Research Spotlight

Posted January 15th 2020

Management of Adults With Normally Functioning Congenitally Bicuspid Aortic Valves and Dilated Ascending Aortas.

William C. Roberts M.D.
William C. Roberts M.D.

Roberts, W. C., S. Siddiquiz, A. E. Rafael-Yarihuaman and C. S. Roberts (2020). “Management of Adults With Normally Functioning Congenitally Bicuspid Aortic Valves and Dilated Ascending Aortas.” Am J Cardiol 125(1): 157-160.

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We describe herein a 65-year-old woman who underwent resection of a dilated (5.1 cm) ascending aorta associated with a normally functioning congenitally bicuspid aortic valve. The patient provided the framework to discuss proper management-operative versus nonoperative-of the dilated ascending aorta associated with a normally functioning bicuspid aortic valve. Unfortunately, there is inadequate data to provide an unequivocal answer to this dilemma. Operative intervention requires that the short-term risk of the prophylactic procedure be considerably lower than the long-term risk of aortic dissection/rupture without operative intervention. Because there is no proof that operative intervention provides less morbidity and lower mortality, nonoperative management at this time seems to be the better approach.


Posted January 15th 2020

Pseudoaneurysm of the Ascending Aorta at the Cannulation Site Diagnosed More Than Four Decades After Repair of Ventricular Septal Defect.

William C. Roberts M.D.E
William C. Roberts M.D.

Roberts, C. S., Y. M. Salam, A. J. Moore and W. C. Roberts (2019). “Pseudoaneurysm of the Ascending Aorta at the Cannulation Site Diagnosed More Than Four Decades After Repair of Ventricular Septal Defect.” Am J Cardiol 124(12): 1962-1965.

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Described herein is a 69-year-old woman who developed a large saccular aortic aneurysm at a previous cannulation site for repair of a ventricular septal defect at age 25 years. The aneurysm was resected and proved histologically to be a false one. The long interval between operations (44 years) exceeds those reported previously.


Posted January 15th 2020

Influence of major histocompatibility complex class I chain-related gene a polymorphisms on cytomegalovirus disease after allogeneic hematopoietic cell transplantation.

Medhat Z. Askar M.D.
Medhat Z. Askar M.D.

Patel, S. S., L. A. Rybicki, M. Yurch, D. Thomas, H. Liu, R. Dean, D. Jagadeesh, B. Hill, B. Pohlman, B. Bolwell, R. Hanna, B. K. Hamilton, M. Kalaycio, A. T. Gerds, E. Cober, S. Mossad, A. Zhang, N. S. Majhail, M. Askar and R. Sobecks (2019). “Influence of major histocompatibility complex class I chain-related gene a polymorphisms on cytomegalovirus disease after allogeneic hematopoietic cell transplantation.” Hematol Oncol Stem Cell Ther Dec 24. [Epub ahead of print].

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OBJECTIVE/BACKGROUND: Cytomegalovirus (CMV) infection and disease are common infectious complications after allogeneic hematopoietic cell transplantation (alloHCT). Major histocompatibility complex (MHC) class I chain-related gene A (MICA) is a ligand of the natural killer (NKG2D) receptor on immune effector cells that helps mediate NK cell alloreactivity. We hypothesized that MICA polymorphisms may influence CMV infection and disease incidence after alloHCT. METHODS: We conducted a retrospective analysis of 423 adults at the Cleveland Clinic with hematologic malignancies treated with a matched related or unrelated donor alloHCT. CMV cases analyzed included a compositive of instances of viral copy replication above detection limits as well as any biopsy-proven tissue invasive disease episodes. Genotypes at the MICA-129 position have been categorized as weak (valine/valine; V/V), intermediate (methionine/valine; M/V), or strong (methionine/methionine; M/M) receptor affinity. RESULTS: In multivariable analysis, V/V donor MICA-129 genotype was associated with CMV infection and disease (hazard ratio [HR]=1.40; 95% confidence interval [CI], 1.00-1.96; p=.05), but not MICA mismatch (HR=1.38; 95% CI, 0.83-2.29; p=.22). There was no association of acute or chronic GVHD with MICA donor-recipient mismatch (HR=1.05; 95% 95% CI, 0.66-1.68; p=.83 and HR=0.94; 95% CI, 0.51-1.76; p=.85, respectively) or V/V donor MICA-129 genotypes (HR=1.02; 95% CI, 0.79-1.31; p=.89 and HR=0.89; 95% CI, 0.65-1.22; p=.47, respectively). CONCLUSION: These findings suggest that the donor MICA-129V/V genotype with weak NKG2D receptor binding affinity is associated with an increased risk of CMV infection and disease after alloHCT.


