Anatomy of the Ventricular Outflow Tracts: An Electrophysiology Perspective.

Outflow tract ventricular arrhythmias are the most common type of idiopathic ventricular arrhythmia. A systematic understanding of the outflow tract anatomy improves procedural efficacy and enables electrophysiologists to anticipate and prevent complications. This review emphasizes the three-dimensional spatial relationships between the ventricular outflow tracts using seven anatomical principles. In turn, each principle is elaborated on from a clinical perspective relevant for the practicing electrophysiologist. The developmental anatomy of the outflow tracts is also discussed and reinforced with a clinical case.

Cardiac resynchronization therapy response in cardiac sarcoidosis.

INTRODUCTION: Cardiac sarcoidosis (CS) is a nonischemic cardiomyopathy (NICM) characterized by infiltration of noncaseating granulomas involving the heart with highly variable clinical manifestations that can include conduction abnormalities and systolic heart failure. Cardiac resynchronization therapy (CRT) has shown significant promise in NICM, though little is known about its efficacy in patients with CS. OBJECTIVE: To determine if CRT improved cardiac remodeling in patients with CS. METHODS: We retrospectively reviewed all patients with a clinical or histological diagnosis of CS who underwent CRT implantation at the Mayo Clinic enterprise from 2000 to 2021. Baseline characteristics, imaging parameters, heart failure hospitalizations and need for advanced therapies, and major adverse cardiac events (MACE) were assessed. RESULTS: Our cohort was comprised of 55 patients with 61.8% male and a mean age of 58.7 ± 10.9 years. Eighteen (32.7%) patients had definite CS, 21 (38.2%) had probable CS, while 16 (29.1%) had presumed CS, and 26 (47.3%) with extracardiac sarcoidosis. The majority underwent CRT-D implantation (n = 52, 94.5%) and 3 (5.5%) underwent CRT-P implantation with 67.3% of implanted devices being upgrades from prior pacemakers or implantable cardioverter defibrillators. At 6 months postimplantation there was no significant improvement in ejection fraction (34.8 ± 10.9% vs. 37.7 ± 14.2%, p = .331) or left ventricular end-diastolic diameter (58.5 ± 10.2 vs. 57.5 ± 8.1 mm, p = .236), though mild improvement in left ventricular end systolic diameter (49.1 ± 9.9 vs. 45.7± 9.9 mm, p < .0001). Within the first 6 months postimplantation, 5 (9.1%) patients sustained a heart failure hospitalization. At a mean follow-up of 4.1± 3.7 years, 14 (25.5%) patients experienced a heart failure hospitalization, 11 (20.0%) underwent cardiac transplantation, 1 (1.8%) underwent left ventricular assist device implantation and 7 (12.7%) patients died. CONCLUSIONS: Our findings suggest variable response to CRT in patients with CS with no overall improvement in ventricular function within 6 months and a substantial proportion of patients progressing to advanced heart failure therapies.

Efficacy of medical and ablation therapy for inappropriate sinus tachycardia: A single-center experience.

BACKGROUND: Effective therapy for inappropriate sinus tachycardia (IST) remains challenging with high rates of treatment failure and symptom recurrence. It is uncertain how effective pharmacotherapy and procedural therapy are long-term, with poor response to medical therapy in general. METHODS: We retrospectively reviewed all patients with the diagnosis of IST at a tertiary academic medical center from 1998 to 2018. We extracted data related to prescribing patterns and symptom response to medical therapy and sinus node modification (SNM), assessing efficacy and periprocedural complication rates. RESULTS: A total of 305 patients with a formal diagnosis of IST were identified, with 259 (84.9%) receiving at least one prescription medication related to the condition. Beta-blockers were the most commonly used medication (n = 245), with a majority of patients reporting no change or worsening of symptoms, and poor response was seen to other medication classes. Improvement was seen significantly more often with ivabradine than beta blockers, though the sample size was limited (p = .003). Fifty-five patients (18.0% of all IST patients), mean age 32.0 ± 9.1 years, underwent a SNM procedure, with an average of 1.8 ± 0.9 procedures per patient. Acute symptomatic improvement (<6 months) was seen in 58.2% of patients. Long-term complete resolution of symptoms was seen in 5.5% of patients, modest improvement in 29.1%, and no long-term benefit was seen in 65.5% of patients. CONCLUSIONS: Among all medical therapies, there were high rates of treatment failure or symptom worsening in over three-quarters of patients in our study. Ivabradine was most beneficial, though the sample size was small. While most patients receiving SNM ablation for IST perceive an acute symptomatic improvement, almost two-thirds of patients have no long-term improvement, and resolution of symptoms is quite rare. AV node ablation with pacemaker implantation following lack of response to SNM offered increased success, though the sample size was limited.

