Transcatheter management of obstructed baffle repairs of partial anomalous pulmonary veins: a case series.

Background: Partial anomalous pulmonary venous return (PAPVR) can be surgically corrected using a pericardial baffle. This baffle can become obstructed post-surgery, leading to pulmonary hypertension and right heart dysfunction if not detected and corrected. Case summary: We describe three patients with occluded PAPVR baffles who underwent drug-coated balloon angioplasty and stenting of the obstructed baffle. In each case, baffle obstruction was detected post-operatively on surveillance cross-sectional imaging, and an invasively measured pulmonary capillary wedge-to-left atrium gradient was noted to be elevated. Post-intervention, each patient had an improvement in baffle flow by angiography as well as lung perfusion as assessed by nuclear medicine scintigraphy. Discussion: Given the subtle symptomatology of obstructed PAPVR pericardial baffle repairs, surveillance imaging is necessary to detect occluded baffles and intervene before downstream right heart disease and pulmonary hypertension develops. Given the high rates of re-stenosis in pulmonary vein stenting, pre-treatment of occluded PAPVR baffles with drug-coated balloons may help reduce re-intervention rates.

A Multicenter Evaluation of the Impact of Therapies on Deep Learning-based Electrocardiographic Hypertrophic Cardiomyopathy Markers.

Background: Artificial intelligence-enhanced electrocardiography (AI-ECG) can identify hypertrophic cardiomyopathy (HCM) on 12-lead ECGs and offers a novel way to monitor treatment response. While the surgical or percutaneous reduction of the interventricular septum (SRT) represented initial HCM therapies, mavacamten offers an oral alternative. Objective: To evaluate biological response to SRT and mavacamten. Methods: We applied an AI-ECG model for HCM detection to ECG images from patients who underwent SRT across three sites: Yale New Haven Health System (YNHHS), Cleveland Clinic Foundation (CCF), and Atlantic Health System (AHS); and to ECG images from patients receiving mavacamten at YNHHS. Results: A total of 70 patients underwent SRT at YNHHS, 100 at CCF, and 145 at AHS. At YNHHS, there was no significant change in the AI-ECG HCM score before versus after SRT (pre-SRT: median 0.55 [IQR 0.24-0.77] vs post-SRT: 0.59 [0.40-0.75]). The AI-ECG HCM scores also did not improve post SRT at CCF (0.61 [0.32-0.79] vs 0.69 [0.52-0.79]) and AHS (0.52 [0.35-0.69] vs 0.61 [0.49-0.70]). Among 36 YNHHS patients on mavacamten therapy, the median AI-ECG score before starting mavacamten was 0.41 (0.22-0.77), which decreased significantly to 0.28 (0.11-0.50, p <0.001 by Wilcoxon signed-rank test) at the end of a median follow-up period of 237 days. Conclusions: The lack of improvement in AI-based HCM score with SRT, in contrast to a significant decrease with mavacamten, suggests the potential role of AI-ECG for serial monitoring of pathophysiological improvement in HCM at the point-of-care using ECG images.

Obstructive hypertrophic cardiomyopathy: a review of new therapies.

Hypertrophic cardiomyopathy (HCM) is a phenotypically heterogeneous disease with a genetic basis and variable penetrance. The hallmarks of HCM include dynamic left ventricular outflow tract obstruction, typically caused by asymmetric septal hypertrophy. However, abnormal papillary muscle placement, abnormal mitral valve and subvalvular apparatus and apical hypertrophic forms have also been described. Typical medical treatment has been stagnant for decades, although there have been significant advances in surgical treatment of patients with obstructive HCM. Herein, we describe a new class of drugs targeting the specific pathophysiology of HCM.

