Incomplete involution of valve of systemic venous sinus can present across a spectrum of anatomical lesions ranging from eustachian valve to division of right atrium (cor triatriatum dexter) with overlapping features. We present the case of a neonate presenting with cyanosis, having persistent valve of systemic venous sinus with anatomical details of the redundant tissue in right atrium suggesting an intermediate form between Chiari network and division of right atrium.

We describe a case of neonatal Marfan syndrome diagnosed because of a family history, dysmorphic features, and cardiac abnormality. The echocardiogram showed aortic root dilatation. Molecular genetic studies showed a mutation in exon 31 of the FBN1 gene in the infant and father. The infant was treated with losartan, which significantly slowed the rate of enlargement of the aorta.

This case report shares the story of a family who sought care elsewhere after their daughter was denied cardiac surgery in their home state because she had trisomy 18. This case report recommends case-by-case assessment of cardiac surgical interventions for children with trisomy 13 or 18 as informed by review of goals, assessment of comorbidities, and literature-informed practice. Coordinated care planning and interdisciplinary communication are relevant in cardiac surgical considerations for children with these underlying genetic conditions.

Exercise is of both physical and psychological benefit in children with congenital heart disease, leading to improved cardiovascular fitness and better quality of life. In such children, however, capacity for exercise is often impaired, and there is a risk of exercise-induced mortality or morbidity. We have reviewed the abnormal cardiovascular response to exercise in children with congenital heart defects and postoperative residua, and the role of exercise testing and training in their diagnosis and treatment. Some patients should be excluded absolutely from all but mild regular exercise because of known high risk; for example, those with severe aortic or subaortic stenosis. Other patients should be encouraged to participate in sports without any limitation; for example, those with small left-to-right shunts or mild valvar regurgitation. In many patients strict recommendations cannot be made, and one must consider the individual, the lesion and its hemodynamic implications, and the type and level of exercise contemplated. Children with congenital heart disease should be encouraged to participate in exercise and recreational sport within the limits provided by their cardiovascular defect. Understanding the pathophysiology of these defects, and knowledge of the risk of exercise in certain conditions, will allow the physician to make sensible recommendations for participation in exercise by individual patients.

Reported here is an obstruction of the right ventricular outflow tract caused by a tuberculoma in a 15-year-old boy who presented with a ventricular septal defect. The obstruction was discovered at surgery and the tuberculous aetiology was only demonstrated histologically. To the authors' knowledge, this is the first report of a tuberculoma of the heart associated with congenital heart disease.

The incidence of congenital heart disease is higher in monozygotic than dizygotic twins, with a higher concordance rate. Although coarctation of the aorta has previously been reported in monozygotic twins, to the best of our knowledge it has not been described in dizygotic twins. We report here such a concurrence in dizygotic twins conceived by in-vitro fertilisation. The finding provides support for both genetic and environmental factors in the aetiology of congenital heart disease. Furthermore, it highlights our lack of data regarding the outcome of in-vitro fertilisation.

Myocarditis is an inflammation of the heart muscle. In this case, a previously healthy, 17-year-old adolescent with myocarditis after BNT162b2 mRNA vaccination was reported. He was admitted to the hospital with severe chest pain, changes in electrocardiography, and elevation in serum troponin level after fourth day of receiving first dose of vaccine. There was no coronary arterial disease in coronary angiogram. A diagnosis of vaccine-induced myocarditis was made, and supportive treatment was initiated.

Abstract Severe Ebstein's malformation has a poor prognosis when diagnosed during the neonatal period. Surgical options that have proved successful in older patients have been associated with high mortality and morbidity in neonates. We report here our success with a policy of induction at term and immediate surgical intervention when Ebstein's malformation was diagnosed prenatally.

Isolated fetal pericardial effusion is generally held to have a good prognosis. We report a study of seven cases identified from a total of 504 fetuses scanned and followed with serial fetal scans.Our results show that it was associated with a poor fetal prognosis in six of the seven pregnancies. Probably due to the high incidence of mid-trimester abortion and the association with other abnormalities only evident postnatally, this has not, hitherto, been reported. We would advocate close follow-up and a guarded prognosis when isolated pericardial effusions are found on fetal scans.

