International Journal of Cardiology 184 (2015) 268–269
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
Primary prophylactic anticoagulation is mandatory if noncompaction is associated with atrial fibrillation or heart failure Josef Finsterer a,⁎, Claudia Stöllberger b a b
Krankenanstalt Rudolfstiftung, Vienna, Austria 2nd Medical Department with Cardiology and Intensive Care Medicine, Krankenanstalt Rudolfstiftung, Vienna, Austria
a r t i c l e
i n f o
Article history: Received 3 January 2015 Accepted 21 February 2015 Available online 24 February 2015 Keywords: Non-compaction Unclassified cardiomyopathy Stroke Embolism Metabolic disease Left ventricular hypertrabeculation
Whether patients with left ventricular hypertrabeculation/ noncompaction (LVHT) require primary prophylaxis with oral anticoagulation (OAC) to prevent stroke/embolism is unsolved [1]. However, if LVHT is associated with atrial fibrillation (AFIB)/atrial flutter (AFLU), or systolic dysfunction or both, OAC for primary prophylaxis of embolism is mandatory, as illustrated by the following case. The patient is a 51 year-old Caucasian male, with a height of 182 cm and weight of 90 kg, who had developed a right posterior borderzone infarction resulting in transient left lower quadrantanopia for 3 h with complete remission. His history was noteworthy for a “big heart” and palpitations since adolescence, resection of lipomas from the abdomen and the lower extremity, an episode with vertigo, double vision and headache at age 46 years, occasional cramping of the intrinsic foot muscles once a month with left-sided predominance, varicositas, hyperlipidemia, and obstructive sleep-apnea syndrome. Work-up for vertigo at age 46 years revealed AFLU, paroxysmal AFIB, some wall irregularities on coronary angiography, dilation of the left ventricle, hypokinesia of the anterior wall, akinesia of the septum, systolic dysfunction (ejection fraction: 40%), and LVHT. He was smoking 10 cigarettes per day until the stroke. He was non-alcoholic. His family history was positive for dilated cardiomyopathy (dCMP) (father), recurrent embolic stroke (father), hypoacusis (son), and visual impairment (daughter). He had taken ⁎ Corresponding author at: Postfach 20, 1180 Vienna, Austria. E-mail address: fifi[email protected] (J. Finsterer).
http://dx.doi.org/10.1016/j.ijcard.2015.02.041 0167-5273/© 2015 Elsevier Ireland Ltd. All rights reserved.
acetyl-salicylic acid and atorvastatin for one year after detection of LVHT but had given up taking any medication since 4 years. Work-up after stroke at age 51 years revealed hypoacusis on neurologic exam exclusively, transient hyper-CKemia (maximal value of 212 U/l, n: − 170 U/l), elevated glutamate pyruvate transaminase (70 U/l, n:0–50 U/l), a proBNP of 8123 ng/l (n: 0–84 ng/l), hyperuricemia (8.4 mg/dl, n: 3.5–7.0 mg/dl), transient renal insufficiency, and latent hypothyroidism. X-ray of the lung showed pulmonary congestion grade 2. Echocardiography showed severely reduced systolic function with an ejection fraction of 30% and LVHT of the apex and the lateral wall (Fig. 1). His medication on dismissal included apixaban (10 mg/d), bisoprolol (5 mg/d), atorvastatin (40 mg/d), allopurinol (100 mg/d), ramipril (5 mg/d), and spironolactone (25 mg/d). Ischemic stroke occurs in up to 15% of the patients with LVHT [2–4]. Stroke in LVHT patients may have various causes in addition to LVHT, such as AFIB/AFLU, heart failure, arterial hypertension, or a coagulopathy [3]. If LVHT is associated with AFIB thrombi may not only be derived from the intertrabecular spaces but also from the left atrium or left atrial appendage. However, there is no consensus under which conditions LVHT patients should be anticoagulated for primary prophylaxis of stroke/embolism. The highest degree of consensus exists for LVHT and AFIB respectively AFLU [3,5]. Concerning the association of LVHT and systolic dysfunction there are some indications that these patients profit from early OAC [6]. The presented patient should have been anticoagulated as soon as LVHT, AF and systolic dysfunction had been diagnosed at age 46 years. However, whether OAC would have prevented the current stroke in case it had been started already earlier remains speculative. Which type of OAC should be applied in patients with LVHT and AFIB/AFLU or LVHT and systolic dysfunction is unsolved [5]. Since all registration trials for direct oral anticoagulants (DOACs) have been carried out in patients with non-valvular AFIB without LVHT, patients with LVHT and AFIB should not receive DOACs for anticoagulation as long as their effectivity and safety in this indication have been proven by a randomised, placebo-controlled, crossover trial. Implantation of an implantable cardioverter defibrillator (ICD) was considered in the presented patient but the decision was postponed seeing if the EF would improve above 35% under heart failure therapy [6]. Whether LVHT was acquired [7] or congenital in the presented patient remains speculative but given the positive family history for dCMP it is quite likely that LVHT was congenital. Both children were invited for echocardiography but did not follow the invitation so far.
