Right ventricular involvement in cardiac sarcoidosis demonstrated with cardiac magnetic resonance.

AIMS: Cardiac involvement in sarcoidosis is reported in up to 30% of patients. Left ventricular involvement demonstrated by contrast-enhanced cardiac magnetic resonance has been well validated. We sought to determine the prevalence and distribution of right ventricular late gadolinium enhancement in patients diagnosed with pulmonary sarcoidosis. METHODS AND
RESULTS: We prospectively evaluated 87 patients diagnosed with pulmonary sarcoidosis with contrast-enhanced cardiac magnetic resonance for right ventricular involvement. Pulmonary artery pressures were non-invasively evaluated with Doppler echocardiography. Patient characteristics were compared between the groups with and without right ventricular involvement, and right ventricular enhancement was correlated with pulmonary hypertension, ventricular mass, volume, and systolic function. Left ventricular late gadolinium enhancement was demonstrated in 30 patients (34%). Fourteen patients (16%) had right ventricular late gadolinium enhancement, with sole right ventricular enhancement in only two patients. The pattern of right ventricular enhancement consisted of right ventricular outflow tract enhancement in 1 patient, free wall enhancement in 8 patients, ventricular insertion point enhancement in 10 patients, and enhancement of the right side of the interventricular septum in 11 patients. Pulmonary arterial hypertension correlated with the presence of right ventricular enhancement (P < 0.001). Right ventricular enhancement correlated with systolic ventricular dysfunction (P < 0.001), hypertrophy (P = 0.001), and dilation (P < 0.001).
CONCLUSIONS: Right ventricular enhancement was present in 16% of patients diagnosed with pulmonary sarcoidosis and in 48% of patients with left ventricular enhancement. The presence of right ventricular enhancement correlated with pulmonary arterial hypertension, right ventricular systolic dysfunction, hypertrophy, and dilation.

Introduction

Sarcoidosis is a rare, inflammatory condition, resulting from an uncontrolled cellular inflammatory response in genetically predisposed individuals, which affects the heart in approximately a third of patients.1 Left ventricular involvement demonstrated by contrast‐enhanced cardiac magnetic resonance has been well validated.2 Until recently, limited attention has been given to right ventricular involvement in cardiac sarcoidosis, its prevalence, relevance, and prognostic value.3 Cardiac magnetic resonance imaging is the preferred imaging tool to evaluate the healthy and diseased right ventricle.4, 5 Right ventricular volumes, mass, and function can be quantified without geometric assumptions and excellent intra‐observer and inter‐observer agreement and inter‐study reproducibility.4, 5, 6 Delayed contrast‐enhanced magnetic resonance allows for the detection and quantification of focal scar and interstitial fibrosis. Although there are numerous reports on delayed contrast‐enhanced cardiac magnetic resonance delineating left ventricular sarcoidosis, relatively few studies have reported on right ventricular involvement.3, 7, 8, 9, 10 We sought to determine the prevalence and distribution of right ventricular late gadolinium enhancement in patients diagnosed with pulmonary sarcoidosis and determine the relationship with pulmonary hypertension, ventricular volume, mass, and systolic function.

Methods

Patient selection

Between July 2001 and March 2014, we enrolled 87 consecutive patients with histologically proven pulmonary sarcoidosis. Cardiac evaluation was performed because of symptoms or routine screening to exclude cardiac involvement. Patients were excluded when the standard contra‐indications for contrast‐enhanced cardiac magnetic resonance existed. Institutional Review Board approval was obtained for this study.

Baseline investigations

Baseline investigations included 12‐lead electrocardiography, Doppler echocardiography, and contrast‐enhanced cardiac magnetic resonance. Pulmonary artery systolic pressure was estimated from the tricuspid regurgitant velocity plus an estimate of right atrial pressure derived from the inferior vena cava.11 Right‐sided heart studies12 were performed in patients with pulmonary hypertension and congestive heart failure and in patients in whom automated cardioverter defibrillators were implanted. Coronary angiography was performed to exclude underlying coronary artery disease in patients with documented ventricular tachy‐arrhythmias, pathological Q‐waves, impaired systolic function, regional wall motion abnormalities, and/or late gadolinium enhancement.

