An update on the diagnosis and treatment of pediatric pulmonary hypertension

References

• Humbert M, Guignabert C, Bonnet S, et al., Pathology and pathobiology of pulmonary hypertension: state of the art and research perspectives. Eur Respir J. 2019;53(1): 1801887.
   Web of Science ®Google Scholar
• Hansmann G. Pulmonary Hypertension in Infants, Children, and Young Adults. J Am Coll Cardiol. 2017;69(20):2551–2569.
   PubMed Web of Science ®Google Scholar
• Ivy DD, Abman SH, Barst RJ, et al. Pediatric pulmonary hypertension. J Am Coll Cardiol. 2013;62(25):D117–126.
   PubMed Web of Science ®Google Scholar
• Barst RJ, Ertel SI, Beghetti M, et al. Pulmonary arteriel hypertension. A comparison between children and adults. Eur Respir J. 2011;37:665–677.
   PubMed Web of Science ®Google Scholar
• Hansmann G, Apitz C, Abdul Khalig H, et al. Executive summary. Heart. 2016;102(Suppl 2):86–100.
   Google Scholar
• Rosenzweig EB, Abman SH, Adatia I, et al. Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management. Eur Respir J. 2019;53:1-18.
   Web of Science ®Google Scholar
• Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019;53(1):1801913.
   Web of Science ®Google Scholar
• Galie N, Channick RN, Frantz RP, et al. Risk stratification and medical therapy of pulmonary arterial hypertension. Eur Respir J. 2019;53(1):1801889.
   Web of Science ®Google Scholar
• Zijlstra WM, Douwes JM, Rosenzweig EB, et al., Survival differences in pediatric pulmonary arterial hypertension: clues to a better understanding of outcome and optimal treatment strategies. J Am Coll Cardiol. 2014;63(20): 2159–2169.
   PubMed Web of Science ®Google Scholar
• Montani D, Chaumais MC, Guignabert C, et al. Targeted therapies in pulmonary arterial hypertension. Pharmacol & Therapeutics. 2014;141:172–191.
   PubMed Web of Science ®Google Scholar
• Hatano S, Strasser T, Primary pulmonary hypertension. Report on a WHO meeting. Geneva, World Health Organization 1975
   Google Scholar
• Kovacs G, Berghold A, Scheidl S, et al. Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review. Eur Respir J. 2009;34(4):888–894.
   PubMed Web of Science ®Google Scholar
• Maron BA, Brittain EL, Choudhary E, et al. Redefining pulmonary hypertension. Lancet Respir Med. 2018;6(3):168–170.
   PubMed Web of Science ®Google Scholar
• Frank BS, Ivy DD. Diagnosis, Evaluation and treatment of pulmonary hypertension in children. Children. 2018;5(4):44.
   Google Scholar
• Rich S, Kaufmann E, Levy PS. The effect of high doses of calcium- channel blockers on survival in primary pulmonary hypertension. N Engl J Med. 1992;327(2):76–81.
   PubMed Web of Science ®Google Scholar
• Sitbon O, Humbert M, Jais X, et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation. 2005;111(23):3105–3111.
   PubMed Web of Science ®Google Scholar
• Hemnes AR, Trammell AW, Archer SL, et al. Peripheral blood signature of vasodilator-responsive pulmonary arterial hypertension. Circulation. 2015;131(4):401–409.
   PubMed Web of Science ®Google Scholar
• Hemnes AR, Zhao M, West J, et al. Critical genomic networks and vasoreactive variants in idiopathic pulmonary arterial hypertension. Am J Respir Crit Care Med. 2016;194(4):464–475.
   PubMed Web of Science ®Google Scholar
• Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25):D34–41.
   PubMed Web of Science ®Google Scholar
• Del Cerro Marin MJ, Sabate RA, Rodriquez OA, et al. Assessing pulmonary hypertensive vascular disease in childhood. Data from Spanish registry. Am J Respir Crit Care Med. 2014;190(12):1421–1429.
   PubMed Web of Science ®Google Scholar
• Van Loon RL, Roofthoft MT, Hillege HL, et al. Pediatric pulmonary hypertension in the Netherlands. Epidemiology and characterization during the period 1991-2005. Circulation. 2011;124:1755–1764.
