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Primary pulmonary hypertension

1. °³¿ä

M:F = 1:1.7, 20-30´ë

°Ç°­ÇÑ »ç¶÷¿¡¼­ ¼­¼­È÷ È£Èí°ï¶õÀÌ ¹ß»ýÇÏ¸ç ¸»±â¿¡ Áø´ÜµÈ´Ù.

pathognomonicÇÑ º´¸®Àû ¼Ò°ßÀº ¾øÀ¸¸ç ´Ù¾çÇÑ Ç÷°üÀÌ»ó¼Ò°ßÀÌ ³ªÅ¸³­´Ù.

2. º´Å»ý¸®

- pul. blood flow¿¡ ´ëÇÑ resistanceÁõ°¡

- Ãʱ⿣ pulmonary a. pressure°¡ ½ÉÇÏ°Ô Áõ°¡µÇ°í C.OÀº Á¤»óÀ» À¯ÁöÇÏÁö¸¸ ½Ã°£ÀÌ

Áö³ª¸é¼­ pul. a. pr°¡ Áõ°¡µÇ´Â °Í º¸´Ù C.O°¨¼Ò°¡ ´õ ºü¸£°Ô ÁøÇàÇÑ´Ù.

¶Ç Ãʱ⿣ pul. a°¡ vasodilator¿¡ Àß ¹ÝÀÀÇÏÁö¸¸ ÁøÇàÇϸ鼭 Áõ°¡µÈ pul. vascular

resistance°¡ fixµÈ´Ù.

- PCWP´Â ¸»±â±îÁö Á¤»óÀ» À¯ÁöÇÏ´Ù°¡ LV diastolic fillingÀÌ ¼Õ»óµÉ ¶§ Áõ°¡ÇÑ´Ù.

°á±¹ RV failure°¡ ¹ß»ýÇÑ´Ù.

- pulmonary functionÀº mild restrictive patternÀ» º¸À̱⵵ ÇÏÁö¸¸ º¸Åë Á¤»óÀÌ´Ù.

- hypoxemia°¡ ÈçÇѵ¥ ÀÌ´Â V/Q mismatch, low C.O ¶§¹®ÀÌ´Ù.

°£È¤ patent foramen ovale¸¦ ÅëÇÏ¿© Rt-to-Lt shunt°¡ ¹ß»ýÇÏ¿© systemic arterial

desaturationÀ» ÀÏÀ¸Å³¼öµµ ÀÖ´Ù.

3. Áø´Ü Fig 260-1

¼­¼­È÷ È£Èí°ï¶õÀÌ ¹ß»ýÇϸç Áõ»óÀÌ »ý±â°í Áø´Ü½Ã±îÁö Æò±Õ 2.5³âÀÌ °É¸°´Ù.

´Ù¸¥ ÈçÇÑ Áõ»óÀ¸·Î´Â fatigue, AP(=RV ischemia¹Ý¿µ), syncope, near syncope, peripheral

edema

¨ç P/E: JVP¡è, carotid pulse¡é, easily palpable RV lift, P2¡è, right-sided S3, S4, TR

peripheral cyanosis and/or edema(later stage)

clubbing(-)

¨è CXR: enlarged central pul. artery, clear lung field

¨é ECG: RAD, RVH

¨ê Echo: RV enlargment, LV cavity size¡é,

¨ë pul fx: hypoxemia, hypocapnia, DLCO¡é

¨ì HRCT: ¾Ö¸ÅÇÒ ¶§ interstitial lung disease°¡ ÀÖ´ÂÁö È®ÀÎÇϱâ À§ÇØ ½ÃÇà

¨í perfusion lung scan: normal or abnormal with multiple diffuse patchy filling defects of

a nonsegmental nature

¨î pul. angiography -°Ë»ç½Ã ÁÖÀÇ

vagally mediated bradycardia¸¦ ¿¹¹æÇϱâ À§ÇØ atropine 1mgÀ» ÀüóġÈÄ ½ÃÇàÇϸç

low-osmolar, nonionic contrast materialÀ» ¼Ò·®»ç¿ëÇÏ¿© °Ë»çÇÑ´Ù.

