Inoperable chronic thromboembolic pulmonary hypertension: Evolution of prognosis over 10 years of new emerging therapies.

Therapies for inoperable chronic thromboembolic pulmonary hypertension (CTEPH) include balloon pulmonary angioplasty (BPA) and PH-specific medical therapy. This study compares survival and its predictors before and after the introduction of BPA. BPA was independently associated with survival; however, there was no difference in overall survival between the two cohorts.

Peripheral microvascular function is linked to cardiac involvement on cardiovascular magnetic resonance in systemic sclerosis-related pulmonary arterial hypertension.

Aims: Systemic sclerosis (SSc) is characterized by vasculopathy, inflammation, and fibrosis, and carries one of the worst prognoses if patients also develop pulmonary arterial hypertension (PAH). Although PAH is a known prognosticator, patients with SSc-PAH demonstrate disproportionately high mortality, presumably due to cardiac involvement. In this cross-sectional study, the relationship between cardiac involvement revealed by cardiovascular magnetic resonance (CMR) and systemic microvascular disease severity measured with nailfold capillaromicroscopy (NCM) in patients with SSc-PAH is evaluated and compared with patients with idiopathic PAH (IPAH).

Echocardiographic right ventricular remodeling after percutaneous atrial septal defect closure.

Background: In order to prevent right ventricular (RV) dysfunction, closure of secundum type atrial septal defects (ASD) is often indicated and percutaneous closure is the preferred treatment modality to do so. The magnitude and time course of RV remodeling is still incompletely understood.

Methods: This retrospective cohort study included consecutive patients who underwent percutaneous secundum ASD closure in two tertiary referral centers in The Netherlands.

Supraventricular arrhythmia in pregnancy.

The physiological changes during pregnancy predispose a woman for the development of new-onset or recurrent arrhythmia. Supraventricular arrhythmia is the most common form of arrhythmia during pregnancy and, although often benign in nature, can be concerning. We describe three complex cases of supraventricular arrhythmia during pregnancy and review the currently available literature on the subject.

Long-term monitoring of arrhythmias with cardiovascular implantable electronic devices in patients with cardiac sarcoidosis.

Background: Risk stratification for sudden cardiac death (SCD) in cardiac sarcoidosis (CS) is challenging in patients without overt cardiac symptoms.

Objective: The purpose of this study was to determine the incidence of ventricular arrhythmias (VAs) and mortality after long-term monitoring with a cardiovascular implantable electronic device (CIED) in CS patients identified after systematic screening of patients with extracardiac sarcoidosis (ECS).

Methods: A retrospective study was performed in 547 predominantly Caucasian patients with ECS screened for cardiac involvement.

An Elderly Woman With Recurrent Transient Loss of Consciousness Preceded by Hallucinatory Attacks.

We report a patient with transient loss of consciousness preceded by hallucinations caused by cardiac syncope. The diagnosis was confirmed by simultaneous video-electrocardiogram-electroencephalogram recording of an event. The attacks no longer occurred after pacemaker implantation.

Hereditary Hemorrhagic Telangiectasia (HHT) and Survival: The Importance of Systematic Screening and Treatment in HHT Centers of Excellence.

Hereditary hemorrhagic telangiectasia (HHT), an autosomal dominant disease, is characterized by telangiectases and arteriovenous malformations (AVMs). Untreated AVMs, especially in the lungs-pulmonary AVMs (PAVMs)-can result in morbidity with a decreased life expectancy. We have investigated whether HHT patients, systematically screened for HHT-related organ involvement and treated if needed, have a similar survival as persons without HHT.

Microtransesophageal Echocardiographic Guidance during Percutaneous Interatrial Septal Closure without General Anaesthesia.

Objective: To study the safety and efficacy of microtransesophageal echocardiography (micro-TEE) and TEE during percutaneous atrial septal defect (ASD) and patent foramen ovale (PFO) closure.

Background: TEE has proven to be safe during ASD and PFO closure under general anaesthesia. Micro-TEE makes it possible to perform these procedures under local anaesthesia.

Prevalence and diagnostic value of nail fold capillary microscopy in hereditary hemorrhagic telangiectasia: A retrospective study.

Abnormal vasculature is a key feature of hereditary hemorrhagic telangiectasia (HHT) and can also present in the nail fold capillary beds. However, the exact prevalence and the clinical diagnostic value in HHT are still largely unknown. The nail fold can be easily and noninvasively inspected with a capillary microscope.

Assessment of Predictors of Left Atrial Volume Response to a Transcatheter InterAtrial Shunt Device (from the REDUCE LAP-HF Trial).

In patients with heart failure and preserved or mildly reduced ejection fractions (EF ≥40%), implantation of an interatrial shunt device (IASD) resulted in heterogenous changes of the left atrial (LA) volume. Baseline characteristics that correlate with a favorable decrease in LA volume are unknown. We hypothesized that a larger ratio of left to right atrial volume at baseline would correlate strongly with LA volume decongestion following IASD implantation.

Non-vitamin K antagonist oral anticoagulants in adults with a Fontan circulation: are they safe.

Background: In Fontan patients with atrial arrhythmias (AA), non-vitamin K antagonist oral anticoagulants(NOACs) have a class III recommendation according to the Pediatric & Congenital Electrophysiology Society (PACES)/Heart Rhythm Society (HRS) guideline in 2014, due to lack of data on outcomes as opposed to evidence of harm. To address this gap in data, we investigated the safety and efficacy of NOACs in adults with a Fontan circulation in a worldwide study.

