4. Congenital heart disease and pulmonary hypertension4.1 Congenital heart disease is present in 0.8–0.9% of live births.Lesions vary in severity, but even patients with complex lesions now survive to childbearing years.In large international surveys of pregnancy and heart disease, two-thirds of cases had congenital heart disease and 5% had a PH.However, congenital heart disease and PH are rare causes of maternal death.

4.先天性心脏病和肺动脉高压

4.1先天性心脏病存在于0.8-0.9％的活产儿中.病变的严重程度不同，但即使是复杂病变的患者现在也可以于生育年龄存活.国际上有大量怀孕和心脏病的调查研究，三分之二的病人患有先天性心脏病，5％的病例有肺动脉高压。但是，先天性心脏病和肺动脉高压是产妇死亡的罕见原因。

In most women with congenital heart disease, pregnancy is well tolerated. The risk of pregnancy depends on the underlying heart 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. Obstetric complications such as (pre-) eclampsia are more often encountered. Offspring complications, including miscarriage, prematurity,and neonatal death,are increased.

在大多数患有先天性心脏病的女性中，怀孕耐受性良好。怀孕的风险取决于潜在的心脏缺陷以及其他因素，如心室功能，功能分级和紫绀。产妇心脏并发症占完成妊娠的10％，并且在患有复杂疾病的母亲中更常见。怀孕期间出现并发症的患者在怀孕后也可能有较高的晚期心脏事件风险.更常发生诸如（先兆子痫）的产科并发症。后代(婴儿)并发症，包括流产，早产和新生儿死亡，都在增加。

4.2 Pulmonary hypertension and Eisenmenger’s syndrome

4.2.1 Pulmonary hypertension 

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.Untreated, idiopathic PH results in death within a median of 2.8years. PAH is frequently encountered in females and the first clinical manifestations may be seen in pregnancy.

4.2肺动脉高压和艾森曼格综合征

4.2.1肺动脉高压

肺动脉高压有许多病因并且是通过右心导管测得肺动脉平均压≥25mmHg来定义的。术语肺动脉高压描述了PH的一个子集，其特征是左室充盈压≤15 mmHg，肺血管阻力> 3木材单位.未经处理，特发性肺动脉高压导致死亡的中位数为2.8年。女性多发肺动脉高压和可以在怀孕期间看到初始症状.

4.2.2 Eisenmenger’s syndrome

4.2.2.1 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(cardiac output

). Maternal mortality is high (20–50%) and termination of pregnancy should be discussed. However, termination also carries a risk.

4.2.2艾森曼格综合征

4.2.2.1艾森曼病患者需要特别考虑，因为紫绀，右向左分流和反常性血栓等额外并发症。在妊娠期间，全身性血管扩张增加了右向左分流并减少肺流量导致紫绀增加，心输出量减少。孕产妇死亡率很高（20-50％），应该讨论终止妊娠.但是，终止妊娠也会有风险。

4.2.3 Cyanotic heart disease without pulmonary hypertension

4.2.3.1 Cyanotic congenital heart disease is usually repaired before pregnancy, but some balanced, inoperable, or palliated cases do reach childbearing age.Maternal complications (HF, thrombosis, arrhythmias, and endocarditis) occur in ≥15% of cyanotic pregnant patients. Maternal outcome will be determined by the underlying condition and the ventricular function rather than the saturation level.

4.2.3无肺动脉高压的发绀性心脏病

4.2.3.1发绀的先天性心脏病通常在妊娠前被修复，但有些血流动力学平衡的，不能手术的或轻度的病例确实到了生育年龄.孕产妇并发症（心衰，血栓形成，心律失常和心内膜炎）发生在≥15％的紫绀患者中。孕产妇结局将由潜在的条件和心室功能而不是饱和度水平决定。

4.4Recommendation for pregnancy and pulmonary arterial hypertension

Right heart catheterization is recommended to confirm the diagnosis of PAH (group 1). This can be performed during pregnancy but with very strict indications.IC

Treatment dose LMWH is recommended in pregnant patients with chronic thrombo-embolic pulmonary hypertension. IC

If a PAH patient conceives on targeted PH therapies, consideration should be given to withdrawing embryotoxic drugs, taking into account the risks of withdrawal. IIaC

In treatment-naive pregnant PAH patients, initiating treatment should be considered.IIaC Pregnancy is not recommended in patients with PAH.IIIB

