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The vulnerable right ventricle: Recurrent, transient right ventricular failure on a background of systemic sclerosis and previous anthracycline exposure
International Journal of Cardiology 178 (2015) 223–225
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
The vulnerable right ventricle: Recurrent, transient right ventricular failure on a background of systemic sclerosis and previous anthracycline exposure☆,☆☆ Rong Bing, Christopher Naoum, Leonard Kritharides ⁎ Concord Repatriation General Hospital, Hospital Road, Concord, NSW, Australia
a r t i c l e
i n f o
Article history: Received 8 October 2014 Accepted 19 October 2014 Available online 24 October 2014 Keywords: Right ventricle Systemic sclerosis Anthracycline
A 51-year-old woman with limited systemic sclerosis (SSc) was diagnosed with small lymphocytic lymphoma. There was no cardiac history. A gated heart pool scan showed normal biventricular size and contractility. The patient underwent six months of chemotherapy, including doxorubicin, which induced partial remission. One month after completing chemotherapy the patient was admitted with pneumonia. Within two days the patient developed signs of right heart failure including sinus tachycardia, elevated jugular venous pressure, a right ventricular (RV) parasternal heave and gallop and peripheral oedema. Transthoracic echocardiography (TTE) demonstrated a normal left ventricle (LV) but a grossly dilated RV with impaired contractility (Fig. 1A–B). The right ventricular–right atrial (RV–RA) pressure gradient was mildly elevated (38 mm Hg). Tricuspid annular plane systolic excursion (TAPSE) was 1.2 cm. CT pulmonary angiography (CTPA) excluded pulmonary embolism. Treatment with antibiotics, fluid restriction and diuretics resulted in resolution of sepsis and heart failure. A repeat TTE one week later showed normalisation of RV size and contractility. During routine follow-up at six months the patient reported exertional dyspnoea. Treadmill stress echocardiography demonstrated normal RV function at rest (Fig. 1C–D) and on exertion but elevated peak ☆ Conflicts of interest: none. ☆☆ Disclosures: none. ⁎ Corresponding author at: Department of Cardiology, Level 3 West, Concord Repatriation General Hospital, Hospital Rd, Concord, New South Wales 2139, Australia. E-mail addresses: [email protected] (R. Bing), [email protected] (C. Naoum), [email protected] (L. Kritharides).
http://dx.doi.org/10.1016/j.ijcard.2014.10.078 0167-5273/© 2014 Elsevier Ireland Ltd. All rights reserved.
exercise pulmonary pressures (RV–RA gradient 31 mm Hg at rest, 63 mm Hg on exercise). Right heart catheterisation with straight leg raising demonstrated exercise-induced pulmonary arterial hypertension (PAH) (mean pulmonary artery pressure 15 mm Hg at rest, 33 mm Hg on exercise; pulmonary vascular resistance 2.24 Woods units at rest, 7.29 Woods units on exercise). Pulmonary capillary wedge pressure was normal (mean 14 mm Hg). 6-Minute walk test (6MWT) distance was 400 m. As SSc can be associated with myocardial fibrosis a cardiac MRI was performed. This demonstrated a mildly dilated RV with normal systolic function and focal delayed gadolinium enhancement of the RV insertion point (Fig. 2A). Subsequent serial TTEs during follow-up confirmed preserved RV contractility. Five years later the patient was again admitted with pneumonia and developed right heart failure two days after admission. TTE (Fig. 2B–C, Video 1) demonstrated severe RV dilatation and dysfunction; RV–RA gradient was 40 mm Hg. CTPA excluded pulmonary embolism and identified bilateral consolidation. The patient required inotropic support. Respiratory viral swabs confirmed viral pneumonia. Ten days later the patient had improved; repeat echocardiography once more demonstrated normalisation of RV size and contractility (TAPSE 2.1 cm). The patient now receives monthly immunoglobulin infusions for acquired immunodeficiency and has remained well. The most recent TTE, 18 months after the second admission, demonstrated normal biventricular size and contractility. This case highlights a vulnerable RV with a propensity for lifethreatening decompensation under systemic stress despite normal baseline echocardiographic systolic function. Our patient has SSc with exercise-induced PAH, prior exposure to anthracycline chemotherapy and subtle RV changes on MRI, all of which may be contributors. PAH on its own is an insufficient explanation for these episodes. Patients with otherwise normal RV function do not develop severe right heart failure in response to the pulmonary pressures exhibited here. The septic milieu is critical and twice resulted in severe decompensation whereas the physiological demands of stress testing did not cause RV dysfunction despite elevated pulmonary pressures. Cardiac involvement in SSc is well described and can include myocardial and pulmonary arterial disease [1]. It is associated with poorer prognosis [2]. Myocardial fibrosis has been reported in 70–80% of cases at autopsy since the 1960s, and there is evidence that focal microvascular ischaemia due to abnormal vasoreactivity may be the
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R. Bing et al. / International Journal of Cardiology 178 (2015) 223–225
Fig. 1. A–B: Parasternal short axis and apical 4-chamber views on TTE showing severe RV dilatation with flattening of the interventricular septum as indicated by arrows. C–D: Identical views showing normalisation of RV size.
