Left main coronary artery aneurysms are very infrequent (0.1%) and the majority is related to atherosclerotic obstructive lesions. We describe a young male having no conventional coronary risk factors presenting with acute inferior wall myocardial infarction. Coronary angiography revealed a prominent aneurysm of the left main coronary artery with selective extension towards the proximal left circumflex artery without associated coronary lesions.

Transposition of the great arteries with left ventricle outflow tract obstruction in combination with situs inversus totalis and dextrocardia is a very rare anomaly and carries high morbidity and mortality. Only few cases have been reported with this anomaly. We describe a 21-day of infant girl with transposition of the great arteries and mirror image dextrocardia and pulmonary stenosis who underwent successful neonatal arterial switch operation and left ventricle outflow tract obstruction resection following PDA stent implantation

The identification of the important relationship between shape and function of ventricular chambers represents a milestone of modern cardiology. Application of the law of Laplace for an ideal sphere furnishes intuitive insights on the progression of heart failure. A dilated heart, by virtue of its large size, must generate greater stress in the myocardial wall to achieve sufficient pressure so as to eject the required amount of blood. The mural hypertrophy represents a compensatory mechanism, guaranteeing a lower stress. When the ratio between the radius of the chamber and the thickness of its wall increases abnormally, the heart fails.

clinical experience in the treatment of patients with functionally univentricular hearts has shown the fontan operation to be much less than a perfect therapy. even in ideal patients, acquired elevation of the venous pressures in the systemic circulation, especially in the regions drained by the inferior caval vein, is only a matter of time. for this reason the treatment of this complex form of congenital heart disease is still the object of active scientific debate, as well as considerable ongoing medical and basic research.

This is a case of a young woman previously completely healthy, with two uneventful pregnancies and deliveries, who presented with bloating, shortness of breath, and signs of right heart failure. A thorough clinical evaluation, along with a work-up including an echocardiogram and a magnetic resonance imaging revealed the diagnosis of Ebstein’s anomaly malformation with tricuspid stenosis. A right ventricular outflow tract obstruction due to thrombus formation was thought to exacerbate her symptoms and lead to the diagnosis. Surgery in the form of right ventricular thrombectomy, right atrial reduction, porcine tricuspid valve replacement and bidirectional cavopulmonary anastomosis “bidirectional Glenn”, was successfully performed. The patient has been stable clinically more than one year after surgery. Discussion about this rare condition and operative details are provided.

Cyanosis in a newborn is commonly due to intracardiac shunts or duct-dependent lesions. Systemic venous anomalies are uncommon and sinus venous atrial septal defects do not usually present with cyanosis. We report the case of a 4-month-old female with persistent hypoxemia due to a right superior vena cava overriding a superior sinus venous atrial septal defect resulting in a right to left shunt.

The technique of deep hypothermia with circulatory arrest has been important in the history of the evolution of cardiac surgery. Wilfred G. Bigelow, working in Toronto in the late 1940s, performed pioneering research on hypothermia, and developed a workable technique of hypothermia in human cardiac surgery.1 Based upon Bigelow's experimental premises, F. John Lewis, at the University of Minnesota, also conducted a number of experiments utilizing hypothermia. On September 2, 1942, Lewis operated on a 5-year-old girl with an atrial septal defect under general hypothermia with inflow occlusion. He was assisted by Richard Varco, Mansur Taufic, and C. Walton Lillehei. Rubberized refrigerated blankets were used to cool the patient to 28°C. The septal defect was closed during five and a half minutes of inflow occlusion. This was the world's first successful open operation on the human heart performed under direct vision, and marked the beginning of the era of open heart surgery. Now, as amazing and as primitive as that methodology may seem, those of you who read Life magazine, or watch the Discovery Channel on television, are aware that, in parts of the Soviet Union, a large fraction of today's open heart surgery is performed not using the technique of cardiopulmonary bypass, but rather using the methodology of immersion hypothermia from the 1950s, with surprisingly good results.

Loeys-Dietz syndrome is a newly recognized constellation that presents with aortic aneurysm or dissection similar to Marfan’s syndrome. We describe successful surgical treatment in a 2-year-old with the syndrome in whom we performed a valve-sparing replacement of the aortic root because of significant dilation of the aortic root and the ascending aorta.

Haematogenous non-contiguous metastatic spread of remote solid tumours to the heart is rare. We describe a previously healthy 5-year-old girl who presented with extensive intracardiac involvement by metastatic pelvic sarcoma.

There has been an increase in the use of extracorporeal membrane oxygenation for severe neonatal cardiac failure. However, the frequency of complications is high, particularly in preterm and low-birth-weight neonates. Herein, we present combination treatment with transcatheter balloon atrioseptostomy and bilateral pulmonary artery banding in a collapsed preterm neonate. This strategy can be an alternative to circulatory support using extracorporeal membrane oxygenation.

We present the case of a 3-year-old boy with bicuspid aortic valve, aortic coarctation, and left ventricular non-compaction. The diagnosis was made post-natally with ultrasonography and was verified by cardiac MRI. Aortic coarctation was initially repaired surgically. At age 3 months, recoarctation and heart failure developed. Balloon angioplasty was performed with immediate improvement. At age 3 years, the patient remains asymptomatic and normotensive.

Therapeutic options are limited for the management of extremely low-birth-weight infants with critical aortic coarctation despite high doses of prostaglandin infusion. We report successful hybrid, fluoroscopy-free, echocardiography-guided primary stenting of native aortic coarctation in a 920-grams premature infant.

A 10-year-old girl with genetically confirmed Jervell-Lange-Nielsen syndrome treated with beta-blocker and developed electrical storm after changing propranolol syrup to tablets. Jervell-Lange-Nielsen is characterised by long QT and congenital sensorineural deafness, with high risk of malignant arrhythmias at early ages. Gastric involvement and achlorhydria may be present, with subsequent alteration of medication bioavailability which can trigger severe arrhythmic complications.

A 29-year-old gravida1 para1 woman presented with increasing fatigue. Multi-modality imaging demonstrated the left coronary artery arising from the main pulmonary artery with large collateral vessels in the interventricular septum, in keeping with unrepaired anomalous left coronary artery from the pulmonary artery. Cardiac MRI T1 mapping demonstrated globally elevated non-contrast T1 and extracellular volume fraction values, which suggested the presence of diffuse interstitial myocardial fibrosis. Cardiac MRI T1 mapping allows for a new dimension of myocardial characterisation, providing insight into subtle, diffuse abnormalities at the tissue level.

Tumours originating from cardiac tissues are rarely encountered during childhood, and fortunately most of these tumours are benign in nature. Inflammatory myofibroblastic tumour, which has unique clinical, pathological, and molecular characteristics, is a relatively new entity compared with previously mentioned tumoural processes originating from the heart. Most of the cardiac intima-media thickness patients are in the age group of 4 months to 17 years. This rarely seen tumoural process has not been subject of any specific research and the prognosis is not well known. Here we present the case of a 3-month-old child who was admitted to our outpatient clinic with massive pericardial effusion and who has shown excellent progress after surgical resection of over 1 year.