Introduction: Hyperthyroidism has been described as a rare cause of pulmonary hypertension (PH) as well as systemic arteriovenous fistulas. However, we describe a case of pulmonary hypertension secondary to an intrathyroid arteriovenous fistula, not previously described in the literature.
Case report: A 56 year old woman followed in our consultations with multinodular goiter and a long standing hyperthyroidism, secondary to Graves Basedow disease with difficult management. She required methimazole and low doses of levothyroxine simultaneously to achieve hormonal control, needing multiple admissions for heart failure (HF). In her last admission to Cardiology, she had right HF stage III-IV of NYHA, and physical examination revealed grade III goiter with murmur auscultation and thrill palpation. The blood tests revealed that she was euthyroid. The echocardiogram showed dilatation of both ventricles, ejection fraction 57%, moderate-severe biauricular dilatation, moderate mitral and tricuspid insufficiency, PAPs 73 mmHg. Cervical thoracic CT evidenced large multinodular goiter with intrathoracic extension and tracheal compression, multiple hypervascular nodules and cervico-vascular venous congestion. Right cardiac catheterization showed moderate precapillary PH with high saturations in the venous territory explored and cardiac output 8.1 l/m. These findings were consistent with the diagnosis of PH secondary to intrathyroid arteriovenous fistula. Definitive treatment was made by performing total thyroidectomy. The pathologic study of the piece was reported as multinodular thyroid goiter (12×13×5 centimeters and 140 grams) with signs of diffuse hyperplasia and arteriovenous fistula. Two months after surgery the patient had improved her functional class and the PAPs had decreased to 30 mmHg.
Conclusions: This is the first case described in which pulmonary hypertension is secondary to an arteriovenous fistula in a giant goiter. Surgery was a successful treatment, and one year after, the patient has a disease free survival.
20 - 23 May 2017
European Society of Endocrinology