66-66.hwp

Size: px

Start display at page:

Download "66-66.hwp"

은경 정
4 years ago
Views:

1 임신중대량객혈로발현된원발성폐고혈압 1 예 가톨릭대학교의과대학내과학교실김명숙, 김형두, 김석찬, 권순석, 김영균, 김관형, 문화식, 송정섭, 박성학 A Case of Primary Pulmonary Hypertension in Pregnancy Presented as Massive Hemoptysis Myung Sook Kim, M.D., Hyoung Doo Kim, M.D., Seok Chan Kim, M.D., Soon Suk Kwan, M.D., Young Kyoon Kim, M.D., Kwan Hyoung Kim, M.D., Hwa Sik Moon, M.D., Jeong Sup Song, M.D. and Sung Hak Park, M.D. Department of Internal Medicine, Daejon St. Mary's Hospital, The Catholic University of Korea 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. (Tuberc Respir Dis 2004; 57:66-67) Key words : Primary pulmonary hypertension, Pregnancy, Hemoptysis. 서 원발성폐고혈압은알려진원인없이폐동맥압과폐혈관저항이상승하는비교적드문질환으로, 평균폐동맥압이안정시 25mmHg 이상이거나운동시 30mmHg 이상인경우폐고혈압으로정의한다. 외관상건강해보이는젊은성인에서서서히진행하는호흡곤란등주로비특이적인증상으로나타나대부분진단이늦어지며, 진단시이미심한폐고혈압의소견을보이는경우가많다. 더욱이임신과동반되는경우, 산모체액의생리적증가는폐동맥압의증가를더욱악화시켜, 심부전을일으키고산모의사망률을증가시킨 론 Address for correspondence : Seok Chan Kim M.D. Ass. Professor Dept. of Internal Medicine, Daejon St. Mary's Hospital, The Catholic University of Korea School of Medicine, 520-2, Dae Heong-Dong, Joong-Gu, Daejon , Korea Phone : Fax : cmcksc@catholic.ac.kr Received : Mar Accepted : May 다. 따라서폐고혈압이있는여성의경우, 임신을피하는것을원칙으로하고있으며, 임신이될경우는가능하면임신의종결을추천하고있다. 저자들은, 임신 27주에대량객혈을주소로내원한여자환자를임상적으로원발성폐고혈압으로진단한후이에대한치료를시행하였으나결국우심부전으로사망한 1예를경험하였기에, 이에문헌고찰과함께보고하는바이다. 증례환자 : 33세여자, 성 주소 : 500cc 가량의대량객혈현병력 : 내원 2개월전부터마른기침이지속되었고, 내원 2주전소량의객혈이한차례발생, 인근병원을경유하여단순흉부방사선사진촬영을시행하였으나특이소견없어경과를관찰하던중, 내원당일발생한대량객혈과호흡곤란을주소로내원하였다. 내원시무월경 27주 5일이었다. 과거력및가족력 : 내원 15개월전의첫분만시를 66

Tuberculosis and Respiratory Diseases Vol. 57. No. 1, Jul, 2004 비롯하여특이사항은없었다.

경정맥의확장소견과흉부청진상좌폐의호흡음감소가있었고, 심음은빈맥이관찰되었으나심잡음은관찰되지않았다. 그외특이소견은관찰되지않았다. 검사실소견 : 내원시말초혈액검사상혈색소 12g/dL, 적혈구용적율 35.

3 mg/dl, 크레아티닌 0.53 mg/dl, 총빌리루빈 0.7 mg/dl, 혈청 AST 16 IU/L, ALT 5 IU/L, 총단백 6.2 g/dl, 알부민 3.6 g/dl 이었고그외검사상특이소견은없었다.

Chest PA radiograph on admission shows mild cardiomegaly with a prominent pulmonary conus and suspicious hazy

4 mmhg, PO 2 56.5 mmhg, HCO - 3 19.9 mmol/l, 산소포화도 91.9% 이었다. Bleeding time(bt) 1.30 min(1-3), Figure 2.

