Terapevticheskii arkhivTerapevticheskii arkhivТерапевтический архив0040-36602309-5342LLC Obyedinennaya Redaktsiya33424Editorial articleПередовая статьяLeft ventricular noncompaction concurrent with bronchoectatic disease complicated by secondary AA-amyloidosis with renal involvementНекомпактный миокард левого желудочка, сочетающийся с бронхоэктатической болезнью, осложнившейся вторичным АА-амилоидозом с поражением почекFominViktor ViktorovichФоминВиктор Викторович

д-р мед. наук, доц. каф. терапии и профболезней; Клиника нефрологии, внутренних и профессиональных болезней им. Е. М. ТарееваКлиника кардиологии ММА им. И. М. Сеченова

fomin_vic@mail.ruRameevV VРамеевВ В

Клиника нефрологии, внутренних и профессиональных болезней им. Е. М. ТарееваКлиника кардиологии ММА им. И. М. Сеченова

SedovV PСедовВ П

Клиника нефрологии, внутренних и профессиональных болезней им. Е. М. ТарееваКлиника кардиологии ММА им. И. М. Сеченова

SeverovaM MСевероваМ М

Клиника нефрологии, внутренних и профессиональных болезней им. Е. М. ТарееваКлиника кардиологии ММА им. И. М. Сеченова

SvetA VСветА В

Клиника нефрологии, внутренних и профессиональных болезней им. Е. М. ТарееваКлиника кардиологии ММА им. И. М. Сеченова

MiroshnichenkoN GМирошниченкоН Г

Клиника нефрологии, внутренних и профессиональных болезней им. Е. М. ТарееваКлиника кардиологии ММА им. И. М. Сеченова

KozlovskayaL VКозловскаяЛ В

Клиника нефрологии, внутренних и профессиональных болезней им. Е. М. ТарееваКлиника кардиологии ММА им. И. М. Сеченова

FominV V

E. M. Tareyev Clinic of Nephrology, Internal and Occupational Diseases

RameyevV V

E. M. Tareyev Clinic of Nephrology, Internal and Occupational Diseases

SedovV P

Department of Radiation Diagnosis

SeverovaM M

E. M. Tareyev Clinic of Nephrology, Internal and Occupational Diseases

SvetA V

Clinic of Cardiology, I. M. Sechenov Moscow Medical Academy

MiroshnichenkoN G

E. M. Tareyev Clinic of Nephrology, Internal and Occupational Diseases

KozlovskayaL V

E. M. Tareyev Clinic of Nephrology, Internal and Occupational Diseases

Клиника нефрологии, внутренних и профессиональных болезней им. Е. М. ТарееваКлиника кардиологии ММА им. И. М. СеченоваE. M. Tareyev Clinic of Nephrology, Internal and Occupational DiseasesDepartment of Radiation DiagnosisClinic of Cardiology, I. M. Sechenov Moscow Medical Academy15012010821NO1 (2010)№1 (2010)737713042020Copyright ©; 2010, Consilium MedicumCopyright ©; 2010, ООО "Консилиум Медикум"2010Consilium MedicumООО "Консилиум Медикум"https://creativecommons.org/licenses/by-nc-sa/4.0https://ter-arkhiv.ru/0040-3660/article/view/33424

The paper describes a clinical case of congenital cardiomyopathy (left ventricular noncompaction) concurrent with secondary amyloidosis and renal involvement that develops at the outcome of long existing brochoectatic disease.

Описано наблюдение сочетания врожденной кардиомиопатии - некомпактного миокарда левого желудочка с вторичным амилоидозом и поражением почек, развившимся в исходе длительно существующей бронхоэктатической болезни.

