We present a rare late manifestation of systemic venous collaterals in a 17-year-old female, 16 years after bidirectional cavopulmonary anastomosis, resulting in clinically unacceptable desaturation with progressive effort intolerance and cyanosis.

This report describes the case of a neonate with d-transposition of the great arteries and severe pulmonary arterial hypertension stabilised in the post-operative period with continuous iloprost nebulisation. To our knowledge, this is the first documented method of treating post-operative severe pulmonary arterial hypertension with continuous inhaled iloprost in a patient with complex CHD. We found this method of delivering the drug very effective in stabilising haemodynamic swings in the setting of severe pulmonary arterial hypertension.

In this study, three patients presenting with early or late postoperative Fontan complications were identified to suffer from restriction of the native atrial septum. This caused significant obstruction to pulmonary venous return and elevated systemic venous pressure. Dobutamine stress testing was used in one patient to identify this lesion. Transcatheter stenting was performed in the other two patients. Patients improved after relief of the obstruction.

A neonate with transposition of the great arteries and intact ventricular septum presented without pulmonary over-circulation, and subsequently developed pulmonary haemorrhage after corrective surgery. Postoperative CT revealed an aortopulmonary collateral artery arising from the descending aorta, and we performed successful embolisation on postoperative day 9. Aggressive imaging modalities such as angiography and/or CT imaging with contrast can detect unexpected extra-pulmonary blood supply and guide further management.

Patients with single ventricle heart disease often undergo staged surgical palliation, ultimately resulting in Fontan anatomy and physiology. Long-term consequences include cirrhosis of the liver, protein-losing enteropathy, and premature death. Elevated central venous pressure and venous congestion transmitted to the abdominal viscera have been implicated in the aetiology of many of these complications. We present a novel operation directed at protecting the liver and intestines by excluding the splanchnic venous return from the Fontan pathway. Instead of exposure to elevated Fontan pressures, the liver and intestines will be exposed to lower common atrial pressures. We hope that this modification will minimise the abdominal complications of Fontan anatomy and physiology.

A 36-year-old woman, who had a history of myomectomy, presented with lightheadedness after changing position from sitting to standing and effort-related shortness of breath. Echocardiography demonstrated a hyperechoic elongated mobile mass extending from the inferior caval vein to the right atrium. The mass was surgically removed, and histological examination established the diagnosis of intravenous leiomyomatosis. This case caught the attention of our cardiology group to consider the diagnosis when an inferior caval vein or right atrium mass is found in a patient with a history of uterine leiomyomatosis.

We describe the case of an asymptomatic girl with sinus bradycardia and short QT interval at birth, junctional bradycardia in infancy requiring single-chamber pacemaker, atrial fibrillation in adolescence, and V141M mutation in the KCNQ1 gene. Atrial fibrillation recurred and became unresponsive to electrical or anti-arrhythmic therapy. During 20 years of follow-up, a progressive evolution from sinus node dysfunction to low-rate atrial fibrillation was observed.

Takayasu arteritis is a chronic, idiopathic, granulomatous vasculitis involving the aorta, its major branches, and occasionally the pulmonary arteries. Although rare in children, it is the third most common vasculitis in the paediatric population. Although aneurysmal disease has been reported in adults with Takayasu arteritis, it is a rare entity in children. We present the case of a 10-year-old boy with a giant ascending and arch aneurysm that necessitated follow-up surgery for a new aneurysm and occlusive disease. This is also the first published case involving endovascular aortic graft placement for the management of vascular sequela of Takayasu arteritis in a child.

This case report documents the successful surgical repair of an aorto-bronchial fistula and a giant aortic pseudoaneurysm at the proximal anastomosis of a dacron interposition tube graft that was balloon dilated for recurrent coarctation. Balloon dilation for recoarctation of a dacron interposition tube graft may lead to serious complications.

Mitral atresia is commonly seen as a part of the spectrum of hypoplastic left heart syndrome, and it is usually associated with multiple levels of systemic outflow tract obstruction. Isolated mitral atresia with a normal aortic valve is extremely rare. We report the rare combination of mitral atresia, transposition of the great arteries, and unobstructed systemic and pulmonary blood flow.

Fatal arrhythmias in asymptomatic Kawasaki disease patients with normal left ventricular function have rarely been reported. In this study, we report the cases of two adult patients with largely unpredictable sudden cardiac arrest, despite almost-normal left ventricular function even after the diagnosis of presumed Kawasaki disease, as well as consider the mechanisms involved with reference to the literature.

Despite several reports describing echocardiography for the guidance of ductal closure, two-dimensional angiography remains the mainstay imaging tool; three-dimensional rotational angiography has the potential to overcome some of the drawbacks of standard angiography, and reconstructed image overlay provides reliable guidance for device placement. We describe arterial duct closure solely from venous approach guided by live three-dimensional image overlay.

We present the case of a 15-year-old boy who underwent arterial switch operation due to transposition of the great arteries with severe scoliosis, obstruction of the right coronary ostium, and severe stenosis of the pulmonary trunk. Balloon angioplasty caused a large aortopulmonary shunt provoking myocardial ischaemia and pulmonary hypertension. The traumatic “aortopulmonary window” was percutaneously occluded using an Amplatzer Septal Occluder device as a bridge to surgical repair.

In this study, we present the case of an extremely low birth weight infant with severe coarctation of the aorta after ductus ligation. We treated the patient with balloon angioplasty using the descending aorta as a new access route. This method reduced many complications typically observed during the intervention for extremely low birth weight infants, which may expand the available treatment options for extremely low birth weight infants.

Congenital anomalies of the pulmonary veins are rare. Meandering right pulmonary vein, considered a part of the Scimitar syndrome spectrum, is often an incidental finding during chest imaging. We present the case of a 4-year-old girl diagnosed with meandering pulmonary vein, who developed pulmonary hypertensive disease with an aggressive course, in spite of absence of hypoxia or elevated pulmonary wedge pressure.

We report a case of aortic pseudoaneurysm associated with a fractured bare Cheatham-Platinum stent following stenting for aortic coarctation. These complications were recognised 6 years after the implantation procedure and were successfully managed by percutaneous stent graft implantation. Staged approach for stent dilatation might prevent development of aortic pseudoaneurysms. In addition, careful follow-up is warranted after stenting for aortic coarctation, particularly in patients with recognised aortic wall injury.