Cardiac magnetic resonance protocol and analysis

Studies were performed using a commercial 1.5 T scanner with a cardiac‐dedicated phased‐array coil. The cardiac magnetic resonance studies were electrocardiographically triggered by standard software. Studies consisted of multi‐phase multi‐slice steady‐state‐free precession and fat‐saturated T2‐weighted (69) breath‐hold sequences of the short axis, vertical long axis, and horizontal long axis views. Outflow tract views were generated in patients with right ventricular abnormalities. The short‐axis images covered the left ventricle from base to apex. The steady‐state‐free precession sequences (typical repetition time 3.5 ms; echo time 1.4 ms; flip angle 55°; temporal resolution 50 ms; voxel size 1.6 × 1.6 × 10 mm, no gap) were performed to assess regional wall‐motion abnormalities, left and right ventricular masses, volumes, and ejection fractions. Papillary muscles were included when determining right ventricular mass and excluded when determining volumes. Contrast‐enhanced and T2‐weighted images were obtained in diastole to minimize artefact due to cardiac motion. Ten minutes after the additional administration of 0.1 mmol/kg gadolinium‐diethylenetriaminepenta‐acetic acid (Schering, Berlin, Germany), a two‐dimensional segmented inversion recovery‐gradient echo breath‐hold sequence (short axis, vertical long axis, horizontal long axis, and right ventricular outflow tract in selected patients, voxel size 1.6 × 1.6 × 10 mm, without gap) was used to assess for late gadolinium enhancement. The inversion time (250 to 400 ms) was determined on an individual basis to obtain optimal nulling of the unenhanced myocardial signal. Two experienced, blinded, and independent observers used commercially available software (CAAS MRV 3.4, Pie Medical Imaging, Maastricht, the Netherlands) to determine the standard parameters delineated in Table 1. The distribution of right ventricular late gadolinium enhancement was determined by consensus and characterized as free wall, apical, outflow tract, right‐sided interventricular septal, and/or including the ventricular insertion points. Late ventricular gadolinium enhancement was considered present only if confirmed on both short‐axis and matching long‐axis myocardial locations. Late left ventricular gadolinium enhancement was quantified by a semiautomatic detection method using the signal intensity threshold of ≥2 SD above a remote reference region. The intra‐observer and inter‐observer variabilities were determined by calculating the variability coefficients and intra‐class correlations for each parameter in 18 randomly selected studies.

Table 1

Characteristics of patients with and without right ventricle late gadolinium enhancement

