Resuscitation, revival, and birth: a case report of cardiac arrest in a pregnant woman with repaired congenital heart disease

Abstract

Background

Surgical correction of congenital cardiac defects in infancy is being increasingly prevalent there is an increasing population of patients reaching adulthood with corrected congenital heart disease. Pregnancy in these patients can be particularly dangerous and risk stratification is key. Complications during pregnancy can still occur, which in the context of corrected congenital cardiac disease may require unique care.

Case Summary

A 30-year-old patient in her third trimester of pregnancy presented to hospital with acute onset chest pain and shortness of breath. She had a background of congenital corrected pulmonary atresia via a Modified Blalock-Thomas-Taussig shunt which required conduit replacements through her adolescence and a ventricular septal defect (VSD) repaired with a Dacron patch. Routine echocardiograms and electrocardiograms had been stable through her pregnancy. On this occasion, she rapidly deteriorated on presentation to the emergency department and suffered a 15-minute ventricular fibrillation (VF) arrest during which she underwent a peri-mortem C-section. During her cardiac arrest, she developed CPR induced consciousness which led to some distressing recall of her treatment. Overall, this was felt to be scar related ventricular fibrillation (VF) and she left hospital after a 3-week admission with an implantable cardiac defibrillator. She and her newly born baby suffered no ill effects of this cardiac arrest.

Discussion

This case highlights the necessity for early recognition, rapid intervention, and multidisciplinary collaboration in managing life-threatening maternal cardiac emergencies.

Learning points.

• Multidisciplinary planning is vital for women with adult congenital heart disease (ACHD) considering pregnancy.

• Cardiopulmonary resuscitation-induced consciousness (CPRIC) is an under-recognized phenomenon during resuscitation and can lead to distressing recall of events for patients.

• Prompt, coordinated resuscitative intervention can lead to excellent maternal and neonatal outcomes even in complex cardiac cases.

Introduction

Congenital cardiac defects such as pulmonary atresia and ventricular septal defects (VSD) constitute significant proportion of cardiac malformations in infant births.^1,2 With improving intrauterine detection, pre/post pregnancy care and novel surgical techniques, mortality of congenital cardiac defects are improving.³ As more of these patients reach adulthood, the proportion of those considering pregnancy is increasing.⁴

Pregnancy leads to significant cardiovascular stress with increased blood volume, heart rate, and cardiac output.⁵ For women with surgically corrected heart defects, these physiological changes can pose significant mortality risk.⁶ Risk stratification for pregnant women with adult congenital heart disease (ACHD) is essential to guide management and optimize outcomes. Two widely adopted tools are CARPREG II (Cardiac Disease in Pregnancy) score⁷ and ZAHARA (Zwangerschap bij Aangeboren HARtAfwijkingen) score.⁸ While both have their limitations, they can be used to tailor counselling, monitoring, and plan delivery in women with ACHD. Despite adequate risk stratification, complications during pregnancy can still occur, which in the context of ACHD requires unique care.

Here, we present the case of a pregnant lady with ACHD who presented with an unheralded ventricular fibrillation cardiac arrest.