   PubMed Web of Science ®Google Scholar
• Moledina S, Hislop AA, Foster H, et al. Childhood idiopathic pulmonary arterial hypertension: A national cohort study. Heart. 2010;96(17):1401–1406.
   PubMed Web of Science ®Google Scholar
• Li L, Jick S, Breitenstein S, et al. Pulmonary arterial hypertension in the USA: an epidemiological study in a large insured pediatric population. Pulm Circ. 2017;7(1):126–136.
   PubMed Web of Science ®Google Scholar
• Fraisse A, Jais X, Schlein JM, et al. Characteristics and prospective 2 year follow up of children with pulmonary hypertension in France. Arch Cardiovasc Dis. 2010;103(2):66–74.
   PubMed Web of Science ®Google Scholar
• Koestenberger M, Ravekes W, Everett AD, et al. Right ventricular function in infants, children and adolescents: reference values of the tricuspid annular plane systolic excursion (TAPSE) in 640 healthy patients and calculation of z score values. J Am Soc Echocardiogr. 2009;22(6):715–719.
   PubMed Web of Science ®Google Scholar
• Cevik A, Kula S, Olgunturk R, et al. Assessment of pulmonary arterial hypertension and vascular resistance by measurement of pulmonary arterial flow velocity curve in absence of measurable tricuspid regurgitation velocity in childhood congenital heart disease. Pediatr Cardiol. 2013;34(3):646–655.
   PubMedGoogle Scholar
• Jone PN, Hinzman J, Wagner BD, et al. Right ventricular to left ventricular diameter ratio at end-systole in evaluating outcomes in children with pulmonary hypertension. J Am Soc Echocardiogr. 2014;27(2):172–178.
   PubMed Web of Science ®Google Scholar
• Del Cerro MJ, Moledina S, Haworth SG, et al. Cardiac catheterization in children with pulmonary hypertensive vascular disease. Consensus statement from the pulmonary vascular research institute, pediatric and congenital heart disease task forces. Pulm Circ. 2016;6:118–125.
   PubMed Web of Science ®Google Scholar
• Takatsuki S, Nakayama T, Ikehara S, et al. Pulmonary Arterial capacitance index is a strong predictor for adverse outcome in children with idiopathic and heritable pulmonary arterial hypertension. J Pedatr. 2017;180:75–79.
   PubMed Web of Science ®Google Scholar
• Hansman G, Koestenberger M, Alastalo TP, et al. updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension: The European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, ESPR and ISHLT. J Heart Lung Transpl. 2019;2019(9):879–901.
   Google Scholar
• Apitz C, Hansman G, Schranz D. Hemodynamic assessment and acute pulmonary vasoreactivity testing in the evaluation of children with pulmonary vascular disease. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European pediatric pulmonary vascular disease network, endorsed by ISHLT and DGPK. Heart. 2016;102(supp12):ii 23–29.
   Google Scholar
• Matsuura H. Cardiac catheterization in children with pulmonary arterial hypertension. Pediatr Int. 2017;59(1):3–9.
   PubMed Web of Science ®Google Scholar
• Barst RJ, Maislin G, Fishman AP. Vasodilator therapy for primary pulmonary hypertension in children. Circulation. 1999;99(9):1197–1208.
   PubMed Web of Science ®Google Scholar
• Sitbon O, Humbert M, Jais X, et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation. 2005;111(23):3105–3111.
   PubMed Web of Science ®Google Scholar
• Douwes JM, Humpl T, Bonnet D, et al. Topp investigators. Acute vasodilator response in pediatric pulmonary arterial hypertension: current clinical practice from the TOPP registry. J Am Coll Cardiol. 2016;67(11):1312–1323.
   PubMed Web of Science ®Google Scholar
• Latus H, Kuehne T, Beerbaum P, et al. Cardiac MR ant CT imaging in children with suspected or confirmed pulmonary hypertension/pulmonary hypertensive vascular disease. Expert consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension. The European pediatric pulmonary vascular disease network, endorsed ISHLT and DGPK. Heart. 2016;102(Suppl 2):ii 14–22.
   Google Scholar
• Pektas A, Olgunturk R, Cevik A, et al. Magnetic resonance imaging in pediatric pulmonary hypertension. Texas Heart Inst J. 2015;42(3):209–216.
