Massive vertical vein aneurysm in a supracardiac total anomalous pulmonary venous connection is rare. Herein, vertical vein aneurysm with total anomalous pulmonary venous connection and additional pathological findings are reported in a young child.

Major aortopulmonary collateral arteries in the setting of pulmonary atresia with intact ventricular septum are very rarely encountered, having been documented in only a handful of case reports. We present the right ventriculogram of a patient found to have this rare combination of findings along with right ventricular-dependent coronary artery circulation and unusual supply of the right pulmonary artery.

The combination of aortopulmonary window, interruption of the aortic arch, and anomalous origin of the right pulmonary artery from the ascending aorta is a rare and complex congenital cardiac malformation. Despite good prenatal care in our case, this cardiac anomaly was not detected prior to birth. Untreated infants who do not undergo surgical correction have a mortality rate of 70% in their first year, and 30% will die within the first 3 months of life.

We report the first successful implantation in the United States of a novel mitral valve (MITRIS RESILIA by Edwards Lifesciences) in a patient with history of mitral valve replacement at a young age. This new bioprosthetic valve offers a unique profile and innovative option for mitral valve replacement in patients who are at risk of left ventricular outflow tract obstruction.

Pulmonary atresia with intact ventricular septum is a complex cyanotic congenital heart lesion with the potential for myocardial ischaemia due to the presence of coronary artery anomalies. We present a case of a two-day-old baby with postnatal diagnosis of pulmonary atresia with intact ventricular septum in whom non-invasive ferumoxytol-enhanced 4D cardiac magnetic resonance angiography was used for the assessment of coronary artery anatomy.

Tuberous sclerosis is a genetic multisystem disorder characterised by hamartomas in several organs. Cardiac rhabdomyomas are the main features of the disease but lipomas can very rarely be associated. Herein, we present a very rare association of tuberous sclerosis and cardiac lipoma detected by echocardiography and diagnosed as a lipoma via MRI and fat suppression technic, aim to report this very rare association, and emphasise usefulness of MRI in cardiac mass lesions.

RASopathies – caused by mutations in the RAS/MAPK signaling pathway – are frequently associated with cardiac diseases, such as hypertrophic obstructive cardiomyopathy. Although cibenzoline is useful for adult hypertrophic obstructive cardiomyopathy patients, little is known about its effect in children. Here, we report two paediatric cases of hypertrophic obstructive cardiomyopathy associated with RASopathies where the condition was improved by cibenzoline.

In hypoplastic left heart syndrome, the size and function of the left ventricle vary and are dependent on the patency of the aortic valve. A patent native aortic valve, permitting left ventricular ejection, can augment cardiac output. We performed a retrospective chart review of patients with hypoplastic left heart syndrome and a stenotic aortic valve who underwent native aortic valvuloplasty at the time of Norwood and found that none of the eight patients identified had clinically significant aortic insufficiency. This case series suggests that surgical aortic valvuloplasty at Norwood is associated with aortic valve patency/augmented systemic cardiac output without the development of clinically significant aortic regurgitation at intermediate follow-up in a limited cohort.

Tetralogy of Fallot with an aortopulmonary window and double aortic arch is very rare. This complex coexistence may be over a wide clinical spectrum. Herein, we present an asymptomatic 8-day-old infant who was diagnosed as having tetralogy of Fallot, double aortic arch, and an aortopulmonary window using transthoracic echocardiography while being examined for microcephaly.

We present a 13-year-old boy who had recurrent chest pain with elevated cardiac enzymes and abnormal ST segments in electrocardiogram 36 hours after the second dose of BNT162b2 vaccination. Cardiac MRI and coronary angiography with acetylcholine provocation confirmed myocarditis and vasospastic angina, respectively. Coronary vasospasm may play a pivotal role in the chest pain in COVID-19 vaccine-related myocarditis.

Transcatheter closure of perimembranous ventricular septal defect is an alternative to surgery in selected small to medium defects with a reasonable distance between the defect and the aortic or tricuspid valves. Surgical closure is generally preferred for large defects with an inlet to outlet extension, aortic malalignment, or deficient aortic rim. We report two successful cases of percutaneous closure of large perimembranous ventricular septal defects with inlet to outlet extension using an Amplatzer Vascular Plug-II via a retrograde approach. Transcatheter closure of large perimembranous ventricular septal defect with inlet to outlet extension is feasible and facilitated by the characteristics of the Amplatzer Vascular Plug-II device and the specific implantation technique.

Reversible cerebral vasoconstriction syndrome presents with thunderclap headache and represents a group of conditions that show reversible multifocal narrowing of cerebral arteries. Some patients who undergo device closure of an atrial septal defect complain of headache, which are posited as a migraine. Here we report a case of severe headache due not to migraine but reversible cerebral vasoconstriction syndrome after device closure of a ventricular septal defect.

Aorto-right-atrial fistula is an uncommon condition with an unclear pathogenesis. We present the case of a 3-year-old girl with a giant omphalocele repaired days after birth and incidentally discovered with a celiacomesenteric trunk-to-right atrium fistula. Three-dimensional reconstruction CT unveiled its anatomical pattern, and the fistula was successfully closed using a Amplatzer vascular plug II percutaneously.

Bicuspid aortic valve is the most common congenital heart malformation and predisposes patients to thoracic aortic aneurysms and aortic dissections. Current peripartum guidelines are extrapolated from other heritable causes of thoracic aortic disease and do not account for unique characteristics of bicuspid aortic valve patients. We therefore evaluated the prevalence of maternal and fetal complications of women with early-onset complications of bicuspid aortic valve disease in the UTHealth Bicuspid Aortic Valve Research Registry. We found that the rate of cardiovascular complications was high and that relatively few women received guideline-recommended care.

Infective endocarditis is a rare complication of atrial septal defect closure using transcatheter procedure. We report about infective endocarditis in an 8-year-old boy 3 months after transcatheter closure using a Figulla Flex II atrial septal defect occluder. Transesophageal echocardiography showed vegetation attached to the left atrium side of the device. Device removal and atrial septal defect closure were performed. The device was less endothelialized on the left than on the right atrium side. Therefore, insufficient endothelialization may cause infective endocarditis.

Behcet’s disease is a multi-systemic inflammatory disease with a clinical spectrum as a triple complex of recurrent oral, genital ulcers, and uveitis. Cardiac involvement in patients with Behcet’s disease is extremely rare and often associated with poor prognosis. Behcet’s disease should be considered in the differential diagnosis of right ventricular mass especially in young adults, even there is no typical clinical features of Behcet’s disease. In this case, a 12-year-old girl who admitted with chest pain and haemoptysis and then was diagnosed with intracardiac thrombus related to Behcet’s disease during follow-up was described.