Alveolar capillary dysplasia with misalignment of the pulmonary veins is an uncommon disorder that affects the lung vasculature development in the neonatal period and leads to pulmonary hypertension. We describe two patients with alveolar capillary dysplasia associated with left-sided obstructive heart defects with two different genetic variants. Our cases highlight the importance of early recognition of this disease in the setting of persistent and supra-systemic pulmonary hypertension despite surgical correction of the associated lesions. Identification of these cases will facilitate the development of a multidisciplinary approach and provide guidance to the affected families.

Atrial fibrillation is one of the most common arrhythmias in the adult population, in children, however, only a few case reports of isolated atrial fibrillation exist.1 Aside from post-operative AF due to atrial scar formation or suture lines and atrial enlargement in CHD (e.g., mitral stenosis), alcohol consumption represents a rare cause of AF in adolescents. We report on the latter.

Sialidosis, a rare autosomal recessive disorder, is caused by a deficiency of NEU1 encoded enzyme alpha-N-acetyl neuraminidase. We report a premature male with neonatal-onset type II sialidosis which was associated with left ventricular dysfunction. The clinical presentation and subsequent progression which culminated in his untimely death at 16 months of age are succinctly described. Early-onset cardiovascular involvement as noted in this patient is not well characterised. The case report is supplemented by a comprehensive review of the determinants, characteristics, and the clinical course of cardiovascular involvement in this rare condition.

Dilated cardiomyopathy is characterised by dilatation and impaired contraction of the left ventricle or both ventricles, which is the most common childhood cardiomyopathy. In recent years, it has been recognised that many sorts of genetic mutations may contribute to dilated cardiomyopathy. We now report a rare association of dilated cardiomyopathy with site mutation of BMPR2 gene. We did not find such an association reported in the medical literature.

The essence of so-called heterotaxy is the potential disharmony between the arrangement of the bronchuses, abdominal organs, and the atrial appendages. Accurate description of the heart, however, can only be provided by specific description of these features, all of which are readily discernible in the clinical setting. We argue that, when accurate description of the atrial and visceral arrangement is provided, along with appropriate description of the intracardiac findings, no further accuracy is gained by suggesting that an individual heart is “heterotaxic”.

Kawasaki disease is an acute systemic vascular disease, generally self-limited and typically affecting children <5 years old, which leads to coronary artery aneurysms in about 25% of untreated cases. Cardiovascular involvement is characterised by transient pancarditis, in acute phase, while coronary illness, ranging from mild dilation to giant CAAs occurs late, rarely before the 10th day since fever onset. Here, we describe a peculiar case of KD, which occurred in a 4-month-old infant and presented with exudate cardiac tamponade and early giant aneurism of both the proximal right coronary artery) and the left circumflex coronary artery, in acute phase of the disease.

In a cross-sectional study, we assessed family functioning prior to outpatient visits and routine heart catheterisations in the families of paediatric heart transplant recipients. Caregivers rated higher short-term family functioning prior to outpatient visits, but not prior to catheterisations. This finding may indicate that family functioning benefits from the support provided during outpatient visits.

We describe an infant with duct-dependent hypoplastic left heart complex with moderate hypoplasia of the left ventricle and aortic arch who was not operated due to resource limitations. The left-sided structures grew remarkably due to favourable loading condition changes of the left ventricle, allowing weaning from prostaglandin at the age of 3 months and discharging the patient without intervention.

A one-and-a-half-month-old patient with isolated unilateral absence of the right proximal pulmonary artery with prominent left pulmonary hypertension was diagnosed using CT. Medication therapy was initiated first. Left pulmonary artery pressure decreased after the initiation of medication therapy, and single-stage reconstruction of the right pulmonary artery was performed. The patient is asymptomatic 14 months postoperatively.

Pulmonary aspergillosis associated with cyanotic congenital heart disease is a rare condition, which is known to have a poor prognosis. We report a case of a 21-year-old woman with truncus arteriosus and major aortopulmonary collateral arteries who underwent primary Rastelli procedure after thoracoscopic lobectomy for the management of progressive pulmonary aspergillosis.

We report a case of a pseudoaneurysm in the sinus of Valsalva, secondary to infective endocarditis in a child with trisomy 21. The patient had a history of subaortic stenosis, bicuspid aortic valve, and ventricular septal defect. Patch closure of the ostium of the pseudoaneurysm and aortic valve replacement was performed. The patient was discharged without severe complications.

There has been an increase in the use of extracorporeal membrane oxygenation for severe neonatal cardiac failure. However, the frequency of complications is high, particularly in preterm and low-birth-weight neonates. Herein, we present combination treatment with transcatheter balloon atrioseptostomy and bilateral pulmonary artery banding in a collapsed preterm neonate. This strategy can be an alternative to circulatory support using extracorporeal membrane oxygenation.

Transcatheter device closure of an atrial septal defect (ASD) may require an additional supportive technique in challenging cases. We report a 15-year-old male with moderate-sized ASD and severe scoliosis. In spite of adequate positioning of the Gore Cardioform ASD occluder, the device was pulled into the right atrium by a retrieval cord due to the acute release of strong tension between the delivery catheter and its device upon locking. This phenomenon was prevented by the use of Mullins sheath, resulting in a successful release of the device. The use of a Mullins sheath may be considered to deliver a Gore Cardioform ASD device in selected cases.

Transcatheter creation of an interatrial communication using the Occlutech Atrial Flow Regulator Device for pulmonary hypertension or heart failure is well described. We report a case of an 8-year-old boy with a failing Fontan circulation, in whom the Atrial Flow Regulator was used to successfully create a fenestration between the pulmonary artery and left atrium, improving his clinical condition.