A Case of Paraganglioma with Cyanotic Congenital Heart Disease

Authors

  • Norasyikin A Wahab Department of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia
  • Beh Hui Chien Department of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia
  • Mohd Rahman Omar Universiti Sains Islam Malaysia
  • Aini Ab Aziz Department of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia
  • Norlaila Mustafa Department of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia
  • Norlela Sukor Department of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia
  • Nor Azmi Kamaruddin Department of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia

Keywords:

cyanotic congenital heart disease, paraganglioma, pheochromocytoma, hypoxia

Abstract

Co-occurrence of cyanotic congenital heart disease (CCHD) and phaeochromocytoma (PCC) and paraganglioma (PGL) are rare, although some cases have been reported. We report a case of left paraganglioma in a 20-year-old lady with an underlying CCHD who underwent palliative Glenn shunt, subsequently developed polycythaemia and cavernous sinus thrombosis presented with palpitation, sweating, headache and hypertension of 3-months duration at the age of 17. The abdominal CT scan revealed an enhancing left paraaortic mass measuring 5.2 cm x 4.4 cm x 3.8 cm. A 24-hour urine catecholamine demonstrated raised noradrenaline level to six times upper limit of normal and hence diagnosis of left sympathetic (sPGL) was made. In view of the delayed diagnosis and significant morbidity associated with her condition, surgical treatment is no longer an option. Therefore, vigilant screening and early treatment of PCC-PGL in patients with CCHD are crucial in order to avoid significant morbidity and ensure a good quality of life.

Author Biographies

Norasyikin A Wahab, Department of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia

Medical department

Beh Hui Chien, Department of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia

Medical department

Mohd Rahman Omar, Universiti Sains Islam Malaysia

Medical department

Aini Ab Aziz, Department of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia

Department of radiology

Norlaila Mustafa, Department of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia

Medical department

Norlela Sukor, Department of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia

Medical department

Nor Azmi Kamaruddin, Department of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia

Medical department

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Published

2021-03-31

How to Cite

Wahab, N. A., Chien, B. H., Omar, M. R., Aziz, A. A., Mustafa, N., Sukor, N., & Kamaruddin, N. A. (2021). A Case of Paraganglioma with Cyanotic Congenital Heart Disease. Acta Medica Indonesiana, 53(1), 77. Retrieved from https://www.actamedindones.org/index.php/ijim/article/view/1065

Issue

Vol. 53 No. 1 (2021): Acta Medica Indonesiana

Section

CASE REPORT

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