   PubMed Web of Science ®Google Scholar
• Swift AJ, Capaner D, Johns C, et al. Magnetic resonance imaging in the prognostic evaluation of patients with pulmonary arterial hypertension. Am J Respir Crit Care Med. 2017;196(2):228–239.
   PubMed Web of Science ®Google Scholar
• Lammars AE, Apitz C, Zartner P, et al. Diagnostic, monitoring and outpatient care in children with suspected pulmonary hypetension/pediatric pulmonary hypertensive disease. Expert consensus statement on the diagnostic and treatment of pediatric pulmonary hypertension. The European pediatric pulmonary vascular disease network, endorsed by ISHLT and DGPK. Heart. 2016;102(suppl2):ii 1–13.
   Google Scholar
• Ten Kate CA, Tibboel D, Kraemer US. B-type natriuretic peptide as a parameter for pulmonary hypertension in children. A systematic review. Eur J Pediatr. 2015;174(10):1267–1275.
   PubMed Web of Science ®Google Scholar
• Ploegstra MJ, Zijlstra WM, Douwes JM, et al. Prognostic factors in paediatric pulmonary arterial hypertension: a systematic review and meta-analysis. Int J Cardiol. 2015;184:198–207.
   PubMed Web of Science ®Google Scholar
• Kula S, Canbeyli F, Atasayan V, et al. A retrospective study on children with pulmonary arterial hypertension: a single center experience. Anatol J Cardiol. 2018;20(1):41–47.
   PubMed Web of Science ®Google Scholar
• Rabinovitch M, Guignabert C, Humbert M, et al. Inflammation and immunity in the pathogenesis of pulmonary arterial hypertension. Circ Res. 2014;115(1):165–175.
   PubMed Web of Science ®Google Scholar
• Nicolls MR, Voelkel NF. The roles of immunity in the prevention and evolution of pulmonary arterial hypertension. Am J Respir Crit Care Med. 2017;195(10):1292–1299.
   PubMed Web of Science ®Google Scholar
• Yin J, You S, Liu H, et al. Role of P2X7R in the development and progression of pulmonary hypertension. Respir Res. 2017;18(1):127–137.
   PubMedGoogle Scholar
• Parpaleix A, Amsellem V, Houssaini A, et al. Role of interleukin-1 receptor 1/MyD88 signalling in the development and progression of pulmonary hypertension. Eur Rep J. 2016;48(2):470–483.
   PubMed Web of Science ®Google Scholar
• Zhang R, Wang XJ, Zhang HD, et al. Profiling nitric oxide metabolites in patients with idiopathic pulmonary arterial hypertension. Eur Respir J. 2016;48(5):1386–1395.
   PubMed Web of Science ®Google Scholar
• Andruska A, Spiekerkoetter E. Consequences of BMPR2 deficiency in the pulmonary vasculature and beyond: contribution to pulmonary arterial hypertension. Int J Mol Sci. 2018;19(9):2499–2533.
   Web of Science ®Google Scholar
• Evans JD, Girerd B, Montani D, et al. BMPR2 mutations and survival in pulmonary arterial hypertension: an individual participant data meta-analysis. Lancet Respir Med. 2016;4(2):129–137.
   PubMed Web of Science ®Google Scholar
• Orriols M, Gomez-Puerto MC, Tin Dijke P. BMP type II receptor as a therapeutic target in pulmonary arterial hypertension. Cell Mol Life Sci. 2017;74(16):2979–2995.
   PubMed Web of Science ®Google Scholar
• Ali IH, Brazil DP. Bone morphogenetic proteins and their antagonists: current and emerging clinical uses. Br J Pharmacol. 2014;171(15):3620–3632.
   PubMed Web of Science ®Google Scholar
• Levy M, Eyries M, Szezepanski I, et al. Genetic analysis in a cohort of children with pulmonary hypertension. Eur Respir J. 2016;48(4):1118–1126.
   PubMed Web of Science ®Google Scholar
• Galie N, Humbert M, Vachiery JL, et al. ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J. 2015;46(4):903–975.
   PubMed Web of Science ®Google Scholar
• Patel SS, Fernie JC, Taylor AL, et al. Evaluation of predictive models for six-minute walk test among children with pulmonary hypertension. Int J Cardiol. 2017;227:393–398.
