Figures
- FIGURE 1
Number of clinical publications on the use of evaluation modalities in humans with pulmonary hypertension (PH), as indexed in PubMed between 1958 and 2018 (unadjusted, searched in October 2019). RHC: right-heart catheterisation; ECHO: echocardiography; CT: computed tomography; CMR: cardiovascular magnetic resonance.
Tables
- TABLE 1
World Health Organization (WHO) groups of pulmonary hypertension (PH) and cardiovascular magnetic resonance (CMR)
Group 1 Group 2 Group 3 Group 4 Group 5 PH category PAH Pulmonary venous hypertension due to left-heart disease PH due to lung disease PH due to chronic thromboembolism PH due to multifactorial or miscellaneous cause Major causes Idiopathic
Heritable
CTD
HIV
Drug/toxin induced
Shunts (CHD)Left heart disease (left-ventricular systolic and diastolic dysfunction)
Valvular heart disease (mitral, aortic)ILD
COPD
Sleep apnoeaChronic thromboembolism Myeloproliferative disorders
Sarcoidosis
Glycogen storage disease
Chronic haemolytic anaemia
Thyroid disease
Chronic kidney diseaseDiagnostic criteria by RHC Mean PAP >20 mmHg
PAWP ≤15 mmHg
PVR ≥3 Wood unitsMean PAP >20 mmHg
PAWP >15 mmHg
PVR <3 or ≥3 Wood unitsMean PAP >20 mmHg
PAWP ≤15 mmHg
PVR ≥3 Wood unitsMean PAP >20 mmHg
PAWP ≤15 mmHg
PVR ≥3 Wood unitsPresence of both precapillary and/or postcapillary PH
Mean PAP >20 mmHg; PAWP ≤15 mmHg; PVR ≥3 Wood units
or
Mean PAP >20 mmHg; PAWP >15 mmHg; PVR <3 or ≥3 Wood unitsMajor diagnostic findings by CMR Aberrant pulmonary veins
ShuntsLeft ventricular hypertrophy
Left ventricular dysfunction
Dilated left atrium
Valvular disease
Pulmonary vein stenosisGross fibrosis of the lung parenchyma Thrombus in the pulmonary vessel Lymphadenopathy
Absence of spleen
Focal LGE (sarcoidosis)Essential diagnostic CMR modality Dedicated cardiac views
Flow mapping (phase contrast flow velocity and/or 4-D flow analysis)
MRAShort and/or long axis views cine SSFP
LGE
Valve lesion quantificationCine SSFP
Flow (phase contrast flow velocity or flow analysis)
MRAMRA LGE CMR prognostic focus Short and/or long axis views cine SSFP
Right ventricular function
Right ventricle–pulmonary artery couplingPAH: pulmonary arterial hypertension; CTD: connective tissue disorder; CHD: congenital heart disease; ILD: interstitial lung disease; COPD: chronic obstructive pulmonary disease; RHC: right-heart catheterisation; PAP: pulmonary arterial pressure; PAWP: pulmonary artery wedge pressure; PVR: pulmonary vascular resistance; LGE: late gadolinium enhancement; MRA: magnetic resonance angiography; SSFP: steady-state free procession.
- TABLE 2
Sequential right-sided changes due to pulmonary hypertension (PH) detected by cardiovascular magnetic resonance (CMR) modalities
Physiologic changes assessed by CMR CMR sequence Stiffening of the pulmonary artery with gradual increase in pressure at rest, leading to worsening of pulmonary artery compliance and reduced RAC Cine
Velocity-encoded phase contrastRight ventricular hypertrophy, progressive right ventricular dilation with leftward shift in IVS or D-septum Cine
Strain imaging
LGE
Adenosine stress perfusion to assess cardiac ischaemiaTricuspid regurgitation due to right ventricular dilation and valve annulus stretch Cine
Velocity-encoded phase contrastIncreased right ventricular pressure leading to a dilated right ventricle with leftward shift of the interatrial septum LGE imaging for myocardial scar Engorged vena cavae with pericardial effusion Cine cardiac structure/function
T1-weighted fast spin echo without fat suppression to assess pericardium
Myocardial oedema imaging with T2 weighted sequencesRAC: relative area change; IVS: interventricular septum; LGE: late gadolinium enhancement.
