83
Views
9
CrossRef citations to date
0
Altmetric
Review

Management and therapeutic options in pediatric pulmonary hypertension

Pages 361-374 | Published online: 10 Jan 2014

References

  • Wood P. The eisenmenger syndrome or pulmonary hypertension with reversed central shunt. Br. Med. J.2, 701–709 (1958).
  • Eisenmenger V. Die angeboren defects des kammerscheidewand des herzen. Z. Klin. Med.32, 1–28 (1897).
  • Heath D, Edwards J. A description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac defects. Circulation18, 533–547 (1958).
  • Rabinovitch M, Keane J, Norwood W. Vascular structure in lung tissue obtained at biopsy correlated with pulmonary haemodynamic findings after repair of congenital heart defects. Circulation69, 655–667 (1984).
  • Rabinovitch M, Pathobiology of pulmonary hypertension: impact on clinical management. Semin. Thorac. Cardiovasc. Surg. Pediatr. Card. Surg. Annu.3, 63–81 (2000).
  • Andrews R, Tulloh R. Pulmonary hypertension in paediatrics. Cur. Opin. Paediatr.14, 603–605 (2002).
  • Gibbs J. Recommendations on the management of pulmonary hypertension in clinical practice. Heart86(Suppl. 1), 1–13 (2001).
  • Proceedings of the 3rd World Symposium on Pulmonary Arterial Hypertension. Venice, Italy, June 23–25. J. Am. Coll. Cardiol.43(12 Suppl. S), S1S–S90 (2003).
  • Rich S. Primary pulmonary hypertension: executive summary from the world symposium – primary pulmonary hypertension. Available at: http;//www.who.int/ncd/cvd/pph.html, (1998).
  • Abman SH. Pathogenesis and treatment of neonatal and postnatal pulmonary hypertension. Curr. Opin. Pediatr.6(3), 239–247 (1994).
  • Humbert M, Nunes H, Sitbon O, Parent F, Herne P, Simonneau G. Risk factors for pulmonary arterial hypertension. Clin. Chest Med.22(3), 459–475 (2001).
  • Luchese S, Manica LJ, Zielinsky P. Intrauterine ductus arteriosus constriction: analysis of a historic cohort of 20 cases. Arq. Bras. Cardiol.81(4), 405–10, 399–404 (2003).
  • Mark EJ, Patalas ED, Chang HT, Evans RJ, Kessler SC. Fatal pulmonary hypertension associated with short-term use of fenfluramine and phentermine. N. Engl. J. Med.337, 602–606 (1997).
  • Chambers CD, Hernandez-Diaz S, van Marter LJ et al. Selective serotonin-reuptake inhibitors and risk of persistent pulmonary hypertension of the newborn. N. Engl. J. Med.354, 579–587 (2006).
  • Rosenzweig EB, Barst JR. Idiopathic pulmonary arterial hypertension in children. Curr. Opin. Pediatr.17(3), 372–380 (2005).
  • Haworth SG. Development of the normal and hypertensive pulmonary vasculature. Exp. Physiol.80(5), 843–853 (1995).
  • Manes A, Campana C. Pulmonary hypertension: classification and diagnostic algorithm. Ital. Heart J.6(10), 834–839 (2005).
  • Ebeid MR, Ferrer PL, Robinson B, Weatherby N, Gebland H. Doppler echocardiographic evaluation of pulmonary vascular resistance in children with congenital heart disease. J. Am. Soc. Echocardiogr.9(6), 822–831 (1996).
  • Lopez-Candales A, Dohi K, Rajagopalan N et al. Right ventricular dyssynchrony in patients with pulmonary hypertension is associated with disease severity and functional class. Cardiovasc. Ultrasound3, 23–26 (2005).
  • Maiya S, Hislop AA, Flynn Y, Haworth SG. Response to bosentan in children with pulmonary hypertension. Heart92(5), 664–670 (2005).
  • Atz AM, Adatia I, Lock JE, Wessel DL. Combined effects of nitric oxide during acute pulmonary vasodilator testing. J. Am. Coll. Cardiol.33, 813–819 (1999).
  • Mikhail G, Gibbs J, Richardson M et al. An evaluation of nebulized prostacycin in patients with primary and secondary pulmonary hypertension. Eur. Heart J.18, 1499–1504 (1997).
