Post-COVID-19 syndrome: morpho-functional abnormalities of the heart and arrhythmias

Aim. To study the myocardial morpho-functional abnormalities, the incidence and nature of cardiac arrhythmias in patients 3 months after the coronavirus disease 2019 (COVID-19).Material and methods. The study included 77 patients (mean age, 35,9 years) treated for coronavirus infection, which underwent echocardiography and 24-hour Holter monitoring 3 months after COVID-19. The patients were divided into 3 groups: group 1 — 31 patients with upper respiratory tract involvement; group 2 — 27 patients with bilateral pneumonia (CT grade 1, 2), 3 — 19 patients with severe pneumonia (CT grade 3, 4). Statistical processing was carried out using Statistica 10.0.Results. According to echocardiography, the peak tricuspid late diastolic velocity and isovolumetric contraction time in all groups increased (P<0,001). The tricuspid and mitral Em/Am ratio decreased depending on the disease severity. In group 3, the right ventricular and atrial size increased (P<0,001). The pulmonary artery systolic pressure, left atrial volume in patients of the 2nd and 3rd groups was higher than in the control one (P<0,001). In group 1 and 2 patients, the regional strain in basal and basal/middle segments decreased, respectively, while, in group 3, not only regional but also global left ventricular (LV) strain decreased (P<0,001). In all groups, cardiac arrhythmias and pericardial effusion were found. The relationship was established between coronavirus activity and the structural and functional myocardial parameters (P<0,001).Conclusion. Cardiovascular injury 3 months after COVID-19 was found in 71%, 93%, and 95% of patients with mild, moderate and severe course. In mild course patients, a decrease in regional myocardial strain in LV basal segments, signs of past pericarditis, and various cardiac arrhythmias were noted. In patients of moderate severity, these changes were more pronounced and were accompanied by an additional decrease in regional strain in LV middle segments, impaired right ventricular diastole and increased pulmonary artery pressure. In severe patients, in addition to the above changes, dilatation of the right heart and inferior vena cava was recorded, as well as LV diastolic and global systolic function decreased.

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Norepinephrine and dobutamine improve cardiac index equally by supporting opposite sides of the heart in an experimental model of chronic pulmonary hypertension

10.21203/rs.3.rs-458360/v1 ◽

2021 ◽

Author(s):

Janus Adler Hyldebrandt ◽

Nikolaj Bøgh ◽

Camilla Omann Christensen ◽

Peter Agger

Keyword(s):

Pulmonary Hypertension ◽

Cardiac Index ◽

Pulmonary Artery ◽

Right Ventricular ◽

Left Ventricular ◽

Ventricular Contractility ◽

End Diastolic Volume ◽

Chronic Pulmonary Hypertension ◽

Blood Pressures ◽

The Right

Abstract Background: Pulmonary hypertension is a significant risk factor in patients undergoing surgery. The combined effects of general anaesthesia and positive pressure ventilation can aggravate this condition and cause increased pulmonary blood pressures, reduced systemic blood pressures and ventricular contractility. Although perioperative use of inotropic support or vasopressors is almost mandatory for these patients, preference is disputed. In this study, we investigated the effects of norepinephrine and dobutamine and their ability to improve the arterio-ventricular relationship and haemodynamics in pigs suffering from chronic pulmonary hypertension.Method: Pulmonary hypertension was induced in five pigs by banding the pulmonary artery at 2–3 weeks of age. Six pigs served as controls. After 16 weeks of pulmonary artery banding, the animals were re-examined under general anaesthesia using biventricular conductance catheters and a pulmonary artery catheter. After baseline measurements, the animals were exposed to both norepinephrine and dobutamine infusions in incremental doses, with a stabilizing period in between the infusions. The hypothesis of differences between norepinephrine and dobutamine with incremental doses was tested using repeated two-way ANOVA and Bonferroni multiple comparisons post-test. Results: At baseline, pulmonary artery banded animals had increased right ventricular pressure (+39%, p=0.04), lower cardiac index (-23% p=0.04), lower systolic blood pressure (-13%, p=0.02) and reduced left ventricular end-diastolic volume (-33%, p=0.02). When incremental doses of norepinephrine and dobutamine were administered, the right ventricular arterio-ventricular coupling was improved only by dobutamine (p<0.05). Norepinephrine increased both left ventricular end-diastolic volume and left ventricular contractility to a greater extent (p<0.05) in pulmonary artery banded animals. While the cardiac index was improved equally by norepinephrine and dobutamine treatments in pulmonary artery banded animals, norepinephrine had a significantly greater effect on mean arterial pressure (p<0.05) and diastolic arterial pressure (p<0.05).Conclusion: While norepinephrine and dobutamine improved cardiac index equally, it was obtained in different manners. Dobutamine significantly improved the right ventricular function and the arterio-ventricular coupling. Norepinephrine increased systemic resistance, thereby improving arterial pressures and left ventricular systolic function by maintaining left ventricular end-diastolic volume.

