Pulmonary arterial hypertension: management in specific medical conditions (RCD code: II‑1A.1)

Sylwia Iwańczyk, Tatiana Mularek-Kubzdela

Full Text:

PDF PDF

Abstract


Pulmonary arterial hypertension (PAH) is a severe and progressive disease. Due to the narrowing of the blood vessels in the lungs, pul- monary vascular resistance and pulmonary pressures increase. This results in reduced cardiac output, right heart failure and ultimately in death. Pulmonary hypertension, particularly PAH, is an independent risk factor for both peri-operative complications and post-operative mortality. The peri-operative management of patients with PAH is particularly challenging and requires a multidisciplinary approach. Appropriate patient preparation requires an accurate assessment of the severity of pulmonary hypertension, comorbidities and the type of surgery to be performed. Additionally, patients with PAH are more likely to develop an infection, particularly of the respiratory system. Infections are important risk factors for disease exacerbation, often affecting prognosis. For this reason, there is a need for effective prophylactic, diagnostic and rapid therapeutic strategies in PAH patients admitted with suspected infection. While pregnancy is not con- sidered a disease, it is associated with a significant mortality and morbidity risk in patients with PAH and therefore it is contraindicated in this group. Thus, the proper education of patients and effective contraception are necessary in order to minimize health risks. If a woman decides to maintain her pregnancy, careful monitoring, specific treatment optimization and close co-operation with an obstetrician are needed. The proper assessment of the severity of PAH and the optimization of specific treatments are crucial to improve the prognosis of PAH patients in all high-risk conditions. Additionally, the early diagnosis of high-risk conditions allows for early intensive treatment or control, which should be performed at a PAH treatment referral centre. JRCD 2018; 3 (6): 194–198

Keywords


rare disease; pulmonary arterial hypertension; surgery; infections; pregnancy

References


Galiè N, Humbert M, Vachiery J-L, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2016; 37: 67–119.

Galiè N, Manes A, Negro L, et al. A meta-analysis of randomized controlled trials in pulmonary arterial hypertension. Eur Heart J 2009; 30: 394–403.

Lajoie AC, Lauzière G, Lega J-C, et al. Combination therapy versus monotherapy for pulmonary arterial hypertension: a meta-analysis. Lancet Respir Med 2016; 4: 291–305.

Rival G, Lacasse Y, Martin S, et al. Effect of pulmonary arterial hypertension-specific therapies on health-related quality of life: a systematic review. Chest 2014;146: 686–708.

Benza RL, Miller DP, Barst RJ, et al. An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. Chest 2012;142: 448–456.

Kaw R, Pasupuleti V, Deshpande A, et al. Pulmonary hypertension: an important predictor of outcomes in patients undergoing non-cardiac surgery. Respir Med 2011;105: 619–624.

Lai H-C, Lai H-C, Wang K-Y, et al. Severe pulmonary hypertension complicates postoperative outcome of non-cardiac surgery. Br J Anaesth 2007; 99: 184–190.

Hosenpud JD, Bennett LE, Keck BM, et al. The Registry of the International Society for Heart and Lung Transplantation: seventeenth official report-2000. J Heart Lung Transplant 2000;19: 909–931.

Ishikawa S, Ohtaki A, Takahashi T, et al. Lung impairment following cardiac surgery in patients with pulmonary hypertension. J Cardiovasc Surg (Torino) 2002; 43: 7–10.

Krowka MJ, Plevak DJ, Findlay JY, et al. Pulmonary hemodynamics and perioperative cardiopulmonary-related mortality in patients with portopulmonary hypertension undergoing liver transplantation. Liver Transpl 2000; 6: 443–450.

Castro M, Krowka MJ, Schroeder DR, et al. Frequency and clinical implications of increased pulmonary artery pressures in liver transplant patients. Mayo Clin Proc 1996; 71: 543–551.

Starkel P, Vera A, Gunson B, et al. Outcome of liver transplantation for patients with pulmonary hypertension. Liver Transpl 2002; 8: 382–388.

Taura P, Garcia-Valdecasas JC, Beltran J, et al. Moderate primary pulmonary hypertension in patients undergoing liver transplantation. Anesth Analg 1996; 83: 675–680.

