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Pheochromocytomas are catecholamine-producing tumors and a rare cause of sudden cardiac death. We describe the case of a previously healthy 28-year-old man who presented after a ventricular fibrillation out-of-hospital cardiac arrest (OHCA). His clinical investigation, including a coronary evaluation, was unremarkable. A protocolized head-to-pelvis computed tomography (CT) scan was ordered and revealed a large right adrenal mass with subsequent laboratory studies showing markedly elevated urine and plasma catecholamines. This raised suspicion for a pheochromocytoma as the underlying etiology behind his OHCA. He received appropriate medical management, underwent adrenalectomy with subsequent normalization of his metanephrines, and fortunately did not have recurrent arrythmias. This case highlights the first documented case of a ventricular fibrillation arrest as the initial presentation of pheochromocytoma crisis in a previously healthy individual, and how the use of early protocolized sudden death CT scan allowed for the prompt diagnosis and management of a rare cause of OHCA. LEARNING OBJECTIVE: We review the typical cardiac manifestations of pheochromocytoma and describe the first case of a pheochromocytoma crisis presenting as sudden cardiac death (SCD) in a previously asymptomatic individual. In young patients with unexplained SCD, it is important to consider pheochromocytoma in the differential diagnosis. We also review why an early head-to-pelvis sudden death computed tomography scan protocol may be helpful in the evaluation of patients resuscitated from SCD without an obvious etiology.

We present a case of a woman with past medical history notable for mild COVID-19 infection who presented with dyspnea on exertion, then developed progressively worsening exertional desaturations and was found to have a patent foramen ovale (PFO). Extensive cardiopulmonary testing revealed no clear alternate etiology for her symptoms. After much discussion, she underwent successful closure of the PFO with complete resolution of her symptoms and significantly improved exertional desaturation.

Advances in breast cancer (BC) treatment have contributed to improved survival, but BC survivors experience significant short-term and long-term cardiovascular mortality and morbidity, including an elevated risk of heart failure with preserved ejection fraction (HFpEF). Most research has focused on HF with reduced ejection fraction (HFrEF) after BC; however, recent studies suggest HFpEF is the more prevalent subtype after BC and is associated with substantial health burden. The increased HFpEF risk observed in BC survivors may be explained by treatment-related toxicity and by shared risk factors that heighten risk for both BC and HFpEF. Beyond risk factors with physiological impacts that drive HFpEF risk, such as hypertension and obesity, social determinants of health (SDOH) likely contribute to HFpEF risk after BC, impacting diagnosis, management and prognosis.Increasing clinical awareness of HFpEF after BC and screening for cardiovascular (CV) risk factors, in particular hypertension, may be beneficial in this high-risk population. When BC survivors develop HFpEF, treatment focuses on initiating guideline-directed medical therapy and addressing underlying comorbidities with pharmacotherapy or behavioural intervention. HFpEF in BC survivors is understudied. Future directions should focus on improving HFpEF prevention and treatment by building a deeper understanding of HFpEF aetiology and elucidating contributing risk factors and their pathogenesis in HFpEF in BC survivors, in particular the association with different BC treatment modalities, including radiation therapy, chemotherapy, biological therapy and endocrine therapy, for example, aromatase inhibitors. In addition, characterising how SDOH intersect with these therapies is of paramount importance to develop future prevention and management strategies.

