Hindawi Case Reports in Anesthesiology Volume 2018, Article ID 2616390, 5 pages https://doi.org/10.1155/2018/2616390

Case Report Anesthetic Implications for Cesarean Section in a Parturient with Complex Congenital Cyanotic Disease

Huili Lim, Chuen Jye Yeoh, Jerry Tan, Harikrishnan Kothandan ,andMayU.S.Mok

Department of Anesthesia and Intensive Care, Singapore General Hospital, Singapore

Correspondence should be addressed to Harikrishnan Kothandan; [email protected]

Received 18 October 2017; Accepted 28 February 2018; Published 29 March 2018

Academic Editor: Latha Hebbar

Copyright © 2018 Huili Lim et al. Tis is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Te discordance between increased physiological demand during pregnancy and congenital cardiac pathology of a parturient is a perilous threat to the maternal-fetal well-being. Early involvement of a multidisciplinary team is essential in improving peripartum morbidity and mortality. Designing the most appropriate anesthetic care will require a concerted efort, with inputs from the obstetricians, obstetric and cardiac anesthesiologists, cardiologists, neonatologists, and cardiothoracic surgeons. We report the multidisciplinary peripartum care and anesthetic management for cesarean section (CS) of a 28-year-old primigravida who has partially corrected transposition of the great arteries, atrial and ventricular septal defect, , right hypoplasia, and .

1. Introduction septal defect (ASD), dextrocardia with atrial and abdominal situs solitus, hypoplastic right ventricle, tricuspid atresia, and In Asia, congenital heart disease occurs in approximately 9.3 multiple aortopulmonary collaterals to the right lung. She had per 1000 live births [1]. Modern medicine has substantially banding at 6 weeks of age and bidirectional improved the survival of patients with major congenital heart cavopulmonary connections (also known as Glenn’s shunt) disease and extended their life expectancy beyond reproduc- were performed at the age of 18 (see Figure 1.) Completion tive age. Te complex nature of the cardiac anomaly coupled of Fontan’s procedure was held of indefnitely because of withthewidearrayofsophisticatedtemporizingorcorrective an incidental fnding of cerebral arteriovenous malformation procedures performed on these patients translates into a (AVM). Te AVM was deemed unsuitable for embolization daunting task for the anesthesiologists. Te physiological duetoitsconnectionwithalargevenouspouch.Shehad changes associated with pregnancy and delivery compound been counselled against pregnancy by her cardiologist before the problem further. conception. Te patient’s other comorbidities include mild Writtenconsentwasobtainedfromthepatientforpub- scoliosis, prolapsed intervertebral disc between the fourth lication with approval from local ethics committee. Tis and ffh lumbar vertebrae, previous infective endocarditis manuscript adheres to the applicable EQUATOR guidelines. complicated by a cerebrovascular accident (full neurological recovery from lef-sided numbness), and compensatory poly- 2. Case Description cythemia (hemoglobin concentration: 20–22 g/dL). At 17 weeks’ gestation, the patient was admitted by her Our patient is a 28-year-old primigravida who presented with cardiologist because of increasing cyanosis and dyspnea. New York Heart Association (NYHA) class III symptoms Her oxygen saturation (SpO2)onairdecreasedfrom90% at 17 weeks’ gestation. She was diagnosed with complex before pregnancy to 80% on admission. Obstetrical work- congenital cyanotic heart disease at birth and her cardiac up revealed a healthy viable fetus. Serial transthoracic echo- malformations included transposition of great arteries, large cardiography showed an ejection fraction of 55% with bidi- nonrestrictive ventricular septal defect (VSD) and atrial rectional shunt across the VSD. Due to pulmonary artery 2 Case Reports in Anesthesiology

Figure 1: Patient’s abnormal cardiac anatomy (picture courtesy of cardiologist Dr. Tan Ju Le). (1) Transposition of great arteries: the morphologic lef ventricle is connected to the pulmonary artery (PA) and ; the hypoplastic right ventricle is connected to the aorta and systemic circulation. (2) Te tight pulmonary artery band limits blood fow from the lef ventricle into the pulmonary circulation to prevent development of pulmonary hypertension and to direct oxygenated blood from lef ventricle to right ventricle through the large nonrestrictive ventricular septal defect (VSD) and then from right ventricle to systemic circulation. (3) Systemic venous blood from the enters the pulmonary circulation for oxygenation via the bidirectional cavopulmonary connection. (4) Systemic venous blood from the inferior vena cava returns to the right and enters lef atrium via the large nonrestrictive (ASD), from lef atrium to the morphologic lef ventricle. Due to the congenital tricuspid atresia, there is no blood fow form right atrium to right ventricle. (5) Oxygenated blood from the lungs enters the pulmonary veins, fows to lef atrium and lef ventricle and then through the ventricular septal defect to right ventricle. Both the hypoplastic right ventricle and the lef ventricle (via the ventricular septal defect) eject blood into the aorta. Bidirectional shunting occurs across the ventricular septal defect with mixing of oxygenated and deoxygenated blood.

