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The Heroic Chamber – an Outlook on the Right Ventricle in Eisenmenger Syndrome

Alecsandra Hernic
Alecsandra Hernic
, 
Roxana Enache
Roxana Enache
, 
Daniela-Noela Radu
Daniela-Noela Radu
, 
Ioan M. Coman
Ioan M. Coman
 y 
Carmen Ginghină
Carmen Ginghină
   | 05 may 2022
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Article Category: Review
Publicado en línea: 05 may 2022
Páginas: 837 - 846
DOI: https://doi.org/10.47803/rjc.2020.31.4.837
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© 2021 Alecsandra Hernic et al., published by Sciendo
This work is licensed under the Creative Commons Attribution 4.0 International License.

Figure 1

Comparison of echocardiographic characteristics in Eisenmenger syndrome secondary to a non-restrictive ventricular septal defect (left column) versus idiopathic pulmonary arterial hypertension (right column) RV, right ventricle. 1A. Transthoracic echocardiography, apical 4-chamber view, 2D examination: adaptive RV hypertrophy with no dilation. 1B. Transthoracic echocardiography, apical 4-chamber view, 2D examination: maladaptive RV hypertrophy and significant dilation. 1C. Transthoracic echocardiography, short-axis view, M-mode examination: adaptive RV hypertrophy with no dilation. 1D. Transthoracic echocardiography, short-axis view, M-mode examination: maladaptive RV hypertrophy and significant dilation. 1E. Transthoracic echocardiography, apical RV-focused view, M-mode examination: normal RV longitudinal function (TAPSE 20mm). 1F. Transthoracic echocardiography, apical RV-focused view, M-mode examination: RV longitudinal dysfunction (TAPSE 15mm). 1G. Transthoracic echocardiography, apical RV-focused view, TDI: normal RV longitudinal function (S’VD 13 cm/s). 1H. Transthoracic echocardiography, apical RV-focused view, TDI: RV longitudinal dysfunction (S’VD 9.5 cm/s). 1I. Transthoracic 2D speckle-tracking echocardiography, apical RV-focused view: mildly reduced RV systolic function (6-segments longitudinal strain −18.7%). 1J. Transthoracic 2D speckle-tracking echocardiography, apical RV-focused view – severe RV systolic dysfunction (6-segments longitudinal strain −8.2%)
Comparison of echocardiographic characteristics in Eisenmenger syndrome secondary to a non-restrictive ventricular septal defect (left column) versus idiopathic pulmonary arterial hypertension (right column) RV, right ventricle. 1A. Transthoracic echocardiography, apical 4-chamber view, 2D examination: adaptive RV hypertrophy with no dilation. 1B. Transthoracic echocardiography, apical 4-chamber view, 2D examination: maladaptive RV hypertrophy and significant dilation. 1C. Transthoracic echocardiography, short-axis view, M-mode examination: adaptive RV hypertrophy with no dilation. 1D. Transthoracic echocardiography, short-axis view, M-mode examination: maladaptive RV hypertrophy and significant dilation. 1E. Transthoracic echocardiography, apical RV-focused view, M-mode examination: normal RV longitudinal function (TAPSE 20mm). 1F. Transthoracic echocardiography, apical RV-focused view, M-mode examination: RV longitudinal dysfunction (TAPSE 15mm). 1G. Transthoracic echocardiography, apical RV-focused view, TDI: normal RV longitudinal function (S’VD 13 cm/s). 1H. Transthoracic echocardiography, apical RV-focused view, TDI: RV longitudinal dysfunction (S’VD 9.5 cm/s). 1I. Transthoracic 2D speckle-tracking echocardiography, apical RV-focused view: mildly reduced RV systolic function (6-segments longitudinal strain −18.7%). 1J. Transthoracic 2D speckle-tracking echocardiography, apical RV-focused view – severe RV systolic dysfunction (6-segments longitudinal strain −8.2%)

