A 28-year-old female with a history of situational syncope and a new-onset right sided hemiparesis is described. Tilt-up table test revealed the postural orthostatic tachycardia syndrome followed by vasovagal syncope. Neurological and internal medicine tests showed no particular disorders. The patient underwent autonomic physical training and the tilt-up test performed three months later showed improvement of the autonomic system in terms of lower heart beat rate of the postural orthostatic tachycardia syndrome and longer duration of the test. This case report describes longstanding idiopathic dysautonomia that can be improved by nonpharmacological treatment, while reminding that this medical condition may also be the cause of syncope.Prikazuje se 28-godišnja bolesnica s anamnezom višegodišnje situacijske sinkope i novonastalom desnostranom hemiparezom. Učinjen tilt-up table testom utvrđen je sindrom posturalne ortostatske tahikardije (engl. POTS , postural orthostatic tachycardia syndrome), nakon kojeg je uslijedila vazovagalna sinkopa. Neurološkom i internističkom obradom nije utvrđen eventualan drugi uzrok sinkope. Tijekom slijedeća tri mjeseca bolesnica je u kućnim uvjetima provodila ortostatske vježbe autonomnog sustava, nakon čega je učinjen kontrolni tilt-up table test kojim je objektivizirano poboljšanje statusa autonomnog sustava u smislu nižih vrijednosti frekvencije tahikardije POTS -a i duljeg trajanja testa, odnosno održavanja ortostaze. Ovaj slučaj govori o višegodišnjoj idiopatskoj disautonomiji na koju je moguće utjecati nefarmakološkim metodama te je ujedno i podsjetnik na jedan od mogućih uzroka sinkope

