We report our experience with an 8-year-old boy with complete atrioventricular block and syncopal bradycardia who required urgent pacing. Each attempt to cross the tricuspid valve with a femoral lead triggered ventricular standstill, followed by fibrillation, and pacing through the coronary sinus failed. Successful ventricular pacing was finally achieved through the oesophagus, allowing subsequent implantation of a transvenous pacemaker.

An asymptomatic 4-year-old child was referred to our Institution with a provisional diagnosis of severe aortic valvar regurgitation in association with a ventricular septal defect. Intraoperatively, the aortic valve appeared bicuspid, and a tunnel of 7 mm diameter extending between the aorta and the left ventricle was found dividing the anterior commissure into two hemi-commissures. Repair was achieved by reconstructing the anterior commissure by direct reapproximation, and simultaneous obliteration of the aortic side of the tunnel. The aortic valvar leaflet was resuspended onto the arterial wall. This combined approach will hopefully reinforce the poorly supported aortic sinus, and may maintain aortic valvar competence over the long term. Our intraoperative diagnosis of aorto-left ventricular tunnel suggests consideration of this diagnosis when evaluating any patient with suspected aortic valvar incompetence.

It is rare to find multiple fistulas arising from all three coronary arteries and draining into the left ventricle. Coronary angiography revealed this anomaly in a one-year-old girl with tricuspid atresia after conversion to the Fontan circulation. To the best of our knowledge, this is the first report of such multiple fistulas in the setting of tricuspid atresia, and also the first report in childhood.

Transplantation is an effective treatment modality for infants1 and children2 with end-stage cardiac diseases. Rejection remains a major complication (Figure 1), even in newborn infants.3 Acute rejection can best be operationally defined by clinical findings, histopathology, and/or abnormalities of ventricular function of new origin that require, and respond to, intensified immunosuppression. Mechanistically, the ability to detect acute rejection is critically dependent on the detection of significant new myocytic injury, damage, and/or death. Surveillance for rejection is critically important in determining both long and short-term outcomes following cardiac transplantation. The ideal strategy for surveillance should have a high negative predictive value, correctly identifying the absence of myocytic injury, with high specificity, such that it does not falsely predict such injury.4

We describe an asymptomatic 1-year-old boy who presented a continuous murmur, and was found to have a tunnel between the right atrium and the aorta. The definitive diagnosis was established by both echocardiography and aortography, and surgical interruption of the tunnel was successful. We emphasise the rarity of this condition, and discuss its major features.

Scimitar syndrome is a rare congenital anomaly that affects classically the right lung and the heart. We present a rare variant that involves both lungs in association with totally anomalous pulmonary venous connection, horseshoe lung, and right pulmonary cyst.

in this review, i will first consider the general physiology induced by hypoplasià of the left ventricle, examining in particular its practical consequences in the fetus and the neonate. i will then explore the particularity of the physiology after the first stage of the norwood sequence of operations, comparing the two approaches of an aorta-pulmonary shunt versus a conduit placed from the right ventricle to the pulmonary arteries.

The acronym PHACES summarizes the most important manifestations of a rare neurocutaneous syndrome. Specifically, “P” accounts for malformation of the brain in the region of the posterior fossa, “H” stands for haemangiomas, “A” is for arterial anomalies, and “C” is for coarctation of the aorta along with cardiac defects, “E” is for abnormalities of the eye, and “S” for clefting of the sternum, and/or a supraumbilical abdominal raphe. Our objective is to introduce the syndrome to paediatric cardiologists. Our patient has stenosis of the aortic arch, multiple malformations of the great vessels arising from the aortic arch, intracranial vascular abnormalities, a sternal malformation with a supraumbilical raphe, and facial haemangiomas. We stress that it is important always to consider the existence of this syndrome in all patients with facial haemangiomas.

