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Erschienen in: Current Cardiovascular Imaging Reports 6/2013

01.12.2013 | Echocardiography (T Buck, Section Editor)

Non-Compaction Cardiomyopathy: Underdiagnosed or Over Diagnosed?

verfasst von: Robert A. Quaife, Ernesto E. Salcedo, Eugene E. Wolfel

Erschienen in: Current Cardiovascular Imaging Reports | Ausgabe 6/2013

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Abstract

Left ventricular non-compaction (LVNC) is a morphologic description of the left ventricle that has genetic, phenotypic, and clinical heterogeneity. The clinical presentation varies from an incidental finding of LVNC in an asymptomatic patient to a patient with a cardiomyopathy with severe left ventricular systolic dysfunction. The diagnosis of LVNC in asymptomatic patients is primarily made by an echocardiogram that is performed either for screening of a first-degree relative of a patient with LVNC. There are no clinical symptoms that are specific for noncompaction. The ECG is abnormal in 87 % of patients with LVNC but there are no pathognomonic findings. Echocardiography is the most frequently used method to diagnose LVNC. The echocardiographic diagnostic criteria for LVNC are well established and it includes the demonstration of a thickened myocardium exhibiting a noncompacted endocardial layer and a compacted epicardial layer. The ratio of the systolic thickness of noncompacted to compacted layer is >2. CMR is well suited to evaluate the layers of myocardium and distinguish the regional characteristic of this disease by the 3-dimensional nature of the technology. This promising imaging modality provides both a high spatial resolution and a good contrast between trabeculations and blood pool. CT similar to CMR possesses inherent high resolution imaging characteristics. It too is well suited to measure the disparity of noncompacted to compacted myocardium. To minimize underdiagnosing or over diagnosing LVNC it is essential to have a sound understanding of the genetics, clinical characteristics and strengths and weaknesses of the imaging modalities used for the evaluation and management of patients with known or suspected LVNC. In this review paper we detail the genetic and clinical background of LVNC, and the added value of echocardiography, CMR and CT for the diagnosis or exclusion of LVNC.
Literatur
1.
Zurück zum Zitat •• Paterick TE, Tajik AJ. Left ventricular noncompaction: a diagnostically challenging cardiomyopathy. Circ J. 2012;76(7):1556–62. Recent review paper highlighting the uncertainties surrounding the diagnosis of LVNC because of a lack of a "perfect diagnostic tool," such as a reproducible genetic marker, the broad spectrum of overlapping cardiomyopathies and the fact that the demarcation between LVNC and normal phenotypic variations is often indistinct. The authors make the point that Echocardiography, used in routine clinical practice to identify the typical morphologic features of LVNC, can be overly sensitive and lack specificity with the presently defined measurements and ratios used to diagnose LVNC and that our future understanding of LVNC depends on an integration of cardiac morphology, physiology, pathophysiology and evolving genetics. •• Paterick TE, Tajik AJ. Left ventricular noncompaction: a diagnostically challenging cardiomyopathy. Circ J. 2012;76(7):1556–62. Recent review paper highlighting the uncertainties surrounding the diagnosis of LVNC because of a lack of a "perfect diagnostic tool," such as a reproducible genetic marker, the broad spectrum of overlapping cardiomyopathies and the fact that the demarcation between LVNC and normal phenotypic variations is often indistinct. The authors make the point that Echocardiography, used in routine clinical practice to identify the typical morphologic features of LVNC, can be overly sensitive and lack specificity with the presently defined measurements and ratios used to diagnose LVNC and that our future understanding of LVNC depends on an integration of cardiac morphology, physiology, pathophysiology and evolving genetics.
2.
Zurück zum Zitat Chrissoheris MP et al. Isolated noncompaction of the ventricular myocardium: contemporary diagnosis and management. Clin Cardiol. 2007;30(4):156–60.PubMedCrossRef Chrissoheris MP et al. Isolated noncompaction of the ventricular myocardium: contemporary diagnosis and management. Clin Cardiol. 2007;30(4):156–60.PubMedCrossRef
3.
