A 29-year-old woman (gravida 2, para 1) at 26 weeks of gestation with monochorionic diamniotic twins presented with 6 weeks of dyspnea, orthopnea, palpitations, lower extremity edema, and enlargement of a facial birthmark (Figure 1). She had a history of heart failure as a newborn secondary to cerebral arteriovenous malformation (AVM), which was resected at 10 weeks of age with no further heart failure symptoms. She had previously carried a singleton pregnancy to term without complications. Family history was significant for cutaneous capillary malformations (CMs) in her mother, sister, and brother.

Figure 1. Superficial appearance of left cheek vascular malformation. Vascular malformation shown (A) before pregnancy, (B) on initial presentation (26 wks, 1d), (C) before embolization (29wks, 3d), (D) 4 d after delivery, and (E) 2wks after delivery.

She presented with a temperature of 37°C, a heart rate of 131 beats per minute, a blood pressure of 129/84 mm Hg, a respiratory rate of 22 breaths per minute, and an oxygen saturation of 98% on room air. Exam demonstrated a pulsatile 12×7.5 cm left-sided facial mass (Figure 1), a 4×2 cm pulsatile left-sided scalp mass, a jugular venous pressure of 10 cm of water, a II/VI systolic murmur at the left sternal border, and 2+ bilateral lower extremity pitting edema. Laboratories revealed a hemoglobin of 8.6 g/dL, cardiac troponin I of <0.04 ng/mL, B-type natriuretic peptide of 93 pg/mL, and a thyroid stimulating hormone of 0.55 mcIU/mL. ECG demonstrated sinus tachycardia at 132 beats per minute. Transthoracic echocardiogram revealed an ejection fraction of 65% to 70%, biatrial enlargement, and a calculated noninvasive cardiac output (CO) of 16.5 L/min (normal CO for twin gestation, 7–8 L/min).¹ Magnetic resonance angiography with ferumoxytol revealed a CO of 14 L/min, and high-flow vascular lesions of the left cheek, left skull vertex, and right lower lateral neck (Figure 2).

Figure 2. Magnetic resonance angiography time resolved series at 27 wk gestation demonstrating left check (green arrow), left scalp (red arrow), and right shoulder (pink arrow) high-flow vascular malformations. The 3 frames show progressive evolution of enhancement from arterial to late venous phases.

The patient was diagnosed with high-output heart failure and was initially managed for 3 weeks with intravenous and oral diuretics in the inpatient and outpatient settings. Despite diuresis, she developed progressive heart failure and subsequently underwent embolization of her cheek and skull vertex vascular malformations with N-butyl cyanoacrylate glue and polyvinyl alcohol particles, resulting in ≈50% flow reduction by angiography (Figure 3). The patient symptomatically improved postoperatively. Transthoracic echocardiogram after the procedure demonstrated a calculated noninvasive CO of 11.9 L/min. On postembolization day 5, she developed thrombocytopenia and acute kidney injury. Given the concern for preeclampsia, she underwent cesarean section at 30 weeks and 1 day with resolution of heart failure symptoms. Twins A and B were born with birthweights of 1300 and 1700 g, respectively. Twin A was born with hypospadias and a small ventricular septal defect. Transesophageal echocardiography immediately after delivery demonstrated a calculated noninvasive CO of 9.3 L/min. The patient was discharged on oral diuretics. Her facial engorgement fully resolved 2 weeks postpartum (Figure 1). At time of writing, the twins are 23 weeks old and doing well.

Figure 3. Angiography of the vascular malformation and surrounding blood vessels.A, Before embolization: left external carotid artery angiogram demonstrates a hypervascular blush overlying the left parietal scalp primarily supplied by a hypertrophied branch of the superficial temporal artery. B, After embolization: left external carotid artery angiogram shows decreased opacification of the vascular malformation nidus. The hypertrophied superficial temporal artery branch has been occluded.

