Abstract
Background
While long-term survival for tetralogy of Fallot continues to improve, residual hemodynamic abnormalities are common and increase risk for cardiovascular events during pregnancy.
Case Summary
A young woman with tetralogy of Fallot with pulmonary atresia (TOF/PA) having undergone biventricular repair presented with heart failure symptoms and near-systemic right ventricular (RV) pressure in the setting of an unplanned pregnancy. Given her desire to continue the pregnancy coupled with the significant maternal and neonatal risk, percutaneous intervention was performed, improving RV pressure and facilitating the eventual delivery of a healthy term child.
Discussion
Though high-risk, percutaneous intervention during pregnancy for complex RV outflow tract/pulmonary artery lesions can be performed safely and effectively.
Take-Home Messages
This case highlights successful percutaneous RV outflow tract/pulmonary artery intervention during pregnancy in a patient with TOF/PA. Multidisciplinary care including adult congenital heart disease, maternal fetal medicine, and cardiac anesthesiology for the pregnant patient with complex congenital heart disease is essential.
Key words: adult congenital heart disease, pregnancy, pulmonic valve, tetralogy of Fallot with pulmonary atresia
Visual Summary
History of Presentation
A 28-year-old woman with repaired tetralogy of Fallot with pulmonary atresia (TOF/PA) and major aortopulmonary collateral arteries presented at 12 weeks of gestation with NYHA functional class III symptoms. She reported dyspnea and chest pressure with mild exertion and difficulty climbing a single flight of stairs.
Past Medical History
Prior surgical interventions included transannular patch at age 5 months, left pulmonary artery unifocalization at age 2 years, complete biventricular repair with intraoperative balloon angioplasty of the right pulmonary artery (RPA) at age 4 years, and repeat right ventricular outflow tract (RVOT) reconstruction with placement of 27-mm Mosaic valve (Medtronic) in the pulmonary valve position and insertion of 27 mm × 12 mm Genesis stents (Cordis) in the bilateral branch pulmonary arteries at age 11.5 years. At age 20, she underwent transcatheter balloon angioplasty of the RPA stent with a 12-mm Vida balloon (Bard Peripheral Vascular), repeat RPA stent placement with PG1910 stent on a 12-mm BIB balloon, and postdilation with 14-mm Vida balloon, with improvement in RV systolic pressure from 88% to 65% of systemic pressure.
Differential Diagnosis and Investigations
In the setting of TOF/PA and prior RVOT interventions including bioprosthetic pulmonary valve replacement and branch pulmonary artery stents, the differential diagnosis included prosthetic pulmonary valve stenosis, branch pulmonary artery in-stent stenosis, RV failure, tricuspid valve regurgitation, and arrhythmia. Transthoracic echocardiogram revealed moderate prosthetic pulmonary valve stenosis and regurgitation, with an estimated RV systolic pressure of 89 mm Hg and preserved systolic function as well as moderate central tricuspid regurgitation (Video 1, Video 2, Video 3). Ambulatory electrocardiogram monitor demonstrated rare supraventricular and ventricular ectopy without prolonged arrhythmia.
After a comprehensive and multidisciplinary assessment of the risks of continuing the pregnancy, both with and without transcatheter intervention, she was recommended to undergo intervention in the early second trimester given the degree of symptomatology and RV pressure and volume overload. The factors that contributed to the patient's elevated cardiovascular risk during pregnancy based on the CARPREG (CARdiac disease in PREGnancy) II score included NYHA functional class III and IV status (+3 points) and pulmonary hypertension (pulmonary artery systolic pressure >50 mm Hg) (+2 points), and the factors that contributed to elevated risk based on the ZAHARA model included cardiac medication before pregnancy (+1.50 points), NYHA functional class ≥II prior to pregnancy (+0.75 points), pulmonic atrioventricular valve regurgitation (moderate/severe) (+0.75 points), and history of cyanotic congenital heart disease at birth (+1.00 points). Based on the modified World Health Organization classification of maternal cardiovascular risk, the patient fell into risk category III, which indicates “significantly increased maternal mortality or severe morbidity risk.” Using these 3 scores for cardiovascular risk assessment in pregnancy, her overall risk of significant cardiac morbidity was between 25% and 70%, with a 10% risk of mortality (Table 1).
Table 1.
