A case of a giant cell myocarditis that developed massive left ventricular thrombus during percutaneous cardiopulmonary support
© The Author(s) 2016
Received: 3 August 2016
Accepted: 18 November 2016
Published: 29 November 2016
Giant cell myocarditis, characterized by infiltration of multinucleated giant cells in the myocardium, is a rare type of myocarditis. It often progresses rapidly into fulminant heart failure and indicates a poor prognosis. When a patient with giant cell myocarditis develops into severe myocarditis presenting with a cardiogenic shock, we should use a percutaneous cardiopulmonary support (PCPS), which could occur complications. We experienced a patient with giant cell myocarditis, who developed left ventricular thrombus formations during the circulation support therapy with PCPS for cardiogenic shock.
A 60-year-old man who developed a cardiogenic shock was transferred to our hospital. After the admission, inotropic agents were increased and an intra-aortic balloon pumping was started. But these therapies did not improve his hemodynamic status. He was placed PCPS. Then, he underwent endomyocardial biopsy and was diagnosed with giant cell myocarditis. On the next morning, he developed complete atrioventricular block, and subsequently, thrombus formations occurred in his left ventricular outlet tract and Valsalva sinus despite an anticoagulant therapy. Thereafter, we intensified the anticoagulant therapy to prevent further thrombus formation, but he developed an intracranial hemorrhage. He did not recover from heart failure and died 16 days after the admission.
We present a patient with giant cell myocarditis who developed widespread thrombosis in the left ventricle during the circulatory support with PCPS, despite anticoagulant therapy. In this case, decreased left myocardial contractility caused by giant cell myocarditis and increased left ventricular afterload by the retrograde perfusion from the PCPS induced the thrombotic tendency and congestion in the left ventricle. In addition, he developed complete atrioventricular block, which reduced the left ventricular ejection and enhanced the thrombus formation. Because patients with giant cell myocarditis have a low probability of spontaneous recovery, heart transplantation or ventricular assist device implantation may be required for circulatory support. We should establish mechanical circulatory support rapidly to improve the prognosis of patients with giant cell myocarditis. Moreover, a ventricular assist device, which can prevent both ventricular congestion and retrograde blood flow, might be suitable to prevent complications as this case.
KeywordsGiant cell myocarditis Ventricular thrombus Percutaneous cardiopulmonary support system
Giant cell myocarditis, characterized by infiltration of multinucleated giant cells in the myocardium, is a rare type of myocarditis. Fulminant myocarditis can occur during its clinical course. When it occurs, prognosis is extremely poor due to acute heart failure [1–4]. Herein, we report a patient with giant cell myocarditis who developed massive left ventricular thrombus during the circulatory support with percutaneous cardiopulmonary support (PCPS) for the treatment of progressive heart failure.
The blood examination on admission and 1 and 10 days after the admission
1 day after the admission (after PCPS placement)
10 days after the admission (after intensive anticoagulant therapy)
White blood cell counts (/μL)
Platelet counts (×104/μL)
Total bilirubin (mg/dL)
Aspartate transaminase (IU/L)
Alanine aminotransferase (IU/L)
Lactate dehydrogenase (IU/L)
Blood urea nitrogen (mg/dL)
C-reactive protein (mg/dL)
Creatine kinase (IU/L)
Creatine kinase-MB (IU/L)
Troponin T (ng/dL)
Brain natriuretic peptide (pg/mL)
International normalized ratio of prothrombin time
Activated partial thromboplastin time (s)
Activated clotting time (s)
Hemodynamic status of the patient on admission, before and after the initiation of PCPS, after the onset of complete atrioventricular block, and after ventricular pacing
Before initiation of PCPS
Initiation of PCPS
Onset of complete AVB
After ventricular pacing
Face mask, 5 L/min of oxygen
Face mask, 6 L/min of oxygen
APRV, FIO2 = 0.6, P high = 20 cm H2O, T high = 9.5 s, P low = 0 cm H2O, T low = 0.5 s
Same as on the left
APRV, FIO2 = 0.6, P high = 15 cm H2O, T high = 5.5 s, P low = 0 cm H2O, T low = 0.5 s
Output of PCPS (L/min)
The sum of PCPS blood flow and his own cardiac output (L/min)
Total gas flow of oxygenator (L/min)
At the initiation of PCPS, the target value of PCPS blood flow was set at 3.0 L/min (rotation speed of 3000 rpm), and the FIO2 and total gas flow of the membrane oxygenator were set at 0.6 and 1 L/min, respectively.
