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• W2024651882 abstract "We describe a combined aortic valve-sparing reimplantation procedure and mitral valve repair in a 20-year-old pregnant Marfan patient who presented at 12 weeks' gestation with an asymptomatic 5.3-cm aortic root aneurysm and moderate mitral regurgitation. Because the risk of aortic dissection and worsening mitral regurgitation becomes significant in the third trimester, elective intervention was undertaken at 14 weeks' gestation. The mother recovered uneventfully, and the baby was delivered at term by cesarean section. This case supports early elective surgery in pregnant Marfan patients with aortic and mitral diseases to avoid potentially fatal cardiovascular complications later in the pregnancy. We describe a combined aortic valve-sparing reimplantation procedure and mitral valve repair in a 20-year-old pregnant Marfan patient who presented at 12 weeks' gestation with an asymptomatic 5.3-cm aortic root aneurysm and moderate mitral regurgitation. Because the risk of aortic dissection and worsening mitral regurgitation becomes significant in the third trimester, elective intervention was undertaken at 14 weeks' gestation. The mother recovered uneventfully, and the baby was delivered at term by cesarean section. This case supports early elective surgery in pregnant Marfan patients with aortic and mitral diseases to avoid potentially fatal cardiovascular complications later in the pregnancy. Pregnant women with Marfan syndrome and dilated aortic root present unique management challenges, given their increased risk of aortic dissection. Although termination of the pregnancy is advised, if it is continued surgical intervention is recommended if the aortic diameter exceeds 4.5 cm [1]. Marfan patients also have a higher prevalence of mitral valve prolapse and mitral regurgitation, which can worsen as a result of increased physiologic stress during pregnancy. We report a successful case of elective valve-sparing aortic root replacement and mitral repair on a pregnant Marfan patient who presented at 12 weeks' gestation with a 5.3-cm aortic root aneurysm and moderate mitral regurgitation. A 20-year-old woman with Marfan syndrome, known dilated aortic root, and mitral valve prolapse presented with pregnancy of 12 weeks' gestation. She had had bilateral lens dislocation requiring lens replacement and surgery for retinal detachment. Her pregnancy workup included echocardiography, revealing mild left ventricular dysfunction with an ejection fraction of 0.53; a 5.3-cm aortic root aneurysm, with effacement of the sinuses of Valsalva; a trileaflet aortic valve, with no aortic insufficiency (Fig 1) ; and mitral valve prolapse with moderate regurgitation. Computed tomographic angiography showed a 5.3-cm dilated aortic root, with normal coronary anatomy. The patient desired to continue her pregnancy, and because the risk of aortic dissection in Marfan syndrome significantly increases during the third trimester, as does the risk of developing severe mitral regurgitation, the decision was made to electively intervene on both the aortic root and mitral valve at an earlier stage of her pregnancy. An obstetrics consult advised that the optimal timing of surgery to minimize maternal and fetal risk would be at 14 weeks' gestation to allow completion of fetal organ and cerebral development. The patient underwent an aortic valve–sparing reimplantation procedure and mitral valve repair. Nonpulsatile cardiopulmonary bypass was conducted under mild hypothermia (34°C), with a target hematocrit of 26%. Pump flow at 2.0 L · min−1 · m−2 and mean arterial pressure in excess of 65 mm Hg were maintained to ensure adequate perfusion and protection of the fetus. Type I mitral dysfunction, with no significant leaflet disease, was found, and a size 40-mm Physio II (Edwards Lifesciences, Irvine, CA) annuloplasty ring was implanted. A valve-sparing reimplantation procedure was performed, using a 28-mm Vascutek Valsalva graft (Terumo Cardiovascular Systems, Ann Arbor, MI). Cross-clamp time was 189 minutes, and cardiopulmonary bypass time was 214 minutes. Intraoperative transesophageal echocardiography showed no residual aortic or mitral regurgitation. The patient recovered uneventfully and was discharged home on postoperative day 7. Transthoracic echocardiography at 4 months showed no recurrent aortic or mitral regurgitation (Fig 2). The patient and fetus remained healthy, and an elective cesarean section was performed uneventfully at term. Hormonal and hemodynamic changes make pregnancy in women with Marfan syndrome a high-risk period for developing aortic dissection, which, depending on aortic root size, may occur in up to 50% in the third trimester and 33% post partum [1Regitz-Zagrosek V. Lundqvist C.B. Borghi C. et al.European Society of Gynecology; Association for European Paediatric Cardiology; German Society for Gender MedicineESC guidelines on the management of cardiovascular diseases during pregnancy: the Task Force on the Management of Cardiovascular Diseases during Pregnancy of the European Society of Cardiology (ESC).Eur Heart J. 2011; 32: 3147-3197Crossref PubMed Scopus (32) Google Scholar]. Meijboom and colleagues [2Meijboom L.J. Vos F.E. Timmermans J. Boers G.H. Zwinderman A.H. Mulder B.J. Pregnancy and aortic root growth in the Marfan syndrome: a prospective study.Eur Heart J. 2005; 26: 914-920Crossref PubMed Scopus (191) Google Scholar] found that women with an aortic root diameter less than 4.5 cm appeared to tolerate pregnancy well. The risk of dissection in a normal aortic root is approximately 1% during pregnancy in Marfan patients, but increases significantly if the aortic root diameter enlarges or exceeds 4.0 cm [3Goland S. Elkayam U. Cardiovascular problems in pregnant women with Marfan syndrome.Circulation. 