left ventricular noncompactionAA- amyloidosisнекомпактный миокард левого желудочкаАА-амилоидозEngberding R., Yelbuz T., Breithardt G. Iolated noncompaction of the left ventricular myocardium -a review of the literature two decades after the initial case description. Clin. Res. Cardiol. 2007; 96(7): 481-488.Chrissoheris M. P., Ali R., Vivas Y. et al. Isolated noncompaction of the ventricular myocardium: contemporary diagnosis and management. Clin. Cardiol. 2007; 30(4): 156-160.Chin T. K., Perloff J. K., Williams R. G. et al. Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation 1990; 82(2): 507-513.Bozic I., Fabijanic D., Carevic V., Polic S. Echocardiography in the diagnosis and management of isolated left ventricular noncompaction: case reports and review of the literature. J. Clin. Ultrasound 2006; 34(8): 416-421.Bodiwala K., Miller A. P., Nanda N. C. et al. Live three-dimensional transthoracic echocardiographic assessment of ventricular noncompaction. Echocardiography 2005; 22(7): 611- 620.Porciani M. C., Rao C. M., Perini A. P. et al. Real-time 3-dimensional transthoracic echocardiography improves the diagnosis of isolated ventricular noncompaction. J. Ultrasound Med. 2007; 26(4): 551-553.Rajdev S., Singh A., Nanda N. C. et al. Comparison of two- and three-dimensional transthoracic echocardiography in the assessment of trabeculations and trabecular mass in left ventricular noncompaction. Echocardiography 2007; 24(7): 760- 767.Yelamanchili P., Nanda N. C., Patel V. et al. Live/real time three-dimensional echocardiographic demonstration of left ventricular noncompaction and thromb. Echocardiography 2006; 23(8): 704-706.Gianfagna P., Badano L. P., Faganello G. 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.Mohrs O. K., Magedanz A., Schlosser T. Noncompaction of the left ventricular myocardium detected by 64-slice multidetector computed tomography. Clin. Cardiol. 2007; 30(1): 48.Goo H. W., Park I. S. Left ventricular noncompaction in an infant: use of non-ECG-gated cardiac CT. Pediatr. Radiol. 2007; 37(2): 217-220.Stцllberger C., Finsterer J. Value of cardiac magnetic resonance imaging in the diagnosis of left ventricular hypertrabeculation/ noncompaction. J. Cardiovasc. Magn. Reson. 2004; 6(4): 959-960.Dodd J. D., Holmvang G., Hoffmann U. et al. Quantification of left ventricular noncompaction and trabecular delayed hyperenhancement with cardiac MRI: correlation with clinical severity. Am. J. Roentgenol. 2007; 189(4): 974-980.Oda Y., Tsunoda S., Doue T. et al. Clinical characteristics and morphologic features of isolated noncompaction of the ventricular myocardium: electrocardiogram-gated can elucidate more accurate biventricular structures Heart Vessels 2005; 20(3): 126-127.Fox E. H., Wood M. L., Trotter J. et al. New onset head failure in a 29-year-old: A case report of isolated left ventricular noncompaction. South. Med. J. 2006; 99(10): 1l30-1133.Stцllberger C., Finsterer J. Cardiologic and neurologic findings in left ventricular hypertrabeculation/non-compaction related to wall thickness, size and systolic function. Eur. J. Heart Fail. 2005; 7(1): 95-97.El-Menyar A. A., Gendi S. M., Numan M. T. Noncompaction cardiomyopathy in the State of Qatar. Saudi Med. J. 2007; 28(3): 429-434.Munehisa Y., Watanabe H., Kosaka T. et al. Successful outcome in a pregnant woman with isolated noncompaction of the left ventricular myocardium. Intern. Med. 2007; 46(6): 285-289.Alehan D. Clinical features of isolated left ventricular noncompaction in children. Int. J. Cardiol. 2004; 97(2): 233-237.Hruda J., Sobotka-Plojhar M. A., Fetter W. P. Transient postnatal heart failure caused by noncompaction of the right ventricular myocardium. Pediatr. Cardiol. 2005; 26(4): 452-454.Ali S. K., Godman M. J. The variable clinical presentation of, and outcome for, noncompaction of the ventricular myocardium in infants and children, an under-diagnosed cardiomyopathy. Cardiol. Young 2004; 14(4): 409-416.Wald R., Veldtman G., Golding F. Determinants of outcome in isolated ventricular noncompaction in childhood. Am. J. Cardiol. 2004; 94(12): 1581-1584.Hughes M. L., Carstensen B., Wilkinson J. L., Weintraub R. G. Angiographic diagnosis, prevalence and outcomes for left ventricular noncompaction in children with congenital cardiac disease. Cardiol. Young 2007; l7(1): 56-63.Timperley J., Becher H., Westaby S. Isolated ventricular noncompaction with left ventricular thrombus. Echocardiography 2005; 22(1): 51-52.