Renal Transplant in a Pediatric Patient With Isolated Left V
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A challenging case of rare cardiomyopathy in a transplant patient was reported. A 13-year-old girl presented for deceased donor kidney transplantation was diagnosed with minimal change disease and focal segmental glomerulosclerosis at 3 years of age and underwent bilateral nephrectomies when she was 10 years old. She was anuric, which was managed by nightly peritoneal dialysis. She was diagnosed with left ventricular noncompaction cardiomyopathy (LVNC) in early childhood, with a current fractional shortening of 21% and a noncompacted-to-compacted layer ratio of 10:3. Strain measurements indicated mild left ventricular diastolic dysfunction.

Vital signs were normal before the procedure. Airway examination was unremarkable with a Mallampati score II. Induction of general anesthesia was performed with propofol, fentanyl and cisatracurium, and the patient was intubated. Then left radial arterial line and left internal jugular double-lumen central venous catheter were placed. Transesophageal echocardiography (TEE) demonstrated an ejection fraction of 45%, and no wall motion or valve abnormalities were noted other than the known noncompaction cardiomyopathy.

The latter was picked up from the trabeculae seen in the apical segment of the left ventricle in the transgastric short axis view. A restrictive fluid strategy was used given the known cardiomyopathy. In addition, 1,100 mL of crystalloid and 500 mL of albumin were given before kidney reperfusion. Monitoring with TEE was used throughout the procedure to assess fluid status.

Unfortunately, after perfusion of the graft kidney, the patient remained anuric. She received furosemide and mannitol to help with urine output, but she continued to be anuric. After reperfusion of the kidney, the hemoglobin level was found to be 6.8 g/dL and blood was transfused. The patient was electively left intubated and transferred in stable condition to the pediatric ICU. Norepinephrine was used to maintain the systolic pressure around 110 mm Hg. Urinary output started some days later in the pediatric ICU with medication management. She had a prolonged course due to the need for plasmapheresis and was discharged home approximately one month later.