the norwood procedure and its modifications have become the most commonly used surgical procedures for patients with hypoplastic left heart syndrome. many efforts have been made to understand the fluid dynamics of the norwood circulation, with the major impetus coming from the lumped-parameter model described by migliavacca et al. in this model, a large number of variables have been analysed to understand their effect on the norwood circulation. in the clinical setting, some of these parameters can be influenced by the surgeon during the operation. the shunt has been demonstrated to be the critical element in regulating the pulmonary and systemic flows, with the goal of the surgeon being to obtain balanced flows. in order to do this, it is possible to influence the flow through the shunt in three ways:

1. by augmenting the pressure of perfusion of the shunt, with a suboptimal aortic reconstruction that can leave some residual pressure gradient. this can work like an increment of systemic vascular resistance;

2. by varying the size of the shunt;

3. by varying the type of architecture of the shunt, since flow through the shunt is dependent on the site of anastomosis, the angle with the vessels, and so on.

in order to validate or verify the results obtained from the lumped-parameter model, we have reviewed our experience with the norwood procedure since 1992.

I discuss a newborn whose mother is addicted to alcohol. On the third day of life, the newborn was found to have ventricular tachycardia. After spontaneous termination of the abnormal rhythm, the duration of the corrected QT interval was 0.48 s. During the next days, the duration of the interval normalized, and has now remained stable for 5 years. I conclude that the so-called “alcohol withdrawal syndrome of the newborn” might cause postnatal prolongation of the QT interval.

The hypoplastic left heart syndrome constitutes a wide spectrum of cardiac malformations, characterized by varying degrees of underdevelopment of the structures of the left heart and aortic arch. At the severe end of the spectrum, there is aortic and mitral atresia, and the left ventricle is vestigial. In contrast, at the mild end, there is hypoplasia of the left ventricle in the absence of overt stenosis of either the aortic or mitral valves, the combination we have described as hypoplastic left heart complex.1 Until recently, debate with regard to the optimal surgical approach has centered on the choice between the Norwood operation and neonatal cardiac transplantation. In the last several years, nonetheless, it has been shown that of biventricular repair can be achieved in those patients at the favorable end of the spectrum that we designated as having the complex rather than the syndrome.1,2 It is well recognized that an ongoing difficulty is the inconsistent and imprecise definition of hypoplastic left heart syndrome. In this presentation, I summarize our own criterions, and our principles for biventricular repair, as based on our experience at the Montreal Children's Hospital, for those patients we judge to have the hypoplastic left heart complex.

We report a rare case of an isolated critical tricuspid regurgitation due to rupture of a papillary muscle. This patient presented with a cyanosis immediately after birth. Despite mechanical ventilation and medical management to decrease the pulmonary vascular resistance, the low cardiac output persisted, along with the cyanosis. Repair of the tricuspid valve was performed, using an artificial tendinous cord, on the 4th day of life. The short-term result of the surgery is satisfactory, but the patient requires long-term follow-up.

Pulmonary atresia with intact ventricular septum has been described extensively, and it is recognized that associated abnormalities of the coronary arteries may cause ischemia. We describe a fetus, diagnosed antenatally as having pulmonary atresia with intact ventricular septum and severe hypoplasia of the right ventricle, who developed severe left ventricular dysfunction. We hypothesize that this is due to reduced coronary blood flow because of falling right ventricular volume in the presence of complete right ventricular coronary arterial dependence.

A 12-year-old boy presented with tachycardia and cardiomegaly. An electrocardiogram showed atrial flutter with a ventricular rate of 170 beats/min. The echocardiogram showed left ventricular dilation, with decreased contractility. Electromyography confirmed the diagnosis of myotonic dystrophy. The atrial flutter was converted to sinus rhythm by electroversion, leading to restoration of normal cardiac function. Our case illustrates that atrial flutter can be an early sign of the cardiac complications of myotonic dystrophy in childhood.

Contrary to what the name implies, the left atrial appendage is increasingly becoming an organ of clinical importance. Its role in the pathogenesis of thromboembolism is now well established. Enlargement of the appendage due to haemodynamic disturbances or anatomic weakness increases this risk, along with the potential for arrhythmia. We report a rare case of gross enlargement of the left atrial appendage within the pericardial cavity.