The report by Danetz, Donofrio and Embrey1 provides an interesting long term follow-up on the first patient diagnosed by me with what is now known as Noonan syndrome. In 1960, when this patient was seen, cardiac ultrasound was unavailable and left-sided cardiac catheterization uncommon. He had severe pulmonary stenosis, and did well following surgery. Unfortunately, his follow-up was not adequate and left-sided obstruction was not recognized until he became symptomatic some thirty years later. Early reports stressed the dysplastic pulmonary valve, with pulmonary stenosis as a common finding.2 By 1972, the report of Ehlers et al.3 brought attention to the left-sided involvement in the Turner phenotype.
Noonan syndrome – then and now