Towards a Diagnosis of Cardiac Amyloidosis: Single Center Experience with (99m) Technetium Pyrophosphate Planar Imaging and Opportunities for Standardization of Diagnostic Workflow

Background and Objectives: Cardiac amyloidosis is a disorder caused by amyloid fibril deposition in the extracellular space of the heart. Almost all forms of clinical cardiac amyloidosis are transthyretin amyloidosis (ATTR) or light chain amyloidosis. (99m) technetium pyrophosphate ((99m)Tc PYP scan) has changed the landscape of the non-biopsy diagnosis of ATTR cardiac amyloidosis (ATTR-CA) by providing remarkably high diagnostic accuracy. We examined our experience with PYP scans in patients undergoing workup for ATTR-CA and evaluated the diagnostic workflow in patients with intermediate PYP scan results. Materials and Methods: Retrospective chart review study in which we analyzed data of 84 patients who underwent c-99m pyrophosphate (PYP) SPECT scan for the diagnosis of ATTR-CA from 2017 till 2021 at our institution. We identified three groups: Low uptake (PYPL uptake ratio \u3c 1.2 + visual grade 1/0), n = 30, Intermediate uptake (PYPI uptake ratio 1.2-1.49, visual grade 2/3), n = 25 and High uptake (PYPH uptake ratio ≥ 1.5 + visual grade 2/3), n = 29. We reviewed patients\u27 demographics, medical histories, echo parameters and diagnostic testing including light chain analysis, cardiac magnetic resonance results, and biopsies. Results: Mean patients\u27 age was 73, male-to=female ratio 3:1, 59% of patients were African American. Cardiovascular comorbidities, cardiac biomarkers (BNP and Troponin) and amyloid-related neuropathy were similar in all groups. A statistically significant difference in septal thickness/posterior wall thickness and final diagnosis were found between the groups. The distribution of overall diagnostic testing ratios for the PYPI group included serum protein electrophoresis 92%, urine protein electrophoresis 65%, free light chain 80%, CMR 32%, tissue biopsy done in 20% and BM biopsy in 16%, which are similar to the ratios of other groups. Overall, 25% (n = 5, 4 TTR-CA and 1 AL Amyloid) of patients in the PYPI group had a final diagnosis of CA established with additional testing (p = 0.001 vs. other groups). Conclusions: The (99m)PYP scan is an accurate noninvasive test for cardiac ATTR-CA. Importantly, 25% of the PYPI group had a final diagnosis of ATTR-CA reiterating that diagnosis needs to be pursued in PYPI cases based on clinical suspicion. Routine evaluation and exclusion of light chain disease and establishing a consistent workflow for amyloid diagnosis and continued education for technologists and readers of PYP scans is key to a successful amyloidosis workup

Towards a Diagnosis of Cardiac Amyloidosis: Single Center Experience with ^99m Technetium Pyrophosphate Planar Imaging and Opportunities for Standardization of Diagnostic Workflow

Background and Objectives: Cardiac amyloidosis is a disorder caused by amyloid fibril deposition in the extracellular space of the heart. Almost all forms of clinical cardiac amyloidosis are transthyretin amyloidosis (ATTR) or light chain amyloidosis. 99m technetium pyrophosphate (99mTc PYP scan) has changed the landscape of the non-biopsy diagnosis of ATTR cardiac amyloidosis (ATTR-CA) by providing remarkably high diagnostic accuracy. We examined our experience with PYP scans in patients undergoing workup for ATTR-CA and evaluated the diagnostic workflow in patients with intermediate PYP scan results. Materials and Methods: Retrospective chart review study in which we analyzed data of 84 patients who underwent c-99m pyrophosphate (PYP) SPECT scan for the diagnosis of ATTR-CA from 2017 till 2021 at our institution. We identified three groups: Low uptake (PYPL uptake ratio n = 30, Intermediate uptake (PYPI uptake ratio 1.2–1.49, visual grade 2/3), n = 25 and High uptake (PYPH uptake ratio ≥ 1.5 + visual grade 2/3), n = 29. We reviewed patients’ demographics, medical histories, echo parameters and diagnostic testing including light chain analysis, cardiac magnetic resonance results, and biopsies. Results: Mean patients’ age was 73, male-to=female ratio 3:1, 59% of patients were African American. Cardiovascular comorbidities, cardiac biomarkers (BNP and Troponin) and amyloid-related neuropathy were similar in all groups. A statistically significant difference in septal thickness/posterior wall thickness and final diagnosis were found between the groups. The distribution of overall diagnostic testing ratios for the PYPI group included serum protein electrophoresis 92%, urine protein electrophoresis 65%, free light chain 80%, CMR 32%, tissue biopsy done in 20% and BM biopsy in 16%, which are similar to the ratios of other groups. Overall, 25% (n = 5, 4 TTR-CA and 1 AL Amyloid) of patients in the PYPI group had a final diagnosis of CA established with additional testing (p = 0.001 vs. other groups). Conclusions: The 99mPYP scan is an accurate noninvasive test for cardiac ATTR-CA. Importantly, 25% of the PYPI group had a final diagnosis of ATTR-CA reiterating that diagnosis needs to be pursued in PYPI cases based on clinical suspicion. Routine evaluation and exclusion of light chain disease and establishing a consistent workflow for amyloid diagnosis and continued education for technologists and readers of PYP scans is key to a successful amyloidosis workup

