Frequency of Arrhythmias and Postural Orthostatic Tachycardia Syndrome in Patients With Marfan Syndrome: A Nationwide Inpatient Study

Syed Emir Irfan Wafa, C. Anwar A. Chahal, Hiroyuki Sawatari, Mohammed Y. Khanji, Hassan Khan, Babken Asatryan, Raheel Ahmed, Saurabh Deshpande, Rui Providencia, Abhishek Deshmukh, Anjali Tiku Owens, Virend K. Somers, Deepak Padmanabhan, Heidi Connolly

Research output: Contribution to journalArticlepeer-review


BACKGROUND: Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder affecting multiple systems, par-ticularly the cardiovascular system. The leading causes of death in MFS are aortopathies and valvular disease. We wanted to identify the frequency of arrhythmia and postural orthostatic tachycardia syndrome, length of hospital stay, health care– associated costs (HAC), and in-hospital mortality in patients with MFS. METHODS AND RESULTS: The National Inpatient Sample database from 2005 to 2014 was queried using International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) codes for MFS and arrhythmias. Patients were classified into subgroups: supraventricular tachycardia, ventricular tachycardia (VT), atrial fibrillation, atrial flutter, and without any type of arrhythmia. Data about length of stay, HAC, and in-hospital mortality were also abstracted from National Inpatient Sample database. Adjusted HAC was calculated as multiplying HAC and cost-to-charge ratio; 12 079 MFS hospitalizations were identified; 1893 patients (15.7%) had an arrhythmia; and 4.9% of the patients had postural orthostatic tachycardia syndrome. Median values of length of stay and adjusted HAC in VT group were the highest among the groups (VT: 6 days, $18 975.8; supraventricular tachycardia: 4 days, $11 906.6; atrial flutter: 4 days, $11 274.5; atrial fibrillation: 5 days, $10431.4; without any type of arrhythmia: 4 days, $8336.6; both P=0.0001). VT group had highest in-patient mortality (VT: 5.3%, atrial fibrillation: 4.1%, without any type of arrhythmia: 2.1%, atrial flutter: 1.7%, supraventricular tachycardia: 0%; P<0.0001) even after adjust-ment for potential confounders (without any type of arrhythmia versus VT; odds ratio [95% CI]: 3.18 [1.62– 6.24], P=0.001). CONCLUSIONS: Arrhythmias and postural orthostatic tachycardia syndrome in MFS were high and associated with increased length of stay, HAC, and in-hospital mortality especially in patients with VT.

Original languageEnglish (US)
Article numbere024939
JournalJournal of the American Heart Association
Issue number17
StatePublished - Sep 6 2022


  • Marfan syndrome
  • cardiac arrhythmia
  • hospitalization
  • postural orthostatic tachycardia syndrome
  • ventricular arrhythmia

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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