Summary figure

Age 1	Repair of a ventricular septal defect (VSD) and congenital pulmonary atresia with a Dacron patch closure of her VSD and creation of a right ventricle (RV) to pulmonary artery (PA) conduit with a 16mm pulmonary homograft
Age 4	Right modified Blalock-Taussig shunt
Age 5	Replacement of RV to PA conduit with 18mmaortic homograft
Age 16	Repair of RV to PA conduit with 22mm Contegra conduit
Week-36	Start of Pregnancy
Week 20	Seen in Obstetric and joint ACHD Clinic on routine basis
Day 0	Presentation to Emergency Department with shortness of breath and chest pain Ventricular Fibrillation Cardiac Arrest
Day 0 (Baby)	Peri-mortem C-section delivery of baby in ED. Mother admitted to Intensive Care Unit, Baby to Neonatal Intensive Care
Day 1	Developed acutely dusky leg in ICU—diagnosed with deep vein thrombosis and started on subcutaneous Dalteparin. Baby discharged from NICU
Day 3	Stepped down to Cardiology Coronary Care Unit. Baby discharged home
Day 9	Cardiac MRI attempted-unable to tolerate. Showed mild systolic function impairment (EF 53%) with a dilated right ventricle
Day 10	CT coronary angiogram showed degenerative thickening and calcification along RV-PA conduit but no significant stenosis, Moderate right heart dilation, RCA origins from the left coronary sinus and left circumflex from non-coronary cusp, although no malignant inter-arterial course
Day 11	Discussed in MDT—felt to be Scar related VF arrest, advised for insertion of implantable cardioverter defibrillator (ICD) and continuation of amiodarone orally
Day 14	Implantable cardioverter defibrillator (ICD) Inserted
Day 15	Discharged from Hospital
Week 3	Pacing follow up—no ICD therapy delivered to date

Case Study

A 30-year-old female at 36 + 3 weeks gestation presented to the emergency department (ED) with chest pain and shortness of breath. She had a background of congenital pulmonary atresia with a VSD. This was surgically corrected within her first year of life with a Dacron patch closure of the VSD and 16 mm pulmonary homograft right ventricle (RV) to pulmonary artery (PA) conduit formed. At age 4, she underwent a right modified Blalock-Taussig shunt. At age 5, her conduit was replaced with an 18 mm aortic homograph. At age 16, she underwent a repair with a 22 mm Contegra conduit.

This was her second pregnancy. Her first pregnancy was completed in 2023. She was referred to local ACHD/cardiology services prior to her first pregnancy. Her pre-pregnancy transthoracic echocardiogram (TTE) showed a Vmax of 1.5 m/s through her conduit, with a mildly dilated RV, her LV size was normal with an ejection fraction (EF) of 50%. She received monthly follow-up by joint ACHD/obstetric service.

She re-presented to clinic with symptoms of shortness of breath and palpitations at 16 weeks in May 2023. She underwent repeat TTE reporting a mild increase in her Vmax through her RV-PA conduit (2.4 m/s). Her biventricular size and function remained unchanged. She underwent a 48-hour Holter monitor which showed bigeminy but no sustained arrythmia and she was initiated on 2.5 mg bisoprolol. Unfortunately, her breathlessness worsened on next review, and she was admitted for inpatient cardiac magnetic resonance imaging (cMRI) (Kawel Boehm normal reference ranges in brackets used locally at our centre). This revealed a normal size left ventricle (LV) with an LV ejection fraction (LVEF) of 57% (56–76%). Her LV end diastolic function (LVEDV) was 148 mL (90–174 mL) and LV end systolic volume (LVESV) was 64mLs (25–63mLs). Her right ventricle end diastolic volume (RVEDV) was 141 mLs (89–177mLs) and RV end systolic volume (RVESV) was 78 mLs (23–75mLs). Her RV ejection fraction was reduced at 45% (52–76%). Her symptoms improved thereafter. She was reviewed every 2–4 weeks in clinic and remained asymptomatic. She had repeat TTE at 34 weeks revealing stable velocities through her RV-PA conduit. She was routinely admitted to hospital at 36 + 4 weeks and delivered a healthy baby at term via Caesarean-section.

Her pregnancy on this occasion was unplanned and she received no pre-pregnancy guidance from her obstetric/cardiology team. She self-presented at 28 weeks through her pregnancy for routine ACHD pregnancy planning and reported no symptoms. She had a TTE which showed an RV-PA conduit Vmax of 1.6 m/s with stable biventricular size and function.