Introduction Spontaneous coronary artery dissection (SCAD) is an important and underreported cause of acute coronary syndrome in women. SCAD has been linked to arteriopathy, pregnancy, as well as hormone replacement therapy in cisgender patients. There is a scarcity of data on the long-term cardiovascular effects of gender affirming hormonal therapy (GAHT) with estrogen. Observational studies have shown increased thromboembolic disease and cardiovascular events in transwomen on estrogen, with oral synthetic or oral combined equine estrogens showing a higher association versus transdermal formulations, however there had been no reports of SCAD in transgender patients on GAHT. Clinical Case A 37-year old transwoman with a history of gender affirming orchiectomy on GAHT with sublingual estradiol 2 mg BID presented with typical symptoms of acute coronary syndrome. No prior history of chest pain. No personal history or family history of cardiovascular disease or risk factors. Initial Troponin I was 9.52 ng/dl ( Discussion This is the first case report showing an association of estrogen GAHT and SCAD in a transwoman. SCAD has been observed to occur more often in cisgender women during the late luteal phase and post-partum period, raising the hypothesis that estrogen level fluctuations could potentially play a role. Sublingual estradiol has been shown to have higher peak and trough serum concentration compared to oral estradiol. Research is needed in this area and on the potential adverse cardiovascular effects of GAHT and whether route of estrogen administration plays a role. We support transdermal estradiol in transwomen with or at risk of cardiovascular disease, and close monitoring of serum levels, as well as aggressive treatment of cardiovascular risk factors. Presentation: No date and time listed

Carcinoid crisis is a life-threatening manifestation of carcinoid syndrome characterized by profound autonomic instability in the setting of catecholamine release from stress, tumor manipulation, or anesthesia. Here, we present an unusual case of carcinoid crisis leading to acute systolic heart failure requiring mechanical circulatory support. (Level of Difficulty: Intermediate.), Graphical abstract

Background Septal myectomy remains the criterion standard for treatment of symptomatic, medically refractory hypertrophic cardiomyopathy (HCM). There is no specific surgical risk calculator for septal myectomy. Methods This study compares the outcomes of septal myectomy at a tertiary referral center with predicted outcomes of mitral valve (MV) repair and aortic valve replacement (AVR) using the Society of Thoracic Surgeons Adult Cardiac Surgery Risk Calculator (STS Calculator). A total of 298 consecutive patients with HCM underwent isolated septal myectomy from 2011 to 2014. Observed outcomes of septal myectomy were compared with the STS Calculator predicted risk of isolated MV repair and AVR predicted within this population using 1-sample tests of proportions. Results Thirty-day mortality for myectomy in this cohort was zero. STS Calculator predicted risk of mortality for MV repair was 0.7% (P = .14) and for AVR = 1.1% (P = .06). Follow-up for vital status was 6.0 ± 0.7 years, at which 294 (98.7%) patients were alive. Hospital stay length was 4.9 ± 1.9 days. One (0.3%) patient experienced a postoperative deep sternal wound infection, and 1 (0.3%) patient experienced a prolonged ventilated state. Postoperative atrial fibrillation occurred in 64 (21.5%) patients. During 30 days of follow-up, no patients experienced stroke, renal failure, or needed dialysis. Conclusions Septal myectomy, performed in a tertiary referral center, had a 30-day mortality rate of 0% and low morbidity rate. There was no difference between observed myectomy mortality and STS Calculator predicted risk for AVR and MV repair. It is possible that a larger sample could reveal lower mortality than STS prediction.

Although Doppler echocardiography is routinely used to assess left ventricle cardiac output, there are limited data about the feasibility of Doppler echocardiography for right ventricular (RV) cardiac output assessment in patients with left-to-right shunt. The purpose of the study was to determine the correlation between Doppler-derived and Fick-derived RV cardiac index (CI), and the interobserver correlation in Doppler-derived RV CI assessment.Retrospective study of patients (age ≥18 years) with unrepaired atrial septal defect who underwent cardiac catheterization and echocardiography (within 3 days), 2004-2017. RV CI was calculated using the hydraulic orifice formula: [.785 × (right ventricle outflow tract diameter)A total of 128 patients (age 52 ± 17 years; female 88 [69%]) met the inclusion criteria. There was a modest correlation between Doppler-derived and Fick-derived RV CI (r = .57, P .001), and the mean difference between Doppler-derived and Fick-derived RV CI was -.3 (95% confidence interval of agreement, -.8 to +.9) L/min/mThe current study demonstrated a modest correlation between Doppler-derived and Fick-derived RV cardiac output, and a modest interobserver correlation in Doppler-derived RV cardiac output assessment. Further studies are required to validate these results and to explore other potential applications such as in patients with chronic pulmonary regurgitation.