underlying aetiology [3]. Cardiac MRI has an increasing role in assessing subclinical myocardial involvement. Fibrosis can be seen on delayed enhancement cardiac MRI and is present in up to 66% of patients in small series, with predominant LV involvement [4]. However, a proportion of patients may have RV systolic dysfunction which is not clinically apparent [5]. Anthracycline-induced cardiomyopathy has been described since the 1970s. The most recognised sequela is LV dysfunction but early radionucleotide studies and more recent MRI data have demonstrated significant RV involvement [6]. In our case, identifying the propensity for acute, life-threatening RV dysfunction unearthed a number of potential underlying pathologies and permitted immediate recognition of her syndrome on the second presentation. Although the predominant contributing pathology is not
clear, the RV here is clearly vulnerable and prone to acute decompensation. It is important to recognise that echocardiographically normal resting RV function may belie an abnormal substrate that is unmasked in a potentially overwhelming fashion under acute systemic stress. Supplementary data to this article can be found online at http://dx. doi.org/10.1016/j.ijcard.2014.10.078.
References [1] D.L. Janosik, T.G. Osborn, T.L. Moore, D.G. Shah, R.G. Kenney, J. Zuckner, Heart disease in systemic sclerosis, Semin. Arthritis Rheum. 19 (3) (1989) 191–200. [2] C. Ferri, G. Valentini, F. Cozzi, M. Sebastiani, C. Michelassi, G. La Montagna, et al., Systemic sclerosis: demographic, clinical, and serologic features and survival in 1,012 Italian patients, Medicine 81 (2) (2002) 139–153.
Fig. 2. A: Short axis view on cardiac MRI showing late gadolinium enhancement of the RV insertion point. B–C: Parasternal short axis and apical 4-chamber views on TTE showing severe RV dilatation with flattening of the interventricular septum as indicated by arrows.
R. Bing et al. / International Journal of Cardiology 178 (2015) 223–225 [3] Y. Allanore, C. Meune, Primary myocardial involvement in systemic sclerosis: evidence for a microvascular origin, Clin. Exp. Rheumatol. 28 (5 Suppl. 62) (2010) S48–S53. [4] G.E. Tzelepis, N.L. Kelekis, S.C. Plastiras, P. Mitseas, N. Economopoulos, C. Kampolis, et al., Pattern and distribution of myocardial fibrosis in systemic sclerosis: a delayed enhanced magnetic resonance imaging study, Arthritis Rheum. 56 (11) (2007) 3827–3836.
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[5] C. Meune, Y. Allanore, J.Y. Devaux, O. Dessault, D. Duboc, S. Weber, et al., High prevalence of right ventricular systolic dysfunction in early systemic sclerosis, J. Rheumatol. 31 (10) (2004) 1941–1945. [6] S. Grover, D.P. Leong, A. Chakrabarty, L. Joerg, D. Kotasek, K. Cheong, et al., Left and right ventricular effects of anthracycline and trastuzumab chemotherapy: a prospective study using novel cardiac imaging and biochemical markers, Int. J. Cardiol. 168 (6) (2013) 5465–5467.
