Pulmonary hypertension with massive megalosplenia: A Case Re
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"A 58-year-old male was admitted to the hospital because of fatigue, breath shortness for 6 months, and got worse in the last 3 months. He had taken an ultrasound cardiogram (UCG) in another hospital 1 week before admission which suggested a mildly enlarged left heart (left ventricular end-diastolic diameter [LVEDD] = 55 mm) with an otherwise normal left ventricular ejection fraction (54%) and a significantly enlarged right heart (right ventricular end-diastolic diameter [RVEDD] = 50 mm). The UCG also indicated a remarkably increased pulmonary artery systolic pressure (PASP = 82 mm Hg), accompanied by a widened inferior vena cava (22 mm) with decreased compression during inspiratory (<50%).

After taking furosemide 20 mg Qd, spironolactone 20 mg Qd, and captopril 6.25 mg Bid for 1 week, the patient did not feel better. Previously, he had hypertension for 15 years. His spleen was found to be enlarged 15 years ago, and was referred to as “massive splenomegaly” since 6 years ago. Four years ago, his portal vein was found to be widened during abdominal ultrasound examination. However, examinations such as abdominal ultrasound and CT had not identified the cause for these abnormalities. He was not a smoker, but he used to drink about 100 g alcohols per day for more than 30 years, which he just quitted 2 months ago. Bone marrow biopsy of the patient revealed myeloproliferative neoplasm (MPN) with severe myelofibrosis (MF).

We treated the patient with diuretics and fosinopril, and also steroids and thalidomide for his MPN/MF. Two weeks later, the pulmonary artery pressure (PAP) was remarkably decreased (PASP = 53.1 mm Hg by UCG, mean PAP = 21 mm Hg by right cardiac catheterization). Within 2 years’ follow-up, his circulatory state and hematological state remained stable.

Source: Medicine: March 2019 - Volume 98 - Issue 12 - p e14594"

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