Methods: This is an international multicentre prospective cohort study, using data from the NOTE (on-vitamin K antagonist ral anticoagulants for hrombombolic prevention in patients with congenital heart disease) registry.

Transcatheter Tricuspid Valve Interventions: An Emerging Field.

Purpose Of Review: This review aims to provide an updated overview and a clinical perspective on novel transcatheter tricuspid valve interventions (TTVI), highlighting potential challenges and future directions.

Recent Findings: Severe tricuspid regurgitation (TR) is a predictor of mortality. However, a sizeable number of patients remain untreated until the end-stage when cardiac surgery presents a prohibitive risk.

Percutaneous patent foramen ovale closure using the Occlutech Figulla device: More than 1,300 patient-years of follow up.

Objective: To evaluate the safety and efficacy of the Occlutech patent foramen ovale (PFO) device at long-term follow-up (FU).

Background: The Occlutech device has been proven safe and effective six-months after percutaneous PFO closure. We describe the safety and efficacy after more than 1,300 patient-years of FU.

Effect of Losartan on Right Ventricular Dysfunction: Results From the Double-Blind, Randomized REDEFINE Trial (Right Ventricular Dysfunction in Tetralogy of Fallot: Inhibition of the Renin-Angiotensin-Aldosterone System) in Adults With Repaired Tetralogy of Fallot.

Background: The effect of angiotensin II receptor blockers on right ventricular (RV) function is still unknown. Angiotensin II receptor blockers are beneficial in patients with acquired left ventricular dysfunction, and recent findings have suggested a favorable effect in symptomatic patients with systemic RV dysfunction. The current study aimed to determine the effect of losartan, an angiotensin II receptor blocker, on subpulmonary RV dysfunction in adults after repaired tetralogy of Fallot.

Cardiac sarcoidosis: challenges in clinical practice.

Purpose Of Review: To address the current recommendations for screening, diagnosis, and treatment of cardiac sarcoidosis and the difficulties to put these recommendations into clinical practice.

Recent Findings: The incidence of cardiac sarcoidosis appears to be higher than earlier reported, probably because of improved imaging techniques. Late gadolinium enhancement with cardiac MRI (LGE-CMR) and fluorodeoxyglucose positron emission tomography obtained a central role in the diagnostic algorithm and monitoring of disease activity.

Right vEntricular Dysfunction in tEtralogy of Fallot: INhibition of the rEnin-angiotensin-aldosterone system (REDEFINE) trial: Rationale and design of a randomized, double-blind, placebo-controlled clinical trial.

Unlabelled: Renin-angiotensin-aldosterone system (RAAS) inhibition with angiotensin II receptor blockers or angiotensin-converting enzyme inhibitors is beneficial in patients with acquired left ventricular dysfunction. Adult patients with tetralogy of Fallot (TOF) with right ventricular (RV) dysfunction are at high risk for heart failure, arrhythmias, and sudden cardiac death. However, the efficacy of RAAS inhibition has not been established in these patients.

Patent Foramen Ovale With Atrial Septal Aneurysm Is Strongly Associated With Migraine With Aura: A Large Observational Study.

Background: A patent foramen ovale (PFO) with atrial septal aneurysm (ASA) has been identified as a risk factor for cryptogenic stroke. Patients with migraine with aura (MA) appear to be at risk for silent brain infarction, which might be related to the presence of a PFO. However, the association between MA and PFO with ASA has never been reported.

One-Year Outcomes After Transcatheter Insertion of an Interatrial Shunt Device for the Management of Heart Failure With Preserved Ejection Fraction.

Background: Heart failure with preserved ejection fraction has a complex pathophysiology and remains a therapeutic challenge. Elevated left atrial pressure, particularly during exercise, is a key contributor to morbidity and mortality. Preliminary analyses have demonstrated that a novel interatrial septal shunt device that allows shunting to reduce the left atrial pressure provides clinical and hemodynamic benefit at 6 months.

QRS fragmentation is superior to QRS duration in predicting mortality in adults with tetralogy of Fallot.

Background: Although QRS duration >180 ms has prognostic value in adults with tetralogy of Fallot (TOF), its sensitivity to predict mortality is low. Fragmented QRS complexes, a simple measurement on ECG, are related to myocardial fibrosis and dysfunction in patients with TOF. Our objective was to determine whether QRS fragmentation predicts major outcomes in TOF.

Follow-up of pulmonary right-to-left shunt in hereditary haemorrhagic telangiectasia.

Pulmonary arteriovenous malformations (PAVMs) are associated with severe neurological complications in hereditary haemorrhagic telangiectasia (HHT). Transthoracic contrast echocardiography (TTCE) is recommended for screening of pulmonary right-to-left shunts (RLS). Although growth of PAVMs is shown in two small studies, no studies on follow-up with TTCE exist.

Ablation for Atrial Fibrillation Combined With Left Atrial Appendage Closure.

Objectives: The study sought to determine long-term clinical effects of combining catheter ablation (CA) and left atrial appendage (LAA) occlusion (LAAO) in a single procedure.

Background: CA relieves symptoms in atrial fibrillation (AF), but freedom from AF is not assured. Thus, oral anticoagulation (OAC) remains necessary in high stroke risk patients.

Aortic valve prosthesis-patient mismatch and exercise capacity in adult patients with congenital heart disease.

Objectives: To report the prevalence of aortic valve prosthesis-patient mismatch (PPM) in an adult population with congenital heart disease (CHD) and its impact on exercise capacity. Adults with congenital heart disease (ACHD) with a history of aortic valve replacement may outgrow their prosthesis later in life. However, the prevalence and clinical consequences of aortic PPM in ACHD are presently unknown.