对于妊娠和肺动脉高压的建议

推荐进行右心导管检查以确认肺动脉高压的诊断（第1组）。这可以在怀孕期间进行，但有非常严格的适应症.IC

治疗剂量低分子肝素推荐用于慢性血栓栓塞性肺动脉高压的孕妇。IC

如果肺动脉高压患者设想有针对性的肺动脉高压治疗，应考虑撤回胚胎毒性药物，同时考虑戒断的风险。IIaC

在未接受过治疗的肺动脉高压患者中，应考虑开始治疗. IIaC

肺动脉高压患者不推荐妊娠.IIIB

Recommendations for congenital heart disease Patients with systemic ventricular dysfunction (EF <40%), or severe TR should be advised against pregnancy. IIaC

Anticoagulation treatment should be considered during pregnancy in Fontan patients. IIaC Symptomatic patients with Ebstein’s anomaly with saturations <85% and/or heart failure should be advised against pregnancy. IIaC

In patients with a Fontan circulation and saturations<85%, depressed ventricular function, moderate–severe 主动脉 regurgitation, refractory arrhythmia, or protein-losing enteropathy, pregnancy is not recommended. III C

有关先天性心脏病的建议

患有全身性心室功能不全（射血分数 <40％）或严重三尖瓣反流的患者应建议不要怀孕。IIaC

在方坦手术的患者，怀孕期间应考虑抗凝治疗。 IIaC 三尖瓣下移畸形且饱和度<85％和/或有心力衰竭的症状性患者应建议不要怀孕。IIaC

对于方坦循环和饱和度<85％，心室功能低下，中重度房室反流，难治性心律失常或蛋白质丢失性肠病的患者，不建议怀孕。III C.

5. Aortic diseaseaSeveral heritable disorders affect the thoracic aorta, predisposing patients to both aneurysm formation and aortic dissection. These include HTAD and either syndromic (Marfan syndrome) or non-syndromic HTAD (only aortic aneurysm). New genes are regularly discovered. Other forms of congenital heart disease (e.g. tetralogy of Fallot ) may also be accompanied by aortic dilatation, and finally non-heritable aortic pathology may occur.Risk factors for aortic dilatation are hypertension and advanced maternal age. Pregnancy is a high-risk period for all patients with aortic pathology, which is rare during pregnancy but associated with very high mortality.Most deaths occur in women not previously known to had an aortopathy. Most of these women will had heritable disease, so autopsy tissue should be sa-ved for DNA analysis and families offered referrals for screening.

5.主动脉疾病

几种可遗传的疾病影响胸主动脉，使患者易于形成动脉瘤和主动脉夹层。这些包括遗传性主动脉疾病并且包括综合征性（马方综合征）或非综合征性遗传性主动脉疾病（即仅主动脉瘤）。定期发现新基因。其他形式的先天性心脏病（例如法洛四联症）也可能伴有主动脉扩张，最终可能会发生非遗传性主动脉病变。主动脉扩张的风险因素是高血压和高龄产妇。妊娠是所有主动脉病变患者的高危时期，在妊娠期间很少见，但死亡率很高。大多数死亡发生在以前不知道患有主动脉病的女性中。这些妇女大多数都有遗传性疾病，因此应保存尸检组织用于DNA分析，并提供可参考的家族以进行筛查。

5.1 Maternal and offspring risk Haemodynamic and hormonal changes during pregnancy increase the susceptibility to dissection. Dissection occurs most often in the last trimester of pregnancy (50%) or the early post-partum period (33%). All women with a genetically proven syndrome or familial aortic pathology should had counselling on the risk of dis- section and the recurrence risk, and had a complete evaluation including imaging of the entire aorta before pregnancy. When assessing aortic diameters, body surface area should be considered, especially in women of small stature. Parity seems associated with increased aortic diameter.The effect of pregnancy on aortic dilatation is not clear.The diagnosis of aortic dissection should be considered in all patients with chest pain during pregnancy.

