Cardionerds: A Cardiology Podcast

The following question refers to Section 4.3 of the 2021 ESC CV Prevention Guidelines. The question is asked by CardioNerds Academy Intern Dr. Maryam Barkhordarian, answered first by medicine resident CardioNerds Academy House Chief Dr. Ahmed Ghoneem, and then by expert faculty Dr. Kim Williams. Dr. Williams is Chief of the Division of Cardiology and is Professor of Medicine and Cardiology at Rush University Medical Center. He has served as President of ASNC, Chairman of the Board of the Association of Black Cardiologists (ABC, 2008-2010), and President of the American College of Cardiology (ACC, 2015-2016). The CardioNerds Decipher The Guidelines Series for the 2021 ESC CV Prevention Guidelines represents a collaboration with the ACC Prevention of CVD Section, the National Lipid Association, and Preventive Cardiovascular Nurses Association. Enjoy this Circulation 2022 Paths to Discovery article to learn about the CardioNerds story, mission, and values. Question #27 Mr. O is a 48-year-old man with a past medical history significant for obesity (BMI is 42kg/m2), hypertension, type 2 diabetes mellitus, and hypercholesterolemia. His calculated ASCVD risk score today is 18.8%. You counsel him on the importance of weight loss in the context of CVD risk reduction. Which of the following weight loss recommendations is appropriate? A Maintaining a weight loss of at least 25% from baseline is required to influence blood pressure, cholesterol, and glycemic control.  B Hypocaloric diets lead to short term weight loss, but a healthy diet should be maintained over time to reduce CVD risk. C Liraglutide can be used to induce weight loss, as an alternative to diet and exercise. D Bariatric surgery is effective for weight loss but has no ASCVD risk reduction benefit. Answer #27 Explanation The correct answer is B. Energy restriction is the cornerstone of management of obesity. All the different types of hypocaloric diets achieve similar short-term weight loss, but these effects tend to diminish by 12 months. It is a class I recommendation to maintain a healthy diet over time to achieve CVD risk reduction. The Mediterranean diet is an example of a diet that can have persistent CV benefit beyond the 12 months. Choice A is incorrect because maintaining even a moderate weight loss of 5 – 10% from baseline has favorable effects on risk factors including blood pressure, cholesterol, and glycemic control, as well as on premature all-cause mortality. Choice C is incorrect because medications approved as aids to weight loss (such as liraglutide, orlistat and naltrexone/bupropion) may be used in addition to lifestyle measures to achieve weight loss and maintenance; they are not alternatives to a healthy lifestyle. Meta-analysis of medication-assisted weight loss found favorable effects on BP, glycemic control, and ASCVD mortality. Choice D is incorrect because patients undergoing bariatric surgery had over 50% lower risks of total ASCVD and cancer mortality compared with people of similar weight who did not have surgery. Bariatric surgery should be considered for obese high-risk individuals when lifestyle change does not result in maintained weight loss (Class IIa). The ACC/AHA guidelines focused primarily on lifestyle interventions for obesity and had no specific recommendations for bariatric surgery or medication-assisted weight loss. Main Takeaway Weight reduction (even as low as 5-10% from baseline) and long-term maintenance of a healthy diet are recommended to improve the CVD risk profile of overweight and obese people. Medication and/or bariatric surgery may have a useful adjunctive role in some patients. Guideline Loc. Section 4.3.3 CardioNerds Decipher the Guidelines – 2021 ESC Prevention SeriesCardioNerds Episode PageCardioNerds AcademyCardionerds Healy Honor Roll CardioNerds Journal ClubSubscribe to The Heartbeat Newsletter!Check out CardioNerds SWAG!Become a CardioNerds Patron!

The following question refers to Section 7.6 of the 2022 AHA/ACC/HFSA Guideline for the Management of Heart Failure. The question is asked by premedical student and CardioNerds Intern Pacey Wetstein, answered first by Mayo Clinic Cardiology Fellow and CardioNerds Academy Chief Dr. Teodora Donisan, and then by expert faculty Dr. Nancy Sweitzer. Dr. Sweitzer is Professor of Medicine, Vice Chair of Clinical Research for the Department of Medicine, and Director of Clinical Research for the Division of Cardiology at Washington University School of Medicine. She is the editor-in-chief of Circulation: Heart Failure. Dr. Sweitzer is a faculty mentor for this Decipher the HF Guidelines series. The Decipher the Guidelines: 2022 AHA / ACC / HFSA Guideline for The Management of Heart Failure series was developed by the CardioNerds and created in collaboration with the American Heart Association and the Heart Failure Society of America. It was created by 30 trainees spanning college through advanced fellowship under the leadership of CardioNerds Cofounders Dr. Amit Goyal and Dr. Dan Ambinder, with mentorship from Dr. Anu Lala, Dr. Robert Mentz, and Dr. Nancy Sweitzer. We thank Dr. Judy Bezanson and Dr. Elliott Antman for tremendous guidance. Enjoy this Circulation 2022 Paths to Discovery article to learn about the CardioNerds story, mission, and values. Clinical Trials Talks Question #21 Ms. Smith is a 56-year-old woman following up in the cardiology clinic for a history of heart failure with reduced ejection fraction. Two years ago, she was diagnosed with non-ischemic cardiomyopathy with a left ventricular ejection fraction (LVEF) of 30%. Over time, she was initiated and optimized on guideline directed medical therapy. She is currently on Carvedilol 12.5 mg BID, Sacubitril/Valsartan 49/51 mg BID, Spironolactone 25 mg daily, Empagliflozin 10 mg daily, and Furosemide PRN for weight gain.   On today’s visit, her BP is 110/80 mmHg, and her HR is 67 bpm. Labs show a creatinine of 0.9 mg/dL, potassium of 5.1 mEq/L, NT-proBNP of 150 ng/L, and a HbA1c of 5.8%. Follow up transthoracic echocardiogram showed an improvement in LVEF to 55%. What are the most appropriate therapy recommendations for Ms. Smith? A Discontinue spironolactone B Discontinue empagliflozin C Decrease the dose of carvedilol D Continue current therapy Answer #21 The correct answer is D – continue current therapy. The patient described above was initially diagnosed with HFrEF and experienced significant symptomatic improvement with GDMT, so she now has heart failure with improved ejection fraction (HFimpEF). In patients with HFimpEF after treatment, GDMT should be continued to prevent relapse of HF and LV dysfunction, even in patients who may become asymptomatic (Class 1, LOE B-R). Although symptoms, functional capacity, LVEF and reverse remodeling can improve with GDMT, structural abnormalities of the LV and its function do not fully normalize, causing symptoms and biomarker changes to persist or recur if treatment is deescalated. Improvements in EF do not always reflect sustained recovery; rather, they signify remission.   Of note, HF relapse can be defined by at least 1 of the following: o   A drop in the EF by >10% and to < 50% o   An increase in LVEDV by >10% and to higher than the normal range o   A 2-fold rise in NT-proBNP concentration and to > 400 ng/L o   Clinical evidence of HF on examination Choice A is incorrect as it would be incorrect to discontinue spironolactone. A potassium of 5.1 is still within the acceptable limit in a patient who has been on Spironolactone for two years, and this medication is an important part of GDMT for HFrEF.   Despite the improvement in Hb A1c, empagliflozin should be continued for heart failure with improved ejection fraction, as it is part of routine GDMT of HFrEF even in the absence of diabetes. Choice B is thus incorrect. Similarly, carvedilol should be continued at the same dose as the patient’s heart rate is within the desired range. Furthermore, all GDMT should be continued in patients with HFimpEF, as emphasized above. Choice C is therefore also incorrect. In patients with HFimpEF after treatment, GDMT should be continued to prevent relapse of HF and LV dysfunction, even in patients who may become asymptomatic. (Class 1, LOE B-R). Section 7.6.2 Decipher the Guidelines: 2022 Heart Failure Guidelines PageCardioNerds Episode PageCardioNerds AcademyCardionerds Healy Honor Roll CardioNerds Journal ClubSubscribe to The Heartbeat Newsletter!Check out CardioNerds SWAG!Become a CardioNerds Patron!