J. Finsterer, C. Stöllberger / International Journal of Cardiology 184 (2015) 268–269
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common metabolic defect is the family history also showing dCMP in the father and hypoacusis in the patient's son. Overall, this case shows that LVHT patients with AFIB/AFLU carry an increased risk to develop cardiac embolism. Patients with LVHT and AFIB/AFLU or LVHT and systolic dysfunction should receive OAC for primary prophylaxis as soon as these combinations of abnormalities are diagnosed. OAC in LVHT should be carried out with vitamin-K antagonists but not with DOACs as long as valid studies showing effectivity of DOACs in this indication are available. Conflict of interest The authors report no relationships that could be construed as a conflict of interest. References Fig. 1. Echocardiographic apical 5 chamber view showing the left ventricular noncompaction/hypertrabeculation in the left ventricular apex and lateral wall.
Whether the father of the presented patient had LVHT in addition to dCMP remains unknown, since no information is available any longer about the cardiac findings in this patient. An indication that he also had developed LVHT is his history of stroke. Unfortunately he had died already in 1975 that's why no medical records were available any longer. Late gadolinium enhancement, which is frequently associated with LVHT, has not been observed in the presented patient. Late gadolinium enhancement may indicate myocardial fibrosis or microvascular compromise. The patient did not undergo cardiac MRI since demonstration of thrombi within the intertrabecular spaces or the left atrium or left atrial appendage remains uncertain because thrombi can be easily mixed up with flow phenomena. Whether there was an underlying common cause of LVHT and the other abnormalities remains speculative but LVHT is frequently associated with metabolic disease [8–11] and metabolic disease is frequently associated with lipoma formation, hypoacusis, AFIB, dCMP, hyperuricemia, renal insufficiency, and hypothyroidism. A further indication of a
[1] Z.Z. Song, Cerebral infarction and right ventricular noncompaction, Int. J. Cardiol. 148 (2011) e45–e46. [2] N.L. Bhatia, A.J. Tajik, S. Wilansky, D.E. Steidley, F. Mookadam, Isolated noncompaction of the left ventricular myocardium in adults: a systematic overview, J. Card. Fail. 17 (2011) 771–778. [3] C. Stöllberger, G. Blazek, C. Dobias, A. Hanafin, C. Wegner, J. Finsterer, Frequency of stroke and embolism in left ventricular hypertrabeculation/noncompaction, Am. J. Cardiol. 108 (2011) 1021–1023. [4] J. Finsterer, C. Stollberger, G. Mölzer, M. Winkler-Dworak, G. Blazek, Cerebrovascular events in left ventricular hypertrabeculation/noncompaction with and without myopathy, Int. J. Cardiol. 130 (2008) 344–348. [5] C. Stöllberger, J. Finsterer, New oral anticoagulants for stroke prevention in left ventricular hypertrabeculation/noncompaction? Int. J. Cardiol. 168 (2013) 2910–2911. [6] M. Sinkovec, M. Kozelj, T. Podnar, Familial biventricular myocardial noncompaction associated with Ebstein's malformation, Int. J. Cardiol. 102 (2005) 297–302. [7] J. Finsterer, C. Stöllberger, Acquired, familial noncompaction and eccentric hypertrophic cardiomyopathy associated with metabolic myopathy and epilepsy, Int. J. Cardiol. 160 (2012) 73–75. [8] J. Finsterer, C. Stöllberger, Disabling myopathy with chorea and noncompaction, Int. J. Cardiol. 181C (2014) 32–34. [9] J. Finsterer, C. Stöllberger, Noncompaction in alleged motor neuron disease suggests myopathy, Int. J. Cardiol. 177 (2014) 639–640. [10] J. Finsterer, C. Stöllberger, Severe rhabdomyolysis after MIHA-bodytec® electrostimulation with previous mild hyper-CK-emia and noncompaction, Int. J. Cardiol. 180C (2014) 100–102. [11] J. Finsterer, C. Stöllberger, M. Motamen, Left ventricular hypertrabeculation/ noncompaction in an Eritrean war invalid with neuromuscular disease, Int. J. Cardiol. 174 (2014) e59–e61.
