Abstract
A 33-year-old pregnant woman with a history of a giant coronary artery aneurysm (CAA) of the right coronary artery owing to Kawasaki disease (KD) was referred to our hospital for the management of pregnancy and delivery. The CAA was detected when she was 10 months old on the 24th day from the onset of KD and showed transient regression followed by progressive dilation and reached a size of 25 mm when she was 30 years old. The baby was delivered at 38 weeks of gestational age. Resection of the CAA and coronary artery bypass grafting were performed 5 months after the delivery. Pathological results suggest that progressive dilation of the CAA was owing to a reduction in elastic recoiling force caused by partial destruction of the internal elastic lamina and degenerated tunica media against an increase in blood pressure that accompanied the growth of the patient. The pathophysiology of CAAs with atypical clinical course may be different from that of typical CAAs owing to KD.
< Learning objective: Kawasaki disease (KD) causes varying sizes of coronary artery aneurysms (CAAs). In this case, rare progressive dilation of the CAA owing to KD was observed in the remote phase. We revealed different pathological features of rare CAAs from those of a typical one; progressive dilation of the CAA could be owing to the reduction of recoiling force. Planned surgical treatment of a CAA showing continuous dilation should be a reasonable option for avoiding emergent CAA rupture.>
Introduction
Kawasaki disease (KD) is a vasculitis of unknown etiology affecting medium-sized extraparenchymal muscular arteries, most commonly the coronary arteries. Intravenous immunoglobulin (IVIG) therapy or the combination of IVIG and steroid or immunosuppressive therapy have dramatically reduced the incidence of coronary artery aneurysms (CAAs); however, 0.13–0.82% of KD patients still suffer from CAAs with varying sizes . Although CAAs sometimes dilate rapidly in the acute phase of KD, progressive dilation of CAAs in the remote phase is fairly rare, and the pathology of CAAs with an atypical clinical course remains to be clarified [ , ].
Here, we report a case of KD with a giant CAA showing progressive dilation over 30 years treated by resection of the aneurysm and coronary artery bypass grafting (CABG), and provide pathological insight of the aneurysm.
Case report
A 33-year-old pregnant woman with a history of KD was referred to our institute for the management of pregnancy and delivery. She was diagnosed with KD when she was 10 months old. Although treatment with aspirin was initiated, a CAA of the right coronary artery (RCA) of 6 mm and transient dilation of the left coronary artery were detected on the 24th day from the fever onset. Coronary angiography (CAG) performed 1 year after the fever onset showed that the CAA regressed to 3 mm in size. However, following regression, a gradual dilation of the CAA was detected, and the patient was referred to our institute when she was 5 years old. CAG at our hospital revealed a right CAA of 8 mm, and time-course dilation over the years was observed using follow-up echocardiography, CAG, and magnetic resonance coronary angiography (MRCA) ( Fig. 1 A ). MRCA revealed the RCA was approximately 25 mm in size with thrombus formation and recanalization inside it when she was 30 years old ( Fig. 1 B ). A combination of two antiplatelet medications, aspirin (200 mg/day) and ticlopidine hydrochloride (200 mg/day), was administered for thromboprophylaxis. During the follow-up period at our institute, no evidence of myocardial ischemia was detected using an exercise electrocardiogram. Thereafter, the patient was followed up at another hospital.
When the patient was referred to our institute again, echocardiography revealed no dramatic change in the size of the RCA compared to that 3 years previously, preserved left ventricular ejection fraction, and no asymmetrical cardiac wall motion. CABG prior to delivery was considered; however, it was abandoned because of the high risk of intrauterine fetal death during surgery. As a rupture of the CAA because of hypertension associated with the delivery was concerned, painless delivery at 38 weeks of gestational age was planned.
At 35 weeks of gestation, she was admitted to our hospital for management of delivery. Her height, weight, and blood pressure (BP) on admission were 166 cm, 64.5 kg, and 112/68 mmHg, respectively. On the day after admission, antiplatelet therapy was stopped, and continuous infusion of heparin was started. At 38 + 3 weeks of gestation, heparin was stopped, and an epidural catheter was inserted for pain management during delivery. At 38 + 4 weeks of gestation, continuous epidural infusion of 0.2% ropivacaine and labor induction were started, and the baby was born after seven hours from the onset of labor. No serious complications occurred during labor, and aspirin administration was started again on the day after delivery. Coronary computed tomography angiography performed after delivery detected the RCA of 27 mm and no evidence of calcification of the CAA ( Fig. 2 ). The scan was performed during an early phase; therefore, it was difficult to elucidate whether a low-density area inside the aneurysm suggested the presence of a thrombus or insufficient enhancement of the aneurysm. Because the patient desired a second child in the future, elected cardiac surgery including resection of CAA and CABG of the RCA was performed 5 months after the delivery. During the surgery, a part of the aneurysm was collected for pathological studies.
