Case report
Marfan syndrome in a term-pregnant woman with aortic root dilatation between 40 and 45mm
Síndrome de Marfan en gestante a término con dilatación de la raíz aórtica entre 40 y 45mm
D.R. Delgado García
, P. Latorre Andreu, B. Fernández Tomás, M.I. Tébar Cuesta
Corresponding author
Servicio de Anestesiología y Reanimación, Hospital 12 de Octubre, Madrid, Spain
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Delgado García, P. Latorre Andreu, B. Fernández Tomás, M.I. Tébar Cuesta" "autores" => array:4 [ 0 => array:4 [ "nombre" => "D.R." "apellidos" => "Delgado García" "email" => array:1 [ 0 => "dadegar@gmail.com" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "P." "apellidos" => "Latorre Andreu" ] 2 => array:2 [ "nombre" => "B." "apellidos" => "Fernández Tomás" ] 3 => array:2 [ "nombre" => "M.I." "apellidos" => "Tébar Cuesta" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Servicio de Anestesiología y Reanimación, Hospital 12 de Octubre, Madrid, Spain" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Síndrome de Marfan en gestante a término con dilatación de la raíz aórtica entre 40 y 45<span class="elsevierStyleHsp" style=""></span>mm" ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Marfan syndrome (MS) is an autosomal dominant hereditary disorder of connective tissue. The alteration is found in the gene that codes for fibrillin-1 in the extracellular matrix, and the main cause of mortality in these patients is cardiovascular complications related to aneurysmal dilatation and dissection of the aortic root.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">The physiological changes that occur during pregnancy are associated with an increased risk of aortic dilatation and dissection (incidence of 1%). In these cases, a pre-pregnancy clinical assessment should be performed, including echocardiography to assess the size of the aortic root, which, if greater that 40<span class="elsevierStyleHsp" style=""></span>mm, is at high risk of rupture.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> Some recent guidelines recommend that women with SM and an aortic root diameter<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>40<span class="elsevierStyleHsp" style=""></span>mm should avoid pregnancy, or should undergo ascending aorta replacement surgery before conception if the aorta measures<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>45<span class="elsevierStyleHsp" style=""></span>mm. The medical treatment of choice is beta-blockers, and labetalol or metoprolol (Food and Drug Administration category <span class="elsevierStyleSmallCaps">C</span>) in pregnant women, which should not be withdrawn at any time (irrespective of whether the birth is by caesarean vaginal delivery).<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a></p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Case report</span><p id="par0015" class="elsevierStylePara elsevierViewall">We report the case of a 32-year-old female weighing 77<span class="elsevierStyleHsp" style=""></span>kg, height 179<span class="elsevierStyleHsp" style=""></span>cm, diagnosed with SM in 2014. She exhibited no cardiovascular symptoms, although echocardiograpy and magnetic resonance angiography showed a dilated ascending aorta which, between 2014 and 2018, had increased in diameter from 38 to 41<span class="elsevierStyleHsp" style=""></span>mm before pregnancy to 41 to 42<span class="elsevierStyleHsp" style=""></span>mm towards the end of pregnancy. She was treated with atenolol, which was replaced with metoprolol during pregnancy, with good blood pressure control. Her family history was significant for her father, who had been diagnosed as marfanoid habitus and was undergoing follow-up for aortic aneurysm.</p><p id="par0020" class="elsevierStylePara elsevierViewall">The mode of delivery was decided by a multidisciplinary team of cardiologists, obstetricians and anaesthesiologists. After evaluating the case, the team recommended scheduled caesarean section under general anaesthesia to avoid the risk associated with physiological changes related to vaginal delivery. General anaesthesia was chosen for 2 reasons: to facilitate haemodynamic management; and because the possible presence of dural ectasia, which had not previously been ruled out by magnetic resonance, could result in ineffective spinal anaesthesia.