   PubMed Web of Science ®Google Scholar
• Hilgendorff A, Apitz C, Bonnet D, et al. Pulmonary hypertension associated with acute or chronic lung disease in the preterm and term neonate and infant. The European paediatric pulmonary vascular disease network, endorsed by ISHLT and DGPK. Heart. 2016;102(Suppl 2):ii 49–56.
   Google Scholar
• Barst RJ, Gibbs JS, Ghofrani HA, et al. Update evidence-based treatment algorithm in pulmonary arterial hypertension. J Am Col Cardiol. 2009;54(Suppl 1):578–584.
   Web of Science ®Google Scholar
• Lador F, Sekarski N, Beghetti M. Treating pulmonary hypertension in pediatrics. Expert Opin Pharmacother. 2015;16(5):1–16.
   PubMed Web of Science ®Google Scholar
• Tuder RM, Cool CD, Geraci MW, et al. Prostacyclin synthase expression is decreased in lungs from patients with severe pulmonary hypertension. Am J Respir Crit Care Med. 1999;159(6):1925–1932.
   PubMed Web of Science ®Google Scholar
• Dadlani GH, Sosa P, Cobb H, et al. Pediatric pulmonary hypertension: diagnosis and management. Curr Opin Cardiol. 2016;31(1):78–87.
   PubMed Web of Science ®Google Scholar
• Barst RJ, Rubin LJ, Long WA, et al. Primary pulmonary hypertension study group. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med. 1996;334(5):296–301.
   PubMed Web of Science ®Google Scholar
• Siehr SL, Ivy DD, Miller Reed K, et al. Children with pulmonary arterial hypertension and prostanoid therapy: long-term hemodynamics. J Heart Lung Transplant. 2013;32:546–552.
   PubMed Web of Science ®Google Scholar
• Murali M, Chakinala MD, Jeremy P, et al. Transition from paranteral to oral treprostinil in pulmonary arterial hypertension. J Heart Lung Transplant. 2017;36(2):193–201.
   PubMed Web of Science ®Google Scholar
• Bourge RC, Waxman AB, Gomberg-Maitland M, et al. Treprostinil administered to treat pulmonary arterial hypertension using a fully implantable programmable intravascular delivery system: results of the DelIVery for PAH trial. Chest. 2016;150(1):27–34.
   PubMed Web of Science ®Google Scholar
• Waxman AB, Mc Eldery HT, Gomberg-Maitland M, et al. Totaly implantable IV treprostinil therapy in pulmonary hypertension: assessment of the implantation procedure. Chest. 2017;152(6):1128–1134.
   PubMed Web of Science ®Google Scholar
• Levy M, Del Cerro MJ, Nadaud S, et al. Safety, efficacy and management of subcutaneous treprostinil infusion in the treatment of severe pediatric pulmonary hypertension. Int J Cardiol. 2018;264:153–157.
   PubMed Web of Science ®Google Scholar
• Tanabe N, Ikeda S, Tahara N, et al. Efficacy and safety of an orally administered selective prostacyclin receptor agonist, Selexipag in Japanese patients with pulmonary arterial hypertension. Circ J. 2017;81(9):1360–1407.
   PubMed Web of Science ®Google Scholar
• Gallotti R, Drogalis-Kim DE, Satou G, et al. Single- center experience using selexipag in a pediatric population. Pediatr Cardiol. 2017;38(7):1405–1409.
   PubMed Web of Science ®Google Scholar
• Vignon-Zellweger N, Heiden S, Myauchi T, et al. Endothelin and endothelin receptors in the renal and cardiovascular system. Life Sci. 2012;91(13–14):490–500.
   PubMed Web of Science ®Google Scholar
• Rubin LJ, Badesch DB, Barst RJ, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med. 2002;346(12):896–903.
   PubMed Web of Science ®Google Scholar
• Beghetti M. Bosentan in pediatric patients with pulmonary arterial hypertension. Curr Vasc Pharmacol. 2009;7(2):225–233.
   PubMed Web of Science ®Google Scholar
• Hislop AA, Moledina S, Foster H, et al. Long-term efficacy of bosentan in treatment of pulmonary arterial hypertension in children. Eur Respir J. 2011;38(1):70–77.