Diana Delić-Brkljačić

Hrvoje Hećimović

Hrvoje Pintarić

Vedrana Škerk

Zvonimir Popović

English

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Acta Clin Croat,  Vol. 51,  No. 1,  2012 93Acta Clin Croat 2012; 51:93-95 Case ReportORthOstAtiC intOleRAnCe: POstuRAl ORthOstAtiC tAChyCARdiA syndROme with OveRlAPPing vAsOvAgAl synCOPevedrana Škerk1, hrvoje Pintarić1, diana delić-Brkljačić1, Zvonimir Popović2 and hrvoje hećimović31department of Cardiology, university department of medicine, sestre milosrdnice university hospital Center, Zagreb; 2school of medicine, Josip Juraj strossmayer university, Osijek; 3university department of neurology, Zagreb epilepsy Center, sestre milosrdnice university hospital Center, Zagreb, CroatiasummARy – A 28-year-old female with a history of situational syncope and a new-onset right sided hemiparesis is described. tilt-up table test revealed the postural orthostatic tachycardia syndrome followed by vasovagal syncope. neurological and internal medicine tests showed no par-ticular disorders. The patient underwent autonomic physical training and the tilt-up test performed three months later showed improvement of the autonomic system in terms of lower heart beat rate of the postural orthostatic tachycardia syndrome and longer duration of the test. This case report describes longstanding idiopathic dysautonomia that can be improved by nonpharmacological trea-tment, while reminding that this medical condition may also be the cause of syncope.Key words: Postural orthostatic tachycardia syndrome; Syncope; DysautonomiaCorrespondence to: Vedrana Škerk, MD, PhD, department of Cardiology, university department of medicine, sestre milosrd-nice university hospital Center, vinogradska c. 29, hR-10000 Zagreb, Croatiae-mail: vedranaskerk@gmail.comReceived April 20, 2011, accepted January 9, 2012IntroductionPostural orthostatic tachycardia syndrome (POts) is observed predominantly in younger women and is defined as a sustained heart rate increase of ≥30 bpm or an increase to ≥120 bpm within the first 10 min of orthostasis associated with symptoms of orthostatic intolerance and without significant orthostatic hy-potension. A higher prevalence of vasovagal syncope has been observed among patients with POts. There are no accurate epidemiological studies regarding this syndrome. its etiology is believed to be multifactorial with a number of possible underlying mechanisms and overlapping syndromes1,2. here we present a 28-year-old female with a history of typical situational syncope and a new-onset right-sided hemiparesis.Case ReportA 28-year-old female was hospitalized at neurol-ogy department because of typical vasovagal syncope (prodromes occurred in a crowded warm place) fol-lowed by new-onset unilateral right-sided hemiparesis of the lower arm and leg. while unconscious, neither physical movements nor post-syncope diuresis/stool were observed in our patient. medical history re-vealed recurrent syncopes in provocative surroundings since puberty, without any neurological disturbances recorded. Other patient history was unremarkable. syncopes and symptoms such as lightheadedness and palpitations have transitorily worsened after each of the two normal pregnancies. The patient is current active smoker and had been using birth control pills for the past four months. she denied infection of any kind or insect bites recent to this episode. MethodsPhysical examination revealed intact cardiopul-monary finding, but showed right-sided neurologi-94 Acta Clin Croat,  Vol. 51,   No. 1,  2012vedrana Škerk et al. Orthostatic intolerance: postural orthostatic tachycardia syndrome with overlapping vasovagal syncopecal performance to be weaker. neurological testing (Ct, mR, eeg, duplex doppler, emng, CsF) showed no pathology. laboratory findings (RBC, wBC, coagulation parameters, Fe, uiBC, tiBC, Fe saturation, thyroid hormones, hbA1c, esR, CRP, electrolytes, blood urea nitrogen, creatinine, amin-otransferases, urine) were normal. Cardiac cause of the syncope was evaluated; there was no orthostatic hypotension, eCg was unremarkable as well as tr-ansthoracic echocardiography. head up tilt table test revealed POts followed by vasovagal syncope in the 25th minute of the test. since then, the patient has been performing orthostatic workout regularly, taking care of daily fluid and salt intake, and has been undergoing physical therapy for three months. The following head up tilt table test showed no trace of hemiparesis and it was positive in the 48th minute of the test.during the first tilt-up test, the heart beat rate was approximately 135-145/bpm with concomitant palpitations, at 95/70 mm hg. vasovagal syncope oc-curred in the 25th min at 45/bpm and blood pressure of 50 mm hg palpatory (Fig. 1a). After three months of autonomic physical training, the second tilt-up test showed improvement in terms of lower heart beat rate and longer duration of the test. during the second tilt-up test, the heart beat was approximately 125/bpm with blood pressure of 90/75 mm hg, while the patient was asymptomatic. in the 43rd minute, pro-dromes occurred at 140/bpm. in the 47th minute, a rapid decrease of pulse was noted, a minute later fol-lowed by vasovagal syncope at 52/bpm and blood pressure of 60 mm hg palpatory (Fig. 1b). Both tilt-up tests were performed according to the 2009 syn-cope