banding, transpulmonary gradient was 41 mmHg. Te obstet- bytheobstetricianandtheanesthesiologists.Teteammet ric anesthesiologist and obstetrician were alerted from the regularly with the following agenda: (1) update on patient’s time of admission. Te patient was again counselled exten- condition, (2) update on fetal condition (afer 24 weeks), (3) sively regarding the risks of further maternal decompensation plans for an early elective delivery, and (4) formulation of and fetal anomaly. However, she remained keen to continue an emergency plan in the event of acute maternal decom- withthepregnancy.Tepatientstayedinthecardiology pensation. Te multidisciplinary team had consensus that high dependency ward for bed rest, oxygen therapy, and maternal condition would deteriorate in the 3rd trimester, monitoring by a cardiologist who was specialized in adult and cesarean section (CS) should happen before 28 weeks congenital heart disease from 17 weeks’ gestation until the of gestation to avoid maternal decompensation. Nearing the point of delivery. end of 2nd trimester, the patient was demonstrating NYHA A multidisciplinary team comprising cardiologists, obste- class IV symptoms and would desaturate during minimal tricians, obstetric and cardiothoracic anesthesiologists, car- eforts such as eating and speaking. Her highest SpO2 value diothoracic surgeons, and neonatologists was established prior to delivery was 85% on a nonrebreathing mask with from the time of admission. Te discussions were led jointly 15 L/min of oxygen. A decision was made for an elective Case Reports in Anesthesiology 3

