Pulmonary hypertension associated with left heart diseases (clinical case)

Main Article Content

G. D. Radchenko
S. M. Kushnir
Yu. M. Sirenko

Abstract

This paper presents clinical case of pulmonary hypertension associated with left heart diseases (PH-LHD) in 63 year old woman with heart failure and preserved ejection fraction (HFpEF). The history of disease (arterial hypertension, atrial fibrillation, diabetes mellitus, acute pulmonary embolism, obesity, chronic obstructive lung disease) and results of standard investigations (no signs of thrombi in pulmonary arteries, significant enlargement of right heart and normal size of left heart, high level of calculated systolic blood pressure in pulmonary artery, high level of B-type natriuretic peptide) did not help to classify the type of pulmonary hypertension (PH). Only after right heart catheterization it was possible to state right diagnosis – post capillary combined PH-LHD. There are also discussed the latest guidelines in diagnosis and treatment of HFpEF and PH-LHD. Some considerations in favor of new step algorithm for diagnosis of HFpEF that was proposed by European Cardiology Congress in Paris (2019) were done and there was demonstrated its possible use in Ukraine. Some questions of morphological and pathogenic differences between precapillary pulmonary arterial hypertension (PAH) and post-capillary PH-LHD were discussed. Conclusion about not using of specific PAH therapy in PH-LHD patients was based on some arguments. There are elucidated some drug interventions in patients with HFpEF. Especial emphasized the necessity of risk factor and concomitant state corrections, including life style modification and non-drug treatment.

Article Details

Keywords:

pulmonary hypertension, diagnosis, treatment, left heart diseases, clinical case, guidelines

References

Albakri A. Heart failure with preserved ejection fraction: A review of clinical status and meta-analysis of diagnosis by myocardial strain and effect of medication on mortality and hospitalization // Int. Med. Care.– 2018.– Vol. 2 (2).– P. 1–12. doi: https://doi.org/10.15761/IMC.1000120.

Badesch D.B., Raskob G.E., Elliott C.G. et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry // Chest.– 2010.– Vol. 137.– P. 376–387. doi: https://doi.org/10.1007/s12471-017-0970-7.

Baumgartner H., Falk V., Bax J.J. et al. ESC Scientific Docu­­­ment Group. 2017 ESC/EACTS Guidelines for the management of valvular heart disease // Eur. Heart J.– 2017.– Vol. 38.– P. 2739–2791. doi: https://doi.org/10.1093/eurheartj/ehx391.

Baumgartner H., Falk V., Bax J.J. et al.; ESC Scientific Document Group. 2017 ESC/EACTS Guidelines for the management of valvular heart disease // Eur. Heart J.– 2017.– Vol. 38.– P. 2739–2791.

Benson L., Brittain E.L., Pugh M.E. et al. Impact of diabetes on survival and right ventricular compensation in pulmonary arterial hypertension // Pulm. Circ.– 2014.– Vol. 4.– P. 311–318. doi: https://doi.org/10.1086/675994.

Bermejo J., Yotti R., García-Orta R. et al. Sildenafil for improving outcomes in patients with corrected valvular heart disease and persistent pulmonary hypertension: a multicenter, double-blind, randomized clinical trial // Eur. Heart J.– 2018.– Vol. 39.– P. 1255–1264. doi: https://doi.org/10.1093/eurheartj/ehx700.

Berthelot E., Montani D., Algalarrondo V. et al. A clinical and echocardiographic score to identify pulmonary hypertension due to HFpEF // J. Card. Fail.– 2017.– Vol. 23.– P. 29–35. doi: https://doi.org/10.1016/j.cardfail.2016.10.002.

Bonderman D., Wexberg P., Martischnig A.M. et al. A noninvasive algorithm to exclude pre-capillary pulmonary hypertension // Eur. Respir. J.– 2011.– Vol. 37.– P. 1096–1103. doi: https://doi.org/10.1183/09031936.00089610.

