We report on a 6-month-old infant (6 Kg/ 64 cm) with perimembranous ventricular septal defect (absent sub-aortic rim, 10 mm left ventricular entry, and 4 and 6 mm right ventricular exists) and successful retrograde closure using an 8x6 mm KONAR-MF™ VSD occluder (Lifetech, China). Immediate and 48 hours post-procedure ultrasounds showed an accurately positioned device and two jets of mild-to-moderate residual shunts. At the 2-week follow-up, the device did not change position and the shunt was stable and intra-prosthetic. The scheduled 3-month follow-up was skipped for familial reasons. The patient came back without alarming symptoms for the regular 6-month follow-up, and the device was found embolised to the left pulmonary artery. The device was retrieved surgically, and the defect was patch-closed with excellent outcomes. There was a pseudoaneurysm involving the tricuspid valve chordae and the device was endothelialized partially on one edge suggesting that embolization occurred somewhere between 3 months and 6 months post-operative. Defects with compromised anatomies should be closed surgically to avoid suboptimal results, especially in small infants.

Isolated left-sided innominate artery, a rare congenital anomaly in which the left-sided innominate artery arises from the main pulmonary trunk, is usually diagnosed incidentally in children and adults. Limited reports exist on its prenatal diagnosis, with none comprehensively describing the associated perinatal haemodynamic changes. We report a case of prenatally diagnosed isolated left-sided innominate artery, describing the postnatal clinical course.

Giant coronary aneurysm with ventricular fistula is rare. Due to the limited data from randomised clinical trials, therapeutic strategies for coronary aneurysms predominantly rely on on case series and anecdotal evidences. Reporting cases that provide practical experience in managing these aneurysms is therefore crucial. In this article, we report a rare case of a successful surgical management for a thrombosed giant left coronary aneurysm with right ventricular fistula, which is larger than any previously reported cases.

We report a case of dilated cardiomyopathy-like hypertensive cardiomyopathy (HTN-CM) with polycystic kidney disease without family history when a 3-month-old boy developed bacteraemia secondary to a urinary tract infection. He was later confirmed as having autosomal recessive inheritance due to the proven PKHD1 gene mutation. The treatment consisted mainly of antihypertensive and anti-heart failure therapies and he was discharged on the 131st day. To prevent the development of heart failure in patients with HTN-CM due to autosomal recessive polycystic kidney disease (ARPKD), it is important to improve the fetal diagnosis rate of ARPKD, detect hypertension early, and strictly control the blood pressure after birth.

We report an uncommon case report of total anomalous pulmonary venous returns into the right atrium at the base of the superior caval vein’s ostium without a sinus venosus defect, in situs solitus, without vertical vein or a posterior pulmonary venous confluence.

We report a case of iatrogenic left pulmonary artery–left atrial appendage fistula following percutaneous transluminal angioplasty for residual pulmonary artery stenosis in an 11-year-old boy. This rare complication could have been predicted by understanding the anatomical relationship of these structures. In this study, simulation using three-dimensional printing greatly contributed to successful stent placement.

Myxomatous degeneration of one or more cardiac valves has been reported in trisomy 18, Noonan, Marfan, and Ehlers-Danlos syndromes. 6q25.1 (TAB2) deletion is one of the notable causes for myxomatous degeneration of cardiac valves. Whole exome sequencing must be considered in these subsets of cases for effective prenatal counselling. A 23-week fetus presented with cardiomegaly, redundant myxomatous tricuspid, mitral valve leaflets, thickened pulmonary valve, and bicuspid aortic valves detected to have 6q25.1 (TAB2) deletion was presented with literature review.

This is a case of a 13-year-old male with frequent premature ventricular contractions with QRS configurations of the left superior axis and left bundle branch block, which originated from the posterior–superior process of the left ventricle. Premature ventricular contractions were successfully eliminated by delivering radiofrequency energy to the inferior wall of the right atrium without causing either junctional rhythm or atrioventricular block. Ventricular arrhythmias originating from this site have been sporadically reported in adults; however, this is the first report in a child.

The atrial switch procedure by Senning or Mustard technique primarily aims in correcting parallel systemic and pulmonary circulations at atrial level. This procedure may be used in late presenting D-transposition of great arteries with a deconditioned left ventricle, congenitally corrected transposition of great arteries and isolated ventricular inversion. We describe the case of a child with dextrocardia, left atrial isomerism with complex pulmonary and systemic venous drainage resulting in mixing at atrial level. She was successfully operated by modified Senning procedure performed through the left-sided atrium.