Posted January 15th 2020

Interdependence of Atrial Fibrillation and Heart Failure With a Preserved Ejection Fraction Reflects a Common Underlying Atrial and Ventricular Myopathy.

Milton Packer M.D.
Milton Packer M.D.

Packer, M., C. S. P. Lam, L. H. Lund and M. M. Redfield (2020). “Interdependence of Atrial Fibrillation and Heart Failure With a Preserved Ejection Fraction Reflects a Common Underlying Atrial and Ventricular Myopathy.” Circulation 141(1): 4-6.

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Atrial fibrillation (AF) and heart failure with a preserved ejection fraction (HFpEF) are closely intertwined disorders that afflict millions of people, many of whom are obese or have diabetes mellitus or other proinflammatory conditions. Conceivably, the convergence of AF and HFpEF might be explained by 2 distinctly different frameworks. On the one hand, it is possible that each phenotype might lead sequentially to the other (ie, the increased left ventricular [LV] filling pressure in HFpEF may cause left atrial [LA] dilatation that triggers AF, and conversely, the rapid heart rate that accompanies AF might lead to LV fibrosis, although there is little evidence to support this hypothesis). On the other hand, and more likely, the 2 disorders may be parallel manifestations of the same underlying myocardial disease, which causes AF (because it affects the LA) and HFpEF (because it afflicts the LV) . . . AF and HFpEF demonstrate an exceptionally high degree of clinical and epidemiological convergence. Regardless of which disorder presents first, both are or will soon become evident in the same patients. AF and HFpEF appear to both be manifestations of a common underlying atrial and ventricular myopathy that is triggered when a systemic inflammatory or metabolic disorder causes coronary microvascular dysfunction and fibrosis of the atrial and ventricular myocardium, a process that may be mediated or exacerbated by inflammation in the adjoining epicardial adipose tissue. (Excerpts from text, p. 4-5; no abstract available.)


Posted January 15th 2020

Is Long-Standing Atrial Fibrillation a Biomarker of or Contributor to the Symptoms or Progression of Chronic Heart Failure?

Milton Packer M.D.
Milton Packer M.D.

Packer, M. (2020). “Is Long-Standing Atrial Fibrillation a Biomarker of or Contributor to the Symptoms or Progression of Chronic Heart Failure?” Am J Med 133(1): 17-18.

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There is consensus that atrial fibrillation is a biomarker of the disease process in heart failure. Prolonged atrial distension due to chronic increases in left ventricular filling pressures contributes to the genesis of the arrhythmia. The disease process in the ventricles can also affect the atrial myocardium. Additionally, the atria are susceptible to fibrosis that results from the inflammation of epicardial adipose tissue that is seen in obesity or diabetes. The degree of atrial fibrosis is particularly marked in patients with persistent and long-standing atrial fibrillation. When the left atrium is severely fibrotic, restoration of sinus rhythm does not fully restore force transmission to the left ventricle. Patients with heart failure are at increased risk of stroke and systemic thromboembolism, whether in sinus rhythm or atrial fibrillation. The risk of stroke is higher in those with atrial fibrillation, perhaps because their atrial disease is more severe. Unless contraindicated, patients with atrial fibrillation should receive non-vitamin K-dependent oral anticoagulants, which have been shown to be noninferior or superior to warfarin in preventing stroke, but carry a lower risk of intracranial bleeding, particularly in patients with heart failure. However, even in sinus rhythm, the use of these drugs (e.g., rivaroxaban) in chronic heart failure appears to reduce the risk of stroke. Does atrial fibrillation itself contribute to the progression of heart failure? (Excerpt from text, p. 17; no abstract available.)