Long QT Syndrome natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]

Overview

Natural History

About half the patients will have an arrhythmia that degenerates into Torsade de Pointes.

Complications

Long QT syndrome can result in fatal heart arrhythmias and death. Certain medications can increase the risk of fatal arrhythmias and death in persons with long QT syndrome.

List of Medications to be Avoided in Congenital Long QT Syndrome



Methadone


Methadone


Methylphenidate


Methylphenidate


Midodrine


Mirtazapine


Moexipril/HCTZ


Moxifloxacin


Nicardipine


Nilotinib


Norepinephrine


Nortriptyline


Octreotide


Ofloxacin


Ondansetron


Oxytocin


Paliperidone


Paroxetine


Pentamidine


Pentamidine


Perflutren lipid microspheres


Phentermine


Phentermine


Phenylephrine


Phenylpropanolamine


Phenylpropanolamine


Pimozide


Probucol


Procainamide


Procainamide


Protriptyline


Pseudoephedrine


Pseudoephedrine


Quetiapine


Quinidine


Quinidine


Ranolazine


Risperidone


Ritodrine


Ritonavir


Roxithromycin*


Salmeterol


Sertindole


Sertindole


Sertraline


Sevoflurane


Sibutramine


Solifenacin


Sotalol


Sparfloxacin


Sunitinib


Tacrolimus


Tamoxifen


Telithromycin


Terbutaline


Terfenadine


Thioridazine


Tizanidine


Tolterodine


Trazodone


Trimethoprim-Sulfa


Trimipramine


Vandetanib


Vardenafil


Venlafaxine


Voriconazole


Ziprasidone

Prognosis

People who are treated with lifestyle modifications and medications live longer than those who are not. For people who are not treated, half of them, mostly those with the inherited form of long QT syndrome, will die within 10 years.

The Jervell and Lange-Nielsen syndrome (JLNS) is an autosomal recessive form of LQTS with associated congenital deafness. It is caused specifically by mutation of the KCNE1 and KCNQ1 genes. In untreated individuals with JLNS, about 50 percent die by the age of 15 years due to ventricular arrhythmias.

References

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