¨ï cardiac cath: underlying cardiac shuntÀÖ´ÂÁö È®ÀÎÇϱâ À§ÇØ ½ÃÇàÇÑ´Ù.

Rt-to-Lt shunt: patent foramen ovale

Lt-to-Rt shunt: congenital defect°¡ ÀÖÀ½À» ÀǹÌ

PCWP°¡ Áõ°¡ÇØ ÀÖ´Ù¸é MS or LVEDPÁõ°¡°¡ ÀÖ´ÂÁö È®ÀÎÇϱâ À§ÇØ Lt heart cathÀ»

½ÃÇàÇØ¾ß ÇÑ´Ù.

4. Natural history

Æò±Õ »ýÁ¸±â°£Àº 2-3³â

functional calss¿¡ ´Þ·ÁÀÖ´Ù.

NYHA class II, III: 3.5³â, NYHA class IV: 6°³¿ù

*»ç¸Á¿øÀÎ : RV failure or sudden death(¸»±â¿¡)

*poor prognosis : RA pressure>15mmHg, C.I<2L/min/m2

5. Ä¡·á

¿îµ¿½Ã¿£ pul. vascular resistance°¡ Áõ°¡ÇϹǷΠÁ¶½ÉÇϵµ·Ï Çϰí, digoxinÀº C.OÀ»

Áõ°¡½Ã۰í circulating norepinephrine levelÀ» ³·Ãá´Ù.

dyspnea, peripheral edema°¡ ÀÖÀ» ¶§ diuretics°¡ µµ¿òÀÌ µÈ´Ù.

* primary pulmonary hypertensionÀ¸·Î Áø´ÜµÈ ¸ðµç ȯÀÚ¿¡¼­ short-acting pul. vasodilator

¸¦ ÀÌ¿ëÇÑ acute drug test¸¦ ÇØ¾ß ÇÑ´Ù.

i) IV adenosine ii) IV prostacyclin iii) inhaled N.O

1) calcium channel antagonist

high dose(nifedipine 120-240mg/d or diltiazem 540-900mg/d)ÇÊ¿ä

pul. a. pr & pul. vascular resistance°¡ »ó´çÈ÷ °¨¼ÒÇÑ È¯ÀÚ´Â Áõ»óÀÌ È£ÀüµÇ°í, RVH°¡

regressionµÇ¸ç chronic tx½Ã survivalÀÌ Çâ»óµÈ´Ù. ±×·¯³ª ÀÌ·± ȯÀÚ´Â ¹Ý¼ö¹Ì¸¸ÀÌ´Ù.

Á¶±â¿¡ Áø´ÜµÇ¾úÀ» ¶§, less advanced disease¿¡¼­ º¸´Ù ¼º°øÀûÀÌ´Ù.

2) prostacyclin

functional class III or IV & convential tx¿¡ ¹ÝÀÀÇÏÁö ¾ÊÀ» ¶§ »ç¿ë

ºÎÀÛ¿ë: flushing, jaw pain, diarrhea, ´ëºÎºÐ tolerable

¿ÀÁ÷ IV·Î¸¸ Åõ¿©°¡´ÉÇϹǷΠpermanent central vein catheter°¡ ÇÊ¿äÇÏ´Ù.

µû¶ó¼­ Ä¡·áÀÇ ÁÖ ¹®Á¦´Â catheter¿Í °ü·ÃÇÑ infectionÀ̹ǷΠÁÖÀǰ¡ ÇÊ¿äÇÏ´Ù.

Àå±â»ç¿ë½Ã thrombocytopenia & serial foot pain°ú °°Àº ºÎÀÛ¿ëÀÌ ÀÖÀ»¼ö ÀÖ´Ù.

3) anticoagulant(warfarin)Àº »ýÁ¸·üÀ» Çâ»ó½ÃŰ¹Ç·Î ¸ðµç ȯÀÚ¿¡¼­ Åõ¿©ÇØ¾ß ÇÑ´Ù.

(INR 2.0-2.5)

disease regression½ÃŰ°Å³ª Áõ»óÀÇ º¯È­¸¦ ÀÏÀ¸Å°Áø ¾Ê´Â´Ù.

4) transplantation

À̽ÄÈÄ Àç¹ßÀº º¸°íµÇÁö ¾Ê¾Ò´Ù.