5.1产妇和后代的风险

妊娠期间的血流动力学和激素变化增加了对夹层的敏感性。夹层最常发生在妊娠的最后三个月（50％）或产后早期（33％）。所有患有遗传学证明的综合征或家族性主动脉病变的妇女均应就夹层风险和复发风险进行咨询，并进行全面评估，包括在怀孕前对整个主动脉进行影像检查。在评估主动脉直径时，应考虑身体表面积，尤其是身材矮小的女性。分娩次数似乎与主动脉直径增加有关。妊娠对主动脉扩张的影响尚不清楚。所有妊娠期胸痛的患者均应考虑主动脉夹层的诊断。

5.2 Specific syndromes

Marfan syndrome is thought to affect 1 in 5000 individuals. Although bicuspid aortic valve is more common (1–2% of the population), associated aortic complications are uncommon, accounting for only 6% of type A dissections during pregnancy.

5.2特定的综合症

据认为，马凡氏综合症会影响5000个个体中的1个。尽管二瓣型主动脉瓣更常见（占总人口的1-2％），但相关的主动脉并发症并不常见，在妊娠期间仅占A型解剖的6％。

5.3 Management

5.3.1 Follow-up and medical therapy

Depending on the aortic diameter, patients with aortic pathology should be monitored by echocardiography at regular intervals through-out the pregnancy and 6 months post-partum. In women with a high-risk of dissection or an already severely dilated aorta, monitoring every month is warranted,while in low-risk women with only a mildly dilated aorta, monitoring every 12 weeks seems reasonable. When needed, cardiac MRI without contrast can be used. Pregnancy should be supervised by a cardiologist and obstetrician who are alert to the possible complications. Strict BP control is advised, and antihypertensive treatment that is safe for the foetus should be initiated if necessary.In women with HTAD(heritable thoracic aorta disorders), beta-blocker therapy throughout pregnancy should be considered. In patients with Ehlers–Danlos syndrome type IV, celiprolol is recommended (also in normotensive women) because of the very high-risk of dissections and the benefit demonstrated in non-pregnant patients. Foetal growth should be monitored when the mother is taking beta-blockers.

5.3管理

5.3.1随访和药物治疗

根据主动脉直径，在整个妊娠期间和产后6个月，应定期以超声心动图监测主动脉病变的患者。对于具有高夹层风险或已经严重扩张主动脉的妇女，应每月进行监测，而对于低危，仅具有轻度扩张主动脉的妇女，每12周进行监测似乎是合理的。需要时，可以使用无对造影剂的心脏磁共振。怀孕应由心脏病专家和产科医生监督，他们要警惕可能的并发症。建议严格控制血压，必要时应采取对胎儿安全的降压治疗。对于患有遗传性主动脉疾病的女性，应考虑在整个妊娠期进行β受体阻滞剂治疗。在患有埃莱尔-当洛综合征IV型的患者中，因为夹层风险很高，建议使用塞利洛尔（在血压正常的女性中也是如此），并且在非怀孕患者中亦获益。当服用β受体阻滞剂时，应监测胎儿的生长情况。

5.4 Recommendations for the management of aortic disease

All aortic diseases

1 It is recommended that women with aortic disease had counselling about the risk of aortic dissection.IC

2 Imaging of the entire aorta (CT/MRI) is recommended before pregnancy in patients with a genetically proven aortic syndrome or known aortic disease.IC

3 In bicuspid aortic valve patients, imaging of the ascending aorta is recommended before pregnancy.IC

4 When a woman with known aortic dilatation (history of) dissection or genetic predisposition for dissection becomes pregnant, strict blood pressure control is recommended. IC

5 Repeated echocardiographic imaging every 4–12 weeks (depending on diagnosis and severity of dilatation) is recommended during pregnancy and 6 months post-partum in patients with ascending aorta dilatation. IC

6 For imaging of pregnant women with dilatation of the distal ascending aorta, aortic arch, or descending aorta, MRI (without gadolinium) is recommended. IC

7 It is recommended to deliver all women with aortic dilatation or (history of) aortic dissection in an experienced centre with a pregnancy heart team, where cardiothoracic surgery is a-vailable. IC

5.4对主动脉疾病的治疗建议

所有主动脉疾病

1建议患主动脉疾病的妇女对主动脉夹层的危险性进行咨询。

2建议经遗传学证明的主动脉综合征或已知的主动脉疾病的患者怀孕前进行完整主动脉的影像学检查（CT / MRI）。

3对于二瓣型主动脉瓣患者，建议在怀孕前对升主动脉进行成像检查.

