The acute chest syndrome of sickle cell disease following aortic valve replacement.
The acute chest syndrome (ACS) of sickle cell disease (SCD) is a leading cause of death in SCD, with a high incidence following surgery, though only one case has been reported following cardiac surgery. We present a case of ACS in an adult undergoing aortic valve replacement (AVR) despite instituting established peri-operative optimization measures to prevent sickling. Early diagnosis of this condition in our patient as a distinct clinical entity facilitated appropriate, specific therapy and a good subsequent postoperative recovery. Greater recognition of this syndrome in the growing number of adult sickle cell patients presenting for cardiac surgery may help improve their outcome.
Murtuza
Bari
B
Department of Cardiothoracic Surgery, St Bartholomew's Hospital, London EC1A 7BE, UK. b.murtuza@ic.ac.uk
Gupta
Prity
P
Lall
Kulvinder S
KS
D056586:Acute Chest Syndrome / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D000280:Administration, Inhalation; D000284:Administration, Oral; D000755:Anemia, Sickle Cell / Q000150:complications*; D001024:Aortic Valve Stenosis / Q000150:complications / Q000601:surgery*; D003131:Combined Modality Therapy; D003422:Critical Care; D042241:Early Diagnosis; D005260:Female; D019918:Heart Valve Prosthesis Implantation / Q000009:adverse effects*; D006801:Humans; D008875:Middle Aged; D009569:Nitric Oxide / Q000008:administration & dosage; D010879:Piperazines / Q000008:administration & dosage; D011687:Purines / Q000008:administration & dosage; D012121:Respiration, Artificial; D012720:Severity of Illness Index; D000068677:Sildenafil Citrate; D013450:Sulfones / Q000008:administration & dosage; D014057:Tomography, X-Ray Computed; D014139:Tracheostomy; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000008:administration & dosage
D010879:Piperazines; D011687:Purines; D013450:Sulfones; D014665:Vasodilator Agents; D009569:Nitric Oxide; D000068677:Sildenafil Citrate