Abstract
Dilated cardiomyopathy (DCMP), the leading type of cardiomyopathy in children, can be either a primary disease or secondary to a host of pathologies. Endocrine diseases are an extremely rare cause of DCMP in children. Hypopituitarism (congenital or acquired), a rare condition in children, may present in a variety of phenotypes depending on the hormonal deficiency (partial vs. pan hypopituitarism) and associated developmental defects. We describe a 10 year old boy with short stature, severely retarded bone age and congestive cardiac failure (CCF). He was diagnosed to have DCMP with hypopituitarism. Review of literature reveal possible mechanisms of DCMP and CCF in hypopituitarism and probably also indicate favorable response to hormonal replacement therapy.
Highlights
Hypopituitarism is defined as absence or reduction in function of two or more hormones produced by the pituitary gland[1]
Congenital hypopituitarism may present in a variety of phenotypes depending on the hormonal deficiency and associated developmental defects[1,2]
We report a 10 years old boy with hypopituitarism and Dilated cardiomyopathy (DCMP) presenting with cardiac failure
Summary
Hypopituitarism is defined as absence or reduction in function of two or more hormones produced by the pituitary gland[1]. Chest x-ray showed massive cardiomegaly (Figure 1c) and echocardiography confirmed dilated ventricles with tricuspid and mitral regurgitation, biventricular dysfunction (ejection fraction 45%) and pericardial effusion (8 mm) but no evidence of tamponade. He was started on moist O2 inhalation, diuretic (Frusemide) and Digoxin with improvement in symptoms. Fig 1a: 10 year old boy with short stature, microcephaly, dry scaly skin, pot belly, frontal bossing, saddle nose, macro-glossia, low set ears and sparse hair; Fig 1b: X-ray of left wrist and hand showing absence of ossification centres of the carpal bones and distal radial epiphysis; Fig 1c: Chest x-ray showing huge cardiomegaly with water bottle shaped cardiac shadow suggestive of pericardial effusion
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