</p><p id="par0025" class="elsevierStylePara elsevierViewall">The patient underwent scheduled caesarean section at 38 weeks and 4 days of gestation. After starting standard monitoring, the radial artery was cannulated for continuous blood pressure monitoring, given the importance of avoiding haemodynamic instability. Following this, preoxygenation and rapid sequence induction of general anaesthesia was performed with 300<span class="elsevierStyleHsp" style=""></span>mg sodium thiopental, remifentanil infusion of around 0.08–0.1<span class="elsevierStyleHsp" style=""></span>mg/kg/min and 75<span class="elsevierStyleHsp" style=""></span>mg of succinylcholine. Intubation was successful at the first attempt using the Airtraq<span class="elsevierStyleSup">®</span> airway (Prodol Meditec, Vizcaya, Spain), and a central venous line was inserted through the right internal jugular vein. Anaesthesia was maintained with sevoflurane at a MAC of around 1.6, together with O<span class="elsevierStyleInf">2</span>/air, neuromuscular relaxation with rocuronium, and continuous perfusion of remifentanil at 0.1<span class="elsevierStyleHsp" style=""></span>μg/kg/min. Paediatrics had already been warned that the infant might need ventilatory support due to the use of opiates.</p><p id="par0030" class="elsevierStylePara elsevierViewall">After delivery, a bolus of 3<span class="elsevierStyleHsp" style=""></span>IU oxytocin was administered, followed by continuous infusion of 1.26<span class="elsevierStyleHsp" style=""></span>IU/h over the following 24<span class="elsevierStyleHsp" style=""></span>h. The patient remained haemodynamically stable during the procedure, with well-controlled blood pressure. The infant had an Apgar score of 3 at 1<span class="elsevierStyleHsp" style=""></span>min of life, 7 at 5<span class="elsevierStyleHsp" style=""></span>min, and 10 at 10<span class="elsevierStyleHsp" style=""></span>min of life.</p><p id="par0035" class="elsevierStylePara elsevierViewall">Non-steroidal anti-inflammatories were given for postoperative analgesia, and bilateral, ultrasound-guided transverse abdominis plane block was performed with 15-ml of 0.25% levobupivacaine.</p><p id="par0040" class="elsevierStylePara elsevierViewall">After reversing neuromuscular blockade with sugammadex, the patient was extubated in the operating room and then transferred to the postoperative care unit for monitoring. She remained haemodynamically stable, with good pain control, good uterine contraction and urine output, and was discharged to the ward 24<span class="elsevierStyleHsp" style=""></span>h after the intervention. The puerperium was uneventful, with a 1-month follow-up echocardiogram showing no progression of ascending aorta dilation.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0045" class="elsevierStylePara elsevierViewall">The best approach to anaesthesia management in pregnant women with MS has yet to be defined. As mentioned above, MS is characterised, among other manifestations, by musculoskeletal involvement, together with dural ectasia and dilatation of the aortic root. In the absence of treatment (beta-blockers), the aortic dilation tends to worsen over time. Pregnancy can accelerate this process due to its effect on connective tissue (more fragile vascular wall) and on haemodynamics.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a> These changes begin in the first and second trimesters, but the third is the period of maximum stress, with an increase in heart rate, stroke volume, blood volume and blood pressure.<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">3,4</span></a> The pregnancy-induced increase in cardiac output reaches a peak immediately after delivery because of the effects of labour pain and autotransfusion secondary to uterine contractions.<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">5</span></a> Therefore, both pregnancy and childbirth in women with MS can be considered a risk situation for aortic dissection, especially when the aortic root is<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>40<span class="elsevierStyleHsp" style=""></span>mm.<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">2,4–6</span></a> The target mean arterial pressure should be about 65–70<span class="elsevierStyleHsp" style=""></span>mmHg, or the lowest blood pressure tolerated by the patient.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Due to changes in the connective tissue of the aortic wall, all pregnant women with MS can be considered to be at risk of aortic dissection, regardless of the size of the aortic diameter. However, a diameter<span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>40<span class="elsevierStyleHsp" style=""></span>mm is associated with low risk (if beta-blockers are administered) and<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>45<span class="elsevierStyleHsp" style=""></span>mm with very high risk<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a>; but, what if the aorta is between 40 and 45<span class="elsevierStyleHsp" style=""></span>mm? Although there is a tendency towards standardisation in the literature, these cases should be approached individually and considered together with other factors. One such factor in the ratio of the aortic diameter to the body surface, particularly in small women, where diameters<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>27<span class="elsevierStyleHsp" style=""></span>mm/m<span class="elsevierStyleSup">2</span> indicate a high risk of dissection.