International Journal of Cardiology 184 (2015) 659–663
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
A case of non-compaction ventricular myocardium and multiple coronary artery-to-right ventricle fistulae Yiwei Chen 1, Zhifang Zhang 1, Fen Li ⁎, Lijun Fu, Jinjin Wu, Yuqi Zhang, Yumin Zhong Department of Radiology, Shanghai Children's Medical Center Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai 200127, PR China
a r t i c l e
i n f o
Article history: Received 6 January 2015 Accepted 21 February 2015 Available online 24 February 2015 Keywords: Non-compaction ventricular myocardium (NVM) Multiple coronary artery-to-right ventricle fistulae Cardiomyopathy
1. Introduction Non-compaction ventricular myocardium (NVM) and multiple coronary artery-to-right ventricle fistulae belong to rare cardiovascular malformations and the combination of these two diseases is rarely reported. NVM is a myocardial disorder and it is caused by the development arrest of the endocardium during embryonic period. The pathological process includes compaction failure of the loose arranged trabecular results in compression failure of sinusoidal connection to capillary, then the trabecular reside in the innermost layer of ventricular muscle and multiple interconnected cavities are presented in the ventricle [1]. Congenital coronary artery fistula (CCAF) may arise due to persistence of sinusoidal connections and trabecular cavities which are presented in the early embryologic period [2]. Therefore, NVM and CCAF shared some common etiology on the basis of their histoembryological characteristics. Until now, few reports on combination case of cardiomyopathy and coronary artery fistula were revealed [3–5] and only 2 cases of NVM and CCAF combination were reported [6,7]. A case of combination of NVM and multiple coronary artery-toright ventricle fistulae diagnosed in the department of cardiovascular medicine of Shanghai Children's Medical Center affiliated to Shanghai Jiaotong university in October 2013 was reported here. The clinical ⁎ Corresponding author at: Department of Cardiology, Shanghai Children's Medical Center Affiliated to Shanghai Jiaotong University School of Medicine, No. 1678 Dongfang Road, Shanghai 200127, PR China. E-mail address: [email protected] (F. Li). 1 Yiwei Chen and Zhifang Zhang contributed equally to this work.
http://dx.doi.org/10.1016/j.ijcard.2015.02.042 0167-5273/© 2015 Elsevier Ireland Ltd. All rights reserved.
course of this complication and therapeutic approaches presented in the literature are also briefly discussed. 1.1. Case report The patient was a 6-month old baby girl and was admitted into the hospital due to the poor appetite and night sweat for 2 weeks. The baby patient was diagnosed with cardiac soufflé during the physical examination 42 days after the birth. Patent foramen ovale and probable cardiomyopathy were revealed by transthoracic echocardiography performed in local hospital. No treatment was applied. Decreased appetite of 5–6 times (daily) breast nursing with 90–100 mL milk occasional stop-eat and copious sweat at usual were found. Average mental state, no anhelation, no edema and normal urine output were also observed in the baby patient. The baby patient was the first child of the family and was delivered on full-term without asphyxiation rescues. The weight of the baby patient at the time of birth and admission was 3.6 and 7.5 kg, respectively. Then she was processed with breast nursing after the birth. The patient was only vaccinated with one dose of Bacillus Calmette–Guérin and hepatitis B vaccine. The parents were confirmed with physical health and the ages of father and mother were 28 and 30, respectively. No consanguineous marriage or history of inheritable disease or similar disease was found in the whole family. 1.1.1. Physical examination Clear mental statue, average mental reaction, free to raise head and sit by herself, no abnormity on face, average nutrition and development, rough breath sounds of bilateral pulmonary, no involving of dry or wet rales, normal heart rhythms with 120 beats/min heart rate, slightly low heart sound, grade II blowy mid- or end-systolic murmur on the third and fourth intercostal spaces of left border of sternum, soft abdomen, liver lies 2.5 cm under the rib, no palpable below xiphoid, soft texture, no tenderness, no palpable spleen under the rib cage and no abnormity on the examination of nervous system. 1.1.2. Auxiliary examination Creatine Kinase (CK): 94 U/L, creatine kinase MB (CK-MB): 21 U/L, fast troponin t (TnT): 202.00 ng/L (normal value b50 ng/L), N-terminal pro-brain natriuretic peptide (NT-proBNP): 15,375 pg/mL (normal range 0–100 pg/mL). No abnormalities on blood routine, prothrombin time (PT), activated partial thromboplastin time (APTT)