Objectives This study hypothesized that atrial fibrillation was associated with heart failure (HF) hospitalization, and that patients who received rhythm control therapy had a lower incidence of HF hospitalization and mortality. Background Atrial fibrillation is a known risk factor for HF hospitalization and mortality in patients with acquired heart disease. Although atrial arrhythmias are common in adults with tetralogy of Fallot (TOF), data about prevalence and outcomes of therapy for atrial fibrillation are very limited. Methods The MACHD (Mayo Adult Congenital Heart Disease) database was queried for adults with repaired TOF and documented atrial fibrillation from 1990 to 2017. Primary endpoint was HF hospitalization defined as admission for volume overload (pulmonary congestion and/or peripheral edema) requiring intravenous diuretics. Secondary endpoint was the effect of rhythm control therapy on HF hospitalization and all-cause mortality. Patients were divided into rhythm control and rate control groups based on the therapy initiated at the time of arrhythmia diagnosis. Results Of 415 patients, 27 (7%) had 42 HF hospitalizations. Of these 415 patients, 88 (21%) had atrial fibrillation at age 49 ± 13 years. Atrial fibrillation was an independent risk factor for HF hospitalization (adjusted hazard ratio: 2.67; 95% confidence interval: 1.04 to 7.34; p = 0.045). The 88 patients were divided into the rhythm control group (n = 61, 69%) and the rate control group (n = 27, 31%). The rate control group had higher unadjusted annual incidence of HF hospitalization (13% vs. 3%; p = 0.001) and all-cause mortality (11% vs. 4%; p = 0.002). Conclusions Atrial fibrillation was a risk factor for HF hospitalization and mortality in TOF patients, and rhythm control therapy was protective against these adverse events.

Aims: Previous data suggest ventricular high rate episodes (VHREs) on pacemakers are frequent and not associated with overall mortality on short term follow up. We sought to determine whether VHREs are associated with mortality, device upgrade, or change in ejection fraction on long term follow up. Methods: A single center, retrospective study was performed on 542 patients with permanent pacemakers followed between 2011 and 2013. Follow-up was extended to 2017 for determination of long term outcomes. “True” VHREs were defined as episodes adjudicated to be due to non-sustained ventricular tachycardia on review of electrograms and “false” VHREs were defined as supraventricular arrhythmias or noise. Results: VHRE occurred in 202(37.2%)/542 included patients. True VHRE was detected in 148(27.3%) while 54(10%) had false VHRE. The mean age of the population was 72 ± 15 years and 46% were women. Mean follow-up was 3.3 ± 1.4 years. The baseline characteristics of the true, false and no VHRE patients were similar. There was no difference in all-cause mortality between groups (27% mortality in true VHRE, 33% in false VHRE and 29% in no VHRE). Furthermore, there was no difference between groups with regards to any device upgrade (5% any upgrades in the VHRE, 9% in false VHRE and 5% in no VHRE.) On follow up, EF declined in all groups: −4% vs −2.4% vs −3.5% for true, false and no VHRE. Conclusion: VHRE are frequently encountered on remote monitoring of pacemakers and not associated with increased risk of mortality or need for downstream device upgrade. Keywords: Non-sustained ventricular tachycardia, Pacemakers, Remote monitoring, Mortality