Here, we describe a case of high-output heart failure secondary to enlarging vascular malformations in the setting of multiple gestation pregnancy. CMs manifest as red patches on the head and neck and are found in 0.3% of newborns.² More rarely, CMs associate with high-flow AVMs in an autosomal dominant disorder called CM-AVM syndrome.³ The patient’s presentation, lesion appearance, and family history of cutaneous CMs (mother, sister, and brother) point to a clinical diagnosis of CM-AVM syndrome. This disorder frequently associates with loss-of-function mutations in RASA1 and EPHB4 and has high penetrance and variable expressivity.^4–6 The patient is still considering genetic testing.

AVMs are hormone sensitive and increase their size and flow in response to hormonal changes. As a result, they may enlarge during adolescence or pregnancy.^3,7,8 The cardiovascular changes associated with pregnancy increase the risk for heart failure in those with vascular malformations. In the first trimester, women experience a drop in peripheral vascular resistance due to systemic vasodilation.¹ Heart rate and stroke volume rise, resulting in an increase in CO. This rise can approach 145% of normal in singleton pregnancies and can reach 7 to 8 L/min in twin gestations.^1,9 The increased estrogen and progesterone from the patient’s multiple gestation pregnancy likely significantly contributed to the increased size and flow of her existing CM-AVMs.

Vascular malformations in pregnancy are typically managed with a conservative watch and wait strategy. Given the patient’s clinical deterioration, embolization of the AVMs was performed. To our knowledge, this represents the first report of CM-AVM embolization during pregnancy. Despite significant postoperative reduction in arteriovenous shunting and mild symptomatic improvement, the patient developed signs of superimposed preeclampsia, ultimately requiring preterm delivery. The regression of her AVMs postpartum confirmed their hormonal sensitivity.

Acute decompensated high-output heart failure in pregnancy due to congenital vascular malformations is rare and can pose significant management challenges. Endovascular embolization may have a role in the management of such conditions following the failure of conservative medical therapies. The postoperative development of preeclampsia necessitating urgent delivery in this report, however, highlights the complexities inherent in such cases.

Dr Hogeling is a Principal Investigator on a study sponsored by Celgene. The other authors report no conflicts.

*Drs Srivastava and Vyas contributed equally to this work.

For Disclosures, see page 3.

一名在怀孕26周时患有单绒毛膜双胎双胎的29岁妇女（妊娠2第1段）出现了6周的呼吸困难，正气呼吸，心pal，下肢浮肿和面部胎记增大（图1）。她有心脏衰竭的病史，是新生儿继发于脑动静脉畸形（AVM）的心脏衰竭，该病在10周龄时被切除，没有进一步的心脏衰竭症状。她以前曾单胎妊娠，没有并发症。她的母亲，姐姐和弟弟的皮肤毛细血管畸形（CM）的家族史非常重要。

图1. 左脸颊血管畸形的表面外观。血管畸形显示为（A）怀孕前，（B）初次出现时（26 wks，1d），（C）栓塞前（29wks，3d），（D）分娩后4 d和（E）分娩后2wks。

她的体温为37°C，每分钟131次心跳，血压为129/84 mm Hg，每分钟呼吸22次，室内空气中的氧饱和度为98％。考试显示搏动性左侧脸部肿块为12×7.5 cm（图1），搏动性左侧头皮肿块为4×2 cm，颈静脉静脉压为10 cm水，左胸骨处有II / VI收缩期杂音边界，以及2+双侧下肢点状浮肿。实验室显示血红蛋白为8.6 g / dL，心肌肌钙蛋白I <0.04 ng / mL，B型利钠肽为93 pg / mL，甲状腺刺激激素为0.55 mcIU / mL。心电图显示窦性心动过速为每分钟132次。经胸超声心动图检查显示射血分数为65％至70％，小儿增大，计算出的无创心输出量（CO）为16。¹使用阿魏酸的磁共振血管造影显示，CO为14 L / min，左脸颊，左颅骨顶点和右下侧颈的高流量血管病变（图2）。

