Hypocalcaemia can have a variety of manifestations including cardiovascular changes. Hypocalcemic cardiomyopathy has been reported in hypoparathyroidism and vitamin D deficiency but association of hypocalcemic cardiomyopathy in pseudohypo-parathyroidism has been reported scarcely in literature. We describe here a case of PHP presenting as congestive cardiac failure

Kaushik, ML

Kumar, B

Mahajan, S

Marwah, R

Raina, R

Tomar, A

Hypocalcaemia can have a variety of manifestations including cardiovascular changes. Hypocalcemic cardiomyopathy has been reported in hypoparathyroidism and vitamin D deficiency but association of hypocalcemic cardiomyopathy in pseudohypoparathyroidism has been reported scarcely in literature. We describe here a case of PHP presenting as congestive cardiac failur

Madan Lal Kaushik

Sanjay Mahajan

Rajiv Raina

Bhupender Kumar

Ashwani Tomar

Rajeev Marwah

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Pseudohypoparathyroidism Presenting as Congestive Cardiac Failure.

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Pseudohypoparathyroidism Presenting as Congestive Cardiac Failure

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Case Report: 
Pseudohypoparathyroidism Presenting as Congestive Cardiac Failure. 
Authors 
Madan Lal Kaushik, Department of Medicine, 
Sanjay Mahajan, Department of Medicine, 
Rajiv Raina, Department of Medicine, 
Bhupender Kumar, Department of Medicine, 
Ashwani Tomar, Department of Radiodiagnosis, 
Rajeev Marwah, Department of Cardiology, 
IG Medical College, Shimla-171 001, Himachal Pradesh, India. 
Address for Correspondence 
Dr. Madan Lal Kaushik, 
Assistant Professor, 
Department of Medicine, 
IG Medical College, 
Shimla-171 001, 
Himachal Pradesh, India.  
E-mail: madankaushik2002@yahoo.com 
Citation 
Kaushik ML, Mahajan S, Raina R, Kumar B, Tomar A, Marwah R. Pseudohypoparathyroidism Presenting as Congestive Cardiac 
Failure. Online J Health Allied Scs. 2013;12(1):7. Available at URL: http://www.ojhas.org/issue45/2013-1-7.html 
Open Access Archives 
http://cogprints.org/view/subjects/OJHAS.html 
http://openmed.nic.in/view/subjects/ojhas.html 
Submitted: Jan 5, 2013; Accepted: Apr 3, 2013; Published: Apr 15, 2013 
Abstract: Hypocalcaemia can have a variety of 
manifestations including cardiovascular changes. 
Hypocalcemic cardiomyopathy has been reported in 
hypoparathyroidism and vitamin D deficiency but association 
of hypocalcemic cardiomyopathy in pseudohypo-
parathyroidism has been reported scarcely in literature. We 
describe here a case of PHP presenting as congestive cardiac 
failure. 
Key Words: Pseudohypoparathyroidism; Hypocalcaemia; 
Cardiomyopathy; Vitamin D 
 
Introduction: 
Plasma calcium level may be decreased due to insufficient 
Parathormone production by the parathyroids 
(hypoparathyroidism), or a resistance against its action in 
target tissues (pseudohypoparathyroidism). In both cases, 
there are significant reduced levels of plasma calcium 
associated with hyperphosphatemia. Hypocalcaemia can have 
a variety of manifestations including cardiovascular 
changes.1  
We describe here a case of Pseudohypoparathyroidism (PHP) 
presenting as congestive cardiac failure. 
 
Case Report 
A 24 years old female presented with insidious and gradually 
progressive shortness of breath for 28 days with orthopnea 
and paroxysmal nocturnal dyspnoea for last few days and 
breathlessness even at rest for 1 day. She was taking 
carbamazepine for seizure control for last eight years. She 
had developmental delay with poor scholastic performance at 
school. She was married and had a 2 years male healthy 
child. Her both parents and younger sister were healthy but 
her younger brother was a diagnosed patient of 
pseudohypoparathyroidism. 
On general physical examination her respiratory rate was 
30/min., pulse rate was 100/min., BP 100/70 mm Hg. and she 
had cyanosis. She developed severe carpopedal spasm during 
examination (Figure 1) and attendant told that she was taking 
carbamazepine for these episodes. Chvostek’s sign was also 
present. There was LV S3 gallop and bilateral basal 
crepitations. Her Mini mental score was 15/30 and deep 
tendons jerks were brisk. On investigation, X-ray chest 
showed bilateral non homogenous opacity in middle and 
lower zones. Echocardiography showed LV enlargement with 
severe LV systolic dysfunction (EF = 22 %) (Figure 2). Her 
serum Ca++ was 4.40 mg/dl (Normal range 8.7–10.2 mg/dL), 
Phosphorus 5.6 mg/dl (Normal range 2.5–4.3 mg/dL with 
marked elevation of serum PTH 201.30 pg/mL (Normal 
range 15.00 – 68.00 pg/ml). Her serum albumin was 4.3 g/dl. 
Alkaline phosphatase was 78 U/L (38 -94 U/L), 25 hydroxy 
Vit. D was 20.12 nmol/L (< 25 nmol /L deficient). Renal and 
liver function tests were normal. ECG revealed a prolonged 
QT interval (QTc 620 ms) (Figure 3). CT scan head showed 
calcification of basal ganglia bilaterally (Figure 4). 
 