  • Holcomb SS. Understanding pulmonary arterial hypertension. Nursing34(9), 50–54 (2004).
  • Muthurangu V, Taylor A, Andriantsimianova R et al. Ovel method of quantifying pulmonary vascular resistance by use of simultaneous invasive pressure monitoring and phase-contrast magnetic resonance flow. Circulation110(7), 826–834 (2004).
  • Muthurangu V, Atkinson D, Sermesant M et al. Measurement of total pulmonary arterial compliance using invasive pressure monitoring and MR flow quantification during MR-guided cardiac catheterization. Am. J. Physiol. Heart Circ. Physiol.289(3), H1301–H1306 (2005).
  • Rabinovitch M, Haworth SG, Castaneda AR, Nadas AS, Reid LM. Lung biopsy in congenital heart disease: a morphometric approach to pulmonary vascular disease. Circulation58, 1107–1122 (1978).
  • Meyrick B, Reid L. Ultrastructural findings in lung biopsy material from children with congenital heart defects. Am. J. Pathol.101, 527–537 (1980).
  • Rabinovitch M, Bothwell T, Hayakawa B. Pulmonary artery endothelial abnormalities in patients with congenital heart defects and pulmonary hypertension: a correlation of light with scanning electron microscopy and transmission electron microscopy. Lab. Invest.55, 632–653 (1986).
  • Taipale J, Lohi J, Saarinen J, Kovanen PT, Keski-Oja J. Human mast cell chymase and leukocyte elastase release latent transforming growth factor B1 from the extracellular matrix of cultured human epithelial and endothelial cells. J. Biol. Chem.270, 4689–4696 (1995).
  • Jones P, Cowan K, Rabinovitch M. Induction of tenascin and fibronectin are features associated with increased smooth muscle cell proliferation during the development of progressive pulmonary hypertension in children. Am. J. Pathol.150, 1349–1360 (1997).
  • Thomson J, Machado RD, Pauciulo MW et al. Sporadic primary pulmonary hypertension is associated with germline mutations of the gene encoding BMPR-2, a receptor member of the TGF-β family. J. Med. Genet.37, 741–745 (2000).
  • Derynck R, Zhang EY. SMAD-dependent and Smad-independent pathways in TGF-family signalling. Nature425, 577–584 (2003).
  • Roberts KE, McElroy JJ, Wong WP et al. BMPR2 mutations in pulmonary arterial hypertension with congenital heart disease. Eur. Respir. J.24(3), 371–374 (2004).
  • Tulloh R, Hislop AA, Boels PJ, Deutsch J, Haworth SG. Chronic hypoxia inhibits postnatal maturation of porcine intrapulmonary artery relaxation. Am. J. Physiol.272, H2436–H2445 (1997).
  • Black SM, Bekker JM, McMullan DM et al. Alterations in nitric oxide production in 8-week-old lambs with increased pulmonary blood flow. Pediatr. Res.52(2), 233–244 (2002).
  • Adatia I, Haworth S. Circulating endothelin levels in children with congenital heart disease. Br. Heart J.69, 233–236 (1993).
  • Bando K, Turrentine MW, Sharp TG et al. Pulmonary hypertension after operations for congenital heart disease: analysis of risk factors and management. J. Thorac. Cardiovasc. Surg.112(6), 1600–1607; discussion 1607–1609 (1996).
  • Yuan JX, Rubin JL. Pathogenesis of pulmonary arterial hypertension: the need for multiple hits. Circulation111(5), 534–538 (2005).
  • Lindberg L, Olsson AK, Jogi P, Jonmarker C. How common is severe pulmonary hypertension after pediatric cardiac surgery? J. Thorac. Cardiovasc. Surg.123(6), 1155–1163 (2002).
  • Ohsumi H, Sakamoto M, Yamazaki T, Okumura F. Effects of fentanyl on carotid sinus baroreflex control of circulation in rabbits. Am. J. Physiol.256, R625–R631 (1989).
