Low gradient severe aortic stenosis with normal ejection fraction.

Sir, A 18 year old female patient was diagnosed to have tubercular cervical lymphadenopathy and started on antitubercular therapy (ATT). The lymphadenopathy regressed, however, after four months of ATT, patient developed jaundice, abdominal distension and swelling of bilateral lower limbs. She had no bleeding manifestation or altered behaviour. She had no history of jaundice in the past, history of alcohol consumption or family history of liver disese. On physical examination, patient had pallor, icterus and bilateral pitting-type pedal edema. She had abdominal distension with shifting dullness. There was no hepatosplenomegaly. Investigations revealed a hemoglobin of 9.6 gm/dl; white cell count was 5,600/cm3 (71 % neutrophils); platelet count was 1,74,000/cm3. Coagulation profile was deranged: Prothrombin time (PT) 42.3 seconds, international normalized ratio (INR) 2.76, activated partial-thromboplastin time (aPTT) showed no clot formation. Biochemistry findings showed 0.6 mg/dL serum creatinine, 2.5 g/dl albumin, 354 Iu/l alkaline phosphatase (35 to 104 Iu/l), 73 Iu/l alanine aminotransferase (5 to 40 Iu/l), 137 Iu/l aspartate aminotransferase (5 to 37 Iu/l), 22.5 mg/dl total serum bilirubin (normal value < 1), 14.2 mg/dl direct bilirubin (< 0.25 mg/dl). Tests for hepatitis C virus antibody, hepatitis B, and hepatitis A were negative, as were tests for human immunodeficiency virus (HIV) types 1 and 2, anti-nuclear (ANA) and antimitochondrial (AMA) antibodies. Alpha-fetoprotein levels were unremarkable. Ascitic fluid examination revealed a transudative picture with total protein 0.3 gm/dl and 190 cells (mainly mononuclear). ATT induced drug induced liver injury (DIlI) was diagnosed by exclusion and all the drugs omitted. Patient was continually observed in-patient. There was no improvement in liver biochemistry following ATT withdrawal. Patient was worked up for other causes of liver injury. ultrasound abdomen revealed coarse echotexture of the liver, small liver, with nodular surface (suggestive of chronic liver disease), with moderate ascites. Contrast-enhanced computed tomography of the abdomen was suggestive of chronic liver disease. Patient underwent evaluation for Wilson’s disease. Slit-lamp examination revealed bilateral Kayser-Fleischer rings in superior and inferior region. Serum ceruloplasmin levels were 21.5mg/dl (normal 22-58) and 24 hour urine copper was 269 microgram/ day (normal < 60). A diagnosis of Wilson’s disease was made. Biopsy could not be done due to coagulopathy. MRI of the brain was done to support the diagnosis, which showed hyperintensities in bilateral globus pallidus suggestive of mineral deposits. Patient most likely had ATT induced acute decompensation of underlying Wilson’s disease. Because of patient’s nonaffordability for liver transplantation, she was started on medical therapy with Low Gradient Severe Aortic Stenosis with Normal Ejection Fraction