There are three cusps in a normally developed aortic valve. Abnormal excavation or fusion, during the embryological development of the aortic valve, results in a varying number of cusps. Bicuspid aortic valve is the most common, but more rarely, unicuspid and quadricuspid aortic valves can be seen.

Here, a case of a 16-year-old male with a unicommissural unicuspid aortic valve and a case of a 13-year-old female with a quadricuspid aortic valve were reported.

Isomerism, also referred to as “heterotaxy” is a complex set of anatomic and functional perturbations. One of the most obvious manifestations of isomerism is the disturbance of organ arrangement, such that the thoracic organs are no longer asymmetric on the left and right. We report the case of a 14-year-old female in whom exercise-induced dyspnea led to a late diagnosis of left isomerism complicated by Abernethy malformation and portopulmonary hypertension. A comprehensive evaluation revealed two anatomic left lungs and hyparterial bronchi, bilateral left atria, an interrupted inferior caval vein with azygos continuation, multiple spleens, sinus node dysfunction, hepatic hypertrophy with focal nodular hyperplasia, and absence of the portal vein. Pulmonary vasodilator therapy was initiated resulting in clinical improvement. This case exhibits unique features including a late diagnosis of isomerism with Abernethy malformation and portopulmonary hypertension. The patient’s presentation, medical workup, and future treatment emphasise the importance of multidisciplinary care in children with complex multisystem disease. We review the multiple cardiac and extracardiac manifestations of isomerism.

We report an innovative treatment strategy for fetal Ebstein’s anomaly with a circular shunt. We used transplacental non-steroidal anti-inflammatory drugs, at the 29th gestational week, to constrict the ductus arteriosus avoiding fetal demise. We addressed the critical neonate with an urgent Starnes procedure. Finally, instead of following the usual single-ventricle palliation pathway after the Starnes procedure, we achieved successful two-ventricle repair with the cone technique at 5 month old.

A young adult with late diagnosis of scimitar syndrome underwent infradiaphragmatic baffling of the scimitar vein to left atrium through an intra-atrial tunnel using PhotoFix® bovine pericardium with recurrent extensive fibrovascular granulation of the patch causing pulmonary and systemic venous obstruction leading to eventual explantation of the bovine pericardium.

On 15 October, 2020, Dr. Paul M. Weinberg, a true giant in the field of paediatric cardiology, succumbed to a prolonged illness. Dr. Weinberg had a 43-year career and was described as a pillar of The Children’s Hospital of Philadelphia and the spirit of the Division of Cardiology, a cherished and beloved teacher, and an outstanding clinician. His impact on the field and on the careers of his students will be remembered for generations to come.

In 2019, Dr. Weinberg wrote for Jefferson Medical School’s 50th year reunion memory book: “In the true spirit of Hippocrates, I seek to mentor the next generation as I was mentored by the last, without expectation of reward. I am forever indebted to these educators for all the knowledge they imparted to me and for the wisdom that I acquired under their tutelage.” These words are a true reflection of his unassuming dedication to teaching the next generation of paediatric cardiologists. His legacy will continue to live on through these trainees and impact the field for generations to come.

Anomalous origin of coronary artery originating from the pulmonary artery in conjunction with the aorticopulmonary window (APW) is a rare but a significant anomaly in the era of congenital cardiac diseases. The occurrence of anomalous origin of the right coronary artery from the pulmonary artery among the associated anomalies is less than 5%. The severity of the clinical condition of these patients depends on the degree of left–right shunt and compromise of the pulmonary blood flow. We report surgical management of a case of a 45-day-old infant with APW, ventricular septal defect, and anomalous origin of coronary artery originated from the pulmonary artery.

Hydatid cyst is a serious parasitic infection in endemic areas. Cardiac hydatid cyst is not a common presentation, and primary pericardial hydatid cyst is rare. Echocardiography, CT, and MRI are important in diagnosing and locating cardiac echinococcosis. Herein, we present the case of an asymptomatic butcher with primary pericardial hydatid cyst and its successful treatment.

Unroofed coronary sinus syndrome complicated by coronary sinus orifice atresia is a rare congenital anomaly. There are two alternate exits for coronary venous return: unroofed coronary sinus and persistent left superior caval vein. The coronary venous direction could be bidirectional depending on the pressure balance between the left atrium and the systemic vein. This anomaly has the risk of heart failure, paradoxical embolism, and cyanosis.

The incidence of paediatric venous thromboembolism has steadily increased in the past decade, by nearly 10% per year. Deep venous thrombosis may remain completely asymptomatic during the acute phase and symptoms may occur later, due to complications. We related the case of a 9-month-old child with increasing cyanosis. A computed tomography (CT) angiography showed a thrombosis of the superior vena cava (SVC) with the development of collateral flow from the systemic to the pulmonary veins. Transcatheter shunt occlusion after SVC recanalization was successfully performed. We discussed the characteristics of these cases and the consequence on our practice in term of treatment (anticoagulation, transcatheter, intervention) and screening.