- TABLE 3
Pulmonary arterial hypertension (PAH) studies, both complete and ongoing, utilising cardiovascular magnetic resonance (CMR) endpoints
Study Type of study Patients n Aim CMR endpoints Key results summary Van Wolferen [10] (2007) Prospective observational study 64 Investigate the relationship between right ventricular structure, function and survival in IPAH CI RVSVI RVMI LVMI RVWT LVWT RVEF LVEF RVEDVI LVEDVI The following are strong independent predictors of mortality and treatment failure: large right ventricular volume; low stroke volume; reduced left ventricular volume Van de Veerdonk [8] (2011) Prospective observational study 110 Examine the relationship between changes in PVR, RVEF and survival in patients with PAH under PAH-targeted therapies RVEDVI RVESVI RVEF LVEDVI LVESVI LVEF RVSVI Loss of right ventricular function is associated with a poor outcome, irrespective of any changes in PVR Wilkins [103] (2005), SERAPH Randomised controlled trial 26 Compare the effect of adding sildenafil, a PDE-5i, to bosentan, an ERA (and the conventional treatment) in PAH RVM RVM decreased with addition of sildenafil to bosentan (mean −8.8 g versus −3 g; p=0.142) Wilkins [104] (2010), SiPHT Randomised controlled trial 42 Assess the therapeutic value of simvastatin in patients with PAH RVM Simvastatin added to conventional therapy produced a small and transient early reduction in RVM but this was not sustained over 12 months Roeleveld [92] (2004) Prospective observational study 11 Assess changes in main pulmonary artery flow, as well as RVM and volumes, in patients with PH during epoprostenol therapy RVM RVEDV No significant changes noted in either parameter Van Wolferen [105] (2006) Prospective observational study 15 Investigate whether the addition of sildenafil reverses right ventricular remodelling and improvement in right ventricular function in PAH treated with bosentan RVM RVEF RVEDV Addition of Sildenafil to bosentan significantly decreased RVM (p<0.01) and increased RVEF (p<0.05) with no significant change in RVEDV Chin [11] (2008) Prospective observational study 16 Investigate changes in CMR measurements in PAH treated with bosentan and compare cardiac MRI results with other known markers of prognosis, including RHC and 6 MWT RVEF RVEDV RVSV No significant changes noted with bosentan therapy Peacock [91] (2014), EURO-MR Prospective observational study 91 Assessment of right ventricular function using CMR to determine response to therapy (ERA or PDE-5i) and to monitor disease progression in PH RVEDVI RVSVI RVEF RVMI Significant improvement noted in RVEF (p<0.001) and RVSVI (p<0.0001) Van de Veerdonk [106] (2017) Retrospective study 80 Assess the impact of upfront combination therapy (ERA+PDE-5i) on right ventricular volume compared with monotherapy (ERA or PDE-5i) in PAH RVEDV RVESV RVM RVEF Relative RVWT Right ventricular volumes, calculated right ventricular wall stress and RVEF improved after combination therapy (p<0.001) but remained unchanged after monotherapy Hassoun [107] (2015), ATPAHSS–O Prospective, multicentre study 24 Evaluate the effect of upfront combination therapy with ambrisentan and tadalafil on left and right ventricular function in patients with SSc-PAH RVM Significantly greater reductions in RVM noted with combination therapy (28.0 g versus 32.5 g; p<0.05) REPAIR (Clinicaltrials.gov NCT02310672) Prospective, multicentre, single-arm study In progress Assess the effect of macitentan on right ventricular and haemodynamic properties (at baseline and 26 weeks) in patients with symptomatic PAH Changes in RVSV Ventriculo-arterial coupling measured by CMR In progress REPLACE (Clinicaltrials.gov NCT02829034) Multicentre, randomised controlled trial versus placebo In progress Assess the effectiveness of riociguat as a replacement for PDE-5i therapy in PAH Change in RVEF measured by CMR In progress IPAH: idiopathic PAH; CI: cardiac index; RVM: right ventricular mass; RVMI: right ventricular mass index; LVMI: left ventricular mass index; RVWT: right ventricular wall thickness; LVWT: left ventricular wall thickness; RVEF: right ventricular ejection fraction; LVEF: left ventricular ejection fraction; RVEDV: right ventricular end diastolic volume; RVEDVI: right ventricular end diastolic volume index; LVEDVI: left ventricular end diastolic volume index; PVR: pulmonary vascular resistance; LVESVI: left ventricular end systolic volume index; RVESV: right ventricular end systolic volume; RVESVI: right ventricular end systolic volume index; RVSV: right ventricular stroke volume; RVSVI: right ventricular stroke volume index; PDE-5i: phosphodiesterase type-5 inhibitor; ERA: endothelin receptor antagonist; MRI: magnetic resonance imaging; RHC: right-heart catheterisation; 6MWT: 6-min walk test; SSc-PAH: systemic sclerosis associated PAH.