Forrest P. Anaesthesia and right ventricular failure. Anaesth Intensive Care 2009; 37: 370–385.

Rodseth RN, Biccard BM, Chu R, et al. Postoperative B-type natriuretic peptide for prediction of major cardiac events in patients undergoing noncardiac surgery: systematic review and individual patient meta-analysis. Anesthesiology 2013; 119: 270–283.

Price LC, Montani D, Jaïs X, et al. Noncardiothoracic nonobstetric surgery in mild-to-moderate pulmonary hypertension. Eur Respir J 2010; 35:1294–1302.

Ramakrishna G, Sprung J, Ravi BS, et al. Impact of pulmonary hypertension on the outcomes of noncardiac surgery: predictors of perioperative morbidity and mortality. J Am Coll Cardiol 2005; 45: 1691–1699.

Minai OA, Venkateshiah SB, Arroliga AC. Surgical intervention in patients with moderate to severe pulmonary arterial hypertension. Conn Med 2006; 70: 239–243.

Meyer S, McLaughlin VV, Seyfarth H-J, et al. Outcomes of noncardiac, nonobstetric surgery in patients with PAH: an international prospective survey. Eur Respir J 2013; 41:1302–1307.

Vachiery J-L. Management of patients with pulmonary hypertension undergoing surgery. [Internet]. 2016 [cited 2018 Feb 27]; available from: https://congress365.escardio.org/Search-Results?vgnextkeyword=vachiery&Years=C365YEAR2016&Medias=C365MEDIATYPEHASSLIDES#.WpTSk7ziZdh

Pilkington SA, Taboada D, Martinez G. Pulmonary hypertension and its management in patients undergoing non-cardiac surgery. Anaesthesia 2015;70: 56–70.

Wang HE, Donnelly JP, Barton D, et al. Assessing Advanced Airway Management Performance in a National Cohort of Emergency Medical Services Agencies. Ann Emerg Med 2018; pii: S0196-0644(17)31 985–6 [Epub ahead of print].

Blaise G, Langleben D, Hubert B. Pulmonary arterial hypertension: pathophysiology and anaesthetic approach. Anesthesiology 2003; 99:1415–1432.

Fischer LG, Van Aken H, Bürkle H. Management of pulmonary hypertension: physiological and pharmacological considerations for anesthesiologists. Anesth Analg 2003; 96:1603–1616.

Kwak YL, Lee CS, Park YH, et al. The effect of phenylephrine and norepinephrine in patients with chronic pulmonary hypertension. Anaesthesia 2002; 57: 9–14.

Leather HA, Segers P, Berends N, et al. Effects of vasopressin on right ventricular function in an experimental model of acute pulmonary hypertension. Crit Care Med 2002; 30: 2548–2552.

Peacock A, Ross K. Pulmonary hypertension: a contraindication to the use of {beta}-adrenoceptor blocking agents. Thorax 2010; 65: 454–455.

Olsson KM, Nickel NP, Tongers J, et al. Atrial flutter and fibrillation in patients with pulmonary hypertension. Int J Cardiol 2013;167: 2300–2305.

Sztrymf B, Souza R, Bertoletti L, et al. Prognostic factors of acute heart failure in patients with pulmonary arterial hypertension. Eur Respir J 2010; 35:1286–1293.

Kurzyna M, Zyłkowska J, Fijałkowska A, et al. Characteristics and prognosis of patients with decompensated right ventricular failure during the course of pulmonary hypertension. Kardiol Pol 2008; 66:1033–1039.

Quarck R, Nawrot T, Meyns B, et al. C-reactive protein: a new predictor of adverse outcome in pulmonary arterial hypertension. J Am Coll Cardiol 2009; 53:1211–1218.

Sharma AC. Sepsis-induced myocardial dysfunction. Shock 2007; 28: 265–269.

Weiss BM, Zemp L, Seifert B, et al. Outcome of pulmonary vascular disease in pregnancy: a systematic overview from 1978 through 1996. J Am Coll Cardiol 1998; 31:1650–1657.

