Download Preoperative Alcohol Septal Ablation for Hypertrophic Obstructive

Case Report
HOCM and Pheochromocytoma
Acta Cardiol Sin 2010;26:189-92
Preoperative Alcohol Septal Ablation for
Hypertrophic Obstructive Cardiomyopathy
before Surgical Resection of Pheochromocytoma:
The First Medical Report
Jia-Feng Chang,1 Chih-Ming Lin,2 Chi-Hung Huang,1 Ching-Ling Lin,3 Shih-Hung Huang4 and Chih-Hui Chin1
A 50-year-old woman was admitted to the hospital because of chest pain accompanied with cold sweating,
palpitation and extremely high blood pressure. Transthoracic echocardiography (TTE) revealed systolic anterior
motion of mitral valve, with peak velocity crossing the left ventricular outflow tract (LVOT) about 4.9 m/s and
severe mitral regurgitation (MR). The study for secondary hypertension revealed high urine vanillylmandelic acid
(82.57 mg/24 hrs) and norepinephrine (916.5 ug/24 hrs). A huge pheochromocytoma was found by abdominal
computed tomography and magnetic resonance imaging. During the hospitalization, her blood pressure could
fluctuate from 188/122 mmHg to 89/46 mmHg, depending on fluid challenge, within three hours with chest pain and
cold sweating. Thus, we decided to apply alcohol septal ablation therapy to provide a more stable hemodynamic
status for tumor resection. TTE was done one week post alcohol septal ablation and showed the peak velocity
crossing the LVOT declined to 2.3 m/s and only mild MR. Subsequently, the patient successfully received surgical
resection and was discharged under long-term outpatient clinic follow-up.
Key Words:
Alcohol septal ablation · Hypertrophic obstructive cardiomyopathy · Pheochromocytoma
CASE REPORT
tum thickness was 17 mm and posterior wall thickness
was 12 mm) with systolic anterior motion of the anterior
mitral valve. The peak velocity cross left ventricular outflow tract (LVOT) was 2.0 m/s at that time. Her baseline
blood pressure had been well-controlled around 130/80
mmHg without significant discomfort in the past years.
Nevertheless, she suffered from intermittent high blood
pressure above 220/110 mmHg with dizziness and had
had several episodes of non-admitted patient emergency
department service since the age of forty-eight. Two
weeks prior to this admission, chest pain, headache, and
palpitation developed in addition to high blood pressure.
Subsequently, the patient received cardiac catheterization under the first impression of acute coronary syndrome, which showed patent coronary artery and hypertrophic obstructive cardiomyopathy. Transthoracic echocardiography (TTE) revealed systolic anterior motion of
mitral valve with peak velocity crossing the LVOT of
A 50-year-old woman was admitted to the hospital
because of chest pain accompanied with cold sweating,
palpitation and extremely high blood pressure. The patient had previously been diagnosed with essential hypertension and started on antihypertensive treatment
when she was thirty-nine years old. Five years later, her
two-dimensional transthoracic echocardiography revealed hypertrophic obstructive cardiomyopathy (sep-
Received: October 7, 2009
Accepted: March 25, 2010
1
Division of Cardiology; 2Division of Urology; 3Division of Endocrine;
4
Pathology, Cathay General Hospital, Taipei, Taiwan.
Address correspondence and reprint requests to: Dr. Chih-Hui Chin,
Division of Cardiology, Department of Medicine, Cathay General
Hospital, No. 280, Section 4, Jen-Ai Road, Taipei 106, Taiwan.
Tel: 886-2-2708-2121 ext. 3118; Fax: 886-2-2932-4969; E-mail:
[email protected]
189
Acta Cardiol Sin 2010;26:189-92
Jia-Feng Chang et al.
