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European Journal of Heart Failure Feb 2020Hypertrophic cardiomyopathy (HCM) is a heterogeneous genetic disorder most often caused by sarcomeric mutations resulting in left ventricular hypertrophy, fibrosis,... (Review)
Review
Hypertrophic cardiomyopathy (HCM) is a heterogeneous genetic disorder most often caused by sarcomeric mutations resulting in left ventricular hypertrophy, fibrosis, hypercontractility, and reduced compliance. It is the most common inherited monogenic cardiac condition, affecting 0.2% of the population. Whereas currently available therapies for HCM have been effective in reducing morbidity, there remain important unmet needs in the treatment of both the obstructive and non-obstructive phenotypes. Novel pharmacotherapies directly target the molecular underpinnings of HCM, while innovative procedural techniques may soon offer minimally-invasive alternatives to current septal reduction therapy. With the advent of embryonic gene editing, there now exists the potential to correct underlying genetic mutations that may result in disease. This article details the recent developments in the treatment of HCM including pharmacotherapy, septal reduction procedures, mitral valve manipulation, and gene-based therapies.
Topics: Cardiac Surgical Procedures; Cardiomyopathy, Hypertrophic; Heart Failure; Humans; Mitral Valve; Treatment Outcome
PubMed: 31919938
DOI: 10.1002/ejhf.1715 -
Circulation Jul 2021Over the past few decades, advances in pharmacological, catheter-based, and surgical reperfusion have improved outcomes for patients with acute myocardial infarctions.... (Review)
Review
Over the past few decades, advances in pharmacological, catheter-based, and surgical reperfusion have improved outcomes for patients with acute myocardial infarctions. However, patients with large infarcts or those who do not receive timely revascularization remain at risk for mechanical complications of acute myocardial infarction. The most commonly encountered mechanical complications are acute mitral regurgitation secondary to papillary muscle rupture, ventricular septal defect, pseudoaneurysm, and free wall rupture; each complication is associated with a significant risk of morbidity, mortality, and hospital resource utilization. The care for patients with mechanical complications is complex and requires a multidisciplinary collaboration for prompt recognition, diagnosis, hemodynamic stabilization, and decision support to assist patients and families in the selection of definitive therapies or palliation. However, because of the relatively small number of high-quality studies that exist to guide clinical practice, there is significant variability in care that mainly depends on local expertise and available resources.
Topics: Acute Disease; American Heart Association; Humans; Myocardial Infarction; United States
PubMed: 34126755
DOI: 10.1161/CIR.0000000000000985 -
Cardiology 2021Carcinoid heart disease (CHD) is a rare and potentially lethal manifestation of an advanced carcinoid (neuroendocrine) tumor. The pathophysiology of CHD is related to... (Review)
Review
Carcinoid heart disease (CHD) is a rare and potentially lethal manifestation of an advanced carcinoid (neuroendocrine) tumor. The pathophysiology of CHD is related to vasoactive substances secreted by the tumor, of which serotonin is most prominent in the pathophysiology of CHD. Serotonin stimulates fibroblast growth and fibrogenesis, which can lead to cardiac valvular fibrosis. CHD primarily affects right heart valves, causing tricuspid and pulmonic regurgitation and less frequently stenosis of these valves. Left heart valves are usually spared because vasoactive substances such as serotonin are enzymatically inactivated in the lung vasculature. The pathology of CHD is characterized by plaque-like deposition of fibrous tissue on valvular cusps, leaflets, papillary muscles, chordae, and ventricular walls. Symptomatic CHD usually presents between 50 and 70 years of age, initially as dyspnea and fatigue. Echocardiography is the mainstay of imaging and demonstrates thickened right heart valves with limited mobility and regurgitation. Treatment focuses on control of the underlying carcinoid syndrome, targeting subsequent valvular heart disease and managing consequent heart failure. Surgical valve replacement and catheter-directed valve procedures may be effective for selected patients with CHD.