Hypertrophic obstructive cardiomyopathy is a genetic condition that leads to increased heart muscle size. This increase in heart muscle can cause blockage of appropriate blood flow out of the heart. In such cases, current medications like ß-blockers, calcium channel blockers and disopyramide can be used, but do not directly target the problem of increased heart muscle size. A new medication class, cardiac myosin inhibitors, decrease the squeezing power of the increased heart muscle to allow for more appropriate blood flow out of the heart. So far, trials have been conducted with mavacamten, with upcoming trials of aficamten (another novel cardiac myosin inhibitor). Recent trials with mavacamten have shown that this medication class has been beneficial for patients in whom other medications have failed. Some trials have also shown that by taking cardiac myosin inhibitors, patients have been able to avoid or delay surgery to correct this problem. Reassuringly, short-term data for this class of medications are positive. However, given that these medications are new, continued monitoring and research is being done to evaluate their long-term effect.

Hypertrophic cardiomyopathy: investigational drugs inhibiting myosin and upcoming agents.

INTRODUCTION: Hypertrophic cardiomyopathy (HCM), a phenotypically variable disorder with a genetic basis, was first described in the late 1800s. Since the discovery of the disease, various medical and surgical treatments have been proposed with surgical treatments proving to be of more benefit than medical in patients with severe symptoms. Although beta blockers, calcium channel blockers, and disopyramide have been used for their negative inotropic effect, the data behind utilization of these medications is weak at best. AREAS COVERED: Herein, we describe a first-in-man class of medications called cardiac myosin inhibitors (CMI), which have been recently approved by the Food and Drug Administration (FDA) for the treatment of symptomatic patients with obstructive HCM. PubMed was the primary database searched. EXPERT OPINION: Whether these medications will stand the test of time remains to be seen. They do appear to provide significant benefit and disease modification in early randomized trials with the drawback of decreasing contractility and ejection fraction. In our opinion, this new class of medications is an option for patients with NYHA class II-III symptoms from obstructive HCM who have EF ≥ 55%.

Invasive therapies for symptomatic obstructive hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is a genetic condition with multiple different genetic and clinical phenotypes. As awareness for HCM increases, it is important to also be familiar with potential treatment options for the disease. Treatment of HCM can be divided into two different categories, medical and interventional. Typically for obstructive forms of the disease, in which increased septal hypertrophy, abnormally placed papillary muscles, abnormalities in mitral valve or subvalvular apparatus, lead to dynamic left ventricular outflow tract (LVOT) obstruction, treatment is targeted at decreasing obstructive gradients and therefore symptoms. Medications like beta blockers, calcium channel blockers, disopyramide can often accomplish this. However, in patients with severe obstruction or symptoms refractory to medical therapy, either surgical correction of the LVOT obstruction or percutaneous via alcohol septal ablation, are treatment options. In this review, we will focus on the invasive treatment of hypertrophic obstructive cardiomyopathy.

Association Between Coffee Consumption and Incident Atrial Fibrillation (from the Multi-Ethnic Study of Atherosclerosis [MESA]).

This study aimed to elucidate a potential dose-dependent relation between coffee intake and atrial fibrillation (AF) incidence in a multi-ethnic setting. Previous studies were comprised mainly of White populations, and an exploration of dose dependency is limited. To address these gaps, we analyzed the Multi-Ethnic Study of Atherosclerosis data, a prospective cohort study. In the primary analysis, we crudely divided patients into 3 groups: nonconsumers, 1 to 3 cups/month, and ≥1 cup/week. For the secondary analysis, we stratified the cohort into 9 groups of gradual increments for coffee consumption. A multivariable cox proportional hazards regression model was adjusted for 6 potential confounders: age, gender, smoking, hypertension, diabetes mellitus, and alcohol. Subjects who drank ≥1 cup of coffee/week had a higher incidence of AF (adjusted hazard ratio 1.40, p = 0.015) than nonconsumers. Furthermore, in the secondary analysis, there was an overall trend, albeit not consistent, of increasing adjusted hazard ratio with progressively increasing doses of coffee in the following groups: 1 to 3 cups/month, 2 to 4 cups/week, 2 to 3 cups/day and ≥6 cups/day. Notably, AF incidence was highest (9.8%) for the group consuming the most coffee, that is, ≥6 cups/day (p = 0.02). Stratification by race/ethnicity suggested the results may be driven by White and Hispanic rather than Black or Chinese-American subgroups. In conclusion, the findings suggest an association between coffee consumption and incident AF in contrast to most previous studies.

