Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare coronary anomaly that can present in childhood or adulthood with a multitude of symptoms depending on the age of presentation. It should be suspected in infants presenting with heart failure in the setting of left ventricular systolic dysfunction and associated mitral regurgitation from papillary muscle ischemia. Adults with ALCAPA may present with cardiac ischemic symptoms. Prompt diagnosis with echocardiography and cross-sectional chest imaging is important to guide surgical intervention and improve the patients’ survival and prognosis. The goal of surgery is to establish a dual-coronary system with mid-term results revealing progressive recovery of left ventricular function and improvement in mitral regurgitation. Patients with ALCAPA should maintain life-long follow-up with a cardiologist with congenital heart disease expertise for surveillance of post-operative complications.
Aims:Increased left ventricular (LV) wall thickness is frequently encountered in transthoracic echocardiography (TTE). While accurate and early diagnosis is clinically important, given the differences in available therapeutic options and prognosis, an extensive workup is often required to establish the diagnosis. We propose the first echo-based, automated deep learning model with a fusion architecture to facilitate the evaluation and diagnosis of increased left ventricular (LV) wall thickness. Methods and Results: Patients with an established diagnosis of increased LV wall thickness (hypertrophic cardiomyopathy (HCM), cardiac amyloidosis (CA), and hypertensive heart disease (HTN)/others) between 1/2015 and 11/2019 at Mayo Clinic Arizona were identified. The cohort was divided into 80%/10%/10% for training, validation, and testing sets, respectively. Six baseline TTE views were used to optimize a pre-trained InceptionResnetV2 model. Each model output was used to train a meta-learner under a fusion architecture. Model performance was assessed by multiclass area under the receiver operating characteristic curve (AUROC). A total of 586 patients were used for the final analysis (194 HCM, 201 CA, and 191 HTN/others). The mean age was 55.0 years, and 57.8% were male. Among the individual view-dependent models, the apical 4-chamber model had the best performance (AUROC: HCM: 0.94, CA: 0.73, and HTN/other: 0.87). The final fusion model outperformed all the view-dependent models (AUROC: HCM: 0.93, CA: 0.90, and HTN/other: 0.92). Conclusion: The echo-based InceptionResnetV2 fusion model can accurately classify the main etiologies of increased LV wall thickness and can facilitate the process of diagnosis and workup.
Background: Prior studies have reported conflicting results of persistent iatrogenic atrial septal defect (iASD) and its impact following a transcatheter edge-to-edge repair (TEER) procedure. This study aims to evaluate the incidence of iASD and its clinical and hemodynamic impact after a TEER. Methods: Consecutive patients who underwent a TEER procedure from June 2014 to September 2020 at the Mayo Clinic were identified. The presence of iASD was retrospectively identified on post-procedure transthoracic echocardiography (TTE) to group patients into an iASD+ group and an iASD− group for comparison of prognosis and cardiac function. Results: A total of 316 patients were included; the mean age was 79.1 ± 9.1 years, and 67.7% were male. Persistent iASD was diagnosed in 108 (34.2%) patients. There was no difference concerning all-cause mortality, heart failure hospitalization, and stroke/TIA between groups at follow-up (median follow-up of 9 months). Post-procedure TTE demonstrated no differences regarding right ventricle (RV) and left ventricle (LV) dimensions and function, including TAPSE (15.2 ± 4.6 vs. 15.4 ± 5.5 mm, p = 0.875), and LV ejection fraction (51.1 ± 14.0% vs. 51.3 ± 13.9%, p = 0.933). However, patients with iASD had higher RV systolic pressure (48.7 ± 14.4 vs. 45.5 ± 14.5 mmHg, p = 0.042) compared with patients without iASD. Conclusion: Notwithstanding higher RV pressures, patients with persistent iASD had no hemodynamic compromise and an equal prognosis compared with those without a residual atrial defect after a TEER procedure. These findings support the mid-term safety of procedures in which an interatrial defect needs to be created and would argue against the need for interventional closure.
To identify prognostically distinct phenotype clusters in transcatheter edge-to-edge repair (TEER) patients based on topological data analysis (TDA), which was never used to assess the heterogeneous TEER population. Patients who underwent TEER (June 2014-September 2020) at Mayo Clinic sites were identified from the institutional database. Thirteen variables were used for TDA. The topological network graph was created using the Python Scikit-TDA Kepler-Mapper package (v. 2.0.1), and clustering was performed at the graph level with Louvain’s modularity method. Kaplan-Meier survival analysis was used to assess the all-cause mortality of each cluster. A total of 389 consecutive patients were included in the final analysis, and 2 major clusters were identified. The mean age was 80.3 ± 8.7 years; 256 (65.8%) were male. Fifty-five (14.5%) patients died during the mean follow-up duration (185 days). Kaplan-Meier analysis showed significant survival differences among the 2 clusters (hazard ratio, 2.70; 95% CI, 1.50-4.87; P=.0005). Cluster 1 (n=227) had moderate or increased residual mitral regurgitation (cluster 0 vs cluster 1: 19.4% vs 41.9%; P<.001) and worse survival performance. Cluster 1 was characterized by worse tricuspid regurgitation severity, higher proportions of patients with atrial fibrillation or flutter, anterior or bileaflet leaflet prolapse, anterior leaflet flail, and mitral annular or leaflet calcification. TDA can identify prognostically distinct clusters in TEER patients based on mitral valve morphology and clinical risk factors. This simple model can facilitate risk stratification for TEER patients regarding procedural success and survival performance.
1 September 2017 in Gastrointestinal Cancer: Targets and Therapy
Cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (CRS-HIPEC) is an effective treatment in a variety of cancers with peritoneal carcinomatosis (PC), especially in pseudomyxoma peritonei for which it is the standard of care. The data for gastric cancer are not as conclusive. This review discusses the effect of HIPEC on gastric cancer survival, the importance of patient selection, the effect of HIPEC on gastric cancer morbidity, translational research on HIPEC, and other recent research on HIPEC in the setting of gastric cancer and/or peritoneal metastases. CRS-HIPEC has been shown to be superior to either CRS alone or systemic chemotherapy alone in terms of improving survival. CRS-HIPEC seems to be the best current treatment for patients with gastric cancer and peritoneal metastases. The international community has recommended it as the treatment for this disease. Prudent patient selection before CRS-HIPEC is recommended as subgroups of patients have been shown to benefit from the treatment, while others have not. Studies on CRS-HIPEC have shown the procedure to have acceptably low rates of morbidity and peri- and postoperative complications as well as significant reductions in the incidence of ascites associated with PC. Translational research on HIPEC supports its use as prophylaxis for prevention of peritoneal metastasis and demonstrates HIPEC to be both effective and safe. Measurement of the tumor marker carcinoembryonic antigen has been shown to be an effective indicator of future outcomes in gastric cancer treated with CRS-HIPEC. Although the treatment outcomes have improved, even current treatment using CRS-HIPEC for gastric cancer with PC the survival rates can be dismal. Thus, the treatment of advanced gastric cancer with PC is an ongoing field of study and future directions of the treatment of gastric cancer with PC may include the use of intraperitoneal immunotherapy.