Natural history and clinical outcomes of inappropriate sinus tachycardia.

BACKGROUND: Limited data are available regarding the demographics, disease associations, and long-term prognosis of patients with inappropriate sinus tachycardia (IST). OBJECTIVE: To establish epidemiologic data for patients with IST, including symptom onset, comorbid disease, and long-term outcomes. METHODS: We retrospectively reviewed all patients with an IST diagnosis at the Mayo Clinic (Rochester, MN) during a 20-year period (1998-2018). We extracted demographic data and clinical outcomes compared to an age and gender-matched control group with atrioventricular nodal reentry tachycardia (AVNRT). RESULTS: Within the study period, a total of 305 patients with IST were identified (mean follow-up 3.5 years) with 92.1% female and mean age 33.2 ± 11.2 years. The most frequently identified circumstances triggering the condition included pregnancy (7.9%) and infectious illness (5.9%) while the most common comorbid conditions were depression (25.6%) and anxiety (24.6%). At diagnosis, the mean left ventricular ejection fraction (LVEF) was 62.3 ± 6.2%, with 77 patients having follow-up echocardiographic data. No significant difference in LVEF was seen after a mean 4.9 ± 4.3-year follow-up (baseline LVEF 59.8 ± 10.7% vs subsequent 61.4 ± 8.1%; P = .2971). Two deaths occurred within the study period, with one related to myocardial infarction and the other noncardiac; compared to an age and gender-matched AVNRT control group there was no excess mortality during the follow-up period. CONCLUSIONS: In our study cohort, IST predominately affects young females with structurally normal hearts and modest coexistent psychiatric disease. In most cases of IST, a major event occurring just before or at the time of diagnosis could not be identified, although nearly 8% of patients first noted symptoms during or shortly after pregnancy. In our cohort, there was no evidence of cardiomyopathy or mortality related to IST.

Role of Abnormal Sperm Morphology in Predicting Pregnancy Outcomes.

The evaluation of strict morphology for predicting successful pregnancy has been controversial, nevertheless remains an essential component of semen analysis. Patients with teratozoospermia (abnormal strict morphology) have traditionally been counseled to undergo assisted reproduction. However, recent studies suggest that patients with abnormal sperm morphology alone should not be precluded from attempting natural conception before undergoing assisted reproduction. The goal of this review is to provide an update on the evaluation of sperm morphology for prognosis in assisted reproductive techniques such as intrauterine insemination and in vitro fertilization with or without intracytoplasmic sperm injection. Additionally, we propose a logical approach to the evaluation of a patient with teratozoospermia seeking fertility treatment.

Lead Management in Special Scenarios.

In this article, the authors review the approach to infections associated with surgically placed leads, leads placed in unusual locations such as azygous veins, extraction of active fixation coronary sinus leads, and the role of hybrid extractions in difficult cases. The authors also review strategies to mitigate the risk of paradoxic embolism among patients undergoing transvenous lead extraction.

Mantle Cell Lymphoma With Cardiac Involvement Presenting as Complete Heart Block.