And the intravenous continuous infusion of heparin (10,000 IU/day) was started in order to maintain his ACT within the range of 150 to 200 s. After the PCPS blood flow reached to 3.0 L/min, the SvO2 increased to 66% and the total blood flow, which was the sum of PCPS blood flow and his own cardiac output, increased from 3.0 to 5.3 L/min (Table 2).
We increased the PCPS blood flow up to 3.5 L/min and total gas flow up to 4.0 L/min, which lead to success of CO2 excretion, but his hemodynamic status remained unstable. After that, we started temporary cardiac pacing to treat complete atrioventricular block, which restored his left ventricular contractility, opened the aortic valve, and subsequently washed out the thrombus.
Thereafter, for the purpose of preventing further thrombus formation, anticoagulant therapy was intensified (20,000 IU/day of heparin) to maintain ACT within the range of 200 to 240 s. Ten days after the admission, his ACT was markedly prolonged up to 273 s (Table 2) and he presented with an anisocoria. Therefore, we performed a computed tomography (CT) scan of the brain, which revealed the intracranial hemorrhage. He did not recover from the heart failure and died 16 days after the admission.
Giant cell myocarditis is characterized by infiltration of multinucleated giant cells in the myocardium and distinguished from cardiac sarcoidosis by the absence of non-caseous necrosis. Cardiac sarcoidosis is generally associated with extracardiac lesions and progresses slowly, whereas giant cell myocarditis develops and progresses to heart failure rapidly, which results in poor prognosis [1–3]. An accurate data about the morbidity of giant cell myocarditis has not been shown because the pathological diagnosis of the disease is difficult. However, approximately 100 cases were reported despite the rarity of this myocarditis type . Mechanical circulatory support, such as IABP and/or PCPS as well as inotropes, is required for the treatment of acute giant cell myocarditis because the disease often progresses to fulminant myocarditis. Some reports showed the efficacy of immunosuppressive therapy for giant cell myocarditis, which suggested a relationship between giant cell myocarditis and autoimmune reaction [5–7]. In addition, a multicenter study of immunosuppressive therapy for giant cell myocarditis showed the prolonged median transplant-free survival from 3 to 12.3 months . However, some studies also showed that few patients recovered from heart failure and weaned from mechanical circulatory support [7–9]. The patients of that study eventually required heart transplantation or circulatory support, such as ventricular assist device (VAD) for destination therapy. In Japan, VAD is a practical option for the rescue treatment of chronic giant cell myocarditis because only 40 heart transplantations are performed per year, and the mean waiting period for transplantation is 636 days. We need to transfer the patients to the hospital capable for left VAD (LVAD) surgery, which is limited in Japan, if needed for LVAD placement.
We could not save this patient because of the disease progression and the complications, including the thrombosis due to the congestion of the left ventricle and intracranial hemorrhage due to the intensive anticoagulant therapy to prevent thrombosis.
Before the onset of the atrioventricular block, the patient had 2.3 L/min of the pulmonary circulation with 2.4 L/min of minute volume of ventilation and 3.0 L/min of the PCPS blood flow with 1 L/min of gas flow of the artificial lung. In this condition, the main pathway of his CO2 elimination was thought to be the pulmonary circulation. After the onset of the atrioventricular block, the pulmonary circulation decreased to far less than 1.0 L/min because of the loss of the cardiac output while 3.0 L/min of the PCPS blood flow was maintained. Hence, the total lung blood flow decreased to approximately 3.0 L/min, and the artificial lung ought to excrete almost whole CO2. However, the gas flow of the artificial lung remained 1.0 L/min, which might be too little to excrete sufficient amount of the CO2. Thereafter, the PaCO2 of the patient rose from 34.8 to 62.5 mmHg.
