Morphologically proved ANCA positive Loeffler's pancarditis: medical and surgical treatment.

Loeffler's endocarditis remains is a very rare disease, develops due to eosinophilic inflammation predominantly of the endocardium with an outcome in fibrosis and massive thrombus formation and. He is generally characterized by an unfavorable prognosis. Clinical case of a 42-year-old patient with Loeffler endocarditis is presented. The development of the disease was preceded by a polyvalent allergy, mild dry eye syndrome and pansinusitis with a single eosinophilia of blood up to 16%. The reason for the hospitalization was the appearance of biventricular heart failure. During the previous year, the level of blood eosinophils remained normal, a threefold increase in the level of eosinophilic cationic protein was observed once. A 20-fold increase in the pANCA level, a 2.5-fold increase in the level of antibodies to DNA, an antibody to the nuclei of cardiomyocytes 1:160 were detected. The diagnosis was made on the basis of electrocardiography data (low QRS voltage, atrial hypertrophy), echocardiography, multispiral computed tomography and magnetic resonance imaging of the heart (thickening and delayed contrasting of the endocardium, massive thrombosis of the left ventricular apex with obliteration of its cavity, encapsulated fluid in the pericardium with compression of the right ventricle). Systolic dysfunction, severe signs of restriction and arrhythmias were absent. Trombectomy, tricuspid valve plasty, pericardial resection, suturing of an open oval window were performed. Signs of active inflammation with single eosinophils, vasculitis, perimuscular sclerosis, endocardial sclerosis were detected in morphological and immunohistochemical studies of endo-, myo-, pericardium. Viral genome was not found. The therapy with methylprednisolone 24 mg/day, azathioprine 75 mg/day was started. Six months after the operation, the symptoms of heart failure are completely absent, the thrombosis did not recur.

[Giant Aneurisms of Coronary Arteries in the Kawasaki Syndrome].

BACKGROUND: Kawasaki disease is an acute systemic vasculitis of childhood with possible coronary artery involvement which can cause acute coronary syndrome in children and young adults. PURPOSE: to study clinical and demographic characteristics of patients with Kawasaki disease with giant coronary aneurysms, to assess long-term prognosis in these patients. MATERIAL AND METHODS: In 2003-2015 262 children with Kawasaki disease were examined in the University Children Hospital of the I.M. Sechenov Moscow State Medical University. The following methods of visualization of coronary arteries were used: echocardiography in all, multispiral computer tomography in 29, invasive coronary angiography in 11 patients. RESULTS: Aneurysms of coronary arteries were found in 84 patients (32.1%), in 15 of them aneurisms were giant (internal diameter >8 mm). During follow-up (from 0.67 to 12, median 5.8 years) intracoronary thrombi were found in 11 patients. Two of them had occlusions of the right coronary artery, 5 had coronary artery stenosis which required surgical intervention. One patient developed myocardial infarction before coronary surgery. CONCLUSION: Patients with Kawasaki disease with giant aneurysms of coronary arteries are predisposed to coronary thrombosis and stenosis. Long-term management of patients with regular examination, prevention of coronary thrombosis, and timely surgical intervention are necessary for lowering the risk of life-threatening complications.

[Multislice spiral computed tomography of the heart in dilated cardiomyopathy: possibilities in the verification of myocarditis (in comparison with myocardial biopsy) and in the evaluation of prognosis]. / Mul'tispiral'naia komp'iuternaia tomografiia serdtsa pri sindrome dilatatsionnoi kardiomiopatii: vozmozhnosti v verifikatsii miokardita (po sravneniiu s biopsiei miokarda) i otsenke prognoza.

