A case report of transthyretin amyloidosis following cardiac transplantation: thick ventricles that look alike.

Background: Transthyretin (ATTR) amyloidosis is more prevalent than initially thought. As much as 13% of patients hospitalized with heart failure with preserved ejection fraction may have ATTR-cardiomyopathy (CM). Conversely, heart transplant patients may manifest left ventricular hypertrophy or diastolic dysfunction, especially late after transplantation. Case summary: We present a case of a 82-year-old male heart transplant patient, 31 years following orthotopic heart transplantation. While he was satisfied with his exercise capacity as an octogenarian, several years before, he required pacemaker implantation due to third-degree atrioventricular block, had bilateral carpal tunnel syndrome treated with carpal tunnel release surgery, and experienced idiopathic sudden deafness. Based on increasing left ventricular wall thickness during routine follow-up, a diagnosis of ATTR amyloidosis was suspected. Ultimately, the diagnosis was confirmed non-invasively with a specific scintigraphic exam, while an additional physicochemical stain on an endomyocardial biopsy taken several years before provided pathological proof. We initiated tafamidis, yet stopped this treatment after 1 month because of gastrointestinal intolerance. Ultimately, our patient died 2 years later due to heart failure. Discussion: Our case shows the long delay between the onset of ATTR deposition, the presence of clinical signs, and the final diagnosis. Echocardiographic findings suggestive for ATTR-CM include left ventricular hypertrophy and diastolic dysfunction, which are both common in heart transplant patients. Yet, ATTR-CM should be considered in the differential diagnosis, especially late after transplantation, in this closely monitored population.

Use of Real-Time Cine MRI to Assess the Respirophasic Variation of the Inferior Vena Cava-Proof-of-Concept and Validation Against Transthoracic Echocardiography.

BACKGROUND: In clinical practice, the right heart filling status is assessed using the respirophasic variation of the inferior vena cava (IVC) assessed by transthoracic echocardiography (TTE) showing moderate correlations with the catheter-based reference standard. PURPOSE: To develop and validate a similar approach using MRI. STUDY TYPE: Prospective. POPULATION: 37 male elite cyclists (mean age 26 ± 4 years). FIELD STRENGTH/SEQUENCE: Real-time balanced steady-state free-precession cine sequence at 1.5 Tesla. ASSESSMENT: Respirophasic variation included assessment of expiratory size of the upper hepatic part of the IVC and degree of inspiratory collapse expressed as collapsibility index (CI). The IVC was studied either in long-axis direction (TTE) or using two transverse slices, separated by 30 mm (MRI) during operator-guided deep breathing. For MRI, in addition to the TTE-like diameter, IVC area and major and minor axis diameters were also assessed, together with the corresponding CIs. STATISTICAL TESTS: Repeated measures ANOVA test with Bonferroni correction. Intraclass correlation coefficient (ICC) and Bland-Altman analysis for intrareader and inter-reader agreement. A P value <0.05 was considered statistically significant. RESULTS: No significant differences in expiratory IVC diameter were found between TTE and MRI, i.e., 25 ± 4 mm vs. 25 ± 3 mm (P = 0.242), but MRI showed a higher CI, i.e., 76% ± 14% vs. 66% ± 14% (P < 0.05). As the IVC presented a noncircular shape, i.e., major and minor expiratory diameter of 28 ± 4 mm and 21 ± 4 mm, respectively, the CI varied according to the orientation, i.e., 63% ± 27% vs. 75% ± 16%, respectively. Alternatively, expiratory IVC area was 4.3 ± 1.1 cm2 and showed a significantly higher CI, i.e., 86% ± 14% than diameter-based CI (P < 0.05). All participants showed a CI >50% with MRI versus 35/37 (94%) with TTE. ICC values ranged 0.546-0.841 for MRI and 0.545-0.704 for TTE. CONCLUSION: Assessment of the respirophasic IVC variation is feasible with MRI. Adding this biomarker may be of particular use in evaluating heart failure patients. LEVEL OF EVIDENCE: 1 TECHNICAL EFFICACY STAGE: 2.

Radiation-Induced Changes in Quartz, A Mineral Analog of Nuclear Power Plant Concrete Aggregates.

Quartz single-crystal samples consisting of α-quartz crystal structure were neutron irradiated to fluences of 5 × 1018, 4 × 1019, and 2 × 1020 n/cm2 (E > 0.1 MeV) at two temperatures (52 and 95 °C). The changes in the α-quartz phase as a function of these two conditions (temperature and fluence) were studied using X-ray powder diffraction (XRD), Raman spectroscopy, and transmission electron microscopy (TEM), and the results acquired using these complementary techniques are presented in a single place for the first time. XRD studies showed that the lattice parameters of α-quartz increased with increasing neutron flux. The lattice growth was larger for the samples that were neutron irradiated at 52 °C than at 95 °C. Moreover, an amorphous content was determined in the quartz samples neutron irradiated at 4 × 1019 n/cm2, with the greater amount being in the 52 °C irradiated sample. Complete amorphization of quartz was observed at a fluence of 2 × 1020 n/cm2 (E > 0.1 MeV) using XRD and confirmed by TEM characterization and Raman spectroscopic studies. The cause for α-quartz lattice expansion and sample amorphization was also explored using XRD and Raman spectroscopic studies.