To our knowledge, no guideline is available for treatment of intraventricular thrombosis in PCPS patients. The treatment choices are thrombectomy and intensive anticoagulant therapy [10–12]. Because of the unstable circulatory status of the patient, intensive systemic anticoagulant therapy and temporary cardiac pacing, which could recover the ventricular contractility and ejection, were the possible choices. Therefore, we started temporary cardiac pacing, which recovered the left ventricular contractility, opened the aortic valve, and washed out the thrombi. Additionally, the efficacy of local thrombolysis in intraventricular thrombosis was recently reported. This technique might be effective in this case .
The washed out thrombi formed at the onset of the complete atrioventricular block could cause cerebral infarctions, and the intensified coagulation therapy might deteriorate the cerebral infarctions into the intracranial hemorrhage. At the onset of complete atrioventricular block, we did not perform a CT scan of the brain or awaken him from the deep sedation due to his unstable circulatory status. But 10 days after the admission, we performed a CT scan of the brain at the risk of circulatory failure because the patient presented with an anisocoria. However, we could not identify the true pathogenesis of the intracranial hemorrhage.
Different types of myocarditis show different clinical courses and prognoses and require different treatments. Therefore, EMB, identifying the histological type of myocarditis, should be performed as the patient’s condition permits. In this case, multidrug immunosuppressive therapy was started after the histological diagnosis of giant cell myocarditis. However, as previously described, heart transplantation or VAD may be required for a definitive therapy within months or years. LVAD, withdrawing the blood from the left ventricular apex and returning the blood to the ascending aorta, can establish physiological perfusion and reduce left ventricular volume, whereas peripheral PCPS perfuses aorta retrogradely, increases left ventricular afterload, and might induce left ventricular distension. In addition, LVAD can cause less bleeding complications than PCPS because LVAD requires weaker anticoagulation than PCPS . If we could place an LVAD on the patient at the diagnosis of giant cell myocarditis, complications such as thrombosis and bleeding might be prevented, which might result in smooth bridge to heart transplantation. To improve the prognosis of myocarditis, full knowledge of the clinical course of myocarditis, precise diagnosis, transfer to an advanced medical center where LVAD placement can be performed and rapid establishment of adequate mechanical circulatory support should be required.
We present a patient with giant cell myocarditis who developed widespread thrombosis in the left ventricle during PCPS support. Retrograde perfusion by PCPS and complete atrioventricular block promoted left ventricular distension, reduced left ventricular ejection, and caused widespread thrombosis. In the circulation management of patients with giant cell myocarditis, we should rapidly establish physiological mechanical circulatory support.
Activated clotting time
- CO2 :
Continuous positive airway pressure
- FIO2 :
Fraction of inspired oxygen
Intra-aortic balloon pumping
Left ventricular assist device
- PaCO2 :
Arterial carbon dioxide tension
- PaO2 :
Arterial oxygen tension
Pulmonary arterial pressure
Percutaneous cardiopulmonary support
- SvO2 :
Mixed venous oxygenation saturation
Ventricular assist device
YT, KT, and TO were anesthesiologists attending to the intensive care. YT drafted the manuscript. YE, HT, and MY supervised the treatment and helped to draft the manuscript. All authors read and approved the final manuscript.
YT, KT, and TO are M.D. and Stuff Anesthesiologists of the Department of Anesthesiology, Tohoku University Hospital. HT is M.D., PhD., and a Lecturer of the Department of Anesthesiology, Tohoku University Hospital. YE is M.D., PhD., and the Associate Director of the Department of Surgical Center and Supply, Sterilization, Tohoku University Hospital. MY is M.D., PhD., and a Professor of Anesthesiology and Perioperative Medicine, Tohoku University School of Medicine.
The authors declare that they have no competing interests.
Consent for publication
Written informed consent was obtained from the patient’s family for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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