Impact of pulmonary stenosis on right ventricular global longitudinal strain in repaired tetralogy of Fallot patients post transcatheter pulmonary valve replacement.

BACKGROUND: Mixed pulmonary disease with pulmonary regurgitation (PR) and stenosis (PS) in repaired tetralogy of Fallot (rTOF) can negatively impact ventricular health. Myocardial strain has been shown to be more sensitive at detecting occult ventricular dysfunction compared to right ventricular ejection fraction (RV EF). We hypothesize that rTOF patients with predominant PS will have lower RV global longitudinal strain (RV GLS) prior to and post-transcatheter pulmonary valve replacement (TPVR). METHODS: A retrospective cohort of rTOF patients who underwent cardiac magnetic resonance (CMR) and cardiac catheterization for right ventricular pressure (RVSP) measurement were analyzed at three time points: before valve implantation, at discharge and within 18 months post-TPVR. Patients were dichotomized into three groups based on RVSP: 0%-49%, 50%-74%, and >75%. RV GLS and left ventricular (LV) GLS by speckle tracking echocardiography (STE) were obtained from the apical 4-chamber using TomTec software (TOMTEC IS, Germany). RESULTS: Forty-eight patients were included. Every 14.3% increase in preimplantation RVSP above 28% was associated with an absolute magnitude 1% lower RV GLS (p = .001). Preimplantation RVSP when 75% or higher had 3.36% worse RV GLS than the lowest bin (p = .014). Overall, average RV strain magnitude was higher when preimplantation RVSP was less than 50% and had greater improvement over the three time points. Higher post implantation RVSP correlated with lower strain magnitude. CONCLUSION: Patients with significant PS (>50%) may benefit from earlier PVR and not depend solely on RV size and EF. Myocardial strain may be a more sensitive marker of function; however, larger, prospective studies are needed.

Patient Driven EKG Device Performance in Adults with Fontan Palliation.

The aim of this study was to evaluate the accuracy of the KardiaMobile (KM) device in adults with a Fontan palliation, and to assess the KM function as a screening tool for atrial arrhythmias. While patient driven electrocardiogram (EKG) devices are becoming a validated way to evaluate cardiac arrhythmias, their role for patients with congenital heart disease is less clear. Patients with single ventricle Fontan palliation have a high prevalence of atrial arrhythmias and represent a unique cohort that could benefit from early detection of atrial arrhythmias. This single center prospective study enrolled adult patients with Fontan palliation to use the KM heart rhythm monitoring device for both symptomatic episodes and asymptomatic weekly screening over a 1-year period. Accuracy was assessed by comparing the automatic KM interpretation (KM-auto) to an electrophysiologist overread (KM-EP) and traditional EKG. Fifty patients were enrolled and 510 follow-up transmissions were received. The sensitivity and specificity of enrollment KM-auto compared to EKG was 65% and 100%, respectively. The sensitivity and specificity of enrollment KM-auto compared to the KM-EP was 75% and 96%, respectively. In the adult Fontan palliation, the accuracy of the KM device to detect a normal rhythm was reliable and best with a physician overread. Abnormal or uninterpretable KM-auto device interpretations, symptomatic transmissions, and any transmissions with a high heart rate compared to a patient's normal baseline should warrant further review.

Arrhythmias in Adult Congenital Heart Disease Heart Failure.

Heart failure and arrhythmias represent 2 major causes of mortality and morbidity in adults with congenital heart disease. Arrhythmias and heart failure are interdependent, and one may exacerbate the other. Treatment of one also has a positive impact on the other. Management approaches need to be multifaceted, including pharmacotherapy, optimization of hemodynamic status with catheter-based or surgical interventions, and specific management of arrhythmia with device or catheter ablation therapy.