Ann Thorac Surg 2013;95:701–3
References 1. Austin EH, Wolfe WG. Aneurysm of aberrant subclavian artery with a review of literature. J Vasc Surg 1985;2:571–7. 2. Cina CS, Althami H, Pasenau J, Abouzahr L. Kommerell’s diverticulum and right-sided aortic arch: a cohort study and review of the literature. J Vasc Surg 2004;39:131–9. 3. Hastrier AR, D’Cruz IA, Cautez T. Right–sided aorta. Part 1. Occurrence of right aortic arch in various types of congenital heart disease. Br Heart J 1966;28:722–5. 4. Ueda T, Fleischmann D, Dake MD, Rubin GD, Sze DY. Incomplete endograft apposition to the aortic arch: bird-beak configuration increases risk of endoleak formation after thoracic endovascular aortic repair. Radiology 2010;255:645–52. 5. Rizvi AZ, Murad MH, Fairman RM, Erwin PJ, Montori VM. The effect of left subclavian artery coverage on morbidity and mortality in patients undergoing endovascular thoracic aortic interventions: a systematic review and meta-analysis. J Vasc Surg 2009;50:1159 – 69. 6. Holt PJ, Johnson C, Hinchliffe RJ, et al. Outcomes of the endovascular management of aortic arch aneurysm: implications for management of the left subclavian artery. J Vasc Surg 2010;51:1329 –38. 7. Matsumura JS, Lee WA, Mitchell RS, et al. The Society for Vascular Surgery Practice Guidelines: management of the left subclavian artery with thoracic endovascular aortic repair. J Vasc Surg 2009;50:1155– 8. © 2013 by The Society of Thoracic Surgeons Published by Elsevier Inc
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Aortic Valve-Sparing Reimplantation and Mitral Repair in a Pregnant, Second Trimester Marfan Patient: Surgical Decision Gilbert H.L. Tang, MD, MSc, Ramin Malekan, MD, Steven L. Lansman, MD, PhD, and David Spielvogel, MD Department of Surgery, Section of Cardiothoracic Surgery, New York Medical College, Westchester Medical Center, Valhalla, New York
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. (Ann Thorac Surg 2013;95:701–3) © 2013 by The Society of Thoracic Surgeons
P
regnant 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
Accepted for publication June 25, 2012. Address correspondence to Dr Spielvogel, Section of Cardiothoracic Surgery, Westchester Medical Center, 100 Woods Rd, Macy 114W, Valhalla, NY 10595; e-mail:
[email protected].
0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2012.06.049
FEATURE ARTICLES
ing aorta, it is not easy to ensure a sufficient proximal landing zone at the descending aorta. This forces the placement of stent-grafts at the aortic arch, which is severely angulated. In patients with a right aortic arch, it is difficult to deliver the stent-grafts through the aortic arch because of the severe angulation of the aortic arch. Furthermore, current stent-grafts are unable to conform to severely angulated or curved arches and are likely to be deployed in incomplete apposition, in a bird-beak configuration [4]. In our case, the proximal descending aorta above the Kommerell diverticulum was a rather hostile neck with a reversetapered configuration. The first proximal stent-graft was migrated to the distal side in performing a touch-up. Therefore, we deployed the additional stent-graft at the proximal landing zone without any touch-up, and immediately we deployed an extra-large Palmaz stent. With this method we deployed the stent-grafts with no migration, and the aneurysm was successfully repaired. The size of the stent-graft has to match the maximum diameter of a reverse-tapered landing zone, and touch-up at the minimum diameter might cause migration of the stent-graft to the area with a larger diameter. The procedure of choice for a reverse-tapered landing zone is deployment of a stent-graft without touch-up followed by immediate deployment of a bare stent. The necessity for revascularization of the left subclavian artery is controversial. Some studies have reported that coverage of a single subclavian artery during thoracic endovascular aortic repair increases the risk of vertebrobasilar ischemia, spinal cord ischemia, and anterior circulation stroke [5, 6]. Moreover, The Society for Vascular Surgery recommends revascularization of the vertebrobasilar vessels when possible [7]. In conclusion, endovascular treatment is a valuable alternative to standard surgical approaches to a ruptured Kommerell diverticulum of the aberrant left subclavian artery with a right-sided aortic arch.
