The medal is her second gold of the championships and her third medal in three days, after gold in the 100m breaststroke on Monday and bronze in the 200m on Sunday.

172 swimmers from 24 countries have been taking part in the competition in Albufeira this week.

"I gave it my all, I'm super happy!" Rattur said after the race.

Coach Eili Paap said the defense tactics had gone to plan. "Keira came here to defend her world championship title and succeeded. Everything went well technically. Keira showed once again that she is among the world's best swimmers with Down syndrome," Paap said.

Mariia Ageeva, competing under a neutral flag, took silver in 50.52 seconds, and Portugal's Matilde Lucia Gaspar took bronze in 51.06.

Rattur's medal haul now stands at two golds and a bronze across three days of competition.

Other Estonians also competed in Portugal. Eric Tött finished sixth in the 100m backstroke in 1:30.42. Kaspar Paul Loik placed 12th in the 200m individual medley in 3:28.13 and 14th in the 100m backstroke in 1:35.39. Hardi Pais finished 12th in the 50m breaststroke in 46.37.

The 12th World Down Syndrome Swimming Championships started on October 30 and run until Friday. Wednesday is a rest day, with competition resuming on Thursday.

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