<Case
Presentation>
A
56-year-old man was admitted to the hospital
because of intolerable orthopnea and paroxysmal nocturnal
dyspnea.
This patient
had a history of diabetes mellitus for more than one year
under regular use of oral hypoglycemic agents. Progressive
dyspnea on exertion and edema of lower legs developed in 1
month before admission. He denied chest pain, symptoms of
upper respiratory infection, or fever. He denied taking
Chinese herbs. On physical examination, the blood pressure was
90/60mmHg, the temperature was 36.6oC, the pulse was 90 and
the respirations were 26. The neck jugular vein was engorged.
Bilateral pulmonary crackles were heard. The heart sounds were
normal. His abdomen was distended with shifting dullness.
There was severe peripheral edema (+++). Electrocardiography
(Fig.
1) and chest X-ray (Fig. 2
) were performed. An echocardiography showed left
ventricular ejection fraction of 45% and concentric left
ventricular hypertrophy. Oral digoxin and intravenous
nitroglycerine were administered.
However, the dyspnea and chest
tightness deteriorated weeks later. A follow-up
echocardiography revealed generalized cardiac hypokinesia and
left ventricular ejection fraction of 30 %. He was sent to the
intensive care unit because of shock status. Low dose dopamine
and angiotensin converting enzyme inhibitor (ACEI) were given
to control his congestive heart failure. Cardiac
catheterization with biopsy was performed. The catheterization
data showed high left and right ventricular end-diastolic
pressure. Restrictive cardiomyopathy was suspected, and
endomyocardial biopsy was performed. The pathology report
showed amyloidosis. Bone marrow biopsy revealed an increased
plasma cell ratio. Under the use of diuretics and dobutamine,
his symptoms improved gradually. The nerve conduction velocity
showed bilateral carpel tunnel syndrome and sensorimotor
neuropathy. The significant laboratory values were summarized
as following.
<Laboratory
values>
- Hematologic laboratory values
Date |
RBC |
Hb |
Hct |
MCV |
Plt |
WBC |
Seg |
Lym |
On admission |
3.26 |
10.3 |
31 |
95.1 |
143 |
5720 |
61.6 |
26.7 |
On 10th hospital day |
2.86 |
9.0 |
27.5 |
96.2 |
170 |
5350 |
64.1 |
22.8 |
On 20th hospital day |
2.7 |
8.4 |
26.6 |
98.3 |
135 |
5430 |
74.7 |
15.5 |
-
Blood chemical values.
Date |
A/G |
Bil-T/D |
GOT |
GPT |
ALP |
GGT |
LDH |
CRP |
On admission |
3.99/4.4 |
0.81/0.3 |
66 |
5 |
88 |
135 |
|
0.95 |
On 10th hospital day |
3.74/3.6 |
0.26 |
28 |
28 |
|
|
|
|
On 20th hospital day |
|
|
|
|
|
|
|
2.18
|
Date |
BUN |
Cre |
Na |
K |
Ca |
Mg |
On admission |
28.6 |
1.02 |
135.9 |
6.76(h3) |
2.23 |
|
On 10th hospital day |
34.5 |
1.59 |
136.8 |
4.4 |
2.19 |
|
On 20th hospital day |
27.5 |
1.26 |
134.6 |
3.93 |
|
|
- On admission CK/CK-MB/Troponin-I:
81/36.8/1.2
- Nerve conduction velocity on 24th hospital day: sensorimotor
polyneuropathy, superimposed with entrapment neuropathy
involving bilateral median nerves at the wrist, carpel
tunnel syndrome.
- Echocardiography:
On admission: LVEF 63%, LA 41
(19-40), AO 23 (20-37), AV 19 (16-26), IVS 16 (7-10), LVPW 18
(8-13), LVEDD 38 (35-53), LVESD 25 (20-35), mild MR, Mild TR,
AV flow 99cm/s (PG 3), MV flow E 111 A 29, Minimal pericardial
effusion On 10th hospital day: LVEF 61%, mitral
flow 112 in inspiration, 96.6 in expiration minimal
pericardial effusion, TR 245 (PG 24)
- Swan-Ganz data on 10th hospital day: C.I. 2.05 SVRI
2220 PVRI 506 PAWP 26 CVP 325
-
Cath data on 5th hospital day: patent coronary
arteries, LVEF 56%, LV 108/31, RV 57/26, thick LV
- Autoimmune profile on 9th hospital day: RA factor
<20, ANA 1:40, C3 87.6, C4 22.8
- Serum IFE on 10th hospital day: a dense
IgA/λmonoclonal gammopathy
- Urine electrophoresis: a monoclonal
gammopathy
- Urine IFE: a dense lambda
Bence-Jones protein
- IgG 594 (1140-1700), IgA 2200
(180-340), IgM 25.2 (90-230), β2-micoglobulin 8.367
(0.7-2.0)
- Anti-HCV (-), HBsAg (-)
病案分析
本病例為一典型之鬱血性心衰竭的患者,其臨床表現包括:dyspnea,orthopnea,paroxysmal
nocturnal dyspnea,neck vein
distension以及extremity
edema等等。慢性之鬱血性心衰竭常伴隨四個房室的擴大,如本病例在胸部X光與心臟超音波所見。下肢水腫雖可見於其他疾病,但同時合併頸靜脈怒張、腹脹與腹水時,再加上腎功能與肝功能正常就幾乎可以排除由心臟以外的疾病所引起的問題。
限制性心肌病變之主要特色為異常的心臟舒張功能。原因為心室壁過度僵硬,而導致心室充填受到阻礙。其可能發生之原因包括:amyloidosis,hemochromatosis,glycogen
deposition,endomyocardial
fibrosis,sarcoidosis等等。各種癌症浸潤引發之心肌病變也是可能因素之一。本病例就是極為罕見的由multiple
myeloma引起的限制性心肌病變。在臨床診斷上,必須仰賴right ventricular transvenous
endomyocardial
biopsy才能確立最後的診斷,治療之預後通常也不理想。
|