Abstract Ref Number = APCP1069
SECONDARY NEPHROTIC SYNDROME ASSOCIATED WITH CYANOTIC CONGENITAL HEART DISEASE A CASE REPORT
Sudung Pardede,Nia Astarina,Partini P Trihono,Henny Adriani Puspitasari
Background : Glomerular dysfunction can be found in cyanotic congenital heart disease (CHD) especially in older children, which occasionally associated with proteinuria and microalbuminuria. Risk of developing renal impairment is particularly high in cyanotic patients particularly in patients with long-standing cyanotic CHD. However, nephrotic syndrome (NS) is an uncommon complication of cyanotic CHD and rarely documented.
Case Presentation Summary : A 3-year-old girl with dyspnea, edema, and cyanosis was referred from primary health care. She was diagnosed as tricuspid atresia, pulmonary atresia, and single atrium based on echocardiography at 6 months of age but lost to follow up. No treatments were given until she came to our hospital with haematuria and oliguria since one day prior. Physical examination revealed an ill, afebrile, cyanosed, anasarca, and grade 4 digital clubbing. Laboratory examination showed hyponatremia, metabolic acidosis, azotemia, and hypoalbuminemia (albumin level 2.1 g/dL) and nephrotic range proteinuria (dipstick urine +2). A diagnosis of CHD and NS was established. Renal biopsy was contraindicated because of deteriorating renal condition and cardiac status. During hospitalization, she was treated specifically for heart failure, associated furosemide (1 mg/kg/dose, every 8 hours). She was given prednisone (60 mg/m2/day) to treat NS. Renal status was deteriorated with declining urinary output and worsening serum biochemical profiles. Her condition was regressed, and finally died on day 3 of admission and parents refused autopsy.
Learning Points/Discussion : The case presented suggest that NS may complicate CHD. The incidence is unknown since proteinuria is not routinely screened in CHD patients. As one of complicating disease in CHD which may worsen the prognosis of CHD, proteinuria should be screened in CHD patient.
Keywords: Nephrotic syndrom cyanotic congenital heart disease complication