A 16-year-old patient underwent successful ablation of ventricular arrhythmia originating from the aortic sinus of Valsalva following surgical unroofing of an anomalous right coronary artery. This case illustrates the complexity of decision making in the management of patients with anomalous coronary arteries and the importance of keeping an open mind when determining ventricular arrhythmia aetiology and origin.

The Fontan connection, originally described in 1971, is used to provide palliation for patients with many forms of CHDs that cannot support a biventricular circulation. An increasing number of females who have undergone these connections in childhood are now surviving into adulthood and some are becoming pregnant. We report a case of a 29-year-old woman who presented with a twin pregnancy at 33 weeks of gestation. She had significant deterioration of her cardiovascular status before the twin babies were delivered by emergency caesarean section owing to associated obstetric complications. This report also highlights the various maternal and fetal complications occurring in pregnancy of Fontan-palliated patients and suggests the need for meticulous pre-conception counselling and strict perinatal care.

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.

Transcatheter closure of atrial septal defects has become more common because of its high success rate and low morbidity; however, this treatment for patients with atrial septal aneurysms is still challenging.

Aortico-left ventricular tunnel and left ventricular non-compaction are rare congenital cardiac anomalies with varied clinical presentations and sparsely described co-existence in the medical literature. Owing to the limited information about these diagnoses in tandem, we present four cases of aortico-left ventricular tunnel and left ventricular non-compaction from our institution and discuss the clinical presentation, management, and follow-up.

Neonates and small infants have unique characteristics that make it possible to obtain echocardiographic views that are inaccessible in older patients. A high transsternal approach through the cartilaginous sternum and the thymus gland allows visualisation of a short-axis view of the pulmonary valve. This view should be included as part of routine protocols for echocardiographic examinations performed in this age group.

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.

A 6-month-old infant with Shone’s complex was found to have left main coronary artery atresia during evaluation for recurrent subaortic stenosis with depressed left ventricular function. The ventricular function improved after surgical subaortic resection without coronary re-vascularisation. This case demonstrates first the rare finding of left main coronary artery atresia and second that coronary re-vascularisation is not necessarily required in all cases of left main coronary artery atresia.

This case report describes chylous ascites associated with a CHD in a 4-month-old infant. Although atraumatic chylous ascites are a rare clinical finding, the recognition and treatment of chylous ascites influence the timing of cardiac surgery.

Hypertrophic cardiomyopathy associated with Friedreich’s ataxia is progressive, and there are few, if any, effective treatments available at present. This case report describes a Friedreich’s ataxia patient who had a septal myectomy for the management of hypertrophic cardiomyopathy with improved symptoms over a 7-year period. This suggests that septal myectomy may be a viable option to relieve symptoms and interrupt progression of heart disease in appropriately selected Friedreich’s ataxia patients.

Interrupted aortic arch is a very rare but well-described congenital anomaly. Concomitant presentation of interrupted aortic arch and giant subclavian artery aneurysm is an unusual event in adulthood. To the best of our knowledge, hybrid intervention for type A aortic arch interruption with a giant aneurysm of the subclavian artery is an alternative approach for the management of these concomitant pathologies in adults.

We have re-investigated an unusual cardiac specimen with juxtaposition of the atrial appendages. The original description dates to 1962, when the autopsy was performed at the Children’s Memorial Hospital in Chicago, now Ann & Robert H. Lurie Children’s Hospital of Chicago. The heart was subsequently stored in the Farouk S. Idriss Cardiac Registry at the same institution. The specimen shows usual atrial arrangement, but with the morphologically left appendage juxtaposed in a rightward manner, passing behind the heart rather than through the transverse sinus so as to reach its location inferior to the morphologically right appendage. The heart also demonstrated an inter-atrial communication between the cavities of the juxtaposed left appendage and the morphologically right atrium. We provide a detailed description of the morphology, and provide images of this lesion, which to the best of our knowledge has not previously been described.

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.

We used three-dimensional printing technology to create an anatomical three-dimensional model of a very rare and complex cyanotic CHD in a newborn, consisting of double-outlet left ventricle, ventricular septal defect, and pulmonary stenosis. This case demonstrates how this new innovative technology allows better understanding of the anatomy in complex CHDs and permits to better plan the surgical repair.

This report describes a rare case of left main coronary artery occlusion caused by embolisation of an intra-cardiac tumour in an 11-year-old patient. The patient presented with severe chest pain with ST-segment depression in the inferolateral leads with evidence of decreased left ventricular function, anterior-apical hypokinesis, and a pedunculated mass attached to the mitral valve. An urgent cardiac catheterisation was performed, which revealed near-complete obstruction of the left anterior descending coronary artery. Transcatheter aspiration of the embolic material was successfully performed. The final pathological examination revealed an inflammatory myofibroblastic tumour, which is a rare benign tumour, characterised by the proliferation of myofibroblasts and inflammatory cell infiltration.

Coronary fistula is defined as an anomalous connection between a coronary artery and any of the four chambers of the heart or any of its great vessels. A coronary fistula connecting the left main coronary artery to the right atrium is the most uncommon. In the present study, we report the surgical management of a very uncommon case of an aneurysm-like fistula connecting the left main coronary artery to the right atrium in a 2-year-old boy.

A 14-year-old boy presented to us with a diagnosis of severe asthma and oxygen desaturation of 76% on a 6-minute-walk test. A contrast echocardiogram revealed echocontrast in the left and right atria simultaneously. A secundum atrial septal defect and partial cor triatriatum dexter were diagnosed, and the atrial defect was closed by cardiac catheterisation.

Intercostal aneurysms are associated with aortic coarctation. Their aetiology is not well-understood but may be related to intrinsic vascular pathology and altered flow dynamics through the intercostal artery. We present the cases of two patients with coarctation and intercostal aneurysms. The aneurysms were recognised on pre-catheterisation imaging studies and were selectively occluded during the same procedure to treat the coarctation. There were no complications; both the patients have no residual coarctation at the most recent follow-up. Intercostal aneurysms associated with coarctation can have significant consequences including late rupture, paralysis, and even death. These aneurysms are common with an incidence of up to 40% with adult-diagnosed coarctation; one treatment plan is to treat both the coarctation and aneurysm during a single catheterisation. Pre-catheterisation CT or MRI may play a role in this strategy.