We present a rare case of scimitar syndrome in which the scimitar vessel, collecting all the right pulmonary veins, was stenotic at its junction, with the inferior caval vein and two anomalous vessels, connecting to the same venous collector, draining most of the flow to the left atrium.

We arbitrarily defined this rare anatomical variant as a congenitally palliated scimitar syndrome.

Oesophageal perforation is a rarely reported complication of transoesophageal echocardiography in infants. This case involves a 3.1-kg neonate with Trisomy 21, atrioventricular septal defect, and hypoplastic aortic arch undergoing aortic arch advancement and pulmonary artery banding. A paediatric transoesophageal echocardiography probe was placed intraoperatively causing a contained false passage from the oesophagus below the cricopharyngeus muscle with extension into the left posterior mediastinum. The perforation healed within 2 weeks without permanent sequelae after conservative medical management.

We report a case of pulmonary atresia with intact ventricular septum, but in the setting of transposed great arteries, and thus the left rather than the right ventricle was hypoplastic.

Major and minor coronary artery anomalies in tetralogy of Fallot is a well-described finding. The importance of determining the coronary distribution impacts upon the decision making for surgery and subsequent management. Traditionally, the coronary distribution is relied classically on echocardiography and cardiac catheterisation; however, they have well-known limitations. The use of CT as a first-line investigation modality for coronary artery distribution is discussed.

Percutaneous treatment of aortic coarctation is a widely used option. Covered stents have increased the profile of efficacy and safety of this procedure. Here we report on a 32-year-old woman with significant aortic recoarctation associated with aortic wall aneurysm and close proximity of both lesions to the origin of both the subclavian arteries.

It was decided to manually and partially uncover the proximal part of the stent to have a hybrid stent that could act as a bare stent at the level of the origin of the subclavian arteries and as a covered stent at the level of the aneurysm.

Background: Children with pulmonary hypertension routinely undergo pulmonary vascular resistance studies to assess the disease severity and vasodilator responsiveness. It is vital that results are accurate and reliable and are not influenced by the choice of anaesthetic agent. However, there are anecdotal data to suggest that propofol and inhalational agents have different effects on pulmonary vascular resistance. Methods: A total of 10 children with pulmonary hypertension were selected sequentially to be included in the study. To avoid confounding because of baseline anatomic or demographic details, a crossover protocol was implemented, using propofol or isoflurane, with time for washout in between each agent and blinding of the interventionalist. Results: Pulmonary and systemic vascular resistance were not significantly different when using propofol or isoflurane. However, the calculated resistance fraction – ratio of pulmonary resistance to systemic resistance – was significantly lower when using propofol than when using isoflurane. Conclusions: Although no difference in pulmonary vascular resistance was demonstrated, this pilot study suggests that the choice of anaesthetic agent may affect the calculation of relative pulmonary and systemic vascular resistance, and provides some preliminary evidence to favour propofol over isoflurane. These findings require replication in a larger study, and thus they should be considered in future calculations to make informed decisions about the management of children with pulmonary hypertension.

We describe a case of an 11-year-old boy who underwent orthotopic heart transplant for dilated cardiomyopathy. He developed a normocytic, normochromic anaemia with a low reticulocyte count 1 month after transplant. A bone marrow biopsy was performed, which showed a mildly hypocellular bone marrow with few red blood cell precursors with giant pro-erythroblasts indicative of a pure red cell aplasia. Parvovirus B19 polymerase chain reaction in the blood was positive 2 months after transplant. Intravenous immunoglobulin administration resulted in a resolution of the anaemia over several months. Unexplained pure red cell aplasia in immunosuppressed patients should alert one to the possibility of parvovirus B19 infection.

We present the case of an infant with three distinct outflow tracts from the right ventricle. Three outlets from the heart have been previously named the “Tritruncal Heart”. We review the two previously reported cases of tritruncal hearts and describe the anatomy, diagnosis, surgical management, and outcome of our case. Embryologic implications are also discussed.

We present a rare congenital heart disease in a 20-year-old man with anomalous origin of the right pulmonary artery from the ascending aorta, accompanied by absent pulmonary valve syndrome, and a right-sided aortic arch suspected initially in transthoracic echocardiography and subsequently confirmed by cardiac catheterisation and computed tomography angiography.

Acute heart failure related to anaphylactic shock is often reversible and necessitates aggressive support to ensure full recovery. We report the case of a 15-year-old boy who developed severe ventricular dysfunction and haemodynamic instability after administration of amiodarone and required temporary mechanical circulatory support with a left ventricular assist device. He had full recovery of cardiac function and returned to baseline neurologic status. This is the first report of successful left ventricular assist device use for recovery from cardiovascular collapse due to anaphylaxis.