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Icariin Attenuates Monocrotaline-Induced Pulmonary Arterial Hypertension via the Inhibition of TGF-β1/Smads Pathway in Rats

Evidence-based Complementary and Alternative Medicine ◽

10.1155/2020/9238428 ◽

2020 ◽

Vol 2020 ◽

pp. 1-8

Author(s):

Yijia Xiang ◽

Changhong Cai ◽

Yonghui Wu ◽

Lebing Yang ◽

Shiyong Ye ◽

...

Keyword(s):

Pulmonary Arterial Hypertension ◽

Pulmonary Artery ◽

Arterial Hypertension ◽

Right Ventricular ◽

Left Ventricular ◽

Matrix Metalloproteinase 2 ◽

Western Blots ◽

Pulmonary Arterial ◽

The Right ◽

Tgf Β1

Background. Pulmonary artery remodeling is important in the development of pulmonary artery hypertension. The TGF-β1/Smads signaling pathway is activated in pulmonary arterial hypertension (PAH) in rats. Icariin (ICA) suppresses the TGF-β1/Smad2 pathway in myocardial fibrosis in rats. Therefore, we investigated the role of icariin in PAH by inhibiting the TGF-β1/Smads pathway. Methods. Rats were randomly divided into control, monocrotaline (MCT), MCT + ICA-low, and MCT + ICA-high groups. MCT (60 mg/kg) was subcutaneously injected to induce PAH, and icariin (50 or 100 mg/kg.d) was orally administered for 2 weeks. At the end of the fourth week, right ventricular systolic pressure (RVSP) was obtained and the right ventricular hypertrophy index (RI) was determined as the ratio of the right ventricular weight to the left ventricular plus septal weight (RV/LV + S). Western blots were used to determine the expression of TGF-β1, Smad2/3, P-Smad2/3, and matrix metalloproteinase-2 (MMP2) in lung tissues. Results. Compared to the control group, RVSP and RI were increased in the MCT group (ρ < 0.05). Additionally, TGF-β1, Smad2/3, P-Smad2/3, and MMP2 expressions were obviously increased (ρ < 0.01). Compared to the MCT group, RVSP and RI were decreased in the MCT + ICA group (ρ < 0.05). TGF-β1, Smad2/3, P-Smad2/3, and MMP2 expressions were also inhibited in the icariin treatment groups (ρ < 0.05). Conclusions. Icariin may suppress MCT-induced PAH via the inhibition of the TGFβ1-Smad2/3 pathway.

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EVALUATION OF THE RELATIONSHIP BETWEEN PULMONARY ARTERY STIFFNESS AND RIGHT VENTRICULAR FUNCTION IN PATIENTS WITH BEHÇET’S DISEASE USING TRANSTHORACIC ECHOCARDIOGRAPHY

10.22541/au.163615894.48445737/v1 ◽

2021 ◽

Author(s):

Salim Yaşar ◽

Murat Unlu ◽

suat gormel ◽

Emre Yalcinkaya ◽

Baris BUGAN ◽

...

Keyword(s):

