ABC | Volume 112, Nº3, March 2019

Case Report Chronic Aortic Dissection and Pregnancy: Clinical Case Report Giulia de Miranda Taglialegna, Leila Katz, Larissa Mayara Aristóteles de Albuquerque, Milena Máximo de Freitas, Alexandre Jorge Gomes de Lucena, Melania Maria Ramos de Amori m Instituto de Medicina Integral Professor Fernando Figueira, Recife, PE – Brazil Mailing Address: Giulia de Miranda Taglialegna • Avenida Santos Dumont, 387, apto 402. Postal Code 52050-050, Aflitos, Recife, PE – Brazil E-mail: giuliademiranda@hotmail.com Manuscript received March 08, 2018, revised manuscript June 19, 2018, accepted July 02, 2018 Keywords Collagen Diseases/complications; Marfan Syndrome Ehlers- Danlos Syndrome; Aortic Coarction; Pregnancy; Postpartum Period; Aneurysm, Dissecting/surgery. DOI: 10.5935/abc.20190044 Introduction Aortic complications during pregnancy can occur in women without risk factors, but they are more often associated with collagen diseases (Marfan and Ehlers-Danlos syndromes) and structural cardiac malformations (aortic coarctation and bivalve aortic valve). 1,2 Approximately half of these events occur during pregnancy in women under 40 years of age, particularly in the third trimester, or in the postpartum period. 3 It is an extremely rare event, accounting for 0.1% to 0.4% of all aortic dissections, affecting 5.5 (95% CI: 4.0 - 7.8) women per 1 million during pregnancy and puerperium. 1,4,5 It is believed that the physiological hemodynamic changes during pregnancy, structural changes determined by compression of the gravid uterus and the hormonal action can aggravate this aortic pathology. 1,6 The most commonly used classifications are based on the affected anatomical portion. The Stanford group considers: type A (dissection involving ascending aorta) and type B (descending aorta only). 7 Depending on the duration of symptoms until the clinical presentation, aortic dissection can be classified as chronic, if it occurs two weeks or more after symptom onset. 6 We present herein a case of Stanford type B chronic aortic dissection in a patient at the 36 th week of pregnancy and discuss the particularities of the diagnosis and management of the potentially severe maternal and fetal complications. Case Report A 35-year-old single pregnant woman, of mixed-race, who had six prior pregnancies with five normal deliveries, at the 36 th week of pregnancy, originally from the municipality of Damião, state of Paraíba, Brazil, was referred from Campina Grande, Paraíba, to the intensive care unit (ICU) of IMIP after undergoing an obstetric ultrasonography (USG) that disclosed significant abdominal aorta dilatation. She reported a history of suggestive lancinating abdominal pain approximately one year ago (three months before the diagnosis of pregnancy). She arrived at the Service asymptomatic, receiving α -Methyldopa 750 mg/day, propranolol 40 mg/day, furosemide 40 mg/day and ASA 100 mg/day. At the physical examination she was conscious, eupneic, acyanotic, afebrile, with normal skin color, blood pressure (BP) of 100x68 mmHg and heart rate (HR) of 84 beats per minute (bpm). Respiratory auscultation showed no alterations and cardiac auscultation showed regular rhythm, normal heart sounds and no murmurs. She had depressible ++ / ++++ edema in the lower limbs; (below the 2.5 th percentile for gestational age), physiological tonus, with fetal movements, fetal heart rate of 132 bpm and absence of visceromegaly. Computed tomography (CT) of the chest and abdomen performed on the first day of hospitalization identified aortic dissection from the aortic arch to the abdominal aorta at the level of the renal arteries (Figures 1 and 2). She underwent an obstetric USG showing a single, live fetus, with estimated weight of 2.316 g, amniotic fluid index of 6 cm, normal umbilical artery Doppler, and gestational age of 35 weeks and 5 days. She remained asymptomatic until the third day of hospitalization, when she developed lower abdominal pain and uterine dynamics (three contractions in ten minutes), BP of 160x100 mmHg during contractions, and thus, a cesarean section was indicated. The procedure was performed under spinal anesthesia, with the cardiology and vascular surgical teams on alert, without complications, when bilateral tubal ligation was also performed. The newborn was a female, weighing 2.475 g, with an APGAR score of 8/9. At the postpartum, she was initially followed in the obstetric ICU and remained stable on propranolol 40 mg/day and amlodipine 5 mg/day, with adequate blood pressure control. She underwent an echocardiogram (2 nd postoperative day) that showed a 52% ejection fraction, dissection of the aorta with false‑lumen flow, and discrete aortic, mitral and tricuspid regurgitation. The heart chambers showed normal dimensions. On the 5 th postoperative day after the cesarean section, she was submitted to a thoracic endoprosthesis implantation without complications. On the 3 rd postoperative day after the implantation the patient developed a fever, of which originwas not determined and ceased after the use of vancomycin, piperacillin and tazobactam for 7 days, enoxaparin and prednisone. She was discharged from the 35 th postoperative day after the cesarean section and 28 th postoperative day after the aortic endoprosthesis implantation, on propranolol 120mg/day, amlodipine 10mg/day, losartan 100 mg/day and digoxin 0.125 mg/day and referred to cardiology outpatient follow-up. Discussion Aortic dissection during pregnancy is considered a potentially devastating clinical calamity for mother and conceptus. 2 Maternal mortality for acute Stanford type A and B dissection corresponds to 21% and 23%, with fetal death rates of 10.3% and 35%, respectively. 2 High rates are related 321