Version May 2024
INTRODUCTION — Approximately 3 to 10 percent of patients with congenital heart disease (CHD) develop pulmonary hypertension (termed pulmonary hypertension-congenital heart disease [PH-CHD]) [1,2]. (See "Pulmonary hypertension with congenital heart disease: Clinical manifestations and diagnosis", section on 'Epidemiology'.)
For patients with PH-CHD, the maternal and fetal risks of pregnancy, contraception methods, and management of pregnancy are discussed here. The clinical manifestations and diagnosis, general management, and disease-specific management of PH-CHD are discussed separately. (See "Pulmonary hypertension with congenital heart disease: Clinical manifestations and diagnosis" and "Pulmonary hypertension in adults with congenital heart disease: General management and prognosis" and "Pulmonary hypertension in adults with congenital heart disease: Disease-specific management".)
DEFINITIONS
●Pulmonary hypertension (PH) – PH is defined as a mean pulmonary artery pressure (PAP) >20 mmHg at rest [3]. This threshold for diagnosis is lower than the previously used threshold of ≥25 mmHg [4].
●Pulmonary hypertension-congenital heart disease (PH-CHD) – Patients with PH-CHD have a variety of types of PH (table 1). In patients with PH-CHD, PH is commonly, but not always, caused by CHD. The most common type of PH-CHD is congenital shunt-related pulmonary arterial hypertension (PAH). (See "Pulmonary hypertension with congenital heart disease: Clinical manifestations and diagnosis", section on 'Classification' and "Pulmonary hypertension with congenital heart disease: Clinical manifestations and diagnosis", section on 'Pathogenesis'.)
●Pulmonary arterial hypertension – PAH is a type of PH diagnosed by demonstration of a mean PAP ≥20 mmHg and a pulmonary vascular resistance (PVR) ≥2 Wood units (WU), along with exclusion of other types of PH (table 1). In the 2022 European Society of Cardiology/European Respiratory Society guidelines, the definition of PAH was revised to >2 WU (from ≥3 WU) to reflect data regarding the prognostic significance of PVR >2 WU [5,6]. However, for treatment purposes, PVR >3 WU is utilized since data regarding utility of treatment for PVR 2 to 3 WU are not available. Severe PAH is identified by a PVR ≥5 WU [7]. PAH associated with CHD (PAH-CHD) is one of many types of PAH. Eisenmenger syndrome is the most severe form of congenital shunt-related PAH. (See "Clinical features and diagnosis of pulmonary hypertension of unclear etiology in adults", section on 'Group 1: Pulmonary arterial hypertension'.)
●Eisenmenger syndrome – This disorder is the most severe form of congenital shunt-related PAH and is characterized by the triad of large intra- or extracardiac defect with an initial systemic-to-pulmonary shunt (ventricular, atrial, or great artery (table 2)), PAH with shunt reversal (right-to-left) or bidirectional shunting, and resulting hypoxemia with cyanosis (figure 1A-B) [8,9]. The pulmonary arterial disease in Eisenmenger syndrome is caused by increased pulmonary blood flow and/or elevated PAP.
RISK OF PREGNANCY — Pregnancy in a patient with PH-CHD is associated with high maternal and fetal risks.
Maternal issues — The risk of maternal mortality and morbidity with PH-CHD is highest for patients with Eisenmenger syndrome [10]. Risk factors for maternal morbidity and mortality include severity of PH, lower arterial oxygen saturation, higher B-type natriuretic peptide level, pericardial effusion on echocardiography, need for hospitalization, and general anesthesia [10,11].
Reported maternal mortality with Eisenmenger syndrome appears to be declining but remains high. The rate of maternal mortality in Eisenmenger syndrome ranged from 38 to 50 percent in reports prior to 1996 [11-16], the mean mortality rate was 25 percent for reports during 1997 to 2007 [11], and mortality rates of 23.3 [17], 16.7 [10], and 10.3 [18] percent were reported by studies performed during various time intervals between 2007 and 2019. The mortality risk cannot be reliably predicted in an individual patient with Eisenmenger syndrome, and death can occur even in patients with few symptoms prior to pregnancy [19].
