right ventricular failure (RVF) is a significant complication after implantation of a left ventricular assist device. We aimed to identify haemodynamic changes in the early postoperative phase that predicted subsequent development of RVF in a cohort of HeartMate II (HMII) implanted patients. METHODS: This was a single-centre observational study of consecutive placement of HMII devices at Rigshospitalet, Copenhagen. Preoperative data (right heart catheterization, biochemistry and clinical status) and postoperative readings from the first 72 h after implantation (haemodynamics, inotropic and vasoactive therapy) were included in the analysis. The data set was examined for significant differences between patients who developed RVF (RVF group, n = 11)-defined as need for inotropic or vasodilator therapy >14 days, nitric oxide therapy ≥ 48 h or right ventricular assist device therapy-and those who did not (non-RVF group, n = 22). RESULTS: Preoperative right heart catheterization data were similar in the two groups. Immediately after HMII implantation, the increase in cardiac index (CI) was significantly larger in the non-RVF than in the RVF group (0.96 ± 0.8 vs 0.2 ± 0.5 L/min, respectively; P = 0.018), whereas right ventricular stroke work index (RVSWI) decreased significantly more in the RVF group (-4.3 ± 2.0 vs -0.9 ± 2.0 g m/m(2); P < 0.001). These differences were present in spite of the RVF group receiving larger doses of catecholaminergic agents (P = 0.034). Over the ensuing 72 h, the CI of the RVF group gradually approached that of the non-RVF group; concurrently, however, the differences in inotropic therapy were further enhanced. Pump settings were similar in the two groups. CONCLUSIONS: The haemodynamic alterations characterizing RVF were present already immediately after HMII implantation. RVF development was not related to pump flow and settings.

Left ventricular assist device as bridge to recovery for anthracycline-induced terminal heart failure.

Anthracycline treatments are hampered by dose-related cardiotoxicity, frequently leading to heart failure (HF) with a very poor prognosis. The authors report a case of a 19-year-old man developing HF after anthracycline treatment for Ewing sarcoma. Despite medical treatment, his condition deteriorated to terminal HF, leading to implantation of a mechanical left ventricular assist device (LVAD). His heart function recovered, allowing explantation of the device 14 months after implantation. Heart transplantation is often contraindicated in the first years after treatment for cancers, and LVAD as "bridge to recovery" may be warranted in similar patients.

[Normalisation of left ventricular function after 13 months of mechanical circulatory support]. / Normalisering af venstre ventrikel efter 13 måneders mekanisk cirkulationsstøtte.

We discuss the first Danish case in which a left ventricular assist device (HeartMate 2) could be explanted after 13 months of support due to cardiac recovery in a young patient who presented with severe dilated cardiomyopathy during pregnancy. Aggressive medical treatment with angiotensin converting enzyme inhibitor, beta blocker and aldosterone antagonist was used, and the patient remained stable without circulatory support several months after device removal.

Incidence of ventricular arrhythmias in patients on long-term support with a continuous-flow assist device (HeartMate II).

The incidence of ventricular tachycardia (VT) or ventricular fibrillation (VF) in patients supported with a continuous-flow left ventricular assist device (LVAD) has not been investigated in detail. In 23 consecutive recipients of a HeartMate II, we analyzed the incidence of VT/VF during a total of 266 months of follow-up. Sustained VT or VF occurred in 52% of the patients, with the majority of arrhythmias occurring in the first 4 weeks after LVAD implantation. VT/VF requiring implantable cardioverter-defibrillator (ICD) shock or external defibrillation occurred in 8 patients and significant hemodynamic instability ensued in 3 patients. There were no clear predictors of VT/VF, and it is argued that prophylactic ICD implantation should be considered in patients supported with a continuous-flow LVAD.

Muscle activation during selected strength exercises in women with chronic neck muscle pain.

BACKGROUND AND PURPOSE: Muscle-specific strength training has previously been shown to be effective in the rehabilitation of chronic neck muscle pain in women. The aim of this study was to determine the level of activation of the neck and shoulder muscles using surface electromyography (EMG) during selected strengthening exercises in women undergoing rehabilitation for chronic neck muscle pain (defined as a clinical diagnosis of trapezius myalgia). SUBJECTS: The subjects were 12 female workers (age=30-60 years) with a clinical diagnosis of trapezius myalgia and a mean baseline pain intensity of 5.6 (range=3-8) on a scale of 0 to 9. METHOD: Electromyographic activity in the trapezius and deltoid muscles was measured during the exercises (lateral raises, upright rows, shrugs, one-arm rows, and reverse flys) and normalized to EMG activity recorded during a maximal voluntary static contraction (MVC). RESULTS: For most exercises, the level of muscle activation was relatively high (>60% of MVC), highlighting the effectiveness and specificity of the respective exercises. For the trapezius muscle, the highest level of muscle activation was found during the shrug (102+/-11% of MVC), lateral raise (97+/-6% of MVC), and upright row (85+/-5% of MVC) exercises, but the latter 2 exercises required smaller training loads (3-10 kg) compared with the shrug exercise (20-30 kg). DISCUSSION AND CONCLUSION: The lateral raise and upright row may be suitable alternatives to shrugs during rehabilitation of chronic neck muscle pain. Several of the strength exercises had high activation of neck and shoulder muscles in women with chronic neck pain. These exercises can be used equally in the attempt to achieve a beneficial treatment effect on chronic neck muscle pain.

Another pitfall in minimally invasive mitral valve repair.

We report a case of severe systolic anterior motion (SAM) and dynamic left ventricular outflow obstruction after repair of a flail posterior leaflet of the mitral valve. The reason for SAM was found to be due to traction on the pericardial stay sutures placed to expose the surgical field. The SAM and the outflow obstruction were completely resolved by cutting these sutures. Our case demonstrates the contribution of geometric factors in the development of SAM and left ventricular outflow obstruction and emphasizes the need to evaluate the heart in its natural position within the mediastinum.