A 36-year-old woman, who had a history of myomectomy, presented with lightheadedness after changing position from sitting to standing and effort-related shortness of breath. Echocardiography demonstrated a hyperechoic elongated mobile mass extending from the inferior caval vein to the right atrium. The mass was surgically removed, and histological examination established the diagnosis of intravenous leiomyomatosis. This case caught the attention of our cardiology group to consider the diagnosis when an inferior caval vein or right atrium mass is found in a patient with a history of uterine leiomyomatosis.

Pulmonary hypertension is a complex and progressive condition that is either idiopathic or heritable, or associated with one or multiple health conditions, with or without congenital or acquired cardiovascular disease. Recent developments have tremendously increased the armamentarium of diagnostic and therapeutic approaches in children and young adults with pulmonary hypertension that is still associated with a high morbidity and mortality. These modalities include non-invasive imaging, pharmacotherapy, interventional and surgical procedures, and supportive measures. The optimal, tailored diagnostic and therapeutic strategies for pulmonary hypertension in the young are rapidly evolving but still face enormous challenges: Healthcare providers need to take the patient’s age, development, disease state, and family concerns into account when initiating advanced diagnostics and treatment. Therefore, there is a need for guidance on core and advanced medical training in paediatric pulmonary hypertension. The Association for European Paediatric and Congenital Cardiology working group “pulmonary hypertension, heart failure and transplantation” has produced this document as an expert consensus statement; however, all recommendations must be considered and applied in the context of the local and national infrastructure and legal regulations.

Corticosteroid therapy has become an important modality of treatment for diseases in which rapid control of immunoinflammatory processes is required. However, one of the serious, but less known adverse effect of this therapy is cardiac arrhythmias. This includes both tachyarrhythmias and bradyarrhythmias. Corticosteroid use may also be associated with electrolyte imbalances like hypokalaemia by its mineralocorticoid activity. Those side effects are mainly seen with high-dose intravenous methyl-prednisolone or oral pulse dose prednisolone therapy. Here we report our experience in a child with warm idiopathic autoimmune haemolytic anaemia who developed sinus bradyarrhythmias and treatment refractory hypokalaemia during low-dose steroid therapy with reduction in heart rate by 60% of baseline.

The patient with a functionally univentricular heart is at increased risk for ventricular dysfunction for a variety of reasons. At birth, the pulmonary and systemic circulations are in parallel, leading to pulmonary overcirculation and a volume-loaded functional ventricle. Significant atrioventricular valvar regurgitation, abnormal ventriculoarterial coupling, diastolic dysfunction, and altered ventricular geometry can also contribute to long-term ventricular dysfunction. These collected circumstances place the patient at increased risk for perioperative morbidity and mortality. We will discuss in this review the pathophysiology that leads to ventricular dysfunction at each stage of surgical palliation, as well as the strategies for perioperative management. In addition, we will highlight novel strategies for management of ventricular dysfunction.

To identify the echocardiographic features and natural history of dilated cardiomyopathy in fetal life, we reviewed records of all fetal echocardiograms over the 10 year period 1980–1989 to identify cases of dilated cardiomyopathy without left ventricular outflow obstruction and conducted a retrospective review of the fetal echocardiograms and clinical course of those fetuses with dilated cardiomyopathy. In addition, all fetal losses, perinatal deaths and live-born infants presenting with dilated cardiomyopathy in the first seven days of life were identified from the British Columbia Pediatric Cardiovascular Pathology Registry and from inpatient records. The pathological findings and outcome for this group were also reviewed. Marked left ventricular dilatation and dysfunction without left ventricular outflow obstruction was diagnosed in five of 1,158 fetuses undergoing feta lechocardiography. These five fetuses were correctly distinguished from two others with critical aortic stenosis and severe left ventricular dysfunction. Two of the five fetuses had additional right ventricular dysfunction and dilatation and developed hydrops, while the remaining three with isolated severe left ventricular dysfunction did not and showed normal somatic growth. All fetuses were live-born with spontaneous labour occurring in four of five cases at a mean gestational age of 36.5 weeks. Four of the five infants died, with three deaths occurring in the first week of life. During the same 10 year period, two of 2450 autopsied stillbirths (both hydropic) were diagnosed with dilated cardiomyopathy. An additional 15 neonates (including the five identified by fetal echocardiography) were diagnosed in the first week of life with dilated cardiomyopathy, congenital myocarditis or endocardial fibroelastosis. Four were hydropic (all with associated right ventricular dysfunction). Only three of the 15 survive. Fetal echocardiography can accurately distinguish dilated cardiomyopathy from left ventricular outflow obstruction. In the absence of right-sided disease or premature closure of the oval fossa, severe left ventricular dysfunction is well tolerated in utero. The prognosis for fetal dilated cardiomyopathy is very poor.