Pulmonary Artery ◽

Right Ventricular ◽

Patient Group ◽

Behcet’S Disease ◽

Left Ventricular ◽

Significant Difference ◽

Artery Stiffness ◽

The Right ◽

The Relationship ◽

Ventricular Functions

Background: Behçet’s disease is a systemic vasculitis that can affect all sizes of arteries and veins. Arterial stiffness is a term used to describe the visco-elastic properties of vessel wall. In this study, we aimed to evaluate the relationship between pulmonary artery stiffness (PAS) and the right ventricular (RV) functions in asymptomatic Behçet’s patients with no cardiovascular risk factors. Methods: We studied 40 patients who were diagnosed by the international diagnostic criteria of Behçet’s disease and 40 healthy individuals who were matching demographic properties with the patients. Two groups were matched by age, gender, clinical history and other clinical features. Substantial medical history concerning the factors that can affect right ventricle diastolic function (such as medications, smoking status, other comorbities, etc.) was taken and general physical examination was carried out. The right and left ventricular functions as well as valvular functions were evaluated by using echocardiography. Also Two-dimensional, M-mode, pulsed wave (PW) Doppler echocardiographic parameters were measured for right ventricular functions. PW Doppler flow trace was obtained from the pulmonary valve with regards to pulmonary artery stiffness. Results: There was no significant difference in terms of clinical and demographic properties. No statistically significant difference was found upon comparison of the left ventricular end of systole and diastole diameters, the diastolic and systolic thicknesses of the interventricular septum (IVS) and left ventricular posterior wall (LVPW), left atrium (LA) diameter and left ventricular ejection fraction (LVEF) values (p>0.05) of the two groups. Right ventricular myocardial performance index (MPI) value was found higher in Behçet’s patients and a statistically significant difference was detected between the groups (p<0.01). Tricuspid annular plane systolic excursion (TAPSE) values were found to be statistically significantly lower in the patient group as compared to the control group (p<0.01). In PW Doppler measurements, early passive filling (E) wave flow velocity and E/A ratio were found to be statistically significantly lower, deceleration time (DT) was higher in the patient group (p<0.01). In Behçet’s patients without clinical pulmonary involvement, the pulmonary artery systolic pressure (PASP) was found to be statistically significantly higher in the patient group (p<0.01). The values of pulmonary artery stiffness (PAS) were found to be significantly higher in the patient group (p<0.01). The relationship between the right ventricular function markers and PAS were evaluated in the patient group. There was no statistically significant relationship between PAS and MPI and TAPSE. But there was a significant correlation between PAS and PASP and duration of illness (p<0.001 and r=0.682 ; p=0.047 and r=0,316). Conclusion: Behçet’s patients without cardiac symptoms and signs, reduction in right ventricular functions and increase in PAS was detected. Although there is no correlation between right ventricular functions and PAS, increased PAS may be an early marker of reduction of the right ventricular functions. Consequently, routine cardiological examination and detailed evaluation of biventricular functions by using echocardiography should be greatly beneficial in Behcet’s patients, even though there are no signs or symptoms.

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Echocardiographic assessment of right heart indices in dogs with elevated pulmonary artery pressure associated with chronic respiratory disorders, heartworm disease, and chronic degenerative mitral valvular disease

Veterinární Medicína ◽

10.17221/7180-vetmed ◽

2013 ◽

Vol 58 (No. 12) ◽

pp. 613-620 ◽

Cited By ~ 7

Author(s):

HP Tai TC Huang

Keyword(s):

Pulmonary Artery ◽

Right Ventricular ◽

Aortic Root ◽

Peak Velocity ◽

Root Diameter ◽

Left Ventricular ◽

Right Heart ◽

Respiratory Disorders ◽

Heartworm Disease ◽

The Right

The aim of this study was to evaluate right ventricular (RV) remodeling and right heart failure associated with different causes of elevated pulmonary arterial pressure (PAP) in dogs. In total, 169 client-owned dogs with tricuspid regurgitation (TR) and 40 client-owned clinically healthy dogs were included. Dogs with TR were further categorised as suffering from chronic respiratory disorders (CRD), heartworm disease (HWD), mild/moderate chronic degenerative mitral valvular disease (CDMD), and severe CDMD. Among the echocardiographic indices of the right heart, no significant difference in the tricuspid annular plane systolic excursion to aortic root diameter ratio was found among the clinical healthy controls and the four different TR categories. No significant differences in TR peak velocity and pulmonic regurgitation peak velocity were found among dogs in the four different TR categories. The ratio of the right to left ventricular basal diameter in the right ventricular-focused view was significantly higher in dogs with chronic respiratory disorders and dogs with heartworms. Left ventricular compression quantified using the eccentricity index and the ratio of the main pulmonary artery to aortic root diameter were significantly higher in dogs with HWD. Their right ventricular acceleration to ejection time (AT/ET) was significantly lower in dogs with HWD. Based on these findings, we conclude that right heart indices are affected by CRD, HWD, and moderate to severe CDMD. However, right heart indices derived from left heart measurements might be underestimated in dogs with CDMD.