4当具有已知主动脉扩张（病史）夹层或遗传倾向夹层的女性怀孕时，应严格采血建议使用压力控制。

5推荐在升主动脉扩张的患者怀孕期间和产后6个月，每4-12周（取决于扩张的诊断和严重程度）进行反复超声心动图检查。

6对扩张的远端升主动脉，主动脉弓或降主动脉的孕妇推荐进行成像的MRI检查。

7建议所有有主动脉扩张或主动脉夹层史的孕妇在有心脏胸腔手术的妊娠心脏小组的经验丰富的中心分娩。

8 In patients with an ascending aorta <40 mm, vaginal delivery is recommended.IC

9 In patients with an ascending aorta >45 mm, caesarean delivery should be considered.IIaC

10 In patients with (history of) aortic dissection, caesarean delivery should be considered.IIaC

11 Prophylactic surgery should be considered during pregnancy if the aorta diameter is >45 mm and increasing rapidly.IIaC

12 When the foetus is viable, delivery before necessary surgery should be considered.IIaC

13 In patients with an aorta 40–45 mm, vaginal delivery with epidural anaesthesia and an expedited second stage should be considered. IIaC 14 In patients with an aorta 40–45 mm, caesarean section may be considered.IIbC

15 Pregnancy is not recommended in patients with (or history of) aortic dissection.IIIC

16 When possible, the use of ergometrine is not recommended in women with aortic disease.IIIC

8对于升主动脉<40 mm的患者，建议阴道分娩。

9在升主动脉> 45 mm的患者中，应考虑剖宫产。

10在有主动脉夹层（病史）的患者中，应考虑剖腹分娩。

11如果主动脉直径> 45 mm并迅速增加，则应在怀孕期间考虑进行预防性手术。

12当胎儿能养活时，应考虑在必要的手术之前分娩。

13在主动脉40-45 mm的患者中，经硬膜外麻醉的阴道分娩并且应该考虑加快第二阶段。

14对于主动脉40-45 mm的患者，可考虑进行剖腹产。

15不建议有主动脉夹层史的患者怀孕。

16如果可能，不建议在患有主动脉疾病的女性中使用麦角新碱。

Specific syndromes

17 In patients with vascular Ehlers–Danlos syndrome, celiprolol is recommended.IC

18 Beta-blocker therapy throughout pregnancy should be considered in women with Marfan syndrome and other heritable thoracic aortic diseases. IIaC

19 Pregnancy is not recommended in patients with severe dilatation of the aorta (heritable thoracic aortic disease such as Marfan syndrome >45 mm, bicuspid aortic valve >50 mm).IIIC

20 Pregnancy is not recommended in patients with vascular Ehlers–Danlos syndrome. IIIC

特定综合征

17对于患有血管性综合征的患者，建议使用塞利洛尔

。

18在马凡综合征和其他遗传性胸主动脉疾病患者中，应考虑在整个妊娠期进行β-受体阻滞剂治疗。

19严重主动脉扩张（遗传性胸主动脉疾病，例如Marfan综合征> 45 mm，双瓣型主动脉瓣> 50 mm）的患者，不建议妊娠.

20血管性Ehlers–Danlos综合征患者不建议怀孕。

6. Valvular heart disease

At childbearing age, valvular heart disease is often due to rheumatic heart disease, particularly in low–middle-income countries. Mechanical valve prostheses raise specific problems during pregnancy. Risk assessment and management need to consider the resources available in high- and low–middle-income countries.

6.1 Stenotic valve lesions

In stenotic valve diseases, increased cardiac output causes an increase in transvalvular gradient of 50%, mainly between the first and second trimesters, which increases the risk of maternal and foetal complications.

6.瓣膜性心脏病

在育龄期，瓣膜性心脏病通常是由于风湿性心脏病，特别是在低中收入国家。机械人工瓣膜在妊娠期间会引发特定的问题.风险评估和管理需要考虑资源在高 和低 - 中等收入国家的可用性。

6.1狭窄性瓣膜病变

在狭窄性瓣膜疾病中，心输出量增加导致经瓣膜压力阶差增加50％，主要发生在早期和妊娠中期，增加了母体和胎儿并发症的风险。

6.1.1 Mitral stenosis

Mild mitral stenosis (MS) is generally well tolerated.HF occurs in one-third of pregnant women with a valve area ≤1.0 cm（centimeter）2 and in one-half of those with a valve area ≤1.5 cm2,most often during the second trimester, even in the absence of symptoms before pregnancy.Sustained AF, although rare (<10%), may precipitate HF and thrombo-embolic events.Mortality is between 0–3% in western countries and higher in low–middle-income countries. NYHA class ≥ II, systolic PAP (pulmonary artery pressure)>30 mmHg, severe stenosis, and older age are associated with maternal complications.

