Clinical Clues and Diagnostic Workup of Cardiac Amyloidosis.

Cardiac amyloidosis is increasingly recognized as an underlying cause of left ventricular wall thickening, heart failure, and arrhythmia with variable clinical presentation. Due to the subtle cardiac findings in early transthyretin cardiac amyloidosis and the availability of therapies that can modify but not reverse the disease progression, early recognition is vital. In light chain amyloidosis, timely diagnosis and treatment can significantly improve survival. In this manuscript, we review the clinical, imaging, and electrocardiographic clues that should raise suspicion for cardiac amyloidosis and provide a simplified diagnostic workup algorithm that ensures an accurate diagnosis. The evolution of the noninvasive diagnosis of cardiac amyloidosis has significantly influenced our understanding of disease prevalence, presentations, and outcomes. However, clinical recognition of clues and red flags remains the most important factor in advancing the care of patients with cardiac amyloidosis.

Pregnancy Management in a Patient With Congenital Heart Disease and Severe Right Ventricle to Pulmonary Artery Conduit Stenosis.

Background: Pregnancy causes multiple hemodynamic changes that place significant stress on the cardiovascular system. With advancements in medical care, individuals with complex congenital heart disease are living into their childbearing years. Much remains to be understood about the effects and management of pregnancy in individuals with complex congenital heart disease. Case Report: We describe the management and delivery of a 29-year-old pregnant female with repaired tetralogy of Fallot or ventricular septal defect with pulmonary atresia. The patient presented at 21 weeks' gestation with New York Heart Association class II symptoms and pulmonary conduit stenosis, with a mean gradient of 52 mmHg. At 36.5 weeks' gestation, she developed severe pulmonary conduit stenosis with a mean gradient of >75 mmHg. The patient was admitted at 37 weeks' gestation for planned delivery. After a successful cesarean section and bilateral tubal ligation, the patient had an uncomplicated postoperative course. She was scheduled for follow-up for severe conduit stenosis at 6 weeks postpartum to discuss management options. Conclusion: Management of a pregnant patient with adult congenital heart disease should involve risk stratification for complications (commonly congestive heart failure exacerbation and arrhythmias) using tools such as the modified World Health Organization pregnancy risk classification. Based on the risk category, decisions must be made about frequency of follow-up, anesthesia, and mode of delivery. Patients in moderate to high-risk stratification should be managed by a multidisciplinary team at a specialty center, and all patients should undergo an anesthesia consultation prior to delivery. The decision for vaginal or cesarean delivery should be made on a case-by-case basis with consideration given to patient preference. Patients with asymptomatic moderate to severe pulmonic stenosis can be managed conservatively with appropriate follow-up and cardiac imaging, allowing intervention to be completed after delivery.