2 Tuberculosis and Respiratory Diseases Vol. 57. No. 1, Jul, 2004 비롯하여특이사항은없었다. 산과력 : 이학적소견 : 내원시혈압 120/70 mmhg, 맥박수분당 120회, 호흡수분당 35회, 체온 36.8 이었고급성병색소견과청색증이관찰되었다. 경정맥의확장소견과흉부청진상좌폐의호흡음감소가있었고, 심음은빈맥이관찰되었으나심잡음은관찰되지않았다. 그외특이소견은관찰되지않았다. 검사실소견 : 내원시말초혈액검사상혈색소 12g/dL, 적혈구용적율 35.3%, 백혈구 12,800/mm 3 ( 호중구 80.7%, 림프구 12.8%, 단핵구 5.5%), 혈소판 83,000/mm 3 이었다. 혈액화학검사상혈청혈당 95 mg/ dl, 요소질소 8.3 mg/dl, 크레아티닌 0.53 mg/dl, 총빌리루빈 0.7 mg/dl, 혈청 AST 16 IU/L, ALT 5 IU/L, 총단백 6.2 g/dl, 알부민 3.6 g/dl 이었고그외검사상특이소견은없었다. 동맥혈가스검사상산소공급없이 ph 7.441, PCO mmhg, PO mmhg, HCO mmol/l, 산소포화도 63.2 % 이었고, 산소 Figure 1.Chest PA radiograph on admission shows mild cardiomegaly with a prominent pulmonary conus and suspicious hazy mottled infiltration in the left lung field, especially the left upper lobe. 마스크로 15L/min 산소공급시 ph 7.48, PCO mmhg, PO mmhg, HCO mmol/l, 산소포화도 91.9% 이었다. Bleeding time(bt) 1.30 min(1-3), Figure 2. Chest CT show irregular lung consolidation in the left upper lobe and mild cardiomegaly with a prominent pulmonary artery. There is no evidence of pulmonary thromboembolism or distinct endobronchial obstructing mass. 67

MS Kim, et al.: A case of primary pulmonary hypertension in pregnancy presented as massive hemoptysis (A) Echocardiogram (B) Doppler Echocardiogram Figure 3.(A) Echocardiogram shows a D-shaped septum.

86), activated Partial thromboplastin time(aptt) 30.7 sec(26-37), Anti-thrombin III 30.6 mg/dl(19-31), Fibrinogen 311 mg/dl(200-400), FDP 10 μg/ ml(0-10), ESR 16 mm/hr(0-20), CRP 0.31 mg/dl(0-0.

