International Journal of Cardiology 177 (2014) e47–e48
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
An unexpected post-partum fulminant heart failure Jenna Berger ⁎, Julia Chabot ⁎, Shawn Pun, Jean-Philippe Pelletier, Line Vautour, Nadia Giannetti a r t i c l e
i n f o
Article history: Received 10 September 2014 Accepted 21 September 2014 Available online 28 September 2014 Keywords: Heart failure Pheochromocytoma Catecholamine cardiomyopathy Impella
A 37 year-old female presented to the emergency department 2 days postpartum, due to a headache and an ongoing, back pain, radiating to her epigastrium. Her presenting vital signs were: blood pressure of 107/83 mm Hg, heart rate 66 beats per minute (regular), and oxygen saturation of 99%. She was non-toxic looking with an unremarkable physical examination. Her ECG demonstrated a sinus rhythm. Her first troponin I was 0.15 μg/L (normal 0–0.06), D dimers were negative at 0.62 ng/mL, WBC was 15.8 × 109/L, and hemoglobin, creatinine, platelets and liver enzymes were normal. Three hours later, she became diaphoretic, her blood pressure rose to 190 mm Hg systolic, and she experienced an episode of a stable, non-sustained wide complex tachycardia. She received 10 mg of intravenous labetalol due to the possibility of an aortic dissection, and 5 g of magnesium sulfate intravenously given the concern of preeclampsia. She returned to sinus rhythm and normalized her blood pressure. A bedside echocardiogram demonstrated basal hypokinesis with an ejection fraction (EF) of 35–40%. Our main differentials were viral myocarditis, postpartum cardiomyopathy, and pericarditis. However, given the elevated blood pressure and chest pain, the patient was sent for a Computed Tomography scan, which was negative for an aortic dissection. She progressed to cardiogenic shock and required intubation and vasopressors. Her repeat Chest X-ray demonstrated remarkable bilateral pulmonary edema. A repeat bedside echocardiogram demonstrated an LVEF of 15–20%, and the troponin rose to 5.99 μg/L. Adding onto our differential were coronary dissection and the unlikely possibility of an acute coronary syndrome.
⁎ Corresponding authors. McGill University. E-mail address: [email protected] (J. Berger).
http://dx.doi.org/10.1016/j.ijcard.2014.09.082 0167-5273/© 2014 Elsevier Ireland Ltd. All rights reserved.
The cardiac catheterization demonstrated an LVEF of 15%, with contractility mainly in the apex, and a left ventricular end diastolic pressure of 45 mm Hg. The right dominant coronary tree was angiographically normal. A Swan–Ganz catheter was inserted via the right jugular vein, demonstrating elevated right-heart and wedge pressures (prominent V waves were also noted on the wedge tracing). Due to the deteriorating cardiac parameters, milrinone was added, and an Impella cVAD, a percutaneous ventricular assist device, was inserted (see Fig. 1). Upon reviewing the imaging, the CT revealed an enhancing right adrenal nodule measuring up to 26 mm in diameter, suspicious for a pheochromocytoma. In light of this finding, levophed was discontinued to prevent a catecholamine surge. The patients' overall status rapidly improved. A repeat echocardiogram 48 h later demonstrated a LVEF of 35%, and the Impella cVAD was removed the subsequent day. Post removal, an echocardiogram demonstrated a LVEF of 55% and the patient was discharged home on phenoxybenzamine prior to surgical intervention. The pathology confirmed a pheochromocytoma. A pheochromocytoma is a rare neuroendocrine tumor that secretes high levels of catecholamines. This leads to the typical clinical manifestations of tachycardia, paroxysms of hypertension, headaches, and diaphoresis [1,2]. The diagnosis can be very challenging as those features are not always present, and some patients may remain completely asymptomatic [3]. The diagnosis was particularly challenging in our patient, as she had never