A Rare Case of Heterotaxy Syndrome: Eisenmenger Syndrome with Dextrocardia in an Adult.

Aims: Heterotaxy syndrome is a rare, complex, and confusing presentation in the realm of situs anomalies. The concurrence of heterotaxy syndrome with dextrocardia and Eisenmenger syndrome has never been reported in an adult. We described, for the first time, an adult patient who had heterotaxy syndrome with dextrocardia, complicated by Eisenmenger syndrome with large ventricular septal defect (VSD). Presentation of Case: A 43 year-old female presented to the emergency room with worsening exertional dyspnea. She carried past medical history of cyanotic congenital heart disease since birth. Her physical examination findings were significant for hypoxia and clubbing with cyanotic fingers and toes, dextrocardia, and a 3/6-holosystolic murmur over the right lower sternal border. Laboratory investigation showed polycythemia andher chest x-ray showed dextrocardia. Echocardiography showed dextrocardia with a dilated left ventricle and a large VSD with left-to-right shunting. Computed tomography (CT) angiogram of the chest showed bilateral bi-lobed lungs with both main stem hyparterial bronchi, right-sided polysplenia, right-sided stomach with left-sided dominant liver. Discussion and Conclusion: Heterotaxy syndrome has a complex variation in clinic presentation. We illustrate the occurrence of heterotaxy syndrome, polysplenia type with Eisenmenger syndrome and dextrocardia. Recognition of this rare anomaly is important for establishing a diagnosis in a patient with multiple organ displacement.

Simultaneous Occurrence of Spontaneous Coronary Artery Dissection and Embolic Stroke.

Acute coronary syndrome (ACS) due to spontaneous coronary artery dissection (SCAD) is rare. Further, concurrent ACS with a cerebrovascular accident is improbable, but possible. We report a case of a young man, with a history of Hodgkin’s lymphoma treated with a combination of chemotherapy and radiotherapy ten years ago, presented with acute coronary syndrome caused by an extensive dissection of the right coronary artery, together with acute ischemic stroke. Survivors of Hodgkin’s lymphoma are at increased risk for cardiovascular complications due to radiation, which can expedite atherosclerosis and can, eventually, give rise to dissection and cerebrovascular disease, as exemplified in our case. This case report and review outlines the incidence, epidemiology, causes, pathophysiology, diagnosis and treatment of spontaneous coronary artery dissection. Our case report is a remainder to clinicians to be mindful of concomitant occurrence of these two conditions and highlights the significant impact the treatment of each has on the other, especially when the literature does not have clear recommendations about simultaneous management. Spontaneous coronary artery dissection with concomitant ischemic cerebrovascular stroke poses a therapeutic dilemma and requires a multi-disciplinary team to appropriately manage the patient.