David S. Majdalany

Double aortic arches are rare congenital malformations resulting from persistence of the right and left aortic arches, as opposed to normal regression of the left aortic arch. These vascular malformations can cause vascular rings since they encompass the trachea and esophagus, and lead to swallowing difficulties or respiratory difficulties from extrinsic compression. Typically, these are diagnosed in childhood but rarely may present in adults. In the presence of symptoms, which require formal gastrointestinal and pulmonary evaluation, surgical resection of the nondominant arch is typically recommended to release the vascular ring. Monitoring of the carotid and subclavian vasculature is performed intraoperatively to ensure distal perfusion is not affected following distal ligation of the arch.

Preeclampsia (PE) is a multifactorial hypertensive disorder of pregnancy that significantly increases both short- and long-term cardiovascular risk for affected women. PE and cardiovascular disease (CVD) share common risk factors, including endothelial dysfunction, obesity, insulin resistance, and dyslipidemia. Women with a history of PE face a markedly elevated risk of chronic hypertension, heart failure, and adverse cardiac remodeling, with evidence suggestive of persistent vascular and myocardial changes after pregnancy. The complex pathophysiology of PE is multifactorial and is thought to involve a combination of abnormal placentation, immune dysregulation, and anti-angiogenic factors, which may induce permanent cardiovascular alterations. Genetic predispositions may further link PE with cardiomyopathies and peripartum cardiomyopathy. However, despite these well-established risks, standardized long-term surveillance and management strategies for women with prior PE remain lacking. Early identification and targeted intervention in women with a history of PE represent critical opportunities to mitigate future cardiovascular morbidity and mortality. This review highlights the urgent need for comprehensive, evidence-based strategies that incorporate personalized follow-up and risk stratification to improve cardiovascular outcomes in this high-risk population.

Background/Objectives: Radiofrequency (RF) wire energy can be used for tissue ablation across many conditions. Adjusting RF generator parameters allows RF energy to puncture tissue with minimal adjacent damage. When RF energy is applied to tissue, however, microbubbles are produced, obstructing intravascular ultrasound (IVUS). Mitigation of RF-generated microbubbles has been studied for ablation but not for puncture. Methods: This paper describes an in vitro bench study using ex vivo bovine live tissue. A model was created with bovine liver tissue and an IVUS catheter submerged in a saline bath. Tissue was punctured with an RF guidewire to recreate microbubbles. Following the puncture, various methods were applied: altering the mechanical index of the IVUS, applying a VF10-5 Linear probe (Siemens), and applying a L12-3 Linear probe (Philips). Regions of interest (ROIs) were selected to track pixel brightness as a proxy for microbubbles. Results: The control increased ROI brightness by 1.5%. Altering the mechanical index of IVUS reduced ROI brightness by 1.2%. VF10-5 probe application increased ROI brightness by 1.2%. L12-3 probe application reduced ROI brightness by 33.0% (p = 0.046, n = 3, one-sample t-test). Brightness reduction was most pronounced at the site of initial RF wire puncture, where microbubbles accumulated. Tip visualization improved, allowing for more precise wire trajectory adjustments. Conclusions: External US with an L12-3 probe was able to dissipate microbubbles effectively to improve IVUS guidance following RF wire puncture in an in vitro exploratory bench model.

Coronary anomalies, although rare, should be considered when young patients present with angina. Clinical suspicion and multi-modality imaging including coronary angiography and tomographic imaging should be pursued for symptomatic patients such as the one we are presenting with anomalous right coronary artery from the pulmonary artery. She was promptly referred for surgical intervention with re-implantation of the right coronary artery onto the aorta.

Pregnancy induces significant physiologic changes that impact the cardiovascular system, potentially exacerbating pre-existing cardiac conditions or precipitating new illnesses. Pregnant patients with cardiac emergencies pose unique challenges, as standard interventions may pose risks to the developing fetus. This article aims to enhance emergency physicians’ confidence in managing pregnancy-related cardiac emergencies by providing a structured approach to initial evaluation and stabilization. We review eight common categories of pregnancy-associated cardiac illness: gestational hypertension and pre-eclampsia, cardiomyopathy, arrhythmias, valvular disease, aortopathies, congenital heart disease and pulmonary hypertension, coronary disease, and anticoagulation-related complications. For each condition, we summarize relevant pregnancy-specific pathophysiology and outline evidence-based, personalized emergency management strategies.