A 50-year-old woman presented with complete heart block on electrocardiogram. Echocardiogram revealed an intracardiac mass with extensive cardiac involvement. The patient was diagnosed with mantle cell lymphoma, confirmed via lymph node biopsy. Pacemaker implantation and chemotherapy were initiated, with subsequent improvement noted. This showcases an unusual manifestation of intracardiac metastasis with conduction system infiltration.

Case Report: Electroanatomic mapping as an early diagnostic tool in arrhythmogenic cardiomyopathy.

Background: Abnormal substrate on invasive electroanatomic mapping (EAM) correlates with areas of myocardial thinning and fibrofatty replacement in Arrhythmogenic Cardiomyopathy (ACM). However, EAM parameters are absent from all sets of diagnostic criteria for ACM. Case summary: A 41-year-old female with no significant family history was referred for evaluation of frequent premature ventricular complexes (PVCs). Twelve-lead ECG showed diffuse low-voltage QRS complexes. Holter monitor showed 28% burden of PVCs with various morphologies consistent with right ventricular (RV) inflow and outflow tract exits. Transthoracic echocardiogram revealed normal biventricular function and dimension. Cardiac magnetic resonance revealed a mildly increased indexed RV end-diastolic volume with normal RV systolic function and no dyssynchrony, akinesia, dyskinesia, or late gadolinium enhancement. Electrophysiologic study demonstrated 2 predominant PVC morphologies that were targeted with ablation, in addition to extensive abnormality with low-voltage and fractionated electrograms in the peri-tricuspid and right ventricular outflow tract free wall regions with septal sparing, suggestive of RV cardiomyopathy. Subsequent genetic testing revealed two pathogenic variants in the desmoplakin and plakophilin-2 genes, confirming the diagnosis of ACM. Conclusion: Advanced RV electropathy can precede RV structural changes in ACM. Invasive evaluation of the electroanatomic substrate should be considered in select cases even when imaging findings are not diagnostic. Future iterations of ACM guidelines may need to consider EAM substrate as one of the diagnostic criteria. A high index of diagnostic suspicion for ACM should be maintained in patients with multifocal RV ectopy.

Left Atrial Appendage Occlusion in Patients With Atrial Fibrillation and Cancer.

Background: The prevention of stroke in patients with atrial fibrillation (AF) and cancer is challenging because patients are at increased bleeding and thrombotic risk. Objectives: The authors sought to assess left atrial appendage occlusion (LAAO) as a safe and effective strategy for reducing stroke at no increased bleeding risk in cancer patients with AF. Methods: We reviewed patients with nonvalvular AF who underwent LAAO at Mayo Clinic sites from 2017 to 2020 and identified those who had undergone prior or current treatment for cancer. We compared the incidence of stroke, bleeding, device complications, and death with a control group who underwent LAAO without malignancy. Results: Fifty-five patients were included; 44 (80.0%) were male, and the mean age was 79.0 ± 6.1 years. The median CHA2Ds2-VASc score was 5 (Q1-Q3: 4-6), with 47 (85.5%) having a prior bleeding event. Over the first year, ischemic stroke occurred in 1 (1.4%) patient, bleeding complications in 5 (10.7%) patients, and death in 3 (6.5%) patients. Compared with controls who underwent LAAO without cancer, there was no significant difference in ischemic stroke (HR: 0.44; 95% CI: 0.10-1.97; P = 0.28), bleeding complication (HR: 0.71; 95% CI: 0.28-1.86; P = 0.19), or death (HR: 1.39; 95% CI: 0.73-2.64; P = 0.32). Conclusions: Within our cohort, LAAO in cancer patients was achieved with good procedural success and offered a reduction in stroke at no increased bleeding risk similar to noncancer patients.

Catheter Ablation in Patients With Neuroendocrine (Carcinoid) Tumors and Carcinoid Heart Disease: Outcomes, Peri-Procedural Complications, and Management Strategies.