had hypertensive episodes documented during her pregnancy. However, it is important to consider that during pregnancy, a supine position may cause physical compression from the gravid uterus leading to paradoxical supine hypertension with a normal blood pressure either in a standing or sitting position [5,6]. Multiple cardiovascular complications may occur secondary to a pheochromocytoma including cardiovascular shock, myocardial infarction, arrhythmias, and cardiomyopathy [7,8]. There have been a few proposed mechanisms which include a catecholamine-induced cardiomyopathy by the direct toxic effect from norepinephrine and other oxidized products, downregulation of beta receptors and a reduction in myofibrils, alpha 1 adrenergic receptor stimulation (leading to vasospasm and subsequent hypoxia of the coronary arteries) [1–3,9]. Additionally, the increased inotropy, chronotropy and afterload may contribute to ischemia and tachyarrhythmias, which may lead to a rate related cardiomyopathy [4]. Our patient's fulminant heart failure may have been exacerbated by the labetalol received. Initiation of beta blockade prior to alpha blockade in patients with a pheochromocytoma may precipitate hemodynamic instability [2,10]. This is believed to occur secondary to a non-selective
e48
J. Berger et al. / International Journal of Cardiology 177 (2014) e47–e48
Fig. 1. Positioning of the Impella cVAD in the left ventricle and aorta.
beta-blockade, leading to the absence of beta-2 receptor-mediated vasodilatation, leaving an unopposed vasoconstrictive effect of alpha receptors [10]. This cascade can lead to an increase in afterload, arterial hypertension, which could lead to myocardial dysfunction and pulmonary edema. Tako-tsubo cardiomyopathy has been described in pheochromocytoma cardiomyopathy. However, similarly to what was found in our patient, it also has been described that a reverse Tako-tsubo can occur. It is believed that this could possibly be due to “regional differences in adrenergic sensitivity or innervation” of the myocardium [11,12]. Our patient was stabilized with an impella cVAD, which is a noninvasive left ventricular assist device that is designed to provide hemodynamic support in patients with cardiogenic shock and poor left ventricular function. Post cardiac angiography, our patient's cardiac parameters were a cardiac index of 1.7 L/min/m2, a cardiac output of 2.8 L/min, a central venous pressure of 28 mm Hg, a pulmonary artery pressure of 35/25 mm Hg, a mixed venous saturation of 60.1%, and an arterial lactate of 2.7. She was initially started on dobutamine, however, due to its beta-adrenergic effects, this was discontinued. A few hours later, the patient's cardiac parameters were worsening and it was decided to insert an Impella cVAD. It provided an average of 3.3 L/min of assistance to the left ventricle. Her CI improved to 2.4 L/min/m2. The following day, her EF had improved to 35%, with a CI of 3.4 L/min/m2. The Impella cVAD was removed 2 days after its insertion. Her EF had improved to 45–50%. Its sole complication occurred on day 1 of its insertion. It had displaced and led to low-grade hemolysis. It was repositioned later that same day. A catecholamine-induced cardiomyopathy can potentially be reversible therefore a diagnosis of a pheochromocytoma should be entertained in patients presenting with acute decompensated heart failure, without a clear etiology. Up until now, there has been no published case of a patient in heart failure, secondary to a pheochromocytoma that