CardioNerds Co-Founder, Dr. Amit Goyal, along with Series Co-Chairs, Dr. Yoav Karpenshif and Dr. Eunice Dugan, and episode Lead, Dr. Sean Dikdan, had the opportunity to expand their knowledge on the topic of ventricular tachycardia and electrical storm from esteemed faculty expert, Dr. Janice Chyou. Audio editing by CardioNerds Academy Intern, Dr. Maryam Barkhordarian. Electrical storm (ES) is a life-threatening arrhythmia syndrome. It is characterized by frequently occurring bouts of unstable cardiac arrythmias. It typically occurs in patients with susceptible substrate, either myocardial scar or a genetic predisposition. The adrenergic input of the sympathetic nervous system can perpetuate arrythmia. In the acute setting, identifying reversible triggers, such as ischemia, electrolyte imbalances, and heart failure, is important. Treatment is complex and varies based on previous treatments received and the presence of intra-cardiac devices. Many options are available to treat ES, including medications, intubation and sedation, procedures and surgeries targeting the autonomic nervous system, and catheter ablation to modulate the myocardial substrate. A multidisciplinary team of cardiologists, intensivists, electrophysiologists, surgeons, and more are necessary to manage this complex disease. The CardioNerds Cardiac Critical Care Series is a multi-institutional collaboration made possible by contributions of stellar fellow leads and expert faculty from several programs, led by series co-chairs, Dr. Mark Belkin, Dr. Eunice Dugan, Dr. Karan Desai, and Dr. Yoav Karpenshif. Enjoy this Circulation 2022 Paths to Discovery article to learn about the CardioNerds story, mission, and values. Pearls • Notes • References • Production Team CardioNerds Cardiac Critical Care PageCardioNerds Episode PageCardioNerds AcademyCardionerds Healy Honor Roll CardioNerds Journal ClubSubscribe to The Heartbeat Newsletter!Check out CardioNerds SWAG!Become a CardioNerds Patron! Pearls and Quotes – Management of Ventricular Tachycardia and Electrical Storm Electrical storm is defined as 3 or more episodes of VF, sustained VT, or appropriate ICD shocks within 24 hours. It occurs more commonly in ischemic compared to non-ischemic cardiomyopathy, and it is associated with a poor prognosis and high cardiovascular mortality. The classic triad of electrical storm is a trigger, a myocardial susceptible substrate, and autonomic input perpetuating the storm. Triggers for electrical storm include ischemia, heart failure, electrolyte abnormalities, hypoxia, drug-related arrhythmogenicity, and thyrotoxicosis. A thorough evaluation of possible triggers is necessary for each patient, but it is uncommonly found. The evaluation may include laboratory studies, genetic testing, advanced imaging, or invasive testing. Acute treatment options involve acute resuscitation, pharmacotherapy with antiarrhythmics and beta-blockers, device interrogation and possible reprogramming, and sedation. Subacute treatment involves autonomic modulation and catheter ablation. Surgical treatments include sympathectomies and, ultimately, heart transplant. Catheter ablation is safe and effective for the treatment of electrical storm. In select patients, hemodynamic peri-procedural hemodynamic support should be considered. Show notes – Management of Ventricular Tachycardia and Electrical Storm Simple diagram of the classic “triad” of ES (see reference 10). Treatment algorithm provided by the 2017 AHA/ACC/HRS guidelines (see reference 1). 1. Define electrical storm. Electrical storm (ES), also called “arrhythmic storm” or “VT storm” refers to a state of cardiac instability associated with 3 or more episodes of VF, sustained VT, or appropriate ICD shocks within 24 hours. Sustained VT refers to 30 seconds of VT or hemodynamically unstable VT requiring termination in < 30 seconds. Incessant VT refers to continued, sustained hemodynamically stable VT that lasts longer than one hour. VT is incessant or recurrent when it recurs promptly despite repeated intervention for termination.1,2 In patients with ICDs for secondary prevention, ES is estimated to occur in 10-28% of patients.3–5 This incidence is much lower in patients who have ICDs implanted for primary prevention in whom the incidence has been estimated as low as 4% at 20 months of follow up.6 ES occurs at similar rates in patients with ischemic or non-ischemic cardiomyopathy.7 ES is associated with a poor prognosis and high cardiovascular mortality. The three-month mortality in patients with an episode of ES has been estimated at up to 18 times higher than in patients without any VT.6 Risk factors for the development of ES include male sex, advanced age, low left ventricular ejection fraction, use of class 1A antiarrhythmic drugs, and the presence of cardiovascular comorbidities.8,9 2. Evaluate the cause of VT storm (e.g., evaluation for ischemia, sarcoidosis , etc) The classic triad of ES is a trigger, a substrate susceptible to ES, and autonomic input perpetuating the storm.10 Potential triggers are varied and typically include myocardial ischemia, decompensated heart failure, electrolyte abnormalities, hypoxia, drug-related arrhythmogenicity, and thyrotoxicosis.4,11 A clear trigger is often not found (only 13% of the time by some estimates).12 Searching for a trigger should not delay management decisions in the acute setting. Structural heart disease unrelated to ischemia such as congenital heart disease and infiltrative cardiomyopathies can serve as the substrate for ES. Conditions related to genetic causes such as long QT syndrome or catecholaminergic polymorphic VT may be a rare etiology. These conditions represent an electrophysiologic substrate as opposed to a structural substrate.13 3. Choose an initial management strategy for patients with electrical storm in the CCU. Treatment of ES is complex. The initial steps in management involve resuscitation, pharmacotherapy, device interrogation and reprogramming, and sedation. ACLS should be used in patients with pulseless VT or VF. Patients with and without cardioverter-defibrillators may be treated differently. Defibrillations from an implanted device accentuate sympathetic tone and may perpetuate further arrhythmia. Once a patient is stabilized, more advanced therapies involving autonomic modulation or catheter ablation (CA) can be utilized. In the patient with ischemia, emergent revascularization should be pursued. The need for mechanical circulatory support (MCS) should be determined. Inotropes and many vasopressors are sympathetic agonists and may worsen the arrhythmia by accentuating adrenergic tone, and so the benefits of improved hemodynamics need to be weighed against the risk of worsening electrical instability. Initial pharmacotherapy in ES includes an antiarrhythmic drug and a beta blocker. Typically loading the patient with IV amiodarone and administering a non-selective beta blocker like propranolol is done. This combination has been shown in ES patients to have superior freedom of arrhythmia compared to using metoprolol.14 Propranolol’s superiority may also be due to its ability to cross the blood-brain barrier. Lidocaine has improved efficacy in ischemic VT.15,16 Procainamide has been shown to be useful in patients with hemodynamically stable VT.17 4. Identify predisposing conditions that should be managed to help treat electrical storm such as ischemia and AHF. Identifying and managing specific triggers is an important initial step in the management of ES. Hypoxia on vital signs or evidence of decompensated HF on exam (with JVD, edema, crackles on auscultation) can implicate volume overload; this can be managed with diuresis. Ischemic ECG changes on the 12-lead ECG when the patient’s ventricular arrhythmia is broken, can suggest myocardial ischemia. If ischemia is believed to be the trigger, urgent revascularization should be pursued while resuscitation is underway. Blood work should include screening for electrolyte abnormalities and thyroid disease. Carefully screening the patient’s medication list and checking a digoxin level  (when appropriate) can help detect drug-induced arrhythmia. Once out of the acute setting, genetic testing may be important in patients without structural disease for determining an etiology. Idiopathic VT, Brugada syndrome, long QT syndrome, short QT syndrome, early repolarization syndrome, catecholaminergic polymorphic VT, arrhythmogenic right ventricular cardiomyopathy, and cardiac sarcoidosis are potential etiologies that may be related to ES.10 5. Recognize when to use general anesthesia to aid