Research Spotlight

Posted October 31st 2020

Ventricular Septal Myectomy for the Treatment of Left Ventricular Outflow Tract Obstruction Due to Fabry Disease.

Peter McCullough, M.D.

Peter McCullough, M.D.

Raju, B., Roberts, C.S., Sathyamoorthy, M., Schiffman, R., Swift, C. and McCullough, P.A. (2020). “Ventricular Septal Myectomy for the Treatment of Left Ventricular Outflow Tract Obstruction Due to Fabry Disease.” Am J Cardiol 132: 160-164.

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Fabry cardiomyopathy can cause symptomatic left ventricular outflow tract obstruction. We review a case of Fabry cardiomyopathy mimicking hypertrophic cardiomyopathy on echocardiography with severe left ventricular outflow tract obstruction treated with ventricular septal myectomy.


Posted October 31st 2020

Considerations for Management of Acute Coronary Syndromes During the SARS-CoV-2 (COVID-19) Pandemic.

Peter McCullough, M.D.

Peter McCullough, M.D.

Briedis, K., Aldujeli, A., Aldujeili, M., Briede, K., Zaliunas, R., Hamadeh, A., Stoler, R.C. and McCullough, P.A. (2020). “Considerations for Management of Acute Coronary Syndromes During the SARS-CoV-2 (COVID-19) Pandemic.” Am J Cardiol 131: 115-119.

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Accumulating evidence suggests that influenza and influenza-like illnesses can act as a trigger for acute myocardial infarction. Despite these unprecedented times providers should not overlook acute coronary syndrome (ACS) guidelines, but may choose to modify the recommended approach in situations with confirmed or suspected COVID-19 disease. In this document, we suggest recommendations as to how to triage patients diagnosed with ACSs and provide with algorithms of how to manage the patients and decide the appropriate treatment options in the era of COVID-19 pandemic. We also address the inpatient logistics and discharge to follow-up considerations for the function of already established ACS network during the pandemic.


Posted October 31st 2020

Impact of Transcatheter Aortic Valve Replacement on Severity of Chronic Kidney Disease.

Michael J. Mack M.D.

Michael J. Mack M.D.

Cubeddu, R.J., Asher, C.R., Lowry, A.M., Blackstone, E.H., Kapadia, S.R., Alu, M.C., Thourani, V.H., Mack, M.J., Kodali, S.K., Herrmann, H.C., Forcillo, J., Babaliaros, V.C., Devireddy, C.M., Malaisrie, S.C., Davidson, C.J., Jaber, W.A., Leon, M.B. and Svensson, L.G. (2020). “Impact of Transcatheter Aortic Valve Replacement on Severity of Chronic Kidney Disease.” J Am Coll Cardiol 76(12): 1410-1421.

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BACKGROUND: The effect of transcatheter aortic valve replacement (TAVR) on kidney function stage in patients with aortic stenosis remains poorly understood. We hypothesized that in some patients, TAVR results in improved kidney function by alleviating cardiorenal syndrome. OBJECTIVES: The purpose of this study was to assess change in chronic kidney disease (CKD) stage following TAVR, identify variables associated with pre- and post-TAVR estimated glomerular filtration rate (eGFR), and assess association of post-TAVR eGFR with mortality. METHODS: Patients (n = 5,190) receiving TAVR in the PARTNER (Placement of Aortic Transcatheter Valves) 1, 2, and PARTNER 2 S3 trials between April 2007 and October 2014 were included. Pre-TAVR and procedural variables associated with post-TAVR eGFR, change in CKD stage at ≤7 days post-TAVR, and association of post-TAVR eGFR on intermediate-term mortality were assessed. RESULTS: At baseline, CKD stage ≥2 was present in 91% of patients. CKD stage either improved or was unchanged following TAVR in the majority of patients (77% stage 1, 90% stage 2, 89% stage 3A, 94% stage 3B, and 99% stage 4). Progression to CKD stage 5 occurred in 1 (0.035%) of 2,892 patients within 7 days post-TAVR. Of 3,546 patients in whom data were available, 70 (2.0%) underwent post-TAVR dialysis. Higher pre-TAVR eGFR and transfemoral approach were strongly associated with higher post-TAVR eGFR. Lower baseline and longitudinal post-TAVR eGFR were associated with lower intermediate-term survival. CONCLUSIONS: In patients with severe aortic stenosis undergoing TAVR, even with baseline impaired eGFR, CKD stage is more likely to stay the same or improve than worsen. Aortic stenosis may contribute to cardiorenal syndrome that improves with TAVR.


Posted October 31st 2020

Impact of Tricuspid Regurgitation on Clinical Outcomes: The COAPT Trial.

Michael J. Mack M.D.

