An unusual case of dilated cardiomyopathy associated with partial hypopituitarism
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Transcript of An unusual case of dilated cardiomyopathy associated with partial hypopituitarism
CE - LETTER TO THE EDITOR
An unusual case of dilated cardiomyopathy associatedwith partial hypopituitarism
Alberto Maria Marra • Michele Arcopinto •
Emanuele Bobbio • Andrea Salzano •
Luigi Sacca • Antonio Cittadini
Received: 22 February 2011 / Accepted: 14 June 2011 / Published online: 29 June 2011
� SIMI 2011
Dilated cardiomyopathy (DCM) secondary to endocrino-
logic disease occurs rarely. In a large clinicopathological
review of 673 patients, only 1.5% fall in the metabolic
category, mostly due to thyroid disorders [1]. Sporadic
reports of DCM associated with other endocrinopathies
have been subsequently published including acromegaly,
GH deficiency, pheochromocytoma, hypoparathyroidism,
Sheehan syndrome, and Addison’s disease.
We herein describe a rare case of DCM secondary to
partial hypopituitarism, in turn related to a previous intra-
cranial surgery, promptly responding to multiple hormonal
replacement therapy.
A 55-year-old woman was admitted to our Intensive
Coronary Unit in February 2008 because of a community-
acquired pneumonia complicated by acute heart failure.
Her chief complaint was shortness of breath, which had
become progressively worse during the prior 4–5 days. She
had a productive yellow cough and blood-tinged sputum.
Examination revealed a tachycardia (115 bpm), tachypnea
22 breaths/min, BP 90/50 mmHg, a raised venous pressure,
fine bilateral basal crepitations, right-sided crackles, and
dullness to percussion. She was treated conventionally in a
territorial hospital for acute pulmonary edema with partial
initial symptomatic recovery. The patient was also
started on a ‘‘pneumonia protocol’’ with cefotaxime and
azithromycin, and oxygen. Notwithstanding standard ther-
apy for acute heart failure including nitrates, furosemide
and digoxin, and the introduction of inotropic support, the
patient was still hypotensive, and for this reason she was
admitted to the intensive care unit of our tertiary care
hospital. The past medical history revealed systemic
hypertension, chronic kidney disease (GFR of 25 ml/min),
subclinical hypothyroidism (TSH 6.3 lU/mL with normal
FT3 and FT4), and surgical intervention for aneurysmec-
tomy of the left middle cerebral artery, which had been
performed in 2006. She never smoked or used alcohol
EtOH or drugs. She lived with her family.
A complete mono-two-dimensional and Doppler echo-
cardiographic examination was performed. The ultrasound
analysis displayed a very enlarged poorly contracting left
ventricle (EF 13%) with moderate-to-severe mitral regur-
gitation (see Fig. 1; Table 1). Diastolic function was
moderately impaired. Interestingly, a previous echocar-
diogram obtained in 2006 was reviewed, and only dis-
played mild LV hypertrophy with normal cavity diameters
and a preserved systolic function (EF 55–60%).
After resolving the decompensated HF and the right
pneumonia, the patient was still in class IV of the NYHA,
and could not undergo a cardiopulmonary stress test that
we routinely perform in CHF patients. To rule out ischemic
etiology, a coronary angiography was performed that
revealed no significant coronary stenosis. An endomyo-
cardial biopsy was suggested but not performed since the
patient refused the procedure.
A complete hormonal panel showed low levels of early-
morning serum cortisol and undetectable levels of serum IGF-
1 without evidence of secondary gonadal failure (Table 1).
Thyroid failure was partial insofar as TSH increased up to
10.9 lU/mL, indicating residual pituitary secretion. We next
performed a GHRH ? arginine stimulation test for diagnosis
A.M. Marra and M. Arcopinto contributed equally to this work.
A. M. Marra � M. Arcopinto � E. Bobbio � A. Salzano �L. Sacca � A. Cittadini
Department of Clinical Medicine and Cardiovascular and
Immunological Sciences, University Federico II, Naples, Italy
A. Cittadini (&)
Department of Internal Medicine and Cardiovascular Sciences,
University Federico II, Via Sergio Pansini 5, 80131 Naples, Italy
e-mail: [email protected]
123
Intern Emerg Med (2012) 7 (Suppl 2):S85–S87
DOI 10.1007/s11739-011-0649-9
of acquired GH deficiency. A CT scan of pituitary region did
not show significant morphologic alterations. No evidence
was found of diabetes insipidus.