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P885 Pulmonary artery stent implantation in an adult with chronic thromboembolic pulmonary stenosis

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jez319.525 ◽

2020 ◽

Vol 21 (Supplement_1) ◽

Author(s):

E A Khalifa ◽

S Helmy ◽

F Elallus ◽

S F Mohamed ◽

M Alkuwari

Keyword(s):

Pulmonary Hypertension ◽

Pulmonary Artery ◽

Right Ventricular ◽

Stent Implantation ◽

Pulmonary Valve ◽

Main Pulmonary Artery ◽

Left Ventricular ◽

Artery Stenosis ◽

Pulmonary Artery Stenosis ◽

The Right

Abstract Introduction Pulmonary artery stenosis presenting in adults is rare. Chronic thromboembolic pulmonary hypertension (CTEPH) is by far the most common cause of pulmonary artery stenosis. Stenosis in these patients are not caused by an abnormality of the arterial wall itself, but by intraluminal narrowing as a result of the only partially resolved and organized thromboembolism. In contrast to paediatric patients, in adults with pulmonary artery stenosis, pulmonary stenting is not routinely performed. Case report A 51-year male, smoker, diabetic, hypertensive, and with chronic kidney disease. He was diagnosed two years earlier with bilateral multiple pulmonary emboli and was maintained on oral anticoagulation therapy. Recently, he presented with gradually progressive shortness of breath and signs of right ventricular failure. Diagnostic imaging: 1-Transthoracic and transesophageal echocardiography showed normal global systolic left ventricular function with no regional wall motion abnormalities, dilated right ventricle (RV) with moderately impaired function, severe pulmonic valve incompetence, mild tricuspid incompetence and a severely elevated right ventricular systolic pressure (RVSP) of 82 mmHg. In addition, a small rounded mass (6 x 11 mm) was visualized attached to the posterior wall of the RV outflow tract (RVOT) about 15mm proximal to the pulmonary valve annulus, (figure A). 2- Computed tomography pulmonary angiography showed a right main pulmonary artery (RPA) with circumferential narrowing, which was highly suggestive of chronic thrombosis. There was an abrupt tapering noted in the segmental branches of the right lower lobar pulmonary artery, with non-opacification of the distal arteries. No contrast opacification was noted in the right upper lobe pulmonary arteries. The left main pulmonary artery showed thickening of its bifurcation, again suggestive of chronic thrombosis, with narrowing of its left upper lobar branch, (figures B&C). 3-Cardiac magnetic resonance (CMR) showed a non enhancing RVOT mass protruding through the incompetent pulmonary valve during systole with features suggestive of a thrombus. Management In view of the clinical history, CTEPH was considered to be the most likely aetiology of the pulmonary hypertension. The decision was to perform balloon angioplasty and stent implantation in the RPA. Immediately after the procedure, RVSP was reduced from 80 to 50 mmHg. The clinical course after this procedure was uncomplicated and the patient showed significant clinical improvement. Follow up CMR showed patent stent with improvement of RV function ( fig D) Abstract P885 Figure.

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Norepinephrine and dobutamine improve cardiac index equally by supporting opposite sides of the heart in an experimental model of chronic pulmonary hypertension

Intensive Care Medicine Experimental ◽

10.1186/s40635-021-00391-x ◽

2021 ◽

Vol 9 (1) ◽

Author(s):

Janus Adler Hyldebrandt ◽

Nikolaj Bøgh ◽

Camilla Omann ◽

Peter Agger

Keyword(s):

