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Obstetric Anaesthesia Annual Scientific Meeting 2025
May 15 - 16, 2025 | ICC, 2 Lanyon Place, Belfast BT1 3WH
6778956
Congenital heart disease, associated pulmonary arterial hypertension and pregnancy: a triple challenge for the obstetric anaesthetist
Poster Presenter
Part of Topic
Case report
Congenital heart disease, associated pulmonary arterial hypertension and pregnancy: a triple challenge for the obstetric anaesthetist
Tamal Ray1, Rachel Davies2, Georgina Margiotta1, Vinnie Sodhi3
1. North West London School of Anaesthesia, 2. Imperial College Healthcare NHS Trust , London, 3. Queen Charlotte’s and Chelsea Hospital, London
Pulmonary hypertension in pregnancy has a mortality of 9-25%1
Atrial isomerism is a rare congenital disorder where a patient has two copies of either the left or right atrium
We present the case of a 33 year old woman with pulmonary arterial hypertension(PH) associated with congenital heart disease who underwent a planned caesarean section(CS) for her third delivery
The pregnancy was complicated by a persistent junctional bradycardia which precipitated a significant clinical deterioration
Case
•
•PMHx: established PH at presentation aged 19 due to congenital abnormalities: atrial septal defect(ASD), partial anomalous pulmonary venous drainage (PAPVD), left atrial isomerism
•Corrective surgery: baffling of PAPVD, fenestrated patch repair of ASD. Low risk PH at follow-up.
• Presented with a 3rd, unplanned pregnancy 3 years after her previous delivery.
•PH stable at booking. PASP 38mmHg with good biventricular function. Noted to have junctional bradycardia 40-45bpm.
•Clinical deterioration from 18 weeks with increasing breathlessness on exertion. Echocardiogram and BNP remained stable.
•Deterioration was attributed to the junctional bradycardia preventing augmentation of cardiac output in response to pregnancy and periods of increased activity.
•24 hour tape showed some augmentation of heart rate on exertion. Electrophysiology(EP) team advised not for invasive pacing unless syncopal episodes occurring.
•Caesarean section(CS) planned for 34/40 gestation due to continuing deterioration.
Management
Pre-op
•Admitted two days prior to CS to administer steroids for fetal lung maturation, insertion of arterial and central lines and to commence isoprenaline infusion in intensive care.
•
Intra-op
•Anaesthesia established with incremental CSE: 2.5mg hyperbaric bupivacaine + 350mcg diamorphine. Subsequent epidural top-ups with 0.5% Levopbupivacaine.
•Heart rate maintained at 60-70bpm with isoprenaline infusion
•Fetus received CPAP on delivery
•Uterotonic = oxytocin 5u given as slow infusion.
•500ml blood loss. 300ml crystalloid given in 50ml aliquots, titrated to CVP in addition to 1 unit of blood.
•
Post-op
•Transferred to ICU for weaning of isoprenaline. HR returned to 40-45bpm.
•Baby received further CPAP in NICU.
•Both discharged home after 7 days.
Discussion
•Pulmonary hypertension is defined as a mean pulmonary artery pressure greater than 20mmHg. 2
•Risk in pregnancy correlated to the degree of right heart failure.
•The normal physiological increase in blood volume (40%) and cardiac output (50%)3 is poorly tolerated by the right ventricle and may lead to catastrophic cardiovascular collapse.
•Atrial isomerism has an incidence of 1 in 10,000.4
•Conduction defects are common; absence of the sino-atrial node and/or atrioventricular node is typical in left atrial isomerism, resulting in brady-arrythmias.
•
•Achieving a safe CS delivery required a multi-disciplinary approach.
•EP team advised on the risk/benefit of invasive pacing and were on standby in case of need for emergency invasive pacing.
•PH team present in theatre to administer Epoprostenol or nitric oxide if acute decompensation occurred eg. when placental auto-transfusion and removal of aortocaval compression produce an increase in right ventricular filling.
•
•Isoprenaline enabled the augmentation of cardiac output.
•The incremental CSE technique was crucial to providing cardio-stable anaesthesia.5
•No vasopressor required but first choice would be Vasopressin due to limited effect on pulmonary vasculature.6
•
•The patient has returned to her pre-pregnancy exercise tolerance.
•Despite counselling after all 3 pregnancies, she declined contraception prior to discharge from hospital.
•She will be closely monitored for 6 months post-partum with regular echocardiography and BNP measurement.
•She is likely to need a permanent pacemaker in the future.
References
1.Krishnan, S., Fricke, E.M., Cordoba, M. et al. Pulmonary Hypertension Complicating Pregnancy. Curr Pulmonol Rep 10, 71–83 (2021). https://doi.org/10.1007/s13665-021-00275-1
2.Maron B. Revised definition of pulmonary hypertension and approach to management: a clinical primer. Journal of the American Heart Association, 2023, https://doi.org/10.1161/JAHA.122.029024
3.Mockridge A, Maclennan K. Physiology of pregnancy, Anaesthesia & Intensive Care Medicine, Volume 20, Issue 7, 2019, https://doi.org/10.1016/j.mpaic.2019.05.001.
4.Dickerson H. Hetertoaxy: The Surprise Curveball, American College of Cardiology, https://www.acc.org/Latest-in-Cardiology/Articles/2021/11/17/14/27/Heterotaxy-The-Surprise-Curveball
5.Hamlyn, EL, Douglass, CA, Plaat, F, Crowhurst, JA, Stocks, GM. Low-dose sequential combined spinal-epidural: an anaesthetic technique for caesarean section in patients with significant cardiac disease. Int J Obstet Anesth. 2005;14:355–361. doi: 10.1016/j.ijoa.2005.01.00
6.Tayama E, Ueda T, Shojima T, Akasu K, Oda T, Fukunaga S, Akashi H, Aoyagi S. Arginine vasopressin is an ideal drug after cardiac surgery for the management of low systemic vascular resistant hypotension concomitant with pulmonary hypertension. Interact Cardiovasc Thorac Surg. 2007 Dec;6(6):715-9. doi: 10.1510/icvts.2007.159624. Epub 2007 Aug 17. PMID: 17704123.10.1510/icvts.2007.159624. Epub 2007 Aug 17.
7.Image – Pre-natal ultrasound scans showing isomerism. Great Ormond Street Hospital, https://www.gosh.nhs.uk/conditions-and-treatments/conditions-we-treat/prenatal-ultrasound-scans-showing-isomerism/