Heart failure with normal heart revealing complex arteriovenous malformation of the lower limb in a child: Case study and literature review

Abstract

Arteriovenous malformations (AVM) are congenital high-flow vascular defects. They are very rare in children. Diagnosis and treatment are often delayed due to their atypical place of occurrence and to their variable clinical manifestations. We report the case of a child treated at the National Centre Hospitalier Albert Royer Children, Dakar. A boy aged 9 years was referred from a health facility in rural areas for the treatment of heart failure. Admission clinical examination showed impaired general condition, global heart failure syndrome and voluminous right warm inguinocrural mass extended to the right abdominal wall (iliac fossa and right flank), with poorly defined limits. Auscultation of this mass showed a thrill and diffuse murmur. Cardiac ultrasound showed severe pulmonary arterial hypertension (PAH) with extensive impairement of the heart cavities, without cardiac structural involvement. The diagnosis of AVM was confirmed by doppler ultrasound of the mass complemented by angiography scan. They showed multiple arteriovenous fistulas within the mass. The diagnosis of complex stage IV AVM (according to Schöbinger classification) of the root of the right thigh was retained. Clinical treatment of heart failure was based on furosemide, spironolactone and captopril to obtain hemodynamic stabilization before possible surgical procedure. Arteriovenous malformations of the limbs, in particular of the proximal end of the lower limb in children are still largely unknown, hence frequent diagnostic errors and delays. Their evolution is unpredictable requiring early diagnosis and careful monitoring involving multidisciplinary interaction between pediatrics, surgeons and radiologists.