- TABLE 4
Comparative assessment of diagnostic modalities in pulmonary hypertension (PH)
Parameter CMR ECHO CT (incl. CTA) RHC Initial diagnosis and confirmation ++
(may suggest)++
(may suggest)++
(may suggest)+++
(gold standard)Aetiology ++ ++ +++
(especially in CTEPH and ILD-related PH)++
(differentiates between various WHO groups)Right ventricle variables Pressure ++ ++ − +++ Ejection fraction +++
(gold standard, stroke volume/end-diastolic volume)++ +/−# − Strain +++ ++ +/−# − Stroke volume +++
(gold standard)+ − +++ Mass +++
(gold standard)− ++ − Right ventricular remodelling +++
(gold standard)++ ++ − Tricuspid regurgitation ++ +++ − + Septal curvature +++ ++ +/−# − Pericardial effusion ++ +++ + − TAPSE − +++ +/−# − Ventricular–arterial coupling (right ventricular end systolic elastance, arterial elastance) +++ − +/−# − Right atrial variables Area/volume ++ ++ + − Pressure − + − +++ Pulmonary artery variables Pressure ++ ++ − +++
(gold standard)RAC/pulsatility +++ + +/−# − Distensibility +++ ++ +/−# + Quantitative blood flow +++ − − − Strengths Gold standard for assessing ventricular mass and structure
No radiationReadily available
Preferred imaging modality for first screening
No radiationGood for evaluation of aetiologies (especially CTEPH and ILD-related PH) Gold standard for confirmation of PH Limitations Limited availability
Significant operator/reader expertise required
Poor evaluation of lung parenchymaLimited role for confirmation/assessment of aetiology
Limited evaluation of right ventricle and pulmonary artery morphologySignificant amount of radiation involved
Poor modality to assess haemodynamicsProvides no information about the morphology and function of the right ventricle CMR: cardiovascular magnetic resonance; CT: computed tomography; CTA: computed tomography angiography; RHC: right-heart catheterisation; CTEPH: chronic thromboembolic pulmonary hypertension; ILD: interstitial lung disease; WHO: World Health Organization; TAPSE: tricuspid annular plane systolic excursion; RAC: relative area change; ECHO: echocardiography; ECG: electrocardiogram. #: CT/CTA can assess these variables if ECG-gating is employed.
Supplementary Material
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Jump To
- Article
- Abstract
- Abstract
- Epidemiology, classification and clinical manifestations of pulmonary hypertension
- Importance of evaluating the right ventricle
- Diagnosis of pulmonary hypertension with other existing modalities and their challenges
- Use of cardiovascular magnetic resonance in management of pulmonary hypertension
- Determining prognosis and risk assessment
- Determining response to therapy
- Using cardiovascular magnetic resonance measurements as trial endpoints
- Advantages and disadvantages of cardiovascular magnetic resonance in pulmonary hypertension
- Conclusion
- Supplementary material
- Footnotes
- References
- Figures & Data
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