Sinus venosus atrial septal defect presenting with platypnea-orthodeoxia syndrome in the setting of COVID-19 infection.

Platypnea-orthodeoxia syndrome is characterised by hypoxemia and dyspnoea while upright that resolves when supine, typically associated with an interatrial communication. We present a case of platypnea-orthodeoxia syndrome associated with a sinus venosus atrial septal defect in a patient with multiple possible aetiologies of hypoxemia, including COVID-19 infection. Cardiac catheterisation with provocative maneuvers confirmed the diagnosis and symptoms resolved following defect closure. We suggest that in patients with platypnea-orthodeoxia syndrome, it is useful to obtain haemodynamic data while supine and upright.

Spontaneous aberrant right subclavian arterio-oesophageal fistula in a previously healthy child.

An aberrant right subclavian artery arising from a left aortic arch is the most frequently described congenital aortic arch anomaly, occurring in 0.5 to 2.3% of the general population. Despite the retro-oesophageal course of the aberrant subclavian artery, an arterio-oesophageal fistula is an uncommon finding, only previously reported as a very rare complication in critically ill patients with oesophageal instrumentation or foreign body ingestion. We describe a unique case of a spontaneous aberrant right subclavian arterio-oesophageal fistula without an inciting event in a 17-month-child.

A Rare Cause of a Continuous Murmur in a Newborn.

We describe what we believe to be the first reported case of a neonate with right coronary ostial atresia with the right coronary artery originating from the left circumflex coronary artery, in conjunction with a right coronary artery to right ventricle fistula in a patient with otherwise normal cardiac anatomy. This was found following an evaluation for a continuous murmur at 2 weeks of life with elevated troponin and abnormal electrocardiogram. Thus far the child has required no intervention and is asymptomatic at 17 months of age, but he will require long-term follow-up to monitor the size of the fistula and potential for myocardial insufficiency.

Total Thrombotic Occlusion of an Extracardiac Fontan Conduit in a Hemodynamically Stable Patient.

Cardiac and extracardiac complications of single-ventricle physiology are well described and carry significant morbidity and mortality risks. We present the case of a 32-year-old patient with a Fontan circulation who presented to the emergency department with epigastric pain, and with chest computed tomography angiography concerning for total thrombosis of the extracardiac conduit.

Unicuspid Aortic Valve With Severe Aortic Stenosis and Progressive Heart Failure During Pregnancy.

A 39-year-old gravida 7 para 6 woman with unicuspid aortic valve and severe symptomatic stenosis was admitted to the hospital at 15 weeks gestation. We describe maternal cardiovascular complications and their implication on obstetric and fetal care. We also describe our multidisciplinary approach to caring for this complex patient.

Differential Impact of Pulmonary Regurgitation on Patients With Surgically Repaired Pulmonary Stenosis Versus Tetralogy of Fallot.

Patients with repaired pulmonary stenosis (PS) or tetralogy of Fallot (TOF) both develop pulmonary regurgitation (PR) leading to right ventricular (RV) dilatation and dysfunction. We aimed to characterize differential effects of chronic PR in these populations. Patients with surgically repaired PS were matched 1:2 by age and PR fraction with patients with TOF. Patients with previous pulmonary valve replacement were excluded. Cardiovascular magnetic resonance data were compared; peak longitudinal and circumferential systolic strain by feature tracking were compared to evaluate differential contribution of the RV sinus and outflow tract, respectively. PS (n = 24, 41 ± 13 years old) and TOF (n = 47, 39 ± 13 years old) patients did not differ in RV end-diastolic volume (153 ± 45 vs 154 ± 45 ml/m(2), p = 0.99) or diastolic function. However, patients with PS had preserved RV ejection fraction (54.3 ± 4.4% vs 48.0 ± 7.1%, p <0.0001). Differences were greater in RV circumferential (-15.8 ± 3.3 vs -11.8 ± 3.4, p <0.0001) than longitudinal strain (-18.0 ± 3.8 vs -15.9 ± 3.8, p = 0.04), with particular decrease in the infundibulum (-17.4 ± 7.5 vs -6.8 ± 6.3, p <0.0001). Late gadolinium enhancement in the RV outflow tract was more frequent in patients with TOF (70.2% vs 45.8%, p = 0.001). In conclusion, surgical repair of PS leads to similar RV dilatation and diastolic dysfunction compared to patients with TOF, but differential effects on ventricular systolic function, largely related to differences in the outflow tract. With different patterns of scarring and ventricular remodeling, further study is needed to clarify whether criteria for pulmonary valve replacement in patients with PS should differ from those with TOF.