Pediatric Cardiology

, Volume 33, Issue 5, pp 775–781

Clinical Manifestations and Long-Term Follow-Up in Pediatric Patients Living at Altitude With Isolated Pulmonary Artery of Ductal Origin

  • Shinichi Takatsuki
  • Jeffrey R. Darst
  • Bibhuti B. Das
  • Thomas E. Fagan
  • Robert Wolfe
  • David Dunbar Ivy
Original Article


This study’s aim was to define the clinical manifestations and long-term outcome of pediatric patients living at altitude with isolated pulmonary artery (PA) of ductal origin (IPADO). This was a retrospective cohort study of 17 consecutive cases of IPADO at a single center. All patients lived at modest altitude (median 2050 m [range 1700 m to 3050 m]). Fifteen children (88%) were symptomatic at presentation. High-altitude pulmonary edema was present in 2 patients (12%) at diagnosis, and only 1 patient had episodes of hemoptysis during follow-up. Fourteen patients (82%) demonstrated evidence of pulmonary arterial hypertension (PAH). Among 14 patients with PAH, 11 patients had surgical interventions. PAH resolved in 5 of 11 patients (45%) undergoing surgical rehabilitation. One patient died during follow-up, and 7 patients are receiving oral vasodilator therapies due to residual PAH; 14 patients remained asymptomatic. Our study showed that early intervention in patients with IPADO at modest altitude can potentially rehabilitate the isolated PA and reverse PAH. Whether surgery is indicated for patients with this disorder in the absence of PAH is unknown.


Isolated pulmonary artery of ductal origin Unilateral absence of pulmonary artery Altitude High-altitude pulmonary edema Pulmonary arterial hypertension 