hrs). The physical examination found a huge abdominal
mass. When touching the abdominal mass, the patient
felt palpitation, headache and sweating. The blood pressure was extremely increased from 128/86 mmHg to
220/130 mmHg, and heart rate was increased from 78
bpm to 116 bpm. Under the impression of pheochromocytoma, a series of imaging studies were done. The
abdominal computed tomography demonstrated a welldefined mass of size about 7.4 ´ 7.0 ´ 5.4 cm with calcification, central necrosis and heterogeneous density at
the right retroperitoneum. The abdominal magnetic resonance imaging also displayed a right retroperitoneal tumor, 7.6 ´ 7.6 ´ 5.9 cm in size with central necrosis
(Figure 2A). Since all the laboratory and imaging studies
were compatible with the diagnosis of pheochromocytoma, surgical intervention was recommended. During
the hospitalization, the patient’s blood pressure could
fluctuate from 188/122 mmHg to 89/46 mmHg depend-
about 4.9 m/s (Figures 1A and B). Septum thickness was
22 mm, posterior wall thickness was 14 mm, left ventricular ejection fraction was 68% and mitral regurgitation (MR) was severe. Because of extremely high
blood pressure, study for secondary hypertension was
done. On the day of admission, the patient had severe
chest pain that was accompanied by cold sweating and
palpitation. The pulse was 95 beats per minute, blood
pressure 262/143 mmHg, respiratory rate 20 breaths per
minute, and temperature 35.3 °C. Electrocardiography
presented sinus rhythm, left ventricular hypertrophy and
ST segment depression with T-wave inversion in leads
II, III, aVF, and V3 through V6. Chest radiography
showed no abnormalities except cardiomegaly. The values of creatinine, sodium, potassium and cardiac enzyme were within normal range. The study for secondary hypertension revealed high urine vanillylmandelic
acid (82.57 mg/24 hrs) and norepinephrine (916.5 ug/24
A
B
C
D
Figure 1. Transthoracic echocardiography (TTE) revealed systolic anterior motion of mitral valve (A) with peak velocity cross left ventricular
outflow tract (LVOT) about 4.9 m/s (B). One week after alcohol septal ablation, systolic anterior motion of mitral valve was much better (C) and peak
velocity cross LVOT declined to 2.3 m/s (D).
Acta Cardiol Sin 2010;26:189-92
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HOCM and Pheochromocytoma
A
B
Figure 2. (A) The abdominal magnetic resonance imaging displayed a right retroperitoneal tumor, 7.6 ´ 7.6 ´ 5.9 cm in size with central necrosis.
(B) Pathology revealed that tumor cells possess mildly to moderately pleomorphic nuclei and abundant finely granular eosinophilic or amphophilic
cytoplasm with nucleolation and few mitotic activity, H&E, x400.
ization, myxoid change and cystic change were noted. In
immunohistochemical study, the tumor cells are positive
for synaptophysin, chromogranin and CD56, and focally
positive for S-100 protein. Taking the histopathological
picture and the results of immunostain together, it is a
paraganglioma. All of the three dissected lymph nodes
revealed no evidence of metastasis.
ing on fluid challenge within three hours with chest pain
and cold sweating. Thus, we decided to apply for alcohol
septal ablation therapy to provide a more stable hemodynamic status for tumor resection. On the fourth hospital day, percutaneous transluminal septal myocardial ablation therapy was performed smoothly. Before the procedure, left ventricular pressure was 205/7 mmHg, aortic
pressure was 115/72 mmHg, and heart rate was 84 bpm.
After procedure, left ventricular pressure was 203/12
mmHg, aortic pressure was 173/104 mmHg and heart
rate was 88 bpm. The pressure gradient crossing the left
ventricular outflow tract decreased from 90 mmHg to 30
mmHg immediately. The peak creatine kinase and creatine kinase MB were 926 U/liter and 238.8 ng/ml post
alcohol septal ablation, respectively. TTE was done one
week post alcohol septal ablation and showed the peak
velocity crossing the LVOT declined to 2.3 m/s and systolic anterior motion of mitral valve was better then
pre-ablation (Figures 1C and D). Subsequently, she successfully received surgical resection and discharged under long-term outpatient clinic follow-up. After postoperative 8 months, echocardiography was repeated and
the data showed that septum thickness was 15 mm, posterior wall thickness was 12 mm, peak velocity crossing
the LVOT was 1.9 m/s, left ventricular ejection fraction
was 62% and MR was mild. The pathology revealed that
tumor cells possess mildly to moderately pleomorphic
nuclei and abundant finely granular eosinophilic or
amphophilic cytoplasm with nucleolation and little mitotic activity (Figure 2B). Focal hemorrhage, hyalin-
DISCUSSION
Hypertrophic obstructive cardiomyopathy (HOCM)
is characterized by asymmetric left ventricular hypertrophy and dynamic LVOT obstruction, which can impair blood flow into and out from left ventricle, leading
to progressive heart failure and sudden death in some
patients. 1,2 The overall probability of death related to
hypertrophic cardiomyopathy was significantly greater
among patients with outflow tract obstruction than among
those without obstruction.3 Studies report higher perioperative risk of inhospital mortality and myocardial infarction associated with noncardiac surgery in patients
with hypertrophic cardiomyopathy. 4 Accordingly, patients with HOCM undergoing elective procedures may
require more careful preoperative assessment, careful
planning in anesthesia and perioperative monitoring to
minimize morbidity and mortality. Also perioperative
morbidity and mortality in patients with pheochromocytoma were 23.6% and 2.4%, respectively.5 There is a
paucity of reports about complications of pheochro191
Acta Cardiol Sin 2010;26:189-92
Jia-Feng Chang et al.