The Medtronic LINQ™ was inserted in an 11-month-old boy for close monitoring of rapid ventricular tachycardia. The device is much smaller than the conventional Medtronic loop recorder. The real advantage of the LINQ™ is that it automatically notifies Carelink if any ventricular tachycardia is seen, which is very advantageous for this particular type of patient. This device is ideal for close monitoring of asymptomatic yet potentially dangerous arrhythmias in smaller children.

We report a baby girl with an antenatal diagnosis of biventricular non-compaction and complete heart block detected at 22 weeks’ gestation. Postnatal echocardiography confirmed severe biventricular non-compaction hypertrophic cardiomyopathy, multiple muscular ventricular septal defects, and mild–moderate pulmonary valve stenosis. Skeletal muscle biopsy confirmed complex 1 mitochondrial respiratory chain deficiency. An epicardial VVI pacemaker was implanted on day 3 of life and revised at 7 years of age. She remains stable at 8 years of age following pacing and medical treatment with carvedilol, aspirin, co-enzyme Q10, and carnitine. This represents the first report of biventricular non-compaction hypertrophic phenotype in association with congenital complete heart block and complex 1 mitochondrial respiratory chain deficiency in a child.

We recently diagnosed two cases of isolated unilateral absence of the pulmonary artery just after birth. Through the therapy, we could not prevent obstruction of the pulmonary artery and lead to complications. There have been no symptoms attributed to isolated unilateral absence of the pulmonary artery so far. We should carefully consider the strategy for therapeutic intervention for asymptomatic cases.

We describe a case of a baby girl born with hypoplastic left heart syndrome consisting of mitral atresia, aortic atresia, hypoplastic ascending aorta, and left ventricle. The pulmonary arteries were hypoplastic, measuring 3 mm. Fluorescence in situ hybridisation analysis demonstrated a microduplication of chromosome 22q11.2. Subsequent array comparative genomic hybridisation showed a gain of 2.3 Mb in one copy of chromosome 22q at band 22q11.21. The proband underwent a successful Norwood procedure with Sano shunt and subsequently underwent bi-directional Glenn shunt and Fontan procedure. This report highlights the association between hypoplastic left heart syndrome with hypoplastic pulmonary arteries and chromosome 22q11.21 microduplication.

Type 1 long QT syndrome is the most common long QT syndrome genetic subtype. Exercise and emotional stress can precipitate sudden cardiac events in patients with type 1 long QT syndrome; however, the precise mechanism remains elusive. We report the case of a teenage girl with type 1 long QT syndrome secondary to a rare frameshift mutation (p. L191fs+90X) in the KCNQ1-encoded Kv7.1 potassium channel. During emotional distress, her continuous QTc recordings precipitously increased, peaking within minutes to 669 ms and then returning to baseline (520 ms) as she calmed without concomitant increase in heart rate. This is the first described case documenting transient, marked accentuation of the QTc interval in a long QT syndrome patient during emotional distress. Such events may be triggered by transient accentuation of the intrinsic perturbation in cardiac repolarisation and increase the risk of degeneration to a ventricular arrhythmia. This case illustrates the need improved understanding of the complex interaction between emotion and cardiac stability in patients with long QT syndrome.

Primary vascular tumour of the heart is rare, especially in neonates and infants. We report a male premature newborn with a right atrial tumour associated with a large amount of pericardial effusion detected by screening foetal echography. Diagnosis of capillary haemangioma was confirmed by histopathological examination after complete surgical resection. Other vascular tumours in the neonates and infants reported in the English literatures are reviewed, and one algorithm for both prenatal and postnatal management is proposed.

May–Thurner syndrome is a rare clinical entity involving venous obstruction of the left lower extremity. The May–Thurner syndrome is a phenomenon commonly described as an acquired stenosis of the left common iliac vein secondary to compression of the left common iliac vein between the right common iliac artery and the underlying vertebral body. We report one case of May–Thurner syndrome, and another rare case of reverse May–Thurner syndrome, incidently detected during intervention, in a case of aortic stenosis and mitral stenosis with dextrocardia and situs inversus.

Uhl’s anomaly is an evolutive disease leading to terminal right ventricular failure. The most difficult differential diagnosis at presentation is the Ebstein disease. We describe the evolution of a foetus with Uhl’s anomaly from 21 to 30 weeks of gestation, with progressive reduction in the right ventricular anterior myocardium suggestive of apoptosis, leading to foetal demise.