Wesley Earl
Resident Physician
New York Presbyterian - Columbia Irving Medical Center
Disclosure information not submitted.
Kemarut Laothamatas, MD
Fellow
New York Presbyterian - Columbia Irving Medical Center, United States
Disclosure information not submitted.
Matthew Baldwin, MD
Attending
New York Presbyterian - Columbia Irving Medical Center, United States
Disclosure information not submitted.
Title:
A Case of Acute Chest Syndrome Resulting in Cor Pulmonale Responsive to Pulmonary Vasodilators
Case Report Body:
Introduction: Optimal management of acute pulmonary hypertension (PH) and ARDS in the context of severe acute chest syndrome (ACS) remains relatively unstudied.
Description: A 30-year-old male presented to the ED with chest pain, low oxygen saturation, and bilateral pulmonary infiltrates, consistent with severe ACS. Supplemental oxygen therapy, antibiotics, and hydration were promptly initiated. CT pulmonary angiography was negative for PE. Admission TTE showed normal LV and RV function. His hypoxemic respiratory failure rapidly progressed, necessitating mechanical ventilation with PaO2 to FiO2 ratio less than 100. Exchange transfusion led to minimal improvement and during transfusion, he developed profound shock with multiorgan failure and lactic acidosis. Findings on subsequent TTE were notable for acute cor pulmonale (ACP) with a right to left shunt through a patent foramen ovale. In addition to lung-protective ventilation, deep sedation, neuromuscular blockade agent, prone positioning, and achieving negative fluid balance via continuous renal replacement therapy, he was started on continuous inhaled nitric oxide at 20 PPM and iloprost every 6 hours. However, within 24 hours on the aforementioned therapeutic regimen, directed at both severe ARDS and acute pulmonary vascular dysfunction, he had a notable improvement in hypoxemia and shock. Follow-up TTE showed resolution of acute cor pulmonale. He continued to improve and was successfully extubated on day 10 of his ICU stay and discharged to an acute rehab on minimal supplemental oxygen therapy.
Discussion: Existing literature suggests that acute PH and ACP are common pathologic processes which could potentiate the severity of shock and hypoxemia among ACS-ARDS patients. The proposed mechanism of ACS-associated pulmonary vascular dysfunction includes nitric oxide depletion, microthrombi formation, and vasoocclusion. Among moderate-to-severe ACS-ARDS patients, acute PH and ACP have a high prevalence, up to 100% and 83%, respectively. There is a paucity of data on the efficacy and mortality benefits of using inhaled pulmonary vasodilators in this setting. However, our case demonstrates safe and effective use of early pulmonary vasodilators, inhaled nitric oxide, and iloprost, in this severely ill patient which was associated with an acceptable outcome.