3 MS Kim, et al.: A case of primary pulmonary hypertension in pregnancy presented as massive hemoptysis (A) Echocardiogram (B) Doppler Echocardiogram Figure 3.(A) Echocardiogram shows a D-shaped septum. (B) Doppler echocardiogram shows tricuspid regurgitation with an estimated peak pressure gradient of 115mmHg. Prothrombin time(pt) 11.5 sec( )(inr: 0.86), activated Partial thromboplastin time(aptt) 30.7 sec(26-37), Anti-thrombin III 30.6 mg/dl(19-31), Fibrinogen 311 mg/dl( ), FDP 10 μg/ ml(0-10), ESR 16 mm/hr(0-20), CRP 0.31 mg/dl(0-0.5), D-dimer 0.10 μg/ml(0-0.5), Factor V Leiden(nega tive), Homocysteine 9.52 μmol/l( ), Coagu lation Factor V/VIII 57%/115%(50-150/50-150), Antiplatelet Ab. (negative), C mg/dl(90-180), C mg/ dl(10-40), FANA(negative), ANCA (nega tive), Anti- DNA Ab IU/ml(0-7), Anti-Cardio lipin Ab. Ig M/G (negative/negative), Anti-Phospho lipid Ab. Ig M/G(negative/negative) 등의혈액응고인자검사, 면역학적검사, 자가항체검사그리고객담검사등에서특이소견은관찰되지않았다. 심전도상우축편향 (right axis devi ation) 그리고빈맥이관찰되었다. 방사선소견 : 흉부 X-선상경도의심비대, pul monary conus 의확대그리고좌폐의불규칙한경도의다발성경화소견이관찰되었고, 이는특히좌상엽에서잘관찰되었다 (Fig. 1). 흉부전산화단층촬영상좌측주기관지의내강이혈종으로추정되는물질로인해광범위하게그리고불규칙적으로좁아져있었고, 좌상엽에서는불규칙한경화소견그리고양폐야에서는광범위한간유리음영이관찰되어혈액의흡인가능성을추정케하였다. 경도의심비대소견과현저한 폐동맥의확장소견이관찰되었으나, 폐동맥색전이나기관지내종괴등은관찰되지않았다 (Fig. 2). 산과적검사상태음과태동은잘관찰되었다. 치료및임상경과 : 내원직후대량객혈과저산소증에대해 transamin 과산소를이용하여보존적인치료를시행하였다. 내원시관찰된흉부 X-선의경도의심비대와 pulmonary conus 확대소견의평가를위해시행한심장초음파검사상좌심실의수축력은정상이나우심과폐동맥의확장 (dilatation) 그리고 D-sha ped septum 이관찰되었고, 도플러초음파검사상삼첨판역류 (Peak Pressure Gradient: 115mmHg) 와심한폐고혈압 (Pulmonary Systolic Arterial Pressure: 120mmHg) 소견을보였다 (Fig. 3). 이에환자의대량객혈의원인을폐고혈압으로판단하고감별진단을위해혈청학적검사와흉부전산화단층촬영을시행하였다. 중요한감별질환인만성혈전색전성폐고혈압의배제를위해폐관류및환기스캔, 폐혈관조영술등의검사가필요하나, 환자의심한호흡곤란과높은폐고혈압으로인해시행하지못하였다. 그러나환자의병력상특이소견없고, 흉부전산화단층촬영상폐동맥색전소견이관찰되지않았고, D-dimer 값이정상범위이며그외혈액응고인자검사, 면역학적검사, 자가항체검사상정상소견으로미루어, 임상적으로일차성폐고혈압으로진단하였다. 내원 3병일까지더이상의객혈은발생하지않았으나흉부 X-선상좌폐의무기폐소견과폐경화소견이관찰되었다 (Fig. 4). 이후추 68

Tuberculosis and Respiratory Diseases Vol. 57. No. 1, Jul, 2004 Figure 4. Chest PA radiograph on hospital day 3 shows collapse and consolidation of the left lung.

5) 환자의호흡곤란은다시악화되고저산소혈증이지속되어 5병일에기관삽관과기계호흡시행, 7병일에태아의조기자연분만을시행하였고, prostaglandin과 low molecular weight heparin을포함한폐고혈압에대한보존적인치료를지속하였으나, 8병일에우심부전의악화로사망하였다. 사망후부검은시행하지못하였다.

