Case Presentation: 77F with COPD, CAD, HFpEF (50%), scimitar syndrome, pHTN, pAfib, type V long QT syndrome, SSS s/p ablation, TIA, and a recent mechanical fall with left pubic ramus fracture and subdural hematoma with recent discharge from acute rehabilitation center presents to the emergency room after a witnessed, mechanical fall at home due patient’s walker catching in rug, fell forward, striking head against the stairs without LOC. In ED, head CT negative, and 10 staples applied to posterior occipital head laceration. CT abdomen and pelvis with acute pubic ramus fracture. Labs significant for leukocytosis, lactate 3.9, and urinalysis concerning for UTI. Patient reported urinary retention and hesitancy, denied dysuria. Afebrile, BP slightly decreased from baseline (SBP 90s), telemetry revealing v-pacing 90s. Pan-cultured, received 1L NS, and started on Vancomycin and Zosyn for presumed sepsis in the setting of a UTI. Admitted to the Medicine Service.
Later, unresponsiveness for approximately 5 seconds correlating with 20 beats of VTach, with subsequent labs revealing lactate 5.6. Received 500ml NS. Continued to decline hemodynamically, SBP 70s. Received additional 500ml bolus; transferred to critical care.
Upon transfer, alert and oriented, afebrile, SBP 90s, HR 90s, paced, RR 20, SpO2 100% on RA. Dyspnea upon exertion while transferring from stretcher, but lungs clear to auscultation, well perfused, no edema, mild JVD, and responsive to passive leg raise. Started on Dobutamine drip; within an hour, respiratory effort worsening, decreased oxygenation, and dyspneic. CXR without evidence of fluid overload, started on BiPAP with improvement. Minute ventilation >20L/min, ABG revealing worsening acidosis, lactate 13. Shortly after, patient seized. Post-ictal pulse palpated, with quick decline to PEA arrest. 30 minutes of ACLS provided without ROSC. Pronounced dead at 1551.
Discussion: Patient with scimitar syndrome, or congenital venolobar syndrome, a defect resulting in either partial or complete anomalous venous return from the right lung with blood returning to systemic venous drainage instead of the left atrium. Early sepsis management centers upon IV fluids and antibiotics. Fluid resuscitation in the context of heart failure is always concerning for fluid overload. In scimitar syndrome, concern should be even higher. In this case, as hemodynamics and lactic acidosis worsened, dobutamine drip started due to potential cardiogenic shock. Presumed cause of death: flash pulmonary edema in the setting of acute on chronic right ventricular failure and overload in the context of severe pulmonary hypertension related to scimitar’s syndrome.
Conclusions: Awareness of Scimitar syndrome’s physiology in relation to fluid resuscitation may have improved early management via limited fluid resuscitation and hemodynamic monitoring. Early recognition, triage, and involvement of cardiology should be employed in the patient with scimitar syndrome and sepsis.