ISHLT consensus statement: Perioperative management of patients with pulmonary hypertension and right heart failure undergoing surgery.

Pulmonary hypertension (PH) is a risk factor for morbidity and mortality in patients undergoing surgery and anesthesia. This document represents the first international consensus statement for the perioperative management of patients with pulmonary hypertension and right heart failure. It includes recommendations for managing patients with PH being considered for surgery, including preoperative risk assessment, planning, intra- and postoperative monitoring and management strategies that can improve outcomes in this vulnerable population. This is a comprehensive document that includes common perioperative patient populations and surgical procedures with unique considerations.

Long-Term Electrocardiographic and Echocardiographic Progression of Arrhythmogenic Right Ventricular Cardiomyopathy and Their Correlation With Ventricular Tachyarrhythmias.

BACKGROUND: Prior studies of structural and electrocardiographic changes in arrhythmogenic right ventricular (RV) cardiomyopathy and their role in predicting ventricular arrhythmias (ventricular tachycardia) have shown conflicting results. METHODS: We reviewed 405 ECGs, 315 transthoracic echocardiographies, and 441 implantable cardioverter defibrillator interrogations in 64 arrhythmogenic RV cardiomyopathy patients (56% men, mean age [SD], 44.2 [14.6] years) over a mean follow-up of 10 (range, 2.3-19) years. Generalized estimating equations were used to identify the association between ECG abnormalities, clinical variables, and transthoracic echocardiographic measurements (>mild degree of tricuspid regurgitation, RV outflow tract diameter in parasternal long axis and short axis, RV end-diastolic area, fractional area change). RESULTS: There was a 4.65 (95% CI, 0.51%-8.8%) increase in RV end-diastolic area, a 3.75 (95% CI, 1.17%-6.34%) decrease in fractional area change, and 1.9 (95% CI, 1.3-2.8) higher odds (odds ratio) of RV wall motion abnormality with every 5-year increase in age after patients' first transthoracic echocardiography. >Mild tricuspid regurgitation was an independent predictor of RV enlargement and dysfunction (hazard ratio of >10% drop in fractional area change from baseline [95% CI], 3.51 [1.77-6.95] and hazard ratio of >10% increase in RV end-diastolic area from baseline [95% CI], 4.90 [2.52-9.52]). Patients with implantable cardioverter defibrillator were more likely to develop >mild tricuspid regurgitation and larger structural and functional disease progression. More pronounced increase in RV end-diastolic area was translated into higher rates of any ventricular tachycardia. Inferior T-wave inversions and sum of R waves (mm) in V1 to V3 were predictors of RV enlargement and dysfunction with the former also predicting risk of any ventricular tachycardia. CONCLUSIONS: Arrhythmogenic RV cardiomyopathy is a progressive disease. Tricuspid regurgitation is an independent predictor of structural disease progression, which may be exacerbated by use of a transvenous implantable cardioverter defibrillator lead.

Atrial arrhythmias in patients with arrhythmogenic right ventricular cardiomyopathy: Prevalence, echocardiographic predictors, and treatment.

INTRODUCTION: The clinical role of atrial arrhythmias (AA) in arrhythmogenic right ventricular cardiomyopathy (ARVC) and the echocardiographic variables that predict them are not well defined. We describe the prevalence, types, echocardiographic predictors, and management of AA in patients with ARVC. METHODS: We retrospectively evaluated medical records of 117 patients with definite ARVC (2010 Task Force Criteria) from two tertiary care centers. We identified those patients with sustained AA (>30 seconds), including atrial fibrillation (AF), atrial flutter (AFL), and atrial tachycardia (AT). We collected demographic, genetic, and clinical data. The median follow-up was 3.4 years (interquartile range = 2.0-5.7). RESULTS: Total 26 patients (22%) had one or more types of AA: AF (n = 19), AFL (n = 9), and AT (n = 8). We performed genetic testing on 84 patients with ARVC (71.8%). Two patients with AA (8%) had peripheral emboli, and one patient (4%) suffered inappropriate implantable cardioverter-defibrillator shock. We performed catheter ablation of AA in eight patients (31%), with no procedural complications. Right atrial area and left atrial volume index were independently associated with increased odds of AA; odds ratio (OR), 1.1 (95% confidence interval [CI]:1.02-1.16) (P = .01) and OR, 1.1 (95% CI:1.03-1.15) (P = .003), respectively. An increase in tricuspid annular plane peak systolic excursion was independently associated with reduced odds; OR, 0.3 (95% CI: 0.1-0.94) (P = .003). CONCLUSIONS: Atrial arrhythmias (AA) are common in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). Inappropriate shocks and systemic emboli may be associated with AA. Atrial size and right ventricular dysfunction may help identify patients with ARVC at increased odds of AA.

Special Situations in Pulmonary Hypertension: Pregnancy and Right Ventricular Failure.

Despite rapid advances in medical therapy, pregnancy and right ventricular (RV) failure predicts a poor prognosis in patients with pulmonary arterial hypertension. Evidence-based therapy for pulmonary arterial hypertension should be initiated early in the disease course to decrease RV wall stress and prevent RV remodeling and fibrosis. In patients with acutely decompensated RV failure, an aggressive and multifaceted approach must be used; a thorough search for triggering factors for the decompensation is a key part of the successful management strategy. Patients with refractory RV failure who are not candidates for surgical intervention should be referred to palliative care to maximize quality of life and symptom relief.

Neoadjuvant use of sunitinib in locally advanced GIST with intolerance to imatinib.

Gastrointestinal stromal tumors (GIST) arise from precursor cells in the myenteric plexus and comprise the most common mesenchymal tumors of the gastrointestinal tract. Surgical resection is the mainstay of therapy for localized disease. Recurrent, unresectable, and metastatic tumors are associated with a poor prognosis given their resistance to conventional chemotherapy and radiation. Advances in the understanding of molecular pathophysiology of GIST and the use of targeted small-molecule therapies have resulted in dramatic increases in survival. Preliminary data have demonstrated benefits in using imatinib in a neoadjuvant setting; however, there are no studies to date analyzing the use of neoadjuvant sunitinib in primary advanced GIST. Here we present the case of a patient with locally advanced primary GIST who developed severe toxicity on imatinib therapy and was successfully treated with sunitinib in the neoadjuvant setting to achieve complete surgical resection.

Atrial fibrillation in elderly patients with heart failure: convergence of two cardiovascular epidemics in the 21st Century.

Atrial fibrillation and heart failure have been called the twin cardiovascular epidemics of the 21st Century. The prevalence of both conditions is increasing in the elderly and often the two conditions coexist in the same patients, leading to worse outcomes. Current data show that rate control and rhythm control are both reasonable strategies for the treatment of atrial fibrillation in heart failure patients. Emerging data suggest the beneficial effects of novel therapeutic approaches such as cardiac resynchronization therapy, and pulmonary vein isolation on left ventricular remodeling and functional outcomes. Anticoagulation remains a mainstay of therapy for stroke prevention in this high-risk population.