	Patients without RV LGE n = 73	Patients with RV LGE n = 14	P value
Male	48 (66)	9 (64)	0.779
Caucasian	58 (79)	8 (57)	0.074
Age (years)	52.8 ± 10.2	55.7 ± 9.1	0.460
Cardiac presentation	18 (25)	10 (71)	<0.001
Syncope	4 (5)	1 (8)	0.564
Palpitations	7 (10)	3 (21)	0.167
Clinical congestive heart failure	4 (5)	6 (43)	0.001
Sustained ventricular Tachycardia	6 (8)	4 (29)	0.039
Chest discomfort	2 (3)	1 (8)	0.388
Dyspnoea
NYHA 0–2	72 (98)	12 (86)	0.388
NYHA 3–4	2 (3)	1 (7)	0.388
Diabetes mellitus	3 (4)	0	1.000
Hypertension	7 (10)	0	0.588
Medication at any time	51 (70)	12 (86)	0.102
Steroids	5 (7)	2 (14)	0.280
Methotrexate	5 (7)	5 (36)	0.006
Loop diuretics	5 (7)	6 (43)	0.001
Spironolactone	5 (7)	7 (50)	<0.001
Ace inhibitors/ATIIRB	7 (9)	7 (50)	0.001
Beta blockers	9 (12)	6 (43)	0.008
Amiodarone
Abnormal ECG	18 (25)	10 (71)	<0.001
Pulmonary hypertension	5 (7)	9 (64)	<0.001
CMR imaging parameters
LVEF, %	60 [54–66]	50 [42–58]	0.015
LVEF ≤ 50%	8 (11)	5 (36)	0.039
LVEDV, mL	113 [90–136]	134 [81–187]	0.261
LVEDV index, mL/m2	58 [47–69]	75 [70–100]	0.142
LV mass	112 [72–152]	122 [83–161]	0.550
LV mass index, g/m2	64 [44–84]	65 [38–92]	0.780
LVH	20 (27)	3 (23)	0.747
LV dilation	5 (8)	4 (29)	0.035
LV LGE	18 (25)	12 (86)	<0.001
LV LGE, %	12 [4–20]	28 [18–38]	0.002
RVEF, %	49 [43–55]	33 [24–42]	0.001
RVEDV, mL	148 [108–188]	188 [141–235]	0.034
RVEDV index, mL/m2	78 [58–98]	96 [68–124]	0.018
RVESV	72 [47–97]	102 [70–134]	0.05
RVESV index, mL/m2	37 [26–48]	58 [38–78]	0.046
RVH	5 (7)	6 (43)	0.001
RV mass, g	42 [34–50]	53 [35–71]	0.068
RV mass index, g/m2	21 [17–25]	28 [22–34]	0.075
RV dilation	3 (4)	6 (43)	<0.001
RVEF ≤ 45%	6 (8)	10 (71)	<0.001
T2 positive	7/60 (12)	3/9 (33)	0.112

CI, confidence interval; CMR, cardiac magnetic resonance; EDV, end‐diastolic volume; LGE, late adolinium enhancement; LV, left ventricle; LVEDV, left ventricular end‐diastolic volume; LVEF, left ventricular ejection fraction; RV, right ventricle; RVEDV, right ventricular end‐diastolic volume; RVEDVI, right ventricular end‐diastolic volume index; RVEF, right ventricular ejection fraction; RVH, right ventricular hypertrophy.

Bold signifies significance i.e. P < 0.05.

Values are n (%), median [IQR], or mean ± SD.

Variables and definitions

Peak systolic right ventricular pressures over 35 mmHg were considered to represent pulmonary hypertension. Right ventricular hypertrophy was defined as right ventricular weight exceeding normal values as published by Maceira et al. and/or right ventricular end‐diastolic wall thickness over 5 mm.6 Right ventricular systolic dysfunction was defined as an ejection fraction below 45%.13

Statistical analysis

All statistical analyses were performed using statistical software (Version 21.0, SPSS; Chicago, IL, USA). Continuous normal distributed variables were expressed as mean ± SD and, between group comparisons, were made using the parametric t‐test for independent samples or the Mann–Whitney test when appropriate. In the non‐normally distributed continuous data, the median and interquartile range were determined and, between group correlations, were made with the Wilcoxon test. Categorical variables were assessed using the χ2 or Fisher's exact test when appropriate. Statistical significance was defined at P < 0.05.

Results

Patient characteristics

Table 1 demonstrates the baseline characteristics in the included 87 patients. Twenty‐seven (31%) patients presented with cardiac symptoms, while the remaining 60 either suffered from non‐specific symptoms or were routinely screened for cardiac sarcoidosis. According to the ACC/AHA/HRS 2008 guidelines for device‐based therapy of cardiac rhythm abnormalities, an implantable cardioverter defibrillator or pacemaker was implanted in respectively 14 and 3 patients after the baseline cardiac magnetic resonance study.14 All had late gadolinium enhancement and suffered from cardiac symptoms. When applying the recently published Heart Rhythm Society expert consensus criteria, we diagnosed 31 patients (36%) with cardiac involvement.15