Even moderate pulmonary vascular disease carries a substantial pregnancy risk since the disease can worsen during pregnancy; thus, no safe cut-off value for pulmonary arterial pressure is known, though risk is likely lower in those with only mildly increased pressure [19].
Lower risk in the setting of PAH without Eisenmenger was suggested by a report from Beijing, in which there were no maternal deaths among 51 patients with PAH-CHD with left-to-right shunt, compared with maternal mortality of 7.7 percent for those with persistent PAH-CHD after previous correction of the defect and 16.7 percent for those with Eisenmenger syndrome [10]. The rates of peripartum heart failure and pulmonary hypertensive crisis were 13.7 and 2.0 percent of those with PAH-CHD with left-to-right shunt, 46.2 and 23.1 percent among those with PAH-CHD with a corrected defect, and 66.7 and 20.0 percent among those with Eisenmenger syndrome.
The majority of maternal deaths occur after delivery during the first week postpartum, but deaths can occur during gestation (particularly the last trimester), labor, or more than one week after delivery [10-13,15,17]. The fixed pulmonary arterial resistance cannot accommodate the hemodynamic fluctuations of labor, delivery, and the puerperium. Most deaths are due to refractory right heart failure, pulmonary hypertensive crisis, thromboembolism, volume depletion (which can augment the right-to-left shunt and precipitate intense cyanosis), and preeclampsia [12-14,19]. When pulmonary hypertensive crisis occurs with sudden increase in pulmonary vascular resistance, right heart failure and systemic hypotension may fatally reduce cerebral blood flow [20].
Fetal issues — In the setting of maternal Eisenmenger syndrome, there is substantial fetal risk because of maternal cyanosis that increases during the course of pregnancy as the fall in systemic vascular resistance enhances the right-to-left shunt. Only 15 to 25 percent of pregnancies progress to term, and at least 50 percent of infants are born prematurely [11-14,16,21]. Spontaneous abortion occurs in 20 to 40 percent of pregnancies, at least 20 to 30 percent of infants born have intrauterine growth retardation, and perinatal mortality has ranged from 7 to 28 percent [11-16].
In the setting of PH-CHD without Eisenmenger syndrome and cyanosis, limited data suggest that the fetal risk may be lower. In a study from Beijing, there were no fetal deaths among 51 pregnancies with maternal PAH with a left-to-right shunt and 13 pregnancies with maternal PAH after defect correction, compared with fetal mortality of 3.3 percent with maternal Eisenmenger syndrome [10]. Numbers were small, but rates of fetal distress were 2.0 percent among those with PAH and left-to-right shunt, 7.7 percent in those with PAH after defect correction, and 10 percent among those with Eisenmenger syndrome.
An additional concern in the setting of maternal PH-CHD is the increased risk of CHD in the fetus. In a literature review of studies published between 1985 and 2007, recurrence was noted in 1 of 20 completed (>20 weeks gestation) pregnancies in women with Eisenmenger syndrome [16]. Prenatal screening and diagnosis of CHD is discussed separately. (See "Pregnancy in women with congenital heart disease: General principles", section on 'Inheritance' and "Congenital heart disease: Prenatal screening, diagnosis, and management".)
CONTRACEPTION METHODS — Safe and effective contraception options are crucial for PH-CHD patients given the very high risks of pregnancy to both the mother and fetus. (See "Contraception: Counseling and selection" and "Pulmonary hypertension in adults with congenital heart disease: General management and prognosis", section on 'Pregnancy'.)