Zurück zum Zitat Biagini E et al. Different types of cardiomyopathy associated with isolated ventricular noncompaction. Am J Cardiol. 2006;98(6):821–4.PubMedCrossRef Biagini E et al. Different types of cardiomyopathy associated with isolated ventricular noncompaction. Am J Cardiol. 2006;98(6):821–4.PubMedCrossRef
4.
Zurück zum Zitat Richardson P et al. Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of cardiomyopathies. Circulation. 1996;93(5):841–2.PubMedCrossRef Richardson P et al. Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of cardiomyopathies. Circulation. 1996;93(5):841–2.PubMedCrossRef
5.
Zurück zum Zitat Maron BJ et al. Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention. Circulation. 2006;113(14):1807–16.PubMedCrossRef Maron BJ et al. Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention. Circulation. 2006;113(14):1807–16.PubMedCrossRef
6.
Zurück zum Zitat Elliott P et al. Classification of the cardiomyopathies: a position statement from the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J. 2008;29(2):270–6.PubMedCrossRef Elliott P et al. Classification of the cardiomyopathies: a position statement from the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J. 2008;29(2):270–6.PubMedCrossRef
7.
Zurück zum Zitat Oechslin E, Jenni R. Left ventricular non-compaction revisited: a distinct phenotype with genetic heterogeneity? Eur Heart J. 2011;32(12):1446–56.PubMedCrossRef Oechslin E, Jenni R. Left ventricular non-compaction revisited: a distinct phenotype with genetic heterogeneity? Eur Heart J. 2011;32(12):1446–56.PubMedCrossRef
8.
Zurück zum Zitat • Kalayci A et al. Noncompaction cardiomyopathy. Is it more than noncompaction? Herz. 2013;38(2):216–8. In this paper the point is made that noncompaction cardiomyopathy resemble the reptile heart and that the defect in myocardial compaction and the frequently seen accompanying anomalies may share a common causative factor during embryogenesis. • Kalayci A et al. Noncompaction cardiomyopathy. Is it more than noncompaction? Herz. 2013;38(2):216–8. In this paper the point is made that noncompaction cardiomyopathy resemble the reptile heart and that the defect in myocardial compaction and the frequently seen accompanying anomalies may share a common causative factor during embryogenesis.
9.
Zurück zum Zitat Hoedemaekers YM et al. Cardiac beta-myosin heavy chain defects in two families with non-compaction cardiomyopathy: linking non-compaction to hypertrophic, restrictive, and dilated cardiomyopathies. Eur Heart J. 2007;28(22):2732–7.PubMedCrossRef Hoedemaekers YM et al. Cardiac beta-myosin heavy chain defects in two families with non-compaction cardiomyopathy: linking non-compaction to hypertrophic, restrictive, and dilated cardiomyopathies. Eur Heart J. 2007;28(22):2732–7.PubMedCrossRef
10.
Zurück zum Zitat Klaassen S et al. Mutations in sarcomere protein genes in left ventricular noncompaction. Circulation. 2008;117(22):2893–901.PubMedCrossRef Klaassen S et al. Mutations in sarcomere protein genes in left ventricular noncompaction. Circulation. 2008;117(22):2893–901.PubMedCrossRef
11.
Zurück zum Zitat Xing Y et al. Genetic analysis in patients with left ventricular noncompaction and evidence for genetic heterogeneity. Mol Genet Metab. 2006;88(1):71–7.PubMedCrossRef Xing Y et al. Genetic analysis in patients with left ventricular noncompaction and evidence for genetic heterogeneity. Mol Genet Metab. 2006;88(1):71–7.PubMedCrossRef
12.
Zurück zum Zitat Ichida F et al. Novel gene mutations in patients with left ventricular noncompaction or Barth syndrome. Circulation. 2001;103(9):1256–63.PubMedCrossRef Ichida F et al. Novel gene mutations in patients with left ventricular noncompaction or Barth syndrome. Circulation. 2001;103(9):1256–63.PubMedCrossRef
13.
