Systemic Sclerosis and Pulmonary Hypertension

HomeCirculation: Heart FailureVol. 14, No. 2Systemic Sclerosis and Pulmonary Hypertension Free AccessResearch ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyRedditDiggEmail Jump toFree ArticlePDF/EPUBSystemic HypertensionThe Great Masquerader Claudia Baratto, MD, Sergio Caravita, PhD, Giovanni Battista Perego, Luigi P. Badano, Jean-Luc Vachiéry, MD Gianfranco Parati, BarattoClaudia Baratto Department of Cardiovascular, Neural Metabolic Sciences, Istituto Auxologico Italiano IRCCS, Ospedale San Luca, Milano, Italy (C.B., S.C., G.B.P., L.P.B., G.P.). Medicine Surgery, University Milano-Bicocca, Search for more papers by this author , CaravitaSergio Caravita Correspondence to: Management, Information Production Engineering, Bergamo, IRCCS Piazzale Brescia 20, 20149 Italy. Email E-mail Address: [email protected] https://orcid.org/0000-0002-3003-6499 Dalmine, (S.C.). PeregoGiovanni Perego BadanoLuigi Badano https://orcid.org/0000-0002-0379-3283 VachiéryJean-Luc Vachiéry Cardiology, Cliniques Universitaires de Bruxelles, Hôpital Académique Erasme, Belgium (J.-L.V.). ParatiGianfranco Parati https://orcid.org/0000-0001-9402-7439 Originally published28 Jan 2021https://doi.org/10.1161/CIRCHEARTFAILURE.120.007554Circulation: Failure. 2021;14:e007554Other version(s) articleYou are viewing the most recent version article. Previous versions: January 28, 2021: Ahead Print The cardiorespiratory involvement in systemic sclerosis quite invariably leads shortness breath,1 but exact pathophysiology underlying symptom might be difficult decipher.A 72-year-old woman complained dyspnea New York Association III-IV after a diagnosis sclerosis. Echocardiography revealed high probability pulmonary hypertension; left atrium was at upper limit normal (33 mL/m2) with reduced reservoir function, witnessed strain 14% (Figure 1). At right heart catheterization, patient showed precapillary hypertension vascular resistance 6.8 WU. An invasive exercise test significant reduction cardiac output (CO) reserve (CO peak, 4.5 L/min; CO/Vo2 slope, 4), accounting moderate-severe peak oxygen consumption (55% predicted). artery wedge pressure (PAWP) remained persistently both rest during exercise, near equalization between PAWP atrial pressure, consistent enhanced ventricular interdependence.Download figureDownload PowerPointFigure 1. first evaluation follow-up. moderately enlarged only follow-up, analysis had already demonstrated function assessment.A arterial associated formulated, an oral combination therapy started clinical improvement.Nine months later, still dyspnea. This time, echocardiography dilation 55 mL/m2 cardiopulmonary study repeated. Mean lower halved (3.5 WU) as compared previous test. CO rest, supernormal (CO/Vo2 9). 12 mm Hg higher than pressure. During tall V wave appeared, rose up 40 2). Proper positioning confirmed saturation 95% on blood sampled tip wedged catheter. Exercise-stress excluded dynamic mitral regurgitation. Accordingly, waves were attributed diastolic dysfunction compliance.2Download 2. Invasive hemodynamics Filling pressures (pulmonary [PAWP] [RAP]) nearly equalized did not markedly increase difference (PAP) peak. second RAP. challenge, appearance (*), development exercise-induced hypertension. gradient abolished.Right overload can enhance interdependence.3 In our patient, hypertension–specific normalized increased CO, thus improving chamber filling. hemodynamic effect particularly evident unmasking presence due failure preserved ejection fraction.4,5 further escalation treatment avoided.This case highlights importance exercise-stress catheterization laboratory, time initial also follow-up monitor possible occurrence changes patients multiple potential confounders comorbidities, such sclerosis.AcknowledgmentsWe grateful Laboratori Guidotti S.p.A. providing free-of-charge software speckle-tracking analysis.Sources FundingThis funded Italian Ministry Health (Progetti di Ricerca Corrente, IRCCS). Dr received 2020 ESC Research Grant. grant “Vera Srebot” from Fondazione CNR/Regione Toscana per la Medica e Sanità Pubblica.Disclosures None.FootnotesFor Sources Funding Disclosures, see page 282.Correspondence s.[email protected]itReferences1. JL, Tedford RJ, Rosenkranz S, Palazzini M, Lang I, Guazzi Coghlan G, Chazova De Marco T. disease.Eur Respir J. 2019; 53:1801897. doi: 10.1183/13993003.01897-2018CrossrefMedlineGoogle Scholar2. Reddy YNV, El-Sabbagh A, Nishimura RA. Comparing end assessment left-sided filling pressures.JAMA Cardiol. 2018; 3:453–454. 10.1001/jamacardio.2018.0318CrossrefMedlineGoogle Scholar3. Naeije R, Badagliacca R. overloaded interdependence.Cardiovasc Res. 2017; 113:1474–1485. 10.1093/cvr/cvx160CrossrefMedlineGoogle Scholar4. Borlaug BA, RA, Sorajja P, Lam CS, Redfield MM. Exercise early fraction.Circ Fail. 2010; 3:588–595. 10.1161/CIRCHEARTFAILURE.109.930701LinkGoogle Scholar5. Kovacs Herve Barbera JA, Chaouat Chemla D, Condliffe Garcia Grünig E, Howard L, Humbert et al.. official European Respiratory Society statement: haemodynamics exercise.Eur 50:1700578. 10.1183/13993003.00578-2017CrossrefMedlineGoogle Scholar Back top Next FiguresReferencesRelatedDetails February 2021Vol Issue 2Article InformationMetrics © 2021 American Association, Inc.https://doi.org/10.1161/CIRCHEARTFAILURE.120.007554PMID: 33504158 publishedJanuary Keywordscardiac outputheart failurehypertension, pulmonaryhemodynamicsatrial pressurePDF download Advertisement SubjectsExercise TestingHeart FailureHemodynamics