Background With the increasing survival of the congenital heart disease population, there is a growing need for in-depth understanding of blood circulation in these patients. Mock loops provide the opportunity for comprehensive hemodynamic studies without burden and risks for patients. This study aimed to evaluate the ability of the presented mock loop to mimic the hemodynamics of the pulmonary circulation with and without stenosis and the MR compatibility of the system. Methods A pulsatile pump with two chambers, separated by a flexible membrane, was designed and 3D printed. A cough assist device applied an alternating positive and negative pressure on the membrane. One adult, and three pediatric pulmonary bifurcations were 3D printed and incorporated in the setup. Two pediatric models had a 50% stenosis of the left branch. Bilateral compliance chambers allowed for individual compliance tuning. A reservoir determined the diastolic pressure. Two carbon heart valves guaranteed unidirectional flow. The positive pressure on the cough assist device was tuned until an adequate stroke volume was reached with a frequency of 60 bpm. Flow and pressure measurements were performed on the main pulmonary artery and the two branches. The MR compatibility of the setup was evaluated. Results A stroke volume with a cardiac index of 2L/min/m2 was achieved in all models. Physiological pressure curves were generated in both normal and stenotic models. The mock loop was MR compatible. Conclusion This MR compatible mock loop, closely resembles the pulmonary circulation thereby providing a controllable environment for hemodynamic studies.
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by replacement of cardiomyocytes by fibrofatty tissue which can lead to ventricular arrhythmias, heart failure or sudden cardiac death. Genetic defects in desmosomal proteins, as plakophilin-2 (PKP2), are known to contribute to disease development. Current electrocardiographic (ECG) criteria for ARVC diagnosis only focus on right precordial leads, but sensitivity of current depolarization criteria is limited. This study aimed to identify additional depolarization criteria with most optimal lead configurations for early detection of ARVC in PKP2 pathogenic mutation carriers. In PKP2-positive ARVC patients (n=7), PKP2 pathogenic variant carriers (n=16) and control subjects without structural heart disease (n=9), 67-lead body surface potential maps (BSPM) were obtained. Terminal QRS-integrals were determined and quantitatively compared to controls using departure mapping. Significantly different terminal QRS-integrals were identified in lead 34 (conventional V3), 40 and 41 (conventional V4). To conclude, a clear distinction between ARVC patients, asymptomatic mutation carriers and healthy controls was observed.
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