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): 465-468.Stцllberger C., Finsterer J. Left ventricular hypertrabeculation/ noncompaction and stroke or embolism. Cardiology 2005; 103(2): 68-72.Walpot J., Klazen C., Sorgedrager J. et al. Isolated ventricular noncompaction in a patient with stroke. Echocardiography 2005; 22(8): 690-692.Nakajima M., Hirano T., Doi H., Uchino M. Stroke and ventricular dysfunction in a patient with isolated left ventricular noncompaction. Intern. Med. 2007; 46(15): 1251-1254.Бобкова И. Н., Полянцева Л. Р., Тареева И. Е., Козловская Л. В. Клиническое значение определения фактора Виллебранда у больных волчаночным нефритом. Тер. арх. 1995; 5: 14-16.Korantzopoulos P., Patsouras D., Siogas K. Transient ischemic attack and electrocardiographic abnormalities revealing left ventricular noncompaction. Int. J. Cardiol. 2007; 119(2): 232-234.Blessing E., Rottbauer W., Mereles D. et al. Isolated left ventricular noncompaction of the myocardium as a cause of embolic superior mesenteric artery occlusion. J. Am. Soc. Echocardiogr. 2005; 18(6): 693.Everett M. E., Kirkpatrick J. N., Lang R. M. Noncompaction of the myocardium complicated by coronary artery embolism. J. Am. Soc. Echocardiogr. 2005; 18(2): 194-196.Fagen K., Lane M., Silverman E. D. Isolated left ventricular noncompaction: a diagnosis to consider with left ventricular wall akinesis. Clin. Nucl. Med. 2005; 30(6): 425-426.Finsterer J., Stцllberger C., Krugluger W. Positive troponin-T in noncompaction is associated with neuromuscular disorders and poor outcome. Clin. Res. Cardiol. 2007; 96(2): 109-113.Jenni R., Wyss C. A., Oechslin E. N. et al. Isolated ventricular noncompaction is associated with coronary microcirculatory dysfunction. J. Am. Coll. Cardiol. 2002; 39(3): 450-454.Sato Y., Matsumoto N., Yoda S. et al. Left ventricular aneurysm associated with isolated noncompaction of the ventricular myocardium. Heart Vessels 2006; 21(3): 192-194.Swinkels B. M., Boersma L. V., Rensing B. J. et al. Isolated left ventricular noncompaction in a patient presenting with a subacute myocardial infarction. Neth. Heart J. 2007; 15(3): 109- 111.Matsumoto N., Sato Y., Kunimasa T. et al. Noncompaction of the ventricular myocardium mimicking ischemic cardiomyopathy. Ann. Nucl. Med. 2006; 20(9): 639-641.Yavuzgil O., Gьrgьn C., Soydas Cinar C. et al. Anterior myocardial infarction in an adult patient with left ventricular hypertrabeculation/noncompaction. Int. J. Cardiol. 2006; 106(3): 394-395.Enriquez S. G., Entem F. R., Cobo M., Olalla J. J. Uncommon etiology of syncope in a patient with isolated ventricular noncompaction. Pacing Clin. Electrophysiol. 2007; 30(4): 577-579.Taniguchi M., Hioka T., Maekawa K. et al. Adult case of isolated ventricular noncompaction discovered by complete atrioventricular block. Circ. J. 2004; 68(9): 873-875.Kawasaki T., Azuma A., Taniguchi T. et al. Heart rate variability in adult patients with isolated left ventricular noncompaction. Int. J. Cardiol. 2005; 99(1): 147-150.Fichet J., Legras A., Bernard A., Babuty D. Aborted sudden cardiac death revealing isolated noncompaction of the left ventricle in a patient with Wolff-Parkinson-White syndrome. Pacing Clin. Electrophysiol. 2007; 30(3): 444-447.Чазов Е. И., Шлевков Н. Б., Харлап М. С. и др. Случай некомпактного миокарда левого желудочка и синдрома Бругада. Сердеч. недостат. 2004; 5(3): 157-162.Kubota S., Nogami A., Sugiyasu A., Kasuya K. Cardiac resynchronization therapy in a patient with isolated noncompaction of the left ventricle and a narrow QRS complex. Heart Rhythm. 2006; 3(5): 619-620.Lim H. E., Pak H. N., Shim W. J. et al. Epicardial ablation of ventricular tachycardia associated with isolated ventricular noncompaction. Pacing Clin. Electrophysiol. 2006; 29(7): 797-799.Friedman M. A., Wiseman S., Haramati L. et al. Noncompaction of the left ventricle in a patient with dextroversion. Eur. J. Echocardiogr. 2007; 8(1): 70-73.Sugiyama H., Hoshiai M., Toda T., Nakazawa S. Double-orifice mitral valve associated with noncompaction of left ventricular myocardium. Pediatr. Cardiol. 2006; 27(6): 746-749.Sadeghpour A., Peighambari M., Dalirrooyfard M. et al. Aorta-to-left ventricle tunnel associated with noncompaction left ventricle. J. Am. Soc. Echocardiogr. 