Skip to main content
SpringerLink
Log in

A case of congenital agenesis of the right pulmonary artery presenting with hemoptysis and mimicking pulmonary hemosiderosis

  • Short Report
  • Published:
European Journal of Pediatrics Aims and scope Submit manuscript

Abstract

Congenital unilateral absence of a pulmonary artery is a rare anomaly most frequently accompanied by other cardiovascular anomalies. We report a 10-year-old girl presenting with fatigue and recurrent hemoptysis who was initially misdiagnosed with idiopathic pulmonary hemosiderosis. Her symptoms did not resolve despite treatment so she was referred to our center for further evaluation. We carried out an angiography which revealed the absence of the right pulmonary artery and multiple collaterals originating from the right subclavian and right internal mammary arteries supplying the right lung. During the follow-up the patient developed a severe episode of pulmonary infection and pulmonary hypertension which responded well to medical treatment. Physicians should be aware of the congenital absence of the right pulmonary artery especially in patients presenting with recurrent respiratory symptoms. Although this condition is generally considered to have a good prognosis, close observation is mandatory in order to prevent further complications and comorbidities.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Abbreviations

FVC:

Forced vital capacity

FEV1 :

Forced expiratory volume in the first second

CT:

Computed tomography

ACE:

Angiotensin-converting enzyme

IV:

Intravenous

MRI:

Magnetic resonance imaging

UAPA:

Unilateral absence of a pulmonary artery

References

  1. Apostolopoulou SC, Kelekis NL, Brountzos EN, Rammos S, Kelekis DA (2002) “Absent” pulmonary artery in one adult and five pediatric patients: imaging, embryology, and therapeutic implications. Am J Roentgenol 179:1253–1260

    Google Scholar 

  2. Bouros D, Pare P, Panagou P, Tsintiris K, Siafakas N (1995) The varied manifestation of pulmonary artery agenesis in adulthood. Chest 108:670–676

    Article  PubMed  CAS  Google Scholar 

  3. Campbell KR, Krasuski R, Wang A, O’Laughlin MP, Harrison JK (2000) Congenital agenesis of the right pulmonary artery. Catheter Cardiovasc Interv 51:460–463

    Article  PubMed  CAS  Google Scholar 

  4. Cucci CE, Doyle EF, Lewis EW Jr (1964) Absence of a primary division of the pulmonary trunk. An ontogenetic theory. Circulation 29:124–131

    PubMed  CAS  Google Scholar 

  5. Farghly E, Bousamra M 2nd (2002) Hemoptysis resulting from unilateral pulmonary artery agenesis. Ann Thorac Surg 74:255–257

    Article  PubMed  Google Scholar 

  6. Fraentzel O (1868) Ein fall von abnormer communication der aorta mit der anterior pulmonalis. Virchow Arch Path Anat 43:420–426

    Article  Google Scholar 

  7. Gamba A, Villani M, Tiraboschi R, Annecchino P, Crupi G, Vanini V, Brunelli F, Parenzan L (1984) Surgical treatment of the tetralogy of Fallot with a single pulmonary artery. G Ital Cardiol 14:499–504

    PubMed  CAS  Google Scholar 

  8. Herraiz Sarachaga I, Pérez González W, Vergara Rodríguez F, Alonso Lastra MJ, Acerete Guillén F, Bermúdez-Cañete Fernández R, Díaz García P, Quero Jiménez M (1993) Unilateral agenesis of the pulmonary artery. Experience with 4 cases. An Esp Pediatr 38:139–144

    PubMed  CAS  Google Scholar 

  9. Jucá ER, Carvalho Júnior W, de Oliveira FM, Araújo JA, Mont’Alverne R, Paes Júnior JN, de Sousa JR (1982) Surgical treatment of tetralogy of fallot with left pulmonary artery agenesis. Report of 2 cases. Arq Bras Cardiol 38:39–43

    PubMed  Google Scholar 

  10. Krall WR, Ploy-Song-Sang Y (1980) Unilateral pulmonary artery aplasia presenting with chest pain and pleural effusion. South Med J 73:233–236

    PubMed  CAS  Google Scholar 

  11. Kucera V, Fiser B, Tůma S, Hucin B (1982) Unilateral absence of pulmonary artery: a report on 19 selected clinical cases. Thorac Cardiovasc Surg 30:152–158

    Article  PubMed  CAS  Google Scholar 

  12. Lin YM, Liang KW, Ting CT (1999) Unilateral pulmonary artery agenesis with presentation of hemoptysis: a case report. Zhonghua Yi Xue Za Zhi (Taipei) 62:644–647

    CAS  Google Scholar 

  13. Mimura S, Kobayashi H, Shinkai M, Kanoh S, Motoyoshi K (2005) A case report of congenital isolated absence of the right pulmonary artery: bronchofibrescopic findings and chest radiological tracings over 9 years. Respirology 10:250–253

    Article  PubMed  Google Scholar 

  14. Pfefferkorn JR, Löser H, Pech G, Toussaint R, Hilgenberg F (1982) Absent pulmonary artery. A hint to its embryogenesis. Pediatr Cardiol 3:283–286

    Article  PubMed  CAS  Google Scholar 

  15. Pool PE, Vogel JHK, Blount SG Jr (1962) Congenital unilateral absence of a pulmonary artery. The importance of flow in pulmonary hypertension. Am J Cardiol 10:706–732

    Article  PubMed  CAS  Google Scholar 

  16. Ten Harkel AD, Blom NA, Ottenkamp J (2002) Isolated unilateral absence of a pulmonary artery: a case report and review of the literature. Chest 122:1471–1477

    Article  PubMed  Google Scholar 

  17. Thompson JW, Nguyen CD, Lazar RH, Stocks RM, Schoumacher RA, Hamdan F, Van Nguyen K (1996) Evaluation and management of hemoptysis in infants and children. A report of nine cases. Ann Otol Rhinol Laryngol 105:516–520

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pediatrics, Hacettepe University, Ankara, Turkey

    Pelin Ozlem Simsek

  2. Pulmonology Unit, Department of Pediatrics, Hacettepe University, Ankara, Turkey

    Ugur Ozcelik, Ebru Yalcin, Nazan Cobanoglu, Sevgi Pekcan, Deniz Dogru & Nural Kiper

  3. Cardiology Unit, Department of Pediatrics, Hacettepe University, Ankara, Turkey

    Alpay Celiker & Dursun Alehan

  4. Hematology Unit, Department of Pediatrics, Selcuk University, Konya, Turkey

    Canan Ucar

  5. Sanliurfa Childrens’ Hospital, Sanliurfa, Turkey

    Pelin Ozlem Simsek

Authors
  1. Pelin Ozlem Simsek
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Ugur Ozcelik
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Alpay Celiker
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Ebru Yalcin
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Nazan Cobanoglu
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Sevgi Pekcan
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Dursun Alehan
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Canan Ucar
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Deniz Dogru
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Nural Kiper
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Pelin Ozlem Simsek.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Simsek, P.O., Ozcelik, U., Celiker, A. et al. A case of congenital agenesis of the right pulmonary artery presenting with hemoptysis and mimicking pulmonary hemosiderosis. Eur J Pediatr 168, 217–220 (2009). https://doi.org/10.1007/s00431-008-0726-x

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00431-008-0726-x

Keywords

Search

Navigation