AIM: To investigate whether intravenous contrast-enhanced multislice spiral computed tomography (computed tomography) (MSCT) versus myocardial morphological examination can diagnose myocarditis and the non-inflammatory causes of dilated cardiomyopathy (DCM) and evaluate prognosis in patients with the latter. SUBJECTS AND METHODS: A study group consisted of 130 patients, including 95 men (46.8±11.9 years), with DCM (mean left ventricular (LV) end-diastolic dimension (EDD), 6.6±0.8 cm; mean LV ejection fraction (EF), 29.8±9.3%; NYHA functional class (FC) III (II; III)). All the patients underwent intravenous contrast-enhanced 320-slice CT of the heart; myocardial morphological examination was made in 48 patients (endomyocardial biopsy in 29 patients, intraoperative biopsy in 7, and autopsy in 9, and study of the explanted heart in 3). In addition, cardiotropic viral DNA in the blood and myocardium and the level of anticardiolipin antibodies were determined; echocardiography (in all the patients), scintigraphy (n = 45), magnetic resonance imaging (MRI) (n = 21), and coronary angiography (CG) (n = 46), and a genetic consultation were performed. A comparison group comprised 20 patients, including 14 men (69.3±9.2 years), with coronary atherosclerosis (40% or more stenoses) according to MSCT findings in the absence of criteria for DCM (mean LV EDD, 4.8±0.5 cm; mean LV EF, 59.4±4.6%). RESULTS: Morphological/comprehensive examination showed that myocarditis as a cause of DCM was diagnosed in 76 (65%) patients; its concurrence with genetic cardiomyopathies was in 17 more patients (17%). MSCT of the heart revealed lower accumulation areas in 2 (1.5%) patients (type 1 based on the proposed rating scale), delayed myocardial contrast agent accumulation (DMCAA) in 81 (62.3%): subendocardial accumulation (type 2) in 8, intramyocardial accumulation in 4 (type 3), subepicardial accumulation in 52 (type 4), and transmural accumulation in 15 (type 5); DMCAA was not noted in 49 patients. DMCAA was not found in the comparison group. As compared with biopsy, the sensitivity, specificity, predictive value of positive and negative results of the tests in detecting active myocarditis for all the types of DMCAA were 77.4, 47.1, 72.7, and 53.3%, respectively; those for types 3-5 of DMCAA were 77.4, 52.9, 75.0, and 56.3%; those in detecting all the morphological types of myocarditis were 68.3, 28.6, 84.8, and 13.3%, and those for types 3-5 were 65.9, 28.6, 84.4, and 12.5%, respectively. Comparison of the data of MSCT and those of comprehensive examination in all the patients with DCM, the diagnostic significance in detecting myocarditis for all the types of DMCAA was 70.6, 67.9, 88.9 and 38.8%, respectively; that for DMCAA types 3-5 was 60.8, 67.9, 87.3, and 32.3%. In the study group, MSCT also identified the non-compacted myocardium (n = 31 (23.8%)), coronary atherosclerosis (n = 31 (23%)), which is confirmed by CG findings in 15 patients. The patients with DMCAA significantly more frequently showed a relationship with previous infection, acute onset, significantly higher NYHA FCs, end-diastolic and end-systolic LV volumes, and insignificantly lower LV EF. During a mean follow-up periods of 12 (6; 37.25) months, the overall mortality rate was 17.7% (23 deaths); the death + transplantation index was 20% (n = 26). All the types of DMCAA were found to be significantly related to prognosis: in the DMCAA group, the mortality rate was 21.5% versus 7.8% in the non-DMCAA group (odds ratio 3.22; 95% confidence interval, 1.02 to 10.21; p < 0.05). CONCLUSION: MSCT with the assessment of delayed contrast enhancement (and simultaneous CT coronary angiography) can be used for the non-invasive diagnosis of myocarditis in patients with DCM, including that in the presence of contraindications to MRI. DMCAA correlates with the presence of myocarditis, its activity, the degree of functional disorders, and prognosis.

[Chronic Heart Failure With Preserved Systolic Function and Reversible Dilatation of Cardiac Chambers].

Diagnosis of dilation (D) cardiomyopathy (CMP) requires exclusion not only of inflammatory and genetically determined forms but also of some rare diseases. This 51 year old patient with history of moderate arterial hypertension approached a cardiologist because of new onset atrial fibrillation and dyspnea. Echocardiography detected dilation of all cardiac chambers with relatively preserved ejection fraction, causing suspicion of DCMP. Among conditions excluded were coronary atherosclerosis, congenital heart defect with left to right shunt, primary pulmonary hypertension, pulmonary embolism, hypertensive heart, tachycardia induced CMP, arrhythmogenic right ventricular dysplasia, noncompaction myocardium. Further examination revealed massive pelvic arteriovenous malformation with branches of right internal iliac artery and inferior mesenteric artery as feeding vessels. This malformation was considered the leading cause of DCMP. Successful multistage embolization of feeding arteries was associated with reduction of cardiac chambers, alleviation of valvular regurgitation and pulmonary hypertension, restoration of sinus rhythm. Presentation of this case is followed by discussion of possible mechanisms of heart failure with high cardiac output and preserved systolic function in patients with arteriovenous malformations with left to right shunt. Approaches to interventional treatment of these malformations are also discussed.

[Noncompaction myocardium as a primary phenomenon or consequence of myocardial dysfunction: clinical masks of the syndrome].