6.1.1二尖瓣狭窄

轻度二尖瓣狭窄通常耐受良好.心衰发生在三分之一的瓣膜面积≤1.0 cm2的和一半瓣膜面积≤1.5 cm2的孕妇中，最常见于妊娠中期，即使在怀孕前没有症状.持续性房颤虽然罕见（<10％），但可能导致心衰和血栓栓塞事件.死亡率在西方国家为0-3％之间和低中收入国家中的更高. NYHA级≥II，收缩期肺动脉压> 30 mmHg，严重狭窄和年龄较大与母亲并发症有关.

6.1.2 Valvular aortic stenosis

The main cause of AS is bicuspid aortic valve followed by rheumatic heart disease.

6.1.2.1 Cardiac morbidity is related to the baseline severity of AS and symptoms.HF is rare (<10%) in women with moderate AS and in those who were asymptomatic before pregnancy, while it occurs in one out of four symptomatic patients.Even in patients with severe AS, pregnancy is often well tolerated if prior exercise tolerance was normal. Mortality is now rare if careful management is provided .Arrhythmias are rare.Women with bicuspid aortic valve have a low-risk of aortic dissection if the aortic diameter is< 50mm。

6.1.2主动脉瓣狭窄

AS的主要原因是二尖瓣型主动脉瓣，其次是风湿性心脏病。

6.1.2.1心脏病发病率与主动脉瓣狭窄和症状的基线严重程度有关.心衰在怀孕前无症状者和在中度主动脉瓣狭窄患者中罕见（<10％），而在四分之一的症状患者中发生.即使在患有严重AS的患者中，如果先前的运动耐受性是正常的，那么怀孕通常也能很好地耐受.如果细心管理，死亡是极罕见的.心律失常是罕见的.如果主动脉直径<50mm，女性患有二尖瓣型主动脉瓣发生主动脉夹层风险较低。

6.2 Regurgitant lesions

6.2.1 Mitral and aortic regurgitation

Mitral and aortic regurgitation can be of rheumatic, congenital, or degenerative origin.

6.2.1.1 Women with severe regurgitation and symptoms or compromised LV function are at high-risk of HF. HF occurs in 20–25% of women with moderate or severe rheumatic MR.Acute severe regurgitation is poorly tolerated. In women with congenital heart disease, significant left aortic valve regurgitation is associated with cardiac complications during pregnancy. A persistent worsening of regurgitation may occur.

6.2返流性病变

6.2.1二尖瓣和主动脉瓣关闭不全

二尖瓣和主动脉瓣关闭不全可能是风湿性，先天性或退行性起源.

6.2.1.1有严重反流和症状或左室功能受损的女性患心衰的风险很高.心衰常发生在在20-25％的中度或重度风湿性二尖瓣关闭不全患者.急性严重的反流耐受性差。在患有先天性心脏病的女性中，显著的左房室瓣反流与妊娠期间的心脏并发症有关。持续恶化的反流可能会反复发作。

6.2.2 Tricuspid regurgitation

Secondary TR is more frequent than primary TR, which may be due to endocarditis or Ebstein’s anomaly. Maternal risk is usually determined by left-sided valve disease or PH. However, maternal risk can be increased in severe symptomatic TR or in women with RV dysfunction.In women with congenital heart disease, moderate/severe aortic valve regurgitation may be associated with maternal cardiac complications, which are mainly arrhythmias.Even severe TR with HF can usually be managed conservatively during pregnancy. When surgery is necessary for left-sided valve lesions, additional tricuspid repair is indicated in severe TR and should be considered in moderate TR with annular dilatation (≥40 mm).In severe symptomatic TR ， repair should be considered pre-pregnancy.

