[Case
presentation]
This 37 year-old woman was sent to the ED due to
progressive dyspnea, abdominal distension and puffy face for
one month.
According to patient’s statement, she denied any other
systemic diseases, including hypertension, diabetes mellitus,
or asthma history. She didn’t smoke or drink. Her mother died
of primary pulmonary hypertension at the age of 65 year-old,
two years after the diagnosis.
She had an uneventful life until she suffered from
progressive exertional dyspnea, easy fatigability 5 years ago.
She had childbirth at the age of 24 and 27 year-old and there
were no complications found. Initially, she came to a local
clinic and no definite diagnosis was told. Due to progressive
dyspnea, she visited to a medical center, where pulmonary
hypertension was informed after workups. Her symptoms improved
a little after medication including diuretics, and digoxin.
However, she lost of follow-up one year later, and begun to
take Chinese herb drugs since then.
On arrival at our ED, her blood pressure was 110/70 mmHg,
body temperature was 36 oC, respiratory rate was 30/ min and
pulse rate was 80/ min. Her consciousness was clear. The
pupils were isocoric and prompt to light reflex. The
conjunctiva was not pale and the sclera was slightly icteric.
The neck was supple, and jugular veins were engorged. There
was neither goiter nor lymphadenopathy noted. The breathing
sounds were clear. The heartbeats were regular. RV heave was
noted. Narrowing of S2 splitting, P2 accentuation and grade
II/VI midsystolic murmur were heard at left lower sternal
border. The abdomen was soft and distended. Shifting dullness
was noted. Liver span was 14 cm along the right mid-clavicle
line. Mild tenderness was at right upper quadrant area and
hepato-jugular reflex was noted. There was pitting edema was
noted over her legs.
Her hemogram was within normal limit. (WBC 5000/μL, Hb
12g/dL, Plt 118K/μL). Abnormal liver function was noted.
(T-Bil 2.8mg/dl, GOT 150 U/L, GPT 168 U/L). Arterial blood gas
disclosed respiratory alkalosis under O2 nasal cannula 4L/min.
[pH 7.31, PCO2 24.1 mmHg, PO2 77.3 mmHg, HCO3- 16 mEq/l, Base excess –1.1mEq/l].
Normal plasma D- dimmer level was disclosed by ELISA . Chest X
ray [Fig
1] and CT [Fig
2] showed clear field, increased cardiac size and
pulmonary trunk enlargement. ECG disclosed RV strain [Fig
3].
Pulmonary ventilation and
perfusion scan disclosed low probability of pulmonary
embolism. Transthoracic UCG showed dilated right ventricle,
moderate TR, and RV systolic hypertension, and no evidence of
intra-cardiac shunt or other anomaly. A cardiac cath was
performed, and pulmonary hypertension was noted (MPA
74/38mmHg).
Under the impression of primary pulmonary hypertension,
oxygen, diuretics and digoxin were described. Continuous
infusion of prostacyclin was tried, and her condition
gradually stabilized. She was discharged smoothly with the
prostacyclin infusion pump.
[Discussion]
Primary pulmonary hypertension
(PPH),泛指所有原因不明之肺動脈高壓症。診斷前需排除次發性之原因,包括肺部疾病 (如吸煙病史、慢性組塞性肺病
(COPD)、肺間質病變 (interstitial lung disease)等),心臟疾病
(如先天性心臟病、瓣膜性疾病等),自體免疫性疾病、HIV或藥物
(fenfluramine-phentermine)等原因引起之肺動脈高壓症。PPH之病理變化為肺動脈及肺小動脈之medial
hypertrophy及其他之血管變化,包括肺小動脈原位性血栓 (thrombotic pulmonary
arteriopathy)。
PPH之盛行率不明,發病族群以女性為多
(佔63%),年齡以20至40歲之間為主,最常見之初期症狀為氣促,約佔80%,疲倦、昏倒或雷諾氏現象 (Raynaud
phenomenon) 也多有報告。部分病人有家族史,為Autosomal
Dominant方式遺傳,但penetrance則不一定。
臨床之症狀表現以右心衰竭為主,理學檢查以P2心音增強,三尖瓣逆流及週邊水腫為多見。最常見死因為右心衰竭
(47%),而猝死 (sudden death) 亦佔有26 %,且絕大部分發生於NYHA Functional class
IV的患者中。
在實驗室檢查中,大部分之檢查均為正常範圍內;當肝功能異常時,多為右心衰竭後,引起的肝臟充血所致。心電圖以右心房心室擴大引起之變化為多。CXR中可能可見main
pulmonary artery
及其分枝之擴大,及肺部血流減少之現象。在心臟超音波中,以右心擴大,左心大小正常或略小,三尖瓣逆流及右心室收縮壓升高之表現為主。然而這些檢查均無絕對之專一性,仍須由臨床表現或其他檢查排除次發性肺動脈高壓疾病。而右心導管之檢查仍為目前判定肺動脈高壓有無之黃金標準,並可同時排除許多心臟疾病及肺動脈栓塞疾病的存在。
目前的治療方式,包括O2 therapy, digoxin, diuretics 等。至於vasodilator
therapy,根據目前文獻,則可能只對部分之病人有效。
可利用adenosine、prostacyclin或NO測試立即之反應,而對有反應之病人可考慮長期給calcium
channel blockers 或 continuous prostacyclin infusion
therapy。至於抗凝血劑之效果則不明。至於接受heart-lung 或 lung
transplantation的時機,則應該在病人處於NYHA Fc
III或IV,對藥物治療反應不佳,或藥效反應開始減弱時,開始考慮並接受移植之評估。
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