had been stabilized with an Impella cVAD. We hope that our case report presents this as a viable option when medical management is limited. Conflict of interest The authors report no relationships that could be construed as a conflict of interest. References [1] Vahdat A, Vahdat O, Chandraratna P. Pheochromocytoma presenting as reversible acute cardiomyopathy. Int J Cardiol 2006;108(3):395–6. [2] Whitelaw BC, Prague JK, Mustafa OG, Schulte KM, Hopkins PA, Gilbert JA, et al. Phaeochromocytoma crisis. Clin Endocrinol 2014;80(1):13–22. [3] Prejbisz A, Lenders JW, Eisenhofer G, Januszewicz A. Cardiovascular manifestations of phaeochromocytoma. J Hypertens 2011;29(11):2049–60. [4] Wu GY, Doshi AA, Haas GJ. Pheochromocytoma induced cardiogenic shock with rapid recovery of ventricular function. Eur J Heart Fail 2007;9(2):212–4. [5] Biggar M, Lennard T. Systematic review of phaeochromocytoma in pregnancy. Br J Surg 2013;100(2):182–90. [6] Keely E. Endocrine causes of hypertension in pregnancy—when to start looking for zebras. Semin Perinatol 1998;22(6):471–84. [7] Galetta F, Franzoni F, Bernini G, Poupak F, Carpi A, Cini G, et al. Cardiovascular complications in patients with pheochromocytoma: a mini-review. Biomed Pharmacother 2010;64(7):505–9. [8] Mobine HR, Baker AB, Wang L, Wakimoto H, Jacobsen KC, Seidman CE, et al. Pheochromocytoma-induced cardiomyopathy is modulated by the synergistic effects of cell-secreted factors. Circulation Heart Fail 2009;2(2):121–8. [9] Mulla CM, Marik PE. Pheochromocytoma presenting as acute decompensated heart failure reversed with medical therapy. BMJ Case Reports; 2012 [2012]. [10] Sibal L, Jovanovic A, Agarwal S, Peaston R, James R, Lennard T, et al. Phaeochromocytomas presenting as acute crises after beta blockade therapy. Clin Endocrinol 2006;65(2):186–90. [11] Naderi N, Amin A, Setayesh A, Pouraliakbar H, Mozaffari K, Maleki M. Pheochromocytoma-induced reverse tako-tsubo with rapid recovery of left ventricular function. Cardiol J 2012;19(5):527–31. [12] Sanchez-Recalde A, Costero O, Oliver JM, Iborra C, Ruiz E, Sobrino JA. Pheochromocytoma-related cardiomyopathy inverted Takotsubo contractile pattern. Circulation 2006;113(17):e738–9.
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
An unexpected post-partum fulminant heart failure Jenna Berger ⁎, Julia Chabot ⁎, Shawn Pun, Jean-Philippe Pelletier, Line Vautour, Nadia Giannetti a r t i c l e
i n f o
Article history: Received 10 September 2014 Accepted 21 September 2014 Available online 28 September 2014 Keywords: Heart failure Pheochromocytoma Catecholamine cardiomyopathy Impella
A 37 year-old female presented to the emergency department 2 days postpartum, due to a headache and an ongoing, back pain, radiating to her epigastrium. Her presenting vital signs were: blood pressure of 107/83 mm Hg, heart rate 66 beats per minute (regular), and oxygen saturation of 99%. She was non-toxic looking with an unremarkable physical examination. Her ECG demonstrated a sinus rhythm. Her first troponin I was 0.15 μg/L (normal 0–0.06), D dimers were negative at 0.62 ng/mL, WBC was 15.8 × 109/L, and hemoglobin, creatinine, platelets and liver enzymes were normal. Three hours later, she became diaphoretic, her blood pressure rose to 190 mm Hg systolic, and she experienced an episode of a stable, non-sustained wide complex tachycardia. She received 10 mg of intravenous labetalol due to the possibility of an aortic dissection, and 5 g of magnesium sulfate intravenously given the concern of preeclampsia. She returned to sinus rhythm and normalized her blood pressure. A bedside echocardiogram demonstrated basal hypokinesis with an ejection fraction (EF) of 35–40%. Our main differentials were viral myocarditis, postpartum cardiomyopathy, and pericarditis. However, given the elevated blood pressure and chest pain, the patient was sent for a Computed Tomography scan, which was negative for an aortic dissection. She progressed to cardiogenic shock and required intubation and vasopressors. Her repeat Chest X-ray demonstrated remarkable bilateral pulmonary edema. A repeat bedside echocardiogram demonstrated an LVEF of 15–20%, and the troponin rose to 5.99 μg/L. Adding onto our differential were coronary dissection and the unlikely possibility of an acute coronary syndrome.