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a> Another factor to consider is the existence of a familial or personal history of aneurysm or aortic dissection. It goes without saying that the risk will increase if the diameter of the aortic root is closer to 45<span class="elsevierStyleHsp" style=""></span>mm than 40<span class="elsevierStyleHsp" style=""></span>mm. Finally, an increase in aortic diameter<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>1<span class="elsevierStyleHsp" style=""></span>mm during pregnancy has been associated with an increased risk of dissection.<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a> What is clear is the need for thorough follow-up with imaging tests during and after pregnancy to monitor the cardiovascular system and the possible progression of dilation.</p><p id="par0055" class="elsevierStylePara elsevierViewall">The choice of delivery mode, leaving aside any strictly obstetric indications, will be made based on the patient's history and the diameter of the aortic root. In women with<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>45-mm aortic dilatation, caesarean section, and even pre-pregnancy aortic replacement appear to be indicated. In patients with 40–45<span class="elsevierStyleHsp" style=""></span>mm aortic dilatation, as in our case, the option of caesarean section should be weighed up by a multidisciplinary team on a case by case basis, taking into consideration the foregoing factors.<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">2,4</span></a> If vaginal delivery is indicated, epidural analgesia should be administered early to relieve pain in the second stage of labour and thus reduce the risk of blood pressure peaks that can induce aortic wall dissection.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a> Suspicion of difficult or impossible epidural puncture could be another reason for suggesting a caesarean section. Mindful of the repeated recommendations in the literature regarding the need for strict control of blood pressure in these patient, we decided to perform a caesarean with general anaesthesia, which would greatly facilitate the management of sudden peaks in cardiac output that could endanger the patient's life.</p><p id="par0060" class="elsevierStylePara elsevierViewall">The choice of anaesthesia for a caesarean section in a woman with MS and aortic dilatation is based on several key considerations, although there is no evidence in the literature to show the superiority of one anaesthesia technique over any other. The first consideration is that general anaesthesia is more likely to facilitate good haemodynamic control. Assuming that these patients have already been prescribed beta-blocker therapy, the hypertensive response to laryngoscopy during the induction of general anaesthesia could be alleviated in several ways, including the administration of drugs, particularly remifentanil, an ultra-short-acting, relatively safe option. If used, the paediatric team should be notified that the newborn could present respiratory depression, which in the case of remifentanil will usually revert spontaneously or with the administration of naloxone. Another method of reducing the haemodynamic response to laryngoscopy is the use of videolaryngoscopy. We used the Airtraq, which in comparative studies with a Macintosh blade has shown greater ease of insertion and less haemodynamic involvement during laryngoscopy.<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a> Given that avoiding hypertension is more important than avoiding mild hypotension, it could be argued this haemodynamic goal is more easily attained using a neuraxial technique than general anaesthesia.<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">5</span></a> However, these patients can present complicating factors, such as dural ectasia, which some series estimate to occur in 92% of MS patients.<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">9</span></a> Due to this, the intrathecal spread of the anaesthetic can be unpredictable and inadequate, resulting in the failure of the technique.<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">9</span></a> Epidural analgesia and anaesthesia in patients with moderate to severe dural ectasia is not recommended due to the increased risk of accidental dural puncture.