CASE REPORT TANG ET AL VALVE REPAIR IN A PREGNANT MARFAN PATIENT
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CASE REPORT TANG ET AL VALVE REPAIR IN A PREGNANT MARFAN PATIENT
Ann Thorac Surg 2013;95:701–3
Fig 1. (A) Preoperative transesophageal echocardiography demonstrated a trileaflet aortic valve and (B) an aortic root aneurysm of 5.3 cm.
FEATURE ARTICLES
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
Fig 2. Transthoracic echocardiography at 4 months after surgery (gestational age, 29 weeks) demonstrated the Valsalva graft with an aortic annular diameter of 2.4 cm and a mitral valve annuloplasty ring (*). There was no aortic or mitral insufficiency.
(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.
Comment 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 [1]. Meijboom and colleagues [2] 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 [3]. 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 [1]. 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 [4]. 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 [5]. 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 [6], 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 [7], 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.
References 1. European Society of Gynecology; Association for European Paediatric Cardiology; German Society for Gender Medicine; Regitz-Zagrosek V, Lundqvist CB, Borghi C, et al. ESC 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;32:3147–97. 2. Meijboom LJ, Vos FE, Timmermans J, Boers GH, Zwinderman AH, Mulder BJ. Pregnancy and aortic root growth in the Marfan syndrome: a prospective study. Eur Heart J 2005;26: 914 –20. 3. Goland S, Elkayam U. Cardiovascular problems in pregnant women with Marfan syndrome. Circulation 2009;119:619 –23. 4. Manallo-Estrella P, Barker AE. Histopathologic findings in human aortic media associated with pregnancy. Arch Pathol 1967;83:336 – 41. 5. Arnoni RT, Arnoni AS, Bonini RCA, et al. Risk factors associated with cardiac surgery during pregnancy. Ann Thorac Surg 2003;76:1605– 8. © 2013 by The Society of Thoracic Surgeons Published by Elsevier Inc
CASE REPORT KUO ET AL AXILLOFEMORAL BYPASS REVERSES MALPERFUSION
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6. Vranes 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 – 6. 7. Patel A, Asopa S, Tang ATM, Ohri SK. Cardiac surgery during pregnancy. Tex Heart Inst J 2008;35:307–12.
Axillofemoral Bypass Relieves Visceral Malperfusion in Type B Aortic Dissection Hsun-Nan Kuo, MD, Hui-Chin Lai, MD, PhD, Yi-Wen Chang, MD, Chung-Chi Wang, MD, Wen-Lieng Lee, MD, PhD, Si-Wa Chan, MD, Kuo-Yang Wang, MD, Chih-Tai Ting, MD, PhD, and Tsun-Jui Liu, MD, PhD Departments of Anesthesiology and Radiology, Cardiovascular Center, Taichung Veterans General Hospital, Division of Cardiology, Department of Internal Medicine, Taichung Armed Forces General Hospital, and Departments of Medicine and Surgery, Cardiovascular Research Center, National Yang Ming University School of Medicine, Taipei; and Department of Medicine, Chung-Shan Medical University, Taichung, Taiwan
A 58-year-old man with acute type B aortic dissection presented with right lower limb cyanosis, mesenteric ischemia, and acute renal failure. He was treated with extraanatomic right axillofemoral bypass surgery alone, recovered completely from renal, mesenteric, and lower extremity malperfusion shortly thereafter, and lived free of symptoms for the following year. Follow-up computed tomography angiograms documented adequate expansion of the true aortic lumen and good perfusion of visceral organs. Thus, managing such patients with coexisting visceral and extremity malperfusion may be accomplished with axillofemoral bypass exclusively, which can relieve ischemia of upstream abdominal organs and downstream lower extremities effectively and durably. (Ann Thorac Surg 2013;95:703–5) © 2013 by The Society of Thoracic Surgeons
T
ype B aortic dissection with vital organ malperfusion carries a grave prognosis despite contemporary medical and surgical therapies [1]. We report a patient who had acute type B aortic dissection with visceral and lower extremity ischemia that was reversed by extraanatomic axillofemoral bypass surgery as the single nonpharmacologic therapy. A 58-year-old hypertensive man presented with abrupt backache, right leg weakness, and abdominal pain. A high brachial blood pressure (157/87 mm Hg), a cyanotic pulseless right lower extremity, and diminished
Accepted for publication June 27, 2012. Address correspondence to Dr Liu, Cardiovascular Center, Taichung Veterans General Hospital, 160, Section 3, Taichungkang Rd, Taichung 407, Taiwan; e-mail:
[email protected].
0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2012.06.056
FEATURE ARTICLES
Ann Thorac Surg 2013;95:703–5