Bonnin M, Mercier FJ, Sitbon O, et al. Severe pulmonary hypertension during pregnancy: mode of delivery and anaesthetic management of 15 consecutive cases. Anesthesiology 2005;102:1133–1137.

Gleicher N, Midwall J, Hochberger D, et al. Eisenmenger’s syndrome and pregnancy. Obstet Gynecol Surv 1979; 34: 721–741.

Pieper PG, Hoendermis ES. Pregnancy in women with pulmonary hypertension. Neth Heart J 2011;19: 504–508.

Olsson KM, Jais X. Birth control and pregnancy management in pulmonary hypertension. Semin Respir Crit Care Med 2013; 34: 681–688.

Thorne S, Nelson-Piercy C, MacGregor A, et al. Pregnancy and contraception in heart disease and pulmonary arterial hypertension. J Fam Plann Reprod Health Care 2006; 32: 75–81.

Mantha S, Karp R, Raghavan V, et al. Assessing the risk of venous thromboembolic events in women taking progestin-only contraception: a meta-analysis. BMJ 2012; 345: e4944.

Jaïs X, Olsson KM, Barbera JA, et al. Pregnancy outcomes in pulmonary arterial hypertension in the modern management era. Eur Respir J 2012; 40: 881–885.

Bédard E, Dimopoulos K, Gatzoulis MA. Has there been any progress made on pregnancy outcomes among women with pulmonary arterial hypertension? Eur Heart J 2009; 30: 256–265.

Terek D, Kayikcioglu M, Kultursay H, et al. Pulmonary arterial hypertension and pregnancy. J Res Med Sci 2013;18:73–76.

European medicines agency. Volibris® (ambrisentan): summary of product characteristics [Internet]. 2016; available from: www.ema.europa.eu/docs/en_GB/document_library/EPAR_-_Product_Information/human/000 839/WC500 053 065.pdf

European medicines agency. Opsumit® (macitentan): summary of product characteristics [Internet] 2016; available from: www.ema.europa.eu/docs/en_GB/document_library/EPAR_-_Product_Information/human/002 697/WC500 160 899.pdf

European medicines agency. Tracleer® (bosentan): summary of product characteristics [Internet]. 2016; available from: 46. European medicines agency. Tracleewww.ema.europa.eu/docs/en_GB/document_library/EPAR_-_Product_Information/human/000 401/WC500 041 597.pdf

Madden BP. Pulmonary hypertension and pregnancy. Int J Obstet Anesth 2009;18:156–164.

Katsuragi S, Yamanaka K, Neki R, et al. Maternal outcome in pregnancy complicated with pulmonary arterial hypertension. Circ J 2012;76:2249–2254.

Daimon A, Kamiya CA, Iwanaga N, et al. Management of pulmonary vasodilator therapy in three pregnancies with pulmonary arterial hypertension. J Obstet Gynaecol Res 2017; 43: 935–938.

Sliwa K, van Hagen IM, Budts W, et al. Pulmonary hypertension and pregnancy outcomes: data from the Registry Of Pregnancy and Cardiac Disease (ROPAC) of the European Society of Cardiology. Eur J Heart Fail 2016;18:1119–1128.

Kaźnica-Wiatr M, Leśniak-Sobelga A, Kopeć G, et al. Pregnancy in pulmonary arterial hypertension (RCD code: VII-II-1). J Rare Cardiovasc Dis 2016;2:215–219.




DOI: http://dx.doi.org/10.20418%2Fjrcd.vol3no6.320

Refbacks

  • There are currently no refbacks.
Journal of Rare Cardiovascular Diseases (JRCD)
John Paul II Hospital in Kraków, 80 Prądnicka Str., 31-202 Kraków, Poland
Phone: +48 (12) 614 33 99, +48 (12) 614 34 88 Fax: +48 (12) 614 34 88
e-mail: rarediseases@szpitaljp2.krakow.pl
Published by SoftQ sp. z o.o.
ul. Oleandry 2, 30-063 Kraków, Poland
Phone: +48 (12) 444 1650 Fax: +48 (12) 444 1659
e-mail: softq@softq.pl