Borbély A., Falcao-Pires I, van Heerebeek L. et al. // Circulation Research.– 2009.– Vol. 104.– P. 780–786. doi: https://doi.org/10.1161/CIRCRESAHA.108.193326.

Charalampopoulos A., Lewis R., Hickey P. et al. Patho­­­physiology and Diagnosis of Pulmonary Hypertension Due to Left Heart Disease // Front. Med. (Lausanne).– 2018.– Vol. 5.– P. 174. doi: https://doi.org/10.3389/fmed.2018.00174.

Cleland J.G.F., Bunting K.V., Flather M.D. et al. Beta-blockers in Heart Failure Collaborative Group. Beta-blockers for heart failure with reduced, mid-range, and preserved ejection fraction: an individual patient-level analysis of double-blind randomized trials // Eur. Heart J.– 2018.– Vol. 39.– P. 26–35. doi: https://doi.org/10.1093/eurheartj/ehx564.

D’Alto M., Romeo E., Argiento P. et al. Echocardiographic prediction of pre-versus postcapillary pulmonary hypertension // J. Am. Soc. Echocardiogr.– 2015.– Vol. 28.– P. 108–115. doi: https://doi.org/10.1016/j.echo.2014.09.004.

Delgado J.F., Conde E., Sanchez V. et al. Pulmonary vascular remodeling in pulmonary hypertension due to chronic heart failure // Eur. Heart Fail.– 2005.– Vol. 7.– P. 1011–1016. doi: https://doi.org/10.1016/j.ejheart.2004.10.021.

Fayyaz A.U., Edwards W.D., Maleszewski J.J. et al. Global pulmonary vascular remodeling in pulmonary hypertension associated with heart failure and preserved or reduced ejection fraction // Circulation.– 2018.– Vol. 137.– P. 1796–1810.

Franssen C., Paulus W. Normal resting pulmonary artery wedge pressure: a diagnostic trap for heart failure with preserved ejection fraction // Eur. J. Heart Failure.– 2015.– Vol. 17.– P. 132–134. doi:https://doi.org/10.1002/ejhf.225.

Friedman S.E., Andrus B.W. Obesity and pulmonary hypertension: a review of pathophysiologic mechanisms // J. Obes.– 2012.– Vol. 2012.– P. 505274. doi: https://doi.org/10.1155/2012/505274.

Galiè N., Barberà J.A., Frost A.E. et al., for the AMBITION Investigators Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension // New Engl. J. Med.– 2015.– Vol. 373.– P. 834–844. doi: https://doi.org/10.1056/NEJMoa1413687.

Galiè N., Humbert M., Vachiery J.L. et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension // Eur. Respir. J.– 2015.– Vol. 46.– P. 903–975. doi: https://doi.org/10.1183/13993003.01032-2015.

Galie N., Manes A., Dardi F., Palazzini M. Aiming at the appropriate target for the treatment of pulmonary hypertension due to left heart disease // Eur. Heart J.– 2018.– Vol. 39.– P. 1265–1268. doi: https://doi.org/10.1093/eurheartj/ehx751.

Galie N., Manes A., Palazzini M. The difficult diagnosis of pulmonary vascular disease in heart failure // Eur. Respir. J.– 2016.– Vol. 48.– P. 311–314. doi: https://doi.org/10.1183/13993003.00854-2016.

Gerges M., Gerges C., Lang I.M. How to define pulmonary hypertension due to left heart disease // Eur. Respir. J.– 2016.– Vol. 48.– P. 553–555. doi: https://doi.org/10.1183/13993003.00432-2016.

Haass M., Kitzman D.W., Anand I.S. et al. Body mass index and adverse cardiovascular outcomes in heart failure patients with preserved ejection fraction: results from the Irbesartan in Heart Failure with Preserved Ejection Fraction (I-PRESERVE) trial // Circ. Heart Fail.– 2011.– Vol. 4 (3).– P. 324–331. doi: https://doi.org/10.1161/CIRCHEARTFAILURE.110.959890.