Cardiac magnetic resonance analyses

Thirty patients (34%) had left ventricular and 14 patients (16%) had right ventricular enhancement, with enhancement limited to the right ventricle in only two patients (2%). The group of patients with right ventricular enhancement had significantly more extensive left ventricular enhancement compared with those without (Table 1, P = 0.007). Table 2 describes the distribution of right ventricular enhancement. Right ventricular enhancement correlated with early diastolic left‐sided septal displacement (P = 0.002), systolic right ventricular dysfunction (P < 0.001), hypertrophy (P < 0.001), and dilation (P = 0.009). Fifteen patients had pulmonary hypertension, eight of which had right ventricular enhancement. The presence of ventricular insertion point enhancement (P = 0.007), right‐sided septal (P < 0.001), and right ventricular free wall enhancement (P = 0.016) correlated with the presence of pulmonary hypertension. Right ventricular hypertrophy correlated with the presence of ventricular insertion point enhancement (P = 0.004) and right‐sided septal (P = 0.001), but not with free wall enhancement (P = 0.076). In five patients, early diastolic septal displacement towards the left ventricle was observed, two of which had insertion point enhancement (P = 0.099), and three of which had right‐sided septal enhancement (P = 0.013). Neither the chronicity of pulmonary sarcoidosis, as determined by the time since diagnosis, nor the extent of lung disease as determined by high‐resolution computed tomography correlated with pulmonary hypertension, right ventricular systolic function, or myocardial enhancement. The right ventricular end‐diastolic volume index in patients with pulmonary Stage 4 (fibro‐cystic disease) was significantly larger compared with the earlier disease Stage 1 (hilar nodes only) (P = 0.027). The T2‐weighted studies suggested active, granulomatous disease in only 10/69 (14%) of patients. The intra‐observer variability for right ventricular end‐diastolic volume was 2%, end‐systolic volume 3.4%, ejection fraction 3.4%, and mass 4%. The interobserver variability for right ventricular end‐diastolic volume was 1.6%, end‐systolic volume 5%, ejection fraction 3.2%, and mass 8%.

Table 2

Characteristics of patients with right ventricle late gadolinium enhancement

Enhanced segments	Patients (n = 14)	Combination of enhanced segments	Patients (n = 14)	Patients with pulmonary hypertension	Patients with end‐systolic septal shift (n = 5)
RV septal	11	RV septal	1	1	0
		VIP
		RV free wall
		RVOT
VIP	10	RV septal	3	2	1
		VIP
		RV free wall
RV free wall	8	RV septal	4	1	0
		VIP
RVOT	1	RV septal	2	2	1
		RV free wall
		RV septal	1	1	1
		RV free wall	1	0
		VIP	1	1	1
		RV free wall	1	0	0
		VIP

RV, right ventricular; RVOT, right ventricular outflow tract; VIP, ventricular insertion points.

Discussion

This is the first prospective cardiac magnetic resonance study to specifically report on the prevalence and distribution of right ventricular involvement in cardiac sarcoidosis. Predominant or isolated right ventricular involvement is rare, with nearly all patients suffering from left ventricular disease.16 With Murtagh et al., Crawford et al., and Cheong et al., we found more extensive left ventricular enhancement to be associated with right ventricular involvement.8, 10, 17 Previous studies reported a correlation between the presence and extent of left ventricular enhancement, impaired left and right ventricular systolic function, and higher adverse event rates. The direct relationship between right ventricular enhancement, its size and systolic function, as previously demonstrated in left ventricular sarcoidosis, has not been reported before.

Right ventricular enhancement, inflammation, and impaired systolic function have been associated with adverse outcome, particularly ventricular tachy‐arrhythmias.10, 17, 18, 19, 20 However, the localizations of the arrhythmogenic foci were not reported on. Because right ventricular sarcoidosis occurs in patients with more extensive left ventricular disease, the reported prognostic relevance of right ventricular disease may at least partly reflect the extent of left ventricular arrhythmogenic substrate.

Several post‐transplant and post‐mortem studies in sarcoidosis patients have reported right ventricular involvement to range from 6%, in patients dying from alternate causes, to as high as 65% in those dying from sudden cardiac deaths.21, 22, 23, 24, 25 Generally, patients with congestive failure were found to have extensive biventricular sarcoid (Figure 1), and in those who had died suddenly, active granulomatous infiltration and patchy scar were present. Right ventricular outflow tract involvement was rare (Figure 2).