We report a case of 2:1 atrioventricular block associated with acquired long QT syndrome. A newborn presented to our neonatal intensive care unit with intermittent bradycardia due to 2:1 atrioventricular block. Initial evaluation showed QT prolongation and significant electrolytic abnormalities. After correction of the electrolytic imbalance, the QT interval normalized and atrioventricular block resolved. Compared to congenital long QT syndrome with 2:1 atrioventricular block, acquired long QT syndrome with comparable atrioventricular block has a benign prognosis, provided treatment is initiated quickly.

We describe the finding of unsuspected aortic coarctation, discovered by catheterisation at 4 months, in a neonate first daignosed on the fourth day of life as having tetralogy of Fallot with pulmonary atresia and major aorto-to-pulmonary collateral arteries. This previously unreported combination contravenes the established theory of patterns of flow during fetal life, and presented difficult issues in clinical management.

Described is a rare association in a patient with the heart in the left chest, namely pulmonary atresia with intact ventricular septum, fistulous coronary arterial connections, a right-sided aortic arch and an aorto-pulmonary collateral artery feeding one lung. The pulmonary arteries were non-confluent, with the right lung supplied by the right arterial duct originating from the under surface of the right-sided aortic arch, and the left lung supplied through the aorto-pulmonary collateral artery arising from the descending aorta. The surgical management is different in the setting of non-confluent pulmonary arteries.

This study reviews the results of the surgical management of 154 cases of ruptured aneurysm of the sinus of Valsalva. Of the patients0 73% were male, with an average age of 28 years. An associated ventricular septal defect was found in 40% and 23% had aortic valvar regurgitation. The aneurysms originated from the right coronary sinus in 79% and from the non-coronary sinus in the remainders. The aneurysms ruptured into the right ventricle in 73%, into the right atrium in 27% and into the left ventricle in less than 1%. Operative mortality was 4.5%. Long-term follow-up was achieved in 80% of patients, with a mean duration of 5.7 years and a range from two months to 29 years. Preoperative aortic regurgitation and preoperative functional class (NYHA III or IV) were both predictive of a worse long-term outcome. The optimal surgical approach was closure of the distal end of the fistula by direct suture together with reinforcement of the aortic sinus with a Dacron patch.

Vitamin C deficiency has been a historical disease rarely seen nowadays. We illustrate a case of a boy with autism presenting with severe pulmonary hypertension and refusal to walk secondary to vitamin C deficiency. Initiating treatment with high-dose vitamin C reversed his symptoms and he regained full power of his lower limbs with total normalisation of his pulmonary pressures.

Tetralogy of Fallot is often found in association with a wide variety of other cardiac lesions, but is rarely found in association with lesions causing obstruction to the left ventricular inflow or outflow. Subaortic stenosis has only rarely been reported in association with tetralogy of Fallot. We report a patient with Marfan syndrome who underwent repair of tetralogy of Fallot at five years of age. Discrete and progressive subaortic stenosis developed two years after the surgical correction, in a previously normal and unobstructed left ventricular outflow tract. Surgical removal of the acquired fibrous subaortic shelf was successful. Clinical signs of obstruction within the left ventricular outflow tract after surgical repair of tetralogy of Fallot should prompt further investigation to exclude the onset of acquired subaortic stenosis.

A symptomatic neonate was referred for aortic coarctation and, at cineangiography, was shown to have an interruption of the aortic arch at the isthmus together with coarctation related to a persistent fifth aortic arch. Balloon dilation of the coarctation was performed, but the coarctation was still evident after six months. A graft was then interposed between the left subclavian artery and the descending aorta. Balloon dilation was repeated five years later leaving a residual gradient of 11 mm Hg.

With advances in surgical technique, more children with complex congenital heart disease are surviving.1 Increasingly, attention is focusing on late morbidities, such as right ventricular dysfunction.2 For many common forms of congenital cardiac malformations, the assessment of right ventricular size and function is now essential so as to make optimal decisions concerning management.3 Commonly encountered examples include the timing for operative replacement of the incompetent pulmonary valve following surgical repair of tetralogy of Fallot, and the assessment of suspected ventricular dysfunction in children with functionally single or systemic right ventricles.4 Echocardiography remains the most practical method for serially determining right ventricular function, despite its limitations.5 The easy accessibility of echocardiography has encouraged its continued development as a tool for determining right ventricular function in neonates, infants, and children. A comparison of values established for the right ventricle is shown in Table 1, along with the references from which they were culled. We will describe these values in sequence.