660
Y. Chen et al. / International Journal of Cardiology 184 (2015) 659–663
and liver and kidney function. Electrocardiography results: left ventricular enlargement, ST-T changes (Fig. 1). 1.1.3. Imageological examination Chest PA (posterioranterior) photo: increased lung markings, enlarged heart shadow; echocardiography: left ventricle enlargement with non-compacted ventricular myocardium, dilation on left and right coronary artery, persistent sinusoidal connection in the myocardium of right ventricle, moderate mitral regurgitation and left ventricular dysfunction (Fig. 2). Angiocardiography and coronary arteriography: multiple coronary artery and left anterior descending branch-to-right ventricle fistula, mitral regurgitation and left ventricular enlargement (Fig. 3). 1.1.4. Diagnosis and therapeutic treatment After completing all the examinations, the baby patient was considered as having NVM, multiple coronary artery-to-right ventricle fistulae and cardiac dysfunction. 10 μg/(kg ∗ d) digoxin, 7.5 μg/(kg ∗ d) furosemide and 7.5 μg/(kg ∗ d) spironolactone were applied to patient to induce cardiotonic, diuresis and cardiac function improvement. Creatine phosphate was also given to provide nutrition to the myocardium. The appetite recovery was found in the baby patient after 1 week and the breast nursing was increased to 6–7 times daily with 130–150 mL milk per nursing. Liver lies 1.5 cm under the rib and the patient was allowed to discharge. The patient was asked to orally take the digoxin, furosemide and spironolactone and follow-up at the outpatient department.
cases of NVM and coronary artery-to-left ventricle fistulae combination [6,7], a case of hypertrophic cardiomyopathy and coronary artery-toright ventricle fistulae combination [11] and no case of NVM and coronary artery-to-right ventricle fistulae combination were found. We also performed a search using Chinese databases named Wanfang and Chongqing Vip and no such disease was found. The first case was a 67-year male in the US and he was admitted into the hospital due to the progressive dyspnea with precordial pain for 3 months. The patient was diagnosed with non-compaction left ventricular myocardium and multiple coronary artery-to-left ventricle fistulae. The author suggested that these two diseases were caused by the development arrest of the endocardium at a certain time point during embryonic period and development failure of sinusoidal connections to capillary. They were the different clinical manifestations of the same disease [6]. The second case was a 46-year female in Germany and admitted into the hospital due to the dyspnea. Non-compaction myocardium was confirmed by transthoracic echocardiography and multiple coronary artery-to-left ventricle fistulae were found by coronary arterioangiography (CAG) [7]. Cardiac insufficiency was found in both cases and no treatment or prognosis description was found in the paper. In the case of hypertrophic cardiomyopathy and coronary artery-to-right ventricle fistulae combination, a 6-year Germany boy was admitted into the hospital due to repeated syncope and he was considered as having severe ischemia and Adams–Stokes syndrome. Transcatheter closure of coronary artery fistulae and implantable cardioverter defibrillator were processed to decrease the risk of sudden death [11]. 2. Discussion
1.2. Literature data Engberding et al. reported the first case of isolated NVM in 1984 [8]. NVM was defined as unclassified heart disease by World Health Organization/International Society and Federation of Cardiology [9]. In 2006, NVM was defined as a kind of primary inheritable cardiomyopathy by the American Heart Association (AHA) [10]. NVM could be combined with coronary artery ventricle fistulae but the low incidence was expected. After performing a search using Pubmed, OVID and Elsevier, 2
Non-compaction myocardium is caused by the development arrest of myocardium compaction during embryonic period. In normal condition, loose arranged trabeculars are gradually compressed, ventricle wall is formed and sinusoidal connection is transformed into capillary network and becomes a part of coronary artery circulation during the 4th and 8th week of the embryonic development. However, if exception occurs, the myocardium remains in the primary state and non-compacted cardiomyopathy will be present. Persistent opening of
Fig. 1. The 12 lead electrocardiogram of the baby patient showed left ventricular enlargement and ST-T segment changes.