   PubMed Web of Science ®Google Scholar
• Beghetti M, Haworth SG, Bonnet D, et al. Pharmacokinetic and clinical profile of a novel formulation of bosentan in children with PAH: the FUTURE-1 study. Br J Clin Pharmacol. 2009;68(6):948–955.
   PubMed Web of Science ®Google Scholar
• Berger RMF, Gehin M, Beghetti M, et al. Future-3 Investigators. A bosentan pharmacokinetic study to investigate dosing regimens in paediatric patients with pulmonary arterial hypertension: FUTURE-3. Br J Clin Pharmacol. 2017;83(8):1734–1744.
   PubMed Web of Science ®Google Scholar
• Gatzoulis MA, Beghetti M, Galie N, et al. Longer term bosentan therapy improves functional capacity in Eisenmenger syndrome: results of the BREATHE-5 open label extension study. Int J Cardiol. 2008;127(1):27–32.
   PubMed Web of Science ®Google Scholar
• Kuang HY, Wu YH, Yi QJ, et al. The efficiency of endothelin receptor antagonist bosentan for pulmonary arterial hypertension associated with congenital heart disease. A systematic review and meta analysis. Medicine (Baltimore). 2018;97:10–19.
   Web of Science ®Google Scholar
• Keating MG. Macitentan: A review in pulmonary arterial hypertension. Am J Cardiovasc Drugs. 2016;16(6):453–460.
   PubMed Web of Science ®Google Scholar
• Herbert S, Gin-Sing W, Howard L, et al. Early experience of macitentan for pulmonary arterial hypertension in adult congenital heart disease. Heart Lung Circ. 2017;26(10):1113–1116.
   PubMed Web of Science ®Google Scholar
• Gatzoulis MA, Landzberg M, Beghetti M, et al. Evaluation of macitentan in patients with Eisenmenger syndrome: results from the randomized, controlled MAESTRO study. Circulation. 2019;139(1):51–63.
   PubMed Web of Science ®Google Scholar
• Kiely D, Rosenkranz S, Galie N, et al. Effects of macitentan on right ventricle remodelling in pulmonary arterial hypertension- results from the REPAIR study interim analysis. Thorax. 2019;741(Suppl 2):A 154.
   Google Scholar
• Takatsuki S, Rosenzweig EB, Zuckerman W, et al. Clinical safety, pharmacokinetics, and efficacy of ambrisentan therapy in children with pulmonary arterial hypertension. Pediatr Pulmonol. 2013;48(1):27–34.
   PubMed Web of Science ®Google Scholar
• Tsihlis ND, Oustwani CS, Vavra AK, et al. Nitric oxide inhibits vascular smooth muscle cell proliferation and neointimal hyperplasia by increasing the ubiquitination and degradation of Ubc H10. Cell Biochem Biophys. 2011;60(1–2)):89–97.
   PubMed Web of Science ®Google Scholar
• Galie N, Ghofrani HA, Torbicki A, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med. 2005;353(20):2148–2157.
   PubMed Web of Science ®Google Scholar
• Barst RJ, Ivy DD, Gaitan G, et al. A randomized double- blind, placebo- controlled, dose-ranging study of oral sildenafil citrate in treatment- naive children with pulmonary arterial hypertension. Circulation. 2012;125(2):324–334.
   PubMed Web of Science ®Google Scholar
• Barst RJ, Beghetti M, Pulido T, et al. STARTS-2 investigators. STARTS-2: long-term survival with oral sildenafil monotherapy in treatment-naive pediatric pulmonary arterial hypertension. Circulation. 2014;129(19):1914–1925.
   PubMed Web of Science ®Google Scholar
• Galie N, Brundage BH, Ghofrani HA, et al. On behalf of the pulmonary arterial hypertension and response to tadalafil (PHIRST) study group. Circulation. 2009;119(22):2894–2903.
   PubMed Web of Science ®Google Scholar
• Qudiz RJ, Brundage BH, Galie N, et al. For the PHIRST study group. Tadalafil for treatment of pulmonary arterial hypertension: a double blind 52 week in controlled extension study. J Am Coll Cardiol. 2012;60(8):768–774.
