We present a case of a 57-year-old man with congenitally corrected transposition of great vessels who was found to have coronary ostial atresia with cranial flow through left superior vena cava on CT with contrast injection in coronary tributaries. As such, he was ineligible for cardiac resynchronization therapy.

Fetal hydrops is a serious complication of immune-mediated congenital complete atrioventricular block. We present the case of a fetus with severe hydrops and profound bradycardia and an unusual favourable outcome. This case enhances the importance of considering the contribution of ventricular ectopic beats to the cardiac output when counselling and predicting outcome of complete heart block.

Congenitally corrected transposition of the great arteries is a rare form of CHD. Situs inversus is a much less common variant of a congenitally corrected transposition of the great arteries. In rare cases, transposition events may be accompanied by various cardiac anomalies. However, situs inversus patients with congenitally corrected transposition, single coronary artery anomaly, and atrioventricular block together have not been reported previously. This combination of abnormalities is presented as a first in the literature.

Pneumopericardium, defined as air in the pericardial cavity, is a rare condition with potentially severe complications and mortality. In the neonatal period, pneumopericardium is associated with prematurity, very low birth weight, and assisted ventilation. We report the occurrence of spontaneous pneumopericardium in a healthy full-term neonate who did not receive any supportive ventilation.

In patients with ductal-dependent CHD, ductal restriction can be life-threatening. We present the use of urgent hybrid palliation involving placement of bilateral pulmonary artery bands and ductus arteriosus stent implantation in a 1.5 kg premature infant with interrupted aortic arch and ductal restriction.

In this article, we report on a newborn with hypoplastic left heart syndrome in whom recoarctation of the aorta was treated with a bare metal stent (Cook Formula 414 Stent) in the early postoperative period after a Norwood procedure. To reduce the risk for scarring and occluding the femoral artery the stent was implanted via 5F Glidesheath Slender sheath.

Anomalous drainage of the right superior caval vein into the left atrium is a rare congenital anomaly that causes cyanosis and occult infection owing to right-to-left shunting. Transcatheter management of this anomaly is unique and rarely reported. We report a 32-year-old man with a history of brain abscess, who was diagnosed with an anomalous right superior caval vein draining to the left atrium; right upper pulmonary vein and right middle pulmonary vein draining into the inferior portion of the right superior caval vein; and a left superior caval vein draining into the right atrium through the coronary sinus without a bridging vein. Pre-procedural planning was guided by three-dimensional printed model. The right superior caval vein was occluded with a 16-mm Amplatzer muscular Ventricular Septal Defect occluder inferior to the azygous vein, but superior to the entries of right upper and middle pulmonary veins. This diverted the right superior caval vein flow to the inferior caval vein system through the azygos vein in a retrograde manner and allowed the right upper pulmonary vein and right middle pulmonary vein flow to drain into the left atrium normally, achieving exclusion of right-to-left shunting and allowing normal drainage of pulmonary veins into the left atrium. At the 6-month follow-up, his saturation improved from 93 to 97% with no symptoms of superior caval vein syndrome.

Gemella is a genus of Gram-positive bacteria found in the digestive tract of humans. They rarely cause systemic illness but have been recently implicated in several serious infections. We report infective endocarditis caused by Gemella bergeri in a 23-year-old with a bicuspid aortic valve status post-intervention in infancy.

Pulmonary valvular stenosis is a relatively common disorder, accounting for approximately 10% of all CHDs. Pulmonic valvular disease can get clinically detected at different ages of life. The more severe the obstruction, the earlier detected the valvular abnormality. Surgical pulmonary valvotomy has been available as a treatment since 1956. This article is about a case of pulmonary annular and valvular stenosis in a 1-year-old child, and it also explores surgical operation of this condition. Transannular patches are usually used within the 1st year of age in pulmonary annular and valvular stenosis. In recent years, anterior leaflet augmentation has been preferred for annulus enlargements. In our 1-year-old case, we expanded the annulus by the anterior leaflet expansion technique and we also augmented other leaflets by polytetrafluoroethylene patch.