6.2.2三尖瓣关闭不全

继发性三尖瓣关闭不全比原发性更常见，可能是由于心内膜炎或埃布斯坦的异常。产妇风险通常由左侧瓣膜病或肺动脉高压决定。然而，严重症状性三尖瓣关闭不全患者或患有右室功能障碍的女性可能会增加产妇风险.对于患有先天性心脏病的女性，中度/重度房室瓣反流可能与母亲心脏并发症有关，主要是心律失常.即使是严重的伴有心衰的三尖瓣关闭不全也可以采取怀孕期间保守治疗。当左侧瓣膜病变需要手术治疗时，严重三尖瓣关闭不全需要额外的三尖瓣修复，中度三尖瓣关闭不全应考虑采用环形扩张（≥ 40 mm）.严重有症状的三尖瓣关闭不全，怀孕前应该考虑修复。

6.3 Atrial fibrillation in native heart valve disease

A high thrombo-embolic risk is associated with AF, particularly in clinically significant MS. Immediate anticoagulation is required, using LMWH at therapeutic doses in the first and last trimesters , and VKAs(Vitamin K antagonist ) with the usual target INRs or LMWH for the second trimester. Non-VKA OACs are contraindicated throughout pregnancy. The choice between cardioversion and rate control using digoxin or beta-blockers depends on the severity of the underlying valve disease and the tolerance 。

6.3自发性心脏瓣膜病的心房颤动

高血栓栓塞风险与房颤相关，特别是在临床显著的二尖瓣狭窄中。需要立即抗凝，在第一次和最后一次使用低分子肝素治疗剂量，并且在妊娠中期使用常用达目标INR的维生素K拮抗剂和低分子肝素。非维生素K拮抗剂口服抗凝药在整个怀孕期间都是禁忌的。使用地高辛或β受体阻滞剂进行心脏复律和速率控制之间的选择取决于潜在瓣膜疾病的严重程度和耐受性。

6.4 Choice of valve prosthesis

When implantation of a prosthetic valve is unavoidable in a woman who wants to become pregnant in the future, valve selection is challenging. Mechanical valves offer excellent haemodynamic performance and long-term durability, but the need for anticoagulation increases maternal and foetal mortality and morbidity, and the risk of major cardiac events during pregnancy is much higher than with bioprosthetic valves.However, bioprosthetic valves in young women are associated with a high-risk of structural valve deterioration resulting in the risk of going through pregnancy with a dysfunctional valve, and eventually in the inevitable need for re-operation. Transcatheter valve implantation (currently especially in pulmonary valves) and the Ross procedure in aortic valve disease (pulmonary autograft in the aortic position and pulmonary homograft) are alternative options to be considered. Data on pregnancy after a Ross procedure are scarce but indicate low-risk in the absence of aortic dilatation.A desire for pregnancy is a class IIa indication for a biological valve.In young women who wish to become pregnant in the future, the pregnancy heart team should be involved in the choice of a specific prosthesis. The final choice should be made after extensive sharing of information and discussion with the patient.

6.4人工瓣膜选择

当一个想要怀孕的女性不可避免地植入人工瓣膜时，选择瓣膜是具有挑战性的。机械瓣膜具有极佳的血液动力学性能和长期耐久性，但抗凝需求会增加孕产妇和胎儿的死亡率和发病率，怀孕期间发生重大心脏事件的风险远高于生物人工瓣膜.但是，年轻女性的生物瓣膜与结构性瓣膜恶化的高风险相关，导致怀孕期间瓣膜功能失调，并最终不可避免地需要再次手术。经导管瓣膜植入术（目前特别是在肺动脉瓣膜）和主动脉瓣膜疾病中的Ross术（主动脉瓣位自体肺动脉瓣以及肺动脉瓣位同种瓣）是可考虑的选择.Ross手术后妊娠的数据很少但表明没有主动脉扩张的低风险.若渴望妊娠，生物瓣膜是IIa类适应症.对于希望在未来怀孕的年轻女性，怀孕期间可能需要妊娠期心脏团队参与对瓣膜种类的选择。最后的选择应该是在与病人进行信息交流和讨论之后做出的。

6.5 Mechanical prostheses and anticoagulation

In women with mechanical valves, pregnancy is associated with a very high-risk of complications (WHO risk classification III). In the ROPAC registry, the chances of an event-free pregnancy with a live birth were 58% for women with a mechanical valve, compared with 79% for women with a bioprosthesis and 78% for women with heart disease but no valve prosthesis.A recent study from the UK reported a favourable outcome for mother and baby in only 28% of cases.The main risks are related to the need for anticoagulation therapy(valve thrombosis and haemorrhagic complications). Additional risks are related to ventricular and valvular dysfunction.

