A 17-year-old male with no previous medical illness was admitted to the hospital with abdominal pain. The patient had a long time history of generalized intermittent abdominal pain that increased postprandial without nausea or vomiting. The patient also had a history of fatigue on exertion. Upon checking vital signs, the patient was found to be hypertensive. There was a discrepancy between the upper (155-160/70-75 mmHg) and lower limbs (100-105/65-70 mmHg) readings. Cardiovascular examination showed a systolic ejection murmur best appreciated at the tip of the left scapula, and bracheo-femoral delay. The abdominal examination was unremarkable. His complete blood count, renal profile, liver profile, troponin, and brain natriuretic peptide were normal. Stool analysis and culture were normal. Electrocardiography revealed sinus rhythm and left ventricular (LV) hypertrophy (Figure ). The chest X-ray revealed the classic rib notching sign (Figure ). An echocardiogram revealed a BAV with mild to moderate aortic regurgitation and dilatation of ascending aorta (Figure ). A post-ductal CoA was observed with a systolic gradient of 80 mmHg (Figure ). His LV function was normal with mild eccentric hypertrophy. Cardiac CT was done and confirmed severe post-ductal CoA with extensive collaterals (Figure ). Another incidental finding was a left-sided IVC, which courses anterior to the abdominal aorta between the aorta itself and superior mesenteric artery crossing the midline to the right before joining the right renal vein and the intra-hepatic IVC segment (Figure ). The patient was discussed in a heart team meeting, and it was decided to proceed with transcatheter intervention with stenting.\nThe procedure was done under general anesthesia through the right femoral access. The pre-stenting peak gradient was 60 mmHg, which improved to 4 mmHg using 14 x 39 mm BeGraft aortic stent (Bentley InnoMed, Hechingen, Germany), which is a balloon-expandable covered stent. This was followed by post-stenting dilation with short non-compliant Z-Med balloon (B. Braun Interventional Systems, Bethlehem, PA) (Figure ). No temporary pacing was used for stenting. No periprocedural complications were encountered. The patient stayed in the hospital for two days, then was eventually discharged home on losartan 100 mg daily. Six weeks later, the patient was called for follow up. His symptoms resolved utterly, and blood pressure was well controlled.
A 44-year-old fit and active female was referred by her general practitioner with palpitations, mostly occurring at rest. Electrocardiography performed in the community demonstrated sudden onset of an atrial tachycardia of 115 beats per min. When assessed in hospital, she was asymptomatic with a blood pressure of 110/62 and a regular pulse rate of 50 beats per min. Resting electrocardiography revealed sinus rhythm with poor R wave progression with no evidence of left ventricular hypertrophy or ischemia. A 24-h electrocardiography demonstrated four beats of atrial tachycardia.\nA transthoracic echocardiogram demonstrated a prominent coronary sinus, moderate mitral regurgitation, and a bicuspid aortic valve []. Transesophageal echocardiography showed normal biventricular systolic function, left atrial dilatation, mild to moderate mitral regurgitation, and tricuspid regurgitation. The aortic valve was bicuspid with trivial aortic regurgitation but no significant stenosis. The ascending aorta was dilated for her age and body surface area, with a maximum diameter of 4.0 cm. There was no evidence of aortic coarctation or dissection.\nSaline contrast echocardiography was performed via the left and right arms [], [], [] and []. This confirmed PLSVC with no obvious shunt. Cardiac magnetic resonance imaging (MRI) demonstrated bicuspid aortic valve with dilated aortic root. A PLSVC was seen draining into the coronary sinus and then the right atrium. A separate right-sided non-hypoplastic superior vena cava also emptied into the right atrium. It was noted that a left-sided IVC drained into the coronary sinus and right atrium. Three hepatic veins were seen emptying into a short right-sided IVC, again draining into the right atrium.