Noncompaction myocardium (NCM) is a genetic heterogeneous primary cardiomyopathy which affects both children and adults and can be either isolated or combined with other congenital heart disorders. It has common pathogenesis of symptoms but is distinguished by pronounced clinical polymorphism. We have observed 25 adult patients (15 men, 10 women aged from 20 to 62 years, mean age 42.9+/-13.3 years) with NCM syndrome. Heart failure have been found in 96% of patients (functional class [FC] I in 7, II - in 6, III in 7, and IV - in 4 patients). Ninety two percent of patients have ventricular extrasystoles, 32% - atrial fibrillation, 28% - FC I-III angina. Mean end diastolic left ventricular dimension is 6.5+/-0.8cm, ejection fraction 29.7+/-13.0%, mean pulmonary artery pressure - 42.6+/-13.5 mm Hg. Intracardiac thrombosis have been found in 24% of patients. In 7 patients morphological study of myocardium has been performed. NCM syndrome was diagnosed at initial investigation just in 1 case. We distinguished the following clinical masks (variants of diagnosis) of NCM: 1) clinically not manifest, is revealed at accidental examination (4%); 2) exists under mask of "idiopathic" rhythm disturbances (8%); 3) has a mask of ischemic heart disease; 4) is revealed in patients with acute or subacute myocarditis (12%); 5) has a mask of dilated cardiomyopathy (52%); 6) NCM in patients with other primary cardiomyopathies (hypertrophic, restrictive, genetic myopathy, arrhythmogenic right ventricular dysplasia). Combination of NCM with congenital heart defects has been found in 20% of patients. In 56% of cases myocarditis was diagnosed (it was viral in no less than 44%). Only in 32% of patients it is possible to consider presence of isolated NCM syndrome. This paper contains discussion of problems of diagnostics (including morphological) and treatment in the presented group of patients, significance of myocarditis for development of decompensation, role of NCM in patients with other primary cardiomyopathies, possibility of compensatory (secondary) character of NCM in severe systolic dysfunction.

[Arrhythmogenic right ventricular dysplasia: polymorphism of clinical manifestations].

We observed 15 patients with arrhythmogenic right ventricular dysplasia (ARVD): 9 with definite and 5 with probable ARVD (modified European Criteria, 2010). Eight patients had typical ARVD (frequent right ventricular extrasystoles, nonsustained right ventricular tachycardia without heart failure with or without myocarditis). Five patients had ARVD with progressive heart failure (right- or biventricular with or without myocarditis). Two patients had full scale arrhythmic form (sustained right ventricular tachycardia without or with right ventricular dilation, with or without myocarditis). In 3 cases diagnosis was confirmed morphologically or with DNA-diagnostics. This material allowed us to highlight the following specific points related to diagnostics of ARVD. Detection of fat at MRT is not obligatory for diagnosis, fat can be detected by MSCT; ventricular arrhythmias can move backwards in the picture of the disease; leading clinical manifestation can be unexplained right ventricular insufficiency; ARVD can be combined with other genetic cardiomyopathies as well as with infectious immune myocarditis (up to 50% of patients); elevated titer of anticardiac antibodies is not characteristic for isolated ARVD; myocardial biopsy allows to verify both ARVD and concomitant myocarditis. The paper also contains discussion of the role of myocarditis in various forms of ARVD and possibilities of its diagnosis and treatments.

[Successful treatment of massive thrombosis of the vena cava inferior with nephrotic syndrome and chronic bilateral pulmonary artery thromboembolism in a patient with genetic thrombophilia].

A case is reported of a 23-year-old male patient who developed, after severe blunt injury of the lumbar region, massive thrombosis of the vena cava inferior (VCI), both renal veins, bilateral pulmonary artery thromboembolism (PATE), nephrotic syndrome (NS). In spite of anticoagulant therapy, the condition of the patient progressively aggravated for 1.5 year: thrombosis involved the ileac and femoral arteries on the right, thrombus floated in the right atrium with PATE recurrent episodes, pulmonary hypertension reached 120 mm Hg with formation of decompensated cor pulmnonale, proteinuria and hypoalbuminemia deteriorated, anasarca edema developed Multigenic thrombophilia was diagnosed (1 homozygous and 5 heterozygous mutations). A radical one-stage operation was successful: thromboectomy from the VCI, right ileac and left renal veins, thrombendarterectomy from the pulmonary arteries, suture of the interatrial septum defect, installation of cava-filter After the operation pulmonary pressure lowered to 40-45 mm Hg, right heart volume normalized, immunosuppressive therapy with prednisolone and cyclosporine led to nephropathy remission. The discussion covers mechanisms and factors (including genetic) of thrombosis progression, correlations between intravascular thrombosis, NS and chronic glomerulonephritis (possible NS development due to bilateral thrombosis of the renal veins and nephropathy role in thrombosis progression), approaches to conservative and surgical treatment of such patients. Global experience in conduction of pulmonary thrombendarterectomy and thrombectomy from VCI is reviewed (one-stage operations were not described earlier).

