|Year : 2016 | Volume
| Issue : 1 | Page : 16-18
Anesthetic experience of two consecutive caesarean sections in a parturient with congenitally corrected transposition of great arteries and complete heart block: A case report
Venkatesh Selvaraj, Revathy Raja, Sreekumar Eranoormana Jayanandan, Shakthy Malathy Venkatesh
Department of Anaesthesiology, Critical Care and Pain Medicine, Sri Ramachandra Medical College and Research Centre, Chennai, Tamil Nadu, India
|Date of Web Publication||22-Apr-2016|
Dr. Venkatesh Selvaraj
Department of Anaesthesiology, Critical Care and Pain Medicine, Sri Ramachandra Medical College and Research Centre, A6 OR Complex, Porur, Chennai - 600 116, Tamil Nadu
Source of Support: None, Conflict of Interest: None
We describe the clinical course of two subsequent caesarean sections in the same parturient with congenitally corrected transposition of great arteries (CCTGA) complicated with complete heart block. CCTGA represents <1% of all forms of congenital heart diseases. In such patients, the hemodynamic effects of pregnancy and surgery may trigger systemic ventricular failure. In these parturients, ventricular dysfunction will also progress rapidly with subsequent pregnancies. The literature review showed no evidence describing the anesthetic management of these high-risk parturients. We used combined spinal epidural anesthesia uneventfully for both the caesarean sections in this patient. This case report will discuss the details of this rare congenital heart disease and the anesthetic management.
Keywords: Anesthetic, heart failure, pregnancy
|How to cite this article:|
Selvaraj V, Raja R, Jayanandan SE, Venkatesh SM. Anesthetic experience of two consecutive caesarean sections in a parturient with congenitally corrected transposition of great arteries and complete heart block: A case report. J Obstet Anaesth Crit Care 2016;6:16-8
|How to cite this URL:|
Selvaraj V, Raja R, Jayanandan SE, Venkatesh SM. Anesthetic experience of two consecutive caesarean sections in a parturient with congenitally corrected transposition of great arteries and complete heart block: A case report. J Obstet Anaesth Crit Care [serial online] 2016 [cited 2022 Jan 24];6:16-8. Available from: https://www.joacc.com/text.asp?2016/6/1/16/181064
| Introduction|| |
Congenitally corrected transposition of great arteries (CCTGA) is a complex congenital heart disease where there is both atrioventricular and ventriculoarterial discordances. The morphological right ventricle (RV) struggles to function as the systemic ventricle pumping against the heavy systemic vascular resistance. With increasing age, these patients tend to develop early systemic ventricular dysfunction.  In such patients, the hemodynamic changes of pregnancy and surgery may accelerate the rate of systemic ventricular failure. We describe the anesthetic management of such a parturient with CCTGA complicated with complete heart block for two consecutive caesarean deliveries. No evidence describing the anesthetic management of the high-risk parturient exists.
| Case Report|| |
A 21-year-old primigravida at 36 weeks of gestational age was admitted to our hospital with labor pain. The obstetrician called for emergency lower segment caesarean section (LSCS) in view of an unfavorable cervix. Earlier at 28 weeks of gestation, she was admitted with shortness of breath. She was diagnosed with CCTGA with complete heart block in systemic ventricle dysfunction. She was treated with diuretics and bronchodilators and then discharged.
Preoperatively screening echocardiogram (echo) [Figure 1]: Two-dimensional (2D) echocardiographic picture] showed dextrocardia, atrioventricular and ventriculoarterial discordances, normal pulmonary artery pressure, and no systemic valvular regurgitation with ejection fraction (EF) of 50%.
The patient's laboratory investigations showed hemoglobin (Hb) of 10.3 g/dL. Her heart rate at rest was 56 beats/min irregularly, blood pressure was 100/70 mmHg, oxygen saturation was 98% in room air, and respiratory rate of 24 breaths/min. She also had basal lung crepitation. Preoperative insertion of a temporary pacemaker was planned. But the cardiologist opined that pacing be done in case of hemodynamic instability.
She received injection furosemide 20 mg intravenous (IV), antibiotic prophylaxis of injection ampicillin 2 g, and injection gentamycin 80 mg IV 1 h prior to surgery. Preinduction monitors included five lead electrocardiograms (ECGs), invasive arterial blood pressure, and pulse oximetry. Central venous line was inserted for emergency pacing. Our plan combined spinal and epidural anesthesia with measures for transvenous pacing kept ready inside the operating room. Epidural catheter was placed in L2-3 space. Subarachnoid block was performed at L3-4 space with 1.5 mL of 0.5% hyperbaric bupivacaine with 25 mcg fentanyl. Intraoperatively, two episodes of hypotension were recorded, which were treated with injection phenylephrine 20 mcg IV boluses. Oxytocin of 20 units was started as a slow IV infusion. Postoperative analgesia was given with epidural infusion of 0.125% bupivacaine with fentanyl. The patient had an uneventful recovery period and was discharged on the 7 th postoperative day.
After 3 years, the same patient was admitted with 36 weeks of gestation G2P1L1 for elective caesarean section in view of the previous caesarean section. She developed gestational diabetes during this pregnancy and was on Tablet. Metformin hydrochloride 500 mg bis in die or twice daily (BD). Her 2D echo showed systemic ventricular dilatation with EF of 45% with mild systemic valvular regurgitation and mild pulmonary artery hypertension. The patient was anesthetized uneventfully with combined spinal epidural anesthesia.
| Discussion|| |
The uniqueness of this article is that it explains the anesthetic course of a parturient with CCTGA during two consecutive caesarean sections over a period of 3 years. Both caesarean sections were managed effectively with combined spinal and epidural anesthesia. The major anesthetic concerns in this patient were risk of systemic ventricular failure, propensity for arrhythmias, and conduction abnormalities and issues with pacemaker insertion.