6.5机械人工瓣膜和抗凝

有机械瓣膜的女性，妊娠与并发症的高风险相关（WHO风险分类III）。在ROPAC登记中，有机械瓣膜的妇女无病怀孕安全降生的概率为58％，而生物瓣膜妇女为79％，无人工瓣膜心脏病妇女为78％。最近英国的一项研究报告显示，只有28％的病例对母婴有利.主要风险与抗凝治疗需求（血栓栓塞和出血并发症）有关。额外风险与心室和瓣膜功能不全相关。

6.6Recommendations for the management of native valvular heart disease

1.Pre-pregnancy evaluation, including echocardiography, and counselling is recommended for any woman with known or sus- pected valvular disease. Ic

2.Mitral stenosis

1)In patients with symptoms or pulmonary hypertension, restricted activities and beta-1-selective blockers are recommended.Ib

2)Diuretics are recommended when congestive symptoms persist despite beta-blockers.Ib

3)Intervention is recommended before pregnancy in patients with MS and valve area <1.0 cm2.Ic

4)Therapeutic anticoagulation using heparins or VKA is recommended in case of atrial fibrillation, left atrial thrombosis, or prior embolism.Ic 5)Intervention should be considered before pregnancy in patients with MS and valve area <1.5cm2.IIa

6) Percutaneous mitral commissurotomy should be considered in pregnant patients with severe symptoms or systolic pulmonary artery pressure >50 mmHg despite medical therapy. IIa

6.6关于管理原发性心脏瓣膜病的建议

1.对于任何已知或疑似瓣膜病的女性，建议进行孕前评估，包括超声心动图和咨询。

2.二尖瓣狭窄

1)患有症状或肺动脉高压的患者，推荐限制活动和β-1-选择性阻滞剂使用.

2)尽管应用了β阻滞剂，充血性心衰持续存在时推荐应用利尿剂.

3)在二尖瓣狭窄和瓣膜面积<1.0平方厘米的患者怀孕前建议进行干预.

4)在心房颤动，左心房血栓形成或先前栓塞的情况下，建议使用肝素或维生素k拮抗剂进行治疗性抗凝治疗。

5) 二尖瓣狭窄患者和瓣膜面积<1.5平方厘米的患者在怀孕前应考虑干预。

6)对于症状严重或收缩期肺动脉压> 50 mmHg的孕妇，尽管进行药物治疗，仍应考虑经皮二尖瓣切开术。

6.6.3.Aortic stenosis

Intervention is recommended before pregnancy in patients with severe aortic stenosis if:

  they are symptomatic  Ib

  OR LV dysfunction (LVEF <50%) is present Ic

  OR when they develop symptoms during exercise testing.  Ic

Intervention should be considered before pregnancy in asymptomatic patients with severe AS when a fall in blood pressure below baseline during exercise testing occurs. IIa

Balloon aortic valvuloplasty should be considered during pregnancy in patients with severe aortic stenosis and severe symptoms.IIa

6.6.4Chronic regurgitant lesions

Surgical treatment is recommended before pregnancy in patients with severe aortic or mitral regurgitation with symptoms of impaired ventricular function or ventricular dilatation.Ic Medical therapy is recommended in pregnant women with regurgitant lesions when symptoms occur. Ic

6.6.3主动脉瓣狭窄如果出现以下情况，建议在严重主动脉瓣狭窄患者怀孕前进行干预：有症状，或左室功能障碍（LVEF <50％）存在，或者当他们在运动测试期间出现症状时。

在运动试验期间血压下降至基线以下时，对于患有严重主动脉狭窄的无症状患者，应在怀孕前考虑干预。

对于严重主动脉瓣狭窄和严重症状的患者，在怀孕期间应考虑主动脉瓣膜球囊成形术。

6.6.4慢性返流性病变

建议在妊娠前患严重主​​动脉或二尖瓣返流，伴有心室功能受损或心室扩张症状明显的患者进行手术治疗.

当症状出现时，建议对有返流性病变的孕妇进行药物治疗。

6.6.5Recommendations for the management of prosthetic heart valves

1)It is recommended that the valve prosthesis for a woman contemplating pregnancy is chosen in consultation with a pregnancy heart team.

2)It is recommended to manage pregnancy in women with mechanical valves in a centre with a pregnancy heart team.

3)If delivery starts while on a VKA or in less than 2 weeks after discontinuation of a VKA, caesarean section is recommended .