  • Levy Tulloh MR, Komai H et al. Maturation of the contractile response and its endothelial modulation in newborn porcine intrapulmonary arteries. Pediatric Res.38, 25–29 (1995).
  • Adatia I, Wessel LD. Therapeutic use of inhaled nitric oxide. Curr. Opin. Pediatr.6(5), 583–590 (1994).
  • Abman SH. Neonatal pulmonary hxypertension: a physiologic approach to treatment. Pediatr. Pulmonol. Suppl.26, 127–128 (2004).
  • Adatia I, Perry S, Landzberg M, Moore P, Thompson JE, Wessel DL. Inhaled nitric oxide and hemodynamic evaluation of patients with pulmonary hypertension before transplantation. J. Am. Coll. Cardiol.25(7), 1656–1664 (1995).
  • Wimmer M, Schlemmer M. Long-term hemodynamic effects of nifedipine on congenital heart disease with Eisenmenger's mechanism in children. Cardiovasc. Drugs Ther.6(2), 183–186 (1992).
  • McMahon CJ, Kadkin J, Nihill RM. Rapid regression of primary pulmonary hypertension. Heart86(1), E1 (2001).
  • Humpl T, Reyes JT, Holtby H, Stephens D, Adatia I. Beneficial effect of oral sildenafil therapy on childhood pulmonary arterial hypertension: twelve-month clinical trial of a single-drug, open-label, pilot study. Circulation111(24), 3274–3280 (2005).
  • Weimann J, Ullrich R, Hromi J et al. Sildenafil is a pulmonary vasodilator in awake lambs with acute pulmonary hypertension. Anesthesiology92, 1702–1712 (2000).
  • Matot I, Gozal Y. Pulmonary responses to selective phosphodiesterase-5 and phosphodiesterase-3 inhibitors. Chest 125(2), 644–651 (2004).
  • Atz AM, Lefler AK, Fairbrother DL, Uber WE, Bradley SM. Sildenafil augments the effect of inhaled nitric oxide for postoperative pulmonary hypertensive crises. J. Thorac. Cardiovasc. Surg.124(3), 628–629 (2002).
  • Bharani A, Mathew V, Sahu A, Lunia B. The efficacy and tolerability of sildenafil in patients with moderate-to-severe pulmonary hypertension. Indian Heart J.55(1), 55–59 (2003).
  • Brun H, Holmstrom H, Thaulow E. Sudden death during a change in treatment for pulmonary hypertension. Cardiol. Young15(2), 223–225 (2005).
  • Della Torre F, Della Torre E, Di Berardino F. Sildenafil in pulmonary hypertension. Sarcoidosis Vasc. Diffuse Lung Dis.22(1), 78–79 (2005).
  • Rosenzweig EB, Ivy DD, Widlitz A et al. Effects of long-term bosentan in children with pulmonary arterial hypertension. J. Am. Coll. Cardiol.46(4), 697–704 (2005).
  • Barst RJ, Ivy D, Dingemanse J et al. Pharmacokinetics, safety, and efficacy of bosentan in pediatric patients with pulmonary arterial hypertension. Clin. Pharmacol. Ther.73(4), 372–382 (2003).
  • Gatzoulis MA, Rogers P, Li W et al. Safety and tolerability of bosentan in adults with Eisenmenger physiology. Int. J. Cardiol.98(1), 147–151 (2005).
  • Apostolopoulou SC, Rammos S. Sitaxsentan in pulmonary arterial hypertension. Chest123(5), 1772; author reply 1772–1723 (2003).
  • Barst RJ, Langleben D, Frost A et al; STRIDE-1 Study Group. Sitaxsentan therapy for pulmonary arterial hypertension. Am. J. Respir. Crit. Care Med.169(4), 441–447 (2004).
  • Barst RJ, Rich S, Widlitz A, Horn EM, McLaughlin V, McFarlin J. Clinical efficacy of sitaxsentan, an endothelin – a receptor antagonist, in patients with pulmonary arterial hypertension: open-label pilot study. Chest121(6), 1860–1868 (2002).
  • Prostacyclin: basic principles and clinical application in congestive heart failure and primary pulmonary hypertension. Proceedings of a symposium. Bologna, Italy, November 20, 1993. Am. J. Cardiol.75(3), 1A–73A (1995).
  • Hermon M, Golej J, Burda G, Marx M, Trittenwein G, Pollak A. Intravenous prostacyclin mitigates inhaled nitric oxide rebound effect: a case control study. Artif. Organs23, 975–978 (1999).