Zurück zum Zitat Luedde M et al. Severe familial left ventricular non-compaction cardiomyopathy due to a novel troponin T (TNNT2) mutation. Cardiovasc Res. 2010;86(3):452–60.PubMedCrossRef Luedde M et al. Severe familial left ventricular non-compaction cardiomyopathy due to a novel troponin T (TNNT2) mutation. Cardiovasc Res. 2010;86(3):452–60.PubMedCrossRef
14.
Zurück zum Zitat Grant R. A unusual anomaly of the coronary vessels in the malformed heart of a child. Heart Fail Rev. 1926;13:273–83. Grant R. A unusual anomaly of the coronary vessels in the malformed heart of a child. Heart Fail Rev. 1926;13:273–83.
15.
Zurück zum Zitat Engberding R, Bender F. Identification of a rare congenital anomaly of the myocardium by two-dimensional echocardiography: persistence of isolated myocardial sinusoids. Am J Cardiol. 1984;53(11):1733–4.PubMedCrossRef Engberding R, Bender F. Identification of a rare congenital anomaly of the myocardium by two-dimensional echocardiography: persistence of isolated myocardial sinusoids. Am J Cardiol. 1984;53(11):1733–4.PubMedCrossRef
16.
Zurück zum Zitat Chin TK et al. Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation. 1990;82(2):507–13.PubMedCrossRef Chin TK et al. Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation. 1990;82(2):507–13.PubMedCrossRef
17.
Zurück zum Zitat Ritter M et al. Isolated noncompaction of the myocardium in adults. Mayo Clin Proc. 1997;72(1):26–31.PubMedCrossRef Ritter M et al. Isolated noncompaction of the myocardium in adults. Mayo Clin Proc. 1997;72(1):26–31.PubMedCrossRef
18.
Zurück zum Zitat Stanton C et al. Isolated left ventricular noncompaction syndrome. Am J Cardiol. 2009;104(8):1135–8.PubMedCrossRef Stanton C et al. Isolated left ventricular noncompaction syndrome. Am J Cardiol. 2009;104(8):1135–8.PubMedCrossRef
19.
Zurück zum Zitat Jenni R et al. Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart. 2001;86(6):666–71.PubMedCrossRef Jenni R et al. Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart. 2001;86(6):666–71.PubMedCrossRef
20.
Zurück zum Zitat Kovacevic-Preradovic T et al. Isolated left ventricular noncompaction as a cause for heart failure and heart transplantation: a single center experience. Cardiology. 2009;112(2):158–64.PubMedCrossRef Kovacevic-Preradovic T et al. Isolated left ventricular noncompaction as a cause for heart failure and heart transplantation: a single center experience. Cardiology. 2009;112(2):158–64.PubMedCrossRef
21.
Zurück zum Zitat Fazio G et al. The chronic heart failure is not so frequent in non-compaction. Eur Heart J. 2007;28(10):1269. author reply 1269-70.PubMedCrossRef Fazio G et al. The chronic heart failure is not so frequent in non-compaction. Eur Heart J. 2007;28(10):1269. author reply 1269-70.PubMedCrossRef
22.
Zurück zum Zitat Bhatia NL et al. Isolated noncompaction of the left ventricular myocardium in adults: a systematic overview. J Card Fail. 2011;17(9):771–8.PubMedCrossRef Bhatia NL et al. Isolated noncompaction of the left ventricular myocardium in adults: a systematic overview. J Card Fail. 2011;17(9):771–8.PubMedCrossRef
23.
Zurück zum Zitat Pitta S, Thatai D, Afonso L. Thromboembolic complications of left ventricular noncompaction: case report and brief review of the literature. J Clin Ultrasound. 2007;35(8):465–8.PubMedCrossRef Pitta S, Thatai D, Afonso L. Thromboembolic complications of left ventricular noncompaction: case report and brief review of the literature. J Clin Ultrasound. 2007;35(8):465–8.PubMedCrossRef
24.