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
The vulnerable right ventricle: Recurrent, transient right ventricular failure on a background of systemic sclerosis and previous anthracycline exposure☆,☆☆ Rong Bing, Christopher Naoum, Leonard Kritharides ⁎ Concord Repatriation General Hospital, Hospital Road, Concord, NSW, Australia
a r t i c l e
i n f o
Article history: Received 8 October 2014 Accepted 19 October 2014 Available online 24 October 2014 Keywords: Right ventricle Systemic sclerosis Anthracycline
A 51-year-old woman with limited systemic sclerosis (SSc) was diagnosed with small lymphocytic lymphoma. There was no cardiac history. A gated heart pool scan showed normal biventricular size and contractility. The patient underwent six months of chemotherapy, including doxorubicin, which induced partial remission. One month after completing chemotherapy the patient was admitted with pneumonia. Within two days the patient developed signs of right heart failure including sinus tachycardia, elevated jugular venous pressure, a right ventricular (RV) parasternal heave and gallop and peripheral oedema. Transthoracic echocardiography (TTE) demonstrated a normal left ventricle (LV) but a grossly dilated RV with impaired contractility (Fig. 1A–B). The right ventricular–right atrial (RV–RA) pressure gradient was mildly elevated (38 mm Hg). Tricuspid annular plane systolic excursion (TAPSE) was 1.2 cm. CT pulmonary angiography (CTPA) excluded pulmonary embolism. Treatment with antibiotics, fluid restriction and diuretics resulted in resolution of sepsis and heart failure. A repeat TTE one week later showed normalisation of RV size and contractility. During routine follow-up at six months the patient reported exertional dyspnoea. Treadmill stress echocardiography demonstrated normal RV function at rest (Fig. 1C–D) and on exertion but elevated peak ☆ Conflicts of interest: none. ☆☆ Disclosures: none. ⁎ Corresponding author at: Department of Cardiology, Level 3 West, Concord Repatriation General Hospital, Hospital Rd, Concord, New South Wales 2139, Australia. E-mail addresses: [email protected] (R. Bing), [email protected] (C. Naoum), [email protected] (L. Kritharides).
http://dx.doi.org/10.1016/j.ijcard.2014.10.078 0167-5273/© 2014 Elsevier Ireland Ltd. All rights reserved.
exercise pulmonary pressures (RV–RA gradient 31 mm Hg at rest, 63 mm Hg on exercise). Right heart catheterisation with straight leg raising demonstrated exercise-induced pulmonary arterial hypertension (PAH) (mean pulmonary artery pressure 15 mm Hg at rest, 33 mm Hg on exercise; pulmonary vascular resistance 2.24 Woods units at rest, 7.29 Woods units on exercise). Pulmonary capillary wedge pressure was normal (mean 14 mm Hg). 6-Minute walk test (6MWT) distance was 400 m. As SSc can be associated with myocardial fibrosis a cardiac MRI was performed. This demonstrated a mildly dilated RV with normal systolic function and focal delayed gadolinium enhancement of the RV insertion point (Fig. 2A). Subsequent serial TTEs during follow-up confirmed preserved RV contractility. Five years later the patient was again admitted with pneumonia and developed right heart failure two days after admission. TTE (Fig. 2B–C, Video 1) demonstrated severe RV dilatation and dysfunction; RV–RA gradient was 40 mm Hg. CTPA excluded pulmonary embolism and identified bilateral consolidation. The patient required inotropic support. Respiratory viral swabs confirmed viral pneumonia. Ten days later the patient had improved; repeat echocardiography once more demonstrated normalisation of RV size and contractility (TAPSE 2.1 cm). The patient now receives monthly immunoglobulin infusions for acquired immunodeficiency and has remained well. The most recent TTE, 18 months after the second admission, demonstrated normal biventricular size and contractility. This case highlights a vulnerable RV with a propensity for lifethreatening decompensation under systemic stress despite normal baseline echocardiographic systolic function. Our patient has SSc with exercise-induced PAH, prior exposure to anthracycline chemotherapy and subtle RV changes on MRI, all of which may be contributors. PAH on its own is an insufficient explanation for these episodes. Patients with otherwise normal RV function do not develop severe right heart failure in response to the pulmonary pressures exhibited here. The septic milieu is critical and twice resulted in severe decompensation whereas the physiological demands of stress testing did not cause RV dysfunction despite elevated pulmonary pressures. Cardiac involvement in SSc is well described and can include myocardial and pulmonary arterial disease [1]. It is associated with poorer prognosis [2]. Myocardial fibrosis has been reported in 70–80% of cases at autopsy since the 1960s, and there is evidence that focal microvascular ischaemia due to abnormal vasoreactivity may be the
224
R. Bing et al. / International Journal of Cardiology 178 (2015) 223–225
Fig. 1. A–B: Parasternal short axis and apical 4-chamber views on TTE showing severe RV dilatation with flattening of the interventricular septum as indicated by arrows. C–D: Identical views showing normalisation of RV size.