Valve-in-valve transcatheter aortic valve replacement versus redo aortic valve replacement: which procedure for which patient?

INTRODUCTION: Bioprosthetic aortic valves are increasingly being utilized in a younger population due to improved durability and possibility for future valve-in-valve replacement. This has resulted in a larger population of patients with bioprosthetic aortic valve degeneration requiring re-intervention. Despite no head-to-head comparisons between redo surgical aortic valve replacement (SAVR) and valve-in-valve transcatheter aortic valve replacement (ViV TAVR), observational studies suggest a comparable long-term risk between which led to the incorporation of ViV TAVR to current guidelines. AREAS COVERED: This article summarizes the comparative performance of redo SAVR versus ViV TAVR in patients with bioprosthetic valve dysfunction and provides a guide to better understand which procedure is best for which patient. EXPERT OPINION: With the rising use of TAVR, we will be confronted with more bioprosthetic aortic valve degeneration requiring re-intervention. Based on the available evidence and expert consensus, we propose that patients with bioprosthetic aortic valve degeneration be treated with ViV TAVR if they have a history of radiation heart disease, prohibitive surgical risk, and multiple sternotomies; while patients with small prostheses, history of infective endocarditis, those at high risk for coronary obstruction, and those with need for other cardiac surgery will be managed with redo SAVR.

Penile cancer with visceral metastasis and p16/human papillomavirus positivity: An unusual case of long-term survival.

Squamous cell carcinoma (SCC) of the penis is a rare cancer in the industrialized countries, including the United States. Risk factors for these cancers include inflammatory conditions as well as infection with the human papilloma virus (HPV). Treatment modalities are based on TNM staging and may include surgical management or chemoradiation. Patients with local or some regional disease can have a favorable prognosis; however, with extranodal metastasis, survival decreases sharply. Here, we present a case of long-term disease-free survival in a patient with widely metastatic SCC of the penis.

Left sinus of Valsalva-Electroanatomic basis and outcomes with ablation for outflow tract arrhythmias.

INTRODUCTION: The ablation of outflow tract premature ventricular contractions (PVCs) is generally safe and effective. In some patients, successful ablation sites may not correlate with the earliest activation. We sought to evaluate mechanistic and anatomic relevance of the region below the left sinus of Valsalva in variable morphology outflow tract ventricular arrhythmias. METHODS: PVC cases where ablation was in the region inferior to the left sinus of Valsalva were identified. Procedural and demographic information and long-term outcomes were obtained. Cadaver dissections to evaluate regional anatomy were done as well. RESULTS: A total of 51 cases were included (age 53 ± 10; 37 [73%] males). Ablation was done for high PVC burden (>20%; mean 27% ± 8%) and presence of symptoms (73%) or ejection fraction less than 50% (78%). QRS morphology included either R wave (8; 16%), Rs (9; 18%), or rS (67%) in lead I, no precordial transition (40; 78%), V2 transition, (7; 14%), or V3 transition (4; 8%). In 31 (61%), the site just below the left coronary cusp was the earliest site, while the remainder had another site earlier. Ablation was acutely successful in 50 of 51 (98%). After 3 months, success was noted in 48 of 51 (94%). In two patients, repeat ablation in the same region resulted in durable suppression. CONCLUSION: The cases presented emphasize the importance of a region centered below the left sinus of Valsalva, where multivariable morphology QRS may be successfully ablated. Consideration of mapping and ablation even when signals are late in this region may be warranted in previously failed ablation attempts or first-line evaluation.