Y. Chen et al. / International Journal of Cardiology 184 (2015) 659–663
661
Fig. 2. Results of 2-D echocardiography. A. Enlarged, hypertrophic left ventricle according to parasternal four chamber view and thick trabeculations in the left ventricle cavity; B, 0.539 cm of noncompacted layer of endocardium and 0.216 cm compacted layer of epicardium on left ventricle lateral wall, the ratio was 2.5; C, sinusoidal connection open and multiple low microblood flow connected with the right ventricular cavity; D, left ventricular end-diastolic dimension was 3.1 cm, left ventricular end-systolic dimension was 2.6 cm, calculated left ventricle ejection factor was 34%, diffused decreasing of ventricular wall motion. Cardiac insufficiency was suggested.
sinusoidal connection, also known as coronary artery ventricle fistula, can simultaneously be presented. Combination of NVM and coronary artery ventricle fistula is rarely seen and its prevalence is only 0.05%– 0.24%. The current study suspected the possibility of familial inheritance and 12%–47% of patients are presented with family history [12]. Klassassen et al. [13] suggested that the etiology of the disease was related with the mutation of a-dystrobrevin, Cypher/ZASP, myotonic dystrophy protein kinase (DMPK), dystrophin, mitochondrial DNA, LMNA gene, etc. In 2013, a study suggested to perform a screening test to the suspected genes such as MYH7, ACTC, TPM1, MYBPC, TNNT2, FKBP-12 and SCN5A [14]. Multiple clinical manifestations with different degrees of severity, such as cardiac insufficiency and arrhythmia, are described in patients with isolated non-compacted cardiomyopathy. When the combination of NVM and coronary artery fistula happens, the myocardial ischemia and cardiac insufficiency caused by ventricle lesion in baby patient will become more severe. Then the blood supply of coronary artery will decrease due to the fistula shunt, the ventricular diastolic perfusion pressure of coronary artery will also decrease and coronary arterial steal syndrome will happen. These will finally result in the myocardial ischemia and decrease of the myocardial contraction. In addition, the abnormal relaxation of the ventricle myocardium could be caused by non-compacted myocardium while myocardial diastolic insufficiency could be induced by restricted ventricular filling. The clinical features such as dysphoria when nursing, pale complexion and night sweat will be present in the baby patient. According to previous description
and the case presented here, cardiac ischemia (ST-segment depression or T wave changes) can be found in the echocardiography. The transthoracic echocardiography and angiocardiography (including CAG) are the most usually used in clinical diagnosis and echocardiography is the most convenient way to process the examination. Jenni et al. [15] summarized the criteria of echocardiography as follows: (1) multiple prominent trabeculations and deep intertrabecular recesses communicating with ventricular cavity: (2) the ratio of non-compacted to compacted myocardial layers at the site of maximal wall thickness N1.4 in child and N2.0 in adult; (3) the color Doppler study showed sinusoidal connection open and low blood flow from the ventricular cavity into the spaces between the prominent trabeculations throughout the cardiac cycle; (4) ventricular cavity enlargement, weak movement, impaired myocardium contraction and diastolic function; and (5) other congenital or acquired heart diseases were excluded. The case described here or reported by other groups is consistent with the first four diagnostic criteria. Controversy still existed on the classification of the NVM and coronary artery ventricular fistula. We supported the opinion of coronary artery ventricular fistula caused by transforming failure of the sinusoidal connection to capillary and it is a distinct manifestation of non-compacted myocardium. The diagnosis of coronary artery ventricle fistula usually depends on CAG that can provide clinical data such as direction of coronary artery, drainage site, size of fistula and distribution of the coronary artery branch at the proximal veins of the fistula. The treatment of the disease includes application of cardiotonic, diuresis and vessel dilation agents
662
Y. Chen et al. / International Journal of Cardiology 184 (2015) 659–663
Fig. 3. Results of selective left coronary artery angiography. A, multiple coronary artery to right ventricle micro-fistulas could be found. Contrast agent was injected into the right ventricle via the right coronary artery and then into the pulmonary artery; B, multiple coronary artery micro-fistulas could be found between left anterior descending branch and right ventricle. Contrast agent was injected into the right ventricle via fistulae; C, multiple coronary artery micro-fistulas could be found between left anterior descending branch and right ventricle.