[Dilated cardiomyopathy as clinical syndrome: experience with nosological diagnostics with biopsy and treatment approaches].

AIM: To study possibility of nosological diagnosis in patients with dilated cardiomyopathy (DCMP) with use of myocardial biopsy. MATERIAL AND METHODS: The trial enrolled 62 patients (23 females) with DCMP syndrome (end diastolic left ventricular size > 5.5 cm, ejection fraction < 55%). Mean age of the patients was 46.0 +/- 12.8 years. The examination included diagnosis of viral infections (Herpes virus, parvovirus B19), measurement of anticardial antibodies titer, 99Tc-MIBI single photon emission computed tomography of the myocardium, multislice computed tomography, MRT of the heart, coronarography, morphological study of the myocardium (n=20) with application of polymerase chain reaction (PCR) for H.simplex viruses of types 1, 2 and 6, herpes zoster, Epstein-Barr, cytomegalovirus, parvovirus B-19, adenoviruses. The control group (20 operated patients with valvular heart disease and coronary heart disease) was examined for viral genome in the blood and myocardium. RESULTS: Complex examination of DCMP patients showed the following distribution by nosological entuities: myocarditis (n=41, 66.1%) including virus-positive (n=14), primary DCMP (n=16, 25.9%) including with non-compact myocarditis (NCM) in 3, with debute at delivery of the child--in 3. Arrhythmogenic right ventricular dysplasia combined with viral myocarditis (n=2), genetic myopathy (n=1) and Takayasu disease (n=1) combined with NCM, isolated NCM (n=1) were diagnosed in the rest cases. Morphological investigation of the myocardium was made in 20 patients: diagnosis of myocarditis and primary DCMP were made in 70% (including in 2 patients with CHD) and 20%. Detection of viral genome was 20 and 15% in the study and control group, respectively, in the myocardium--in 57.9 (test for parvovirus B19 was not made in 26%) and 65.0% (complete diagnosis). All the virus-positive patients with DCMP were diagnosed to have signs of active/borderline myocarditis. Diagnostic criteria and poor prognosis factors were defined. CONCLUSION: The nosological diagnosis of DCMP was made in all the examinees basing on the complex of clinical, case history and device evidence. The diagnosis was morphologically verified in 33.9% patients. Treatment approaches are developed.

[Primary lymphoma of the heart: difficulties in diagnosis and treatment].

Primary lymphoma of the heart (non-Hodgkin's lymphoma primarily of B-cells affecting first the heart and pericardium) is a rare disease which accounts for 1.3-2.0% of all primary tumors of the heart. A case is reported of a 43-year-old male patient with a cardioverter-defibrillator implanted at the age of 38 years for management of ventricular tachycardia paroxysms and AB-block of the second-third degree. The patient also had hypertrophic cardiomyopathy, moderate pressure gradient on the pulmonary artery valve and pericardial effusion. Later he had two cardiac tamponades (serous punctuate). From the age of 42 years breathlessness, right ventricular wall thickness, right heart pressure and pulmonary artery pressure aggravated. The patient has undergone ballon plastic surgery of the pulmonary artery valve. At the age of 43 computed tomography detected massive tumor of the heart and mediastinum first diagnosed as sarcoma but later specified as lymphoma. Endobronchial biopsy specimens contained the tissue of diffuse large B-cell lymphoma, the liver and lymph nodes were also involved. After the first course of effective polychemotherapy (CHOP) the patient died of mesenterial thrombosis. Diagnostic difficulties and problems of treatment policy are discussed.

[Capacities of diagnostic volumetric multislice spiral computed tomography in the diagnosis of gastric cancer].

In the period 2009 to 2010, diagnostic multislice spiral computed tomography (MSCT) was carried out in 49 patients with gastric cancer. The efficiency of dynamic MSCT in detecting a tumor and determining its locoregional extent was evaluated. The results of dynamic MSCT were compared with those of postoperative histological examination. The possibility of detecting gastric cancer by dynamic MSCT was ascertained to depend on the depth of tumor invasion and to average 29, 71, 72, and 77% for T1, T2, T3, and T4, respectively. Dynamic MSCT showed a correlation between the frequency of metastatic lesion and the sizes of lymph nodes (r = 0.54; p = 0.0001) and their number (r = 0.82; p < 0.0001).