Pulmonary Hypertension ◽

Cardiac Index ◽

Pulmonary Artery ◽

Right Ventricular ◽

Left Ventricular ◽

Ventricular Contractility ◽

End Diastolic Volume ◽

Chronic Pulmonary Hypertension ◽

Blood Pressures ◽

The Right

Abstract Background Pulmonary hypertension is a significant risk factor in patients undergoing surgery. The combined effects of general anaesthesia and positive pressure ventilation can aggravate this condition and cause increased pulmonary blood pressures, reduced systemic blood pressures and ventricular contractility. Although perioperative use of inotropic support or vasopressors is almost mandatory for these patients, preference is disputed. In this study, we investigated the effects of norepinephrine and dobutamine and their ability to improve the arterio-ventricular relationship and haemodynamics in pigs suffering from chronic pulmonary hypertension. Method Pulmonary hypertension was induced in five pigs by banding the pulmonary artery at 2–3 weeks of age. Six pigs served as controls. After 16 weeks of pulmonary artery banding, the animals were re-examined under general anaesthesia using biventricular conductance catheters and a pulmonary artery catheter. After baseline measurements, the animals were exposed to both norepinephrine and dobutamine infusions in incremental doses, with a stabilising period in between the infusions. The hypothesis of differences between norepinephrine and dobutamine with incremental doses was tested using repeated two-way ANOVA and Bonferroni multiple comparisons post-test. Results At baseline, pulmonary artery-banded animals had increased right ventricular pressure (+ 39%, p = 0.04), lower cardiac index (− 23% p = 0.04), lower systolic blood pressure (− 13%, p = 0.02) and reduced left ventricular end-diastolic volume (− 33%, p = 0.02). When incremental doses of norepinephrine and dobutamine were administered, the right ventricular arterio-ventricular coupling was improved only by dobutamine (p < 0.05). Norepinephrine increased both left ventricular end-diastolic volume and left ventricular contractility to a greater extent (p < 0.05) in pulmonary artery-banded animals. While the cardiac index was improved equally by norepinephrine and dobutamine treatments in pulmonary artery-banded animals, norepinephrine had a significantly greater effect on mean arterial pressure (p < 0.05) and diastolic arterial pressure (p < 0.05). Conclusion While norepinephrine and dobutamine improved cardiac index equally, it was obtained in different manners. Dobutamine significantly improved the right ventricular function and the arterio-ventricular coupling. Norepinephrine increased systemic resistance, thereby improving arterial pressures and left ventricular systolic function by maintaining left ventricular end-diastolic volume.

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Comparison of Doppler Flow Tei-Indexes With Pulmonary Artery Thermodilution Measurement of Cardiac Output in an Experimental Porcine Model

Physiological Research ◽

10.33549/physiolres.932015 ◽

2011 ◽

pp. 483-492 ◽

Cited By ~ 1

Author(s):

J. KOBR ◽

V. TŘEŠKA ◽

J. MOLÁČEK ◽

V. KUNTSCHER ◽

V. LIŠKA ◽

...

Keyword(s):

Cardiac Output ◽

Pulmonary Artery ◽

Right Ventricular ◽

Left Ventricular ◽

Average Weight ◽

Ventricular Afterload ◽

Echocardiographic Examination ◽

The Right ◽

Right Ventricular Preload ◽

Thermodilution Measurement

The objective of our study was to compare Doppler echocardiography imaging with pulmonary artery thermodilution measurement during mechanical ventilation. Total 78 piglets (6 weeks old, average weight 24 kg, under general anesthesia) were divided into 4 groups under different cardiac loading conditions (at rest, with increased left ventricular afterload, with increased right ventricular preload, and with increased afterload of both heart ventricles). At 60 and 120 min the animals were examined by echocardiography and simultaneously pulmonary artery thermodilution was used to measure cardiac output. Tei-indexes data were compared with invasively monitored hemodynamic data and cardiac output values together with calculated vascular resistance indices. A total of 224 parallel measurements were obtained. Correlation was found between values of right Tei-index of myocardial performance and changes in right ventricular preload (p<0.05) and afterload (p<0.01). Significant correlation was also found between left index values and changes of left ventricular preload (p<0.001), afterload (p<0.001), stroke volume (p<0.01), and cardiac output (p<0.01). In conclusion, echocardiographic examination and determination of the global performance selectively for the right and left ventricle can be recommended as a suitable non-invasive supplement to the whole set of methods used for monitoring of circulation and cardiac performance.

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Echocardiographic parameters as predictors of three-months mortality in patients with 2019 Coronavirus infection

European Heart Journal ◽

10.1093/eurheartj/ehab724.0137 ◽

2021 ◽

Vol 42 (Supplement_1) ◽

Author(s):

T Miranda Aquino ◽

J M Pereira-Forcado ◽

B Ordonez-Salazar ◽

G Dominguez-Trejo ◽

A Rangel-Guerra ◽

...

Keyword(s):

