An 18-month-old male with pulmonary atresia and ventricular septal defect presented with stridor after neonatal systemic-to-pulmonary artery shunt surgery, that persisted on follow-up. CT angiography revealed a vascular ring with balanced double aortic arch.

We report a 5-month-old infant who developed an unexpected acute ischaemia of the right lower limb following a surgical perimembranous interventricular communication closure. This rare case of ischaemia was due to an occlusive right common iliac artery dissection. It was early managed by angioplasty with two ABSORB® bioresorbable stents, resulted in complete revascularisation of the right leg.

We report a case of a right ventricular outpouching diagnosed during prenatal period. We defined it as an aneurysm because of its thin, hypokinetic wall, and wide neck connecting to the ventricle. Ventricular aneurysms, especially right ventricular aneurysms, are very rare cardiac malformations. Therefore, we describe a rare case of right ventricular aneurysm and the diagnostic features, differential diagnosis, and clinical features of ventricular aneurysms.

Anomalous origin of the left coronary artery from the pulmonary artery is an exceedingly rare and potentially fatal congenital coronary anomaly that typically presents early in infancy. We report an unusual case of anomalous origin of the left coronary artery from the pulmonary artery in a 4-year-old child who presented later in life with vague respiratory and gastrointestinal symptoms and was found to have severe global cardiac dysfunction with evidence myocardial ischaemia.

We report the first successful Glenn procedure for pulmonary atresia with intact ventricular septum in the setting of D-transposition of the great arteries. This case was complicated by hypoplastic left ventricle and severe mitral regurgitation. Partial closure of the mitral valve at the time of Blalock-Taussig shunting resulted in improvement of heart failure.

Although larger stent placement could be effective for pulmonary vein stenosis, stents extrusion tends to occur due to caliber narrowing, small landing area, and the slippery nature. We placed stents with diameter ≧8 mm for four stenotic lesions using the “half-uncovered technique”. All stents were precisely placed and successfully resolved the stenosis. This technique allows us to avoid extrusion and to perform safe and effective dilatation when placing larger stents for pulmonary vein stenosis.

Pulmonary atresia with ventricular septal defect is a complex congenital cardiac anomaly. The blood is supplied to the lungs through a patent ductus arteriosus, a major aortopulmonary collateral artery, or in very rare cases from a coronary artery–pulmonary artery fistula. We present two cases with coronary artery–pulmonary artery fistula which underwent surgical intervention. In our first patient, the main pulmonary artery was supplied from the left main coronary artery. In the second patient, the right pulmonary artery originated from the left main coronary artery and continued to the right lung posteriorly to the aorta, while the left pulmonary artery originated from the patent ductus arteriosus. The difference in our cases is that the coronary artery pulmonary artery fistulas behave like major aortopulmonary collateral arteries originating from the coronary arteries. These fistulas were the main source of pulmonary blood flow.

Patients with erythrokeratodermia cardiomyopathy syndrome exhibit congenital, generalised erythrokeratoderma and dilated cardiomyopathy during early childhood. We report a case of erythrokeratodermia cardiomyopathy syndrome in a 15-year-old male patient and focus this report on cardiac features that were present.

We present the case of 28-year-old woman with a history of complex congenital cardiac surgery who developed cardiovascular collapse with reperfusion pulmonary oedema and right ventricular failure after surgical replacement of a severely stenosed right ventricle to pulmonary artery conduit. She required two separate episodes of support with extracorporeal membrane oxygenation and is alive and well 6 months after her initial cardiorespiratory crisis. We believe that consideration of a second period of support with extracorporeal membrane oxygenation is appropriate for select adults with CHD, provided they have a potentially reversible cause of postoperative cardiorespiratory collapse.

Our case provides several important lessons: (1) adults with CHD with severe postcardiotomy cardiorespiratory failure may potentially be salvaged even if they require multiple runs of extracorporeal membrane oxygenation; (2) adults with CHD with severe postcardiotomy respiratory failure with adequate cardiac function may potentially be salvaged with veno-venous extracorporeal membrane oxygenation; and (3) patients supported with extracorporeal membrane oxygenation will benefit from care from a skilled multidisciplinary team who are able to focus on the support of the function of the organs of the patient whilst providing nutrition and mobilisation.

Combined tricuspid atresia and absent pulmonary valve with dysplasia of the right ventricular myocardium is a very rare congenital heart anomaly with a poor prognosis. We present three cases of this rare disease that reached the Fontan operation without prior surgical intervention of the right ventricle or pulmonary artery; no right ventriclar dilation was detected. All patients had uneventful post-operative courses.

We report a neonate who presented with spontaneous thrombus formation in the native aortic root after Norwood palliation for hypoplastic left heart syndrome. Thrombus formation led to myocardial ischaemia due to decreased coronary blood flow with electrocardiographic signs of ischaemia and elevated cardiac enzymes. Aggressive thrombolysis and anticoagulation therapy were major contributors to successful outcome.

A foetal echocardiogram, in a 27-week foetus referred for cardiomegaly, demonstrated dextrocardia, absence of the ductus venosus, and an unrestricted unusual umbilical venous drainage to a left posterior intercostal vein, which continued to left hemiazygos vein and drained into the coronary sinus. Progressive cardiomegaly led to early delivery. To the best of our knowledge, no case with similar umbilical venous drainage has been previously reported.

Persistent pulmonary hypertension of the neonate can cause acute and life-threatening hypoxia, but preterm neonates are not suitable candidate to extra-corporeal life support. We report the unique case of an extremely preterm neonates with life-threatening persistent pulmonary hypertension refractory to triple vasodilator therapy (nitric oxide, iloprost, and bosentan), which has been successfully treated with the addition of adenosine continuous infusion.

A newborn girl was referred for a suspected abnormal pulmonary venous return in coronary sinus. Echocardiogram revealed a large outpouching of the left ventricle creating an accessory cardiac chamber that was connected to the left ventricle behind the mural leaflet of the mitral valve, and with the right atrium via the coronary sinus. Ten years later, the orifice between the submitral aneurysm and the left ventricle was closed percutaneously.