Figure 1

(A) Contrast‐enhanced magnetic resonance study in a patient with biventricular congestive heart failure (inversion‐recovery gradient echo sequence and horizontal long axis view) demonstrates enhancement of the right ventricular free wall and right‐sided interventricular septum. (B) Short axis view in the same patient demonstrates enhancement of the right‐sided interventricular septum and inferior right ventricular insertion point.

Ten recent studies employing contrast‐enhanced magnetic resonance in the assessment of cardiac sarcoidosis reported on right ventricular disease in sarcoidosis (Table 3).3, 7, 8, 9, 10, 17, 18, 19, 20, 26 Our findings in an unselected population with pulmonary sarcoidosis compare. Right ventricular enhancement was reported in five of these studies and ranged from 2% in unselected to 48% in high‐risk populations, which compares with our 47% of patients with left ventricular sarcoidosis.3, 7, 8, 9, 10 Similar to our findings, nearly all patients with right ventricular enhancement had left ventricular enhancement. Samar et al., Patel et al., and Crawford et al. were the only researchers to report on right ventricular enhancement in more detail.3, 7, 10 Contrary to our and other studies, Samar et al. reported that right ventricular enhancement did not correlate with a difference in left ventricular ejection fraction. His data were included in a poster presentation, which precluded detail, while relevant data were not available in 19% of patients.3 Crawford reported multi‐focal right ventricular enhancement in nearly half of those with left ventricular enhancement, basal, mid, or apical right ventricular segments being equally involved.10 Similar to Crawford et al., the majority of our patients (70%) had multi‐focal right ventricular enhancement. However, most of our patients had right‐sided septal enhancement, similar to Patel's findings.7 Right‐sided septal and insertion point enhancement were related to pulmonary hypertension in eight of our patients. Crawford et al. and Patel et al. did not include data on right ventricular or pulmonary pressures. Compared with Crawford's population, our patients with right ventricular enhancement had on average poorer systolic function and more extensive left ventricular enhancement.

Table 3

Cardiac magnetic resonance studies reporting on right ventricular involvement in cardiac sarcoidosis

Authors	Type of study	Patients	Conclusion
Cheong et al.8	Prospective, single centre	31 patients asymptomatic biopsy proven systemic sarcoidosis, 8 (26%) LV LGE of whom 2 (25%) with RV LGE, inferobasal RV LGE in patients with most LV LGE	Asymptomatic small amount of LGE (average 3.2% of LV) in 26%, no cardiac events after 1 year
Patel et al.7	Prospective, single centre	81 patients with extra‐cardiac sarcoidosis, 21 (26%) with LV LGE (average 6 g), 14 (67%) had right‐sided septal LGE incl 4 RV free wall/outflow tract/anterobasal segments	Patients with LGE had 9‐fold higher rate of adverse events
Patel et al.19	Retrospective, single centre	152 patients extra‐cardiac sarcoidosis, LVEF ≥ 50%, 29 (19%) LV LGE, no data on RV LGE	Patients with LV LGE had lower RVEF, because of either presumed biventricular disease or pulmonary hypertension
Schuller et al.18	Retrospective, multi‐centre	112 CS patients with ICDs for primary or secondary prevention, no data on LGE	Impaired systolic LV and RV function correlates with more ICD therapy
Samar et al. (poster)3	Retrospective, single centre	122 sarcoidosis patients, 37 (22%) LV LGE, 18 (49%) of these also RV LGE	LVEF, LVEDV, RVEDV similar in groups with/without RV LGE
Crawford et al.10	Retrospective, multi‐centre	52 CS patients, all LVEF > 35% 32 (62%) with LV LGE of which 13 (41%) also had RV LGE	Multi‐focal LGE correlated with VT/VF, patients with RV LGE had more extensive LV LGE
Nadel et al.9	Retrospective, single centre	106 sarcoidosis patients, 32 CS‐defined by CMR LGE—32 LV LGE, 2 (6%) RV LGE	LGE only independent predictor of adverse outcome
Muser et al.20	Prospective, single centre	31 CS patients with VTs pre‐ablation, 23 had CMR, 21 (68%) LV LGE, 11 (35%) RV LGE, no data on RV distribution or extent	LGE extent predicted VT‐free survival
Ekström et al.26	Retrospective, single centre	50 CS patients, 48 (96%) with LV LGE, not reported on RV LGE	LV extent of LGE and RVEF, correlated with adverse outcome
Murtagh et al.17	Retrospective, single centre	205 patients extra‐cardiac sarcoidosis, LVEF ≥ 50%, 41 (20%) LV LGE, ≥4 patients with VIP LGE, no specific data on RV LGE	For every 1% in LGE burden, the hazard for an event increased by 8%; mild impaired RV dysfunction correlated with increased event rate

