Complete heart block in association with graft-versus-host disease

Abstract

Summary:An infant who received haploidentical BM for severe combined immunodeficiency (SCID) developed acute, reversible complete heart block in association with an exacerbation of GVHD. Respiratory distress and myocardial dysfunction were also seen with this and previous GVHD exacerbations. The patient had not received chemotherapy or radiation prior to BMT. The complete heart block resolved after 1 week of intensive immunosuppression. The association of complete heart block with GVHD is important because the heart block is potentially reversible with prompt, aggressive control of the GVHD. Keywords: cardiac; heart block; arrhythmia; graftversus-host disease; bone marrow transplantation Graft-versus-host disease is a major cause of morbidity and mortality after bone marrow transplantation. GVHD manifestations are predominantly limited to the skin, gastrointestinal tract, and liver. 1 However, severe GVHD is associated with a marked release of cytokines ('cytokine storm') 2 that may result in more widespread organ involvement. Cardiac involvement during acute or chronic GVHD has been reported to be infrequent, mild and generally asymptomatic. 3,4 We report the second case of complete atrioventricular block occurring in association with GVHD. A case of complete heart block has been reported in a patient with SCID who received an haploidentical parental marrow transplant. The patient had acute GVHD of the skin, liver and gut which was treated with high-dose steroids and monoclonal antibody. He had recurrent symptoms and signs of chronic GVHD and was maintained on low-dose steroids. Skin and liver biopsies were consistent with chronic GVHD. The patient developed complete heart block at day +97 and required a pacemaker. A myocardial biopsy showed interstitial edema, focal mononuclear cell infiltrate, and individual fiber degeneration. The lymphohistiocytic infiltrate resembled that from the skin biopsy. The patient Correspondence: Dr A Gilman, Pediatric Hematology/Oncology, The Children's Mercy Hospital, 2401 Gillham Road, Kansas City, MO 64108, USA Received 14 March 1997; accepted 14 July 1997 died 1 month later and autopsy results showed similar infiltrates with foci of necrosis and scarring present in the atrioventricular node, bundle of His and myocardium. No evidence of viral infection was found on the premortem biopsy specimen or at autopsy. 5 The patient in our report was treated with intensive immunosuppression, had reversal of his complete heart block, and remains well. Case reportThe patient was diagnosed with SCID when he presented at 4 months of age with Pneumocystis carinii pneumonia and failure to thrive. He had severe pneumonia and required a high-frequency ventilator and nitric oxide for 2 weeks. He had no clinical evidence of cardiac dysfunction. Immunophenotyping showed a lack of T lymphocytes and NK cells, and the presence of B lymphocytes. He received haploidentical T cell-depleted bone marrow from his father. T cell depletion was performed with OKT3 antibody and rabbit complement, which resulted...

Acute heart failure after allogeneic blood stem cell transplantation due to massive myocardial infiltration by cytotoxic T cells of donor origin

“…To our knowledge, there is only one case reported of reversible complete heart block occurring during acute GVHD. 1 The patient had not shown any clinical signs of heart failure before conditioning for the second transplant was started. Therefore, echocardiography was not repeated.…”

“…To our knowledge, there is only one report in the literature suggesting an acute GVHD of the heart with reversible complete heart block. 1 We report a case of allogeneic unrelated PBSC transplantation in a patient with AML which relapsed early after first transplant. Acute GVHD occurred and the patient died due to acute heart failure on day +15 after a second PBSCT.…”

Cardiac and cardiovascular consequences after haematopoietic stem cell transplantation

“…The heart and the cardiovascular system have been rarely reported as a target of GvHD. There are a few single reports (Gilman et al, 1998;Platzbecker et al, 2001;Prevost et al, 2004) and a small series of patients with cardiac GvHD. Patients with cardiac GvHD developed dysrhythmia, mainly bradycardia, coronary artery disease, or cardiomyolysis (Rackley et al, 2005).…”

Risk factors affecting cardiac left-ventricular hypertrophy and systolic and diastolic function in the chronic phase of allogeneic hematopoietic cell transplantation

“…There are some reports of cardiac dysfunction during GVHD but these are just case series. [21][22][23][24] However, a larger, retrospective cohort study showed no statistically significant difference in the incidence of congestive heart failure among patients with acute GVHD, those with a history of chronic GVHD and Cardiac dysfunction in the chronic phase of HCT M Nishimoto et al those with active GVHD. 25 Uderzo et al 13 report similar findings in childhood HCT.…”