Pulmonary Artery ◽

Right Ventricular ◽

Longitudinal Strain ◽

Clinical Laboratory ◽

Systolic Pressure ◽

Left Ventricular ◽

Ventricular Dilation ◽

Echocardiographic Parameters ◽

The Right ◽

Right Ventricular Dilation

Abstract Background Coronavirus disease 2019 is a systemic entity, where cardiac involvement has been described. The echocardiogram is a diagnostic tool that describes myocardial damage with good certainty. Objectives Determine which echocardiographic parameters are predictors of mortality. Analyze if there is a difference in clinical, laboratory and echocardiographic variables in terms of patients who died versus those who survived. Investigate the cut-off point of the echocardiographic parameters that is best associated with mortality. Methods Prospective, analytical, comparative study. Patients admitted to the hospital with Coronavirus 2019 infection. Clinical, laboratory and echocardiographic variables will be assessed. The association with three-month mortality of the different variables will be determined. We used ROC-curves for the best cut-off associated with mortality. The association with three-month mortality was analized using Cox regression, unadjusted analysis of the variables was performed, as well as adjusted analysis for age and gender. Results 84 patients were included, a mortality of 29% was documented. Significant differences were found in the left atrial volumen index, the E/e', the proportion of dilatation of the right ventricle and diastolic dysfunction. Tricuspid annulus anterior systolic excursion (TAPSE), pulmonary artery acceleration time (PAA), tricuspid regurgitation velocity (TRV), pulmonary artery systolic pressure (PASP), left ventricular longitudinal strain (LVGLS), of the left atrium (LAGLS) and the right ventricular free wall longitudinal strain (RVFWLS). Right ventricular dilation, right ventricular shortening fraction, TAPSE, PASP, TRV, LVGLS, LAGLS, and RVFWLS were associated with mortality. Conclusion Right ventricular dilation, right ventricular shortening fraction, TAPSE, PASP, TRV, LVGLS, LAGLS, and RVFWLS are the echocardiographic parameters that were associated with three-month mortality. FUNDunding Acknowledgement Type of funding sources: None. Table 2

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Hemodynamic Support Using Percutaneous Transfemoral Impella 5.0 and Impella RP for Refractory Cardiogenic Shock

Case Reports in Cardiology ◽

10.1155/2019/4591250 ◽

2019 ◽

Vol 2019 ◽

pp. 1-6 ◽

Cited By ~ 1

Author(s):

Pratik K. Dalal ◽

Amy Mertens ◽

Dinesh Shah ◽

Ivan Hanson

Keyword(s):

Left Ventricle ◽

Pulmonary Artery ◽

Cardiogenic Shock ◽

Ventricular Function ◽

Standard Of Care ◽

Left Ventricular ◽

Biventricular Failure ◽

Ventricular Support ◽

The Right ◽

Flow Pump

Acute myocardial infarction (AMI) resulting in cardiogenic shock continues to be a substantial source of morbidity and mortality despite advances in recognition and treatment. Prior to the advent of percutaneous and more durable left ventricular support devices, prompt revascularization with the addition of vasopressors and inotropes were the standard of care in the management of this critical population. Recent published studies have shown that in addition to prompt revascularization, unloading of the left ventricle with the placement of the Impella percutaneous axillary flow pump can lead to improvement in mortality. Parameters such as the cardiac power output (CPO) and pulmonary artery pulsatility index (PAPi), obtained through pulmonary artery catheterization, can help ascertain the productivity of right and left ventricular function. Utilization of these parameters can provide the information necessary to escalate support to the right ventricle with the insertion of an Impella RP or the left ventricle with the insertion of larger devices, which provide more forward flow. Herein, we present a case of AMI complicated by cardiogenic shock resulting in biventricular failure treated with the percutaneous insertion of an Impella RP and Impella 5.0 utilizing invasive markers of left and right ventricular function to guide the management and escalation of care.

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Hypertrophy of unaffected cardiomyocytes correlates with severity of cardiomyopathy in female patients with Fabry disease

Orphanet Journal of Rare Diseases ◽

10.1186/s13023-021-01803-7 ◽

2021 ◽

Vol 16 (1) ◽

Author(s):

Cristina Chimenti ◽

Romina Verardo ◽

Andrea Frustaci

Keyword(s):

Fabry Disease ◽

Wall Thickness ◽

Left Ventricular ◽

Female Patients ◽

Maximal Wall Thickness ◽

Group 4 ◽

Group 3 ◽

Group 2 ◽

Group 1

Abstract Aim To investigate the contribution of unaffected cardiomyocytes in Fabry disease cardiomyopathy. Findings Left ventricular (LV) endomyocardial biopsies from twenty-four females (mean age 53 ± 11 ys) with Fabry disease cardiomyopathy were studied. Diagnosis of FD was based on the presence of pathogenic GLA mutation, Patients were divided in four groups according with LV maximal wall thickness (MWT): group 1 MWT ≤ 10.5 mm, group 2 MWT 10.5–15 mm, group 3 MWT 16–20 mm, group 4 MWT > 20 mm. At histology mosaic of affected and unaffected cardiomyocytes was documented. Unaffected myocytes’ size ranged from normal to severe hypertrophy. Hypertrophy of unaffected cardiomyocytes correlated with severity of MWT (p < 0.0001, Sperman r 0,95). Hypertrophy of unaffected myocytes appear to concur to progression and severity of FDCM. It is likely a paracrine role from neighboring affected myocytes.

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