Severe Aortic Root Dilatation in a Patient With Larsen Syndrome.

We present a five-year-old female diagnosed with Larsen syndrome at birth with severe aortic root dilatation, failure to thrive, and developmental delay. This report highlights the specific physical findings, imaging, genetics, and surgical treatment, along with a brief overview of Larsen syndrome.

Idiopathic Fascicular Ventricular Tachycardia in a Pediatric Patient With Suicidal Ideation and Self-Injurious Behavior.

Idiopathic fascicular ventricular tachycardia (IFVT) is an arrhythmia that occurs in a structurally normal heart and may present with sudden onset in a healthy individual. We present the case of a 10-year-old female child, with no pertinent medical history, who complained of palpitations and shortness of breath, which was followed by suicidal ideations. On presentation to the ER, tachycardia was noted with other vital signs within normal limits. Labs were unremarkable. EKG showed wide-complex tachycardia with right bundle branch block and left superior axis, consistent with idiopathic left ventricular fascicular tachycardia. The echocardiogram showed normal cardiac structure. She was transferred to the cardiovascular care unit and intravenous verapamil was given with the resolution of symptoms and reversal of tachycardia. She remained hemodynamically stable and was subsequently discharged on oral verapamil. This case report is aimed at raising awareness of the different ways IFVT can manifest, aiding physicians to easily recognize the zebra among the horses.

Accidental Ingestion of Tetrahydrocannabinol-Laced Gummies Causing Bradycardia and First-Degree Atrioventricular Block in a Pediatric Patient: A Case Report.

An increasing trend of cannabis use places children at risk for the detrimental effects of marijuana. Poison control centers in the United States have been experiencing an upsurge in calls involving marijuana ingestion among children in the past years, specifically in states where marijuana is legal. With marijuana ingestion, neurologic symptoms predominate but cardiovascular manifestations have also been observed. Bradycardia and bradyarrhythmia are both uncommon cardiac manifestations of cannabis ingestion in children. Here, we present the case of a previously healthy two-year-old male with sinus bradycardia and first-degree atrioventricular (AV) block following accidental ingestion of tetrahydrocannabinol-laced gummies. Although bradycardia and first-degree AV block are uncommon after cannabis ingestion in children, clinicians should be aware of these findings and must consider evaluating for marijuana toxicity whenever presented with these acute signs. Prevention is crucial and can be achieved through supervision, parental education, and support.

Incomplete Kawasaki Disease in an Infant: A Case Report and Literature Review.

The term "incomplete Kawasaki Disease (IKD)" was first used to describe patients with coronary complications who did not fulfill the classical diagnostic criteria for Kawasaki Disease (KD). The risk of coronary artery involvement is similar if not greater in cases of IKD. However, the recognition of IKD is challenging and often delayed, especially in infants. Multiple algorithms have been formulated to identify cases of IKD utilizing supplemental clinical, echocardiographic, and laboratory features. Although fever is not required for a diagnosis of KD in the Japanese guideline, most of the current guidelines, including those of the American Heart Association (AHA), consider the presence of fever for at least seven days a requirement for the diagnosis of both KD and IKD in infants. We present a case of IKD in a four-month-old female who presented with fever for less than three days and did not follow the current AHA algorithm for IKD. An echocardiogram obtained 10 days later revealed a coronary artery aneurysm, and a retrospective diagnosis of IKD was made. A review of the literature identified similar cases with a growing consensus on the need to redefine the role of fever. Pediatricians should search for coronary artery lesions in cases of high clinical suspicion, even if the fever period is short, particularly in those less than six months. Additionally, further innovative research is directly needed to identify immunological and cellular markers that could be tested early in the course of the disease and guide the management.

Cardiopulmonary stress testing in children and adults with congenital heart disease.

Cardiopulmonary exercise stress testing (CPET) is a vital tool used to assess patients with a history of congenital heart disease. There are several tests in the cardiologist's armamentarium that allow for assessment of cardiac anatomy and function. The majority of these tests are only performed with the body at rest and some even require sedation. Exercise stress testing is unique in allowing assessment of the hemodynamic status of a patient in motion. In addition to providing all the information obtained during an exercise stress test, such as heart rate, rhythm, ST-segment analysis, and blood pressure, the CPET provides critical metabolic information. Parameters such as VO2, oxygen pulse, and VE/VCO2 slope help to detail the patient's physiology in a dynamic state. Decisions can then be better made regarding follow-up plans, acceptable exercise recommendations, and future interventions, if necessary. It allows insight into the patient's exercise capacity and quality of life. Norms for both children and adults with many forms of congenital heart disease are now available allowing appropriate comparisons to be made. This review will discuss in detail the CPET and its application in congenital heart disease.