in the stabilization of electrical storm and incessant VT. Intubation and deep sedation are immediate next steps to minimize the sympathetic drive contributing to the arrhythmia. This treatment is very effective at terminating arrythmia and preventing immediate recurrence.18,19 This step is used in the acute setting for ES that persists despite pharmacotherapy. Note that propofol is a negative inotrope with the potential to worsen heart failure in decompensated patients and precipitate shock. In addition to breaking the sympathetic cycle that drives this pathophysiology, sedation mitigates some of the psychological stress that repeated ICD shocks can cause in patients.20 6. Describe considerations specific to patients with implanted ICDs. If a patient with an ICD presents with ES, the device should be interrogated. It is important to confirm the shocks are appropriate. Inappropriate shocks can occur in up to 40% of patients with an ICD; causes may include atrial arrythmia, oversensing, and lead fracture.21,22 Inappropriate ICD shocks are associated with a worse outcome. Overdrive pacing is a possible therapy to prevent ES. If the ES is hemodynamically stable, then the ICD therapies may be disabled manually or with the use of a magnet. If anti-tachycardia pacing (ATP) treats the ventricular arrythmia effectively, adjusting these settings and increasing the use of ATP can mitigate unnecessary shocks in the future.23 7. Understand the role of catheter ablation in the management of electrical storm. Research has shown an excellent response of ES to catheter ablation (CA). CA has a class 1 indication in patients with ES due to anti-arrhythmic drug refractory VA in both ischemic and nonischemic cardiomyopathy.2 Treatment of an initial episode of ES with CA has shown a reduction in all-cause mortality compared to other modalities.24 At nearly 1-2 years of follow up, nearly 90% of patients with ES that undergo CA are free from further ES, and roughly two-thirds of these patients are free from any ventricular arrythmia (VA) recurrence.25,26 CA is also relatively safe in this setting, with procedure-related mortality estimated to be less than 1%.27 Rapid transfer to an experienced catheter ablation capable facility is important in all critically ill patients with ES. 8. Consider when it may be appropriate to use mechanical support such as IABP, pVAD and ECMO. Mechanical circulatory support (MCS) may be necessary to maintain adequate perfusion when the patient is suffering from cardiogenic shock due to unstable arrhythmia. Patients with high risk for hemodynamic decompensation during CA can be preemptively supported with MCS. This practice has been shown to improve mortality compared to rescue or no MCS. 28,29 The PAAINESD score may be useful in identifying high risk patients. This score assigns numerical values to the following risk factors: pulmonary disease, age over 60 years, general anesthesia, ischemic cardiomyopathy, NYHA class III or IV, LV EF < 25%, VT storm, and diabetes mellitus. 2,28 An intra-aortic balloon pump may be sufficient but requires the patient to have enough adequate forward flow to generate a pulse. Extracorporeal membrane oxygenation (ECMO) has been studied and shows good long-term outcomes.29 Guidelines have a IIa recommendation for hemodynamic support with ECMO or a temporary LVAD during CA in select patients. 2 9. Discuss other strategies such as sympathectomies (stellate ganglion block vs. surgical), stereotactic radio ablation, and transplant for refractory cases. There are several therapies available to treat ES that specifically target the autonomic nervous system (ANS).30 While sedation is used for this purpose acutely, other interventions seek to mitigate sympathetic activity in the subacute or chronic setting. These include stellate ganglion blockade (SGB), thoracic epidural anesthesia (TEA), cardiac sympathetic denervation (CSD), and renal artery sympathetic denervation (RSD). Percutaneous SBG involves an injection of anesthetic directly into the stellate ganglia with or without ultrasound guidance. This is a temporizing measure that can be performed in the acute or subacute setting. It has shown complete suppression of VA in 50% of patients for the subsequent 48 hours.31 TEA involves the percutaneous administration of a local anesthetic directly into the thoracic epidural space. This is also a temporary treatment best used as a bridge to definitive treatment, such as CA or surgical denervation. In ES patients with a failed CA TEA can reduce VA up to 80% in most patients.32 CSD is a surgical measure that offers a more permanent solution. It can be useful in refractory ES that has not responded to multiple treatments. CSD has achieved 80% event-free survival up to 2 years.33 Guidelines recommend CSD in ES when beta-blockade, anti-arrhythmic drugs, and CA are deemed ineffective with a class IIb recommendation.1 RSD functions similarly but has the added benefit of being non-surgical and directly reducing catecholamine secretion. Cardiac transplantation would be indicated in a patient that has unrelenting ES despite these aggressive measures. Patients with MCS and life-threatening arrhythmias qualify as status 1 for OHT.34 Whereas VT/VF without MCS by itself would qualify a patient as status 2. References Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death. Circulation 2018;138(13):e272–391. Cronin EM, Bogun FM, Maury P, et al. 2019 HRS/EHRA/APHRS/LAHRS expert consensus statement on catheter ablation of ventricular arrhythmias. Europace 2019;21(8):1143–4. Exner D v, Pinski SL, Wyse DG, et al. Electrical Storm Presages Nonsudden Death The Antiarrhythmics Versus Implantable Defibrillators (AVID) Trial. Circulation [Internet] 2001;103:2066–71. Available from: http://www.circulationaha.org Credner SC, Klingenheben T, Mauss O, Sticherling C, Hohnloser SH. Electrical Storm in Patients With Transvenous Implantable Cardioverter-Defibrillators Incidence, Management and Prognostic Implications. J Am Coll Cardiol 1998;32(7):1909–15. Bänsch D, Böcker D, Brunn J, Weber M, Breithardt G, Block M. Clusters of Ventricular Tachycardias Signify Impaired Survival in Patients With Idiopathic Dilated Cardiomyopathy and Implantable Cardioverter Defibrillators. J Am Coll Cardiol 2000;36(2):566–73. Sesselberg HW, Moss AJ, McNitt S, et al. Ventricular arrhythmia storms in postinfarction patients with implantable defibrillators for primary prevention indications: A MADIT-II substudy. Heart Rhythm 2007;4(11):1395–402. Streitner F, Kuschyk J, Dietrich C, et al. Comparison of ventricular tachyarrhythmia characteristics in patients with idiopathic dilated or ischemic cardiomyopathy and defibrillators implanted for primary prevention. Clin Cardiol 2011;34(10):604–9. Vergara P, Tung R, Vaseghi M, et al. Successful ventricular tachycardia ablation in patients with electrical storm reduces recurrences and improves survival. Heart Rhythm 2018;15(1):48–55. Emkanjoo Z, Alihasani N, Alizadeh A, et al. Electrical Storm in Patients with Implantable Cardioverter-Defibrillators Can It Be Forecast? Tex Heart Inst J 2009;36(6):563–7. Kowlgi GN, Cha YM. Management of ventricular electrical storm: A contemporary appraisal. Europace 2020;22(12):1768–80. Muser D, Liang J, Santangeli P. Electrical Storm in Patients with Implantable Cardioverter-defibrillators: A Practical Overview. J Innov Card Rhythm Manag 2017;8(10):2853–61. Stefan H. Hohnloser, Hussein R. Al-Khalidi, Craig M. Pratt, et al. Electrical storm in patients with an implantable defibrillator: incidence, features, and preventive therapy: insights from a randomized trial. Eur Heart J 2006;27(24):3027–32. Geraghty L, Santangeli P, Tedrow UB, Shivkumar K, Kumar S. Contemporary Management of Electrical Storm. Heart Lung Circ 2019;28(1):123–33. Chatzidou S, Kontogiannis C, Tsilimigras DI, et al. Propranolol Versus Metoprolol for Treatment of Electrical Storm in Patients With Implantable Cardioverter-Defibrillator. J Am Coll Cardiol 2018;71(17):1897–906. MacMahon S, Collins R, Peto R, Koster RW, Yusuf S, MacMahon M. Effects of Prophylactic Lidocaine in Suspected Acute Myocardial Infarction. J Am Med Assoc [Internet] 1988;260(13):1910–6. Available from: https://jamanetwork.com/ Collinsworth KA, Kalman SM, Harrison DC. The Clinical Pharmacology of Lidocaine as an Antiarrhythymic Drug. Circulation [Internet] 1974;50(6):1217–30. Available from: http://ahajournals.org Ortiz M, Martin A, Arribas F, et al. Randomized comparison of intravenous procainamide vs. intravenous amiodarone for the acute treatment of tolerated wide QRS tachycardia: The