REFERENCES
mocytoma associated with clinical and echocardiographic features simulating HOCM. 6,7 Both pheochromocytoma and HOCM can lead to perioperative morbidity and mortality, therefore, patients who suffer from
both diseases are at especially high risk for surgery. According to preoperative assessment of the patient, the
peak velocity crossing the LVOT by was up to 4.9 m/s,
with severe MR. Her clinical paroxysmal blood pressure
fluctuation was stressful and made us hesitate to administer the preoperative medicine of alfa-adrenergic
blockade. As a result, we decided to perform alcohol
septal ablation therapy for relative hemodynamic stability. After the septal myocardial ablation, there was neither hypotension nor complication. Therefore, alcohol
septal ablation of HOCM in such condition may be one
of the options.
Systemic hypertension due to pheochromocytoma
may be associated with left ventricular hypertrophy that
is often concentric and symmetric.8-10 However, a few
cases simulating HOCM revealed asymmetric septal hypertrophy, systolic anterior motion of mitral valve, MR
and dynamic LVOT obstruction. 5 Huddle et al. found
only partial regression of abnormal septum and posterior
wall thickness in patients with pheochromocytoma simulating asymmetric septal hypertrophy. The present case
also showed asymmetric septal hypertrophy and partial
regression of abnormal septum and posterior wall thickness after successful removal of pheochromocytoma.
Acta Cardiol Sin 2010;26:189-92
1. Wigle ED, Rakowski H, Kimball BP, et al. Hypertrophic cardiomyopathy: clinical spectrum and treatment. Circulation 1995;
92:1680.
2. Maron BJ. Hypertrophic cardiomyopathy: a systematic review.
JAMA 2002;287:1308.
3. Maron MS, Olivotto I, Betocchi S, et al. Effect of left ventricular
outflow tract obstructionon clinical outcome in hypertrophic
cardiomyopathy. N Engl J Med 2003;348:295-303.
4. Hreybe H, Zahid M, Sonel A, et al. Noncardiac surgery and the
risk of death and other cardiovascular events in patients with
hypertrophic cardiomyopathy. Clin Cardiol 2006;29:65-8.
5. Plouin PF, Duclos JM, Soppelsa F, et al. Factors associated with
perioperative morbidity and mortality in patients withpheochromocytoma: analysis of 165 operations at a single center. J Clin
Endocrinol Metab 2001;86:1480-6.
6. Huddle KR, Kalliatakis B, Skoularigis J. Pheochromocytoma associated with clinical and echocardiographic features simulating
hypertrophic obstructive cardiomyopathy. Chest 1996;109:1394-7.
7. Kassim TA, Clarke DD, Mai VQ, et al. Catecholamine-induced
cardiomyopathy. Endocr Pract 2008;14:1137-49.
8. Mardini MK. Echocardiographic findings in pheochromocytoma.
Chest 1982;81:394-5.
9. Yang YN, Chuang YC, Yin WH, et al. Catecholamine-induced
cardiomyopathy secondary to pheochromocytoma mimicking
fulminant acute myocarditis. Acta Cardiol Sin 2007;23:125-30.
10. Lin CT, Rim R, Lin TK, et al. Hypertrophyic cardiomyopathy associated with midventricular obstruction and apical aneurysm.
Acta Cardiol Sin 2008;24:221-5.
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