⁎ Corresponding authors. McGill University. E-mail address: [email protected] (J. Berger).
http://dx.doi.org/10.1016/j.ijcard.2014.09.082 0167-5273/© 2014 Elsevier Ireland Ltd. All rights reserved.
The cardiac catheterization demonstrated an LVEF of 15%, with contractility mainly in the apex, and a left ventricular end diastolic pressure of 45 mm Hg. The right dominant coronary tree was angiographically normal. A Swan–Ganz catheter was inserted via the right jugular vein, demonstrating elevated right-heart and wedge pressures (prominent V waves were also noted on the wedge tracing). Due to the deteriorating cardiac parameters, milrinone was added, and an Impella cVAD, a percutaneous ventricular assist device, was inserted (see Fig. 1). Upon reviewing the imaging, the CT revealed an enhancing right adrenal nodule measuring up to 26 mm in diameter, suspicious for a pheochromocytoma. In light of this finding, levophed was discontinued to prevent a catecholamine surge. The patients' overall status rapidly improved. A repeat echocardiogram 48 h later demonstrated a LVEF of 35%, and the Impella cVAD was removed the subsequent day. Post removal, an echocardiogram demonstrated a LVEF of 55% and the patient was discharged home on phenoxybenzamine prior to surgical intervention. The pathology confirmed a pheochromocytoma. A pheochromocytoma is a rare neuroendocrine tumor that secretes high levels of catecholamines. This leads to the typical clinical manifestations of tachycardia, paroxysms of hypertension, headaches, and diaphoresis [1,2]. The diagnosis can be very challenging as those features are not always present, and some patients may remain completely asymptomatic [3]. The diagnosis was particularly challenging in our patient, as she had never had hypertensive episodes documented during her pregnancy. However, it is important to consider that during pregnancy, a supine position may cause physical compression from the gravid uterus leading to paradoxical supine hypertension with a normal blood pressure either in a standing or sitting position [5,6]. Multiple cardiovascular complications may occur secondary to a pheochromocytoma including cardiovascular shock, myocardial infarction, arrhythmias, and cardiomyopathy [7,8]. There have been a few proposed mechanisms which include a catecholamine-induced cardiomyopathy by the direct toxic effect from norepinephrine and other oxidized products, downregulation of beta receptors and a reduction in myofibrils, alpha 1 adrenergic receptor stimulation (leading to vasospasm and subsequent hypoxia of the coronary arteries) [1–3,9]. Additionally, the increased inotropy, chronotropy and afterload may contribute to ischemia and tachyarrhythmias, which may lead to a rate related cardiomyopathy [4]. Our patient's fulminant heart failure may have been exacerbated by the labetalol received. Initiation of beta blockade prior to alpha blockade in patients with a pheochromocytoma may precipitate hemodynamic instability [2,10]. This is believed to occur secondary to a non-selective
e48
J. Berger et al. / International Journal of Cardiology 177 (2014) e47–e48
Fig. 1. Positioning of the Impella cVAD in the left ventricle and aorta.