PROCAMIO study. Eur Heart J 2017;38(17):1329–35. Martins RP, Urien JM, Barbarot N, et al. Effectiveness of Deep Sedation for Patients With Intractable Electrical Storm Refractory to Antiarrhythmic Drugs. Circulation 2020;142(16):1599–601. Bundgaard JS, Jacobsen PK, Grand J, et al. Deep sedation as temporary bridge to definitive treatment of ventricular arrhythmia storm. Eur Heart J Acute Cardiovasc Care 2020;9(6):657–64. Passman R, Subacius H, Ruo B, et al. Implantable Cardioverter Defibrillators and Quality of Life Results From the Defibrillators in Nonischemic Cardiomyopathy Treatment Evaluation Study. Journal of the American Medical Association Internal Medicine [Internet] 2007;167(20):2226–32. Available from: https://jamanetwork.com/ Powell BD, Saxon LA, Boehmer JP, et al. Survival after shock therapy in implantable cardioverter-defibrillator and cardiac resynchronization therapy-defibrillator recipients according to rhythm shocked: The altitude survival by rhythm study. J Am Coll Cardiol 2013;62(18):1674–9. van Rees JB, Borleffs CJW, de Bie MK, et al. Inappropriate implantable cardioverter-defibrillator shocks: Incidence, predictors, and impact on mortality. J Am Coll Cardiol 2011;57(5):556–62. Wathen MS, DeGroot PJ, Sweeney MO, et al. Prospective randomized multicenter trial of empirical antitachycardia pacing versus shocks for spontaneous rapid ventricular tachycardia in patients with implantable cardioverter-defibrillators: Pacing fast ventricular tachycardia reduces shock therapies (PainFREE Rx II) trial results. Circulation 2004;110(17):2591–6. Morawski S, Pruszkowska P, Sredniawa B, Lenarczyk R, Kalarus Z. Long-term outcome of catheter ablation and other form of therapy for electrical storm in patients with implantable cardioverter-defibrillators. Journal of Interventional Cardiac Electrophysiology 2017;50(3):227–34. Carbucicchio C, Santamaria M, Trevisi N, et al. Catheter ablation for the treatment of electrical storm in patients with implantable cardioverter-defibrillators : Short-and long-term outcomes in a prospective single-center study. Circulation 2008;117(4):462–9. Deneke T, Shin DI, Lawo T, et al. Catheter ablation of electrical storm in a collaborative hospital network. American Journal of Cardiology 2011;108(2):233–9. Nayyar S, Ganesan AN, Brooks AG, Sullivan T, Roberts-Thomson KC, Sanders P. Venturing into ventricular arrhythmia storm: A systematic review and meta-analysis. Eur Heart J 2013;34(8):560–9. Mariani S, Napp LC, lo Coco V, et al. Mechanical circulatory support for life-threatening arrhythmia: A systematic review. Int J Cardiol 2020;308:42–9. Baratto F, Pappalardo F, Oloriz T, et al. Extracorporeal Membrane Oxygenation for Hemodynamic Support of Ventricular Tachycardia Ablation. Circ Arrhythm Electrophysiol 2016;9(12). Zhu C, Hanna P, Rajendran PS, Shivkumar K. Neuromodulation for Ventricular Tachycardia and Atrial Fibrillation: A Clinical Scenario-Based Review. JACC Clin Electrophysiol 2019;5(8):881–96. Fudim M, Qadri YJ, Waldron NH, et al. Stellate Ganglion Blockade for the Treatment of Refractory Ventricular Arrhythmias. JACC Clin Electrophysiol 2020;6(5):562–71. Bourke T, Vaseghi M, Michowitz Y, et al. Neuraxial modulation for refractory ventricular arrhythmias: Value of thoracic epidural anesthesia and surgical left cardiac sympathetic denervation. Circulation 2010;121(21):2255–62. Li J, Liu Y, Yang F, et al. Video-Assisted Thoracoscopic Left Cardiac Sympathetic Denervation: A Reliable Minimally Invasive Approach for Congenital Long-QT Syndrome. Annals of Thoracic Surgery 2008;86(6):1955–8. Stevenson LW, Kormos RL, Young JB, Kirklin JK, Hunt SA. Major advantages and critical challenge for the proposed United States heart allocation system. Journal of Heart and Lung Transplantation 2016;35(5):547–9.

The following question refers to Sections 3.3 and 3.4 of the 2021 ESC CV Prevention Guidelines. The question is asked by CardioNerds Academy Intern student Dr. Adriana Mares, answered first by Brigham & Women’s medicine resident & Director of CardioNerds Internship Dr. Gurleen Kaur, and then by expert faculty Dr. Allison Bailey. Dr. Bailey is an advanced heart failure and transplant cardiologist at Centennial Heart. She is the editor-in-chief of the American College of Cardiology’s Extended Learning (ACCEL) editorial board and was a member of the writing group for the 2018 American Lipid Guidelines.  The CardioNerds Decipher The Guidelines Series for the 2021 ESC CV Prevention Guidelines represents a collaboration with the ACC Prevention of CVD Section, the National Lipid Association, and Preventive Cardiovascular Nurses Association. Enjoy this Circulation 2022 Paths to Discovery article to learn about the CardioNerds story, mission, and values. Question #26 Ms. Priya Clampsia is a 58-year-old never-smoker with a history of hypertension. Her BMI is 29 kg/m2. She also mentions having pre-eclampsia during her pregnancy many years ago. She describes a predominately sedentary lifestyle and works as a receptionist. You see her in the clinic to discuss routine preventive care. Her most recent lipid panel results were LDL of 101 mg/dL, HDL of 45 mg/dL, and triglycerides of 190 mg/dL. What additional step will provide valuable information regarding her CVD risk profile? A Send additional lab workup including C-reactive protein and lipoprotein (a) B Measure her waist circumference C Assess her work stress D Ask her about history of preterm birth E B, C, and D Answer #26 Explanation The correct answer is E – measuring her waist circumference, assessing her occupational stress, and obtaining history about adverse pregnancy outcomes including preterm birth all add valuable information for CVD risk stratification. BMI is easily measured and can be used to define categories of body weight. However, body fat stores in visceral tissue carry higher risk than subcutaneous fat and therefore, waist circumference can be a simple way to measure global and abdominal fat. When waist circumference is ≥102 cm in men and ≥88 cm in women, weight reduction is advised. While these WHO thresholds are widely accepted in Europe, it is important to note that different cut-offs may be appropriate in different ethnic groups. Work stress is important to ascertain as well because there is preliminary evidence of the detrimental impact of worse stress on ASCVD health, independent of conventional risk factors and their treatment. Work stress is determined by job strain (i.e., the combination of high demands and low control at work) and effort-reward imbalance. Pre-eclampsia is associated with increase in CVD risk by factor of 1.5-2.7 compared with all women. Both preterm (RR 1.6) and still birth (RR 1.5) are also associated with a moderate increase in CVD risk. Taking a thorough pregnancy history is important in determining future cardiovascular risk in women. The ESC guidelines give a Class IIb (LOE B) recommendation that in women with history of premature or stillbirth, periodic screening for hypertension and DM may be considered. Of note, the 2018 ACC/AHA guidelines include preeclampsia and premature menopause (occurring at age <40 years) as risk-enhancing factors for statin therapy but state that the mechanism or cause of preterm birth is often unknown, so it is difficult to include it as a risk-enhancing factor. Choice A (sending additional lab workup including CRP and LPa) is incorrect. The ESC guidelines do not recommend using routine circulating biomarkers as they do not improve risk prediction and publication bias distorts the evidence (Class III, LOE B). While some biomarkers like lipoprotein (a) are promising, further work is still needed. Conversely, the 2019 ACC/AHA guidelines do include, if measured, elevated high-sensitivity C-reactive protein (≥2mg/L) and elevated Lp(a) (>50mg/dL or >125nmol/L) and elevated apoB (≥130 mg/dL) as risk-enhancing factors. Specific indications for measuring Lp(a) include family history of premature ASCVD and specific indications for measuring apoB include triglyceride ≥200mg/dL. Main Takeaway The ESC guidelines do not recommend routine measurement of additional circulating and urine biomarkers as further data and research is still needed in this area; however, there are specific situations in which these biomarkers may be warranted. Guideline Loc. Section 3.3.7, 3.3.9, 3.3.10, 3.4.12 CardioNerds Decipher the Guidelines – 2021 ESC Prevention Series CardioNerds Episode Page CardioNerds Academy Cardionerds Healy Honor Roll CardioNerds Journal Club Subscribe to The Heartbeat Newsletter! Check out CardioNerds SWAG! Become a CardioNerds Patron!

