Table 1.
Summary of the available studies addressing cardiac amyloidosis and aortic stenosis coexistence and outcomes.
| Study/Authors | Year | Study Design | Patients (n) | Age (years) | Intervention | Prevalence | Follow-up | Results | Complications | Conclusions |
|---|---|---|---|---|---|---|---|---|---|---|
| Castaño et al. | 2017 | Prospective study of patients receiving TAVR screened for ATTR-CA with 99mTc-PYP cardiac scintigraphy. | 151 | 84±6 | TAVR with 99mTc-PYP cardiac scintigraphy screening for ATTR-CA. | ATTR in 16% of post-TAVR patients | n/a | Patients with ATTR-CA had a thicker interventricular septum, higher LV mass index, lower SV index, advanced diastolic dysfunction with a high E/A ratio, lower EF, and more impaired global longitudinal strain. | Post-TAVR 17.4% of patients with ATTR-CA needed a permanent pacemaker, versus 14.4% without ATTR-CA. | ATTR-CA is prevalent in 16% of patients with severe AS undergoing TAVR and is associated with severe AS phenotype of low-flow low-gradient with a reduced EF. |
| Cavalcante J et al. | 2017 | Retrospective study of patients with AS referred for CMR, and assessment of the associations between CA & AS and all-cause mortality. | 113 | 74±14 | Transthoracic echocardiogram, CMR, LGE and identification of CA. 59 patients received AVR. | 9/96 (9.4%) patients with severe AS had CA. | Median follow-up was 18 months. | 8% patients had combined CA-AS, 16% patients >74 years-old had combined CA-AS, excluding women 32% of men >74 years-old had CA-AS, combined CA-AS had low flow low gradient physiology, 1 year all-cause mortality in AS-CA patients was 56% versus 20% in isolated AS. | 40 patients died within the median 18-month follow-up, | Significant CA prevalence in patients with moderate-severe AS, particularly among older males. Combined CA-AS often presents with A-fib and a low flow low gradient physiology. Combined CA-AS has an increased 1-year all cause mortality. |
| Java AP et al. | 2018 | Retrospective study of patients with diagnosed ATTR-CA who underwent AVR. | 16 | 76±6 | 11/16 patients had surgical AVR, 5/16 patients had TAVR. | n/a | Median follow-up was 1.9 year. | No 30-day mortality post-op. First post-op mortality occurred 1.5 years after surgery. 4 total deaths during the follow-up period all occurring >1 year post-op ranging from 1.5 to 7.4 years. | 3/16 patients had procedure related complications including femoral artery pseudoaneurysm, post-op tamponade, and low-output syndrome. | AVR has low risk of operative morbidity and mortality in patients with combined CA-AS, TAVR has a reduced hospital stay, 1 year survival rate is excellent. |
| Longhi S et al. | 2016 | Prospective study of patients referred for treatment of AS. Screening for ATTR-CA with echocardiogram and confirmed with DPD scintigraphy. | 43 | 84±6 | DPD scintigraphy in patients with red flags for ATTR-CA on echocardiogram, all patients underwent balloon aortic valvuloplasty and currently undergoing clinical follow-up. | 11.6% patients had combined ATTR-CA and AS. | Currently under-going clinical follow-up | 5 patients had combined ATTR-CA and AS, 4 were men, all had advanced HF, 3 had carpal tunnel syndrome, 4 patients had low flow low gradient physiology. | None reported, pending clinical follow-up. | Combined ATTR-CA & AS can be suspected on the basis of clinical and echocardiographic features, and effectively diagnosed with DPD scintigraphy. |
| Study/Authors | Year | Study Design | Patients (n) | Age (years) | Intervention | Prevalence | Follow-up | Results | Complications | Conclusions |
| Scully PR et al. | 2018 | Prospective study of DPD screening pre-TAVR for patients with severe AS. Patients will be followed post-op through April 2019. | 125 | 86±5 | DPD bone scintigraphy, TAVR, patients positive for ATTR-CA referred to National Amyloidosis Center. | ATTR in 12.8% of patients pre-TAVR. | Patients will under-go follow-up through April 2019. Most recent update was May 2018 [34]. | ATTR in 12.8% of patients pre-TAVR. These patients had lower mean AV gradient and stroke volume index. | 1 periprocedural permanent pacemaker placed, 1 implantable cardiac defibrillator placed, 1 spinal cord infarction. 2 deaths pre-TAVR. | ATTR-CA is prevalent in 12.8% of patients undergoing TAVR and is likely to alter clinical presentation and mortality. |
| Seki T | 2017 | Case report of a 77 year-old patient with 19 year history of hemodialysis, mixed systemic amyloidosis, and carpal tunnel syndrome who underwent AVR. | 1 | 77 | Surgical AVR. | n/a | n/a | After significant hemodynamic instability, metabolic acidosis, and cardiac arrest the patient expired. Autopsy revealed a mixed systemic amyloidosis in virtually all major organs and extensive whole body edema. | Hemodynamic deterioration unresponsive to aggressive therapy, metabolic acidosis, cardiac arrest, and eventual death. | Systemic amyloidosis may be a risk factor for hemodynamic deterioration due to increased vascular permeability after AVR. |
| Treibel TA et al. | 2016 | Prospective study of patients receiving AVR surgery who underwent magnetic resonance and intraoperative biopsies. ATTR was confirmed histologically and typed with immune-histochemistry. | 146 | 75±6 | AVR with intra-operative biopsy. | ATTR in 4.1% of all patients pre-AVR and 5.6% patients >65 years-old. | Median follow-up was 2.3 years. | 6 patients undergoing AVR surgery tested positive for ATTR. These patients had severe hypertrophy and left ventricular impairment. | 3 of the ATTR positive patients died at follow-up (50%), versus 8 ATTR-negative who died at follow-up (7.5%). | ATTR is an important prognostic indicator for elderly patients with AS receiving AVR. |
Note: Transcatheter Aortic Valve Replacement (TAVR), Transthyretin Cardiac Amyloidosis (ATTR-CA), Technetium-99m Pyrophosphate (99mTc-PYP) cardiac scintigraphy, Monoclonal gammopathy of undetermined significance (MGUS), Cardiovascular Magnetic Resonance (CMR), Late Gadolinium Enhancement (LGE), Dihosphono-1,2-Propanodicarboxylic acid (DPD) scintigraphy.