OBJECTIVES: This report describes a series of patients with neuroendocrine tumors with or without carcinoid heart disease undergoing catheter ablation at the authors' institution. BACKGROUND: Neuroendocrine (carcinoid) tumors are a rare form of neoplasm with the potential for systemic vasoactive effects and cardiac valvular involvement. These tumors can create peri-operative management challenges for the electrophysiologist. However, there are few data regarding ablation outcomes, periprocedural complications, and management of these patients. METHODS: All patients with neuroendocrine tumors undergoing catheter ablation at the Mayo Clinic, Rochester, Minnesota over a 25-year period were retrospectively reviewed. From this cohort, the type of arrhythmias ablated, the recurrence of arrhythmia, perioperative complications, and mortality were reviewed and analyzed. RESULTS: A total of 17 patients (52.9% male; mean age 62.4 ± 9.3 years) with neuroendocrine tumors underwent catheter ablation during the study period. Primary tumor sites included the gastrointestinal tract (n = 11), lung (n = 4), ovary (n = 1), and lymph node (n = 1). Nine patients had metastatic disease, 5 of whom were on somatostatin analog therapy at the time of ablation. Three patients had active symptoms of carcinoid syndrome at the time of ablation, and 2 of those patients had carcinoid heart disease. Ablations were performed mainly for atrial arrhythmias (76.5%): atrioventricular nodal re-entry tachycardia (n = 7), atrial fibrillation (n = 4), and atrial flutter (n = 2). Four patients underwent ablation of ventricular arrhythmias. During a mean follow-up of 19.2 ± 26.2 months, arrhythmia recurred in 35.3% of patients. Three patients (17.6%) had periprocedural complications: pericardial effusion (n = 1), groin site hematoma (n = 1), and carcinoid crisis (n = 1). No deaths were noted in the peri-operative period. CONCLUSIONS: In a unique cohort of patients with neuroendocrine tumors, catheter ablation was feasible in patients with or without carcinoid syndrome. Carcinoid crisis may occur during the periprocedural period, which can be life-threatening, and a specified protocol for management is important to mitigate this risk.

Outcomes and periprocedural management of cardiac implantable electronic devices in patients with carcinoid heart disease.

BACKGROUND: Carcinoid heart disease (CHD) is a rare complication of hormonally active neuroendocrine tumors that often requires surgical intervention. Data on cardiac implantable electronic device (CIED) implantation in patients with CHD are limited. OBJECTIVE: The purpose of this study was to evaluate the experience of CIED implantation in patients with CHD. METHODS: Patients with a diagnosis of CHD and a CIED procedure from January 1, 1995, through June 1, 2020, were identified using a Mayo Clinic proprietary data retrieval tool. Retrospective review was performed to extract relevant data, which included indications for implant, procedural details, complications, and mortality. RESULTS: A total of 27 patients (55.6% male; mean age at device implant 65.6 ± 8.8 years) with cumulative follow-up of 75 patient-years (median 1.1 years; interquartile range 0.4-4.6 years) were included for analysis. The majority of implanted devices were dual-chamber permanent pacemakers (63%). Among all CHD patients who underwent any cardiac surgery, the incidence of CIED implantation was 12%. The most common indication for implantation was high-grade heart block (66.7%). Device implant complication rates were modest (14.8%). No patient suffered carcinoid crisis during implantation, and there was no periimplant mortality. Median time from implant to death was 2.5 years, with 1-year mortality of 15%. CONCLUSION: CHD is a morbid condition, and surgical valve intervention carries associated risks, particularly a high requirement for postoperative pacing needs. Our data suggest that CIED implantation can be performed relatively safely. Clinicians must be aware of the relevant carcinoid physiology and take appropriate precautions to mitigate risks.

Putting down the phone: the obsolescence of transtelephonic monitoring for pacemaker follow-up.

