Adam W. Powell

Rare Cause for a Continuous Murmur: Large Left Coronary Artery to Coronary Sinus Fistula

A one-year-old asymptomatic male infant was referred for evaluation of a murmur. Examination revealed a grade III/VI continuous murmur heard throughout the upper precordium. The clinical presentation was thought to be consistent with a patent ductus arteriosus and an echocardiogram was performed. This demonstrated a large left coronary artery to coronary sinus fistula (measuring 5 mm 6 mm in the largest diameter) which originated proximal to the left anterior descending and circumflex coronary artery (Supplemental Video; Figures 1A and B and 2.). The coronary sinus was dilated but drained unobstructed to the right atrium. The left ventricle was mildly dilated, but the rest of the cardiac structure and function was normal. Due to the reported risk of progressive congestive heart failure, infective endocarditis, and coronary ischemia secondary to thrombosis or steal phenomenon, a decision was made to proceed with transcatheter device closure of the fistulous connection. An 8 mm 7 mm Amplatzer Vascular Plug II (St Jude Medical, St Paul, Minnesota) was used to successfully occlude the fistulous connection (Figure 1C and D). At the two-month follow-up visit, the patient was asymptomatic. His echocardiogram demonstrated no residual shunting, unchanged device location, persistent dilation of the left main coronary artery, and no flow compromise to any of the distal branches. Aspirin was continued due to the potential risk of coronary thrombosis, especially among those with large fistulous connection as in the present report. This report highlights the diagnosis and management of a rare form of fistulous connection arising from the left main coronary artery and draining to the coronary sinus which accounts for <5% of patients in a large series of coronary fistulous connections. Percutaneous catheter-based closure offers a viable and less invasive alternative to surgical ligation.

Diastolic dysfunction is associated with exercise impairment in patients with sickle cell anemia

Left ventricular diastolic dysfunction (DD) is an independent risk factor for mortality in sickle cell anemia (SCA) and is associated with increased extracellular volume (ECV) on cardiac MRI (CMR). Exercise impairment is common in SCA, but its causes and prognostic value are not well understood.

Cardiac morphology for the millennial cardiology fellow: Nomenclature and advances in morphologic imaging: Cardiac Morphology for the Millennial Cardiology Fellow

Cardiology fellows-in-training, both in adult and pediatric hospitals, need structured education in regards to congenital heart disease (CHD) nomenclature. With improved survival of patients with CHD, it is not uncommon for these patients to seek care in multiple adult and pediatric hospitals. A deep understanding of CHD nomenclature would aid in providing accurate medical and surgical care for these patients. In this forum, we share our experience with such structured education and also comment on recent advances in morphologic imaging that would aid in understanding the nomenclature.

Cardiopulmonary fitness assessment on maximal and submaximal exercise testing in patients with Fabry disease

The cardiopulmonary exercise test (CPET) is a valuable tool to assess a patients aerobic fitness and cardiac function, including the response to stress. There have been few studies using CPET to evaluate cardiopulmonary exercise capacity in patients with Fabry disease. We performed a retrospective chart review of patients with Fabry disease from 2001 to 2016, compared to age, gender, and size‐matched normal controls. A total of 18 patients were evaluated using the Bruce protocol (treadmill) and 11 patients were evaluated with the ramp protocol (cycle ergometer). The Fabry group demonstrated significantly lower heart rate at peak exercise (151.2 ± 22.5 vs. 178.6 ± 16.2, p < .05), max indexed VO2 (23.7 ± 7 vs. 33.9 ± 8.4, p < .05), and peak index oxygen pulse (12.1 ± 3 vs. 15.2 ± 4.2, p < .05). When the groups were further separated into treadmill or cycle ergometry testing only, there remained statistically significant differences in peak indexed oxygen pulse, heart rate at peak exercise, and max indexed VO2. There was a statistically significant difference between the Fabry patients evaluated by treadmill testing for systolic blood pressure at peak exercise that was not seen in the cycle ergometry group. Additionally, when looking at the patients who had concurrent cardiac MRI (cMRI) with their CPET, there was a positive correlation with max indexed VO2 and right ventricular end‐diastolic volume (r = .55, p = .007) and end‐systolic volume (r = .59, p = .007). Patients with Fabry disease have impaired cardiopulmonary exercise capacity as measured by CPET. Additionally, in patients with Fabry disease there is a positive correlation with functional capacity and right ventricular volumes on cMRI.

Left Ventricular Noncompaction With Muscular Ventricular Septal Defect in Mother and Son

A 34-year-old previously healthy woman at 34 weeks’ gestation presented to our clinic for advice following an abnormal prenatal echocardiogram. Fetal echocardiogram demonstrated severe biventricular dysfunction, mild tricuspid regurgitation, and prominent right ventricular trabeculations. Infant was born at 37 weeks. A postnatal echocardiogram demonstrated moderately diminished biventricular dysfunction, major left ventricular trabeculations most prominent in the inferior and lateral segments extending to the apex, and two small muscular ventricular septal defects (Figure 1). He was diagnosed with left ventricular noncompaction (LVNC) and left ventricular dysfunction. The mother’s screening echocardiogram demonstrated apical LVNC with mildly diminished left ventricular function, a mildly hypoplastic right ventricle, and a small muscular ventricular septal defect (Figure 2). Her end-systolic ratio of compacted to noncompacted layers was 3.3:1. Cardiac magnetic resonance imaging delineated the abnormal

Widespread Vasculopathy in a Patient with Morquio A Syndrome

Morquio A syndrome (mucopolysaccharidosis IV type A), an autosomal recessive lysosomal storage disorder caused by a defective N-acetylgalactosamine 6-sulfatase gene, leads to lysosomal accumulation of keratan sulfate and chondroitin 6-sulfate. This accumulation affects multiple systems and causes notable cardiovascular manifestations, such as thickening of the left-sided valves, ventricular hypertrophy, and intimal stenosis of the coronary arteries. There have been few reports of vasculopathy in this population. We present the case of a 58-year-old woman with Morquio A syndrome who was found to have aortic dilation on a routine screening echocardiogram. Magnetic resonance images revealed multiple tortuous, dilated arteries in her head, neck, and abdomen. The diffuse vasculopathy seen in this patient should prompt further study to determine whether this is an underreported phenomenon of clinical significance or an unusual finding in this rare disorder.

Incorrect ventricular lead placement into the systemic right ventricle of a patient with D-transposition of the great vessels after Mustard procedure.

Incorrect pacemaker lead placement into the systemic ventricle is a complication that has rarely been described in patients with D-transposition status after atrial baffle palliation. We present a case of ventricular lead misplacement in the systemic right ventricle of a patient with D-transposition of the great arteries after Mustard procedure. This case demonstrates the challenges with proper imaging of lead placement in patients with atrial baffles and long-term management of a lead in the systemic ventricle.