ObjectiveThis tertiary centre study aims to identify factors associated with adverse outcomes in adult survivors with total cavopulmonary connection (TCPC) Fontan palliation for single ventricle.MethodsThis retrospective review of medical records identified adult (≥18 years) survivors of TCPC Fontan palliation who were followed at a single tertiary centre between 1 January 2000 and 1 July 2019. Adverse outcomes were defined as arrhythmia, pacemaker/implantable cardioverter defibrillator placement, liver cirrhosis, protein losing enteropathy, hospitalisation for heart failure, thromboembolic complication and/or death.Results160 adult TCPC patients met the inclusion criteria: 117 (73.1%) extracardiac and 43 (26.9%) lateral tunnel. The median (IQR) duration of follow-up since TCPC palliation was 17.5 (11.8–21.3) years. An adverse outcome occurred in 87 (54.4%) patients. Adverse outcome-free survival rates at 10, 20 and 25 years post TCPC were 89% (95% CI 82% to 93%), 60% (95% CI 50% to 69%) and 24% (95% CI 15% to 35%), respectively. On multivariate analysis, extracardiac Fontan (HR 2.21, 95% CI 1.20 to 4.08, p=0.011) was observed to be an independent risk factor for adverse outcomes after adjusting for age, race, morphology of the systemic ventricle and history of fenestration.ConclusionsIn this single-centre retrospective study of adult survivors of TCPC palliation, extracardiac Fontan was associated with an increased hazard for adverse outcomes. This finding could guide clinicians in developing risk modification strategies and management decisions to improve long-term outcomes in these patients.

Background There are limited studies of thrombotic and embolic complications (TEC) in the adult Fontan population. The purpose of the study was to determine the prevalence, risk factors, and outcomes of TECs in this population. Methods Retrospective review of adults with a previous Fontan operation, with follow-up at Mayo Clinic, 1994-2014. Systemic TEC was defined as intracardiac thrombus, ischemic stroke, or systemic arterial embolus. Nonsystemic TEC was defined as Fontan conduit/right atrial thrombus or pulmonary embolus. Results We identified 387 patients with a mean (SD) age of 28 (7) years and a mean follow-up of 8 (2) years. An atriopulmonary connection (APC) was done for 286 patients (74%). Atrial arrhythmias were present in 278 (72%). There were 121 TECs (systemic n=36, nonsystemic n=85) in 98 patients (25%). Risk factors for systemic TEC were atrial arrhythmia (hazard ratio 2.28, P =.001) and APC (hazard ratio 1.98, P =.02); nonsystemic TEC also had similar risk factors. All 98 patients received warfarin. Warfarin was discontinued in 10 of 98 because of bleeding, and 8 of these 10 subsequently had a second TEC. Among the 82 patients who had follow-up imaging, 16 (20%) had resolution of thrombus. In total, 24 of 98 patients had a second TEC, most of whom had inadequate anticoagulation. Conclusions Thrombotic and embolic complication was not uncommon; risk factors for TEC were APC and atrial arrhythmias. Most patients were treated successfully with warfarin alone. A second TEC occurred in most patients whose anticoagulation was discontinued because of bleeding events.

Background There are limited data about the risk of thrombotic and embolic complication (TEC) in adults with atrial arrhythmia after Fontan operation. Objectives This study sought to determine the risk of TEC in this population and the role of anticoagulation therapy in TEC prevention. Methods This was a retrospective review of adults with atrial arrhythmia after Fontan operation who were evaluated at the Mayo Clinic between 1994 to 2014. TEC was classified into 2 groups: systemic TEC, defined as intracardiac thrombus, ischemic stroke, or systemic arterial embolus; and nonsystemic TEC, defined as Fontan conduit/right atrial thrombus or pulmonary embolus. Patients were divided into 3 groups: anticoagulation, antiplatelet, and no therapy cohorts. Results We followed 278 patients, mean age 31 ± 9 years, for 88 ± 14 months (1,464 patient-years). Patient groups included antiplatelet (n = 181), anticoagulation (n = 91), and no therapy (n = 6). There were 97 TEC in 81 patients (29%); 32 were systemic, yielding an event rate of 2.1 systemic TEC per 100 patient-years, and 65 were nonsystemic TEC, yielding an event rate of 4.4 nonsystemic TEC per 100 patient-years. Prevalence of TEC was 18% and 55% at 5 and 10 years, respectively. Atriopulmonary connection was a risk factor for TEC (hazard ratio: 2.31; 95% confidence interval: 1.61 to 4.64), and TEC were associated with higher risk of death and hospitalization (p Conclusions Anticoagulation was associated with lower TEC rate and lower risk of death and hospitalization, without a significant increase in bleeding risk. Perhaps anticoagulation should be the preferred preventive strategy.

