Abstract

DIFFICULTY IN MANAGING EISENMENGER SYNDROME: ABOUT A CASE SEEN IN THE TOAMASINA PEDIATRICS DEPARTMENT

Rakotomahefa Narison Mbolanirina Lala*, Ratsimbazafy Arthur Bien Aimé, Rakotoarisoa Lalaina Andonirina, Razafindrakoto Hery, Rakotoarisoa Heriniaina, Raobijaona Solofoniaina Honoré

ABSTRACT

Introduction: Eisenmenger's syndrome is the ultimate stage of congenital heart disease with untreated left-right shunt. Unfortunately, these patients are not always followed up in specialized centres. This makes their management in daily paediatric practice very complex. Justification: The objective of this study is to raise awareness among family doctors of the need to detect and then refer all heart diseases to a specialized centre at an early stage. All this in order not to delay treatment and to avoid their evolution towards the Eisenmenger complex. Observation: Our case concerns a 4.5 year old boy, admitted to the Paediatrics Department for an oedematous syndrome, with no personal or family cardiovascular history or reported malformation in the family, but in his personal history, we can retain a cough associated with repeated cyanosis, which may be in favour of a heart problem. On cardiac ultrasound, we objected to a heart disease with an Eisenmenger complex type with an aorto-pulmonary window. The patient was treated symptomatically, showing a transient improvement in his clinical condition. The subsequent evolution was enamelled by a succession of complex complications with multivisceral disorders: renal, pulmonary, hematological and cutaneous. After 16 days in hospital, the patient died following a pulmonary embolism. Conclusion: Our observation illustrates the difficulty of managing this disease. Collaboration with family physicians for early diagnosis of any heart disease, in order to quickly refer it to a specialized centre, affects the immediate prognosis and long-term survival of these patients. Once installed, the Eisenmenger complex will be managed in a specialized and multidisciplinary environment, including a palliative care unit that will ensure a better quality of life for the patient.

Keywords: Congenital heart disease, cyanosis, Eisenmenger's syndrome, polyglobulia.