Predicted Morbidity Risk According to Models Assessing Cardiovascular Risk in Pregnancy Models
| Cardiovascular Pregnancy Risk Score | Category/Point Total | Maternal Risk Factors | Predicted Morbidity Risk |
|---|---|---|---|
| mWHO classification | 3 | Complex congenital heart disease | 24.5% |
| CARPREG II | 5 | NYHA functional class III and IV status (3 points); pulmonary hypertension (pulmonary artery systolic pressure >50 mm Hg) (2 points) | 41% |
| ZAHARA | 4.00 | Cardiac medication before pregnancy (1.50 points); NYHA functional class ≥II before pregnancy (0.75 points); pulmonic atrioventricular valve regurgitation (moderate/severe) (0.75 points); corrected/uncorrected cyanotic congenital heart disease (1.00 points) | 70% |
CARPREG = CARdiac disease in PREGnancy; mWHO = modified World Health Organization; ZAHARA = Zwangerschap bij Aangeboren HARtAfwijking [pregnancy in women with congenital heart disease].
Management
After multidisciplinary discussion, the decision was made to proceed with percutaneous intervention. Prior to the procedure, fetal heart tones were checked and found appropriate for a gestational age of 17 weeks. The patient was placed under general anesthesia, and radiation exposure was minimized by using only single-plane imaging, decreasing fluoroscopy dosing, and avoiding cineangiography. Hemodynamic assessment revealed baseline RV pressure of 73/11 mm Hg (90% systemic), a peak-to-peak gradient across the bioprosthetic pulmonary valve of 23 mm Hg, and a gradient across the RPA stent of 32 mm Hg. Pulmonary angiography showed a narrowed bioprosthetic pulmonary valve measuring approximately 20 mm in diameter on anteroposterior projection, as well as narrowing in the RPA stent to 11 mm.
Balloon angioplasty of the RPA stent was performed with a 16 mm × 2 cm Vida balloon (Bard Peripheral Vascular) (Figure 1A), followed by placement of a Palmaz 3110 stent (Cordis) mounted on a 14-mm BIB balloon (NuMed) (Figure 1B) that was postdilated using a 14-mm Vida balloon (Figure 1C). There was subsequent angiographic resolution of the stent narrowing. After RPA stent placement, there was modest improvement in RV systolic pressure, decreasing from 88% to 81% of systemic blood pressure, so balloon valvuloplasty of the bioprosthetic pulmonary valve was performed using a 26 mm × 2 cm Vida balloon. Owing to interaction with one of the RPA stent struts, the balloon ruptured and was removed. RV pressure had decreased to 72% systemic pressure at this time. Repeat balloon valvuloplasty was performed with frame fracture at 14 atm, successfully decreasing the RV systolic pressure to 65% systemic. Given the potential for interaction between the RPA stent strut and the deployment balloon of a Sapien pulmonary valve, transcatheter pulmonary valve replacement was not pursued at this time. The patient recovered well after the cardiac catheterization, with ongoing stable fetal heart tones. She ultimately delivered a healthy term child with appropriate birthweight via caesarean section at 37 weeks and 4 days of gestation.
Figure 1.
Intraprocedural Images During Right Pulmonary Artery Balloon Angioplasty and Stent Placement
(A) RPA balloon angioplasty. Cineangiography was avoided, and the RPA stent was ballooned with a 16-mm high-pressure Vida balloon showing distal stent stenosis down to a minimal diameter of 11 mm. (B) Stent positioning. A Palmaz Genesis 1910 stent lined up within the previously placed RPA stent. (C) Stent dilation. Postdilation of the newly placed RPA stent with a 14-mm high-pressure Vida balloon. RPA = right pulmonary artery.
Discussion
This case exemplifies the challenging clinical decision-making and multidisciplinary management of patients with complex congenital heart disease during pregnancy. While pregnancy outcomes among patients without severe RVOT lesions are generally good, those with moderate to severe hemodynamic lesions as well as those with underlying TOF/PA anatomy are at higher risk for cardiovascular events as well as for spontaneous abortion and neonatal complications.1, 2, 3
Our patient demonstrated not only severe residual hemodynamic lesions including pulmonary valve regurgitation, pulmonary stenosis, and branch pulmonary stenosis, but also symptoms of heart failure. Taken together in the context of pregnancy, these factors placed her at high risk for further cardiovascular complications and significantly influenced the decision to intervene during pregnancy.1,4, 5, 6 The consideration of intervention was also weighed against risk to the fetus from ionizing radiation. Given the low overall radiation exposure and the timing of intervention (the procedure was not performed during the most critical period of organogenesis), the risk to the fetus was determined to be very low.
From an intraprocedural standpoint, the decision to forego high-risk transcatheter pulmonary valve replacement was multifaceted and challenging. Alhough the patient's multilevel RVOT obstruction improved after RPA stent placement and balloon pulmonary valvuloplasty, RV pressure remained moderately elevated. We accepted this result rather than attempt placement of a Sapien valve, as rupture of the deployment balloon and inadequate expansion of a prosthetic valve within the main pulmonary artery would have been significantly more dangerous to the patient and fetus, potentially necessitating surgical intervention.