by targeting cardiac insufficiency or the transcatheter closure of coronary artery fistulae. In the case described here, transcatheter closure was not suitable due to 6 month age and digoxin, furosemide and spironolactone were applied to improve cardiac function. Improved clinical symptoms were observed at 2 months after discharge. However, the exact therapeutic outcome could not completely follow up due to the lack of imageological follow-up data. In conclusion, the combination of NVM and multiple coronary artery-to-right ventricle fistulae is a kind of rarely seen congenital heart malformations. In baby patients, poor appetite and night sweat were the main clinical features. The diagnosis of the disease is mainly dependent on the echocardiography and cardioangiography. The applications of cardiotonic, diuresis and vessel dilation agents are the main strategy in treatment. The early screening and diagnosis of the disease may be achieved through the improvement of the imageological examinations. Funding The Natural Science Foundation of Science and Technology Commission of Shanghai Municipality (No. 13ZR1457500); Innovation Program of Shanghai Municipal Education (No. 14YZ039, No. 11ZZ14); Shanghai City funded training plan of young teachers in Colleges and Universities(No. ZZjdyx12116); The National Natural Science Foundation of China (No. 81170152). Conflict of interest None declared.
Acknowledgment Special thanks to Dr. Shubao Chen for his numerous contributions to this article, without his help, this article will never come into being. References [1] P. Thavendiranathan, A. Dahiya, D. Phelan, M.Y. Desai, W.H. Tang, Isolated left ventricular non-compaction controversies in diagnostic criteria, adverse outcomes and management, Heart 99 (2013) 681–689. [2] C.V. Mangukia, Coronary artery fistula, Ann. Thorac. Surg. 93 (2012) 2084–2092. [3] C. Dresios, S. Apostolakis, S. Tzortzis, K. Lazaridis, A. Gardikiotis, Apical hypertrophic cardiomyopathy associated with multiple coronary artery-left ventricular fistulae: a report of a case and review of the literature, Eur. J. Echocardiogr. 11 (2010) E9. [4] O. Ucar, H. Cicekcioglu, M. Cetin, M. Ileri, S. Aydogdu, Coronary artery-left ventricular microfistulae associated with apical hypertrophic cardiomyopathy, Cardiol. J. 18 (2011) 307–309. [5] O. Alyan, O. Ozeke, Z. Golbasi, Coronary artery-left ventricular fistulae associated with apical hypertrophic cardiomyopathy, Eur. J. Echocardiogr. 7 (2006) 326–329. [6] V. Dias, S. Cabral, M. Vieira, I. Sa, D. Anjo, C. Gomes, et al., Noncompaction cardiomyopathy and multiple coronary arterioventricular fistulae: 1 or 2 distinct disease entities? J. Am. Coll. Cardiol. 57 (2011) e377. [7] J. Wilhelm, K. Heinroth, D. Stoevesandt, K. Werdan, A. Plehn, Non-compaction cardiomyopathy with diffuse left coronary artery fistulae as a rare cause of congestive heart failure, Eur. Heart J. 34 (2013) 12. [8] R. Engberding, F. Bender, Identification of a rare congenital anomaly of the myocardium by two-dimensional echocardiography: persistence of isolated myocardial sinusoids, Am. J. Cardiol. 53 (1984) 1733–1734. [9] P. Richardson, W. McKenna, M. Bristow, B. Maisch, B. Mautner, J. O'Connell, 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 93 (1996) 841–842. [10] B.J. Maron, J.A. Towbin, G. Thiene, C. Antzelevitch, D. Corrado, D. Arnett, 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
