Case Report: Primigravida with Coronary Artery Aneurysm and History of Kawasaki Disease

Issue 36 | Volume 1

Introduction

Kawasaki Disease (KD) is a multisystem febrile vasculitis, presumably infectious in etiology, that primarily affects children. Serious complications include coronary arteritis, coronary artery aneurysm (CAA) and stenosis, coronary thrombosis, and rare rupture of a coronary aneurysm.1 There are limited case reports of patients with KD, resultant coronary aneurysms and their management in pregnancy and for delivery.2–7 We present a case of a patient with a giant coronary aneurysm secondary to KD in childhood who underwent an uneventful pregnancy and delivery via primary cesarean section.

Case Description

A 30 y/o primigravida presented to the high-risk cardio-OB clinic at nine weeks gestation with a history of KD. At age 13, the patient experienced a prolonged febrile illness with subsequent diagnosis of KD treated with IVIG. At age 16, she was diagnosed with a 10 mm x 23 mm giant CAA of the left anterior descending coronary artery on cardiac catheterization. Patient followed with cardiology and had normal EKGs and echocardiograms. Stress test at age 26 demonstrated no evidence of ischemia, and cardiac CT performed at age 28 showed stable CAA. Anticoagulation was maintained with apixaban and aspirin until pregnancy, when she was transitioned to enoxaparin BID and aspirin.

Cardiac MRI was performed at 22 weeks, but coronary sequences were not adequate for assessment; echocardiogram remained normal. Delivery planning included discussion of elective cesarean section versus vaginal delivery with assisted second stage to avoid valsalva, avoiding terbutaline and methergine as absolute and relative contraindications. At 36 weeks, the patient was transitioned to heparin 10,000 units SQ BID and aspirin. At approximately 37 weeks, the patient was admitted for finding of a critically high calcium level of 14.8 mg/dL caused by primary hyperparathyroidism, and non-sustained severe range pressures. Per the MFM team, delivery was now indicated in the setting of worsening blood pressure control at term and finding of breech presentation. A Cesarean section was planned for the following morning to allow for calcium correction via diuresis with furosemide, intravenous fluid, and calcitonin. Cesarean delivery was performed in cardiac operating room with cardiac anesthesia, cardiac surgery, perfusion, and NICU teams on standby. Heparin had been discontinued for >24 hours. Spinal anesthesia was performed with the patient on standard ASA monitors and an arterial line placed in the operating room. A female infant was born with normal Apgars, and the patient recovered well.

Discussion

Pregnant patients with a history of KD should be managed under the care of a multidisciplinary team including maternal fetal medicine, cardiology, and anesthesiology. Mode and timing of delivery should be based on obstetric considerations.4,7 The preanesthetic evaluation should focus on cardiac function, known cardiac lesions, arrhythmias, and patient’s functional status.7,8 Coronary artery aneurysms, which occur in 25% of untreated cases, can develop as early as 7 days after onset of fever and continue to increase in size up to 6 weeks after disease onset.4,8 Approximately half of CAAs will regress to normal diameter within 2 years.8 Giant aneurysms are classified as greater than 8 mm internal diameter and have an estimated 33% mortality.8 Some patients have required coronary artery bypass grafting due to giant CAA.4 The goal for these patients is to minimize cardiac stress and balance cardiac oxygen demand with supply, as they may not be able to compensate for increased demand.4,5,7–9 Labor epidural is likely beneficial in blocking sympathetic output from painful contractions.7 Neuraxial anesthesia was shown to be safe in a small case series of 19 pregnancies in 13 women with history of KD and known coronary artery lesion.7 For cesarean sections spinal blockade was safe, though 5 of 7 cases in that case series required vasopressor to treat hypotension.7 Invasive monitoring should be determined based on patient’s functional status and cardiac function. Only 54% of patients in the Inoue case series had an arterial line for hemodynamic monitoring.7

While aneurysm rupture could be catastrophic, pregnant patients are at higher risk of thrombosis and arrythmias.4–6 There is not a clear consensus on the best practice regarding anticoagulation. Some patients are managed with therapeutic enoxaparin, while others are managed with low dose aspirin, and others were not on any anticoagulation or antiplatelet medications.4,6,7 Anticoagulation must be factored into timing of neuraxial anesthesia and may increase risk of postpartum hemorrhage. Pitocin can be given intravenously or by direct intrauterine injection; however, methylergonovine is contraindicated due to risk of coronary artery spasm.5–7 Delivery is associated with significant increase in maternal cardiac output and is presumed to be the time of highest risk; however, most complications have occurred postpartum.7

REFERENCES:

1. Rowley AH, Jaggi P. Kawasaki Disease. In: Cherry J, Kaplan S, Harrison G, Steinbach W, Hotex P, Williams J, eds. Feigin and Cherry’s Textbook of Pediatric Infectious Diseases (Ninth Edition). 9th ed. ; 2025. Accessed May 8, 2025. https://doi.org/10.1016/ B978-0-323-82763-8.00078-9
2. Shear R, Leduc L. Successful Pregnancy Following Kawasaki Disease.; 1999.
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5. Alam S, Sakura S, Kosaka Y. Anaesthetic management for Caesarean section in a patient with Kawasaki disease. Canadian Journal of Anaesthesia. 1995;42(11):1024-1026. doi:10.1007/ BF03011076
6. McAndrew P, Hughes D, Adams P. Pregnancy and Kawasaki disease. Int J Obstet Anesth. 2000;9(4):279-281. doi:10.1054/ ijoa.2000.0723
7. Inoue R, Mazda Y, Tanaka H, et al. Management of pregnancy and delivery in women with Kawasaki disease and residual coronary artery lesion with detailed analysis of labor analgesia: combined experience of 13 cases in two institutions. JA Clin Rep. 2020;6(1):67. doi:10.1186/s40981-020-00375-y
8. To L, Krazit ST, Kaye AD. Perioperative considerations of kawasaki disease. Ochsner journal. 2013;13(2):208-213.
9. Rowley AH, Jaggi P. Kawasaki Disease. In: Feigin and Cherry’s Textbook of Pediatric Infectious Diseases, 9th Edition: 2-Volume Set. Vol 1-2. Elsevier; 2025:777-792.e6. doi:10.1016/B978-0-323- 82763-8.00078-9

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