On top of optimized therapy for CHF that included beta-
blockers, loop diuretics, CEI, nitrates, and low doses of
aldosterone receptor antagonists, we added the following
hormone replacement therapy: cortisone acetate 37.5 mg/day
and levotyroxin 12.5 mg/day for the first 30 days and then
25 mg/day. After 2 weeks, recombinant human GH (rhGH)
was started at the replacement dose 0.25 IU/kg/week. The
patient was re-evaluated after 6 months, and showed a
remarkable improvement of clinical status and cardiovascular
performance. She gained weight, approximately 2–3 kg,
NYHA class shifted from IV to I, BP rose to 110/80 mmHg,
and GFR increased from 25 to 50 ml/min. The hormonal
profile displayed the normalization of the main axes as
depicted in the table. Echocardiography detected a dramatic
increase of both systolic and diastolic function. Cavity
dimensions decreased accordingly, and particularly impres-
sive was the change in the end-systolic LV volume (see
Fig. 1). Cardiopulmonary stress test was performed, and the
patient displayed a markedly improved performance.
The current case represents a very unusual DCM with
severe symptomatic CHF in a patient with hypopituitarism
promptly responding to multiple replacement therapy. The
peculiarities of the case report are many and include the
following:
Fig. 1 Transthoracic echocardiography performed at baseline and after 6 months of hormone replacement therapy
Table 1 Echocardiography, cardiopulmonary exercise testing and
hormones at baseline and after 6 months of therapy
Baseline After
6 months
Heart rate (bpm) 110 80
Systolic/diastolic blood pressure (mmHg) 90/50 110/70
End-Systolic volume (mL) 128 63
End-Diastolic volume (mL) 148 103
Ejection Fraction (%) 13 38
E/A ratio 1.57 0.82
E/E0 19 4.7
TSH (lU/mL) 10.9 7.63
FT3 (pg/mL) 1 1.6
FT4 (ng/dL) 0.27 0.42
Early-morning cortisol (lg/dL) 9.98 27.2
IGF-1 (ng/mL) Unfeasible 140
ACTH (pg/mL) 9.88 20.1
Maximal oxygen consumption (ml/kg/min) Unfeasible 14.4
Workload (watt) Unfeasible 39
S86 Intern Emerg Med (2012) 7 (Suppl 2):S85–S87
123
(1) to our knowledge this is the first case of partial
hypopituitarism associated with dilated cardiomyopa-
thy, insofar as all previously reported cases only describe
cardiomyopathies following panhypopituitarism;
(2) although hypopituitarism of various degree may
develop in patients undergoing neurosurgery even
far from hypothalamic–pituitary region, most diseases
occur following excision of primary brain tumors and
not because of aneurysmectomy;
(3) the response to the replacement therapy was impres-
sive, to a larger extent than reported so far;
(4) at variance with previous reports, we started almost
simultaneously glucocorticoid, thyroid, and GH
replacement therapy, and the GH dose was lower.
The most likely explanation for the hypopituitarism is the
previous intracranial surgery. Indeed, Ghigo et al. [2] report
that hypopituitarism of various degrees may develop 1 year
after neurosurgery even in areas far from the hypothalamic–
pituitary region because of the particular fragility of these
structures. Most cases were secondary to primary brain
tumors, and the incidence of hypopituitarism is 43.2%.
Very few cases have been described so far linking
hypopituitarism to dilated cardiomyopathy. Cuneo et al. [3]
describe a case of a 53-year-old man in whom a total
hypophysectomy was performed for Cushing’s disease.
Then Frustaci et al. [4] describe a case of a 49-year-old
woman with clinical and biochemical signs of panhypopi-
tuitarism who presented with severe DCM. Fazio et al. [5]
report a case of CHF in a 48-year-old woman with a history
of hypopituitarism treated only with partial replacement
therapy. While the interaction between the GH/IGF-1 axis
is well recognized, the role of chronic hypothyroidism in
the development of severe heart failure is less often
described. Most cases of acute hypothyroidism in fact
mimic acute coronary syndromes rather than dilated
cardiomyopathy.
We are aware that association of partial hypopituitarism
and dilated cardiomyopathy does not imply a causal rela-
tionship. In this regard, we cannot exclude that the standard
therapy for CHF significantly contributed to the cardio-
vascular improvement of our patient. Doubts also remain as
to which of the hormone replacement therapies had the
major impact on the cardiovascular system.
Conflict of interest None.
References
1. Kasper EK, Agema WR, Hutchins GM, Deckers JW, Hare JM,
Baughman KL (1994) The causes of dilated cardiomyopathy: a
clinicopathologic review of 673 consecutive patients. J Am Coll
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Scaroni C, Cannavo S, Di Somma C, Mantero F, degliUberti EC,
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with primary brain tumors 1 year after neurosurgical treatment:
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3. Cuneo RC, Wilmshurst P, Lowy C, McGauley G, Sonksen PH
(1989) Cardiac failure responding to growth hormone. Lancet
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specific replacement therapy. J Clin Endocrinol Metab 81:887–890
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