Y. Chen et al. / International Journal of Cardiology 184 (2015) 659–663 Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention, Circulation 113 (2006) 1807–1816. [11] S. Caputo, G. Capozzi, G. Santoro, G. Pacileo, M.C. Bigazzi, M.G. Russo, et al., Multiple right coronary artery fistulae in a patient with diffuse hypertrophic cardiomyopathy: a case report, J. Am. Soc. Echocardiogr. 18 (2005) 884. [12] E.J. Topol, R.M. Califf, Textbook of Cardiovascular Medicine, Lippincott Williams & Wilkins, 2007. [13] S. Klaassen, S. Probst, E. Oechslin, B. Gerull, G. Krings, P. Schuler, et al., Mutations in sarcomere protein genes in left ventricular noncompaction, Circulation 117 (2008) 2893–2901.
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[14] M.J. Ackerman, S.G. Priori, S. Willems, C. Berul, R. Brugada, H. Calkins, et al., HRS/ EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies this document was developed as a partnership between the Heart Rhythm Society (HRS) and the European Heart Rhythm Association (EHRA), Europace 13 (2011) 1077–1109. [15] R. Jenni, E. Oechslin, J. Schneider, C. Attenhofer Jost, P.A. Kaufmann, Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy, Heart 86 (2001) 666–671.
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
A case of non-compaction ventricular myocardium and multiple coronary artery-to-right ventricle fistulae Yiwei Chen 1, Zhifang Zhang 1, Fen Li ⁎, Lijun Fu, Jinjin Wu, Yuqi Zhang, Yumin Zhong Department of Radiology, Shanghai Children's Medical Center Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai 200127, PR China
a r t i c l e
i n f o
Article history: Received 6 January 2015 Accepted 21 February 2015 Available online 24 February 2015 Keywords: Non-compaction ventricular myocardium (NVM) Multiple coronary artery-to-right ventricle fistulae Cardiomyopathy
1. Introduction Non-compaction ventricular myocardium (NVM) and multiple coronary artery-to-right ventricle fistulae belong to rare cardiovascular malformations and the combination of these two diseases is rarely reported. NVM is a myocardial disorder and it is caused by the development arrest of the endocardium during embryonic period. The pathological process includes compaction failure of the loose arranged trabecular results in compression failure of sinusoidal connection to capillary, then the trabecular reside in the innermost layer of ventricular muscle and multiple interconnected cavities are presented in the ventricle [1]. Congenital coronary artery fistula (CCAF) may arise due to persistence of sinusoidal connections and trabecular cavities which are presented in the early embryologic period [2]. Therefore, NVM and CCAF shared some common etiology on the basis of their histoembryological characteristics. Until now, few reports on combination case of cardiomyopathy and coronary artery fistula were revealed [3–5] and only 2 cases of NVM and CCAF combination were reported [6,7]. A case of combination of NVM and multiple coronary artery-toright ventricle fistulae diagnosed in the department of cardiovascular medicine of Shanghai Children's Medical Center affiliated to Shanghai Jiaotong university in October 2013 was reported here. The clinical ⁎ Corresponding author at: Department of Cardiology, Shanghai Children's Medical Center Affiliated to Shanghai Jiaotong University School of Medicine, No. 1678 Dongfang Road, Shanghai 200127, PR China. E-mail address: [email protected] (F. Li). 1 Yiwei Chen and Zhifang Zhang contributed equally to this work.