Known for the pathological connection to atrial fibrillation (AF), the left atrial appendage (LAA) is the most common source of thromboembolism in patients with AF and may be an arrhythmogenic source for the maintenance of AF. Potential interventions of the LAA for stroke prevention have recently been developed through better understanding its anatomy and physiology. Occlusion of the LAA is an alternative to the use of life-long anticoagulation in selected nonvalvular AF cases. The PROTECT-AF (The WATCHMAN LAA Closure Device for Embolic PROTECTion in Patients with Atrial Fibrillation) and PREVAIL (Randomized Trial of LAA Closure vs. Warfarin for Stroke/Thromboembolic Prevention in Patients with Non-valvular Atrial Fibrillation) randomized controlled trials demonstrated that LAA exclusion using the Watchman percutaneous device is not inferior to warfarin. However, the appendage is structurally complex and has considerable morphological variations among individuals, and it can be challenging to generalize the device for all patients. Continued technological developments including occlusion/ligation through epicardial, endocardial, or surgical approaches, as well as operator expertise regarding LAA anatomy, physiology, and pathophysiology, should improve interventional outcomes. Furthermore, the optimal strategy for re-entrant tachyarrhythmias arising from LAA remains unknown. Whereas an observational study suggested that LAA isolation was more effective than focal ablation, LAA isolation may be associated with significant impairments in LAA contractility, predisposing individuals to a risk of thrombosis.

Background We have previously shown that sympathetic ganglia stimulation via the renal vein rapidly increases blood pressure. This study further investigated the optimal target sites and effective energy levels for stimulation of the renal vasculatures and nearby sympathetic ganglia for rapid increase in blood pressure. Methods The pre-study protocol for endovascular stimulations included 2 minutes of stimulation (1-150 V and 10 pulses per second) and at least 2 minutes of rest during poststimulation. If blood pressure and/or heart rate were changed during the stimulation, time to return to baseline was allowed prior to the next stimulation. Results In 11 acute canine studies, we performed 85 renal artery, 30 renal vein, and 8 hepatic vasculature stimulations. The mean arterial pressure (MAP) rapidly increased during stimulation of renal artery (95 ± 18 mmHg vs. 103 ± 15 mmHg; P 10 V focused on the left renal artery, bilateral renal arteries, and bilateral renal veins (especially the mid segment). Overall, heart rate was unchanged, but muscle fasciculation was observed in 22.0% with an output >10 V (range 15-150 V). Analysis after excluding the stimulations that resulted in fasciculation yielded similar results to the main findings. Conclusions Stimulation of intra-abdominal vasculatures promptly increased the MAP and thus may be a potential treatment option for hypotension in autonomic disorders. Predictors of optimal stimulation include energy delivery and the site of stimulation (for the renal vasculatures), which informs the design of subsequent research.

Background: Right ventricular systolic dysfunction (RVD) often coexists with various cardiopulmonary diseases. However, the association between RVD and risk of sudden cardiac death (SCD) has not been well studied. This study examined the risk of SCD associated with RVD in patients with heterogeneous underlying cardiac diseases. Methods: The Mayo Clinic cardiac care unit database included 5463 consecutive patients with complete echocardiographic evaluation to assess right ventricular systolic function and RVD severity. Prospective surveillance follow-up was obtained for all patients. SCD was adjudicated when a malignant ventricular arrhythmia was documented as the primary rhythm leading to death. RESULTS: The prevalence of mild RVD and moderate-severe RVD was 14.9% and 17.1%, respectively. Patients with RVD were more likely to have a history of congestive heart failure, cardiac arrest, pulmonary disease, and lower baseline left ventricular ejection fraction compared with those with normal right ventricular systolic function. During a median follow-up of 14 months, the incidence of SCD was highest in patients with moderate-severe RVD (7.4% versus 4.4% in mild RVD versus 1.6% in normal right ventricular function; P P =0.046) and moderate-severe RVD (adjusted hazard ratio, 1.91; P =0.006) were independently associated with an increased risk of SCD. Moderate-severe RVD remained an independent predictor of SCD for patients with left ventricular ejection fraction >35% without or with preexisting implantable cardioverter-defibrillator (adjusted hazard ratio, 4.12; P =0.003 and adjusted hazard ratio, 5.04; P Conclusions: Presence of RVD in patients with a history of preexisting cardiac disease is an independent predictor of SCD irrespective of left ventricular ejection fraction.