This work is licensed under a 
Creative Commons Attribution- 
No Derivative Works 2.5 India License 
Online Journal of Health and Allied Sciences 
Peer Reviewed, Open Access, Free Online Journal 
Published Quarterly :  Mangalore, South India : ISSN 0972-5997 
Volume 12, Issue 1; Jan-Mar 2013 
2 
 
Figure 1: Photograph showing carpal spasm on 
compression of forearm. 
 
Figure 2: Echocardiography showing LV Dysfunction in 
hypocalcemia. 
Figure 3: ECG showing prolonged QTc Interval. 
Figure 4: CT Scan head showing bilateral calcification in 
basal ganglia. 
 
Figure 5: Echocardiography after correction of 
hypocalcemia. 
Diagnosis of Pseudohypoparathyroidism and Vitamin D 
deficiency was considered. She was managed with calcium 
gluconate infusion for 24 hours and injection cholecalciferol 
6 lakh IU intramuscular was given. After that oral calcium 
and 1, 25(OH) 2D supplements were started. Carbamazepine 
was tapered off. After 2 weeks her serum Ca++ was 8 mg/dl 
and Phosphorus was 4.6 mg/dl and Echocardiography 
parameters of LV function along with LV dimensions 
showed improvement and left ventricular ejection fraction 
was 25%. Chest X ray showed clearing of the opacities. QTc 
improved to 472 milliseconds. Patient came for follow up 
after 4month. She was asymptomatic and her serum calcium 
was 9.4 mg/dl and phosphate was 4.5mg/dl. she never 
developed any seizure or tetany after treatment. Her echo was 
normal and ejection fraction was 60% (Figure 5). She was 
advised to continue the same treatment and to follow up after 
6 months. 
 
Discussion 
Hypocalcaemia characteristically causes prolongation of the 
QT interval in the electrocardiogram.2 Torsades de pointes 
can potentially be triggered by hypocalcaemia but is much 
less common than with hypokalemia or hypomagnesemia. In 
addition, decreased myocardial performance and even 
congestive heart failure have been reported.3-7 Myocardial 
dysfunction is reversible with calcium 
repletion.5,8 Hypocalcemic cardiomyopathy in PHP has been 
reported scarcely in literature.9 
Our patient had PHP along with vitamin D deficiency 
causing hypocalcemia leading to QTc prolongation along 
with congestive cardiac failure which improved with 
restoration of calcium level and calcitriol supplementation. In 
this patient, vitamin D deficiency can be due to prolonged 
carbamazepine use and moreover deficiency of Vitamin D is 
very common in India.10 Furthermore, conversion of 25-
hydroxy vitamin D to 1, 25(OH)2D is impaired in PHP due to 
parathormone resistance. 
This case demonstrates that hypocalcemic cardiomyopathy 
should be considered in the differential diagnosis of 
congestive cardiac failure in PHP who do not respond 
adequately to the usual treatment. Recognition of the 
condition is important as myocardial impairment is reversible 
after administration of calcium. 
References 
1. Maeda SS, Fortes EM, Oliveira UM et al. 
Hypoparathyroidism and Pseudohypoparathyroidism: a 
review. Arq Bras Endocrinol Metab2006;50/4:664-673. 
2. Benoit SR, Mendelsohn AB, Nourjah P, et al. Risk 
factors for prolonged QTc among US adults: Third 
National Health and Nutrition Examination Survey. Eur 
J Cardiovasc Prev Rehabil 2005;12:363. 
3 
 
3. Kazmi AS, Wall BM. Reversible congestive heart 
failure related to profound hypocalcemia secondary to 
hypoparathyroidism. Am J Med Sci 2007;333:226. 
4. Levine SN, Rheams CN. Hypocalcemic heart 
failure. Am J Med 1985;78:1033. 
5. Wong CK, Lau CP, Cheng CH, et al. Hypocalcemic 
myocardial dysfunction: short- and long-term 
improvement with calcium replacement. Am Heart J. 
1990;120:38. 
6. Kudoh C, Tanaka S, Marusaki S, et al. Hypocalcemic 
cardiomyopathy in a patient with idiopathic 
hypoparathyroidism. Intern Med 1992;31:561. 
7. Brunvand L, Haga P, Tangsrud SE, Haug E. Congestive 
heart failure caused by vitamin D deficiency? Acta 
Paediatr 1995;84:106. 
8. Rimailho A, Bouchard P, Schaison G, et al. 
Improvement of hypocalcemic cardiomyopathy by 
correction of serum calcium level. Am Heart J. 
1985;109:611. 
9. Mönig H, Föh KP, Schulte HM, Simon R. 
Hypocalcemic cardiomyopathy as the cause of severe 
left heart failure. Dtsch Med Wochenschr. 1994 Sep 
23;119(38):1270-1275. 
10. Harinarayan CV, Joshi SR. Vitamin D status in India-
Its implications and Remedial Measures. J Assoc 
Physicians India. 2009;57:40-48. 


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Pseudohypoparathyroidism Presenting as Congestive Cardiac Failure

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