http://dx.doi.org/10.1016/j.ijcard.2015.02.042 0167-5273/© 2015 Elsevier Ireland Ltd. All rights reserved.
course of this complication and therapeutic approaches presented in the literature are also briefly discussed. 1.1. Case report The patient was a 6-month old baby girl and was admitted into the hospital due to the poor appetite and night sweat for 2 weeks. The baby patient was diagnosed with cardiac soufflé during the physical examination 42 days after the birth. Patent foramen ovale and probable cardiomyopathy were revealed by transthoracic echocardiography performed in local hospital. No treatment was applied. Decreased appetite of 5–6 times (daily) breast nursing with 90–100 mL milk occasional stop-eat and copious sweat at usual were found. Average mental state, no anhelation, no edema and normal urine output were also observed in the baby patient. The baby patient was the first child of the family and was delivered on full-term without asphyxiation rescues. The weight of the baby patient at the time of birth and admission was 3.6 and 7.5 kg, respectively. Then she was processed with breast nursing after the birth. The patient was only vaccinated with one dose of Bacillus Calmette–Guérin and hepatitis B vaccine. The parents were confirmed with physical health and the ages of father and mother were 28 and 30, respectively. No consanguineous marriage or history of inheritable disease or similar disease was found in the whole family. 1.1.1. Physical examination Clear mental statue, average mental reaction, free to raise head and sit by herself, no abnormity on face, average nutrition and development, rough breath sounds of bilateral pulmonary, no involving of dry or wet rales, normal heart rhythms with 120 beats/min heart rate, slightly low heart sound, grade II blowy mid- or end-systolic murmur on the third and fourth intercostal spaces of left border of sternum, soft abdomen, liver lies 2.5 cm under the rib, no palpable below xiphoid, soft texture, no tenderness, no palpable spleen under the rib cage and no abnormity on the examination of nervous system. 1.1.2. Auxiliary examination Creatine Kinase (CK): 94 U/L, creatine kinase MB (CK-MB): 21 U/L, fast troponin t (TnT): 202.00 ng/L (normal value b50 ng/L), N-terminal pro-brain natriuretic peptide (NT-proBNP): 15,375 pg/mL (normal range 0–100 pg/mL). No abnormalities on blood routine, prothrombin time (PT), activated partial thromboplastin time (APTT)
660
Y. Chen et al. / International Journal of Cardiology 184 (2015) 659–663
and liver and kidney function. Electrocardiography results: left ventricular enlargement, ST-T changes (Fig. 1). 1.1.3. Imageological examination Chest PA (posterioranterior) photo: increased lung markings, enlarged heart shadow; echocardiography: left ventricle enlargement with non-compacted ventricular myocardium, dilation on left and right coronary artery, persistent sinusoidal connection in the myocardium of right ventricle, moderate mitral regurgitation and left ventricular dysfunction (Fig. 2). Angiocardiography and coronary arteriography: multiple coronary artery and left anterior descending branch-to-right ventricle fistula, mitral regurgitation and left ventricular enlargement (Fig. 3). 1.1.4. Diagnosis and therapeutic treatment After completing all the examinations, the baby patient was considered as having NVM, multiple coronary artery-to-right ventricle fistulae and cardiac dysfunction. 10 μg/(kg ∗ d) digoxin, 7.5 μg/(kg ∗ d) furosemide and 7.5 μg/(kg ∗ d) spironolactone were applied to patient to induce cardiotonic, diuresis and cardiac function improvement. Creatine phosphate was also given to provide nutrition to the myocardium. The appetite recovery was found in the baby patient after 1 week and the breast nursing was increased to 6–7 times daily with 130–150 mL milk per nursing. Liver lies 1.5 cm under the rib and the patient was allowed to discharge. The patient was asked to orally take the digoxin, furosemide and spironolactone and follow-up at the outpatient department.