<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">10</span></a> However, experts now agree that epidural anaesthesia (at titrated doses) should be considered the technique of choice, provided the caesarean is scheduled. This technique gives good perioperative haemodynamic and analgesic control, and its effect is more predictable than intradural techniques, except in cases of haemodynamic decompensation. The degree of dural ectasia can be measured by magnetic resonance. However, if it has not been performed, as was the case in our patient, it must be assumed to be present.</p><p id="par0065" class="elsevierStylePara elsevierViewall">In conclusion, it is best to approach the anaesthetic and obstetrical management of pregnant women with SM and aortic dilatation (particularly 40–45<span class="elsevierStyleHsp" style=""></span>mm diameter) on a case by case basis. Management should be undertaken by a multidisciplinary team that will decide the best and safest delivery mode for both the mother and the foetus. The type of anaesthesia used depends on the experience of the anaesthesiologist and the patient's status and comorbidities.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conflicts of interest</span><p id="par0070" class="elsevierStylePara elsevierViewall">None.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:9 [ 0 => array:3 [ "identificador" => "xres1135250" "titulo" => "Abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0005" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec1067349" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres1135249" "titulo" => "Resumen" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0010" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec1067348" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Case report" ] 6 => array:2 [ "identificador" => "sec0015" "titulo" => "Discussion" ] 7 => array:2 [ "identificador" => "sec0020" "titulo" => "Conflicts of interest" ] 8 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2018-06-11" "fechaAceptado" => "2018-08-31" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec1067349" "palabras" => array:7 [ 0 => "Marfan syndrome" 1 => "Aortic root dilation" 2 => "Caesarean section" 3 => "Anaesthesia" 4 => "Obstetrics" 5 => "Obstetric anaesthesia" 6 => "Aortic root diameter" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec1067348" "palabras" => array:7 [ 0 => "Síndrome de Marfan" 1 => "Dilatación raíz aórtica" 2 => "Cesárea" 3 => "Anestesia" 4 => "Obstetricia" 5 => "Anestesia obstétrica" 6 => "Diámetro raíz aórtica" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Marfan syndrome is a hereditary connective tissue disorder. The main cause of mortality in these patients is due to cardiovascular complications related to dilation of an aneurysm and dissection of the aortic root, a situation that increases their risk due to the physiological changes that occur during pregnancy, childbirth and puerperium. The case is presented of a pregnant woman with Marfan syndrome and aortic root dilatation of 42<span class="elsevierStyleHsp" style=""></span>mm. The issues are discussed, such as the mode of delivery (vaginal delivery vs. caesarean section) depending on the aortic root diameter or the choice of type of anaesthesia (general vs. neuraxial) in these cases.</p></span>" ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">El síndrome de Marfan es un trastorno hereditario del tejido conectivo. La principal causa de mortalidad en estas pacientes es debida a complicaciones cardiovasculares relacionadas con dilatación aneurismática de la raíz aórtica y disección de la misma, situación que aumenta su riesgo con los cambios fisiológicos que ocurren durante el embarazo, el parto y el puerperio. Presentamos el caso de una paciente embarazada que presentaba síndrome de Marfan y dilatación de la raíz aórtica de 42<span class="elsevierStyleHsp" style=""></span>mm, e intentamos arrojar luz sobre temas como son la vía de parto (parto vaginal vs. cesárea) en función del diámetro aórtico o la elección del tipo de anestesia (general vs. neuroaxial) en estos casos.</p></span>" ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Delgado García DR, Latorre Andreu P, Fernández Tomás B, Tébar Cuesta MI. Síndrome de Marfan en gestante a término con dilatación de la raíz aórtica entre 40 y 45<span class="elsevierStyleHsp" style=""></span>mm. Rev Esp Anestesiol Reanim. 2019;66:49–52.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:10 [ 0 => array:3 [ "identificador" => "bib0055" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Epidural anesthesia for cesarean section in a pregnant woman with marfan syndrome and dural ectasia" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "F. Pepe" 1 => "M. Stracquadanio" 2 => "F. de Luca" 3 => "A. Privitera" 4 => "E. Sanalitro" 5 => "P. 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