New England Journal of Medicine

Auxologico and Mayo Clinic global leaders in Long QT

Review by the world's most authoritative medical journal confirms Italy's centrality in the management of the leading cause of sudden death under 20 years of age

2' min read

Translated by AI
Versione italiana

2' min read

Translated by AI
Versione italiana

The New England Journal of Medicine (Nejm), the world's most prestigious medical journal, has published a review entirely authored by Italian specialists from Irccs Istituto Auxologico Italiano, recognising the Centre for Genetic Arrhythmias' global leadership on Long QT Syndrome (Lqts).

Lqts, the leading cause of sudden death under 20, is a genetic disorder that can trigger fatal arrhythmias under stressful conditions, during sport, swimming or even at the sound of an alarm clock. Today, thanks to therapeutic advances developed in Italy, the risk of death has fallen from 50% in the 1960s and 1970s to less than 1%.

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A certification of worldwide authority

When the Nejm entrusts a review to a specific group of specialists, it actually recognises those authors as the world's leading experts on the subject. In the case of Lqts, the choice fell entirely on the Italians of the Auxologico, indicated as the organisation with the most experience in the world on Lqts, together with the famous US Mayo Clinic.

The review represents 'the most advanced framework' for the clinical management of the condition, defining therapeutic priorities and prevention strategies for the most dangerous arrhythmias.

Among the points highlighted, the review emphasises the great effectiveness of left-sided cardiac sympathetic denervation: an operation lasting about 50 minutes, without opening the chest, which drastically reduces the risk of the most serious arrhythmias when beta-blocker drugs are insufficient. This approach significantly improves patients' quality of life and, above all, reduces the need for implantable defibrillators, which are reserved for only a very limited number of cases.

Peter Schwartz: the pioneer who changed the history of the disease

Peter Schwartz, a pivotal figure and for decades the world's leading expert on Long QT Syndrome, recounts in his own words the origin of this technique, which he introduced when he was just 30 years old: 'It was in 1973 that I first did it in my first patient, Agostina, who was not protected by beta-blocker drugs (which are almost always very effective) and who had had a new cardiac arrest. The child's father asked me: "But why do you want to cut these nerves?" I answered because of some experiments in dogs done in America and my own experiments on cats. We did the surgery on 25 March 1973 and my patient had nothing more until 2017 when she died in an accident.

Schwartz also recalls how, for almost twenty years, he was the only one in the world to perform the procedure, travelling with his surgeon 'from Russia to China, from Israel to Holland' to operate on young patients suffering from the syndrome. It wasn't until the 2000s that the Mayo Clinic began practising it regularly. Today it is recognised as one of the most effective therapies for patients not protected by beta-blockers. Working alongside him for over 25 years is Lia Crotti, co-author of the review and international reference in genetics and arrhythmology.

Global leadership that speaks Italian

Nejm's recognition is not just an academic achievement: it is confirmation of Italy's central role in the research and treatment of Long QT Syndrome. A rare but potentially fatal disease, which thanks to innovations born in our country, and now shared with the Mayo Clinic, can be successfully managed in almost all cases.

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