  1. 1.
    Agostoni P, Cattadori G, Guazzi M, Bussotti M, Conca C, Lomanto M et al (2000) Effects of simulated altitude-induced hypoxia on exercise capacity in patients with chronic heart failure. Am J Med 109:450–455PubMedCrossRefGoogle Scholar
  2. 2.
    Alison M, Garel L, Bigras JL, Dery J, Lapierre C (2011) Unilateral absence of pulmonary artery in children: bronchovascular anatomy, natural course and effect of treatment on lung growth. Pediatr Radiol 41:459–468PubMedCrossRefGoogle Scholar
  3. 3.
    Barer GR, Howard P, Shaw JW (1970) Stimulus-response curves for the pulmonary vascular bed to hypoxia and hypercapnia. J Physiol 211:139–155PubMedGoogle Scholar
  4. 4.
    Bouros D, Pare P, Panagou P, Tsintiris K, Siafakas N (1995) The varied manifestation of pulmonary artery agenesis in adulthood. Chest 108:670–676PubMedCrossRefGoogle Scholar
  5. 5.
    Das BB, Wolfe RR, Chan KC, Larsen GL, Reeves JT, Ivy D (2004) High-altitude pulmonary edema in children with underlying cardiopulmonary disorders and pulmonary hypertension living at altitude. Arch Pediatr Adolesc Med 158:1170–1176PubMedCrossRefGoogle Scholar
  6. 6.
    Fagan T, Kay J, Carroll J, Neubauer A (2012) 3-D guidance of complex pulmonary artery stent placement using reconstructed rotational angiography with live overlay. Catheter Cardiovasc Interv 79:414–421Google Scholar
  7. 7.
    Freedom RM, Pongiglione G, Williams WG, Trusler GA, Moes CA, Rowe RD (1983) Pulmonary vein wedge angiography: indications, results, and surgical correlates in 25 patients. Am J Cardiol 51:936–941PubMedCrossRefGoogle Scholar
  8. 8.
    Groves BM, Reeves JT, Sutton JR, Wagner PD, Cymerman A, Malconian MK et al (1987) Operation Everest II: elevated high-altitude pulmonary resistance unresponsive to oxygen. J Appl Physiol 63:521–530PubMedGoogle Scholar
  9. 9.
    Hackett PH, Creagh CE, Grover RF, Honigman B, Houston CS, Reeves JT et al (1980) High-altitude pulmonary edema in persons without the right pulmonary artery. N Engl J Med 302:1070–1073PubMedCrossRefGoogle Scholar
  10. 10.
    Hamdan MA, Al Meshham Y, Najm HK (2005) Successful one-stage repair of unilateral agenesis of pulmonary artery. Pediatr Cardiol 26:724–726PubMedCrossRefGoogle Scholar
  11. 11.
    Kim GB, Ban JE, Bae EJ, Noh CI, Kim WH, Lee JR et al (2011) Rehabilitation of pulmonary artery in congenital unilateral absence of intrapericardial pulmonary artery. J Thorac Cardiovasc Surg 141:171–178PubMedCrossRefGoogle Scholar
  12. 12.
    Koga H, Hidaka T, Miyako K, Suga N, Takahashi N (2010) Age-related clinical characteristics of isolated congenital unilateral absence of a pulmonary artery. Pediatr Cardiol 31:1186–1190PubMedCrossRefGoogle Scholar
  13. 13.
    Kosaka Y, Kurosawa H, Hoshino S, Shin’oka T, Isomatsu Y, Tsuji Y (2003) Surgery for unilateral absence of pulmonary artery using autologous tissue. Ann Thorac Surg 76:1281–1283PubMedCrossRefGoogle Scholar
  14. 14.
    Kucera V, Fiser B, Tuma S, Hucin B (1982) Unilateral absence of pulmonary artery: a report on 19 selected clinical cases. Thorac Cardiovasc Surg 30:152–158PubMedCrossRefGoogle Scholar
  15. 15.
    Maignan M, Rivera-Ch M, Privat C, Leon-Velarde F, Richalet JP, Pham I (2009) Pulmonary pressure and cardiac function in chronic mountain sickness patients. Chest 135:499–504PubMedCrossRefGoogle Scholar
  16. 16.
    Mehta AC, Livingston DR, Kawalek W, Golish JA, O’Donnell JK (1987) Pulmonary artery agenesis presenting as massive hemoptysis—a case report. Angiology 38:67–71PubMedCrossRefGoogle Scholar
  17. 17.
    Moreno-Cabral RJ, McNamara JJ, Reddy VJ, Caldwell P (1991) Unilateral absent pulmonary artery: surgical repair with a new technique. J Thorac Cardiovasc Surg 102:463–465PubMedGoogle Scholar
  18. 18.
    Paralikar SJ, Paralikar JH (2010) High-altitude medicine. Indian J Occup Environ Med 14:6–12PubMedCrossRefGoogle Scholar
  19. 19.
    Pasha MA, Newman JH (2010) High-altitude disorders: Pulmonary hypertension: Pulmonary vascular disease: the global perspective. Chest 137(Suppl 6):13S–19SPubMedCrossRefGoogle Scholar
  20. 20.
    Penaloza D, Arias-Stella J (2007) The heart and pulmonary circulation at high altitudes: healthy highlanders and chronic mountain sickness. Circulation 115:1132–1146PubMedCrossRefGoogle Scholar
  21. 21.
    Pool PE, Vogel JH, Blount SG Jr (1962) Congenital unilateral absence of a pulmonary artery. The importance of flow in pulmonary hypertension. Am J Cardiol 10:706–732PubMedCrossRefGoogle Scholar
  22. 22.
    Presbitero P, Bull C, Haworth SG, de Leval MR (1984) Absent or occult pulmonary artery. Br Heart J 52:178–185PubMedCrossRefGoogle Scholar
  23. 23.
    Rios B, Driscoll DJ, McNamara DG (1985) High-altitude pulmonary edema with absent right pulmonary artery. Pediatrics 75:314–317PubMedGoogle Scholar
  24. 24.
    Schoene RB (2001) Fatal high altitude pulmonary edema associated with absence of the left pulmonary artery. High Alt Med Biol 2:405–406PubMedCrossRefGoogle Scholar
  25. 25.
    Sebbane M, Wuyam B, Pin I, Pendlebury S, Plasse M, Durand C et al (1997) Unilateral agenesis of the pulmonary artery and high-altitude pulmonary edema (HAPE) at moderate altitude. Pediatr Pulmonol 24:111–114PubMedCrossRefGoogle Scholar
  26. 26.
    Shakibi JG, Rastan H, Nazarian I, Paydar M, Aryanpour I, Siassi B (1978) Isolated unilateral absence of the pulmonary artery. Review of the world literature and guidelines for surgical repair. Jpn Heart J 19:439–451PubMedCrossRefGoogle Scholar
  27. 27.
    Sotomora RF, Edwards JE (1978) Anatomic identification of so-called absent pulmonary artery. Circulation 57:624–633PubMedGoogle Scholar
  28. 28.
    Taguchi T, Ikeda K, Kume K, Nagasaki A, Goto S, Matsuo S et al (1987) Isolated unilateral absence of left pulmonary artery with peribronchial arteriovenous malformation showing recurrent hemoptysis. Pediatr Radiol 17:316–318PubMedCrossRefGoogle Scholar
  29. 29.
    Ten Harkel AD, Blom NA, Ottenkamp J (2002) Isolated unilateral absence of a pulmonary artery: a case report and review of the literature. Chest 122:1471–1477PubMedCrossRefGoogle Scholar
  30. 30.
    Trivedi KR, Karamlou T, Yoo SJ, Williams WG, Freedom RM, McCrindle BW (2006) Outcomes in 45 children with ductal origin of the distal pulmonary artery. Ann Thorac Surg 81:950–957PubMedCrossRefGoogle Scholar
  31. 31.
    Wagenvoort CA, Wagenvoort N, Vogel JH (1969) The pulmonary vasculature in cattle at an altitude of 1,600 metres with and without one-sided pulmonary arterial ligation. J Comp Pathol 79:517–523PubMedCrossRefGoogle Scholar
  32. 32.
    Welch K, Hanley F, Johnston T, Cailes C, Shah MJ (2005) Isolated unilateral absence of right proximal pulmonary artery: surgical repair and follow-up. Ann Thorac Surg 79:1399–1402PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  • Shinichi Takatsuki
    • 1
  • Jeffrey R. Darst
    • 1
  • Bibhuti B. Das
    • 2
  • Thomas E. Fagan
    • 1
  • Robert Wolfe
    • 1
  • David Dunbar Ivy
    • 1
  1. 1.Section of Cardiology, Department of PediatricsChildren’s Hospital Colorado, University of Colorado DenverAuroraUSA
  2. 2.Section of Cardiology, Department of PediatricsChildren’s Medical Center, University of Texas SouthwesternDallasUSA

Personalised recommendations