Table 1.

Summary of the 35 studies retained for analysis

First author of study, year of publication [reference number]	N (size of cohort)	Country	Diagnostic method(s)	Baseline periodicitya	Follow-up (years)b	Evidence class, risk of biasc
Adams, 2000 [19]	45	Multiplei	Tissue biopsy + genetic testing	1993–1999	6	1, Low
Adams, 2018 [20]	225	Multiple	NR	2013–2016	7	1, Low
Algalarrondo, 2015/2016 [3, 21]d	215	France	Tissue biopsy + genetic testing	1993–2011	10	1, Low
Arruda-Olson, 2013 [22]	37	USA	Tissue biopsy	1980–2010	6	2, Low
Barrosso, 2017 [23]	75	Mixed	Tissue biopsy + genetic testing	NR–2014	10	1, Low
Bittencourt, 2002 [24]	24	Brazil	Tissue biopsy + genetic testingo	1993–1999	7	2, Moderate
Carvalho, 2002 [25]	35	Portugal	Genetic testing	1992–2000	5	2, Moderate
Coelho, 2018 [26]	3160	Portugal	Mixed	1992–2016	24	2, Moderate
Coutinho, 2013 [27]	106e	Portugal	Genetic testing	1998–2010	13	1, Moderate
Coutinho, 2017 [27]	232	Portugal	NR	1998–2015	15	1, Moderate
Ericzon, 2015 [28]	1623	Multiplej	NR	1990–2010	20	2, High
Franz, 2013 [29]	21	Germany	Genetic testing	1996–2011	10	2, Moderate
Gonzalez-Lopez, 2018 [30]	118	Spain	NR	NR	25	3, High
Ines, 2018 [31]	3160	Portugalk	NR	NR–2016	35	3, High
Kawaji, 2014 [32]	27	Japan	Tissue biopsy + genetic testing	1987–2011	7	1, Low
Lagarto, 2016 [33]	116	Portugal	Genetic testing	2006–2014	7	2, Moderate
Loavenbruck, 2016 [34]	41f	USA	Tissue biopsy + genetic testing	1993–2013	20	1, Low
Lobato, 2004 [35]	62	Portugal	Tissue biopsy + genetic testingp	1989–2000	9	1, Moderate
Mariani, 2015 [36]	194	Francel	Tissue biopsy + genetic testing	1988–2010	25	2, Low
Munar-Ques, 2005 [37]	102	Spain	Tissue biopsy ± genetic testing	1976–2003	29r	3, Moderate
Ohya, 2011 [38]	34	Japan	Tissue biopsy ± genetic testing	1994–2006	10	3, Moderate
Okamoto, 2011 [39]	104	Sweden	Tissue biopsy ± genetic testing	1990–2008	25	1, Low
Okamoto, 2009 [40]	141	Sweden	Tissue biopsy ± genetic testing	1990–2008	20	1, Low
Okumura, 2016 [41]	65	Japan	Genetic testing	1990–2010	20	2, Moderate
Plante-Bordeneuve, 1998 [42]	65	France	Genetic testing ± tissue biopsy	1988–1997	10	2, High
Suhr, 2016 [11]	264	Multiplej	NR	1991–2012	10	2, High
Suhr, 2002 [43]	51g	Sweden	Tissue biopsy + genetic testing	1990–2000	20	2, Moderate
Swiecicki, 2015 [44]	110 h	USA	Family history ± genetic testing	1970–2013	8	4, High
Takei, 2005 [45]	43	Japan	Genetic testing	1993–2004	10	2, Moderate
Tashima, 1998 [46]	120	Multiplem	Tissue biopsy + genetic testingq	1973–1998	24	3, Moderate
Wixner, 2015 [47]	115	Sweden	Tissue biopsy + genetic testing	1990–2011	9	1, Low
Yamamoto, 1998 [48]	116	Japan	Tissue biopsy + family history	1974–1995	20	4, High
Yamamoto, 2007 [49]	86	Multiplem	Tissue biopsy + genetic testing	1990–2005	16	1, Low
Yamashita, 2012 [50]	80	Multiplen	Tissue biopsy + genetic testing	1990–2010	20	2, Low
Yamashita, 2018 [51]	104	Japan	Tissue biopsy ± genetic testing	2000–2017	10	2, Low

HTx Heart transplantation, KTx kidney transplantation, LTx liver transplantation, mBMI modified body mass index, NR not reported

^aRefers to the time-period over which the study was conducted

^bRefers to maximum number of years from the baseline event at which survival outcomes were reported

^cEvidence grades range from 1 (best) to 4 (worst)

^dNote that two studies are reported here

^eRefers to the matched cohort of LTx (n = 53) ves. non-LTx (n  = 53)

^fRefers to the transthyretin amyloidosis cohort (n = 41) and excludes the primary amyloidosis cohort (n = 60)

^gRefers to the transthyretin amyloidosis cohort (n  = 51) and excludes the control cohort (n  = 19)

^hRefers to Val30Met (n  = 42), Ser77Tyr (n  = 15), and other mutations (n  = 53) and excludes Thr60Ala (n  = 68) and Val122Ile (n  = 28)

ⁱFrance, Germany, and Portugal

^jPortugal, France, Sweden, USA, Brazil, Spain, Japan, UK, Germany, Italy, Australia, Switzerland, Netherlands, Belgium, Argentina, Canada, Denmark, China, and Singapore

^kThe geographical origin of the patient cohort was not explicitly stated in the report. Portugal was assumed based on authors’ affiliations

^lAll patients were treated in France, but n  = 110 (57%) were Portuguese

^mSweden and Japan

ⁿJapan, Sweden, Australia, and USA

^oRefers to definitive testing performed after LTx

^pGenetic testing performed on first-degree relatives for n  = 3 (5%) patients

^qGenetic testing was performed only on patients living past 1997

^rNot reported and so imputed as the year of publication (2005) minus the earliest baseline year (1976)