Figure 2

Comparison of echocardiographic characteristics in Eisenmenger syndrome secondary to a non-restrictive ventricular septal defect (left column) vs. non-restrictive atrial septal defect (right column) RV, right ventricle. 1A. Transthoracic echocardiography, apical RV-focused view, 2D examination: adaptive RV hypertrophy with no dilation. 1B. Transthoracic echocardiography, apical RV-focused view, 2D examination: maladaptive RV hypertrophy and significant dilation. 1C. Transthoracic echocardiography, short-axis view, M-mode examination: adaptive RV hypertrophy with no dilation. 1D. Transthoracic echocardiography, short-axis view, M-mode examination: maladaptive RV hypertrophy and significant dilation. 1E. Transthoracic echocardiography, apical RV-focused view, M-mode examination: normal RV longitudinal function (TAPSE 21mm). 1F. Transthoracic echocardiography, apical RV-focused view, M-mode examination: mild RV longitudinal dysfunction (TAPSE 17mm). 1G. Transthoracic echocardiography, apical RV-focused view, TDI: normal RV longitudinal function (S’VD 12.6 cm/s). 1H. Transthoracic echocardiography, apical RV-focused view, TDI: mild RV longitudinal dysfunction (S’VD 10.4 cm/s). 1I. Transthoracic 2D speckle-tracking echocardiography, apical RV-focused view: mildly reduced RV systolic function (6-segments longitudinal strain −18%). 1J. Transthoracic 2D speckle-tracking echocardiography, apical RV-focused view: severe RV systolic dysfunction (6-segments longitudinal strain −5.9%)
Comparison of echocardiographic characteristics in Eisenmenger syndrome secondary to a non-restrictive ventricular septal defect (left column) vs. non-restrictive atrial septal defect (right column) RV, right ventricle. 1A. Transthoracic echocardiography, apical RV-focused view, 2D examination: adaptive RV hypertrophy with no dilation. 1B. Transthoracic echocardiography, apical RV-focused view, 2D examination: maladaptive RV hypertrophy and significant dilation. 1C. Transthoracic echocardiography, short-axis view, M-mode examination: adaptive RV hypertrophy with no dilation. 1D. Transthoracic echocardiography, short-axis view, M-mode examination: maladaptive RV hypertrophy and significant dilation. 1E. Transthoracic echocardiography, apical RV-focused view, M-mode examination: normal RV longitudinal function (TAPSE 21mm). 1F. Transthoracic echocardiography, apical RV-focused view, M-mode examination: mild RV longitudinal dysfunction (TAPSE 17mm). 1G. Transthoracic echocardiography, apical RV-focused view, TDI: normal RV longitudinal function (S’VD 12.6 cm/s). 1H. Transthoracic echocardiography, apical RV-focused view, TDI: mild RV longitudinal dysfunction (S’VD 10.4 cm/s). 1I. Transthoracic 2D speckle-tracking echocardiography, apical RV-focused view: mildly reduced RV systolic function (6-segments longitudinal strain −18%). 1J. Transthoracic 2D speckle-tracking echocardiography, apical RV-focused view: severe RV systolic dysfunction (6-segments longitudinal strain −5.9%)

Diagnostic work-up in Eisenmenger Syndrome (adapted from [4])

Clinical evaluation SymptomsShortness of breath at rest/on exertionLimited exercise capacityPalpitationsHaemoptysisAnginaHeadacheDizziness Syncope/presyncope Physical examinationWeightResting SpO2%Blood pressureCyanosisDigital clubbingHeart rate, arrhythmiaSystemic congestion: oedema, jugular vein distension, hepatomegaly
ECG Presence of sinus rhythmHeart rateSupraventricular/ventricular arrhythmiasConduction abnormalities (right bundle branch block/atrioventricular block)RV/biventricular hypertrophy Holter monitoring may be considered in case of syncope, palpitations, baseline ECG abnormalities
Non-invasive imaging Chest X-ray Position of cardiac apex (RV hypertrophy)Cardiothoracic ratioPosition of aortic arch (left/right)Pulmonary outflow tract dilation/calcificationDilation of pulmonary arteriesPruning of peripheral pulmonary vesselsPleural/pericardial effusion
TTE Systematic analysis of cardiac morphology and ventriculo-arterial connectionsDescription of the shunt (location, direction, haemodynamic significance)RV and LV dimensions, systolic and diastolic functionLV eccentricity indexRA dimensions and areaPresence of pericardial effusionEstimation of PAP and RVEDP
TEE (unanswered questions on TTE) Shunt descriptionVentricular functionCo-existing valve diseaseCo-existing morphologic anomalies (i.e. anomalous pulmonary venous drainage)Suspicion of complications (intracardiac thrombosis/endocarditis)
CMR (complex lesions/inadequate patient echogenicity) Detailed description of cardiac morphologyShunt description and quantificationQuantification of RV volumes and RVEFRV fibrosis (LGE)
Non-invasive imaging CT (specific indications) Pulmonary artery diameters/calcificationPulmonary artery in situ thrombosisCompression of left main stem in case of pulmonary artery aneurysmSource of haemoptysis
Exercise testing 6MWD Systematic at baseline and follow-up visits
Cardiopulmonary exercise testing Exercice capacityVO2 max %
Cardiac catheterization Confirmation of diagnosis and haemodynamic assessment–right atrial pressure, sPAP, mPAP, dPAP, capillary wedge pressure, pulmonary vascular resistances cardiac output, SVO2 %, pulmonary-to-systemic flow ratio Differential diagnosis: ES, PAH with left-to-right shunt, iPAH, segmental PH
Biomarkers Full blood countRenal functionHepatic testsCoagulation panelNT-proBNPUric acidCRPSerum iron, ferritin, total iron binding capacity, transferrin saturation coefficientFolic acid, vitamin B12
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Medicine, Clinical Medicine, Internal Medicine, Cardiology, Pediatrics and Juvenile Medicine, Paediatric Cardiology, Surgery, Cardiac Surgery
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