Ductal arterial spasm is a very potentially dangerous incidence during percutaneous device closure of patent ductus arteriosus (PDA), which, otherwise, is a very safe catheter intervention. It is essential to notice its occurrence before device sizing and deploying. Without awareness, it can mislead device selection and can result in serious complication. In this report, we shared our nightmare of ductal spasm during transcatheter closure of PDA in two children which had led to death in one patient.

Truncus arteriosus is a rare cyanotic congenital heart defect that involves septation failure of the heart’s main arterial outflow tract. Varying morphologies of the truncal valve and aorta have been reported; however, the ascending aorta is typically supplied via anterograde blood flow through the truncal valve. We present the first reported case of neonatal truncus arteriosus with the ascending aorta being supplied entirely by retrograde flow.

Known as a benign arrhythmia and normally requiring no specific treatment, accelerated idioventricular rhythm can rarely degenerate to a life-threatening arrhythmia. Here, we present a child with left coronary cusp-originating accelerated idioventricular rhythm, degenerating into torsades de pointes and resulting in cardiac arrest, which was ablated with a cryocatheter. An 11-year-old boy, followed due to asymptomatic accelerated idioventricular rhythm before, was referred to our department because he had experienced an aborted cardiac arrest during sleep. He had been resuscitated for 5 minutes. Twenty-four-hour Holter-ECG revealed incessant accelerated idioventricular rhythm, consisting up to 90% of the whole record and two torsades de pointes attacks, triggered by accelerated idioventricular rhythm-induced “R on T” phenomenon, and resulting in syncope and cardiac arrest. Transthoracic echocardiography revealed no structural cardiac defect but mild left ventricular systolic dysfunction with an ejection fraction of 45% and shortening fraction 23%. An electrophysiologic study was conducted, and accelerated idioventricular rhythm focus was mapped to left aortic coronary cusp. A cryocatheter with an 8-mm tip was preferred for successful ablation of the accelerated idioventricular rhythm focus, due to close neighbourhood to coronary ostium. The patient was discharged in 3 days without any premature ventricular contractions or accelerated idioventricular rhythm and with normalised cardiac functions. After 9 months on follow-up, he was still asymptomatic, without any premature ventricular contractions or accelerated idioventricular rhythm and with normal cardiac functions. Although the clinical course of accelerated idioventricular rhythm is known as benign, accelerated idioventricular rhythm can rarely degenerate to a life-threatening arrhythmia. In such cases, electrophysiologic study and catheter ablation are a good option in such cases with accelerated idioventricular rhythm for an ultimate cure.

An 11-year-old male was presented with exertional chest pain and was diagnosed with atresia of the left main coronary artery. A stress nuclear perfusion imaging was negative at initial presentation, and a vasodilator stress cardiac MRI was again negative 5 years later. The patient has fully participated in competitive sports for 6 years with no occurrence of cardiac events.

We report the case of a fetus with anamnios sequence and VACTERL syndrome, having a circumflex right aortic arch. Two arterial ducts join anteriorly to form a common vessel that connects to the pulmonary trunk with confluent pulmonary branches. Embryologically, the dorsal right 6th aortic arch did not disappear and the aortic arch development stopped in a symmetrical state with an exceptional “Y-shaped” merged bilateral arterial duct.

An increased incidence of CHD has been noted in twin gestations and in infants conceived using assisted reproductive technologies. However, CHD in these populations remains understudied and the mechanisms underlying these phenomena remain unclear. We present the case of twins conceived via in vitro fertilisation both with Tetralogy of Fallot and additional cardiac and extracardiac malformations.

We report an extremely rare case of a 14-month-old girl who was diagnosed with a single right coronary artery with coronary artery fistula communicating with the right ventricle and congenital absence of left coronary artery. Angiography showed a dilated and tortuous single right coronary artery draining into the right ventricle, absence of left coronary system, and left ventricular coronary circulation supplied via collateral vessels.

A right aortic arch with an isolated left innominate artery is a rare form of aortic arch anomaly. We present a case of neonatal diagnosis of this anomaly with concerning findings of global cerebral white matter atrophy at 13 months of age.

We present an unusual case of concordant ventriculoarterial connections, subpulmonary infundibulum, and parallel arterial trunks. This case was complicated by extreme pulmonary artery tortuosity and low arching aorta causing severe tracheal compression. We discuss the difficulty in prenatal diagnosis, necessity for advanced imaging postnatally, and associated airway complications.

Anomalous single coronary artery from pulmonary artery is a very rare congenital heart anomaly. Anomalous single coronary artery from pulmonary artery has high mortality rates and poor surgical outcome despite advanced surgical techniques. We report a 4-month-old infant presented by congestive heart failure findings and diagnosed with anomalous single trunk coronary arteries arising from right pulmonary artery.