Michael J. Mack M.D.

Hahn, R.T., Asch, F., Weissman, N.J., Grayburn, P., Kar, S., Lim, S., Ben-Yehuda, O., Shahim, B., Chen, S., Liu, M., Redfors, B., Medvedofsky, D., Puri, R., Kapadia, S., Sannino, A., Lindenfeld, J., Abraham, W.T., Mack, M.J. and Stone, G.W. (2020). “Impact of Tricuspid Regurgitation on Clinical Outcomes: The COAPT Trial.” J Am Coll Cardiol 76(11): 1305-1314.

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BACKGROUND: The presence of tricuspid regurgitation (TR) may affect prognosis in patients with mitral regurgitation (MR). OBJECTIVES: This study sought to determine the impact of TR on outcomes in patients with heart failure and severe secondary MR randomized to guideline-directed medical therapy (GDMT) or edge-to-edge repair with the MitraClip in the COAPT (Cardiovascular Outcomes Assessment of the MitraClip Percutaneous Therapy for Heart Failure Patients With Functional Mitral Regurgitation) trial. METHODS: A total of 614 patients with symptomatic heart failure with moderate to severe (3+) or severe (4+) secondary MR were randomized to maximally tolerated GDMT plus MitraClip or GDMT alone; 599 had core laboratory evaluable echocardiograms. Patients were divided into 2 groups by baseline TR severity: none/trace/mild TR (≤Mild TR) (n = 501 [83.6%]) and moderate/severe TR (≥Mod TR) (n = 98 [16.4%]). Two-year composite endpoints of death or heart failure hospitalization (HFH) and the individual endpoints were analyzed. RESULTS: Patients with ≥Mod TR were more likely to be New York Heart Association functional class III/IV (p < 0.0001) and have a Society of Thoracic Surgeons score of ≥8 (p < 0.0001), anemia (p = 0.02), chronic kidney disease (p = 0.003), and higher N-terminal pro-B-type natriuretic peptide (p = 0.02) than those with ≤Mild TR. Patients with ≥Mod TR had more severe MR (p = 0.0005) despite smaller left ventricular volumes (p = 0.005) and higher right ventricular systolic pressure (p < 0.0001). At 2 years, the composite rate of death or HFH was higher in patients with ≥Mod TR compared with ≤Mild TR treated with GDMT alone (83.0% vs. 64.3%; hazard ratio: 1.74; 95% confidence interval: 1.24 to 2.45; p = 0.001) but not following MitraClip (48.2% vs. 44.0%; hazard ratio: 1.14; 95% confidence interval: 0.71 to 1.84; p = 0.59). Rates of death or HFH, as well as death and HFH alone, were reduced by MitraClip compared with GDMT, irrespective of baseline TR grade (p(interaction) = 0.16, 0.29, and 0.21 respectively). CONCLUSIONS: Patients with severe secondary MR who also had ≥Mod TR had worse clinical and echocardiographic characteristics and worse clinical outcomes compared to those with ≤Mild TR. Within the COAPT trial, MitraClip improved outcomes in patients with and without ≥Mod TR severity compared with GDMT alone. (Cardiovascular Outcomes Assessment of the MitraClip Percutaneous Therapy for Heart Failure Patients With Functional Mitral Regurgitation [COAPT]; NCT01626079).


Posted October 31st 2020

Angiomyolipoma of the Nasal Cavity: A Distinct Entity. A Case Report and Literature Review.

Lina Liu, M.D.

Lina Liu, M.D.

Wang, L., Leng, B. and Liu, L. (2020). “Angiomyolipoma of the Nasal Cavity: A Distinct Entity. A Case Report and Literature Review.” Head Neck Pathol Sept 16. [Epub ahead of print.].

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Angiomyolipoma (AML) is a mesenchymal tumor commonly found in the kidneys. Extra-renal AML is uncommon and especially rare in the nasal cavity. To our knowledge, fewer than five cases of nasal AML are reported in the United States. We present a case of nasal AML in a 65-year-old man with a history of rhinosinusitis and obstruction of the left nasal cavity. The lesion comprised of admixed spindled smooth muscle cells, mature adipose tissue, and thick-walled blood vessels. Smooth muscle differentiation of the spindled cells was confirmed by expression of smooth muscle actin. Surprisingly, melanocytic markers, such as HMB45, were negative. The histologic features and immunoprofile suggest that nasal AML is pathologically distinct from neoplastic AMLs/perivascular epithelioid cell tumors (PEComas) that typically occur in the kidney. We propose that nasal AML is a hamartomatous lesion rather than neoplastic. Although AML is rare in the nasal cavity, it should be considered in the differential diagnosis of clinically benign nasal masses.