Zurück zum Zitat Stollberger C, Finsterer J. Left ventricular hypertrabeculation/noncompaction and stroke or embolism. Cardiology. 2005;103(2):68–72.PubMedCrossRef Stollberger C, Finsterer J. Left ventricular hypertrabeculation/noncompaction and stroke or embolism. Cardiology. 2005;103(2):68–72.PubMedCrossRef
25.
Zurück zum Zitat Aras D et al. Clinical features of isolated ventricular noncompaction in adults long-term clinical course, echocardiographic properties, and predictors of left ventricular failure. J Card Fail. 2006;12(9):726–33.PubMedCrossRef Aras D et al. Clinical features of isolated ventricular noncompaction in adults long-term clinical course, echocardiographic properties, and predictors of left ventricular failure. J Card Fail. 2006;12(9):726–33.PubMedCrossRef
26.
Zurück zum Zitat Lofiego C et al. Wide spectrum of presentation and variable outcomes of isolated left ventricular non-compaction. Heart. 2007;93(1):65–71.PubMedCrossRef Lofiego C et al. Wide spectrum of presentation and variable outcomes of isolated left ventricular non-compaction. Heart. 2007;93(1):65–71.PubMedCrossRef
27.
Zurück zum Zitat Ichida F et al. Clinical features of isolated noncompaction of the ventricular myocardium: long-term clinical course, hemodynamic properties, and genetic background. J Am Coll Cardiol. 1999;34(1):233–40.PubMedCrossRef Ichida F et al. Clinical features of isolated noncompaction of the ventricular myocardium: long-term clinical course, hemodynamic properties, and genetic background. J Am Coll Cardiol. 1999;34(1):233–40.PubMedCrossRef
28.
Zurück zum Zitat •• Niemann M, Stork S, Weidemann F. Left ventricular noncompaction cardiomyopathy: an overdiagnosed disease. Circulation. 2012;126(16):e240–3. Although this is only a case report, it highlights some of the difficulties in establishing the diagnosis of LCNC. The authors report a patient with the typical echocardiographic findings of LVNC, who actually had myocarditis. •• Niemann M, Stork S, Weidemann F. Left ventricular noncompaction cardiomyopathy: an overdiagnosed disease. Circulation. 2012;126(16):e240–3. Although this is only a case report, it highlights some of the difficulties in establishing the diagnosis of LCNC. The authors report a patient with the typical echocardiographic findings of LVNC, who actually had myocarditis.
29.
Zurück zum Zitat Stollberger C, Finsterer J, Blazek G. Left ventricular hypertrabeculation/noncompaction and association with additional cardiac abnormalities and neuromuscular disorders. Am J Cardiol. 2002;90(8):899–902.PubMedCrossRef Stollberger C, Finsterer J, Blazek G. Left ventricular hypertrabeculation/noncompaction and association with additional cardiac abnormalities and neuromuscular disorders. Am J Cardiol. 2002;90(8):899–902.PubMedCrossRef
30.
Zurück zum Zitat •• Habib G et al. Isolated left ventricular non-compaction in adults: clinical and echocardiographic features in 105 patients. Results from a French registry. Eur J Heart Fail. 2011;13(2):177–85. In this large series of LVNC, the authors concluded that Left ventricular non-compaction was detected by familial screening in asymptomatic patients in 8% of cases; left ventricular non-compaction was frequently over-diagnosed by echocardiography and patients identified as LVNC presented with a high risk of severe complications, transplantation or death and needed close follow-up. •• Habib G et al. Isolated left ventricular non-compaction in adults: clinical and echocardiographic features in 105 patients. Results from a French registry. Eur J Heart Fail. 2011;13(2):177–85. In this large series of LVNC, the authors concluded that Left ventricular non-compaction was detected by familial screening in asymptomatic patients in 8% of cases; left ventricular non-compaction was frequently over-diagnosed by echocardiography and patients identified as LVNC presented with a high risk of severe complications, transplantation or death and needed close follow-up.
31.