4 Tuberculosis and Respiratory Diseases Vol. 57. No. 1, Jul, 2004 Figure 4. Chest PA radiograph on hospital day 3 shows collapse and consolidation of the left lung. 적흉부 X-선상무기폐와폐경화소견이호전됨에따라환자의호흡곤란증상은점차호전되었고, 동맥혈가스검사상산소마스크로 10L/min 산소공급시 - ph 7.454, PCO mmhg, PO mmhg, HCO mmol/l, 산소포화도 97.4% 이었다. 내원 4병일, 좌폐의무기폐소견은거의회복되었으나 (Fig. 5) 환자의호흡곤란은다시악화되고저산소혈증이지속되어 5병일에기관삽관과기계호흡시행, 7병일에태아의조기자연분만을시행하였고, prostaglandin과 low molecular weight heparin을포함한폐고혈압에대한보존적인치료를지속하였으나, 8병일에우심부전의악화로사망하였다. 사망후부검은시행하지못하였다. 고찰 원발성폐고혈압의발생에가장중요한일차적역할을하는것은폐동맥내피세포로알려져왔다 1. prostacycline 과 nitric oxide 의생산은감소하고 endo thelin 의생산은증가하는형질전환 (phenotypic tran sformation) 을일으켜혈관수축, 혈관구조재형성 (re modeling) 과혈전증을유발하고이로인해폐고혈압이발생하는것으로추측된다 2. 또한폐동맥내피세포는단일클론성증식으로특징적인총상병변 (plexiform lesion) 을구성하기도하고, 이외에 thromboxane, 5- hydroxytryptamine 등여러성장인자들과시토카인들 Figure 5. Cheat PA radiograph on hospital day 4 shows a much improved state of the collapse and consolidation of the left lung. 이혈관구조재형성에관여할것으로주목받고있다 1,3. 폐고혈압환자에서관찰되는또다른특징적인폐혈관의변화는중막비후와혈관외막의섬유모세포증식으로알려져있다. 원발성폐고혈압이있는여성환자는피임이원칙이며이때경구피임제의사용은피해야한다. 임신했을경우에는인공유산을유도하는것이원칙이나, 만약환자가임신을원하는경우는신체적스트레스를유발하거나폐혈관저항을증가시킬수있는상황등은피하고, 산소, 폐혈관확장제, 항응고제등을치료에이용할수있다. 혈관확장제는단기작용혈관확장제를이용한약물검사를시행하여, 심각한부작용없이유의한폐혈관저항의감소효과를얻을수있는환자를선택하여사용해야하겠다. Nifedipine 혹은 diltiazem 등의 calcium channel 차단제뿐만아니라 nitric oxide (NO) 와 prostacyclin(pgi2) 등이치료약제로시도되고있고 4, 최근에는흡입형태로사용하여얻은좋은결과들이보고되고있다. 분만전, 후에사용시관찰되는폐동맥압의감소효과를심도자술로증명한보고와임신중사용시폐고혈압환자의폐동맥압감소와저산소증개선등의효과를발표한증례보고들이 NO사용의근거를제시하나 4-6, 주의해야할사항은혈소판기능억제혹은 methemoglobinemia 발생등이다. Prosta cyclin은혈관확장, 혈관구성세포의성장억제그리고항응고작용을통해혈관확장제에반응이없는환자에 69