2006; 19(8): 1073.Arslan S., Gurlertop H. Y., Gundogdu F., Senocak H. Left ventricular noncompaction and mid-caviter narrowing associated with Ebstein's anomaly: three-dimensional transthoracic echocardiographic image. Int. J. Cardiol. 2007; 115(1): e52- e55.Song Z. Z., Ma J. A rare combination of left ventricular noncompaction and a right coronary artery-to-right ventricle fistula: echocardiographic features. J. Ultrasound Med. 2007; 26(4): 547-550.Park J. S., Shin D. G., Kim Y. J. et al. Left ventricular noncompaction with a single coronary artery of anomalous origin. Int. J. Cardiol. 2007; 119(2): e35-e37.Koh Y. Y., Seo Y. U., Woo J. J. et al. Familial isolated noncompaction of the ventricular myocardium in asymptomatic phase. Yonsei Med. J. 2004; 45(5): 931-935.Kitao K., Ohara N., Funakoshi T. et al. Noncompaction of the left ventricular myocardium diagnosed in pregnant woman and neonate. J. Perinat. Med. 2004: 32(6): 527-531.Limongelli G., Pacileo G., Marino B. et al. Prevalence and clinical significance of cardiovascular abnormalities in patients with the LEOPARD syndrome. Am. J. Cardiol. 2007; 100(4): 736-741.Finsterer J., Stцllberger C., Steger C., Cozzarini W. Complete heart block associated with noncompaction, nail-patella syndrome, and mitochondrial myopathy. J. Electrocardiol. 2007; 40(4): 352-354.Finsterer J., Stцllberger C. Myopathy associated with spherocytosis and left ventricular hypertrabeculation/noncompaction. Eur. J. Heart Fail. 2007; 9(1): 100.Corrado G., Checcarelli N., Santarone M. et al. Left ventricular hypertrabeculation/noncompaction with PMP22 duplication-based Charcot-Marie-Tooth disease type 1a. Cardiology 2006; 105(3): 142-145.Finsterer J., Stцllberger C., Blazek G. Neuromuscular implications in left ventricular hypertrabeculation/noncompaction. Int. J. Cardiol. 2006; 110(3): 288-300.Pauli R. M., Scheib-Wixted S., Cripe L. et al. Ventricular noncompaction and distal chromosome 5q deletion. Am. J. Med. Genet. 1999; 85(4): 419-423.Hermida-Prieto M., Monserrat L., Castro-Beiras A. et al. Familial dilated cardiomyopathy and isolated left ventricular noncompaction associated with lamin A/C gene mutations. Am. J. Cardiol. 2004; 94(1): 50-54.Xing Y., Ichida F., Matsuoka T. et al. Genetic analysis in patients with left ventricular noncompaction and evidence for genetic heterogeneity. Mol. Genet. Metab. 2006; 88(1): 71-77.Notarangelo L. D., Plebani A., Mazzolari E. et al. Genetic causes of bronchiectasis: primary immune deficiencies and the lung. Respiration 2007; 74(3): 264-275.Kennedy M. P., Omran H., Leigh M. W. et al. Congenital heart disease and other heterotaxic defects in a large cohort of patients with primary ciliary dyskinesia. Circulation 2007; 115(22): 2814-2821.Zhao J., Chen H., Peschon J. J. et al. Pulmonary hypoplasia in mice lacking tumor necrosis factor-alpha converting enzyme indicates an indispensable role for cell surface protein shedding during embryonic lung branching morphogenesis. Dev. Biol. 2001; 232(1); 204-218.Shi W., Chen H., Sun J. et al. TACE is required for fetal murine cardiac development and modeling. Dev. Biol. 2003; 261(2): 371-380.Мухин Н. А. Клинические проблемы амилоидоза почек. Клин. мед. 1983; 10: 12-17.Козловская Л. В., Рамеев В. В., Саркисова И. А. Амилоидоз у пожилых. Клин. мед. 2005; 6: 12-16.Моисеев С. В. Инфильтративные поражения миокарда. Рестриктивная кардиомиопатия. М.: Фарма Пресс; 1998.Stцllberger C., Winkler-Dworak M., Blazek G., Finsterer J. Cardiologic and neuromuscular co-morbidity influences mortality of patients with left ventricular hypertrabeculation/noncompaction: comparison with the Austrian general population. Acta Cardiol. 2007; 62(1): 1-5.Oechslin E. N., Attenhofer Jost C. H., Rojas J. R. et al. Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J. Am. Coll. Cardiol. 2000; 36(2): 493-500.Aras D., Tufekcioglu O., Ergun K. 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): 726-733.Spieker T., Krasemann T., Hoffmeier A. et al. Heart transplantation for isolated noncompaction of the left ventricle in an infant. Thorac. Cardiovasc. Surg. 2007; 55(2): 127-129.Shimamoto T., Marui A., Yamanaka K. et al. Left ventricular restoration surgery for isolated left ventricular noncompaction: report of the first successful case. J. Thorac. Cardiovasc. Surg. 2007; 134(1): 246-247.