CStobeperformedat27weeksofgestationinviewofher view of the presence of VSD. Cardiac function was contin- worsening functional capacity. Tis was to allow the conduct uously monitored by TEE performed by the cardiologist in of anesthesia and surgery under controlled conditions. direct contact with the anesthesiologist. A general anesthetic technique was jointly agreed upon Following the delivery of the baby, three intravenous by the cardiac and obstetric anesthesiologists because of the boluses of oxytocin 1 U were administered, which achieved potential for less hemodynamic instability. Members of the satisfactory uterine contraction. Tere were no noticeable multidisciplinary team were present in the operating theatre changes in the vital signs during autotransfusion. on the day of surgery. Te perfusionists were also on standby At the end of surgery and before tracheal extubation, in preparation for the possible need for extracorporeal mem- bilateral transversus abdominis plane blocks were performed. brane oxygenation (ECMO). Te muscle relaxant was reversed with sugammadex 2 mg/kg. Equipment necessary for both maternal and neonatal care Te hemodynamic stimulation during tracheal extubation and monitoring were available, including transesophageal was blunted by titrated boluses of esmolol. echocardiography (TEE), nitric oxide for inhalation, and Afer tracheal extubation, she was monitored in the neonatal resuscitator (medical device). Intravenous cardio- intensivecareunitfor1day.Shewassubsequentlytransferred vascular drugs (nitroglycerine, norepinephrine, epinephrine, to a high dependency ward under the care of the cardiologist and milrinone) were prepared. Before the induction of and had no episodes of acute desaturation during her stay. She anesthesia, the vascular access sites for emergency ECMO was discharged home on postoperative day 10. support were identifed and marked. Standard monitors were Te baby was delivered weighing 870 g and was sent to attached, and the lef radial artery and lef internal jugular neonatalintensivecareunit.Hewassubsequentlydischarged vein were cannulated for intra-arterial and central venous well. pressure lines. Te lef internal jugular vein was cannulated as the right internal jugular vein was noted to be small on 3. Discussion ultrasound imaging. A pulmonary artery catheter was not considered in this patient as it would be technically difcult. In industrialized countries, maternal congenital heart disease Tis was because the superior vena cava was anastomosed to is the most common cause of mortality in parturients with the right pulmonary artery. Typical pulmonary artery tracing preexisting cardiovascular disease. Mortality risk of up to would not be obtainable as the blood fow to the lung is 30% is associated with poor NYHA class status, severe passive in nature due to her altered anatomy. ventricular dysfunction, severe , Marfan’s syn- Tepatient’sairwaywaslocallyanesthetizedwith5mLof drome with lesion or aortic dilatation, or pul- 1% nebulized lidocaine and cophenylcaine sprays to attenuate monary hypertension [2]. the sympathetic response to laryngoscopy and endotracheal A best evidence topic presented by Asfour et al. in 2013 intubation. Before induction of anesthesia, an antibiotic was deems vaginal delivery to be safer in patients who are NYHA prophylactically administered as per institutional protocol. classes I and II in labour, but an expedited instrument- Te surgical site was cleansed with antiseptic solution, and assisted vaginal delivery in patients with poorer NYHA status surgical drapes were attached. Rapid sequence induction of is feasible with good analgesia. Right ventricular failure, pul- anesthesia was performed with cricoid pressure. Induction monary regurgitation, and pulmonary hypertension impart drugs used include fentanyl 100 mcg, ketamine 25 mg, etomi- the greatest risk to both mother and baby [3]. Due to the poor date 10 mg, and rocuronium at 1 mg/kg (research papers have maternal state of our patient antenatally and the prematurity supportedtheuseof1mg/kgforrapidsequenceinduction). of the fetus, the multidisciplinary team decided that CS under Te trachea was intubated with a 7.5 mm endotracheal tube. controlled conditions is the safest option. Intraoperatively, the patient was hemodynamically stable Afer multidisciplinary discussions, we decided to per- with mean arterial pressure (MAP) remaining more than form the cesarean section under general anesthesia. We con- 70 mmHg throughout. Te SpO2 value remained within sidered general anesthesia as the method of choice because of 10% of her baseline at 81–87%. Excessive airway pressure a possibly lower risk of hemodynamic instability compared was avoided by setting a low tidal volume (350 mL) and a to neuraxial anesthesia. Spinal and to a lesser extent epidural higher respiratory rate (15) for volume-controlled mechanical anesthesia will lead to profound sympathetic block. General ventilation. Peak end expiratory pressure was set at zero. anesthesia using carefully selected anesthetic agents known to Peak airway pressure was consistently at or below 20 cm produce less hemodynamic efects will ofer more predictabil- H2O. Te patient was closely monitored for hypercapnia and ity and stability. In addition, general anesthesia will enable the hypoxia. Hypothermia was prevented with the use of Bair use of TEE intraoperatively. Te underlying lumbar scoliosis hugger warming blanket and Hotline fuid warmer. Preload and disc prolapse might have complicated the administra- was optimized by trending the central venous pressure (CVP tion of neuraxial anesthesia. Accidental or deliberate dural readings) and was guided by real-time TEE imaging. Te puncture occurring during attempted epidural or spinal anes- CVP readings were, in fact, pulmonary artery pressure read- thesia,respectively,mighthavecausedruptureofthecerebral ings as the superior vena cava in this patient was anastomosed AVM secondary to a change in transmural pressure [4]. Te to the right pulmonary artery. Te hemoglobin concentration patient was also too breathless to tolerate the supine position was kept above 16 g/dL (patient’s baseline was approximately required for the surgery. 