ABC | Volume 110, Nº1, January 2018

Case Report Pregnancy in Woman with Kawasaki Disease and Multiple Coronary Artery Aneurysms Walkiria Samuel Avila, Antônio Fernando Diniz Freire, Alexandre Anderson de Sousa Soares, Ana Neri Rodrigues Epitácio Pereira, José Carlos Nicolau Instituto do Coração (InCor) - Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HCFMUSP), São Paulo, SP – Brazil Correspondência: Walkiria Samuel Avila • Rua Dr. Enéas Carvalho Aguiar, 44, Cerqueira César. Postal Code 05403-000, São Paulo, SP - Brazil E-mail: wsavila@cardiol.br , walkiria@incor.usp.br Manuscript received August 05, 2017, revised manuscript March 21, 2017, accepted March 29, 2017 Keywords Pregnancy; Mucocutaneous Lymph Node Syndrome; Coronary Aneurysm. DOI: 10.5935/abc.20170185 Introduction Kawasaki disease (KD), first described in 1967, is a systemic vasculitis of unknown cause. 1 It is an important cause of cardiac diseases in children aged younger than five years. 2 KD is an autoimmune disorder whose clinical features include high fever, exanthema, conjunctivitis, cervical linfadenopathy and peripheral edema. Laboratory tests are compatible with acute inflammatory condition. KD predominantly affects the coronary arteries, which is the most important clinical manifestation of the disease, varying from dilation and stenosis to aneurysm (incidence of 5% in patients with adequate treatment, and 25% in untreated patients). Giant coronary artery aneurysms (CAAs), i.e., CAAs with diameters > 8 mm, are associated with increased risk of thrombosis, acute myocardial infarction (AMI) and sudden death. 2 Lack of diagnosis and treatment of KD at its acute phase during childhood contributes to increased prevalence of pregnant women with vascular sequelae of KD. 3 Management of these patients has not been established, especially in symptomatic patients and, in existing literature, premature labor has been performed in these cases. There are no Brazilian reports on the theme, which are more commonly found in the American and Japanese literature. The aim of this study is to describe a successful management of a patient with giant CAA, sequela of KD with thrombotic complication, from the first trimester of pregnancy until term birth. Case report A 32-year old patient was admitted to the emergency care at week nine of first pregnancy, with dyspnea and slight, precordial pain during great efforts, of short duration and well tolerated. The patient had a history of ST-elevation myocardial infarction of inferior wall at the age of 30, with giant aneurysms and coronary artery thrombosis evidenced by coronary angiography (Figure 1C and 1D). The patient underwent coronary computed tomography angiography, which confirmed the previous findings, with evidence of coronary artery ectasia with multiple aneurysmatic dilatations and mural thrombus (Figure 2). The patient received the diagnosis of KD and was referred to outpatient follow-up. At the occasion, the patient was using simvastatin, clopidogrel, atenolol and acetylsalicylic acid (ASA). At physical examination on admission, the patient was eupneic, with blood pressure of 110/60 mmHg, heart rate of 80 bpm, normal heart sounds without murmurs, normal pulmonary auscultation, abdomen free of abnormal signs, and normal pulse rate. Regarding complementary tests, electrocardiography showed sinus rhythm with diffuse ventricular repolarization; transthoracic echocardiogram is depicted in Figure 1A and 1B. Despite recommendations received in outpatient visits on contraindications for pregnancy, the patient got pregnant, and a close monitoring of the patient was started. A daily dose of 100 mg of ASA, 60 mg of propranolol and 40 mg of enoxaparin were prescribed, and routine obstetric exams showed normal fetal vital signs. After 29 weeks of pregnancy, the patient had progressive worsening of cardiac function to New York Heart Association (NYHA) class III, with daily, atypical palpitations, which caused the patient to get a sick leave to rest at home. During the week 34, the patient had diffuse chest pain, dyspnea and uterine contractions. The patient was then hospitalized for a rest and adjustment of medication. Obstetric examination revealed irregular, weak contractions, fundal height of 33 cm, impenetrable cervix, a single fetus in longitudinal position, cephalic presentation, regular heart beat at 128 bpm. Fetal assessment was performed by fetal biophysical profile and normal doppler velocimetry of umbilical arteries. Estimated fetal weight was adequate (percentile of Hadlock growth curve). There was a marked improvement in clinical and obstetric conditions as result of adjustments in propranolol (80 mg/day orally) and enoxaparin (60 mg/2x day subcutaneously) doses, as well as administration of sublingual nitrate (only if needed) and vaginal tablets of natural micronized progesterone (200 mg/2x day). There were no changes in electrocardiographic or echocardiographic patterns during hospitalization. At week 37, cesarean section and tubal ligation were indicated. The procedure was successfully performed by the Central Institute obstetric staff at the Heart Institute (InCor) of the General Hospital of the University of Sao Paulo Medical School. Newborn was born healthy, weight 2,860 g, appropriate for gestational age, Apgar score of 9 and 10 at fifth and tenth minute of life, respectively. Tubal ligation was performed following delivery, with previous consent of the spouse. Enoxaparin was discontinued 12 hours before and restarted 24 hours after cesarean section. Forty‑eight hours later, warfarin was prescribed and readjusted until the prothrombin international normalized ratio (INR) was 2; 97