Management of Terminal Stage CHF Due to Shones Syndrome

Abstract

Background: we report a case of a 23-year-old male with CHF due to Shone’s syndrome s.p multiple open-heart surgeries, aggressive medical therapy, and implanted CRT-D device. He has been managed by pediatric and adult cardiologists, cardiovascular surgeons, nephrologists, and general internists for many years. He came to us with acute respiratory failure, acute kidney injury and fragility due to advanced stage of CHF with reduced ejection fraction. After decent attempts trying to treat him, eventually the decision of transition to hospice care was made. Case Report: He was started furosemide 120 mg IV; the plan was to continue him on diuretics with temporal improvement recorded. As per his Pediatric cardiologist he is an advanced case of CHF with too frequent exacerbations and hospital admissions, and not a candidate of Heart transplant because of guarded prognosis It was also noted that the patient still had frequent hospitalizations with CHF exacerbations complicated with AKI despite maximum medical therapy. With Palliative care, pediatric cardiologist and Nephrologist all involved, the ultimate decision made to discharge home under strict hospice care plan which will be taken care by a home visit nurse provided thought hospice care Agency. Conclusion: The report shed lights on shone syndrome related CHF, the course of the disease, treatment options and ultimate goals of care. In patients who continues to be symptomatic despite maximum medical and surgical therapy, the decision of implementing more invasive treatment options will greatly depend on goals of care and overall weight of risk vs. benefits.