Ebstein’s anomaly (EA) is a rare cyanotic form of congenital heart disease (CHD) characterized by apical displacement of the tricuspid valve, with resultant hemodynamic and electrical manifestations. The severity of symptoms is determined by the degree of apical displacement and deformity and incompetence of the tricuspid valve. As a result, patients with EA can be severely symptomatic during infancy and childhood or can be incidentally discovered in the sixth or seventh decade of life. Hallmarks of Ebstein’s anomaly include progressive cyanosis, right-sided heart failure, and tachyarrhythmias, among which tachyarrhythmias (most commonly atrial, but also ventricular) are the most common presenting symptoms in Ebstein’s anomaly patients during adulthood. This review aims to provide insight into the genetic and electrophysiological (EP) basis underlying the tachyarrhythmias encountered when managing patients with EA.

Coarctation of the aorta (CoA) comprises 5–7% of congenital heart disease and can present as an isolated narrowing in the aortic arch just distal to the left subclavian artery or can be associated with cardiac abnormalities such as a bicuspid aortic valve, aortopathy, or ventricular septal defects. With the advances in the medical field, intervention on CoA can either be via surgical repair or endovascular stenting. Echocardiography is the mainstay in diagnosing CoA, with tomographic imaging such as magnetic resonance imaging (MRI) or computed tomography providing supplementary assessment of the aorta, valves, and collateral vessels. We present a case of a young hypertensive male who was noted to have a continuous cardiac murmur with diagnostic Doppler pattern of CoA on echocardiography that normalized soon after percutaneous stenting.

Congenital heart disease in adult patients (ACHD) includes individuals with native anatomic deformities and those who have benefited from corrective, ameliorative, or interventional heart and vascular interventions. Congenital heart disease is the most common birth defect, although with interventions most survive into adulthood. Newborns and children with complex congenital heart diseases that feature cyanosis fail to thrive, and once this is identified, heart failure can promptly undergo diagnostic evaluations and treatment. However, patients with simple congenital heart disease and subtle clinical signs and symptoms may escape diagnosis until adulthood or experience changes in their cardiac hemodynamics and physiology in settings such as pregnancy or newly diagnosed arrhythmias. The chest X-ray (CXR) is the most common X-ray among all radiological procedures. Individual features or a constellation of features on a CXR are often present in patients who have congenital heart disease. The ability to recognize these CXR features is a valuable skill for making the diagnosis of ACHD and for following these patients as they age, and can complement echocardiographic findings. When used well to diagnose ACHD, the CXR will be the sharpest arrow in the quiver.

The electrocardiogram is the first test that is undertaken when evaluating a patient’s heart. Diagnosing congenital heart disease in an adult (ACHD) can be facilitated by knowing the classical electrocardiographic (EKG) findings. These EKG findings often result from the congenital defect that prevents a part of the cardiac conduction system from occupying its normal anatomic position. When these classical EKG findings are not present, the clinician should consider alternate diagnoses. As the patient with congenital heart disease ages, with native anatomy or after surgical or device repair, the EKG can be used to assess the patient’s status and to decide if and when treatment requires adjustment. This is because the electrocardiogram (EKG) can diagnose the hypertrophy or enlargement in a cardiac chamber that results from the congenital defect or anomaly and can diagnose an arrhythmia that might compromise an otherwise stable anatomy. While ACHD often involves intracardiac shunting, in many cases the abnormality only involves cardiac electrical conduction block or ventricular repolarization. These life-threatening diseases can be diagnosed with an EKG. This review will demonstrate and explain how the EKG can be used to diagnose and follow adults with congenital heart disease. When coupled with history and physical examination, the value of the EKG in ACHD will be apparent. A diagnosis can then be made or a differential diagnosis proposed, before an imaging study is ordered.