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............................................................................................................................................................................. defect as well as on additional factors such as ventricular function, functional class, and cyanosis. Maternal cardiac complications are present in 10% of completed pregnancies and are more frequent in mothers with complex disease. Patients who experience complications during pregnancy may also be at higher risk of late cardiac events after pregnancy.139 Obstetric complications such as (pre-) eclampsia are more often encountered. Offspring complications, including miscarriage, prematurity, and neonatal death, are increased.

Diagnosis. In most cases, congenital heart disease is diagnosed well before pregnancy, giving the opportunity for a full pre-pregnancy risk assessment. The mWHO classes (Table 3) outline the broad risk categories.

4.2 Pulmonary hypertension and

Eisenmenger’s syndrome 4.2.1 Pulmonary hypertension 4.2.1.1 Introduction PH has many causes and is defined by an elevation in mean pulmonary arterial pressure (PAP) >_25 mmHg at right heart catheterization. The term PAH describes a subset of PH characterized by an LV filling pressure <_15 mmHg and a pulmonary vascular resistance >3 Wood Units.23 Untreated, idiopathic PH results in death within a median of 2.8 years. PAH is frequently encountered in females and the first clinical manifestations may be seen in pregnancy.140

4.2.1.2 Maternal risk Maternal outcome, which varies according to the PH subset, has improved with the availability of new targeted therapies and the use of a team-based, multidisciplinary approach.141–143 While pregnancy appears safer today, mortality remains high in women with PAH (16–30% maternal mortality).137,138 Therefore, the recommendation to avoid pregnancy remains and, when pregnancy occurs, termination should be discussed. The greatest period of risk is the puerperium and early post-partum. These women should be managed by a multidisciplinary team, with a PH expert included, in an expert centre for pregnancy and cardiac disease. Pulmonary hypertensive crisis, pulmonary thrombosis, and right HF are the most common causes of death. This may occur even in patients with few symptoms prior to pregnancy. Risk factors for maternal death are: severity of PH, late hospitalization, and perhaps the use of general anaesthesia.144 Even moderate forms of pulmonary vascular disease can worsen during pregnancy.138 Although there is no safe cut-off for elevated PAP risk, it is thought to be less in those with only mildly increased pressure.138

4.2.1.3 Obstetric and offspring risk There is increased foetal and neonatal (0–30%) mortality, particularly if there is preterm delivery, reduced maternal CO, and/or hypoxaemia.

4.2.1.4 Management The usual diagnostic algorithm of PH should be followed when a pregnant patient presents with new PH. Echocardiography is key and other diagnostic steps, in keeping with the PH guideline, are planned individually. Invasive right heart catheterization is recommended if there is diagnostic uncertainty and to assist important therapeutic decisions. If this is required, it should be performed in a specialist centre. Genetic counselling is appropriate in familial cases. A multidisciplinary team is required to care for the pregnant PH patient. This should be tailored to the patient, but will require very regular follow-up (often weekly in the third trimester). A full assessment, including oxygen saturation and assessment of RV function, should occur at each visit. Bed rest may be required in symptomatic patients and additional risk factors (such as air travel) avoided.

Thrombo-embolism is a major risk and anticoagulation should be considered (see section 11). Diuretics may be needed in patients with HF and iron deficiency should be treated. Pregnancy in PAH patients is a high-risk condition and a proactive approach should be taken to commencing advanced therapies. Risk stratification should be performed as in non-pregnant patients. There is no evidence of benefit comparing a stepwise approach vs. early combination therapy in pregnant patients, although the latter is often favoured, as per our Guidelines. Bosentan and other endothelin receptor antagonists are associated with embryopathy, and should be discontinued unless doing so would greatly increase maternal risk. An individualized approach is required and many units start therapy with oral sildenafil. The subset of patients with true vasodilator responsiveness who are well controlled on calcium channel blocker (CCB) therapy may be at lower risk and this therapy should be continued, as should all i.v. therapies. Section 12 discusses specific medications, including potential interactions with contraceptive drugs and anticoagulants.

4.2.1.5 Delivery A detailed delivery plan, including the optimal mode and timing of delivery, should be decided by the pregnancy heart team. This should include the post-partum need for intensive care and mechanical support. Regional anaesthesia is usually favoured over general anaesthesia.145 Meticulous fluid balance and optimization of RV function are important determinants of a good outcome. Patients remain at highrisk for many months post-delivery, and individualized counselling is needed to discuss the need for ongoing therapies and the avoidance of future pregnancies. Therapies should not be discontinued in the early post-delivery period.

4.2.2 Eisenmenger’s syndrome 4.2.2.1 Maternal risk Eisenmenger patients require special consideration because of the additional complications of cyanosis, right-to-left shunting, and paradoxical embolism. During pregnancy, systemic vasodilatation increases the right-to-left shunt and decreases pulmonary flow, leading to increased cyanosis and a low CO. Maternal mortality is high (20–50%) and termination of pregnancy should be discussed.146 However, termination also carries a risk.

4.2.2.2 Foetal risk Foetal and neonatal risks are increased and relate to maternal CO and cyanosis. Miscarriage is common. Maternal hypoxaemia is the most important predictor of outcome.

4.2.2.3 Management Many of the principles of caring for non-Eisenmenger PAH apply. However, patients with Eisenmenger’s syndrome are at increased risk of thrombocytopenia, deficiencies in vitamin K-dependent Downloaded from academic.oup.com/eurheartj/article/39/34/3165/5078465 by guest on 07 October 2026