Aneurysm–osteoarthritis syndrome is a recently discovered inherited autosomal dominant connective tissue disease caused by SMAD3 mutations. Aneurysm–osteoarthritis syndrome is responsible for 2% of familial thoracic aortic aneurysms and dissections and is characterised by aneurysms, dissections, and tortuosity throughout the arterial tree in combination with osteoarthritis. Early-onset osteoarthritis is present in almost all patients. We present the case of a non-syndromic young boy with SMAD3 mutation isolated from the dilated aortic root and ascending aorta without osteoarthritis.

A 17-year-old girl with situs ambiguous, hypoplastic right ventricle with a large ventricular septal defect, and severe pulmonary stenosis had undergone Kawashima operation 10 years back. She had significant desaturation because of a large Abernethy malformation, with reverse shunting from the inferior caval vein to the portal vein. It was closed with a vascular plug, with improvement in oxygen saturation. She developed extensive inferior caval vein thrombus following the procedure, which was managed conservatively by anti-coagulation.

Hypoglycaemia is a well-known side effect of Propranolol. We described the case of a child presenting severe and recurrent Propranolol-induced hypoglycaemia. Those episodes were not related to prolonged fasting and were associated with only mild ketosis. Thus, therapy with β blockers may not only aggravate classical ketotic hypoglycaemia but also interfere with glucose metabolism.

Spontaneous regression of severe aortic coarctation with ductus dependency has not been reported. We experienced a case of trisomy 18 with spontaneous regression of severe aortic coarctation complicated by ventricular septal defect and patent ductus arteriosus. The aortic isthmus diameter was 1.2 mm at birth. After 5 months, it increased to 4.5 mm, and the shape of the isthmus was fully normalised.

We describe the case of a 21-year-old patient who underwent repairs for multiple lesions including aortic and pulmonary valve replacements, right ventricular outflow tract reconstruction, revision of the right pulmonary artery route, and a repair of partial anomalous pulmonary venous drainage, which was diagnosed during this fourth sternotomy. For these patients with adult CHD, it is most important to address all underlying factors as much as possible at the redo surgery.

If coronary artery sequelae are the only suggestive signs of previous Kawasaki disease, the diagnosis may easily be missed. We describe a rare case of a child with severe occlusive coronary disease likely owing to missed Kawasaki disease. This diagnosis was not initially considered given the age and absence of suggestive history. Careful echocardiographic assessment and low-radiation coronary CT angiogram resulted in successful diagnosis and treatment.

Diaphragm excursion method is unsuitable for diagnosis of diaphragm paralysis during positive pressure ventilation. We diagnosed diaphragm paralysis in a neonate, without interrupting positive pressure respiratory support after cardiac surgery, using the diaphragm thickness fraction, which could be evaluated during mechanical ventilation and was unaffected by bowel gases. The diaphragm thickness fraction method can help diagnose diaphragm dysfunction using only echography.

We have previously shown that children with a bioelectrical impedance spectroscopy phase angle at 50° (PA 50°) of <2.7 on postoperative day 2 had a four-fold increase in the risk of prolonged paediatric intensive care length of stay. In this study, we demonstrate a relationship between a baseline measure of phase angle 200/5° and postoperative length of stay.

Pulmonary artery slings and vascular rings are very rare congenital anomalies. It is even rarer to have both anomalies in the same setting. We present a case of a toddler who was diagnosed with a left pulmonary artery sling and a vascular ring as part of the screening process for the VACTERL association – co-occurrence of vertebral, anorectal, cardiac, tracheoesophageal, renal, and limb malformations. He underwent a successful surgical repair via median sternotomy and on cardiopulmonary bypass with an uneventful postoperative course.

Cardiac involvement has been reported in various mucopolysaccharidoses syndromes. Cardiac valve pathology is the most prominent cardiac manifestation of patients with these syndromes. To date, there have been no reports of early childhood onset of high-grade atrioventricular block in patients with Hunter syndrome. We present a case of a 3-year-old boy with Hunter syndrome who was found to have various degrees of atrioventricular block. This case highlights the importance of early routine cardiac screening for conduction abnormalities and close follow-up in patients with mucopolysaccharidoses syndromes.