The following question refers to Sections 7.3.2, 7.3.8, and 7.6.2 of the 2022 AHA/ACC/HFSA Guideline for the Management of Heart Failure. The question is asked by Palisades Medical Center medicine resident & CardioNerds Intern Dr. Maryam Barkhordarian, answered first by Hopkins Bayview medicine resident & CardioNerds Academy Fellow Dr. Ty Sweeny, and then by expert faculty Dr. Robert Mentz. Dr. Mentz is associate professor of medicine and section chief for Heart Failure at Duke University, a clinical researcher at the Duke Clinical Research Institute, and editor-in-chief of the Journal of Cardiac Failure. Dr. Mentz is a mentor for the CardioNerds Clinical Trials Network as lead principal investigator for PARAGLIDE-HF and is a series mentor for this very Decipher the Guidelines Series. For these reasons and many more, he was awarded the Master CardioNerd Award during ACC22. The Decipher the Guidelines: 2022 AHA / ACC / HFSA Guideline for The Management of Heart Failure series was developed by the CardioNerds and created in collaboration with the American Heart Association and the Heart Failure Society of America. It was created by 30 trainees spanning college through advanced fellowship under the leadership of CardioNerds Cofounders Dr. Amit Goyal and Dr. Dan Ambinder, with mentorship from Dr. Anu Lala, Dr. Robert Mentz, and Dr. Nancy Sweitzer. We thank Dr. Judy Bezanson and Dr. Elliott Antman for tremendous guidance. Enjoy this Circulation 2022 Paths to Discovery article to learn about the CardioNerds story, mission, and values. Question #20 Ms. Betty Blocker is a 60-year-old woman with a history of alcohol-related dilated cardiomyopathy who presents for follow up. She has been working hard to improve her health and is glad to report that she has just reached her 5-year sobriety milestone. Her current medications include metoprolol succinate 100mg daily, sacubitril-valsartan 97-103mg BID, spironolactone 25mg daily, and empagliflozin 10mg daily. She is asymptomatic at rest and up to moderate exercise, including chasing her grandchildren around the yard. A recent transthoracic echocardiogram shows recovered LVEF from previously 35% now to 60%. Ms. Blocker does not love taking so many medications and asks about discontinuing her metoprolol. Which of the following is the most appropriate response to Ms. Blocker’s request? A Since the patient is asymptomatic, metoprolol can be stopped without risk B Stopping metoprolol increases this patient’s risk of worsening cardiomyopathy regardless of current LVEF or symptoms C Because the LVEF is now >50%, the patient is now classified as having HFpEF and beta-blockade is no longer indicated; metoprolol can be safely discontinued D Metoprolol should be continued, but it is safe to discontinue either ARNi or spironolactone Answer #20 Explanation The correct answer is B – stopping metoprolol would increase her risk of worsening cardiomyopathy. Heart failure tends to be a chronically sympathetic state. The use of beta-blockers (specifically bisoprolol, metoprolol succinate, and carvedilol) targets this excess adrenergic output and has been shown to reduce the risk of death in patients with HFrEF. Beyond their mortality benefit, beta-blockers can improve LVEF, lessen the symptoms of HF, and improve clinical status. Therefore, in patients with HFrEF, with current or previous symptoms, use of 1 of the 3 beta blockers proven to reduce mortality (e.g., bisoprolol, carvedilol, sustained-release metoprolol succinate) is recommended to reduce mortality and hospitalizations (Class 1, LOE A). Beta-blockers in this setting provide a high economic value. Table 14 of the guidelines provides recommendations for target doses for GDMT medications. Specifically for beta blockers, those targets are 25-50mg twice daily for carvedilol (or 80mg once daily for the continuous release formulation), 200mg once daily for metoprolol succinate, and 10mg once daily for bisoprolol. While we should be cognizant of pill-burden and other barriers to our patients’ quality of life, we must counsel them about the risks of discontinuing any element of guideline directed medical therapy (GDMT). The 2022 heart failure guidelines recommend the long-term use of beta blockers for patients diagnosed with HFrEF, even if symptoms improve (Option A). Conversely, long-term treatment should also be maintained even if symptoms do not improve to reduce the risk of major cardiovascular events. Importantly, the abrupt withdrawal of beta blockers can lead to clinical deterioration. Our patient here has heart failure with improved ejection fraction (HFimpEF) defined as having a previous LVEF ≤ 40% and a ≥ 10-point increase from baseline with a follow-up measurement of LVEF > 40%. HFimpEF is distinct from HFpEF and was proposed in the “Universal Definition and Classification of Heart Failure” by Bozkurt et al published in JCF 2021 in order to distinguish those who benefit from continued GDMT. Accordingly, in patients with HFimpEF after treatment, GDMT should be continued to prevent relapse of HF and LV dysfunction, even in patients who may become asymptomatic (Class 1, LOE B-R). While GDMT may improve symptoms, functional capacity, LVEF, and reverse remodeling in patients with HFrEF, these favorable changes do not reflect full and sustained recovery but rather remission with susceptibility to worsening with GDMT withdrawal. Therefore, stopping any element of her GDMT (BB, ARNi, or MRA) would be incorrect (Options A, C, D). Main Takeaway In patients with HFrEF who experience improvement in heart failure symptoms and cardiac function on GDMT (develop HFimpEF), it is important to continue optimizing GDMT to prevent relapse, even if asymptomatic. Guideline Loc. Section 7.3.2 Section 7.3.8, Table 14 Section 7.6.2 Decipher the Guidelines: 2022 Heart Failure Guidelines Page CardioNerds Episode Page CardioNerds Academy Cardionerds Healy Honor Roll CardioNerds Journal Club Subscribe to The Heartbeat Newsletter! Check out CardioNerds SWAG! Become a CardioNerds Patron!