   PubMed Web of Science ®Google Scholar
• Takatsuki S, Calderbank M, Ivy DD. Initial experience with tadalafil in pediatric pulmonary arterial hypertension. Pediatr Cardiol. 2012;33(5):683–688.
   PubMed Web of Science ®Google Scholar
• Yamazaki H, Kobayashi N, Taketsuma M, et al., Safety and effectiveness of tadalafil in pediatric patients with pulmonary arterial hypertension: a sub group analysis based on Japan post- marketing surveillance. Curr Med Res Opin. 2017;33(12): 2241–2249.
   PubMed Web of Science ®Google Scholar
• Shiva A, Shiran M, Rafati M, et al. Oral tadalafil in children with pulmonary arterial hypertension. Drug Res. 2016;66:7–10.
   PubMed Web of Science ®Google Scholar
• Ghofrani HA, Galie N, Grimminger F, et al. Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med. 2013;369(4):330–340.
   PubMed Web of Science ®Google Scholar
• Rosenkranz S, Ghofrani HA, Beghetti M, et al. Riociguat for pulmonary arterial hypertension associated with congenital heart disease. Heart. 2015;101(22):1792–1799.
   PubMed Web of Science ®Google Scholar
• Spreeman T, Bertram H, Happel CM, et al. First-in-child use of the oral soluble guanylate cyclase stimulator riociguat in pulmonary arterial hypertension. Pulm Circ. 2018;8(1):204589321774312.
   Web of Science ®Google Scholar
• Segura- Ibarra V, Suhong W, Hassan N, et al. Nanotherapeutics for treatment of pulmonary arterial hypertension. Front Physiol. 2018;9:890–906.
   PubMed Web of Science ®Google Scholar
• Li HH, Hsu HH, Chang GJ, et al., Prostanoid EP4 agonist L-902,688 activates PPARγ and attenuates pulmonary arterial hypertension. Am J Physiol Lung Cell Mol Physiol. 2018;314(3): L349–L359.
   PubMed Web of Science ®Google Scholar
• Scott TE, Kemp-Harper BK, Hobbs AJ. Inflammasomes: a novel therapeutic target in pulmonary hypertension? Brit J Pharm. 2019;176:1880-1896.
   PubMed Web of Science ®Google Scholar
• Tuncer GO, Olgunturk R, Pektas A, et al. The role of inflammatory biomarkers in congenital heart disease associated pulmonary hypertension in children. Cardiol Young. 2017;27(2):255–260.
   PubMed Web of Science ®Google Scholar
• Kojonazarov B, Myrzaakhmatova A, Sooronbaev T, et al. effects of fasudil in patients with high altitude pulmonary hypertension. Eur Respir J. 2012;39(2):496–498.
   PubMed Web of Science ®Google Scholar
• Galie N. Effects of inhaled aviptadil (vasoactive intestinal peptide) in patients with pulmonary arterial hypertension (PAH). Am J Respir Crit Care Med. 2010;181(1):B116.
   PubMedGoogle Scholar
• Zhu JH, Wang XX, Zhang FR, et al. Safety and efficacy of autologous endothelial progenitor cells transplantation in children with idiopathic pulmonary arterial hypertension: open-label pilot study. Pediatr Transplant. 2008;12(6):650–655.
   PubMed Web of Science ®Google Scholar
• Sa S, Gu M, Chappe J II, et al. İnduced pluripotent stem cell model of pulmonary arterial hypertension reveals novel gene expression and patient specifity. Am J Respir Crit Care Med. 2017;195:930–941.
   PubMed Web of Science ®Google Scholar
• Sabiniewicz R. Baloon atrial septoplasty in pulmonary arterial hypertension: atrial flow regülatör- New therapeutic option. Cardiol J. 2017;24(4):455–456.
   PubMed Web of Science ®Google Scholar
• Kula S, Atasayan V. Surgical and transcatheter management alternatives in refractory pulmonary hypertension. Anatol J Cardiol. 2015;15(10):843–850.
   PubMed Web of Science ®Google Scholar
• Benden C, Edwards LB, Kucheryavaya AY, et al. The registry of the international society for heart and lung transplantation: official pediatric lung and heart lung transplantation report. Focus theme: age. J Heart Lung Transplant. 2013;32:989–999.
   PubMedGoogle Scholar