A 34-year-old African American male presented to the emergency department (ED) due to sudden-onset severe chest pain. Thirty minutes prior to arrival, the patient experienced severe, tearing chest pain radiating to the back while smoking a cigarette. The patient reported shortness of breath and chest pain worsening with deep inspiration. He did not report any vision disturbances, headaches, or abdominal/leg pain. His past medical history was significant for current cigarette use (5 pack-years), 4 years of uncontrolled hypertension, and 5 years of chronic kidney disease (stage 3b). His home medications included amlodipine, clonidine, losartan, and metoprolol. The patient denied any diagnosis of connective tissue diseases such as Marfan or Ehlers-Danlos syndromes or Systemic Lupus Erythematosus. Family history was significant for type 2 diabetes in his mother and hypertension and kidney disease in his father who required dialysis and died in his 60s. Six months ago, the patient visited his primary care provider, admitting to non-adherence with antihypertensive medications. Five months prior, he began taking MDMA (“ecstasy”) three times a month, stating most recent ingestion was 2 days prior to hospitalization.
Upon arrival to the hospital, the patient was awake, oriented, afebrile, hypertensive (156/75 mmHg), and tachycardic (105 beats per minute). On auscultation, he was tachycardic with regular rhythm, with a holosystolic murmur. His lungs were clear to auscultation bilaterally. He had palpable, equal pulses, intact sensation, and 5/5 strength in all extremities. Labs demonstrated elevations in white blood cell count (24,200/μL), lactic acid (4.1 mmol/L), D-dimer (3150 ng/mL), blood urea nitrogen (24 mg/dL), and creatinine (2.5 mg/dL, baseline 3.0 mg/dL).
SARS-CoV-2 (COVID-19) RT-PCR test was negative, and the patient denied recent symptoms consistent with COVID-19 infection. Electrocardiogram showed sinus rhythm with nonspecific ST changes, left ventricular hypertrophy, T-wave inversion in inferior/lateral leads, and questionable ST elevations in leads V1-V4. Computed tomography angiography (CTA) revealed a type A aortic dissection extending from the aortic root involving the ascending aorta, arch, descending thoracic aorta, and extending into the iliac and common femoral arteries (Fig. 1).
Upon hospital admission, the patient was started on a nicardipine drip with labetalol pushes for goal systolic blood pressure under 120 mmHg, and cardiac and vascular surgery were consulted for emergent surgery. The patient underwent median sternotomy, ascending aortic aneurysm and dissection repair with a gelatin-sealed, woven polyester thoracic endovascular stent graft to treat the descending thoracic aorta, aortic valve resuspension, right axillary artery, and right femoral venous cut down for cardiopulmonary bypass and intraoperative transesophageal echocardiogram.
Two days later, the patient had an acute worsening of hypertension and reported right lower limb pain, right calf swelling, and decreased function. On exam, the right leg had detectable pedal pulses but was tense and tender with pain on passive range of motion along with decreased strength on dorsiflexion and minimal plantarflexion. Labs revealed worsening creatinine to 5.4 (2.5 on admission) with rhabdomyolysis and a creatinine phosphokinase level of 1,105 U/L. Venous Dopplers were negative.
Urgent right lower limb 4-compartment fasciotomy for compartment syndrome was performed. Plastic surgery was consulted for evaluation and management of necrotic muscle and skin in the lateral compartment. Following evaluation, the patient underwent excision of peroneus longus and brevis muscles, lateral gastrocnemius, lateral hemisoleus and partial excision of flexor hallucis longus, and neurolysis of right common peroneal and superficial peroneal nerves. Following muscle excision, he underwent a debridement by plastic surgery, and wound vacuum-assisted closure (VAC) was applied.
The remainder of his hospital course was complicated by worsening acute kidney injury requiring dialysis. He underwent debridement two more times. The patient’s renal function continued to improve with good urine output. He was discharged to acute rehab with VAC to the medial and lateral compartments with dressing changes every 3 days with potential plan for skin graft in 2 to 4 months. He participated in acute rehabilitation for 10 days with continued wound VAC changes. Strict blood pressure control was recommended, and the patient was discharged home at an independent level with adaptive devices, including a rolling walker and straight cane.