PURPOSE: The evolution of heart rhythm monitoring technology over the past few decades has seen a decline in the use and need of transtelephonic monitoring (TTM). We sought to establish a predicted date for the sun setting of TTM at our institution, as well as establish the current demographics of the patients still using this technology. METHODS: We retrospectively reviewed all patients with permanent pacemakers receiving routine device follow-up at our institution (Mayo Clinic-Rochester) between 2015 and 2018. From this cohort, we reviewed and analyzed patients using TTM for device follow-up and utilized projected battery longevity to determine cessation date. Pacemaker implantation date, underlying arrhythmia, and most recent device interrogation reports were also collected. RESULTS: As of March 2018, a total of 3543 patients with permanent pacemakers were being followed at our institution and 289 (8.2%) are using TTM for monitoring device function (147 male, mean age 79.9 ± 12.0 years). Of those currently using TTM, by January of 2020, only 122 (42.2%) are predicted to be using this technology for device follow-up, 40 (13.8%) by January 2022, with zero patients by November of 2024. CONCLUSIONS: The use of TTM will continue to significantly diminish over the next few years. Based on battery longevity estimates, we predict that by the end of 2024 TTM will no longer be used for device follow-up at our institution.

Concurrent Sweet's syndrome and myopericarditis following mesalamine therapy.

Mesalamine, or 5-aminosalicylic acid, is a frequently used medication for the treatment of inflammatory bowel disease (IBD). We report the case of a 40-year-old woman recently diagnosed with IBD and started on mesalamine, who presented with new onset tender skin lesions 3 days following medication administration. One day following the onset of skin lesions, the patient developed acute chest pain, shortness of breath, ECG changes, troponemia, C-reactive protein elevation and pericardial enhancement on cardiac MRI consistent with myopericarditis. Subsequent skin biopsy confirmed the diagnosis of Sweet's syndrome. On cessation of the drug, both the skin lesions and the cardiac symptoms resolved in combination with anti-inflammatory therapy. While mesalamine has been previously associated with myocarditis and pericarditis, to our knowledge this is the first case of coexisting Sweet's syndrome with myopericarditis in the context of mesalamine therapy.

Accessory pancreatic lobe in association with a gastric duplication cyst.

Gastric duplication cysts are an extremely rare anomaly with few reported cases in association with accessory pancreatic tissue. Diagnosis can be challenging given a presentation of recurrent pancreatitis and resemblance to pancreatic pseudocysts. We report the case of a 6-year old boy with multiple episodes of pancreatitis who was discovered to have an accessory pancreatic lobe connected to a gastric duplication cyst, successfully treated with surgical excision.

Splenic Cysts.

Splenic cysts are rare in the United States but more common in regions of the world where Echinococcus is endemic. Cysts are typically classified as true cysts or pseudocysts. True cysts can be parasitic or nonparasitic in origin, whereas most pseudocysts are a result of previous trauma. Recent recognition of features shared by true cysts and pseudocysts suggests the classification system may need to be revised. The prevalence of splenic cysts has increased secondary to the widespread use of abdominal imaging and successful nonoperative management of traumatic splenic injuries. Treatment previously consisted primarily of total splenectomy. However, recognition of the importance of the spleen throughout a patient's life has led to changes in the management of splenic disease. Advances in the testing and preoperative localization of splenic lesions have also led to increased efforts in splenic conservation. [Pediatr Ann. 2016;45(7):e251-e256.].

Neonatal kaposiform hemangioendothelioma of the spleen associated with Kasabach-Merritt phenomenon.

Kaposiform hemangioendothelioma is a rare locally aggressive vascular tumor that usually manifests during early childhood. Typically the lesion presents with skin, soft tissue and bone involvement and is characterized histologically by ill-defined nodularity and the presence of spindle cells with resemblance to Kaposi's sarcoma. We report a rare neonatal case of a splenic kaposiform hemangioendothelioma associated with Kasabach-Merritt phenomenon that was diagnosed with radiographic imaging. Because of the rapid onset of thrombocytopenia and anemia, the patient required urgent splenectomy with subsequent resolution of the blood dyscrasias.