Arterial tortuosity syndrome (ATS) is a rare, autosomal recessive, connective tissue disorder. It predominantly involves the arterial tree with clinical features reflecting the systems involved. There have been few cases of ATS suspected during antenatal screening ultrasound in high-risk families, but none confirmed. We present the first case of ATS confirmed antenatally in the fetus with cascade testing, detecting the disease in the mother as well.

Ductus arteriosus is an essential component of fetal circulation. Due to occurring changes in the cardiopulmonary system physiology after birth, ductus arteriosus closes. Patent ductus arteriosus can be closed by medical or invasive (percutaneous or surgical) treatment methods. Percutaneous or surgical closure of patent ductus arteriosus can be performed for the cases that medical closure failed. Surgical treatment is often preferred method for closure of patent ductus arteriosus in the neonatal period. The most common surgical complications are pneumothorax, recurrent laryngeal nerve injury, bleeding, and recanalisation. A very rare surgical complication is left pulmonary artery ligation that has been presented in a few cases in the literature. Echocardiography control should be performed in the early post-operative period, especially in patients with clinical suspicion. If reoperation is required, it should never be delayed. We report a newborn patient whose left pulmonary artery ligated accidentally during patent ductus arteriosus closure surgery and surgical correction of this complication at the early post-operative period.

We report a case of a 2-month-old infant with incomplete Kawasaki disease with multiple coronary and systemic arteries aneurysms complicated with internal iliac arteries thrombosis. The atypical clinical presentations and severity of systemic vascular involvements discuss the importance of high index of suspicions in younger infants and treatment options in such cases.

We report a case of Figulla-II Occlutech septal occluder malposition with residual shunt at posteriosuperior margin of an atrial septal defect. Improvising its bioptome type delivery cable, same system was used to recapture the device and redeploy it successfully. This report highlights a potential malfunction of Figulla-II Occlutech disc and the advantage of its delivery system for retrieval of the device.

Isolated atrial defects usually lead to left-to-right shunt and right ventricular volume load. Descriptions of cyanosis with this congenital heart defect are rare.

We describe a rare case of inferior caval vein flow directed through an atrial septal defect in the fossa ovalis leading to severe cyanosis, but without any additional intracardiac anatomical abnormalities. The baby with clinical features of Marfan’s syndrome had an eventration of the right-sided diaphragm. Surgical closure of the defect resolved the cyanosis, but the child died 10 weeks later of severe valvar dysfunction, related to Marfan’s syndrome.

Mechanisms of cyanosis in patients with atrial septal defects are discussed. Echocardiographic bubble studies both from the lower and upper half of the body may help to clarify the mechanism of an otherwise unexplained cyanosis.

Anomalous origin of one pulmonary artery from the ascending aorta is a rare cardiac anomaly in which the pulmonary artery abnormally arises from the ascending aorta. Physiologically, most patients develop signs of cardiac failure due to high flow to both lungs. The purpose of this study is to demonstrate, with this rare anomaly, the accurate place of the echocardiography to establish diagnosis especially in the systemic or supra-systemic pulmonary hypertension.

Isolated systemic arterial supply to a normal lung, a type of bronchopulmonary vascular malformation, is a rare cause of extracardiac left-to-right shunt. We describe such a case that was successfully managed by transcatheter closure of the anomalous arterial supply to otherwise normal lung.

A 14 -year-old boy presented with chest pain and breathlessness. Echocardiography showed a large pericardial effusion with cardiac tamponade features and suspicion of cardiac mass. Cardiovascular magnetic resonance demonstrated a large, well-defined pericardial mass, suggesting atypical large coronary fistula with pericardial haematoma or primary cardiac/pericardial tumour such as angiosarcoma. Histology confirmed a mixed-type vascular malformation. Sirolimus therapy was initiated.

In young patients with unexplained ventricular fibrillation, coronary occlusion may be missed by echocardiogram and misinterpreted by CT. We report two patients presenting with ventricular fibrillation and initially negative workup, later identified to have occlusion of left main coronary artery. We demonstrate the importance of angiography to rule out coronary occlusion in patients with unexplained ventricular fibrillation.

We present a 31 gestational weeks’ premature baby whose fetal echocardiogram showed ectopia cordis, single ventricle and severe pulmonary stenosis. At 31 gestational weeks, an emergency caesarean section was performed, and his birth weight was 1756 g, SpO2 was 80% on 100% O2. Epicardial echocardiogram showed double inlet right ventricle, severe valvular pulmonary stenosis and no ductus arteriosus. The risk of surgery was very high, so we decided to perform balloon valvuloplasty by direct puncture of the heart. We punctured the apex of the ventricle using a 16-gauge needle under echo guidance, advanced the guidewire to the pulmonary artery and performed balloon valvuloplasty. Soon after the procedure, the cyanosis improved dramatically. This is the first report of a transcatheter procedure performed by direct puncture of the heart for ectopia cordis with complex congenital heart disease.

We report the case of a 2-year-old girl who developed catastrophic haemoptysis due to an arterio-bronchial fistula after transcatheter balloon dilatation for a narrowing aortopulmonary shunt. We embolised the fistula while haemoptysis was controlled with the left bronchial block ventilation and haemostatic balloon occlusion of the left subclavian artery. An arterio-bronchial fistula is an extremely rare complication for balloon dilatation of an aortopulmonary shunt.

Rhabdomyomas are the most common paediatric cardiac tumours. The natural history of these tumours is mostly benign, and the tumour usually regresses spontaneously. Although surgical resection of these tumours is one of the considerations in patients with ventricular outflow obstruction, a palliation with Blalock–Taussig shunt is an alternative approach with the hope of regression of the tumour over time. We report a case of prenatally diagnosed rhabdomyomas in the right ventricle and its outflow presenting as hemodynamic simulating hypoplastic right ventricle in a newborn. She required prostaglandin and Blalock–Taussig shunts palliation for pulmonary flow and subsequent regression of tumours.