CardioNerds (Drs. Amit Goyal and Dan Ambinder) join Dr. Mina Fares, Dr. Johannes Bergehr, and Dr. Christina Peter from Cambridge University Hospitals in the UK. They discuss a case involving a man man in his 40’s presented with progressive heart failure symptoms. He has extensive background cardiac history including prior episodes of myocarditis and complete heart block status post permanent pacemaker implantation. Ultimately a diagnosis of Danon disease is made. Dr. Sharon Wilson provides the E-CPR for this episode. Audio editing by CardioNerds Academy Intern, Hirsh Elhence. “To study the phenomena of disease without books is to sail an uncharted sea, while to study books without patients is not to go to sea at all.” – Sir William Osler. CardioNerds thank the patients and their loved ones whose stories teach us the Art of Medicine and support our Mission to Democratize Cardiovascular Medicine. CardioNerds is collaborating with Radcliffe Cardiology and US Cardiology Review journal (USC) for a ‘call for cases’, with the intention to co-publish high impact cardiovascular case reports, subject to double-blind peer review. Case Reports that are accepted in USC journal and published as the version of record (VOR), will also be indexed in Scopus and the Directory of Open Access Journals (DOAJ). Enjoy this Circulation 2022 Paths to Discovery article to learn about the CardioNerds story, mission, and values. CardioNerds Case Reports PageCardioNerds Episode PageCardioNerds AcademyCardionerds Healy Honor Roll CardioNerds Journal ClubSubscribe to The Heartbeat Newsletter!Check out CardioNerds SWAG!Become a CardioNerds Patron! Case Summary – A Presentation of Heart Failure and Heart Block with Elusive Genetic Origins – Cambridge University A man in his 40s with a history of cardiac issues, including prior myocarditis and complete heart block, presented with progressive heart failure symptoms. Extensive cardiac investigations were conducted, revealing dilated left ventricle, mild to moderate left ventricular systolic dysfunction, normal coronaries, infero-lateral late gadolinium enhancement on cardiac MRI, and low-level uptake on PET-CT. Differential diagnosis included worsening underlying cardiomyopathy, recurrent myocarditis, tachycardia-related cardiomyopathy, pacemaker-induced LV dysfunction, and sarcoidosis. The patient’s condition improved with heart failure medications, and cardiac MRI showed a mildly dilated left ventricle with moderate systolic dysfunction and active inflammation in the anterior wall. Further evaluation indicated a family history of hereditary cardiomyopathy, and the patient exhibited phenotypic features such as early-onset heart disease, arrhythmias, family history of cardiomyopathy, learning problems, intellectual disability, and mild proximal myopathy. Genetic testing confirmed a LAMP2 mutation, leading to the diagnosis of Danon disease. Case Media – A Presentation of Heart Failure and Heart Block with Elusive Genetic Origins – Cambridge University Show Notes -A Presentation of Heart Failure and Heart Block with Elusive Genetic Origins – Cambridge University References – Danon, M. J., Oh, S. J., DiMauro, S., Miranda, A., De Vivo, D. C., & Rowland, L. P. (1981). Lysosomal glycogen storage disease with normal acid maltase. Neurology, 31(1), 51-7. Nishino, I., Fu, J., Tanji, K., Nonaka, I., & Ozawa, T. (2000). Mutations in the gene encoding LAMP2 cause Danon disease. Nature, 406(6798), 906-10. Tanaka, K., Nishino, I., Nonaka, I., Fu, J., & Ozawa, T. (2000). Danon disease is caused by mutations in the gene encoding LAMP2, a lysosomal membrane protein. Nature, 406(6798), 902-6. Maron, B. J., Haas, T. S., Ackerman, M. J., Ahluwalia, A., Spirito, P., Nishino, I., … & Seidman, C. E. (2009). Hypertrophic cardiomyopathy and sudden death in a family with Danon disease. JAMA, 301(12), 1253-9. Hashem, S., Zhang, J., Zhang, Y., Wang, H., Zhang, H., Liu, L., … & Wang, J. (2015). AAV-mediated gene transfer of LAMP2 improves cardiac function in Danon disease mice. Stem cells, 33(11), 2343-2350. Chi, L., Wang, H., Zhang, J., Zhang, Y., Liu, L., Wang, J., … & Hashem, S. (2019). CRISPR/Cas9-mediated gene editing of LAMP2 in patient-derived iPSCs ameliorates Danon disease phenotypes. Proceedings of the National Academy of Sciences, 116(4), 556-565.

The following question refers to Section 3.2 of the 2021 ESC CV Prevention Guidelines. The question is asked by student Dr. Hirsh Elhence, answered first by Mayo Clinic Fellow Dr. Teodora Donisan, and then by expert faculty Dr. Eugene Yang. Dr. Yang is professor of medicine of the University of Washington where he is medical director of the Eastside Specialty Center and the co-Director of the Cardiovascular Wellness and Prevention Program. Dr. Yang is former Governor of the ACC Washington Chapter and chair of the ACC Prevention of CVD Section. The CardioNerds Decipher The Guidelines Series for the 2021 ESC CV Prevention Guidelines represents a collaboration with the ACC Prevention of CVD Section, the National Lipid Association, and Preventive Cardiovascular Nurses Association. Enjoy this Circulation 2022 Paths to Discovery article to learn about the CardioNerds story, mission, and values. Question #25 Please choose the CORRECT statement from the ones below. A CAC scoring can be considered to improve ASCVD risk classification around treatment decision thresholds. B Patients with type 1 or type 2 diabetes are considered very high CV risk, regardless of comorbidities and other risk factors. C CKD does not increase the cardiovascular risk in the absence of other risk factors. D Men and women older than 65 years old are at high cardiovascular risk. Answer #25 Explanation Option A is correct. Coronary artery calcium (CAC) scoring can reclassify CVD risk upwards and downwards in addition to conventional risk factors and may thus be considered in men and women with calculated risks around decision thresholds (Class IIb, Level B). If CAC is detected, its extent should be compared with what would be expected for a patient of the same sex and age. CAC scoring does not provide direct information on total plaque burden or stenosis severity and can be low or even zero in middle-aged patients with soft non-calcified plaque. Option B is false. Not all patients with diabetes are very high risk by default. ·       Moderate risk: well controlled diabetes, <10 years duration, without evidence of target organ damage and no additional ASCVD risk factors. ·       High risk: patients not fulfilling the criteria above, without ASCVD and/or severe target organ damage. ·       Very high risk: diabetic patients with established ASCVD and/or severe target organ damage. Severe target organ damage is defined by: ·       eGFR <45 mL/min/1.73 m2 ·       eGFR 45-59 mL/min/1.73 m2 and microalbuminuria (albumin-to-creatinine ratio, ACR 30 -300 mg/g) ·       Proteinuria (ACR >300 mg/g) ·       Presence of microvascular disease in at least 3 different sites (e.g., microalbuminuria + retinopathy + neuropathy Option C is false. CKD carries at least a high CVD risk even in the absence of diabetes or ASCVD. ·       Moderate CKD carries a high CVD risk: o   eGFR 30−44 mL/min/1.73 m2 and ACR <30 o   eGFR 45−59 mL/min/1.73 m2 and ACR 30−300 o   eGFR ≥60 mL/min/1.73 m2 and ACR >300 ·       Severe CKD carries a very high CVD risk: o   eGFR<30 mL/min/1.73 m2 o   eGFR 30−44 mL/min/1.73 m2 and ACR >30   Option D is false. There is an age difference between men and women with regards to cardiovascular risk. Age is a major CVD risk driver, but age cutoffs should be used with flexibility. ·       Women < 50 years-old and men < 40 years old are usually at low 10-year CVD risk. It is still important to be aware of unfavorable modifiable risk factors that can sharply increase their lifetime CVD risk. ·       Women > 75 years-old and men > 65 years-old are usually at high 10-year CVD risk. ·       Only between the ages of 55 – 75 years in women and 40 – 65 years in men does the 10-year CVD risk vary around commonly used thresholds for intervention.   Of note: ·       In younger, apparently healthy patients, we also discuss lifetime CVD risk estimates since 10-year risk assessments often underestimate risk. ·       In an aging population, treatment decisions should take competing non-CVD risk into account. ·       In patients with established ASCVD we can discuss about residual CVD – defined as the risk estimated after initial lifestyle changes and risk factor treatment. Main Takeaway Estimating CVD risk is not only important in apparently healthy patients, but also in patients with diabetes, renal disease, established ASCVD, or older patients. This can provide information to allow shared decision making and personalized approach for our patients. Guideline Loc. Table 3, page 3237; Section 3.2.3., page 3243; Table 4, page 3244 CardioNerds Decipher the Guidelines – 2021 ESC Prevention SeriesCardioNerds Episode PageCardioNerds AcademyCardionerds Healy Honor Roll CardioNerds Journal ClubSubscribe to The Heartbeat Newsletter!Check out CardioNerds SWAG!Become a CardioNerds Patron!