Anatomic Considerations Relevant to Atrial and Ventricular Arrhythmias.

Knowledge of relevant cardiac anatomy is crucial in understanding the pathophysiology and treatment of arrhythmias, and helps avoid potential complications in mapping and ablation. This article explores the anatomy, relevant to electrophysiologists, relating to atrial flutter and atrial fibrillation, ventricular tachycardia relating to the outflow tracts as well as endocardial structure, and also epicardial considerations for mapping and ablation.

Coronary Artery Disease in Adults With Coarctation of Aorta: Incidence, Risk Factors, and Outcomes.

Background Premature coronary artery disease ( CAD ) is common in patients with coarctation of aorta ( COA ), but there are limited data about any direct relationship (or lack thereof) between COA and CAD . We hypothesized that atherosclerotic cardiovascular disease risk factors, rather than COA diagnosis, was the primary determinant of CAD occurrence in patients with COA . Methods and Results This is a retrospective study of 654 COA patients and a control group of 876 patients with valvular pulmonic stenosis and tetralogy of Fallot to determine prevalence and independent risk factors for CAD . There was no evidence of a difference in the unadjusted CAD prevalence between the COA and control groups (7.8% versus 6.3%, P=0.247), but premature CAD was more common in COA patients (4.4% versus 1.8%, P=0.002). In the analysis of a propensity-matched cohort of 126 COA and 126 control patients, there was no evidence of a difference in overall CAD prevalence (6.3% versus 5.6% versus P=0.742) and premature CAD prevalence (4.8% versus 3.2%, P=0.518). The multivariable risk factors for CAD were hypertension (odds ratio [ OR ] 2.14; 95% CI 1.36-3.38), hyperlipidemia ( OR 3.33; 95% CI 2.02-5.47), diabetes mellitus ( OR 1.98; 95% CI 1.31-3.61), male sex ( OR 2.05; 95% CI 1.33-3.17), and older age per year ( OR 1.06; 95% CI 1.04-1.07). Conclusions After adjusting for atherosclerotic cardiovascular disease risk factors, we did not find evidence of a difference in CAD risk between the patients with COA and other patients with congenital heart disease.

Role of QRS Fragmentation for Risk Stratification in Adults With Tetralogy of Fallot.

Background Patients with tetralogy of Fallot ( TOF ) remain at risk for cardiovascular events despite successful repair. Some of the current risk stratification tools require advanced imaging and invasive studies, and hence are difficult to apply to routine patient care. A recent study showed that QRS fragmentation ( QRS -f) is predictive of mortality in patients with TOF. The current study aims to validate this result by assessing whether severity of QRS -f could predict all-cause mortality in a different TOF population. Methods and Results The authors reviewed the Mayo Adult Congenital Heart Disease database for patients with TOF who had ECG from 1990-2017. QRS -f was defined as notches in QRS complex in ≥2 contiguous leads on ECG , not related to bundle branch block, and classified as none, mild (≤3 leads), moderate (4 leads), or severe (≥5 leads). Of 465 patients (age 37±14 years) in the study, QRS -f was present in 161 (35%): mild (n=43, 9%), moderate (n=77, 17%), and severe (n=41, 9%). There were 55 deaths (12%) during 13.6±8.2 years of follow-up. Severity of QRS -f remained an independent predictor of all-cause mortality after adjustment for other ECG parameters, patient demographics, and atrial and ventricular arrhythmia (hazard ratio, 1.74 per class; 95% confidence interval, 1.08-2.93 [ P=0.041]). Conclusions The presence of severe QRS -f may be used as complementary data to the usual clinical indices to determine whether interventions such as invasive electrophysiology study should be performed in patients with nonsustained ventricular tachycardia or to proceed with pulmonary valve replacement in patients with severe pulmonary regurgitation with ventricular volumes below the guideline-directed threshold for intervention.