2009; 119: 619-623Crossref PubMed Scopus (61) Google Scholar]. Current European Society of Cardiology guidelines recommend that Marfan women should be discouraged from becoming pregnant if their aortic root diameter exceeds 4.5 cm or undergo surgery before pregnancy [1Regitz-Zagrosek V. Lundqvist C.B. Borghi C. et al.European Society of Gynecology; Association for European Paediatric Cardiology; German Society for Gender MedicineESC guidelines on the management of cardiovascular diseases during pregnancy: the Task Force on the Management of Cardiovascular Diseases during Pregnancy of the European Society of Cardiology (ESC).Eur Heart J. 2011; 32: 3147-3197Crossref PubMed Scopus (32) Google Scholar]. In the present case, although the patient had a history of aortic root aneurysm, she was lost to follow-up, presenting at 12 weeks' gestation with a 5.3-cm aortic root aneurysm, making the risk of dissection very high. As she desired to continue her pregnancy, an elective aortic root procedure was deemed advisable. In consultation with obstetrics and neonatology, early intervention was recommended for two reasons. First, elevated levels of estrogen and progesterone could inhibit maternal collagen and elastin deposition in the aorta during pregnancy, leading to reticulin fiber fragmentation, elastin loss, and weakening of the aortic wall [4Manallo-Estrella P. Barker A.E. Histopathologic findings in human aortic media associated with pregnancy.Arch Pathol. 1967; 83: 336-341PubMed Google Scholar]. Also, hemodynamic stress significantly increases as pregnancy advances as a result of increased cardiac output and circulating blood volume, increasing the susceptibility of the Marfan aorta to dissection and rupture. Second, the growing fetus progressively compresses the abdominal aorta and inferior vena cava, complicating the management of cardiopulmonary bypass and increasing the operative risk to both the mother and fetus. Surgery performed relatively early in the pregnancy minimizes these risks. The optimal timing for surgical intervention in pregnant women has been reported to range from 13 to 28 weeks [5Arnoni R.T. Arnoni A.S. Bonini R.C.A. et al.Risk factors associated with cardiac surgery during pregnancy.Ann Thorac Surg. 2003; 76: 1605-1608Abstract Full Text Full Text PDF PubMed Scopus (91) Google Scholar]. Intervening during the first trimester may result in fetal malformation, and intervening during the third trimester may risk preterm delivery and mortality to both mother and fetus. Our patient presented at 12 weeks' gestation, when fetal organogenesis should have been completed. Given that delaying intervention until after birth increases the risk of aortic dissection in Marfan patients with a dilated aortic root [6Vranes M. Velinovic M. Kovacevic-Kostic N. Savic D. Nikolic D. Karan R. Pregnancy-related aortic aneurysm and dissection in patients with Marfan's syndrome: medical and surgical management during pregnancy and after delivery.Medicina (Kaunas). 2011; 47: 604-606PubMed Google Scholar], it was elected to proceed with surgery. Pregnant women with Marfan syndrome may also experience worsening mitral regurgitation, especially if moderate to severe regurgitation predated pregnancy. In the present case, the patient had an aortic root aneurysm and moderate mitral regurgitation and therefore needed concomitant repairs. The conduct of cardiopulmonary bypass during pregnancy should include maintaining mild hypothermia, high blood flow and systemic pressure, and adequate hematocrit to protect the fetus. Fetal cardiac monitoring is important if surgery is performed in the late second or third trimester, and a high-risk obstetrician and neonatologist should be present during the operation in case an emergency cesarean section is required. Animal studies have suggested that pulsatile perfusion on cardiopulmonary bypass may help to maintain placental perfusion and limit a rise in placental vascular resistance [7Patel A. Asopa S. Tang A.T.M. Ohri S.K. Cardiac surgery during pregnancy.Tex Heart Inst J. 2008; 35: 307-312PubMed Google Scholar], but no definitive clinical benefit has been observed. In the present case, mild hypothermia (34°C), a hematocrit of 26%, and mean arterial pressure greater than 65 mm Hg were maintained during cardiopulmonary bypass. The decision to proceed with early elective surgery for an aortic root aneurysm and mitral regurgitation in a pregnant woman with Marfan syndrome led to an uneventful delivery and recovery for the mother and child in this case." @default.
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• W2024651882 title "Aortic Valve-Sparing Reimplantation and Mitral Repair in a Pregnant, Second Trimester Marfan Patient: Surgical Decision" @default.
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