We report the first-stage percutaneous palliation in a newborn with a rare heterotaxy syndrome variant including interrupted inferior vena cava, partial anomalous pulmonary venous drainage, and restrictive interatrial communication. Virtual reality imaging aided visualisation, decision-making, and planning. Successful atrial septoplasty performed via the internal jugular vein and anomalous pulmonary vein was followed by stenting of ductus arteriosus.

While infradiaphragmatic total anomalous pulmonary venous drainage to portal vein is well described, hemianomalous drainage of right pulmonary veins to portal vein in Scimitar syndrome has not yet been reported.

Submitral aneurysm is a rare type of non-ischaemic ventricular aneurysm. We describe the case of a 26-year-old woman with progressive dyspnoea for approximately 1 year who was diagnosed with a ruptured submitral aneurysm in the left atrium with severe regurgitation. The patient underwent aneurysm correction and mitral valve repair, and post-operative echocardiography showed no residual abnormalities. The patient remains asymptomatic.

Percutaneous pulmonary valve implantation is established as a safe and effective method of treating patients with disfunction of right ventricular outflow tract. Modifications of this method allow for an increasingly wider use of this less invasive treatment. We present a staged percutaneous pulmonary valve implantation into a single-branch pulmonary artery in a paediatric patient with tetralogy of Fallot after patch repair.

We report the case of a 9-year-old male with severe congenital pulmonary valve stenosis referred to our centre for percutaneous valvotomy. On admission, trans-thoracic echocardiogram confirmed a unicuspid pulmonary valve with a peak/mean pulmonary valve gradient of 91/53 mmHg and a pulmonary annulus of 13.8 mm (−0.8 Z Score). It also showed an enlarged RV (RV/LV ratio 0,9). During cardiac catheterisation, an additional atrial septal defect (secundum) with significant left to right shunt (Qp/Qs > 2) was diagnosed, which was not amenable to percutaneous closure. The patient was referred for surgical repair.

The atrial septal defect was closed by a direct running suture. The repair of the unicuspid valve consisted in bicuspidisation by a large commissurotomy to the left anterior wall of the pulmonary artery. The neo-commissure was created with two separate patches of autologous pericardium secured to the wall of the pulmonary root. The adjustment of the effective height of the pulmonary valve leaflets was done by trimming the patches and a triangular plication of the newly created posterior leaflet. Perioperative echocardiogram showed a peak gradient of 15 mm Hg and trivial pulmonary regurgitation. The total cross-clamp time was 92 min and the bypass time 123 min with a favourable evolution after the surgery.

The particularity of the case is represented by the complexity of the bicuspidisation procedure. Using this technique, a tailored approach is needed for every patient.

We present the case of a premature neonate with pericardial effusion secondary to extravasation of total parenteral nutrition from a mispositioned/migrated umbilical venous catheter. Emergency pericardiocentesis was complicated by an intrapericardial thrombus, which was managed conservatively with spontaneous resolution within 24 hours. This case illustrates that the rare complication of an intrapericardial thrombus after pericardiocentesis can be successfully managed conservatively with close monitoring in haemodynamically stable paediatric patients.

Danon disease is a rare X-linked disorder caused by deficiency of the lysosome-associated membrane protein-2. We report a case of hypertrophic obstructive cardiomyopathy secondary to a novel mutation in the lysosome-associated membrane protein-2 gene in a 10-year-old male adolescent. We performed a modified extended Morrow procedure to minimise the risk of death and improve the patient’s quality of life. The patient did not have exertional dyspnoea, and auscultation did not reveal a cardiac murmur at 1-year follow-up.

Prinzmetal angina is a rare condition more likely to be reported in adults. Vasospasm is by far notable for causing this condition. We present a case of Prinzmetal angina in a 17-year-old male who experienced unexplained resting chest pain for 2 days.

Atresia of the aortic valve is usually associated with hypoplasia of the mitral valve and the left ventricle. In very rare cases, a ventricular septal defect may be associated with aortic atresia, when left ventricle and mitral valve are normal-sized, due to the presence of an outflow for the left ventricle through the ventricular septal defect. We present the multi-modality imaging findings of an adolescent girl who presented with breathlessness and was later found to have aortic valvar atresia with a normal-sized left ventricle.