Upon arrival to ED, she collapsed at 08:04 and was found to be in cardiac arrest. Immediate advanced life support was initiated in accordance with Resuscitation Council guidelines⁹ alongside left lateral displacement of her uterus. In total she received 5 cycles of cardiopulmonary resuscitation (CPR) and was in ventricular fibrillation (VF) throughout. She received 5 external defibrillation shocks during each cycle of resuscitation. She received 300 mg of amiodarone in the first cycle of CPR and a further 150 mg in the second cycle. She received 1 mg IV adrenaline in her third cycle, during which she had a perimortem Caesarean section. This resulted in delivery of a live infant with normal Apgar scores. The baby was admitted to Neonatal Intensive Care Unit for ongoing monitoring/observation. In her fourth CPR cycle her uterus was atonic, she received 5 IU of syntometrine IM. Return of spontaneous circulation was achieved at 0820. Her post cardiac arrest bloods showed: sodium: 140; potassium: 5.2; GFR: >90; ALT: 55; CRP: 8; haemoglobin: 113; white cell count: 32.9; neutrophils: 28.6; arterial blood gas: pH: 7.01; CO₂: 7.54; O₂:11.7; lactate: 7.2; bicarbonate: 11.9. She was stabilized haemodynamically with an adrenaline and then noradrenaline infusion. She received IV Ceftriaxone due to her emergency Caesarean-section. Her ECG is shown in Figure 1 which appeared similar to her previous ECG.

Figure 1.

Post cardiac arrest ECG showing sinus rhythm with right bundle branch block.

Notably, during resuscitation efforts, she exhibited cardiopulmonary resuscitation-induced consciousness (CPRIC), demonstrated by brief episodes of consciousness during chest compressions. She required no pharmacological intervention for this but was briefly manually restrained.

Following resuscitation, she was transferred to operating theatre for post-operative care of her abdominal wound including, abdominal closure and insertion of an intraperitoneal drain. Her post arrest computerized tomography (CT) scan revealed no evidence of pulmonary embolism. She had a post cardiac arrest bedside transthoracic echocardiogram which showed a normal LV cavity size and a visually dilated RV but unable to accurately assess left or right ventricular systolic function due to tachycardia, 120bpm. Her RV-PA conduit had a Vmax of 1.8 m/s alongside a mild regurgitant jet.

She was admitted to intensive care unit (ICU) for standard post-cardiac arrest care. During this admission she developed a cold, dusky leg with absent pulses. A CT angiogram revealed no significant occlusion, but ultrasound Doppler confirmed a deep vein thrombosis, she was initiated on subcutaneous Dalteparin. Once stepped down to the cardiology ward she underwent a CT coronary angiogram. This showed mild degenerative thickening and calcification along RV-PA conduit but no significant stenosis, moderate right heart dilation, right coronary artery origin from the left coronary sinus and left circumflex origin from non-coronary cusp, although there was no malignant inter-arterial course (Figure 2A,2B). She also underwent a cMRI showing mild systolic function impairment (biplane LVEF 53%). Unfortunately, she was unable to tolerate the full cMRI study and parametric mapping alongside gadolinium sequences were not performed. Short axis cines were also not obtained, and thus quantitative assessment of RV volume and function was not possible but visually her right ventricle was mildly dilated with mildly impaired function. (Figure 2C, 2D).

Figure 2.

Radiographic images. (A) Computerised tomography coronary angiogram image revealing an aberrant left circumflex artery from non-coronary cusp (solid arrow). (B) Computerised tomography coronary angiogram image revealing an aberrant right coronary artery from the left coronary cusp (dotted arrow). (C) Still frame of steady-state free precession sine cardiac magnetic resonance apical four chamber image revealing dilated right ventricle (dashed arrows). (D) Still frame of steady-state free precession cardiac magnetic resonance RVOT image revealing the RVOT is of a normal size with no stenosis or aneurysm.

Her care was discussed in an electrophysiology multidisciplinary meeting (MDT). Overall, it was felt that this was likely scar related VF from her multiple previous cardiothoracic procedures. The MDT recommended continuation of oral amiodarone and insertion of an implantable cardioverter defibrillator (ICD) prior to discharge. She had some recall of the events of resuscitative efforts which may be attributed to CPRIC. She found this distressing and received input from psychology services.