Zurück zum Zitat Belanger AR et al. New classification scheme of left ventricular noncompaction and correlation with ventricular performance. Am J Cardiol. 2008;102(1):92–6.PubMedCrossRef Belanger AR et al. New classification scheme of left ventricular noncompaction and correlation with ventricular performance. Am J Cardiol. 2008;102(1):92–6.PubMedCrossRef
32.
Zurück zum Zitat McMahon CJ et al. Left ventricular non-compaction cardiomyopathy in children: characterisation of clinical status using tissue Doppler-derived indices of left ventricular diastolic relaxation. Heart. 2007;93(6):676–81.PubMedCrossRef McMahon CJ et al. Left ventricular non-compaction cardiomyopathy in children: characterisation of clinical status using tissue Doppler-derived indices of left ventricular diastolic relaxation. Heart. 2007;93(6):676–81.PubMedCrossRef
33.
Zurück zum Zitat de Groot-de Laat LE et al. Usefulness of contrast echocardiography for diagnosis of left ventricular noncompaction. Am J Cardiol. 2005;95(9):1131–4.PubMedCrossRef de Groot-de Laat LE et al. Usefulness of contrast echocardiography for diagnosis of left ventricular noncompaction. Am J Cardiol. 2005;95(9):1131–4.PubMedCrossRef
34.
Zurück zum Zitat Gianfagna P et al. Additive value of contrast echocardiography for the diagnosis of noncompaction of the left ventricular myocardium. Eur J Echocardiogr. 2006;7(1):67–70.PubMedCrossRef Gianfagna P et al. Additive value of contrast echocardiography for the diagnosis of noncompaction of the left ventricular myocardium. Eur J Echocardiogr. 2006;7(1):67–70.PubMedCrossRef
35.
Zurück zum Zitat Bodiwala K et al. Live three-dimensional transthoracic echocardiographic assessment of ventricular noncompaction. Echocardiography. 2005;22(7):611–20.PubMedCrossRef Bodiwala K et al. Live three-dimensional transthoracic echocardiographic assessment of ventricular noncompaction. Echocardiography. 2005;22(7):611–20.PubMedCrossRef
36.
Zurück zum Zitat •• Parsai C et al. Diagnostic and prognostic value of cardiovascular magnetic resonance in non-ischaemic cardiomyopathies. J Cardiovasc Magn Reson. 2012;14:54. The potential application of CMR in the clinical approach of a patient with suspected non-ischaemic cardiomyopathy is discussed in this review. •• Parsai C et al. Diagnostic and prognostic value of cardiovascular magnetic resonance in non-ischaemic cardiomyopathies. J Cardiovasc Magn Reson. 2012;14:54. The potential application of CMR in the clinical approach of a patient with suspected non-ischaemic cardiomyopathy is discussed in this review.
37.
Zurück zum Zitat Kohli SK et al. Diagnosis of left-ventricular non-compaction in patients with left-ventricular systolic dysfunction: time for a reappraisal of diagnostic criteria? Eur Heart J. 2008;29(1):89–95.PubMedCrossRef Kohli SK et al. Diagnosis of left-ventricular non-compaction in patients with left-ventricular systolic dysfunction: time for a reappraisal of diagnostic criteria? Eur Heart J. 2008;29(1):89–95.PubMedCrossRef
38.
Zurück zum Zitat •• Karamitsos TD, Francis JM, Neubauer S. The current and emerging role of cardiovascular magnetic resonance in the diagnosis of nonischemic cardiomyopathies. Prog Cardiovasc Dis. 2011;54(3):253–65. Tako-tsubo cardiomyopathy is a poorly understood syndrome. This paper is a case report of a 76-year-old female patient with apical ballooning syndrome and features of left ventricular non-compaction that was followed up by Cardiovascular Magnetic Resonance (CMR) imaging. •• Karamitsos TD, Francis JM, Neubauer S. The current and emerging role of cardiovascular magnetic resonance in the diagnosis of nonischemic cardiomyopathies. Prog Cardiovasc Dis. 2011;54(3):253–65. Tako-tsubo cardiomyopathy is a poorly understood syndrome. This paper is a case report of a 76-year-old female patient with apical ballooning syndrome and features of left ventricular non-compaction that was followed up by Cardiovascular Magnetic Resonance (CMR) imaging.