Clinical impact of pre-kidney transplant pulmonary hypertension on post-transplant outcomes

Outcomes of kidney transplant (KT) patients with pre-transplant pulmonary hypertension (PH) are poorly understood. PH patients are often considered high risk and excluded from KT. We investigated the association of pre-transplant PH with KT recipient\u27s outcomes. A single-center, retrospective study that reviewed all patients transplanted from 2010 to 2016, who had a transthoracic echocardiogram (TTE) before KT and at least one TTE post-KT. The TTE closest to the KT was used for analyses. PH is defined as pulmonary artery systolic pressure (PASP) ≥ 40 mm Hg. Of 204 patients, 61 had PASP ≥ 40 mm Hg (with PH) and 143 had PASP \u3c 40 mm Hg (without PH) prior to KT. No statistically significant differences existed between the two groups in baseline demographics, renal failure etiologies, dialysis access type, and cardiovascular risk factors. The mean difference in pre-KT PASP was 18.1 ± 7 mm Hg (P \u3c 0.001). Patients with PH had a statistically significant decrease in PASP post-KT compared to the patients without PH with a mean change of -7.03 ± 12.28 mm Hg vs. + 3.96 ± 11.98 mm Hg (p \u3c 0.001), respectively. Moderate mitral and moderate-severe tricuspid regurgitation were the only factors found to be independently associated with PH (p = 0.001) on multivariable analysis. No statistically significant difference was notable in patient survival, graft function, and creatinine post-KT in both groups. PH pre-KT particularly mild-moderate PH did not adversely affect intermediate (90-day) and long-term allograft and patient survival. Patients with mild-moderate PH should not be excluded from KT

LARGE SINGLE CENTER EXPERIENCE WITH MANTA VASCULAR CLOSURE DEVICE IN TRANSCATHETER AORTIC VALVE REPLACEMENT AND MECHANICAL CIRCULATORY SUPPORT

Background: Transcatheter aortic valve replacement (TAVR) and mechanical circulatory support (MCS) are common procedures requiring large-bore arterial access. Data on suture-based VCD for large-bore arterial access closure have been comprehensive. The safety and efficacy of the MANTA VCD using real-world data are scarce. The present study aims to evaluate the safety and efficacy of the MANTA vascular closure device (VCD) (Teleflex, Morrisville, NC, USA) in arterial transfemoral large-bore sheath size from 14 to 24 Fr using real-world data in a tertiary-care center. Methods: A single-center evaluation of patients who underwent TAVR and MCS with the use of Manta VCD from June 2019 to September 2020. The primary efficacy outcome is defined as immediate hemostasis post closure in absence of major bleeding or access site endovascular or surgical intervention. The secondary safety outcomes assessed using Valve Academic Research Consortium-2 (VARC-2) criteria, including major and minor bleeding and vascular complications. Results: Twelve different operators consecutively treated 174 patients with the MANTA device. TAVR was performed in 94 (54%), percutaneous MCS was performed in 76 (43.6%), and other structural interventions in five (3%) of patients. The 14Fr MANTA was used in 59 cases (34%) and the 18Fr was used in 115 cases (66%). The median sheath size was 14 Fr. A single device was deployed in 173 (99.4%) of cases. The primary efficacy outcome was obtained in 92.6% of cases. The secondary safety outcomes using VARC-2 criteria were major bleeding 0.5%, minor bleeding 4.5%, major vascular complication 1.13%, minor vascular complication 4.5%. To achieve hemostasis 6.7% of the cases required endovascular balloon tamponade and 1.7% required covered stents. There was no access site infection, collagen-plug embolization, surgical repair, and retroperitoneal bleeding. Conclusion: In this single-center experience, the use of MANTA VCD can safely and effectively close large bore arteriotomies, including TAVR and MCS. Larger multicenter studies of efficacy, safety, and cost-effectiveness are needed