A Case of Primary Pulmonary Hypertension in Pregnancy Presented as Massive Hemoptysis

임신 중 대량객혈로 발현된 원발성 폐고혈압 1예

  • Kim, Myung Sook (Department of Internal Medicine, Daejon St. Mary's Hospital, The Catholic University of Korea) ;
  • Kim, Hyoung Doo (Department of Internal Medicine, Daejon St. Mary's Hospital, The Catholic University of Korea) ;
  • Kim, Seok Chan (Department of Internal Medicine, Daejon St. Mary's Hospital, The Catholic University of Korea) ;
  • Kwan, Soon Suk (Department of Internal Medicine, Daejon St. Mary's Hospital, The Catholic University of Korea) ;
  • Kim, Young Kyoon (Department of Internal Medicine, Daejon St. Mary's Hospital, The Catholic University of Korea) ;
  • Kim, Kwan Hyoung (Department of Internal Medicine, Daejon St. Mary's Hospital, The Catholic University of Korea) ;
  • Moon, Hwa Sik (Department of Internal Medicine, Daejon St. Mary's Hospital, The Catholic University of Korea) ;
  • Song, Jeong Sup (Department of Internal Medicine, Daejon St. Mary's Hospital, The Catholic University of Korea) ;
  • Park, Sung Hak (Department of Internal Medicine, Daejon St. Mary's Hospital, The Catholic University of Korea)
  • 김명숙 (가톨릭대학교 의과대학 내과학교실) ;
  • 김형두 (가톨릭대학교 의과대학 내과학교실) ;
  • 김석찬 (가톨릭대학교 의과대학 내과학교실) ;
  • 권순석 (가톨릭대학교 의과대학 내과학교실) ;
  • 김영균 (가톨릭대학교 의과대학 내과학교실) ;
  • 김관형 (가톨릭대학교 의과대학 내과학교실) ;
  • 문화식 (가톨릭대학교 의과대학 내과학교실) ;
  • 송정섭 (가톨릭대학교 의과대학 내과학교실) ;
  • 박성학 (가톨릭대학교 의과대학 내과학교실)
  • Received : 2004.03.26
  • Accepted : 2004.05.31
  • Published : 2004.07.30

Abstract

Primary pulmonary hypertension (PPH) is a rare, progressive and incurable disease, which is characterized by an increase in the pulmonary artery pressure without a demonstrable cause. The most common presenting symptom is dyspnea on exertion, with other symptoms comprising of chest pain, syncope and hemoptysis. The diagnosis is one of exclusion of any of the known causes of pulmonary hypertension. When associated with pregnancy, the maternal mortality ranges from 30 to 50%. Because pregnancy and labor are very serious problems for patients with PPH, the available evidence suggests that pregnancy when afflicted with PPH should be avoided. In account the case of a 33-year old patient, reporting with massive hemoptysis, and diagnosed with PPH during her twenty seventh week of gestation, is presented. She was treated with conservative management, including oxygen and a vasodilator, and underwent a pregnancy termination. However, due to aggravation of right heart failure, she presented with severe systemic hypotension and hypoxemia, and eventually died. This case is reported, with brief review of the literature.

저자들은 임신중기에 대량객혈로 내원하여 안정, 산소공급, 폐혈관확장제, 치료적 유산 그리고 항응고제 사용 등으로 치료하였으나 결국 우심부전으로 사망한 원발성 폐고혈압 환자 1예를 경험하였다. 본 증례는 폐관류 및 환기스캔, 폐혈관조영술 등의 검사와 사망후 부검은 시행하지 못하였으나 현재까지의 검사결과에 의거 시 임신 중 대량객혈로 발현된 일차성 폐고혈압으로 사료되어, 이에 문헌고찰과 함께 보고하는 바이다.

Keywords

References

  1. Voelkel NF, Tuder RM. Cellular and molecular mechanisms in the pathogenesis of severe pulmonary hypertension. Eur Respir J 1995;8(12):2129-38
  2. Christman BW, McPherson CD, Newman JH, King GA, Bernard GR, Groves BM, et al. An imbalance between the excretion of thromboxane and prostacyclin metabolites in pulmonary hypertension. N Engl J Med 1992;327:70-5
  3. Lee SD, Shroyer KR, Markham NE, Cool CD, Voelkel NF, Tuder RM. Monoclonal endothelial cell proliferation is present in primary but not secondary pulmonary hypertension. J Clin Invest 1998;101(5):927-34
  4. Monnery L, Nanson J, Charlton G. Primary pulmonary hypertension in pregnancy; a role for novel vasodilators. Br J Anaesth 2001;87:295-8
  5. Goodwin TM, Gherman RB, Hameed A, Elkayam U. Favorable response of Eisenmenger syndrome to inhaled nitric oxide during pregnancy. Am J ObstetGynecol 1999;180:64-7
  6. Lam GK, Stafford RE, Thorp J, Moise KJ Jr, Cairns BA. Inhaled nitric oxide for primary pulmonary hypertension in pregnancy. Obstet Gynecol 2001;98:895-8
  7. Barst RJ, Rubin LJ, Long WA, McGoon MD, Rich S, Badesch DB, et al. A comparison of continuous intravenous epoprostenol(prostacyclin) with conventional therapy for primary pulmonary hypertension: The Primary Pulmonary Hypertension Study Group. N Engl J Med 1996;334:296-302
  8. Olschewski H, Walmrath D, Schermuly R, Ghofrani A, Grimminger F, Seeger W. Aerosolized prostacyclin and iloprost in severe pulmonary hypertension. AnnIntern Med 1996;124:820-4
  9. Mikhail G, Gibbs J, Richardson M, Wright G, Khaghani A, Banner N, Yacoub M. An evaluation of nebulized prostacyclin in patients with primary and secondary pulmonary hypertension. Eur Heart J 1997;18: 1499-504
  10. Easterling TR, Ralph DD, Schmucker BC. Pulmonary hypertension in pregnancy: treatment with pulmonary vasodilators. Obstet Gynecol 1999;93:494-8
  11. Weiss BM, Zemp L, Seifert B, Hess OM. Outcome of pulmonary vascular disease in pregnancy: a systematic overview from 1978 through 1996. J Am CollCardiol 1998;31:1650-7
  12. Mccaffrey RM, Dunn LJ. Primary pulmonary hypertension in pregnancy. Obstet Gynecol Surv 1964;19:567-91
  13. Robinson JN, Banerjee R, Landzberg MJ, Thiet MP. Inhaled nitric oxide therapy in pregnancy complicated by pulmonary hypertension. Am J Obstet Gynecol1999;180:1045-6