CMR, cardiac magnetic resonance; CS, cardiac sarcoidosis; LGE, late gadolinium enhancement; LV, left ventricle; LVEF, left ventricular ejection fraction; RV, right ventricle; RVEF, right ventricular ejection fraction.

The presence of right ventricular enhancement correlated with hypertrophy, dilation, and systolic dysfunction. The relationship between pulmonary hypertension, right ventricular hypertrophy, dilation, systolic dysfunction, septal displacement, and septal and insertion point enhancement has been demonstrated before12 (Figure 3). Right ventricular enhancement in sarcoidosis may be caused by direct granulomatous infiltration but also be related to pulmonary hypertension12 (Figures 1 and 3). Right ventricular hypertrophy, dilation, dysfunction, and enhancement may be secondary to pulmonary hypertension and are associated with worse prognosis.12, 27 Pulmonary hypertension is found in 6–28% of the general sarcoidosis outpatient setting and may be secondary to pulmonary fibrosis, angiitis, and/or congestive heart failure.27 The fact that pulmonary hypertension in our population did not correlate with disease extent as determined by computed tomography illustrates the variety in pathophysiology of this condition in sarcoidosis.28 Right ventricular dilation, systolic impairment, and inflammation, as demonstrated with positron emission tomography, in sarcoidosis have been demonstrated to predict adverse outcome in sarcoidosis.10, 13, 17, 18, 19, 26, 29

In patients with predominant right ventricular disease, cardiac sarcoidosis needs to be differentiated from arrhythmogenic right ventricular cardiomyopathy. Distinguishing features favouring sarcoidosis consist of an older age of onset, a non‐familiar pattern, wider QRS complexes, septal involvement with atrio‐ventricular conduction disease, multiple arrhythmogenic foci, particularly right ventricular apical tachycardia, concomitant left ventricular disease, and the presence of mediastinal lymphadenopathy.30 Electro‐anatomic mapping, contrast‐enhanced magnetic resonance, and/or positron emission tomography may guide endomyocardial biopsies needed to obtain histological confirmation of the diagnosis.

We report on ventricular insertion point enhancement, a distribution pattern not specifically mentioned in the other studies. Late enhancement of the septum and insertion points may result from delayed wash‐out of gadolinium due to altered myocardial fibre strain, fibre disarray, ischaemia, and fibrosis, secondary to right ventricular pressure or volume overload, and resulting septal shift.12, 31, 32 Ventricular insertion point enhancement has been reported in hypertrophic cardiomyopathy, atrial septal defects, severe pulmonary hypertension, tetralogy of Fallot, transposition of the great arteries, and even a proportion of veteran healthy endurance athletes31, 32, 33, 34, 35 (Figures 2, 3, 4, 5). The amount of insertion point enhancement correlates with mean pulmonary arterial pressures, right ventricular mass, volume, and ejection fraction.12 Pulmonary hypertension in sarcoidosis is associated with adverse outcome, particularly when accompanied by right ventricular dysfunction, and/or lung fibrosis.12, 27 Recently, Swift et al. reported septal extension of insertion point enhancement in pulmonary hypertension to mark more severe disease, with associated right ventricular dilation, but found it not to be an independent predictor of overall mortality.12

Figure 2

Contrast‐enhanced magnetic resonance study (inversion‐recovery gradient echo sequence, end‐diastolic frame, and short axis view) demonstrates enhancement of the right ventricular free wall (arrow), ventricular insertion points (triangles), and right‐sided septum (arrow).