20–22 g/dL). Blood products were available on standby. Air For patients with Glenn shunt in situ, the superior vena bubbles in intravenous drip sets were carefully avoided in cava is anastomosed to the right pulmonary artery, such that 4 Case Reports in Anesthesiology blood bypasses the malformed chambers of the right heart should be formulated in anticipation of maternal deteriora- andisshunteddirectlyintothelungsforoxygenation.Anes- tion requiring emergency surgery. thetic goals include (a) maintenance of adequate preload, (b) Te mode of anesthesia and delivery should be decided preservation of sinus rhythm and myocardial contractility, based on patient-specifc factors, and the decision should be (c) maintenance of a low pulmonary and normal systemic made in conjunction with the obstetrician, cardiologist, and vascular resistance, (d) avoidance of hypoxia, hypercarbia, neonatologist. acidosis, and hypothermia, which may increase right to lef shunt, (e) avoidance of air bubbles in intravenous drip sets, (f) avoidance of excessive airway pressure by maintaining low Conflicts of Interest tidal volume ventilation and avoiding the use of peak end Te authors declare that there are no conficts of interest expiratory pressure, and (g) maintenance of hemoglobin level regarding the publication of this paper. above 16 g/dL. Te stress response to intubation as well as surgeryshouldbewellblunted. Central venous pressure (CVP) line was inserted in the Authors’ Contributions lef internal jugular vein. CVP in this patient denotes the pulmonary artery (PA) pressure as the superior vena cava Huili Lim, Chuen Jye Yeoh, and Jerry Tan contributed to is directly connected to the right PA. In addition, a central literature review and drafing of the paper. Huili Lim, Chuen venous line is necessary for administration of inotropic drugs Jye Yeoh, Jerry Tan, May U. S. Mok, and Harikrishnan if required. Kothandan contributed to revision of the paper. Huili Lim General anesthesia is not without its perils. A reduction was responsible for submitting the paper. in venous return due to vasodilation, myocardial depression due to anesthetic agents, and reduction in systemic vascular Acknowledgments resistance will result in worsening of the right to lef shunt in this patient [5]. Atelectasis following general anesthesia Te authors would like to thank Adjunct Assistant Professor mayalsoworsentherighttolefshunt.Sympatheticdischarge Tan Ju Le (MBBS (UK), MRCP(UK), FAMS (Cardiology), during laryngoscopy which is known to be more difcult FACC (USA), and FESC (France)) for allowing them to use in parturients [6] can lead to rupture of the AVM with her diagram of the patient’s altered cardiac anatomy in this devastating consequences. case report. Adjunct Assistant Professor Tan Ju Le is a senior Etomidate and ketamine were used as induction agents in consultant with the Department of Cardiology in National view of their cardiovascular stability. Etomidate causes min- Heart Centre Singapore (NHCS); she is also the Director of imalchangesinsystemicandpulmonaryvascularresistance the Adult Congenital Heart Disease Programme at NHCS. and the cardiac output. Specifcally, ketamine administration in patients with congenital heart disease has been shown to produce minimal changes in shunt direction or systemic References oxygenation [7] and improve myocardial contractility [8]. [1]D.VanderLinde,E.E.M.Konings,M.A.Slageretal.,“Birth Etomidate and ketamine are safe to use in obstetric anesthesia prevalence of congenital heart disease worldwide: a systematic with minimal changes to uterine blood fow and no adverse review and meta-analysis,” Journal of the American College of neonatal outcomes [5]. Cardiology,vol.58,no.21,pp.2241–2247,2011. Afer delivery of the baby, autotransfusion of blood from [2] MBRRACE-UK, Saving Lives, Improving Mothers’ Care - Lessons the uteroplacental bed back into the maternal circulation LearnedtoInformFutureMaternityCarefromtheUKandIre- together with blood loss can increase or decrease preload land Confdential Enquiries into Maternal Deaths and Morbidity causing unpredictable efects on right heart function and 2009–1, National Perinatal Epidemiology Unit, University of degree of right to lef shunt. For this reason, glyceryl Oxford, Oxford, 2014. trinitrate, vasoconstrictors, and inotropes were made avail- [3] V. Asfour, M. O. Murphy, and R. Attia, “Is vaginal delivery or able intraoperatively to counteract these efects during sur- caesarean section the safer mode of delivery in patients with gery. adult congenital heart disease?” Interactive CardioVascular and Tis complicated patient was under the care of the mul- Toracic Surgery,vol.17,no.1,pp.144–150,2013. tidisciplinary team during her stay in the hospital. Without [4] C. A. Davie and P. O’Brien, “Stroke and pregnancy,” Journal of such conjoint efort, a desirable outcome would not have been Neurology, Neurosurgery & Psychiatry,vol.79,no.3,pp.240– possible in this complicated cardiac patient. 245, 2008. In conclusion, a multidisciplinary team involving obste- [5] D. H. Chestnut, “Principles and practice of obstetric,” in Anes- tricians, adult congenital cardiologists, anesthesiologists, car- thesia, pp. 703–733, Elsevier Mosby, Philadelphia, 3rd edition, diothoracic surgeons, and neonatologists should be initiated 2004. at the earliest opportunity during pregnancy. Patients with [6]B.-S.Kodali,S.Chandrasekhar,L.N.Bulich,G.P.Topulos, partially or fully corrected congenital heart disease should and S. Datta, “Airway changes during labor and delivery,” Anes- also be managed in centres experienced in the care of these thesiology,vol.108,no.3,pp.357–362,2008. conditions. In the multidisciplinary team meetings, patient- [7] N. Dhawan, S. Chauhan, S. S. Kothari, U. Kiran, S. Das, and N. specifc concerns should be discussed. Detailed plans for Makhija, “Hemodynamic responses to etomidate in pediatric delivery should be mapped out, and a contingency plan patients with congenital cardiac shunt lesions,” Journal of Case Reports in Anesthesiology 5

Cardiothoracic and Vascular Anesthesia,vol.24,no.5,pp.802– 807, 2010. [8] J.HanouzandE.Persehaye,“Teinotropicandlusitropicefects of ketamine in isolated human atrial myocardium: the efect of adrenoceptor blockade,” Analgesia,pp.1689–1695,2004. M EDIATORSof INFLAMMATION

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