Hanipah Z.N., Mulcahy M.J., Sharma G. et al. Bariatric surgery in patients with pulmonary hypertension // Surg. Obes. Relat. Dis.– 2018.– Vol. 14.– P. 1581–1586. doi: https://doi.org/10.1016/j.soard.2018.07.015.

Hummel S.L., Seymour E.M., Brook R.D. et al. Low-sodium dietary approaches to stop hypertension diet reduces blood pressure, arterial stiffness, and oxidative stress in hypertensive heart failure with preserved ejection fraction // Hypertension.– 2012.– Vol. 60 (5).– P. 1200–1206. doi: https://doi.org/10.1161/HYPERTENSIONAHA.112.202705.

Hunt S.A. American College of Cardiology, American Heart Association Task Force on Practice Guidelines (Writing Committee to Update the 2001 Guidelines for the Evaluation and Management of Heart Failure). ACC/AHA 2005 guideline update for the diagnosis and management of chronic heart failure in the adult: a report of the American college of Cardiology / American heart association task force on practice guidelines (writing committee to update the 2001 guidelines for the evaluation and management of heart failure) // J. Amer. Coll. Cardiology.– 2005.– Vol. 46 (6).– P. e1–e82. doi:https://doi.org/10.1016/j.jacc.2005.08.022.

Jacobs W., Konings T.C., Heymans M.W. et al. Noninvasive identification of left-sided heart failure in a population suspected of pulmonary arterial hypertension // Eur. Respir. J.– 2015.– Vol. 46.– P. 299–302. doi: https://doi.org/10.1183/09031936.00202814.

Kenchaiah S., Evans J.C, Levy D. et al. Obesity and the risk of heart failure // The New Engl. J. Medicine.– 2002.– Vol. 347 (5).– P. 305–313. doi: https://doi.org/10.1056/NEJMoa020245.

Lam C.S., Lyass A., Kraigher-Krainer E. et al. Cardiac dysfunction and non-cardiac dysfunction as precursors of heart failure with reduced and preserved ejection fraction in the community // Circulation.– 2011.– Vol. 124 (1).– P. 24–30. doi: https://doi.org/10.1161/CIRCULATIONAHA. 110.979203.

Lang I., Palazzini M. The burden of comorbidities in pulmonary arterial hypertension // Eur. Heart J. 2019.– Vol. 21 (Suppl. K).– P. K21–K28. doi: https://doi.org/10.1093/eurheartj/suz205.

LeVarge B., Pomerantsev E., Channick R. Reliance on end-expiratory wedge pressure leads to misclassification of pulmonary hypertension // Eur. Respiratory J.– 2014.– Vol. 44.– P. 425–434. doi: https://doi.org/10.1183/09031936.00209313.

Loh E., Stamler J.S., Hare J.M. et al. Cardiovascular effects of inhaled nitric oxide in patients with left ventricular dysfunction // Circulation.– 1994.– Vol. 90.– P. 2780–2785. doi: https://doi.org/10.1161/01.cir.90.6.2780.

Lund L., Claggett B., Liu J. et al. Heart failure with mid‐range ejection fraction in CHARM: characteristics, outcomes and effect of candesartan across the entire ejection fraction spectrum // Eur. J. Heart Failure.– 2018.– Vol. 20.– P. 1230–1239. doi:https://doi.org/10.1002/ejhf.1149.

Lund L.H., Donal E., Oger E. et al.; KaRen Investigators. Association between cardiovascular vs. non-cardiovascular co-morbidities and outcomes in heart failure with preserved ejection fraction // Eur. J. Heart Fail.– 2014.– Vol. 16.– P. 992–1001. doi: https://doi.org/10.1002/ejhf.137.

Macdougall I.C., Canaud B., de Francisco A.L. et al. Beyond the cardiorenal anaemia syndrome: recognizing the role of iron deficiency // Eur. J. Heart Fail.– 2012.– Vol. 14.– P. 882–886. doi: https://doi.org/10.1093/eurjhf/hfs056.