We have previously shown that sympathetic ganglia stimulation via the renal vein rapidly increases blood pressure. This study further investigated the optimal target sites and effective energy levels for stimulation of the renal vasculatures and nearby sympathetic ganglia for rapid increase in blood pressure.The pre-study protocol for endovascular stimulations included 2 minutes of stimulation (1-150 V and 10 pulses per second) and at least 2 minutes of rest during poststimulation. If blood pressure and/or heart rate were changed during the stimulation, time to return to baseline was allowed prior to the next stimulation.In 11 acute canine studies, we performed 85 renal artery, 30 renal vein, and 8 hepatic vasculature stimulations. The mean arterial pressure (MAP) rapidly increased during stimulation of renal artery (95 ± 18 mmHg vs. 103 ± 15 mmHg; P0.0001), renal vein (90 ± 16 mmHg vs. 102 ± 20 mmHg; P = 0.001), and hepatic vasculatures (74 ± 8 mmHg vs. 82 ± 11 mmHg; P = 0.04). Predictors of a significant increase in MAP were energy10 V focused on the left renal artery, bilateral renal arteries, and bilateral renal veins (especially the mid segment). Overall, heart rate was unchanged, but muscle fasciculation was observed in 22.0% with an output10 V (range 15-150 V). Analysis after excluding the stimulations that resulted in fasciculation yielded similar results to the main findings.Stimulation of intra-abdominal vasculatures promptly increased the MAP and thus may be a potential treatment option for hypotension in autonomic disorders. Predictors of optimal stimulation include energy delivery and the site of stimulation (for the renal vasculatures), which informs the design of subsequent research.

Known for the pathological connection to atrial fibrillation (AF), the left atrial appendage (LAA) is the most common source of thromboembolism in patients with AF and may be an arrhythmogenic source for the maintenance of AF. Potential interventions of the LAA for stroke prevention have recently been developed through better understanding its anatomy and physiology. Occlusion of the LAA is an alternative to the use of life-long anticoagulation in selected nonvalvular AF cases. The PROTECT-AF (The WATCHMAN LAA Closure Device for Embolic PROTECTion in Patients with Atrial Fibrillation) and PREVAIL (Randomized Trial of LAA Closure vs. Warfarin for Stroke/Thromboembolic Prevention in Patients with Non-valvular Atrial Fibrillation) randomized controlled trials demonstrated that LAA exclusion using the Watchman percutaneous device is not inferior to warfarin. However, the appendage is structurally complex and has considerable morphological variations among individuals, and it can be challenging to generalize the device for all patients. Continued technological developments including occlusion/ligation through epicardial, endocardial, or surgical approaches, as well as operator expertise regarding LAA anatomy, physiology, and pathophysiology, should improve interventional outcomes. Furthermore, the optimal strategy for re-entrant tachyarrhythmias arising from LAA remains unknown. Whereas an observational study suggested that LAA isolation was more effective than focal ablation, LAA isolation may be associated with significant impairments in LAA contractility, predisposing individuals to a risk of thrombosis.

Determine prevalence of hypertensive response to exercise (HRE) and its association with cardiovascular adverse events (CAE) in patients (pt) with repaired coarctation of aorta (rCOA). Retrospective review of adult pt with rCOA who had cardiopulmonary exercise tests (CPET) at Mayo Clinic, 1994-2014

Neural modulation with pulmonary vein isolation (PVI) may be more successful than PVI alone for atrial fibrillation (AF) ablation and may be signaled by changes in sinus rhythm (SR) heart rate post ablation. We sought to determine if a change in SR heart rate predicted outcomes post PVI. Patients