The following question refers to Section 7.1 of the 2022 AHA/ACC/HFSA Guideline for the Management of Heart Failure. The question is asked by New York Medical College medical student and CardioNerds Intern Akiva Rosenzveig, answered first by Lahey Hospital and Medical Center internal medicine resident and CardioNerds Academy House Faculty Leader Dr. Ahmed Ghoneem, and then by expert faculty Dr. Clyde Yancy. Dr. Yancy is Professor of Medicine and Medical Social Sciences, Chief of Cardiology, and Vice Dean for Diversity and Inclusion at Northwestern University, and a member of the ACC/AHA Joint Committee on Clinical Practice Guidelines. The Decipher the Guidelines: 2022 AHA / ACC / HFSA Guideline for The Management of Heart Failure series was developed by the CardioNerds and created in collaboration with the American Heart Association and the Heart Failure Society of America. It was created by 30 trainees spanning college through advanced fellowship under the leadership of CardioNerds Cofounders Dr. Amit Goyal and Dr. Dan Ambinder, with mentorship from Dr. Anu Lala, Dr. Robert Mentz, and Dr. Nancy Sweitzer. We thank Dr. Judy Bezanson and Dr. Elliott Antman for tremendous guidance. Enjoy this Circulation 2022 Paths to Discovery article to learn about the CardioNerds story, mission, and values. Question #19 Ms. M is a 36-year-old G1P1 woman 6 months postpartum who was diagnosed with peripartum cardiomyopathy at the end of her pregnancy. She is presenting for a follow up visit today and notes that while her leg edema has resolved, she continues to have dyspnea when carrying her child up the stairs. She also describes significant difficulty sleeping, though denies orthopnea, and notes she is not participating in hobbies she had previously enjoyed. She is currently prescribed a regimen of sacubitril-valsartan, metoprolol succinate, spironolactone, and empagliflozin.  What are the next best steps? A Screen for depression B Counsel her to follow a strict low sodium diet with goal of < 1.5g Na daily C Recommend exercise therapy and refer to cardiac rehabilitation D A & C Answer #19 Explanation The correct answer is D – both A (screening for depression) and C (referring to cardiac rehabilitation) are appropriate at this time. Choice A is correct. Depression is a risk factor for poor self-care, rehospitalization, and all-cause mortality among patients with HF. Interventions that focus on improving HF self-care have been reported to be effective among patients with moderate/severe depression with reductions in hospitalization and mortality risk. Social isolation, frailty, and marginal health literacy have similarly been associated with poor HF self-care and worse outcomes in patients with HF. Therefore, in adults with HF, screening for depression, social isolation, frailty, and low health literacy as risk factors for poor self-care is reasonable to improve management (Class 2a, LOE B-NR).  Choice C is correct. In patients with HF, cardiac rehabilitation has a Class 2a recommendation (LOE B-NR) to improve functional capacity, exercise tolerance, and health-related QOL; exercise training (or regular physical activity) for those able to participate has a Class 1 recommendation (LOE A) to improve functional status, exercise performance, and QOL. Choice B is incorrect. For patients with stage C HF, avoiding excessive sodium intake is reasonable to reduce congestive symptoms (Class 2a, LOE C-LD). However, strict sodium restriction does not have strong supportive data and is not recommended. There are ongoing studies to better understand the impact of sodium restriction on clinical outcomes and quality of life. The AHA currently recommends a reduction of sodium intake to <2300 mg/d for general cardiovascular health promotion; however, there are no trials to support this level of restriction in patients with HF. Main Takeaway Depression is a risk factor for poor HF self-care and worse outcomes in patients with heart failure and so it is reasonable to screen for depression in these patients. Exercise therapy and cardiac rehabilitation have been shown to improve outcomes in HF patients. While avoiding excess sodium intake is reasonable in HF patients to reduce congestive symptoms, there is no specific strict sodium level recommended. Guideline Loc. Section 7.1 Decipher the Guidelines: 2022 Heart Failure Guidelines Page CardioNerds Episode Page CardioNerds Academy Cardionerds Healy Honor Roll CardioNerds Journal Club Subscribe to The Heartbeat Newsletter! Check out CardioNerds SWAG! Become a CardioNerds Patron!

CardioNerds (Daniel Ambinder) join Dr. Tomio Tran, Dr. Vid Yogeswaran, and Dr. Amanda Cai from the University of Washington for a break from the rain at the waterfront near Pike Place Market. They discuss the following case: A 46-year-old woman presents with cardiac arrest and was found to have cor triatriatum sinistrum (CTS). CTS is a rare congenital cardiac malformation in which the left atrium is divided by a fenestrated membrane, which can restrict blood flow and cause symptoms of congestive heart failure. Rarely, the condition can present in adulthood. To date, there have been no cases of sudden cardiac death attributed to CTS. Dr. Jill Steiner provides the E-CPR for this episode. Audio editing by CardioNerds Academy Intern, student doctor Akiva Rosenzveig. “To study the phenomena of disease without books is to sail an uncharted sea, while to study books without patients is not to go to sea at all.” – Sir William Osler. CardioNerds thank the patients and their loved ones whose stories teach us the Art of Medicine and support our Mission to Democratize Cardiovascular Medicine. CardioNerds is collaborating with Radcliffe Cardiology and US Cardiology Review journal (USC) for a ‘call for cases’, with the intention to co-publish high impact cardiovascular case reports, subject to double-blind peer review. Case Reports that are accepted in USC journal and published as the version of record (VOR), will also be indexed in Scopus and the Directory of Open Access Journals (DOAJ). Enjoy this Circulation 2022 Paths to Discovery article to learn about the CardioNerds story, mission, and values. CardioNerds Case Reports PageCardioNerds Episode PageCardioNerds AcademyCardionerds Healy Honor Roll CardioNerds Journal ClubSubscribe to The Heartbeat Newsletter!Check out CardioNerds SWAG!Become a CardioNerds Patron! Case Media – A Sinister Cause of Sudden Cardiac Death – University of Washington A 40-year-old woman with a history of recurrent exertional syncope had sudden loss of consciousness while kissing her partner. The patient received bystander CPR while 911 was called. EMS arrived within 10 minutes of the call and found the patient apneic and unresponsive. Initial rhythm check showed narrow complex tachycardia at a rate of 136 BPM. ROSC was eventually achieved. A 12-lead ECG showed that the patient was in atrial fibrillation with rapid ventricular rate. The patient was intubated and brought to the emergency department. The patient spontaneously converted to sinus rhythm en route to the hospital. In the emergency department, vital signs were remarkable for hypotension (76/64 mmHg) and sinus tachycardia (110 BPM). The physical exam was remarkable for an inability to follow commands. Laboratory data was remarkable for hypokalemia (2.5 mmol/L), transaminitis (AST 138 units/L, ALT 98 units/L), acidemia (pH 7.12), and hyperlactatemia (11.2 mmol/L). CT scan of the chest revealed a thin membrane within the left atrium. Transthoracic echocardiogram showed normal biventricular size and function, severe tricuspid regurgitation, pulmonary artery systolic pressure of 93 mmHg, and the presence of a membrane within the left atrium with a mean gradient of 25 mmHg between the proximal and distal left atrial chambers. Vasopressors and targeted temperature management were initiated. The patient was able to be re-warmed with eventual discontinuation of vasopressors, however she had ongoing encephalopathy and seizures concerning for hypoxic brain injury. There was discussion with the adult congenital heart disease team about next steps in management, however the patient was too sick to undergo any definitive treatment for the intracardiac membrane within the left atrium. The patient developed ventilator associated pneumonia and antibiotics were initiated. The patient ultimately developed  bradycardia and pulseless electrical activity; ROSC was unable to be achieved, resulting in death. Autopsy was remarkable for the presence of a fenestrated intracardiac membrane within the left atrium and lack of other apparent congenital heart defects. There was right ventricular hypertrophy and pulmonary artery intimal thickening with interstitial fibrosis suggestive of pulmonary hypertension. There were bilateral acute subsegmental pulmonary emboli present. The cause of death was declared to be arrhythmia in the setting of pulmonary hypertension and right sided heart failure caused by cor triatriatum sinistrum with a significant contribution from acute subsegmental pulmonary emboli. Case Media – A Sinister Cause of Sudden Cardiac Death – University of Washington Pearls – A Sinister Cause of Sudden Cardiac Death – University of Washington In a patient presenting with syncope, the following feature may indicate an underlying cardiac etiology: exertional syncope, sudden syncope without a prodrome, structural heart disease, advanced age, and family history of sudden cardiac or unexplained death. Cor triatriatum sinistrum is diagnosed by CT, echocardiography, or MRI and is often found incidentally in adults.   