Topics: Carcinoid Heart Disease; Heart Valve Diseases; Heart Valves; Humans; Pathology, Clinical; Pulmonary Valve Insufficiency
PubMed: 33070143
DOI: 10.1159/000507847 -
JACC. Clinical Electrophysiology Oct 2023Of the monomorphic ventricular tachycardias, there are 4 specific tachycardias related to the Purkinje system: 1) idiopathic verapamil-sensitive fascicular ventricular... (Review)
Review
Of the monomorphic ventricular tachycardias, there are 4 specific tachycardias related to the Purkinje system: 1) idiopathic verapamil-sensitive fascicular ventricular tachycardia (FVT); 2) non-re-entrant FVT; 3) bundle branch re-entry and interfascicular re-entry; and 4) Purkinje-mediated VT in structural heart disease. Verapamil-sensitive FVT is classified into 4 types according to the location of the circuit: 1) left posterior type; 2) left anterior type; 3) left upper septal type;and 4) reverse type. And, in the left anterior and posterior types, there are septal and papillary muscle subtypes. Although macro-re-entry has been reported to be the mechanism underlying verapamil-sensitive FVT, recording the entire circuit is challenging. One possible reason is that the Purkinje-muscle junction may penetrate the myocardial layer as a part of the circuit. The Purkinje network may thus play an important role in the initiation and maintenance of ventricular fibrillation. Further, it has been reported that the development and the abnormalities of the Purkinje system are associated with the arrhythmogenesis of ventricular fibrillation. Furthermore, it has been reported that catheter ablation of trigger ventricular premature complexes, and/or "de-networking" of the Purkinje system, can be used as electrical bailout therapy. There is a hypothesis that the intramural Purkinje system is involved in the generation of J waves. Nevertheless, as there are still unresolved issues that must be debated and accurately analyzed, this review aims to discuss the solved and unsolved questions related to Purkinje-related arrhythmias.
Topics: Humans; Ventricular Fibrillation; Purkinje Fibers; Tachycardia, Ventricular; Verapamil; Ventricular Premature Complexes
PubMed: 37498247
DOI: 10.1016/j.jacep.2023.05.040 -
JACC. Cardiovascular Interventions Sep 2019The aim of this study was to examine the temporal trends and outcomes of mechanical complications after myocardial infarction in the contemporary era. (Observational Study)
Observational Study
OBJECTIVES
The aim of this study was to examine the temporal trends and outcomes of mechanical complications after myocardial infarction in the contemporary era.
BACKGROUND
Data regarding temporal trends and outcomes of mechanical complications after ST-segment elevation myocardial infarction (STEMI) and non-ST-segment elevation myocardial infarction (NSTEMI) are limited in the contemporary era.
METHODS
The National Inpatient Sample database (2003 to September 2015) was queried to identify all STEMI and NSTEMI hospitalizations. Temporal trends and outcomes of mechanical complications after STEMI and NSTEMI, including papillary muscle rupture, ventricular septal defect, and free wall rupture, were described.
RESULTS
The analysis included 3,951,861 STEMI and 5,114,270 NSTEMI hospitalizations. Mechanical complications occurred in 10,726 of STEMI hospitalizations (0.27%) and 3,041 of NSTEMI hospitalizations (0.06%), with no changes in trends (p = 0.13 and p = 0.83, respectively). The rates of in-hospital mortality in patients with mechanical complications were 42.4% after STEMI and 18.0% after NSTEMI, with no significant trend changes (p = 0.62 and p = 0.12, respectively). After multivariate adjustment, patients who had mechanical complications after myocardial infarction had higher in-hospital mortality, cardiogenic shock, acute kidney injury, hemodialysis, and respiratory complications compared with those without mechanical complications. Predictors of lower mortality in patients with mechanical complications who developed cardiogenic shock included surgical repair in the STEMI and NSTEMI cohorts and percutaneous coronary intervention in the STEMI cohort.
CONCLUSIONS
Contemporary data from a large national database show that the rates of mechanical complications are low in patients presenting with STEMI and NSTEMI. Post-myocardial infarction mechanical complications continue to be associated with high mortality rates, which did not improve during the study period.
Topics: Aged; Aged, 80 and over; Databases, Factual; Female; Heart Rupture, Post-Infarction; Hospital Mortality; Humans; Inpatients; Male; Middle Aged; Non-ST Elevated Myocardial Infarction; Prognosis; Risk Assessment; Risk Factors; ST Elevation Myocardial Infarction; Time Factors; United States; Ventricular Septal Rupture
PubMed: 31537282
DOI: 10.1016/j.jcin.2019.04.039 -
Journal of Clinical Medicine Jan 2023Idiopathic ventricular tachycardia (VT) is an important cause of morbidity and less commonly, mortality in patients with structurally normal hearts. Appropriate... (Review)
Review
Idiopathic ventricular tachycardia (VT) is an important cause of morbidity and less commonly, mortality in patients with structurally normal hearts. Appropriate diagnosis and management are predicated on an understanding of the mechanism, relevant cardiac anatomy, and associated ECG signatures. Catheter ablation is a viable strategy to adequately treat and potentially provide a cure in patients that are intolerant to medications or when these are ineffective. In this review, we discuss special approaches and considerations for effective and safe ablation of VT arising from the right ventricular outflow tract, left ventricular outflow tract, left ventricular fascicles, papillary muscles, and moderator band.
PubMed: 36769578
DOI: 10.3390/jcm12030930