cases of NVM and coronary artery-to-left ventricle fistulae combination [6,7], a case of hypertrophic cardiomyopathy and coronary artery-toright ventricle fistulae combination [11] and no case of NVM and coronary artery-to-right ventricle fistulae combination were found. We also performed a search using Chinese databases named Wanfang and Chongqing Vip and no such disease was found. The first case was a 67-year male in the US and he was admitted into the hospital due to the progressive dyspnea with precordial pain for 3 months. The patient was diagnosed with non-compaction left ventricular myocardium and multiple coronary artery-to-left ventricle fistulae. The author suggested that these two diseases were caused by the development arrest of the endocardium at a certain time point during embryonic period and development failure of sinusoidal connections to capillary. They were the different clinical manifestations of the same disease [6]. The second case was a 46-year female in Germany and admitted into the hospital due to the dyspnea. Non-compaction myocardium was confirmed by transthoracic echocardiography and multiple coronary artery-to-left ventricle fistulae were found by coronary arterioangiography (CAG) [7]. Cardiac insufficiency was found in both cases and no treatment or prognosis description was found in the paper. In the case of hypertrophic cardiomyopathy and coronary artery-to-right ventricle fistulae combination, a 6-year Germany boy was admitted into the hospital due to repeated syncope and he was considered as having severe ischemia and Adams–Stokes syndrome. Transcatheter closure of coronary artery fistulae and implantable cardioverter defibrillator were processed to decrease the risk of sudden death [11]. 2. Discussion
1.2. Literature data Engberding et al. reported the first case of isolated NVM in 1984 [8]. NVM was defined as unclassified heart disease by World Health Organization/International Society and Federation of Cardiology [9]. In 2006, NVM was defined as a kind of primary inheritable cardiomyopathy by the American Heart Association (AHA) [10]. NVM could be combined with coronary artery ventricle fistulae but the low incidence was expected. After performing a search using Pubmed, OVID and Elsevier, 2
Non-compaction myocardium is caused by the development arrest of myocardium compaction during embryonic period. In normal condition, loose arranged trabeculars are gradually compressed, ventricle wall is formed and sinusoidal connection is transformed into capillary network and becomes a part of coronary artery circulation during the 4th and 8th week of the embryonic development. However, if exception occurs, the myocardium remains in the primary state and non-compacted cardiomyopathy will be present. Persistent opening of
Fig. 1. The 12 lead electrocardiogram of the baby patient showed left ventricular enlargement and ST-T segment changes.
Y. Chen et al. / International Journal of Cardiology 184 (2015) 659–663
661
Fig. 2. Results of 2-D echocardiography. A. Enlarged, hypertrophic left ventricle according to parasternal four chamber view and thick trabeculations in the left ventricle cavity; B, 0.539 cm of noncompacted layer of endocardium and 0.216 cm compacted layer of epicardium on left ventricle lateral wall, the ratio was 2.5; C, sinusoidal connection open and multiple low microblood flow connected with the right ventricular cavity; D, left ventricular end-diastolic dimension was 3.1 cm, left ventricular end-systolic dimension was 2.6 cm, calculated left ventricle ejection factor was 34%, diffused decreasing of ventricular wall motion. Cardiac insufficiency was suggested.
