Spontaneous Cardiac Tamponade in a Patient with Severe Hemophilia A: A Case Report

Q: What is the clinical evidence from this study?

Study design: Case Report. Population: Severe hemophilia A with factor VIII inhibitors complicated by spontaneous hemorrhagic pericardial effusion and cardiac tamponade (n=1). Intervention: Ultrasound-guided pericardiocentesis with recombinant factor VIIa, emicizumab, and fresh frozen plasma. Primary outcome: Resolution of cardiac tamponade and hemodynamic recovery.

Q: What does this research mean for the field?

Timely factor-supported ultrasound-guided pericardiocentesis can safely and effectively resolve spontaneous cardiac tamponade in patients with severe hemophilia A complicated by factor VIII inhibitors. Novelty: ClaimNovelty.INCREMENTAL. Consensus alignment: ConsensusAlignment.NEUTRAL.

Teenu Singh; Sachin Wali; Shreyas Hanumantrao Gutte; Afzal Azim; Banani Poddar

doi:10.5811/cpcem.64682

October 20, 2026Clinical Practice and Cases in Emergency MedicineOpen Access

Spontaneous Cardiac Tamponade in a Patient with Severe Hemophilia A: A Case Report

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Key result

Factor-supported ultrasound-guided pericardiocentesis successfully resolves spontaneous cardiac tamponade in severe hemophilia A.

n=1

Why the study?

Hemophilia A is an X-linked recessive bleeding disorder characterized by factor VIII deficiency in which hemorrhage can be life-threatening when major organ systems are involved.

Key Points

To describe the diagnosis and emergency management of spontaneous hemorrhagic cardiac tamponade in an adolescent with severe hemophilia A and factor VIII inhibitors.
Clinical evaluation and management of a 13-year-old boy with severe hemophilia A and low-titer factor VIII inhibitors presenting with septic arthritis, refractory shock, and tachyarrhythmia.
Bedside point-of-care ultrasound (POCUS) examination to differentiate shock etiology.
Hemostatic rescue using recombinant factor VIIa, fresh frozen plasma, and emicizumab followed by ultrasound-guided pericardiocentesis.
POCUS identified a large circumferential hemorrhagic pericardial effusion with right ventricular diastolic collapse, confirming cardiac tamponade physiology over suspected sepsis-related myocarditis.
Ultrasound-guided pericardiocentesis performed after hemostatic rescue with recombinant factor VIIa resulted in rapid hemodynamic recovery without reported procedural bleeding complications.

Study Design

Type

Case Report (n=1)

Multicenter

No

Structured PICO

P

Population

A 13-year-old boy in India with severe hemophilia A and factor VIII inhibitors who developed septic arthritis and spontaneous cardiac tamponade due to a 3.5 cm hemorrhagic pericardial effusion, followed through hospital day 28.

I

Intervention

Hemostatic rescue therapy with recombinant factor VIIa (250 µg/kg), emicizumab (90 µg initially, then 90 µg daily), and intravenous fresh frozen plasma (15 mL/kg), followed 30 minutes later by ultrasound-guided subxiphoid pericardiocentesis with an 8-French pericardial drain left in place for 7 days, along with packed red blood cells (7 units), fresh frozen plasma (7 units), pooled cryoprecipitate (6 units), and factor VIII (1,000 U twice daily).

O

Outcome

Hemodynamic recovery and resolution of cardiac tamponade and pericardial effusion.hard clinical

This case report demonstrates that spontaneous hemorrhagic cardiac tamponade in a patient with severe hemophilia A and factor VIII inhibitors can be rapidly diagnosed with bedside point-of-care ultrasound and safely treated with ultrasound-guided pericardiocentesis following hemostatic support with recombinant factor VIIa, fresh frozen plasma, and emicizumab.

Limitations

Inability to definitively determine the exact precipitating mechanism of the hemorrhagic pericardial effusion due to concurrent severe hemophilia A with FVIII inhibitors, sepsis-associated coagulopathy, and mild hepatic dysfunction.
Single-patient case report design.
Inability to definitively determine the exact precipitating mechanism of the hemorrhagic pericardial effusion (whether primarily severe hemophilia A with factor VIII inhibitors or exacerbated by concurrent sepsis-associated coagulopathy and mild hepatic dysfunction).

Implication

Supports factor-supported pericardiocentesis for tamponade in pediatric hemophilia A; hypothesis-generating pending larger studies.

Abstract

Introduction: Hemophilia A is an X-linked recessive bleeding disorder characterized by deficiency of factor VIII. The clinical features of this disease are hemorrhage and can be life-threatening when major organ systems are involved. Case Report: We report the case of a 13-year-old boy with severe hemophilia A and factor VIII inhibitors who presented with fever and septic arthritis, later developing refractory shock with tachyarrhythmia. Despite initial suspicion of sepsis-related myocarditis, point-of-care ultrasound (POCUS) revealed massive hemorrhagic pericardial effusion with tamponade physiology. Recombinant factor VIIa was transfused as hemostatic rescue therapy; the patient was subsequently started on fresh frozen plasma and emicizumab. Ultrasound-guided pericardiocentesis resulted in rapid hemodynamic recovery. Conclusion: This case highlights the importance of considering obstructive shock in unstable hemophilia patients and demonstrates that timely POCUS-based diagnosis and factor-supported pericardiocentesis can be lifesaving.

Discussion

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Cite This Study

Singh et al. (2026) conducted a case report in Severe hemophilia A with factor VIII inhibitors complicated by spontaneous hemorrhagic pericardial effusion and cardiac tamponade (n=1). Ultrasound-guided pericardiocentesis with recombinant factor VIIa, emicizumab, and fresh frozen plasma was evaluated on Resolution of cardiac tamponade and hemodynamic recovery. Factor-supported ultrasound-guided pericardiocentesis drained 900 mL of blood on day 1 and resolved spontaneous cardiac tamponade in a 13-year-old boy with severe hemophilia A and FVIII inhibitors.

synapsesocial.com/papers/6ae28e94cbadf70daf6c4349 https://doi.org/10.5811/cpcem.64682

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Spontaneous Cardiac Tamponade in a Patient with Severe Hemophilia A: A Case Report

Key Points

Study Design

Structured PICO

Limitations

Implication

Abstract

Discussion

Cite This Study

Spontaneous Cardiac Tamponade in a Patient with Severe Hemophilia A: A Case Report

Key Points

Study Design

Structured PICO

Limitations

Implication

Abstract

Discussion

Cite This Study

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