39.
Zurück zum Zitat •• Martin M et al. Usefulness of cardiac magnetic resonance imaging in left ventricular non-compaction cardiomyopathy. Eur J Heart Fail. 2011;13(5):577. In this paper the authors make the point that although many questions remain about left ventricular non-compaction disease, cardiac MRI can provide some of the answers through a more precise diagnosis based on a better endomyocardial definition and tissue characterization that can also help to determine patient prognosis. •• Martin M et al. Usefulness of cardiac magnetic resonance imaging in left ventricular non-compaction cardiomyopathy. Eur J Heart Fail. 2011;13(5):577. In this paper the authors make the point that although many questions remain about left ventricular non-compaction disease, cardiac MRI can provide some of the answers through a more precise diagnosis based on a better endomyocardial definition and tissue characterization that can also help to determine patient prognosis.
40.
Zurück zum Zitat Petersen SE et al. Left ventricular non-compaction: insights from cardiovascular magnetic resonance imaging. J Am Coll Cardiol. 2005;46(1):101–5.PubMedCrossRef Petersen SE et al. Left ventricular non-compaction: insights from cardiovascular magnetic resonance imaging. J Am Coll Cardiol. 2005;46(1):101–5.PubMedCrossRef
41.
Zurück zum Zitat •• Jacquier A et al. Measurement of trabeculated left ventricular mass using cardiac magnetic resonance imaging in the diagnosis of left ventricular non-compaction. Eur Heart J. 2010;31(9):1098–104. In this paper the authors describe a method for measuring trabeculated left ventricular (LV) mass using cardiac magnetic resonance imaging and to assess its value in the diagnosis of left ventricular non-compaction. This method is felt to be reproducible and provides an assessment of the global amount of LV trabeculation. A trabeculated LV mass above 20% of the global LV mass is highly sensitive and specific for the diagnosis of LVNC. •• Jacquier A et al. Measurement of trabeculated left ventricular mass using cardiac magnetic resonance imaging in the diagnosis of left ventricular non-compaction. Eur Heart J. 2010;31(9):1098–104. In this paper the authors describe a method for measuring trabeculated left ventricular (LV) mass using cardiac magnetic resonance imaging and to assess its value in the diagnosis of left ventricular non-compaction. This method is felt to be reproducible and provides an assessment of the global amount of LV trabeculation. A trabeculated LV mass above 20% of the global LV mass is highly sensitive and specific for the diagnosis of LVNC.
42.
Zurück zum Zitat •• Grothoff M et al. Value of cardiovascular MR in diagnosing left ventricular non-compaction cardiomyopathy and in discriminating between other cardiomyopathies. Eur Radiol. 2012;22(12):2699–709. The authors of this paper maintain that cardiac magnetic resonance imaging can reliably diagnose left ventricular non-compaction cardiomyopathy. Furthermore differentiation of LVNC from other cardiomyopathies and normal hearts is possible. The best diagnostic performance can be achieved if combined MRI criteria for the diagnosis are used. •• Grothoff M et al. Value of cardiovascular MR in diagnosing left ventricular non-compaction cardiomyopathy and in discriminating between other cardiomyopathies. Eur Radiol. 2012;22(12):2699–709. The authors of this paper maintain that cardiac magnetic resonance imaging can reliably diagnose left ventricular non-compaction cardiomyopathy. Furthermore differentiation of LVNC from other cardiomyopathies and normal hearts is possible. The best diagnostic performance can be achieved if combined MRI criteria for the diagnosis are used.
43.