Mclaughlin V., Galie N., Barbera J. et al. A Comparison of Characteristics and Outcomes of Patients with Atypical and Classical Pulmonary Arterial Hypertension from the AMBITION Trial // Amer. J. Respiratory Critical Care Medicine.– 2015.– Vol. 191.– P. A2196 https://www.atsjournals.org/doi/abs/10.1164/ajrccm-conference.2015.191.1_Meeting Abstracts. A2196.

Opitz C., Hoeper M., Gibbs S. et al. Pre-Capillary, Combined, and Post-Capillary Pulmonary Hypertension A Pathophysiological Continuum // J. Amer. Coll. Cardiology.– 2016.– Vol. 68.– P. 368–378. doi: https://doi.org/10.1016/j.jacc.2016.05.047.

Opotowsky A.R., Ojeda J., Rogers F. et al. A simple echocardiographic prediction rule for hemodynamics in pulmonary hypertension // Circ. Cardiovasc. Imaging.– 2012.– Vol. 5.– P. 765–775 doi: https://doi.org/10.1161/CIRCIMAGING.112.976654.

Paulus W.J., Tschöpe C., Sanderson J.E. et al. How to diagnose diastolic heart failure: a consensus statement on the diagnosis of heart failure with normal left ventricular ejection fraction by the Heart Failure and Echocardiography Associations of the European Society of Cardiology // Eur. Heart J.– 2007.– Vol. 28.– P. 2539–2550. doi: https://doi.org/10.1093/eurheartj/ehm037.

Pieske B., Tschöpe C., de Boer R.A. et al. How to diagnose heart failure with preserved ejection fraction: the HFA-PEFF diagnostic algorithm: a consensus recommendation from the Heart Failure Association (HFA) of the European Society of Cardiology (ESC) // Eur. Heart J.– 2019.– Vol. 40 (40).– P. 3297–3317. doi: https://doi.org/10.1093/eurheartj/ehz641.

Poms A.D., Turner M., Farber H.W. et al. Comorbid conditions and outcomes in patients with pulmonary arterial hypertension: a REVEAL registry analysis // Chest.– 2013.– Vol. 144.– P. 169–176. doi: https://doi.org/10.1378/chest.11-3241.

Ponikowski P., Voors A.A., Anker S.D. et al.; Authors/Task Force Members. 2016 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure The Task Force for the diagnosis and treatment of acute and chronic heart failure of the European Society of Cardiology (ESC) Developed with the special contribution of the Heart Failure Association (HFA) of the ESC // Eur. Heart J.– 2016.– Vol. 37.– P. 2129–2200. doi: https://doi.org/10.1093/eurheartj/ehw128.

Reddy Y.N.V., Carter R.E., Obokata M. et al. A simple, evidence-based approach to help guide diagnosis of heart failure with preserved ejection fraction // Circulation.– 2018.– Vol. 138.– P. 861–870. doi: https://doi.org/10.1161/CIRCULATIONAHA.118.034646.

Rijzewijk L.J., van der Meer R.W., Smit J.W. et al. Myocardial steatosis is an independent predictor of diastolic dysfunction in type 2 diabetes mellitus // J. Amer. Coll. Cardiology.– 2008.– Vol. 52 (22).– P. 1793–1799. doi: https://doi.org/10.1016/j.jacc.2008.07.062.

Sheu E.G., Channick R., Gee D.W. Improvement in severe pulmonary hypertension in obese patients after laparoscopic gastric bypass or sleeve gastrectomy // Surg. Endosc.– 2016.– Vol. 30.– P. 633–637. doi: https://doi.org/10.1007/s00464-015-4251-5.

Strange G., Playford D., Stewart S. et al. Pulmonary hypertension: prevalence and mortality in the Armadale echocardiography cohort // Heart.– 2012.– Vol. 98 (24).– P. 1805–1811. doi: https://doi.org/10.1136/heartjnl-2012-301992.

