Cardiomyopathy associated with Wegener's granulomatosis

To, Andrew; Zoysa, Janak de; Christiansen, J.

doi:10.1136/hrt.2006.098038

Cited by 19 publications

(22 citation statements)

References 1 publication

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“…The most frequent abnormality observed in the present study was LGE lesions in both RV and LV myocardium. LGE lesions were previously described in case reports of WG and similar to the present study, they were distributed in all myocardial layers (subendocardial, midwall, subepicardial) [9–11]. Interestingly, many of them were localized in apical and posteroinferior LV wall segments and a similar distribution of myocarditis was reported at autopsy in a patient with rapidly progressive, fatal WG [22].…”

Section: Discussionsupporting

confidence: 87%

Cardiac involvement in Wegener’s granulomatosis resistant to induction therapy

et al. 2011

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ObjectivesThe aim of the study was to assess cardiac involvement in patients with Wegener’s granulomatosis (WG), who failed to achieve remission following >6 months induction therapy for life or organ threatening disease.MethodsEleven WG patients (eight males, mean age 47 ± 13 years), who failed to achieve remission despite >6 months induction therapy, underwent transthoracic echocardiography (TTE) and cardiac magnetic resonance (CMR).ResultsCardiac involvement was present in 9 (82%) patients. Regional wall motion abnormalities were found in two individuals, but none had left ventricular (LV) ejection fraction <50%. Nine patients had late gadolinium enhancement (LGE) lesions involving LV myocardium and right ventricle free wall was involved in four patients. LGE lesions were found in subepicardial, midwall and subendocardial LV myocardial layers. CMR revealed myocarditis in six patients. Patients with myocarditis had a higher number of LV segments with LGE (5.2 ± 3.4 vs 1.0 ± 1.2, p = 0.03) and more frequent diastolic dysfunction by TTE (5 vs 0, p = 0.02) than those without. Pericardial effusion was observed in five patients, while localized pericardial thickening in six patients.ConclusionsIn WG resistant to >6 months induction therapy cardiac involvement is frequent and is characterized by foci of LGE lesions and signs of myocardial inflammatory process.

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Section: Discussionsupporting

confidence: 87%

Cardiac involvement in Wegener’s granulomatosis resistant to induction therapy

et al. 2011

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“…As previously reported the lesions were present in all myocardial layers (subendocardial, midwall, subepicardial) both in the posterior LV portion and interventricular septum [8, 10–12, 14, 15]. Interestingly, these LGE areas were not associated with CMR signs of myocardial edema or hyperemia/hyperpermeability of capillaries, which suggests the presence of healed rather than necrotic lesions.…”

Section: Discussionsupporting

confidence: 67%

Standard and feature tracking magnetic resonance evidence of myocardial involvement in Churg–Strauss syndrome and granulomatosis with polyangiitis (Wegener’s) in patients with normal electrocardiograms and transthoracic echocardiography

Miszalski−Jamka

Szczeklik

Sokołowska

et al. 2012

Int J Cardiovasc Imaging

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The aim of the study was to evaluate the presence and spectrum of cardiac abnormalities identified by cardiac magnetic resonance (CMR) in subjects in clinical remission of Churg-Strauss syndrome (CSS) and granulomatosis with polyangiitis (Wegener's) (WG) with normal ECG and transthoracic echocardiography (TTE). Eleven (7 females, 4 males, mean age 42.4 ± 9.6 years) CSS and 10 (4 females, 6 males, mean age 45.3 ± 10.9 years) WG patients in clinical remission with normal ECG and TTE underwent CMR. Segmental peaksystolic myocardial strain (e ps ) was measured using feature tracking cine-sequence based technique. Left ventricular (LV) ejection fraction, end-diastolic volume and myocardial mass indexes were 66.2 ± 5.8 %, 66.1 ± 6.6 ml/m 2 , and 61.0 ± 8.9 g/m 2 , respectively. No patient showed regional wall motion abnormalities and signs of myocarditis. Nine CSS and 8 WG patients demonstrated decreased segmental longitudinal, circumferential or radial e ps and myocardial late gadolinium enhancement (LGE) (6 subendocardial, 10 midwall, 8 subepicardial) areas. In CSS and WG subjects with LVLGE lesions the mean LVLGE extent was 2.0 ± 1.6 % and 2.3 ± 1.5 % (p = 0.65), respectively. Segmental e ps was decreased longitudinally (-11.8 ± 5.6 %) for subendocardialLGE, radially (13.7 ± 8.7 %) for subepicardial LGE, and circumferentially (-16.6 ± 4.2 %), longitudinally (-13.2 ± 5.5 %) and radially (18.8 ± 8.1 %) for midwall LGE, if compared to longitudinal (-22.7 ± 5.1 %), circumferential (-23.6 ± 5.6 %) and radial (34.2 ± 15.7 %) e ps in controls (11 females, 10 males, mean age 43.9 ± 10.5 years) (all p \ 0.01). Despite clinical remission, normal ECG and TTE, most CSS and WG patients demonstrate decreased segmental e ps and non-ischemic LGE lesions without signs of myocarditis.

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“…Later reports found that heart failure was the cause of death in 48 % of patients with EPGA (Lanham et al 1984). In GPA, dilated cardiomyopathy has been described in patients with both new and established disease and may be due to myocardial inflammation and damage (Shanahan et al 1999;To et al 2007) (see Fig. 6).…”

Section: Other Clinical Manifestationsmentioning

confidence: 97%

“…Likewise, there are some case reports of AAV-associated cardiomyopathy that can be improved with the combination of immunosuppression and plasmapheresis ). Along with corticosteroids, CYC, azathioprine, and cyclosporine (Schiavone et al 1985;Shanahan et al 1999;To et al 2007;Kim et al 2013) have also been tried with variable success for patients with AAV and evidence of cardiac involvement. However, in many cases, immunosuppression alone cannot reverse the cardiac manifestations.…”

Section: Treatmentmentioning

confidence: 99%

Pulmonary Vasculitis

Orlowsky¹,

Kaul²,

Clair³

2014

PanVascular Medicine

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The systemic vasculitides are rare conditions characterized by inflammation and necrosis of blood vessels. Those with predominately small vessel involvement, such as the ANCA-associated vasculitides (AAV), are the only ones to target the lungs with any regularity. However, these conditions can affect almost any organ system, including the cardiovascular system. Obtaining an accurate diagnosis is crucial before committing a patient to long-term immunosuppressive therapy. The choice of treatment is guided by the organ systems involved, the severity of disease, and the presence of comorbid conditions. Newer treatments are emerging which provide more options in the therapeutic armamentarium.

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Cardiomyopathy associated with Wegener's granulomatosis

Cited by 19 publications

References 1 publication

Cardiac involvement in Wegener’s granulomatosis resistant to induction therapy

Cardiac involvement in Wegener’s granulomatosis resistant to induction therapy

Standard and feature tracking magnetic resonance evidence of myocardial involvement in Churg–Strauss syndrome and granulomatosis with polyangiitis (Wegener’s) in patients with normal electrocardiograms and transthoracic echocardiography

Pulmonary Vasculitis

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