Women with severe PH-CHD, especially those with Eisenmenger syndrome, and their partners should be counseled about the importance of avoidance of pregnancy in view of the high risk of maternal death and should be educated regarding safe and appropriate methods of contraception. Our approach to counseling is informed by the Centers for Disease Control and Prevention's Medical Eligibility Criteria for Contraceptive Use, the European Society of Cardiology (ESC) guidelines for the management of cardiovascular disease during pregnancy, and established contraceptive failure rates (figure 2) [19,22]. (See "Contraception: Counseling and selection".)
Options for contraception include male vasectomy, long-acting reversible contraception (LARC), and female sterilization. Vasectomy has a first-year typical-use failure rate of 0.15 percent and is the safest sterilization option as it does not require intraabdominal surgery or general anesthesia [23] When considering vasectomy as a permanent contraception method for the male partner of a female with severe PH-CHD, one consideration is the limited life-expectancy for individuals with severe PH-CHD.
If vasectomy is declined, then we discuss LARC, including intrauterine devices and the etonogestrel implant, and female sterilization. For women who desire female sterilization, the risks of abdominal surgery, general anesthesia, and pneumoperitoneum need to be weighed against the minimal risks of nonsurgical LARC methods, which have similar efficacy (first-year typical-use failure rates of 0.5 percent for female tubal surgery and 0.1 to 0.8 percent for LARC) [24]. Of note, tubal hysteroscopic sterilization is no longer available. (See "Vasectomy" and "Overview of female permanent contraception".)
For women who decline permanent sterilization, available LARC methods include levonorgestrel-releasing intrauterine devices and the etonogestrel implant. Of the intrauterine devices, we prefer the 52 mg levonorgestrel-releasing intrauterine devices (IUDs; commercial names Mirena, Liletta) because they reduce menstrual blood loss and result in an approximately 20 percent amenorrhea rate after one year of use. As the copper IUD (commercial name Paragard) is associated with heavier and longer menses, we prefer to avoid this device, particularly in cyanotic women with hematocrit levels above 55 percent, because intrinsic hemostatic defects further increase the risk of excessive menstrual bleeding. An equally effective LARC is the etonogestrel implant. While the etonogestrel implant can cause longer or shorter duration of menstrual bleeding, users generally experience decreased overall menstrual bleeding compared with nonusers, but the pattern is less predictable [25,26]. Some women prefer the upper arm insertion of the etonogestrel implant rather than intrauterine insertion of IUDs. While uncommon, syncope can occur during placement of any of these devices. For this reason, the ESC guidelines advise insertion in a monitored setting, such as an operating room, although supporting data are limited and are based in part on the Lippes Loop IUD, which is no longer in use [19,27].
Given the high risk of maternal mortality should pregnancy occur, we do not advise use of other less-effective contraceptive methods, including hormonal pills, patches, and rings; injectable contraception; pericoital contraceptives (diaphragm, cervical cap, spermicidal sponge); and barrier contraceptives when used alone (male and female condoms) (figure 2). However, some women may elect to combine less-effective contraceptive methods to increase contraceptive efficacy (eg, oral progesterone pills plus a barrier method such as male or female condom).
Estrogen-containing contraceptives are contraindicated because of the increased risks of hypertension and thromboembolism and resultant risk of paradoxical embolism [19].
MANAGEMENT OF PREGNANCY — As noted above, pregnancy in a patient with PH-CHD is associated with high maternal and fetal risks and should be discouraged [5,19]. Careful preconception risk assessment and counseling at a specialist center are recommended.
Counseling if pregnancy occurs — Women with PH-CHD who become pregnant should receive individualized counseling regarding maternal and fetal risks from cardiovascular and obstetric caregivers with expertise in the management of PH-CHD. The earliest possible pregnancy termination should be discussed, as termination may be life-saving for the mother. However, termination carries a risk and should only be performed at a care facility that cares for patients with complex PH-CHD.