图2. 妊娠27 wk时的磁共振血管造影时间分辨系列，显示左检查（绿色箭头），左头皮（红色箭头）和右肩（粉红色箭头）高流量血管畸形。3个帧显示了从动脉阶段到晚期静脉阶段的增强过程。

该患者被诊断为高输出心力衰竭，最初在住院和门诊均接受静脉和口服利尿剂治疗3周。尽管利尿，她仍发展为进行性心力衰竭，随后用氰基丙烯酸丁酯胶和聚乙烯醇颗粒栓塞了脸颊和颅骨顶点血管畸形，通过血管造影术导致流量减少了约50％（图3）。术后症状改善。手术后经胸超声心动图显示计算出的无创CO为11.9 L / min。栓塞后第5天，她出现了血小板减少症和急性肾损伤。考虑到先兆子痫，她在第30周和第1天进行了剖宫产，以解决心力衰竭症状。双胞胎A和B的出生体重分别为1300和1700 g。双胞胎A出生时患有尿道下裂和小室间隔缺损。分娩后立即经食管超声心动图检查显示无创CO的计算值为9.3 L / min。该患者因口服利尿剂出院。产后2周她的面部红肿完全消退（图1）。在撰写本文时，这对双胞胎已经23周大了，表现很好。

图3. 血管畸形和周围血管的血管造影。A，栓塞前：左颈外动脉血管造影显示，主要由浅颞动脉的肥大分支提供的覆盖在左顶头皮上的高血管腮红。B，栓塞后：左颈外血管造影显示血管畸形的混浊减少。肥厚的颞浅动脉分支已被阻塞。

在这里，我们描述了在多胎妊娠的情况下继发于血管畸形的继发性高输出心力衰竭的病例。CM在头和脖子上表现为红色斑点，在0.3％的新生儿中发现。²罕见的是，CM与高流量AVM相关，称为常染色体显性遗传疾病，称为CM-AVM综合征。³患者的表现，病变外观和皮肤CM（母亲，姐姐和兄弟）的家族史指出了CM-AVM综合征的临床诊断。这种疾病经常与RASA1和EPHB4中的功能丧失突变相关，并具有较高的渗透性和可变的表达能力。^4–6该患者仍在考虑进行基因检测。

AVM对激素敏感，并会随着荷尔蒙的变化而增加其大小和流量。结果，它们可能在青春期或怀孕期间增大。^3,7,8与妊娠相关的心血管变化会增加血管畸形者发生心力衰竭的风险。在头三个月中，由于全身血管扩张，女性的外周血管阻力下降。¹心率和中风量增加，导致CO升高。这种升高在单胎妊娠中可达到正常值的145％，在双胎妊娠中可达到7至8 L / min。^1,9患者多胎妊娠增加的雌激素和孕酮可能极大地促进了她现有CM-AVM的体积和流量的增加。

妊娠中的血管畸形通常通过保守的观察和等待策略来处理。鉴于患者的临床恶化，进行了AVM的栓塞治疗。据我们所知，这是妊娠期CM-AVM栓塞的首次报道。尽管术后动静脉分流明显减少，症状轻度改善，但患者仍出现子痫前期叠加的体征，最终需要早产。产后AVM的消退证实了其激素敏感性。

由于先天性血管畸形而导致的急性失代偿性高输出心力衰竭在妊娠中很少见，并且可能带来重大的管理挑战。保守医学治疗失败后，血管内栓塞可能在此类疾病的治疗中起作用。子痫前期的术后发展需要在本报告中紧急分娩，但强调了这种情况下固有的复杂性。

Hogeling博士是由Celgene赞助的一项研究的首席研究员。其他作者报告没有冲突。

* Srivastava博士和Vyas博士为这项工作做出了同样的贡献。

有关披露，请参阅第3页。