underlying aetiology [3]. Cardiac MRI has an increasing role in assessing subclinical myocardial involvement. Fibrosis can be seen on delayed enhancement cardiac MRI and is present in up to 66% of patients in small series, with predominant LV involvement [4]. However, a proportion of patients may have RV systolic dysfunction which is not clinically apparent [5]. Anthracycline-induced cardiomyopathy has been described since the 1970s. The most recognised sequela is LV dysfunction but early radionucleotide studies and more recent MRI data have demonstrated significant RV involvement [6]. In our case, identifying the propensity for acute, life-threatening RV dysfunction unearthed a number of potential underlying pathologies and permitted immediate recognition of her syndrome on the second presentation. Although the predominant contributing pathology is not
clear, the RV here is clearly vulnerable and prone to acute decompensation. It is important to recognise that echocardiographically normal resting RV function may belie an abnormal substrate that is unmasked in a potentially overwhelming fashion under acute systemic stress. Supplementary data to this article can be found online at http://dx. doi.org/10.1016/j.ijcard.2014.10.078.
References [1] D.L. Janosik, T.G. Osborn, T.L. Moore, D.G. Shah, R.G. Kenney, J. Zuckner, Heart disease in systemic sclerosis, Semin. Arthritis Rheum. 19 (3) (1989) 191–200. [2] C. Ferri, G. Valentini, F. Cozzi, M. Sebastiani, C. Michelassi, G. La Montagna, et al., Systemic sclerosis: demographic, clinical, and serologic features and survival in 1,012 Italian patients, Medicine 81 (2) (2002) 139–153.
Fig. 2. A: Short axis view on cardiac MRI showing late gadolinium enhancement of the RV insertion point. B–C: Parasternal short axis and apical 4-chamber views on TTE showing severe RV dilatation with flattening of the interventricular septum as indicated by arrows.
R. Bing et al. / International Journal of Cardiology 178 (2015) 223–225 [3] Y. Allanore, C. Meune, Primary myocardial involvement in systemic sclerosis: evidence for a microvascular origin, Clin. Exp. Rheumatol. 28 (5 Suppl. 62) (2010) S48–S53. [4] G.E. Tzelepis, N.L. Kelekis, S.C. Plastiras, P. Mitseas, N. Economopoulos, C. Kampolis, et al., Pattern and distribution of myocardial fibrosis in systemic sclerosis: a delayed enhanced magnetic resonance imaging study, Arthritis Rheum. 56 (11) (2007) 3827–3836.
225
[5] C. Meune, Y. Allanore, J.Y. Devaux, O. Dessault, D. Duboc, S. Weber, et al., High prevalence of right ventricular systolic dysfunction in early systemic sclerosis, J. Rheumatol. 31 (10) (2004) 1941–1945. [6] S. Grover, D.P. Leong, A. Chakrabarty, L. Joerg, D. Kotasek, K. Cheong, et al., Left and right ventricular effects of anthracycline and trastuzumab chemotherapy: a prospective study using novel cardiac imaging and biochemical markers, Int. J. Cardiol. 168 (6) (2013) 5465–5467.