Majdalany, David

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.

While congenital heart disease historically was a pathology primarily restricted to specialized pediatric centers, advances in technology have dramatically increased the number of people living into adulthood, the number of complications faced by these patients, and the number of patients visiting non-specialized emergency departments for these concerns. Clinicians need to be aware of the issues specific to patients’ individual congenital defects but also have an understanding of how typical cardiac pathology may manifest in this special group of patients. This manuscript attempts to provide an overview of this diverse but increasingly common group of adult patients with congenital heart diseases, including a review of their anatomical variants, the complications they face at the highest rates, and ways that emergency physicians may need to manage these patients differently to avoid causing harm.

Current management of patients with congenital heart disease has increased their survival into adulthood. This is accompanied by potential cardiac complications, including pulmonary hypertension associated with congenital heart disease (PAH-CHD). PAH-CHD constitutes a challenging subgroup of pulmonary hypertension and requires expert management to improve quality of life and prognosis. Novel agents have shown a significant improvement in morbidity and mortality in patients with pulmonary arterial hypertension. However, the long-term effects of these medications on PAH-CHD patients remain somewhat uncertain, necessitating treatment plans largely founded on the clinical experience of the healthcare providers. The aim of this review is to summarize the current evidence and future perspectives regarding treatment strategies for PAH-CHD to help better guide management of this complex disease.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare coronary anomaly that can present in childhood or adulthood with a multitude of symptoms depending on the age of presentation. It should be suspected in infants presenting with heart failure in the setting of left ventricular systolic dysfunction and associated mitral regurgitation from papillary muscle ischemia. Adults with ALCAPA may present with cardiac ischemic symptoms. Prompt diagnosis with echocardiography and cross-sectional chest imaging is important to guide surgical intervention and improve the patients’ survival and prognosis. The goal of surgery is to establish a dual-coronary system with mid-term results revealing progressive recovery of left ventricular function and improvement in mitral regurgitation. Patients with ALCAPA should maintain life-long follow-up with a cardiologist with congenital heart disease expertise for surveillance of post-operative complications.

Congenital heart disease (CHD) is the most frequent disorder encountered in the newborn period and the most frequent cause of infant mortality [...]

Congenital heart disease (CHD), which affects 1% to 2% of all births, is the most common abnormality in women contemplating pregnancy in western countries. With diagnostic and interventional advances, most patients with CHD survive into adulthood and require lifelong cardiac follow-up with cardiac imaging, particularly echocardiography and cardiac computed tomography. Multiple hemodynamic and physiologic changes of pregnancy may predispose patients with CHD to clinical decompensation and an inability to tolerate pregnancy. This manuscript reviews common CHD lesions, their repair or palliative interventions, long-term sequelae, important features to assess on cardiac imaging, and the impact of pregnancy on these types of lesions. Moreover, the review bridges the fields of CHD, cardiac imaging, and maternal cardiology, which will aid clinicians in counseling patients and managing pregnancies.