5 MS Kim, et al.: A case of primary pulmonary hypertension in pregnancy presented as massive hemoptysis 서도증상을호전시키고운동능력의향상및생존율을증가시킨다고보고하고있다 7. 흡입용 prostacyclin 은비교적전신혈관저항에는적은영향을미치면서좀더효과적으로폐혈관압과폐혈관저항을낮출수있다고보고되고있다 8,9. 이렇듯 prostacyclin의폐고혈압감소효과에대한긍정적인보고가있으나, 일부에서는자궁혈류에대한문제로인하여사용이추천되지않는다는보고 4 도있어, 향후이의사용에대한좀더광범위한연구가필요할것으로사료된다. 폐고혈압이동반된 4명의산모를대상으로한혈관확장제사용결과에대한보고는임신전최소한 1년여간의혈관확장제의사용을추천하고있다 10. 원발성폐고혈압환자는혈전증이폐혈관내에서흔히관찰되는소견으로항응고제의투여가환자의생존률을증가시킨다고알려져모든환자가치료의적응이되나, 항응고제의투여가증상을호전시키거나이미진행된질환을복귀 (regres sion) 시키지는않는다고알려져있다. 원발성폐고혈압이임신과관련되는경우는문헌고찰에의하면 30% 11-56% 12 의사망률을보고하고있다. 임상적악화는대부분임신중기에발생하는데, 40% 가량의생리적체액량의증가로우심실의전부하뿐만아니라후부하의증가를초래하여우심실비후와심기능저하를일으켜증상의악화를유발한다 13. 폐고혈압이있는임신부의사망위험은분만이후 10일이내가가장높다고보고되고있다. 이는임신자궁의압박에서풀려난하대정맥 (inferior vena cava) 으로부터의정맥환류량 (venous return) 의증가로인한우심부전의악화와심근수축력의감소로인한것으로알려져있다 11. 따라서환자의조기발견, 적절한약물선택과환자치료를위한전문적인의료팀의구성등이산모의예후향상을위해필요하리라사료된다. 요약저자들은임신중기에대량객혈로내원하여안정, 산소공급, 폐혈관확장제, 치료적유산그리고항응고제사용등으로치료하였으나결국우심부전으로사망한원발성폐고혈압환자 1예를경험하였다. 본증례는 폐관류및환기스캔, 폐혈관조영술등의검사와사망후부검은시행하지못하였으나현재까지의검사결과에의거시임신중대량객혈로발현된일차성폐고혈압으로사료되어, 이에문헌고찰과함께보고하는바이다. 참고문헌 1. Voelkel NF, Tuder RM. Cellular and molecular mechanisms in the pathogenesis of severe pulmonary hypertension. Eur Respir J 1995;8(12): Christman BW, McPherson CD, Newman JH, King GA, Bernard GR, Groves BM, et al. An imbalance between the excretion of thromboxane and prostacy clin metabolites in pulmonary hypertension. N Engl J Med 1992;327: Lee SD, Shroyer KR, Markham NE, Cool CD, Voelkel NF, Tuder RM. Monoclonal endothelial cell prolifer ation is present in primary but not secondary pul monary hypertension. J Clin Invest 1998;101(5): Monnery L, Nanson J, Charlton G. Primary pul monary hypertension in pregnancy; a role for novel vasodilators. Br J Anaesth 2001;87: Goodwin TM, Gherman RB, Hameed A, Elkayam U. Favorable response of Eisenmenger syndrome to inhaled nitric oxide during pregnancy. Am J Obstet Gynecol 1999;180: Lam GK, Stafford RE, Thorp J, Moise KJ Jr, Cairns BA. Inhaled nitric oxide for primary pulmonary hypertension in pregnancy. Obstet Gynecol 2001;98: Barst RJ, Rubin LJ, Long WA, McGoon MD, Rich S, Badesch DB, et al. A comparison of continuous in travenous epoprostenol(prostacyclin) with conventio nal therapy for primary pulmonary hypertension: The Primary Pulmonary Hypertension Study Group. N Engl J Med 1996;334: Olschewski H, Walmrath D, Schermuly R, Ghofrani A, Grimminger F, Seeger W. Aerosolized prostacyclin and iloprost in severe pulmonary hypertension. Ann Intern Med 1996;124: Mikhail G, Gibbs J, Richardson M, Wright G, Kha ghani A, Banner N, Yacoub M. An evaluation of nebu lized prostacyclin in patients with primary and secondary pulmonary hypertension. Eur Heart J 1997;18: Easterling TR, Ralph DD, Schmucker BC. Pulmonary hypertension in pregnancy: treatment with pulmonary vasodilators. Obstet Gynecol 1999;93:

6 Tuberculosis and Respiratory Diseases Vol. 57. No. 1, Jul, Weiss BM, Zemp L, Seifert B, Hess OM. Outcome of pulmonary vascular disease in pregnancy: a syste matic overview from 1978 through J Am Coll Cardiol 1998;31: Mccaffrey RM, Dunn LJ. Primary pulmonary hyper tension in pregnancy. Obstet Gynecol Surv 1964; 19: Robinson JN, Banerjee R, Landzberg MJ, Thiet MP. Inhaled nitric oxide therapy in pregnancy complicated by pulmonary hypertension. Am J Obstet Gynecol 1999;180:

Trd022.hwp

Trd022.hwp 김신태, 이선녕, 이석정, 정필문, 박홍준, 신명상, 김종환, 이부길, 김상하, 리원연, 신계철, 용석중 Shin-Tae Kim, M.D., Shun Nyung Lee, M.D., Seok Jeong Lee, M.D., Pil Moon Jung, M.D., Hong Jun Park, M.D., Myung Sang Shin, M.D., Chong Whan Kim,