beta-blockade, leading to the absence of beta-2 receptor-mediated vasodilatation, leaving an unopposed vasoconstrictive effect of alpha receptors [10]. This cascade can lead to an increase in afterload, arterial hypertension, which could lead to myocardial dysfunction and pulmonary edema. Tako-tsubo cardiomyopathy has been described in pheochromocytoma cardiomyopathy. However, similarly to what was found in our patient, it also has been described that a reverse Tako-tsubo can occur. It is believed that this could possibly be due to “regional differences in adrenergic sensitivity or innervation” of the myocardium [11,12]. Our patient was stabilized with an impella cVAD, which is a noninvasive left ventricular assist device that is designed to provide hemodynamic support in patients with cardiogenic shock and poor left ventricular function. Post cardiac angiography, our patient's cardiac parameters were a cardiac index of 1.7 L/min/m2, a cardiac output of 2.8 L/min, a central venous pressure of 28 mm Hg, a pulmonary artery pressure of 35/25 mm Hg, a mixed venous saturation of 60.1%, and an arterial lactate of 2.7. She was initially started on dobutamine, however, due to its beta-adrenergic effects, this was discontinued. A few hours later, the patient's cardiac parameters were worsening and it was decided to insert an Impella cVAD. It provided an average of 3.3 L/min of assistance to the left ventricle. Her CI improved to 2.4 L/min/m2. The following day, her EF had improved to 35%, with a CI of 3.4 L/min/m2. The Impella cVAD was removed 2 days after its insertion. Her EF had improved to 45–50%. Its sole complication occurred on day 1 of its insertion. It had displaced and led to low-grade hemolysis. It was repositioned later that same day. A catecholamine-induced cardiomyopathy can potentially be reversible therefore a diagnosis of a pheochromocytoma should be entertained in patients presenting with acute decompensated heart failure, without a clear etiology. Up until now, there has been no published case of a patient in heart failure, secondary to a pheochromocytoma that
had been stabilized with an Impella cVAD. We hope that our case report presents this as a viable option when medical management is limited. Conflict of interest The authors report no relationships that could be construed as a conflict of interest. References [1] Vahdat A, Vahdat O, Chandraratna P. Pheochromocytoma presenting as reversible acute cardiomyopathy. Int J Cardiol 2006;108(3):395–6. [2] Whitelaw BC, Prague JK, Mustafa OG, Schulte KM, Hopkins PA, Gilbert JA, et al. Phaeochromocytoma crisis. Clin Endocrinol 2014;80(1):13–22. [3] Prejbisz A, Lenders JW, Eisenhofer G, Januszewicz A. Cardiovascular manifestations of phaeochromocytoma. J Hypertens 2011;29(11):2049–60. [4] Wu GY, Doshi AA, Haas GJ. Pheochromocytoma induced cardiogenic shock with rapid recovery of ventricular function. Eur J Heart Fail 2007;9(2):212–4. [5] Biggar M, Lennard T. Systematic review of phaeochromocytoma in pregnancy. Br J Surg 2013;100(2):182–90. [6] Keely E. Endocrine causes of hypertension in pregnancy—when to start looking for zebras. Semin Perinatol 1998;22(6):471–84. [7] Galetta F, Franzoni F, Bernini G, Poupak F, Carpi A, Cini G, et al. Cardiovascular complications in patients with pheochromocytoma: a mini-review. Biomed Pharmacother 2010;64(7):505–9. [8] Mobine HR, Baker AB, Wang L, Wakimoto H, Jacobsen KC, Seidman CE, et al. Pheochromocytoma-induced cardiomyopathy is modulated by the synergistic effects of cell-secreted factors. Circulation Heart Fail 2009;2(2):121–8. [9] Mulla CM, Marik PE. Pheochromocytoma presenting as acute decompensated heart failure reversed with medical therapy. BMJ Case Reports; 2012 [2012]. [10] Sibal L, Jovanovic A, Agarwal S, Peaston R, James R, Lennard T, et al. Phaeochromocytomas presenting as acute crises after beta blockade therapy. Clin Endocrinol 2006;65(2):186–90. [11] Naderi N, Amin A, Setayesh A, Pouraliakbar H, Mozaffari K, Maleki M. Pheochromocytoma-induced reverse tako-tsubo with rapid recovery of left ventricular function. Cardiol J 2012;19(5):527–31. [12] Sanchez-Recalde A, Costero O, Oliver JM, Iborra C, Ruiz E, Sobrino JA. Pheochromocytoma-related cardiomyopathy inverted Takotsubo contractile pattern. Circulation 2006;113(17):e738–9.