Post discharge, this patient was enrolled in cardiac rehabilitation. She has since been reviewed in cardiology outpatient clinic and is doing well post discharge with no functional limitations. She attended pacing follow up routinely and has not received any ICD therapy. She underwent a repeat transthoracic echocardiogram at 6 weeks post discharge. This showed a normal LV cavity size with overall visual LV ejection fraction of 50–55%. The RV appeared dilated with impaired function (TAPSE 14 mm; RV S^’ wave 6 cm/s). Her RV-PA conduit had a Vmax of 2.2 m/s alongside mild regurgitation. The neonate suffered no ill effects of the emergency delivery and was discharged after 3 days. She was advised against breastfeeding due to her medication regimen. She was counselled not to consider further pregnancies and opted for insertion of a contraceptive coil. She continues to see the psychology team as an outpatient.

Discussion

Congenital cardiac defects such as pulmonary atresia and VSDs remain rare; however, advances in surgical management of these conditions in early life, has led to increased number of patients surviving to adulthood. For women reaching adulthood with ACHD, discussions around pregnancy planning are vital and for patients that are considering pregnancy, appropriate risk assessment and planning is essential to predict patients that may need closer follow up during pregnancy.

During pregnancy, a host of physiological changes occur that place significant additional stress on the cardiovascular system. These include increases in blood volume and cardiac output, which increases up to 30–50% above baseline, as well as reductions in systemic vascular resistance and blood pressure.¹⁰ In women with ACHD, these physiological demands may precipitate decompensation or life-threatening events such as arrhythmias, heart failure, or cardiac arrest.¹¹

As surgical and medical management of patients with ACHD continues to improve, the number of women reaching reproductive age will continue to rise. This shift will lead to increasingly complex pregnancies requiring individualized risk assessment and coordinated care plans.¹²

We highlight in this case the increasingly recognized phenomenon of CPRIC. Although CPRIC remains poorly understood, physiologic variables, such as autoregulation, comorbidities, and cerebral ischaemic thresholds are all suggested as mechanisms which predispose its occurrence.¹³ CPRIC presents unique challenges during resuscitation, namely due to confusion within the resuscitative team about whether the conscious represents CPRIC or return in spontaneous circulation, which can lead to inappropriate rhythm checks and interruption of CPR.¹⁴ For patients experiencing CPRIC evidence that total patient recall of resuscitative efforts can occur in up to 40% of cases¹⁵ which can have serious psychological effects for survivors. Witnessing CPRIC as a member of a resuscitative team can also be distressing.¹⁶ Treating CRPIC is difficult and currently poorly evidence based, there are no universally adapted current guidelines, but medical professions need to consider use of sedation or physical restraint to ensure effective CPR while minimizing distress to both patient and team.¹⁷

Conclusion

This case highlights the necessity for early recognition, rapid intervention, and multidisciplinary collaboration in managing life-threatening maternal cardiac emergencies. Early identification, resuscitative treatment and management of subsequent cardiac arrest complications, resulted in an excellent outcome for this patient and her baby.

Lead author biography

Dr Gupta is a Cardiology Consultant with a subspecialty interest in imaging.

Author contributions

Mehak Gupta (Visualisation, Project administration, Writing—original draft), Andrew Cole (Conceptualisation, Visualisation, Writing—original draft), Tamara Hameed (Investigation, Project administration, Writing—review & editing), Marcus Brumpton (Visualisation, Validation, Writing—review & editing), and Diane Barker (Supervision, Validation, Writing—review & editing)

Funding

None declared.

Data availability

No new data were generated or analysed in support of this research.

Patient consent

The authors confirm that written consent for submission and publication of this case report including images and associated text has been obtained from the patient, in line with COPE guidance.

Ethics

The patient involved in this case report has signed a consent form this anonymized case report to be published.