Abstract
Carcinoid heart disease is a rare disease, which develops in 20-50% of patients with carcinoid syndrome and is a main predictor of clinical outcome in those patients. Typical cardiac involvement of this disease presents as primary diseases of the tricuspid or pulmonary valves with a rare affection of left sided heart valves. The characteristic pathological findings are endocardial plaques of fibrous which may involve not only the valve leaflets, but also the subvalvar apparatus.Typical management of patients with this condition consist of the treatment of right heart failure (HF), pharmacotherapy to reduce the secretion of tumour products, and surgical valve replacement. Here we report a 56-year old male admitted to the Department of Endocrinology because of flushing with abdominal pain and diarrhea 2-3 times a week for 2 years. During hospitalization carcinoid syndrome with metastases to the liver and abdominal lymph nodes was diagnosed. Treatment with a long-acting somatostatin analog was initiated, resulting in a good control of the symptoms of the carcinoid syndrome. Trans-thoracic echocardiography revealed right atrial and right ventricular enlargement with degenerative lesions of tricuspid valve leaflets and its subvalvular apparatus, with leaflet stiffening, retraction and malcoaptation, resulting in severe tricuspid regurgitation (vena contracta [VC] width - 7 mm). Pulmonic valve was also involved with thickening of pulmonary valve cusps leading to mild pulmonary stenosis (peak gradient [PG] - 27 mmHg, mean gradient [MG] - 14 mmHg) and mild pulmonary regurgitation. Moreover, signs of pulmonary hypertension (with tricuspid regurgitation pressure gradient [TRPG] of 50 mmHg and estimated systolic pulmonary artery pressure [SPAP] of 50-55 mmHg) was observed. There were no signs of hemodynamically significant left-sided valve disease, nor of any abnormalities in segmental or global left ventricular function. After 12 months of treatment with a long-acting somatostatin analog, the patient was reassessed. Despite a good control of carcinoid syndrome symptoms and reduction in carcinoid syndrome marker (5-hydroxyindoloacetic acid), exacerbates the tricuspid regurgitation and worsens right HF was observed. Control echocardiographic examination showed significant progression of the pulmonary valve disease with severe pulmonary regurgitation (VC width - 10 mm) and mild pulmonary stenosis (PG 18 mmHg, MG 8 mmHg), a deterioration of tricuspid regurgitation (VC width - 11 mm, effective regurgitant orifice area - 0.94 cm2, regurgitant volume - 64 ml) with further enlargement of the right ventricle and right atrium, and with a consequent decrease in TRPG value (25 mmHg), despite well preserved right ventricular systolic function (TAPSE - 24 mm). Due to disease progression, treatment of HF and peptide receptor radionuclide therapy were initiated. The patient was presented for surgical valve replacement, however he did not agree to surgical treatment.
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Case Presentation - Biventricular Hypertrophy and Valvular Pulmonary Stenosis in Adult Patient with Noonan Syndrome: A Rare Case