Tian N., Moore R.S., Braddy S. et al. Interactions between oxidative stress and inflammation in salt-sensitive hypertension // Am. J. Physiol. Heart Circ. Physiol.– 2007.– Vol. 293.– P. H3388–H3395. doi: https://doi.org/10.1152/ajpheart.00981.200.

Vachiéry J., Tedford R., Rosenkranz S. et al. Number 9 in the series «Proceedings of the 6th World Symposium on Pulmonary Hypertension» / Ed. N. Galiè, V.V. McLaughlin, L.J. Rubin, G. Simonneau // ERJ Express. 2018; in press [doi: https://doi.org/10.1183/13993003.01897-2018].

Vachiery J.L., Adir Y., Barbera J.A. et al. Pulmonary hypertension due to left heart disease // J. Am. Coll. Cardiol.– 2013.– Vol. 62.– P. D100–D108.

Voelkel M.A., Wynne K.M., Badesch D.B. et al. Hyperuric­­emia in severe pulmonary hypertension // Chest.– 2000.– Vol. 117 (1).– P. 19–24. doi: https://doi.org/10.1378/chest.117.1.19.

Weatherald J., Huertas A., Boucly A. et al. Association between BMI and obesity with survival in pulmonary arterial hypertension // Chest.– 2018.– Vol. 154.– P. 872–881. doi: https://doi.org/10.1016/j.chest.2018.05.006.

Weatherald J., Huertas A., Boucly A. et al. Is there an obesity paradox in pulmonary arterial hypertension? // Eur. Respir J.– 2017.– Vol. 50.– P. PA3521. doi: https://doi.org/10.1183/1393003.congress-2017.PA3521.

Whitaker M.E., Nair V., Sinari S. et al. Diabetes mellitus associates with increased right ventricular afterload and remodeling in pulmonary arterial hypertension // Am. J. Med.– 2018.– Vol. 131.– P. 702.e7–702.e13. doi: https://doi.org/10.1016/j.amjmed.2017.12.046.

Wirth A., Kroger H. Improvement of left ventricular morphology and function in obese subjects following a diet and exercise program // Intern. J. Obesity.– 1995.– Vol. 19 (1).– P. 61–66. PMID: 7719394.

Wong C., Marwick T. Obesity cardiomyopathy: pathogenesis and pathophysiology // Nature Clin. Practice Cardiovasc. Medicine.– 2007.– Vol. 4 (8).– P. 436–443. doi: https://doi.org/10.1038/ncpcardio0943.

Wong C.Y., Byrne N.M., O’Moore-Sullivan T. et al. Effect of weight loss due to lifestyle intervention on subclinical cardiovascular dysfunction in obesity (body mass index > 30 kg/m2) // Amer. J. Cardiology.– 2006.– Vol. 98 (12).– P. 1593–1598. doi: https://doi.org/10.1016/j.amjcard.2006.07.037.

Wong C.Y., Moore-Sullivan T.O., Leano R. et al. Alterations of left ventricular myocardial characteristics associated with obesity // Circulation.– 2004.– Vol. 110 (19).– P. 3081–3087 doi: https://doi.org/10.1161/01.CIR.0000147184.13872.0F.

Zharikov S.I., Swenson E.R., Lanaspa M. et al. Could uric acid be a modifiable risk factor in subjects with pulmonary hypertension? // Medical Hypotheses.– 2010.– Vol. 74 (6).– P. 1069–1074. doi: https://doi.org/10.1016/j.mehy.2009.12.023.

Zimpfer D., Zrunek P., Roethy W. et al. Left ventricular assist devices decrease fixed pulmonary hypertension in cardiac transplant candidates // J. Thorac. Cardiovasc. Surg.– 2007.– Vol. 133.– P. 689–695. doi: https://doi.org/10.1016/j.jtcvs.2006.08.104.