Abstract: PUB167
Not Your Typical Heart Failure: A Rare Case of Adult-Onset Bartter Syndrome
Session Information
Category: Fluid‚ Electrolyte‚ and Acid-Base Disorders
- 1002 Fluid‚ Electrolyte‚ and Acid-Base Disorders: Clinical
Authors
- Williams, Hanah L., Western University of Health Sciences, Pomona, California, United States
- Ho, Andrew T., Temecula Valley Hospital, Temecula, California, United States
- Ahoubim, Joseph, UHS Southern California Medical Education Consortium, Temecula, CA, USA, Temecula, California, United States
- Kim, Andrew Y., UHS Southern California Medical Education Consortium, Temecula, CA, USA, Temecula, California, United States
- Salari, Ellika, UHS Southern California Medical Education Consortium, Temecula, CA, USA, Temecula, California, United States
- Myers, Sheldon James, Western University of Health Sciences, Pomona, California, United States
Introduction
Bartter Syndrome (BS) is a renal tubulopathy characterized by hypokalemia, hypochloremia, metabolic alkalosis, hyperreninemia, and hyperaldosteronism. It causes a mutation in the loop of Henle’s thick ascending limb’s Na-K-Cl (NKCC) cotransporter. This gives BS a characteristic mechanism mimicking the loop diuretic, furosemide. Understandably, this complicates the treatment of comorbid conditions, specifically heart failure (HF). In HF, if potassium (K+) falls below 3.5 mmol/L (4-5 mmol/L) in the setting of aggressive replenishment, an alternative diagnosis should be considered. Furthermore, if an electrolyte imbalance cannot be explained, tubulopathies should be on the differential. The complexity of HF and renal response can be exacerbated by BS, thus playing a role in overall management.
Case Description
We report an unusual case of a 55-year-old female with a history of HF with reduced ejection fraction (HFrEF) and chronic hypotension, who presented with HFrEF (EF 10%) exacerbation. Her course was complicated by metabolic alkalosis and severe hypokalemia (as low as 1.8 mmol/L) refractory to aggressive potassium repletion protocols. Serum renin, serum chloride (Cl-), urine Cl-, and urine K+ levels were 32.092 ng/ml/hr (<5.82 ng/ml/hr), 81 mmol/L (96-106 mmol/L), 87 mmol/L (<40 mmol/L), and 108 mmol/L (<62 mmol/L), respectively. These findings are consistent with BS. Ultimately, she required ICU monitoring and emergent airway protection. The patient developed recurrent ventricular arrhythmias and passed from cardiac arrest.
Discussion
In the setting of BS, a HFrEF patient’s survival is futile if not caught early. Both HF and hypokalemia carry a risk of sudden cardiac death as a single entity, so when seen concurrently, treatment becomes complicated. While BS is underrepresented in adults, early recognition and understanding of this tubulopathies’ effects on heart failure management could reduce mortality and morbidity in this complicated patient population.
KCl Response in Patient with BS