SPC365

Primary cardiac angiosarcoma presenting as cardiac tamponade in pregnancy

Session: Prémio Melhor Caso Clínico
Speaker: Luana Alves

Congress: CPC 2026
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Session Type: Sessão de Prémios
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Authors: Luana Raquel Lorenzo Alves; Mariana Vasconcelos; Roberto Pinto; Catarina Costa; Carla Sousa; Elson Salgueiro; Teresa Pinho; Daniel Seabra; Carlos Xavier Resende; Roberto Roncon; Sandra Amorim; Rui André Rodrigues
<p style="text-align:justify"><span style="font-size:medium"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="color:#000000"><strong><span style="color:black">Background:</span></strong>&nbsp;<span style="color:black">Primary cardiac angiosarcoma is a rare and aggressive malignancy, frequently diagnosed late due to non-specific clinical presentation. Its occurrence during pregnancy is exceptional and poses major diagnostic and therapeutic challenges, particularly when presenting as an acute cardiovascular emergency.</span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:medium"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="color:#000000"><strong><span style="color:black">Case summary:</span></strong>&nbsp;<span style="color:black">A 39-year-old woman at </span><span style="color:black">32 + 6 weeks </span><span style="color:black">of gestation presented to the emergency department with pleuritic chest pain </span><span style="color:black">and presyncope. </span><span style="color:black">On admission, she was </span><span style="color:black">hypotensive</span><span style="font-size:13.5pt"><span style="color:black"> and </span></span><span style="color:black">tachycardic. Transthoracic echocardiography (TTE) revealed a large circumferential pericardial effusion with right ventricular diastolic collapse, consistent with cardiac tamponade. Urgent echocardiography-guided pericardiocentesis via the apical approach drained 950 mL of haemorrhagic fluid, with immediate haemodynamic stabilisation. Pericardial fluid cytology, infectious, inflammatory and autoimmune investigations were negative.</span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:medium"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="color:#000000"><span style="color:black">Four days later, recurrence of</span> <span style="color:black">symptoms p</span><span style="color:black">rompted repeat TTE, which identified a right atrial mass. Given the suspicion of malignancy and advanced gestational age, foetal lung maturation was completed, and caesarean delivery was performed at 33 + 6 weeks, with favourable maternal and neonatal outcomes. Multimodality imaging, including cardiac magnetic resonance imaging and <sup>18</sup>F-FDG PET-CT, demonstrated a large</span> <span style="color:black">invasive heterogeneous and </span><span style="color:black">hypermetabolic right atrial mass without distant metastases.</span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:medium"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="color:#000000"><span style="color:black">Histological diagnosis obtained by TOE-guided biopsy confirmed primary cardiac angiosarcoma. After multidisciplinary discussion, surgical resection followed by adjuvant chemotherapy was pursued. Extensive tumour excision with </span><span style="color:black">interatrial septum, inferior vena cava, and right atrium </span><span style="color:black">reconstruction and tricuspid valvuloplasty was performed. The postoperative course was complicated by transient atrioventricular block, managed with temporary ventricular pacing support. Adjuvant paclitaxel (175 mg/m&sup2; every 3 weeks) was administered over four months.</span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:medium"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="color:#000000"><span style="color:black">The patient remained free of recurrence for approximately nine months after diagnosis. At ten months, surveillance imaging identified a new hypermetabolic left pericardiophrenic lesion, consistent with suspected locoregional metastatic disease. Given its isolated nature, resection via a minimally invasive robotic-assisted thoracic approach was proposed. At the most recent follow-up, both mother and child remain stable, and the patient is awaiting surgical management.</span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:medium"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="color:#000000"><strong><span style="color:black">Conclusion:</span></strong>&nbsp;<span style="color:black">This case illustrates the diagnostic and therapeutic complexity of primary cardiac angiosarcoma presenting during pregnancy and highlights the importance of multimodality imaging and coordinated multidisciplinary management approach </span><span style="color:black">to the outcome.</span></span></span></span></p>