Termination in the first trimester appears to be safer than allowing pregnancy continuation. For termination of pregnancy, uterine aspiration (also known as dilation and curettage) in the first trimester is the choice of most experts. Prostaglandin F2 alpha should be avoided due to reports of arterial oxygen desaturation in pregnant patients with PH [28]. Second- or third-trimester termination may carry a risk equal to or greater than continuation of pregnancy, but may be reasonable after the risks of termination are balanced against the risks of continuation of pregnancy [29]. (See "Overview of pregnancy termination".)
Management during pregnancy — When a patient elects to continue pregnancy, comprehensive multidisciplinary care, including (at a minimum) maternal-fetal medicine, PH-CHD, and cardiac/obstetric anesthesia experts, is recommended. A multidisciplinary approach and targeted PAH therapy may result in improved pregnancy outcomes [19,30]; in one series, pregnancy outcome was most favorable in long-term responders to calcium channel blockers [31].
If the patient desires to continue the pregnancy, the following steps should be followed to try to ensure the best possible outcome for the mother and child [32,33]:
●Referral – Patients are referred to a center with PH-CHD and maternal-fetal medicine expertise, as these centers provide the optimal combination of clinical expertise, facilities for advanced diagnosis and therapy, structured care, and access to new and evolving therapies. Patients with PH-CHD are the highest-risk patients with preexisting cardiovascular disease during pregnancy. Care of patients with PH-CHD includes management of issues related to the intracardiac shunt, cyanotic heart disease, PH, and Eisenmenger syndrome. (See "Medical management of cyanotic congenital heart disease in adults" and "Pulmonary hypertension in adults with congenital heart disease: General management and prognosis" and "Pulmonary hypertension in adults with congenital heart disease: Disease-specific management".)
●Evaluation for VTE risk – Evaluation is performed during early pregnancy to classify the risk of venous thromboembolism (VTE) and determine whether prophylaxis against VTE is indicated [19]. A careful assessment of maternal and fetal risks and benefits is required since the risk of exacerbating intrinsic hemostatic defects with prophylactic heparin therapy may outweigh any theoretical benefits, and the care of each patient requires careful review and discussion [34,35]. (See "Medical management of cyanotic congenital heart disease in adults", section on 'Thromboembolism' and "Use of anticoagulants during pregnancy and postpartum".)
All patients require vigilant clinical surveillance for symptoms and signs of VTE.
●Medication adjustment
•Discontinue any endothelin receptor antagonists (eg, bosentan) and guanylate cyclase stimulants (riociguat). These medications are contraindicated during pregnancy and in women who may become pregnant.
•If the patient is on a long-acting calcium channel blocker, this therapy is continued. As discussed separately, most patients with PH-CHD are not candidates for calcium channel blocker therapy. (See "Pulmonary hypertension in adults with congenital heart disease: Disease-specific management", section on 'Calcium channel blocker not used'.)
•For patients with World Health Organization (WHO) functional class II or III symptoms (table 3), continue or start a phosphodiesterase-5 inhibitor (eg, sildenafil).
•For patients judged to be at risk of clinical deterioration (ie, WHO functional class III with progressive disease or right heart dysfunction or WHO class IV symptoms (table 3)), epoprostenol is an option. For patients already treated with prostacyclin pathway agonist, such therapy is generally continued.
●Oxygen – Continue oxygen therapy, if indicated. (See "Pulmonary hypertension in adults with congenital heart disease: General management and prognosis", section on 'General approach'.)
●Follow-up – This should include at least monthly visits with maternal-fetal medicine and cardiology caregivers with echocardiograms and B-type natriuretic peptide levels to screen for right ventricular decompensation and right heart failure. Weekly or biweekly visits may be required during the third trimester [19].
●Role of inpatient care – Early hospitalization (eg, at 20 weeks) for close clinical monitoring may be required for some patients [12,36].
Delivery — The optimum timing and mode of delivery in this setting is controversial. The multidisciplinary team should prepare a detailed delivery plan including preparation for postpartum intensive care and circulatory support, if needed.