Although it is recommended that all women with congenital heart disease (CHD) receive prepregnancy counseling with input from an adult congenital heart disease (ACHD) cardiologist, and that women with highly complex CHD should be managed collaboratively during pregnancy by ACHD cardiologists, maternal fetal medicine specialists, and anesthesiologists experienced in ACHD, this is often not the case.7 This reality, coupled with the fact that increasing numbers of women with CHD are becoming pregnant, underscores the importance of describing complex cases such as this one. Interestingly, there have been several reported pulmonary valve interventions for CHD during pregnancy,8, 9, 10 however none have been in the setting of such complex RVOT and branch pulmonary artery reconstruction.
Follow-Up
After delivery and before discharge, we had an extensive discussion with the patient regarding need for preconception planning prior to the next pregnancy. Four years later, the patient decided that she would like to pursue another pregnancy and underwent transcatheter pulmonary valve replacement and rehabilitation of the branch pulmonary arteries with angioplasty and stent placement.
Funding Support and Author Disclosures
Dr Aboulhosn has been a consultant for Abbott Medical and Edwards Lifesciences; has been a proctor for Edwards Lifesciences and Medtronic; and has received research funding from Venus Medtech. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
Take-Home Messages
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•
Patients with right ventricular outflow tract obstruction with moderate to severe hemodynamic lesions as well as those with underlying TOF/PA anatomy are at higher risk for cardiovascular events during pregnancy, spontaneous abortion, and neonatal complications.
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•
All women with congenital heart disease should receive prepregnancy counseling with input from an ACHD cardiologist and should be managed collaboratively during pregnancy by ACHD cardiologists, maternal fetal medicine specialists, and anesthesiologists experienced in ACHD.
Footnotes
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the Author Center.
Appendix
Visual Summary.
| Time | Events |
|---|---|
| Day 1 (week 12 gestation) | A 28 year-old woman with TOF/PA at 12 weeks' gestation presented with NHYA functional class III symptoms. The pregnancy was unplanned, but she desired to continue it. Echocardiography showed severe RVOT obstruction, moderate tricuspid regurgitation, and preserved RV systolic function. |
| Day 7 (week 13 gestation) | Underwent consultation with maternal fetal medicine, who recommended deferring any transcatheter intervention, if possible, until second trimester, after organogenesis. |
| Day 18 (week 14 gestation) | Followed up with ACHD cardiologist, who recommended the patient undergo transcatheter pulmonary valve/pulmonary artery intervention and planned the procedure for the second trimester. |
| Day 48 (week 19 gestation) | Presented to emergency room with worsening dyspnea on mild exertion and chest pressure. |
| Day 49 (week 19 gestation) | Underwent cardiac catheterization including percutaneous pulmonary artery angioplasty, RPA stent placement, and balloon pulmonary valvuloplasty while admitted. There was improvement in RV systolic pressure from 88% to 65% systemic. |
| Day 50 (week 19 gestation) | Discharged home on postprocedure day 1. |
| Day 177 (week 37 gestation) | Admitted for induction of labor but developed prolonged labor (failure to progress) and underwent cesarean section, delivering a healthy term child at 37 weeks and 4 days' gestation. Discharged home on postpartum day 3. |
Appendix
Parasternal Long-Axis 2D Interrogation Demonstrating Hyperdynamic Left Ventricular Systolic Function, Moderate Right Ventricular Hypertrophy, and Bowing of the Interventricular Septum in Systole, Consistent With Elevated Right Ventricular Systolic Pressure
Parasternal Short-Axis 2D Interrogation Demonstrating Hyperdynamic Left Ventricular Systolic Function, Moderate Right Ventricular Hypertrophy, and Bowing of the Interventricular Septum in Systole, Consistent With Elevated Right Ventricular Systolic Pressure
Parasternal Long-Axis Color Flow Doppler Interrogation of the Right Ventricular Outflow Tract Demonstrating Flow Acceleration, Moderate Stenosis, and Moderate Regurgitation
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Associated Data
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Supplementary Materials
Parasternal Long-Axis 2D Interrogation Demonstrating Hyperdynamic Left Ventricular Systolic Function, Moderate Right Ventricular Hypertrophy, and Bowing of the Interventricular Septum in Systole, Consistent With Elevated Right Ventricular Systolic Pressure
Parasternal Short-Axis 2D Interrogation Demonstrating Hyperdynamic Left Ventricular Systolic Function, Moderate Right Ventricular Hypertrophy, and Bowing of the Interventricular Septum in Systole, Consistent With Elevated Right Ventricular Systolic Pressure
Parasternal Long-Axis Color Flow Doppler Interrogation of the Right Ventricular Outflow Tract Demonstrating Flow Acceleration, Moderate Stenosis, and Moderate Regurgitation