sinusoidal connection, also known as coronary artery ventricle fistula, can simultaneously be presented. Combination of NVM and coronary artery ventricle fistula is rarely seen and its prevalence is only 0.05%– 0.24%. The current study suspected the possibility of familial inheritance and 12%–47% of patients are presented with family history [12]. Klassassen et al. [13] suggested that the etiology of the disease was related with the mutation of a-dystrobrevin, Cypher/ZASP, myotonic dystrophy protein kinase (DMPK), dystrophin, mitochondrial DNA, LMNA gene, etc. In 2013, a study suggested to perform a screening test to the suspected genes such as MYH7, ACTC, TPM1, MYBPC, TNNT2, FKBP-12 and SCN5A [14]. Multiple clinical manifestations with different degrees of severity, such as cardiac insufficiency and arrhythmia, are described in patients with isolated non-compacted cardiomyopathy. When the combination of NVM and coronary artery fistula happens, the myocardial ischemia and cardiac insufficiency caused by ventricle lesion in baby patient will become more severe. Then the blood supply of coronary artery will decrease due to the fistula shunt, the ventricular diastolic perfusion pressure of coronary artery will also decrease and coronary arterial steal syndrome will happen. These will finally result in the myocardial ischemia and decrease of the myocardial contraction. In addition, the abnormal relaxation of the ventricle myocardium could be caused by non-compacted myocardium while myocardial diastolic insufficiency could be induced by restricted ventricular filling. The clinical features such as dysphoria when nursing, pale complexion and night sweat will be present in the baby patient. According to previous description
and the case presented here, cardiac ischemia (ST-segment depression or T wave changes) can be found in the echocardiography. The transthoracic echocardiography and angiocardiography (including CAG) are the most usually used in clinical diagnosis and echocardiography is the most convenient way to process the examination. Jenni et al. [15] summarized the criteria of echocardiography as follows: (1) multiple prominent trabeculations and deep intertrabecular recesses communicating with ventricular cavity: (2) the ratio of non-compacted to compacted myocardial layers at the site of maximal wall thickness N1.4 in child and N2.0 in adult; (3) the color Doppler study showed sinusoidal connection open and low blood flow from the ventricular cavity into the spaces between the prominent trabeculations throughout the cardiac cycle; (4) ventricular cavity enlargement, weak movement, impaired myocardium contraction and diastolic function; and (5) other congenital or acquired heart diseases were excluded. The case described here or reported by other groups is consistent with the first four diagnostic criteria. Controversy still existed on the classification of the NVM and coronary artery ventricular fistula. We supported the opinion of coronary artery ventricular fistula caused by transforming failure of the sinusoidal connection to capillary and it is a distinct manifestation of non-compacted myocardium. The diagnosis of coronary artery ventricle fistula usually depends on CAG that can provide clinical data such as direction of coronary artery, drainage site, size of fistula and distribution of the coronary artery branch at the proximal veins of the fistula. The treatment of the disease includes application of cardiotonic, diuresis and vessel dilation agents
662
Y. Chen et al. / International Journal of Cardiology 184 (2015) 659–663
Fig. 3. Results of selective left coronary artery angiography. A, multiple coronary artery to right ventricle micro-fistulas could be found. Contrast agent was injected into the right ventricle via the right coronary artery and then into the pulmonary artery; B, multiple coronary artery micro-fistulas could be found between left anterior descending branch and right ventricle. Contrast agent was injected into the right ventricle via fistulae; C, multiple coronary artery micro-fistulas could be found between left anterior descending branch and right ventricle.
by targeting cardiac insufficiency or the transcatheter closure of coronary artery fistulae. In the case described here, transcatheter closure was not suitable due to 6 month age and digoxin, furosemide and spironolactone were applied to improve cardiac function. Improved clinical symptoms were observed at 2 months after discharge. However, the exact therapeutic outcome could not completely follow up due to the lack of imageological follow-up data. In conclusion, the combination of NVM and multiple coronary artery-to-right ventricle fistulae is a kind of rarely seen congenital heart malformations. In baby patients, poor appetite and night sweat were the main clinical features. The diagnosis of the disease is mainly dependent on the echocardiography and cardioangiography. The applications of cardiotonic, diuresis and vessel dilation agents are the main strategy in treatment. The early screening and diagnosis of the disease may be achieved through the improvement of the imageological examinations. Funding The Natural Science Foundation of Science and Technology Commission of Shanghai Municipality (No. 13ZR1457500); Innovation Program of Shanghai Municipal Education (No. 14YZ039, No. 11ZZ14); Shanghai City funded training plan of young teachers in Colleges and Universities(No. ZZjdyx12116); The National Natural Science Foundation of China (No. 81170152). Conflict of interest None declared.
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