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
Primary prophylactic anticoagulation is mandatory if noncompaction is associated with atrial fibrillation or heart failure Josef Finsterer a,⁎, Claudia Stöllberger b a b
Krankenanstalt Rudolfstiftung, Vienna, Austria 2nd Medical Department with Cardiology and Intensive Care Medicine, Krankenanstalt Rudolfstiftung, Vienna, Austria
a r t i c l e
i n f o
Article history: Received 3 January 2015 Accepted 21 February 2015 Available online 24 February 2015 Keywords: Non-compaction Unclassified cardiomyopathy Stroke Embolism Metabolic disease Left ventricular hypertrabeculation
Whether patients with left ventricular hypertrabeculation/ noncompaction (LVHT) require primary prophylaxis with oral anticoagulation (OAC) to prevent stroke/embolism is unsolved [1]. However, if LVHT is associated with atrial fibrillation (AFIB)/atrial flutter (AFLU), or systolic dysfunction or both, OAC for primary prophylaxis of embolism is mandatory, as illustrated by the following case. The patient is a 51 year-old Caucasian male, with a height of 182 cm and weight of 90 kg, who had developed a right posterior borderzone infarction resulting in transient left lower quadrantanopia for 3 h with complete remission. His history was noteworthy for a “big heart” and palpitations since adolescence, resection of lipomas from the abdomen and the lower extremity, an episode with vertigo, double vision and headache at age 46 years, occasional cramping of the intrinsic foot muscles once a month with left-sided predominance, varicositas, hyperlipidemia, and obstructive sleep-apnea syndrome. Work-up for vertigo at age 46 years revealed AFLU, paroxysmal AFIB, some wall irregularities on coronary angiography, dilation of the left ventricle, hypokinesia of the anterior wall, akinesia of the septum, systolic dysfunction (ejection fraction: 40%), and LVHT. He was smoking 10 cigarettes per day until the stroke. He was non-alcoholic. His family history was positive for dilated cardiomyopathy (dCMP) (father), recurrent embolic stroke (father), hypoacusis (son), and visual impairment (daughter). He had taken ⁎ Corresponding author at: Postfach 20, 1180 Vienna, Austria. E-mail address: fifi[email protected] (J. Finsterer).
http://dx.doi.org/10.1016/j.ijcard.2015.02.041 0167-5273/© 2015 Elsevier Ireland Ltd. All rights reserved.
acetyl-salicylic acid and atorvastatin for one year after detection of LVHT but had given up taking any medication since 4 years. Work-up after stroke at age 51 years revealed hypoacusis on neurologic exam exclusively, transient hyper-CKemia (maximal value of 212 U/l, n: − 170 U/l), elevated glutamate pyruvate transaminase (70 U/l, n:0–50 U/l), a proBNP of 8123 ng/l (n: 0–84 ng/l), hyperuricemia (8.4 mg/dl, n: 3.5–7.0 mg/dl), transient renal insufficiency, and latent hypothyroidism. X-ray of the lung showed pulmonary congestion grade 2. Echocardiography showed severely reduced systolic function with an ejection fraction of 30% and LVHT of the apex and the lateral wall (Fig. 1). His medication on dismissal included apixaban (10 mg/d), bisoprolol (5 mg/d), atorvastatin (40 mg/d), allopurinol (100 mg/d), ramipril (5 mg/d), and spironolactone (25 mg/d). Ischemic stroke occurs in up to 15% of the patients with LVHT [2–4]. Stroke in LVHT patients may have various causes in addition to LVHT, such as AFIB/AFLU, heart failure, arterial hypertension, or a coagulopathy [3]. If LVHT is associated with AFIB thrombi may not only be derived from the intertrabecular spaces but also from the left atrium or left atrial appendage. However, there is no consensus under which conditions LVHT patients should be anticoagulated for primary prophylaxis of stroke/embolism. The highest degree of consensus exists for LVHT and AFIB respectively AFLU [3,5]. Concerning the association of LVHT and systolic dysfunction there are some indications that these patients profit from early OAC [6]. The presented patient should have been anticoagulated as soon as LVHT, AF and systolic dysfunction had been diagnosed at age 46 years. However, whether OAC would have prevented the current stroke in case it had been started already earlier remains speculative. Which type of OAC should be applied in patients with LVHT and AFIB/AFLU or LVHT and systolic dysfunction is unsolved [5]. Since all registration trials for direct oral anticoagulants (DOACs) have been carried out in patients with non-valvular AFIB without LVHT, patients with LVHT and AFIB should not receive DOACs for anticoagulation as long as their effectivity and safety in this indication have been proven by a randomised, placebo-controlled, crossover trial. Implantation of an implantable cardioverter defibrillator (ICD) was considered in the presented patient but the decision was postponed seeing if the EF would improve above 35% under heart failure therapy [6]. Whether LVHT was acquired [7] or congenital in the presented patient remains speculative but given the positive family history for dCMP it is quite likely that LVHT was congenital. Both children were invited for echocardiography but did not follow the invitation so far.