Acute management of CTS is similar to mitral stenosis and consists of 1) careful volume management to avoid both hypovolemia and hypervolemia, and 2) avoidance of tachycardia to allow for adequate LV filling during diastole. Surgical resection of the membrane is definitive. A mean gradient of ≥ 8 mmHg across a CTS membrane is hemodynamically significant and should prompt surgical evaluation for membrane resection. Show Notes -A Sinister Cause of Sudden Cardiac Death – University of Washington Syncope Loss of consciousness due to transient decrease in cerebral blood flow Differential Orthostatic Neurally mediated Cardiogenic Mimickers of syncope: seizures, head trauma causing loss of consciousness, hypoglycemia Red flag symptoms of cardiogenic syncope Advanced age Exertional or while lying down Palpitations prior to event Structural heart disease Family history of unexplained or sudden cardiac death Structural heart disease etiologies Generally left sided and causes obstruction to blood flow Valvular stenoses Hypertrophic cardiomyopathy Cardiac tumors Arrhythmia etiologies Can be caused by any brady- or tachyarrhythmia, especially in the setting of structural heart disease Most commonly Sinus node disease High degree heart block Ventricular arrhythmia Pulse pressure Difference between systolic and diastolic pressure Normal ~ 40 mmHg Narrow – <25% of systolic blood pressure Wide – >100 mmHg Wide pulse pressure etiologies Physical conditioning (normal variant) Aortic regurgitation Severe iron deficiency anemia Hyperthyroidism Arteriosclerosis Shunting from arteriovenous fistulas Narrow pulse pressure etiologies – indicates low stroke volume/cardiac output Heart failure Hypovolemia Blood loss Valvular stenosis Cardiac tamponade Pulmonary embolism Cor triatriatum sinister Presence of a membrane within the left atrium that divides the left atrium into 2 chambers Pathophysiology Theorized to be caused by misincorporation of the pulmonary veins within the left atrium causing a membrane within the left atrium If restrictive, sequelae include congestive heart failure, pulmonary hypertension, and right ventricular dysfunction Often associated with other congenital heart disease (ASD, pulmonary venous return, mitral regurgitation) Epidemiology Among the rarest of all congenital heart disease (up to 0.4% of all congenital heart disease, but true incidence is unknown as many can be asymptomatic) Found more often in infancy/childhood; often found incidentally in adults Signs/symptoms Many are asymptomatic Over time, membrane may become fibrotic or calcified and cause significant obstruction Infants/children – pulmonary congestion, respiratory infections, cyanosis, growth restriction Adults – dyspnea, chest pain, palpitations, syncope Thrombotic events are common likely from vascular injury and congestion/stasis Atrial arrhythmia is associated with CTS, possibly from scarring of the membrane vs chronic elevation of left atrial pressure Diagnosis Made by imaging (CT chest/cardiac, echocardiography, MRI) Primary competing differential diagnosis is supravalvular mitral ring If intra-atrial membrane contains the left atrial appendage and pulmonary veins -> supravalvular mitral ring Associated with Schone complex (supravalvular mitral ring, parachute mitral valve, subaortic stenosis, aortic coarctation CTS rarely associated with Shone complex If intra-atrial membrane contains only the pulmonary veins -> CTS Can be found incidentally and cause technical issues in cardiac procedures that require transeptal atrial punctures Quantify degree of restriction with echocardiography; gradients ≥ 8 mmHg are significant per AHA guidelines Treatment Acute: Diuretics to treat congestion Fluids to avoid hypovolemia due to preload dependence for cardiac output Treat underlying causes of tachycardia to optimize diastolic filling Treat tachyarrhythmia with anti-arrhythmis and AV nodal blockers Chronic/definitive: Surgical resection of the membrane offers a good and durable outcome Low recurrence rate, residual gradients likely due to incomplete resection Pulmonary hypertension WHO classification Group 1 – Pulmonary arterial hypertension (idiopathic, toxin induced, HIV, connective tissue disease, congenital heart disease Congenital heart disease comprises a small portion of group 1, typically from shunt lesions Group 2 – Left sided heart disease Group 3 – Pulmonary disease Group 4 – CTEPH Group 5 – Unclear mechanisms (sickle cell, sarcoid, metabolic disease) References – Albassam OT, Redelmeier RJ, Shadowitz S, Husain AM, Simel D, Etchells EE. Did This Patient Have Cardiac Syncope?: The Rational Clinical Examination Systematic Review. JAMA. 2019;321(24):2448-2457. doi:10.1001/jama.2019.8001 Jegier W, Gibbons JE, Wigglesworth FW. Cortriatriatum: clinical, hemodynamic and pathological studies surgical correction in early life. Pediatrics. 1963;31:255-267. Jha AK, Makhija N. Cor Triatriatum: A Review. Semin Cardiothorac Vasc Anesth. 2017;21(2):178-185. doi:10.1177/1089253216680495 Rudienė V, Hjortshøj CMS, Glaveckaitė S, et al. Cor triatriatum sinistrum diagnosed in the adulthood: a systematic review. Heart. 2019;105(15):1197-1202. doi:10.1136/heartjnl-2019-314714 Saxena P, Burkhart HM, Schaff HV, Daly R, Joyce LD, Dearani JA. Surgical repair of cor triatriatum sinister: the Mayo Clinic 50-year experience. Ann Thorac Surg. 2014;97(5):1659-1663. doi:10.1016/j.athoracsur.2013.12.046 Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines [published correction appears in J Am Coll Cardiol. 2019 May 14;73(18):2361]. J Am Coll Cardiol. 2019;73(12):1494-1563. doi:10.1016/j.jacc.2018.08.1028 Yaroglu Kazanci S, Emani S, McElhinney DB. Outcome after repair of cor triatriatum. Am J Cardiol. 2012;109(3):412-416. doi:10.1016/j.amjcard.2011.09.029

The following question refers to Section 6.3 of the 2021 ESC CV Prevention Guidelines. The question is asked by Dr. Christian Faaborg-Andersen, answered first by UCSD cardiology fellow Dr. Harpreet Bhatia, and then by expert faculty Dr. Jaideep Patel. Dr. Patel recently graduated from Virginia Commonwealth University cardiology fellowship and is now a preventive cardiologist at the Johns Hopkins Hospital. The CardioNerds Decipher The Guidelines Series for the 2021 ESC CV Prevention Guidelines represents a collaboration with the ACC Prevention of CVD Section, the National Lipid Association, and Preventive Cardiovascular Nurses Association. Enjoy this Circulation 2022 Paths to Discovery article to learn about the CardioNerds story, mission, and values. Question #24 A 65-year-old man with a history of ischemic stroke 6 months ago presents to cardiology clinic to establish care. An event monitor was negative for atrial fibrillation and TTE with agitated saline study was negative for a patent foramen ovale. Therefore, his ischemic stroke was presumed to be non-cardioembolic in origin. He is currently taking lisinopril 5 mg daily for hypertension (BP in clinic is 115/70) and atorvastatin 40 mg daily. He has no history of significant gastrointestinal or other bleeding. What do you recommend next? A Start apixaban 5 mg BID B Increase lisinopril to 10 mg daily C Start aspirin 81 mg daily D Start aspirin 81 mg daily and clopidogrel 75 mg daily E Start aspirin 81 mg daily and ticagrelor 90 mg BID Answer #24 Explanation The correct answer is C – start aspirin 81mg daily. For the secondary prevention of non-cardioembolic ischemic stroke or TIA, anti-platelet therapy is recommended with aspirin only (75-150 mg/day), dipyridamole + aspirin (slightly superior to aspirin), or clopidogrel alone (slightly superior to aspirin) (Class I, LOE A). DAPT with aspirin and clopidogrel or aspirin and ticagrelor should be considered in the immediate period after a minor ischemic stroke or TIA (3 weeks after event, Class IIa), but not 6 months after an ischemic stroke. Dual antiplatelet therapy with aspirin and clopidogrel increases bleeding risk without a significant benefit over either agent alone. Dual antiplatelet therapy with aspirin and ticagrelor increases bleeding risk, but does not improve disability incidence. Oral anticoagulation would be recommended for a cardioembolic stroke, which does not fit the clinical picture. His BP is well controlled so increasing lisinopril is not necessary. Main Takeaway For the secondary prevention of an ischemic stroke or TIA, anti-platelet therapy with aspirin, aspirin + dipyridamole, or clopidogrel alone is recommended. Guideline Loc. 6.3, page 3296-3297 CardioNerds Decipher the Guidelines – 2021 ESC Prevention SeriesCardioNerds Episode PageCardioNerds AcademyCardionerds Healy Honor Roll CardioNerds Journal ClubSubscribe to The Heartbeat Newsletter!Check out CardioNerds SWAG!Become a CardioNerds Patron!