Faculty Bibliography

Background: There is limited data regarding the interplay of anatomic and physiologic parameters with exercise capacity in adults with native or recurrent nonsevere coarctation of the aorta (CoA). Objectives: The objective of this study was to use exercise stress echocardiography and cardiac magnetic resonance imaging to identify anatomic and physiologic correlates of exercise capacity in these patients. Methods: We conducted a single-center retrospective analysis of 54 adults with nonsevere CoA (repaired or unrepaired) followed at our institution between 2015 and 2020. Resting coarctation gradients were obtained using echocardiography. Exercise gradients and functional capacity were assessed using exercise stress echocardiography. Aorta anatomy was obtained using magnetic resonance imaging. Results: Coarctation-to-diaphragm ratio correlated with minutes of exercise (r = 0.56, P < 0.01) and metabolic equivalents (r = 0.49, P < 0.01). These relationships remained significant after controlling for use of beta-blockers, valvular disease, and type of coarctation repair. Minutes of exercise correlated with mean resting gradients (r = −0.39, P < 0.05). Coarctation-to-diaphragm ratio correlated with peak and mean resting gradients (r = −0.34, P < 0.05; r = −0.48, P < 0.01). Patients with coarctation-to-diaphragm ratio ≤0.7 achieved fewer metabolic equivalents (11.1 ± 1.9 vs 12.8 ± 2.2, P < 0.05) and minutes of exercise (10.3 ± 2.0 vs 12.6 ± 2.7, P < 0.05). Conclusions: In patients with nonsevere native or recurrent CoA, reduced exercise capacity is correlated with coarctation severity by anatomic size and gradients. Those with a coarctation-to-diaphragm ratio ≤0.7 may represent a subset of patients with nonsevere CoA whose clinical symptoms are only elicited with exercise stress testing. Exercise stress testing and cross-sectional imaging may help identify those who could be considered for earlier coarctation intervention.

Specialty care is associated with improved outcomes for adults with adult CHD and must be extended to the underserved. A retrospective cohort study was performed to describe the provision of care to adult CHD patients in America's largest municipal public health system including patient demographics, diagnostic and therapeutic procedures, and adherence to guideline-recommended surveillance. We identified 229 adult CHD patients aged >18 years through electronic medical records. The most common diagnoses were atrial septal defect, ventricular septal defect, patent ductus arteriosus, and valvular pulmonary stenosis. In total, 65% had moderate or greater anatomic complexity. A large number of patients were uninsured (45%), non-white (96%), and non-English speaking (44%). One hundred forty-six patients (64%) presented with unrepaired primary defects. Fifty eight patients underwent primary repair during the study period; 48 of those repairs were surgical and 10 were transcatheter. Collaboration with an affiliated Comprehensive Care Center was utilised for 28% of patients. A high proportion of patients received adult CHD speciality visits (78%), echocardiograms (66%), and electrocardiograms (56%) at the guideline-recommended frequency throughout the study period. There was no significant difference in the rate of adherence to guideline-recommended surveillance based on insurance status, race/ethnicity, or primary language status. The proportion of patients who had guideline-recommended adult CHD visits, echocardiograms, and electrocardiograms was significantly lower for those with more advanced physiological stages. These results can inform the provision of adult CHD care in other public health system settings.

There remains significant controversy in the risk stratification and management of patients with anomalous right coronary artery originating from the opposite sinus (R-ACAOS). We present the case of a patient with an inferior ST-elevation myocardial infarction, found to have R-ACAOS and severe atherosclerotic right coronary artery disease, treated with fractional flow reserve and intravascular ultrasound-guided percutaneous coronary intervention.

A 39-year-old gravida 2, para 1 woman presented for evaluation of palpitations in pregnancy. She was found to have supraventricular tachycardia. The initial echocardiogram demonstrated a color Doppler signal in the proximal pulmonary artery with diastolic-dominant flow, suggestive of coronary flow. She received a diagnosis of anomalous right coronary artery from the pulmonary artery. Her arrhythmia was managed medically. We discuss techniques for risk stratification of cardiac lesions in pregnancy and for the management of anomalous coronary origin from the pulmonary artery. (Level of Difficulty: Intermediate.).

The prevalence of congenital heart disease (CHD) in pregnancy is rising due to the improved survival of patients with CHD into childbearing age. The profound physiological changes that occur during pregnancy may worsen or unmask CHD, affecting both mother and fetus. Successful management of CHD during pregnancy requires knowledge of both the physiological changes of pregnancy and the potential complications of congenital heart lesions. Care of the CHD patient should be based on a multidisciplinary team approach beginning with preconception counseling and continuing into conception, pregnancy, and postpartum periods. This review summarizes the published data, available guidelines and recommendations for the care of CHD during pregnancy.

Ventricular septal defect (VSD) is the most common congenital heart lesion among children. In most cases, however, it is identified and corrected in childhood, before long-term sequelae such as pulmonary hypertension develop. In this case report, we present a young man with an undiagnosed VSD with consequent Eisenmenger syndrome who initially presented to medical attention with diplopia found to be caused by cerebral infarcts.