guidelines, without medical provocation3.DiscussionThis is an interesting case presenting dysautonomia of an untraceable cause. Our patient’s dysautonomia manifested as transitory hemiparesis and orthostatic impairment presenting as POts with overlapping va-sovagal syncope.POts etiology is still unknown and is supposed to be multifactorial, in the sense of sympathetic hy-persensitivity, venous and arterial dilatation mecha-nisms at the microcirculatory level4-7. POts is part of a wide spectrum of disorders that exhibit auto-nomic dysfunction. These include severe orthostatic hypotension in the presence of autonomic neuropathy and vasovagal syncope in the absence of other evi-dence of autonomic dysfunction. POts manifests as an excessive orthostatic tachycardia without signifi-cant orthostatic hypotension in patients without overt autonomic neuropathy. it is associated with numerous other symptoms such as exercise intolerance, palpita-tions, weakness, and lightheadedness; most of these symptoms are also autonomically mediated8. Our patient’s clinical impairment was improved by nonpharmacological maneuvers, proved by bet-ter physical performance (absence of no-tilt induced syncope and no manifest hemiparesis), objectified by longer maintaining of orthostatic stability at the rec-ommended duration of the tilt up table test in persons with POts9. here we considered orthostatic home training and care of daily fluid and salt intake as suf-ficient therapy for this neurocardiovascular condition. This case report can be perceived as a contribution to the overall knowledge of POts, as it is a relatively new medical term, and as a reminder to consider this Fig. 1a. Tilt up table test 1. Fig. 1b. Tilt up table test 2.1601401201008060supine position orthostasis0 20 0 5 10 15 20 25 30 35 40 45 48heart ratesystolic bloodpressure1801601401201008060 0 20 0 5 10 15 20 25supine position orthostasisheart ratesystolic bloodpressureActa Clin Croat,  Vol. 51,  No. 1,  2012 95vedrana Škerk et al. Orthostatic intolerance: postural orthostatic tachycardia syndrome with overlapping vasovagal syncopecondition when evaluating the potential causes of syn-cope. References1. CARew s, COnnOR mO, COOKe J, COnwAy R, sheehy C, COstellOe A, et al. A review of postural orthostatic tachycardia syndrome. europace 2009;11:18-25.2. FReemAn R, KAuFmAnn h. Postural tachycardia syndrome. http://www.uptodate.com/patients/content/topic.do?topicKey=~lC342gtmi__iKC (17 november 2010, data last accessed).3. european heart Rhythm Association (ehRA); heart Fail-ure Association (hFA); heart Rhythm society (hRs); eu-ropean society of emergency medicine (eusem); european Federation of internal medicine (eFim); european union geriatric medicine society (eugms), et al. guidelines for the diagnosis and management of syncope (version 2009): the task Force for the diagnosis and management of syncope of the european society of Cardiology (esC). eur heart J 2009;30:2631-71.4. Fu Q , vAngundy tB, gAlBReAth mm, shi-BAtA s, JAin m, hAstings Jl, et al. Cardiac origins of the postural orthostatic tachycardia syndrome. J Am Coll Cardiol 2010;55:2858-68.5. ZyŚKO d, gAJeK J, sCiBORsKi R, smeReKA J, CheCiŃsKi i, mAZuReK w. The pattern of activation of the sympathetic nervous system during tilt-induced syn-cope. europace 2007;9:225-7.6. gRAssi g. vasovagal syncope, sympathetic mechanisms and prognosis: the shape of things to come. eur heart J 2010;31:1951-3.7. PARRy sw, Kenny RA. The role of tilt table testing in neurocardiovascular instability in older adults. eur heart J 2001;22:370-2.8. BlOmstRÖm-lundQvist C, sCheinmAn mm. ACC/AhA/esC guidelines for the management of Pa-tients with supraventricular Arrhythmias. http://www.escardio.org/guidelines-surveys/esc-guidelines/guidelines-documents/guidelines-svA-Ft.pdf (7 April 2011, data last accessed).9. CARew s, COOKe J, O’COnnOR m, dOnnelly t, COstellOe A, sheehy C, et al. what is the op-timal duration of tilt testing for the assessment of patients with suspected postural tachycardia syndrome? europace 2009;11:635-7.sažetakORtOstAtsKA intOleRAnCiJA: sindROm POstuRAlne ORtOstAtsKe tAhiKARdiJe s vAZOvAgAlnOm sinKOPOmV. Škerk, H. Pintarić, D. Delić-Brkljačić, Z. Popović i H. HećimovićPrikazuje se 28-godišnja bolesnica s anamnezom višegodišnje situacijske sinkope i novonastalom desnostranom hemi-parezom. učinjen tilt-up table testom utvrđen je sindrom posturalne ortostatske tahikardije (engl. POts, postural orthosta-tic tachycardia syndrome), nakon kojeg je uslijedila vazovagalna sinkopa. neurološkom i internističkom obradom nije utvr-đen eventualan drugi uzrok sinkope. tijekom slijedeća tri mjeseca bolesnica je u kućnim uvjetima provodila ortostatske vježbe autonomnog sustava, nakon čega je učinjen kontrolni tilt-up table test kojim je objektivizirano poboljšanje statusa autonomnog sustava u smislu nižih vrijednosti frekvencije tahikardije POts-a i duljeg trajanja testa, odnosno održavanja ortostaze. Ovaj slučaj govori o višegodišnjoj idiopatskoj disautonomiji na koju je moguće utjecati nefarmakološkim meto-dama te je ujedno i podsjetnik na jedan od mogućih uzroka sinkope. Ključne riječi: Sindrom posturalne ortostatske tahikardije; Sinkopa; Disautonomija

Ortostatska intolerancija: sindrom posturalne ortostatske tahikardije s vazovagalnom sinkopom

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