J. Finsterer, C. Stöllberger / International Journal of Cardiology 184 (2015) 268–269
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common metabolic defect is the family history also showing dCMP in the father and hypoacusis in the patient's son. Overall, this case shows that LVHT patients with AFIB/AFLU carry an increased risk to develop cardiac embolism. Patients with LVHT and AFIB/AFLU or LVHT and systolic dysfunction should receive OAC for primary prophylaxis as soon as these combinations of abnormalities are diagnosed. OAC in LVHT should be carried out with vitamin-K antagonists but not with DOACs as long as valid studies showing effectivity of DOACs in this indication are available. Conflict of interest The authors report no relationships that could be construed as a conflict of interest. References Fig. 1. Echocardiographic apical 5 chamber view showing the left ventricular noncompaction/hypertrabeculation in the left ventricular apex and lateral wall.
Whether the father of the presented patient had LVHT in addition to dCMP remains unknown, since no information is available any longer about the cardiac findings in this patient. An indication that he also had developed LVHT is his history of stroke. Unfortunately he had died already in 1975 that's why no medical records were available any longer. Late gadolinium enhancement, which is frequently associated with LVHT, has not been observed in the presented patient. Late gadolinium enhancement may indicate myocardial fibrosis or microvascular compromise. The patient did not undergo cardiac MRI since demonstration of thrombi within the intertrabecular spaces or the left atrium or left atrial appendage remains uncertain because thrombi can be easily mixed up with flow phenomena. Whether there was an underlying common cause of LVHT and the other abnormalities remains speculative but LVHT is frequently associated with metabolic disease [8–11] and metabolic disease is frequently associated with lipoma formation, hypoacusis, AFIB, dCMP, hyperuricemia, renal insufficiency, and hypothyroidism. A further indication of a
[1] Z.Z. Song, Cerebral infarction and right ventricular noncompaction, Int. J. Cardiol. 148 (2011) e45–e46. [2] N.L. Bhatia, A.J. Tajik, S. Wilansky, D.E. Steidley, F. Mookadam, Isolated noncompaction of the left ventricular myocardium in adults: a systematic overview, J. Card. Fail. 17 (2011) 771–778. [3] C. Stöllberger, G. Blazek, C. Dobias, A. Hanafin, C. Wegner, J. Finsterer, Frequency of stroke and embolism in left ventricular hypertrabeculation/noncompaction, Am. J. Cardiol. 108 (2011) 1021–1023. [4] J. Finsterer, C. Stollberger, G. Mölzer, M. Winkler-Dworak, G. Blazek, Cerebrovascular events in left ventricular hypertrabeculation/noncompaction with and without myopathy, Int. J. Cardiol. 130 (2008) 344–348. [5] C. Stöllberger, J. Finsterer, New oral anticoagulants for stroke prevention in left ventricular hypertrabeculation/noncompaction? Int. J. Cardiol. 168 (2013) 2910–2911. [6] M. Sinkovec, M. Kozelj, T. Podnar, Familial biventricular myocardial noncompaction associated with Ebstein's malformation, Int. J. Cardiol. 102 (2005) 297–302. [7] J. Finsterer, C. Stöllberger, Acquired, familial noncompaction and eccentric hypertrophic cardiomyopathy associated with metabolic myopathy and epilepsy, Int. J. Cardiol. 160 (2012) 73–75. [8] J. Finsterer, C. Stöllberger, Disabling myopathy with chorea and noncompaction, Int. J. Cardiol. 181C (2014) 32–34. [9] J. Finsterer, C. Stöllberger, Noncompaction in alleged motor neuron disease suggests myopathy, Int. J. Cardiol. 177 (2014) 639–640. [10] J. Finsterer, C. Stöllberger, Severe rhabdomyolysis after MIHA-bodytec® electrostimulation with previous mild hyper-CK-emia and noncompaction, Int. J. Cardiol. 180C (2014) 100–102. [11] J. Finsterer, C. Stöllberger, M. Motamen, Left ventricular hypertrabeculation/ noncompaction in an Eritrean war invalid with neuromuscular disease, Int. J. Cardiol. 174 (2014) e59–e61.