●Timing – Many experts favor an individualized approach with delivery performed at 34 to 37 weeks of gestation, with later delivery favored when the woman and fetus are stable.
●Mode – The mode of delivery is individualized, with obstetric principles applied to select the least hemodynamically stressful mode of delivery unless there is a specific overriding obstetric indication for cesarean delivery. Options include controlled vaginal delivery with early epidural analgesia and vacuum extraction or low forceps delivery to minimize expulsive efforts. Some experts favor scheduled cesarean delivery with epidural anesthesia at 34 weeks of gestation. Regional anesthesia is generally favored over general anesthesia in this setting [19].
●Monitoring – Pulmonary artery catheter monitoring is avoided because of general lack of benefit in this setting and high risk for hemorrhagic and other complications [37].
●Medical support – Supplemental oxygen should be given during delivery because of its beneficial effects on pulmonary vascular resistance and shunt flow [12,38]. Peripartum hypotension should be treated promptly with fluids and pressors to prevent an increase in the right-to-left shunt.
Circulatory support options such as extracorporeal membrane oxygenation should be available in the event of peripartum hemodynamic collapse. (See "Extracorporeal life support in adults in the intensive care unit: Overview" and "Sudden cardiac arrest and death in pregnancy".)
SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Pulmonary hypertension in adults" and "Society guideline links: Congenital heart disease in adults" and "Society guideline links: Management of cardiovascular diseases during pregnancy".)
SUMMARY AND RECOMMENDATIONS
●Risk of pregnancy – Pregnancy in patients with pulmonary hypertension-congenital heart disease (PH-CHD) is associated with high maternal and fetal risks, with the highest risks seen in patients with Eisenmenger syndrome. (See 'Risk of pregnancy' above.)
•Maternal risk – The reported maternal mortality with Eisenmenger syndrome appears to be declining but remains above 10 percent. The mortality risk cannot be reliably predicted and death can occur even in patients with few symptoms prior to pregnancy. Causes of death include refractory right heart failure, pulmonary hypertensive crisis, thromboembolism, volume depletion, and preeclampsia. (See 'Maternal issues' above.)
•Fetal risk – In the setting of Eisenmenger syndrome, spontaneous abortion occurs in 20 to 40 percent of pregnancies, at least 50 percent of infants are born prematurely, at least 20 to 30 percent of infants born have intrauterine growth retardation, and perinatal mortality has ranged from 7 to 28 percent. An additional concern is the elevated risk of recurrent CHD in the fetus. (See 'Fetal issues' above.)
●Contraception – Safe and effective contraception options are crucial for patients with PH-CHD given the very high risks of pregnancy. Options for contraception include male vasectomy, long-acting reversible contraception (LARC), and female sterilization. (See 'Contraception methods' above.)
●Counseling if pregnancy occurs – Women with PH-CHD who become pregnant should receive individualized counseling regarding maternal and fetal risks from cardiovascular and maternal-fetal medicine caregivers with expertise in the management of PH-CHD. The earliest possible pregnancy termination should be discussed, as termination may be life-saving for the mother. (See 'Counseling if pregnancy occurs' above.)
●Management during pregnancy – When a patient elects to continue pregnancy, comprehensive multidisciplinary care, including (at a minimum) maternal-fetal medicine, PH-CHD experts, and cardiac/obstetric anesthesia experts, is recommended. A multidisciplinary approach and targeted pulmonary arterial hypertension (PAH) therapy may result in improved pregnancy outcomes.
●Delivery – The optimum timing and mode of delivery in this setting is controversial. The multidisciplinary team should prepare a detailed delivery plan including preparation for postpartum intensive care and circulatory support, if needed.
Many experts favor an individualized approach with delivery performed at 34 to 37 weeks of gestation, with later delivery favored when the woman and fetus are stable. Options include controlled vaginal delivery with early epidural anesthesia. (See 'Delivery' above.)
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