Heart conduction system defects and sustained ventricular tachycardia complications in a patient with granulomatosis with polyangiitis. A case report and literature review

Abstract

Granulomatosis with polyangiitis is a rare systemic inflammatory disorder characterized by vasculitis of the small arteries, the arterioles and the capillaries together with necrotizing granulomatous lesions. This case reports on a young female patient, previously diagnosed with granulomatosis with polyangiitis, who was admitted to the intensive care unit with seizures and hemodynamic instability due to a complete atrioventricular heart block. The event was associated with multiple episodes of sustained ventricular tachycardia without any structural heart changes or electrolyte disturbances. In the intensive care unit, the patient was fitted with a provisory pacemaker, followed by immunosuppression with corticosteroids and immunobiological therapy, resulting in a total hemodynamic improvement. Severe conduction disorders in patients presenting granulomatosis with polyangiitis are rare but can contribute to increased morbidity. Early detection and specific intervention can prevent unfavorable outcomes, specifically in the intensive care unit.

A 60-year-old with heart failure symptoms due to 1st degree AV-Block caused by granulomatosis with polyangiitis (Wegener’s granulomatosis)

“…There have been several case reports showing a severe conduction disorder in GPA [9], however we found no mention of persisting heart failure symptoms under this condition in the literature. The patient was admitted to the hospital initially with an NT-proBNP serum level of 1108 pg/ml, at the second admission it was measured as high as 2250 pg/ml, whereas two weeks after treatment initiation and clinical improvement a 480 pg/ml NT-proBNP level has been measured and the patient did not have any shortness of breath or other complaints resembling the clinical symptoms of heart failure.…”

Case Report: Case report: An unusual presentation of granulomatosis with polyangiitis

“…13 Valve disease and conduction disorder including intraventricular conduction defects and atrioventricular blockage are less common. 14 As a result of aggressive treatment, cardiac involvement in patients with GPA was not associated with poorer outcome. 14 In this case the patient died from a respiratory failure due to diffuse alveolar hemorrhage which can be, either another rare and mortal complication of GPA occurring in only 5% to 10% of the patients, or a complication of the acute heart failure.…”

“…14 As a result of aggressive treatment, cardiac involvement in patients with GPA was not associated with poorer outcome. 14 In this case the patient died from a respiratory failure due to diffuse alveolar hemorrhage which can be, either another rare and mortal complication of GPA occurring in only 5% to 10% of the patients, or a complication of the acute heart failure. 15 In another hand, necrotizing granulomatosis hepatitis could increase the risk of heart failure.…”

An unusual precipitant of acute heart failure—ANCA-associated vasculitis in a patient with ischaemic cardiomyopathy: a case report

“…It has been reported in only two cases in the past and both patients had histopathological evidence of myocarditis. In the report by Santos et al , 6 a 44-year-old female with GPA developed sustained VT preceded by bradycardia which improved after temporary pacing followed by a permanent pacemaker insertion. In another report by Hanna et al , 7 a patient with a history of recurrent otitis media presented with myocarditis, renal dysfunction, hyperkalaemia, and VT. She was treated with corticosteroids, cyclophosphamide, and plasma exchange.…”