Figure 3

(A) Magnetic resonance study (steady‐state‐free precession sequence, horizontal long axis view, and end‐diastolic frame) demonstrates dilation of the right ventricle (RV) and right atrium (RA), marked right ventricular hypertrophy, with displacement of the interventricular septum towards the left ventricle; both left ventricle and atrium are compressed. (B) Contrast‐enhanced magnetic resonance study (inversion recovery‐gradient echo sequence, short axis view, and end‐diastolic frame) of the identical patient with pulmonary vascular sarcoidosis and resulting severe pulmonary arterial hypertension demonstrates contrast‐enhancement of the right ventricular hinge points (triangles) and free wall (arrows).

Figure 4

Contrast‐enhanced magnetic resonance study (inversion‐recovery gradient echo sequence and short axis view) in a patient without pulmonary hypertension demonstrates enhancement of the ventricular insertion points (arrows), papillary muscles (asterisks), and postero‐lateral left ventricular segments (triangle).

Figure 5

(A) Contrast‐enhanced magnetic resonance study (inversion‐recovery gradient echo sequence, end‐diastolic frame, and short axis view) in a patient diagnosed with a high‐degree atrio‐ventricular block secondary to active cardiac sarcoidosis. A dual chamber pacemaker had been inserted. Ventricular insertion point enhancement is demonstrated (triangles). Pulmonary pressures were normal (asterisk—artefact of right ventricular pace lead). (B) Contrast‐enhanced magnetic imaging study (inversion‐recovery gradient echo sequence, end‐diastolic frame, and short axis view) in the identical patient when reassessed 7 years later demonstrates substantially more enhancement of the right‐sided septum (arrow) and the insertion points (triangles). The percentage time pacing had increased from 5% to 15% of the time (asterisk—artefact produced by right ventricular pace lead).

Late gadolinium enhancement includes active, potentially reversible, granulomatous inflammation, and chronic focal scar.8, 20, 36 Immune suppressive treatment, currently a work in progress, may potentially improve systolic function and decrease arrhythmogenic substrate.10, 20, 36 The T2‐weighted spin echo‐based assessment used in our study to evaluate for active granulomatous infiltration and associated oedema is rather insensitive.8 T2 mapping and positron emission tomography have shown promise and will be included in future projects.28, 37

Conclusions

Approximately 30% of an unselected patient population with pulmonary sarcoidosis had left ventricular involvement, half of which had right ventricular involvement. More extensive left ventricular enhancement correlated with right ventricular involvement. Right ventricular enhancement may result from direct infiltration and resulting scar or pulmonary hypertension. Previous studies associated impaired systolic right ventricular function and right ventricular enhancement with ventricular tachy‐arrhythmias. We demonstrate right ventricular enhancement with cardiac magnetic resonance to be mostly multi‐focal, involve the septum, and correlate with increased right ventricular volumes, hypertrophy, and impaired systolic function.

Limitations

Pulmonary pressures were routinely determined non‐invasively in the majority of our patients, and we may have underestimated the pulmonary pressures. Our imaging protocol was not primarily adapted to evaluate the right ventricle, our slice thickness, and potentially suboptimal myocardial nulling may have resulted in an underestimation of the presence and extent of right ventricular infiltration and scarring. Because T2 mapping was not performed, we likely underestimated active granulomatous inflammation. The relatively small number of patients included limits our findings and conclusions.

Future focus of development

Customized hybrid approaches including electrocardiography, ultrasound, positron emission tomography, and contrast‐enhanced magnetic resonance will provide us with more sensitive, accurate, and comprehensive information on haemodynamic, electrical, mechanical, and inflammatory characteristics of the atriae and ventricles.

Conflict of interest

None declared.