The systemic ventricular failure can be attributed to the inherent vulnerability of RV to failure, worsening atrioventricular valve (AV) regurgitation with age, ischemia prone systemic ventricle due to a single main artery supply unlike the morphological left ventricle, or due to the associated anomalies.  The commonly associated anomalies are ventricular septal defect, tricuspid regurgitation, pulmonary valve stenosis, and conduction abnormalities. Two-thirds of the patients with significant associated defects had congestive heart failure by the age of 45 years. 
The systemic ventricle often dilates with progressive tricuspid regurgitation during subsequent pregnancies in patients with transposition of the great arteries in whom the mustard procedure has been performed.  In our patient, echo showed a progressive systemic ventricular dysfunction and increasing systemic valvular regurgitation over a period of 3 years. In fact, CCTGA patients with an EF of 40% and less are often counseled to avoid pregnancy.  Monthly echo surveillance of systemic ventricular function by the cardiologist is recommended.  If ventricular function deteriorates, caesarean delivery should be done early to avoid worsening of heart failure. 
The conduction abnormalities can be due to the unusual position and number of AV node  or the longer course of the bundle of His, making it prone to fibrosis and increased incidence of complete AV block at about 2% per year  increasing with age. Atrial dilatation due to systemic AV insufficiency can cause dysrhythmias. Spontaneous ventricular tachycardia has also been reported. 
Supportive pacing is usually not necessary for isolated congenital complete heart block with good ventricular function, especially when the escape rhythm has a narrow QRS complex as in our patient.  Achieving pacemaker insertion may be technically difficult due to the absence of trabeculation in the morphological RV. Insertion of a pacemaker may also precipitate systemic ventricular dysfunction by inducing "septal shift"  and worsening the AV valve regurgitation. But we had expertise and equipment for transvenous pacing in case of hemodynamic instability. The preexisting conduction abnormalities, pulmonary stenosis, and abnormal position of atrioventricular node  may complicate pulmonary artery catheterization and monitoring. This monitoring may be valuable in a patient with severe ventricular dysfunction.
Literature evidence for the choice of anesthetic technique in these parturients is almost nonexistent. The afterload reduction in neuraxial anesthesia reduces stress on the systemic ventricle and also decreases the degree of systemic AV regurgitation. The exceptions are those with significant right-to-left intracardiac shunting and severe systemic ventricular dysfunction, which may worsen with neuraxial anesthesia. Successful management with general anesthesia has been reported in one parturient who presented with severe ventricular dysfunction.  The stress of labor may increase the risk of cardiac dysrhythmias, ischemia, and congestive cardiac failure. Early initiation and safe management of neuraxial labor analgesia has also been reported, which may be prudent in these patients. 
| Conclusion|| |
The presence of coexisting intracardiac defects and shunt, dysrhythmias, and ventricular dysfunction are the main considerations before planning the anesthetic method in these patients. Neuraxial anesthesia may be safe in these patients for caesarean section.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Dimas AP, Moodie DS, Sterba R, Gill CC. Long-term function of the morphologic right ventricle in adult patients with corrected transposition of the great arteries. Am Heart J 1989;118:526-30.
Warnes CA. Transposition of the great arteries. Circulation 2006;114:2699-709.
Graham TP Jr, Bernard YD, Mellen BG, Celermajer D, Baumgartner H, Cetta F, et al
. Long-term outcome in congenitally corrected transposition of the great arteries: A multi-institutional study. J Am Coll Cardiol 2000;36:255-61.
Guédès A, Mercier LA, Leduc L, Bérubé L, Marcotte F, Dore A. Impact of pregnancy on the systemic right ventricle after a Mustard operation for transposition of the great arteries. J Am Coll Cardiol 2004;44:433-7.
Arendt KW, Connolly HM, Warnes CA, Watson WJ, Hebl JR, Craigo PA. Anesthetic management of parturients with congenitally corrected transposition of the great arteries: Three cases and a review of the literature. Anesth Analg 2008;107:1973-7.
Regitz-Zagrosek V, Blomstrom Lundqvist C, Borghi C, Cifkova R, Ferreira R, Foidart JM, et al
.; European Society of Gynecology (ESG); Association for European Paediatric Cardiology (AEPC); German Society for Gender Medicine (DGesGM), 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.
Colman JM, Sermer M, Seaward PG, Siu SC. Congenital heart disease in pregnancy. Cardiol Rev 2000;8:166-73.
Anderson RH, Becker AE, Arnold R, Wilkinson JL. The conducting tissues in congenitally corrected transposition. Circulation 1974;50:911-23.
Fontaine JM, Kamal BM, Sokil AB, Wolf NM. Ventricular tachycardia: A life-threatening arrhythmia in a patient with congenitally corrected transposition of the great arteries. J Cardiovasc Electrophysiol 1998;9:517-22.
Tsuda A, Nagaoka K, Hashiguchi H, Shibata S, Shibata I, Nagatani A. Anesthetic management of a pregnant patient with congenitally corrected transposition of the great arteries for emergency cesarean delivery. Masui 2003;52:187-9.
Schabel JE, Jasiewicz RC. Anesthetic management of a pregnant patient with congenitally corrected transposition of the great arteries for labor and vaginal delivery. J Clin Anesth 2001;13:517-20.