Cardiac amyloidosis in non‐transplant cardiac surgery

Abstract

Cardiac amyloidosis is a rare infiltrative cardiomyopathy that portends a poor prognosis. There is a growing recognition of co‐existent aortic valve stenosis and transthyretin cardiac amyloidosis, with some studies suggesting that dual pathology may be associated increased risk of complication and mortality during surgical intervention. This review aims to evaluate the available literature on non‐transplant cardiac surgical interventions in patients with cardiac amyloidosis, with particular focus on diagnosis, high surgical risk and areas of uncertainty that require further research.

Cardiac amyloidosis and surgery: What do we know about rare diseases?

“…With the assumption that heart transplantation works acceptably in selected patients, an important statement in this contribution by Smith et al 9 is that it is difficult to find a strong justification to recommend a cardiac surgical intervention in patients with cardiac amyloidosis. There are reasons to understand that these patients, regardless of the clinical problem eventually requiring cardiac surgery, namely aortic stenosis, mitral disease, or coronary artery disease amenable for surgical revascularization, have a clear profile for intra‐ and postoperative complications in the form of low‐output syndrome entailing significant mortality.…”

“…After their review with a focus on diagnosis, surgical risk, and areas of uncertainty that require further research, some take‐home messages from Smith et al 9 are that cardiac amyloidosis, per se, has an intrinsic poor prognosis, that surgical treatment of diseases that may have an amyloid component like aortic stenosis need an extremely careful and accurate individualized assessment and that, currently, heart transplantation has to be considered in specific subgroups. For the time being, the indications for non‐transplant cardiac surgery seem to be restrictive.…”

“…6 Its potential role in heart failure with preserved ejection fraction and eventual therapeutic approach have recently been highlighted in some reviews. 7,8 Having said that, Smith et al 9 from London present in this issue of the journal, an interesting review proposal with regards to cardiac amyloidosis in non-transplant cardiac surgery. As briefly stated by the authors, heart transplantation is becoming the ultimate therapeutic tool in light-chain amyloidosis and transthyretin amyloidosis, with some interesting short-term outcomes reported in some contributions, like the recent one from Vaidya et al 10 These authors report an overall 3-year survival of 81.6% in a series of 51 patients.…”

“…12 However, it is not to be forgotten that this is technically a rare disease and collective experience, especially in the surgical field, is still scanty. In their review, Smith et al 9 discuss a number of small-sized studies and mortality. Most of these studies do not report the actual cause of death.…”