  • Barst RJ, Rubin LJ, Long WA et al.; The Primary Pulmonary Hypertension Study Group. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N. Engl. J. Med.334(5), 296–302 (1996).
  • Simonneau G, Barst RJ, Galie N et al; Treprostinil Study Group. Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial. Am. J. Respir. Crit. Care Med.165(6), 800–804 (2002).
  • Vizza CD, Sciomer S, Morelli S et al. Long term treatment of pulmonary arterial hypertension with beraprost, an oral prostacyclin analogue. Heart86(6), 661–5 (2001).
  • Barst RJ, McGoon M, McLaughlin V et al ; Beraprost Study Group. Beraprost therapy for pulmonary arterial hypertension. J. Am. Coll. Cardiol.41(12), 2119–2125 (2003).
  • Baker SE, Hockman HR. Inhaled iloprost in pulmonary arterial hypertension. Ann. Pharmacother.39(7–8), 1265–1274 (2005).
  • Clark RH, Huckaby JL, Kueser TJ et al ; Clinical Inhaled Nitric Oxide Research Group. Low-dose nitric oxide therapy for persistent pulmonary hypertension: 1-year follow-up. J. Perinatol.23(4), 300–303 (2003).
  • Cheever KH. An overview of pulmonary arterial hypertension: risks, pathogenesis, clinical manifestations, and management. J. Cardiovasc. Nurs.20(2), 108–116; quiz 117–118 (2005).
  • Rosenzweig EB, Barst RJ. Eisenmenger's syndrome: current management. Prog. Cardiovasc. Dis.45, 129–138 (2002).
  • Andrews R, Tulloh R, Magee A, Anderson D. Atrial septal defect with failure to thrive in infancy: hidden pulmonary vascular disease? Pediatr. Cardiol.23(5), 528–530 (2002).
  • Kumar A, Taylor GP, Sandor GG, Patterson MW. Pulmonary vascular disease in neonates with transposition of the great arteries and intact ventricular septum. Br. Heart J.69(5), 442–445 (1993).
  • Holcomb R, Tyson RW, Ivy DD, Abman SH, Kinsella JP. Congenital pulmonary venous stenosis presenting as persistent pulmonary hypertension of the newborn. Pedia. Pulmonology28, 301–306 (1999).
  • Ricci M, Elliott M, Cohen GA et al. Management of pulmonary venous obstruction after correction of TAPVC: risk factors for adverse outcome. Eur. J. Cardiothorac. Surg.24(1), 28–36; discussion 36 (2003).
  • Rich S, Kaufmann E, Levy SP. The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension. N. Engl. J. Med.327(2), 76–81 (1992).
  • Higenbottam T. Intravenous epoprostenol for primary pulmonary hypertension. N. Engl. J. Med.334(22), 1477; author reply 1477–1478 (1996).
  • Sandoval J, Aguirre JS, Pulido T et al. Nocturnal oxygen therapy in patients with the Eisenmenger syndrome. Am. J. Respir. Crit. Care Med.164(9), 1682–1687 (2001).
  • Kerstein D, Levy PS, Hsu DT, Hordof AJ, Gersony WM, Barst RJ. Blade balloon atrial septostomy in patients with severe primary pulmonary hypertension. Circulation91(7), 2028–2035 (1995).
  • Micheletti A, Hislop A, Lammers A et al. Role of atrial septostomy in the treatment of children with pulmonary arterial hypertension. Heart (2005) (Epub ahead of print).

Website

  • Department of Health: National Specialist Comissioning Advisory Group (NSCAG) www.advisorybodies.doh.gov.uk/NSCAG

Reprints and Corporate Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

To request a reprint or corporate permissions for this article, please click on the relevant link below:

Academic Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

Obtain permissions instantly via Rightslink by clicking on the button below:

If you are unable to obtain permissions via Rightslink, please complete and submit this Permissions form. For more information, please visit our Permissions help page.