Zurück zum Zitat • Caliskan K et al. Frequency of asymptomatic disease among family members with noncompaction cardiomyopathy. Am J Cardiol. 2012;110(10):1512–7. In this paper authors evaluated 156, mostly first-degree, family members of 44 adult patients with LVNC and found a high prevalence, mostly asymptomatic, of cardiac disease (26%) among first- and second-degree family members of patients. They felt this supports the concept of screening and offers an opportunity for early intervention. • Caliskan K et al. Frequency of asymptomatic disease among family members with noncompaction cardiomyopathy. Am J Cardiol. 2012;110(10):1512–7. In this paper authors evaluated 156, mostly first-degree, family members of 44 adult patients with LVNC and found a high prevalence, mostly asymptomatic, of cardiac disease (26%) among first- and second-degree family members of patients. They felt this supports the concept of screening and offers an opportunity for early intervention.
44.
Zurück zum Zitat •• Melendez-Ramirez G et al. Left ventricular noncompaction: a proposal of new diagnostic criteria by multidetector computed tomography. J Cardiovasc Comput Tomogr. 2012;6(5):346–54. In this paper authors maintain that LVNC can be accurately diagnosed with MDCT when using a cutoff NC/C ratio of 2.2 at end diastole involving >/=2 segments. •• Melendez-Ramirez G et al. Left ventricular noncompaction: a proposal of new diagnostic criteria by multidetector computed tomography. J Cardiovasc Comput Tomogr. 2012;6(5):346–54. In this paper authors maintain that LVNC can be accurately diagnosed with MDCT when using a cutoff NC/C ratio of 2.2 at end diastole involving >/=2 segments.
45.
Zurück zum Zitat • Nucifora G et al. Myocardial fibrosis in isolated left ventricular non-compaction and its relation to disease severity. Eur J Heart Fail. 2011;13(2):170–6. This study evaluated the prevalence and extent of myocardial fibrosis in patients with isolated left ventricular non-compaction to determine its relation to clinical status and LV systolic function and determined that myocardial fibrosis is related to clinical disease severity and LV systolic dysfunction in isolated LVNC. • Nucifora G et al. Myocardial fibrosis in isolated left ventricular non-compaction and its relation to disease severity. Eur J Heart Fail. 2011;13(2):170–6. This study evaluated the prevalence and extent of myocardial fibrosis in patients with isolated left ventricular non-compaction to determine its relation to clinical status and LV systolic function and determined that myocardial fibrosis is related to clinical disease severity and LV systolic dysfunction in isolated LVNC.
46.
Zurück zum Zitat • Quarta G, Sado DM, Moon JC. Cardiomyopathies: focus on cardiovascular magnetic resonance. Br J Radiol. 2011;84 Spec No 3:p. S296–305. This paper reviews the role of CMR in the clinical assessment of patients with cardiomyopathies. • Quarta G, Sado DM, Moon JC. Cardiomyopathies: focus on cardiovascular magnetic resonance. Br J Radiol. 2011;84 Spec No 3:p. S296–305. This paper reviews the role of CMR in the clinical assessment of patients with cardiomyopathies.
47.
Zurück zum Zitat • Ismail TF, Prasad SK, Pennell DJ. Prognostic importance of late gadolinium enhancement cardiovascular magnetic resonance in cardiomyopathy. Heart. 2012;98(6):438–42. This paper reviews the evidence for late gadolinium enhancement as a prognostic marker in dilated and hypertrophic cardiomyopathy and highlights the challenges ahead. • Ismail TF, Prasad SK, Pennell DJ. Prognostic importance of late gadolinium enhancement cardiovascular magnetic resonance in cardiomyopathy. Heart. 2012;98(6):438–42. This paper reviews the evidence for late gadolinium enhancement as a prognostic marker in dilated and hypertrophic cardiomyopathy and highlights the challenges ahead.
Metadaten
Titel
Non-Compaction Cardiomyopathy: Underdiagnosed or Over Diagnosed?
verfasst von
Robert A. Quaife
Ernesto E. Salcedo
Eugene E. Wolfel
Publikationsdatum
01.12.2013
Verlag
Springer US
Erschienen in
Current Cardiovascular Imaging Reports / Ausgabe 6/2013
Print ISSN: 1941-9066
Elektronische ISSN: 1941-9074
DOI
https://doi.org/10.1007/s12410-013-9227-z

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