4)It is recommended to discontinue VKAs and start adjusted-dose intravenous UFH (unfractionated heparin)(aPTT ≥2x control) or adjusted-dose LMWH at the 36th week of gestation.

5) In pregnant women on LMWH or UFH, it is recommended to perform weekly anti-Xa level monitoring or aPTT monitoring with dose adjustment (within 36 h).

6) In pregnant women on a VKA, it is recommended to perform INR monitoring weekly or every 2 weeks

7)In pregnant women with LMWH, it is recommended to target anti-Xa levels 4–6 h post-dose at 0.8–1.2 U/l (aortic valve prosthesis) or 1.0–1.2 IU/mL (mitral and right-sided valve prostheses).

6.6.5关于人工心脏瓣膜管理的建议

1)建议与妊娠心脏团队协商选择适合怀孕的女性的人工瓣膜。

2)建议在妊娠心脏中心对机械瓣膜的妇女进行妊娠管理.

3)如果在应用维生素k拮抗剂期间开始分娩或在停用VKA后不到2周内开始分娩，推荐剖腹产。

4)建议在妊娠第36周停用维生素k拮抗剂并开始调整 静脉剂量用普通肝素（aPTT≥2x对照）或调整皮下剂量的低分子肝素钠。

5)对于应用低分子肝素钠或普通肝素的孕妇，建议每周进行抗Xa水平监测或aPTT监测，并调整剂量（36小时内）。

6)对于应用维生素k拮抗剂的孕妇，建议每周或每2例进行一次INR监测。

7)对于应用低分子肝素钠孕妇，建议在以0.8-1.2 U / l（主动脉瓣人工瓣膜）或1.0-1.2 IU / mL（二尖瓣和右侧人工瓣膜）给药后4-6小时达到目标抗-Xa水平。

7)It is recommended to replace LMWH with intravenous UFH (aPTT ≥2x control) at least 36 h before planned delivery.

8)UFH should be continued until 4–6 h before planned delivery and restarted 4–6 h after delivery if there are no bleeding complications.

9) It is recommended to anticipate the timing of delivery to ensure safe and effective peripartum anticoagulation.

10)Immediate echocardiography is recommended in women with mechanical valves presenting with dyspnoea and/or an embolic event.

11) It is recommended to implement changes in the anticoagulation regimen during pregnancy in hospital.

12)During the second and third trimesters until the 36th week, VKAs are recommended in women needing a low dose.

13)A bioprostheses should be considered in young women contemplating pregnancy.

14)During the second and third trimesters until the 36th week, VKAs should be considered in women needing a high dose.

15)Continuation of VKAs should be considered during the first trimester if the warfarin dose required for therapeutic anticoagulation is <5 mg/day (or phenprocoumon <3 mg/day or acenocoumarol <2 mg/day) after patient information and consent.

16)Discontinuation of VKAs between weeks 6 and 12, and replacement with adjusted-dose intravenous UFH (aPTT ≥2x control) or adjusted- dose LMWH twice daily，should be considered in patients with a warfarin dose >5 mg/day (or phenprocoumon >3 mg/day or acenocoumarol >2 mg/day).

7)建议在计划分娩前至少36小时用静脉应用普通肝素（aPTT> _2x对照）替换低分子肝素。

8)如果没有出血并发症，普通肝素应持续到计划分娩前4-6小时，并在分娩后4-6小时重新开始。

9)建议预测分娩的时间，以确保安全有效的围产期抗凝治疗。

10)建议在机械瓣膜患者出现呼吸困难和/或栓塞事件时立即进行超声心动图检查。

11)建议在住院怀孕期间实施抗凝治疗方案的变化。

12)在妊娠中期和孕晚期至第36周，建议在需要较低剂量的女性使用维生素k拮抗剂。

13)在计划妊娠年轻女性应考虑生物瓣膜。

14)在妊娠中期和孕晚期至第36周，建议在需要高剂量的女性应该考虑维生素k拮抗剂。

15)在患者知情和同意后，如果治疗性抗凝治疗所需的华法林剂量<5 mg /天（或苯丙氨酸<3 mg /天或醋硝香豆素<2 mg），应在孕早期考虑维生素k拮抗剂持续时间。

16) 华法林剂量> 5 mg /天的患者应考虑在第6周至第12周停用VKA，并每日更换调整剂量静脉注射普通肝素（aPTT≥2倍对照值）或调整剂量每日2次皮下注射低分子肝素钠。