[Quantitative analysis of coronary artery calcinosis: comparative informative value of multispiral computed tomography and electron-beam tomography].

AIM: To compare informative value (IV) of electron-beam tomography (EBT) and multispiral computed tomography (MSCT) in the assay of coronary artery calcinosis with participation of one group of patients. MATERIAL AND METHODS: EBT and MSCT were conducted in 210 patients whose mean age was 60.6+/-7.7years (67% males, mean age 60.2+/-7.6 years; 33% females, mean age 61.5+/-8.3 years) with a verified diagnosis of ischemic heart disease (IHD). RESULTS: 185 patients had calcium index (CI) from 21 to 2636 units. EBT and MSCT have not detected coronary artery calcinates in 34 patients. In one patient CI was 2 units by EBT and 0 units by MSCT. Mean CI by EBT was 499.4+/-598.2 units, by MSCT--533.7 +/-594.9 units (p = 0.8). The correlation coefficient between IV of the two methods was 0.976 (p < 0.0001). Mean difference between CI by both methods was 34.3 +/-133.6, variation coefficient 22%. There were differences between compatibility of CI in groups of patients with different CI, correlation and variation coefficients in dividing the patients into the subgroups depending on CI. In CI equal to 0-99 units EBT and MSCT correlation coefficient made up 0. 72, coefficient of variation 66%, in CI being 100-399 units these were 0.81 and 27%, in CI of 400-1000 units--0.85 and 16%, in CI exceeding 1000 units--0.95 and 0.4%, respectively. CONCLUSION. High IV correlation was found of MSCT and EBT for coronary artery calcinosis assay. Compatability of the MSCT and EBT data depends on CI

[Multispiral computed tomography in diagnosis of remote complications following surgical interventions in obliterating diseases of lower limb arteries].

Multispiral computed tomography opened new horizons in diagnosis of vascular pathology. The study was aimed at evaluating the significance of MSCT in diagnosis of complications following major and minor surgical interventions for obliterating diseases of the lower-limb arteries, using the findings of the intraoperative revision as a reference method. Each of the 68 examined patients underwent MSCT (4-spiral unit) and duplex scanning. The obtained findings suggest a high informative value of MSCT, hence making it possible to recommend it for use as an alternative means of the routinely used roentgen-contrast angiography.

[The place of multispiral computed tomography in diagnostics of ischemic heart disease.]. / Mesto mul'tispiral'noi komp'iuternoi tomografii v diagnostike ishemicheskoi bolezni serdtsa.

Multispiral computed tomography (CT) of the heart with detection of coronary artery calcification as well as clinical examination, stress tests, single photon emission computer tomography at rest and during stress, and/or stress echocardiography were used in the study of 56 patients with and 26 without ischemic heart disease (mean age 58+/-1 and 55+/-1.5 years, respectively). In 44 patients multispiral CT was performed with intravenous contrast injection and 27 patients were subjected to coronary angiography. Specificity, sensitivity of multispiral CT for detection of hemodynamically significant (>50%) stenoses compared with angiography and for diagnosis of structural changes of the myocardium were 92, 90%, and 89, 91%, respectively. Calcium index was significantly higher in patients with than without ischemic heart disease (253+/-28 and 3+/-1.5 U, respectively) and could be a significant diagnostic criterion of ischemic heart disease.

[Role of computed angiopulmonography in current diagnosis of pulmonary artery thromboembolism]. / Rol' komp'iuternoi angiopul'monografii v sovremennoi diagnostike tromboémbolii legochnoi arterii.

AIM: To study potentialities of tomographic methods in diagnosis of pulmonary artery thromboembolism (PATE). MATERIAL AND METHODS: Electron-beam tomography (EBT) was used for diagnosis in 34 patients (mean age 41.3 +/- 16.5 years) suspected of PATE (Imatron C-150 unit, USA). The procedure included standard examination of the lungs and EBT angiopulmonography in intravenous introduction of the contrast agent (80-100 ml, the rate 3-3.5 ml/s). RESULTS: The pulmonary artery was found to contain thrombi in 56% patients. 18% of PATE patients had pulmonary infarction. A complete coincidence of EBT and scintigraphy was registered in 31% cases, no PATE by both methods was in 19% cases. Angiopulmonography and EBT findings were the same. In 1 case the result of pulmonary scintigraphy was false negative. 24% patients showed symptoms of thrombophlebitis or varicosity of the lower limbs. EBT has detected signs of PATE in 83% of them. CONCLUSION: EBT-angiopulmonography is a precise, highly informative non-invasive method in diagnosis of PATE.