Objectives: This publication aims to report the cases of four children with pseudoaneurysm of the mitral-aortic intervalvular fibrosa and carry out a review of the literature. Background: Pseudoaneurysm of the mitral-aortic intervalvular fibrosa is a very rare anomaly in children. It can be either congenital or acquired, namely, after bacterial endocarditis or cardiac trauma. This pathology does not usually cause specific symptoms but its outcome may be potentially fatal. Methods: We report the cases of four patients presenting with pseudoaneurysm of the mitral-aortic intervalvular fibrosa, referred for treatment in a paediatric cardiology clinic. Patient clinical notes were retrospectively reviewed for aetiology, clinical presentation, diagnostic work-up, surgical treatment, and follow-up. Literature on the subject was extensively reviewed. Results: In three patients, pseudoaneurysm of the mitral-aortic intervalvular fibrosa was acquired, being secondary to bacterial endocarditis in two cases and establishing after mitral surgery in another case. The remaining patient had a “congenital” aetiology – no other cause could be traced. The diagnosis was achieved by transthoracic echocardiography for all patients, and confirmed in all by trans-oesophageal echocardiography, to better define morphological details and to access flow into the aneurysmal formation. All patients were submitted to corrective cardiac surgery. Of the patients, three survived and were cured by surgery, staying asymptomatic, and one died after repeated interventions, for persistent endocarditis. Conclusions: Pseudoaneurysm of the mitral-aortic intervalvular fibrosa is a rare but potentially fatal anomaly. In our experience, surgical cure was achieved for the majority of the cases, except for a case for which infection could not be locally eradicated, leading to multiple reinterventions.

The authors describe a case of Kawasaki disease who presented fever, coronary artery aneurysm, and ischaemic intestinal obstruction requiring stage resections. A review of literature of such association is performed. Atypical Kawasaki disease is more complex to diagnose and in combination of persistent fever with no definite cause and surgical abdominal pain, Kawasaki disease should be considered in the differential diagnosis.

Background: CHD is the leading cause of mortality due to birth defects. Array comparative genomic hybridisation (aCGH) detects submicroscopic copy number changes and may improve identification of the genetic basis of CHD. Methods: This is a retrospective analysis of 1252 patients from a regional referral centre who had undergone aCGH. Of the patients, 173 had CHD. A whole-genome custom-designed oligonucleotide array with >44,000 probes was used to detect copy number changes. Results: Of the 1252 patients, 335 (26.76%) had abnormal aCGH results. Of the 173 patients with CHD, 50 (28.9%) had abnormal aCGH results versus 284 (26.3%) of 1079 non-cardiac patients. There were six patients with CHD who had well-described syndromes such as Wolf–Hirschhorn, trisomy 13, DiGeorge, and Williams. Of the patients with CHD, those with left-sided heart disease had the highest proportion (14/31; 45.13%) of abnormal aCGH results, followed by those with conotruncal heart disease (10/29; 34.48%), endocardial cushion defects (13/50; 26%), complex/other heart disease (12/52; 23.08%), and patent ductus arteriosus (1/11; 9.09%). Conclusions: Patients with CHD are at a substantial risk of having microdeletions and microduplications. The incidence of abnormalities on aCGH analysis is higher than identified with karyotype, and identification of copy number changes may help identify the genetic basis of the specific heart defects. However, aCGH may not have a significant diagnostic yield in those with isolated CHD. Further research using larger data sets may help identify candidate genes associated with CHD.

more than 30 years ago, fontan and baudet proposed bypass of a dysfunctional right ventricle by connecting the pulmonary arteries directly to the right atrium, the so-called atriopulmonary anastomosis. since then, much experience has been accrued in the field of the functionally univentricular circulation. the proposed connections have been subjected to several modifications, aiming towards minimizing the losses of energy in the cavopulmonary system, and thereby improving the clinical outcomes. a remarkable improvement was achieved with the introduction of the concept of the total cavopulmonary connection, specifically the combination of a bi-directional glenn anastomosis with a tubular intracardiac extension from the inferior caval venous to the pulmonary arteries. this design was shown to avoid the dissipation of energy associated with the swirling patterns seen in the traditional atrio-pulmonary anastomosis.