Prinzmetal angina in a child with actin gene ACTC1 mutation.

Prinzmetal angina is a rare cause of intermittent chest pain in paediatrics. Here, we report the case of a 2-year-old female who presented with episodic chest pain, malaise, diaphoresis, fatigue, and poor perfusion on exam. During her hospitalisation, these episodes were associated with significant low cardiac output as evidenced by lactic acidosis and low mixed venous oxygen saturations. Her workup revealed an actin alpha cardiac muscle 1 (ACTC1) gene mutation and associated left ventricular non-compaction with decreased systolic function. She was started on oral heart failure medications as well as a calcium channel blocker but continued to have episodes which were found to promptly resolve with nitroglycerine. She was ultimately listed for cardiac transplant given her perceived risk of sudden death.

Prenatal detection of congenital heart disease: Recent experience across the state of Arizona.

OBJECTIVE: To determine the prenatal detection rate (PDR) of congenital heart disease (CHD) in Arizona as well as describe various factors that may influence detection rates. METHODS: This was a retrospective chart review using the Society of Thoracic Surgeons and Phoenix Children's Fetal Cardiology databases. We included all cases of CHD requiring surgery <1 year of age between 2013 and 2018. A total of 1137 patients met the criteria, and various demographic, socioeconomic, and patient outcome data were collected. RESULTS: The overall PDR was 58% with an improving detection rate over the course of our study, with the final year having a PDR of 67%. Over time, PDR improved in urban communities, but this was not seen in rural communities. Rural address, public insurance, and Native American ethnicity were associated with lower PDR. Postnatal outcomes, including Apgars, initial pH, and lactate, did not differ with the presence of a prenatal diagnosis. Diagnoses typically identified with the outflow tract and 3-vessel views on the fetal echocardiogram were less likely to be detected prenatally. CONCLUSIONS: The PDR of CHD continues to improve with evolving technologies and guidelines. We highlight a discrepancy between urban, rural, and Native American populations. Additionally, by supplying descriptors of missed diagnosis and associated echocardiography views, we hope to provide data for future interventions.

Inosculation and perfusion of pre-vascularized tissue patches containing aligned human microvessels after myocardial infarction.

A major goal of tissue engineering is the creation of pre-vascularized tissues that have a high density of organized microvessels that can be rapidly perfused following implantation. This is especially critical for highly metabolic tissues like myocardium, where a thick myocardial engineered tissue would require rapid perfusion within the first several days to survive transplantation. In the present work, tissue patches containing human microvessels that were either randomly oriented or aligned were placed acutely on rat hearts post-infarction and for each case it was determined whether rapid inosculation could occur and perfusion of the patch could be maintained for 6 days in an infarct environment. Patches containing self-assembled microvessels were formed by co-entrapment of human blood outgrowth endothelial cells and human pericytes in fibrin gel. Cell-induced gel contraction was mechanically-constrained resulting in samples with high densities of microvessels that were either randomly oriented (with 420 ± 140 lumens/mm(2)) or uniaxially aligned (with 940 ± 240 lumens/mm(2)) at the time of implantation. These patches were sutured onto the epicardial surface of the hearts of athymic rats following permanent ligation of the left anterior descending artery. In both aligned and randomly oriented microvessel patches, inosculation occurred and perfusion of the transplanted human microvessels was maintained, proving the in vivo vascularization potential of these engineered tissues. No difference was found in the number of human microvessels that were perfused in the randomly oriented (111 ± 75 perfused lumens/mm(2)) and aligned (173 ± 97 perfused lumens/mm(2)) patches. Our results demonstrate that tissue patches containing a high density of either aligned or randomly oriented human pre-formed microvessels achieve rapid perfusion in the myocardial infarct environment - a necessary first-step toward the creation of a thick, perfusable heart patch.