We report a unique case of an adolescent patient with Fontan physiology presenting with unconjugated hyperbilirubinemia due to dehiscence of a tricuspid valve annuloplasty ring.

The timing of pulmonary valve replacement in patients with pulmonary regurgitation following treatment of pulmonary stenosis is undefined. Although cardiac magnetic resonance-based right ventricular volumes in tetralogy of Fallot patients have been used as a guide in pulmonary stenosis patients, anatomic differences between tetralogy of Fallot and pulmonary stenosis patients complicate their application to pulmonary stenosis patients and could result in late referral for pulmonary valve replacement. We sought to determine if pulmonary stenosis patients referred for pulmonary valve replacement were at greater risk for morbidity or need for tricuspid valve intervention at the time of pulmonary valve replacement. A retrospective cohort study was performed on all adult patients with a diagnosis of pulmonary stenosis or tetralogy of Fallot followed at our centre. Clinical and imaging-based exposures were collected. Pre-specified endpoints included need for concomitant tricuspid valve repair or replacement and pre- and post-pulmonary valve replacement cardiac magnetic resonance-based volumetric measurements. Between 1/1999 and 1/2020, 235 patients underwent pulmonary valve replacement for pulmonary regurgitation (52 with pulmonary stenosis, 183 with tetralogy of Fallot). Pulmonary stenosis patients were more likely to have at least moderate tricuspid regurgitation (p = 0.010), undergo concomitant tricuspid valve intervention (p = 0.003), and require tricuspid valve repair or replacement secondary to annular dilation (p = 0.027) compared to tetralogy of Fallot patients. There was no difference in pre-pulmonary valve replacement right ventricular size between pulmonary stenosis and tetralogy of Fallot patients. These findings suggest that referral for pulmonary valve replacement may be occurring later in the disease course for pulmonary stenosis patients.

We report an innovative treatment strategy for fetal Ebstein’s anomaly with a circular shunt. We used transplacental non-steroidal anti-inflammatory drugs, at the 29th gestational week, to constrict the ductus arteriosus avoiding fetal demise. We addressed the critical neonate with an urgent Starnes procedure. Finally, instead of following the usual single-ventricle palliation pathway after the Starnes procedure, we achieved successful two-ventricle repair with the cone technique at 5 month old.

Systemic right ventricular function in congenitally corrected transposition depends on septal geometry. Suprasystemic left ventricular systolic pressures and high end-diastolic pressures after Fontan surgery paradoxically preserve right ventricular function.

This brief report describes a case of flail anterior tricuspid valve leaflet in a neonate associated with maternal antiphospholipid syndrome. Fetal echocardiography at 27 weeks of gestation showed competent atrioventricular valves with biventricular echogenic chordae. Fetal distress was noted at delivery, and echocardiography showed a flail anterior leaflet of the tricuspid valve with severe regurgitation. Possible causation and implications of maternal antiphospholipid syndrome are discussed.

Cor triatriatum sinister is a very rare cardiac anomaly that may lead to pulmonary hypertension, right ventricular dilation, and eventually right heart failure. We report a case of a toddler who presented with respiratory distress and cardiomegaly and was found to have cor triatriatum sinister with a restrictive communication, decompressing vertical vein, pulmonary hypertension, severe tricuspid regurgitation, and severe right ventricular dysfunction. She underwent a successful surgical repair, with normalisation of right ventricular function and pulmonary artery pressure.

Valvar cardiac disease accounts for a large percentage of heart lesions in the paediatric population. There are a variety of congenital and acquired valvar lesions produced by different mechanisms that produce unique physiologies. With the development of more advanced cardiac interventional procedures and congenital cardiac surgery techniques, most of these lesions can be repaired in a single or multi-stage approach. Given the potential for significant symptomatology before intervention, it is imperative to review the critical care management of paediatric valvar heart disease. The purpose of this article is to provide an overview of the current critical care management of valvar cardiac disease. We approach each cardiac valve individually in order to review the pathophysiology caused by valve dysfunction (obstruction and incompetence) and elaborate on the most frequent diagnoses, specific cardiac physiology, potential for other organ system associations, and perioperative management.

A 24-year-old female without previous symptoms or known heart disease consulted a general practitioner with dyspnoea which had slowly worsened over a period of two years. She was treated for asthma, without investigations, until she presented with rapid atrial fibrillation, chest pain and pulmonary oedema necessitating admission to hospital, where she was found to have “mitral” regur-gitation (of the left-sided atrioventricular valve) and congenitally corrected transposition. At operation the grossly regurgitant left-sided morphologically tricuspid valve was replaced by a “top-hat” inverted aortic valve homograft. The procedure resulted in intermittent heart block, needing a pacemaker, and continued myocardial failure which progressed with new right-sided valvar regurgitation. She died a few months later. Post mortem examination confirmed the double discordance, and showed a fistula around the tricuspid valvar prosthesis, left atrial thrombus and changes in the mitral valve. There was also an unsuspected area of old myocardial ischaemia, associated with severe hypoplasia of the circumflex branch of the right-sided coronary artery. This was considered probably to have been of embolic origin.

Background: Our study focusses on echocardiographic assessment of the right ventricular diastolic function and adaptive right ventricular response to volume overload resulting from pulmonary and tricuspid regurgitation in surgically treated patients with tetralogy of Fallot. Method and results: We included 60 patients subsequent to surgical correction of tetralogy of Fallot, dividing them into two groups – a group of 18 patients with restrictive physiology, having antegrade flow to the pulmonary arteries greater than 30 centimetres per second in late diastole in five consecutive beats, and a group of 42 patients with non-restrictive physiology. Based on the cardiothoracic ratio, being more or equal to, or less than 0.55, we further divided those with restrictive physiology into a group of 14 patients deemed to have primary restriction, and the other 4 patients considered to have secondary or acquired restriction. Those with non-restrictive physiology were divided into groups of 16 patients with a small heart, and 26 patients with a large heart. A fraction of the venous retrograde diastolic flow in the hepatic vein greater or equal to 30 centimetres is important for distinguishing between the subgroup with primary restriction and the other subgroups. In the four patients with secondary restriction, anterograde diastolic flow in the pulmonary artery greater than 30 centimetres per second was recorded after the average period of follow-up of 2.4 years. The mean value of the pulmonary regurgitant jet pressure half-time was higher in the subgroup with the secondary restriction in comparison to the nonrestrictive subgroup with large hearts at 152 milliseconds with standard deviation of 36 milliseconds versus 85 milliseconds with standard deviation of 11 milliseconds, p less than 0.05. This was significantly lower in comparison to those with primary restriction, where the value was 238 milliseconds, with standard deviation of 42 milliseconds, p less than 0.02. Conclusion: Echocardiographic analysis offers great possibilities for assessment of right ventricular diastolic function, identifying in particular those with restrictive physiology, its interrelation with pulmonary and tricuspid regurgitation, as well as timing and selection of patients for re-intervention.

The outlook for patients with hypoplastic left heart syndrome has dramatically improved over the past two decades. Universally fatal only 25 years ago, since that time outcomes for staged palliation have shown consistent improvement. Recent reports show that eight to nine patients from every ten can now leave the hospital after the Norwood procedure.1 Attrition following the Norwood procedure, nonetheless, remains significant, with from five to fifteen percent of patients dying between the first and second stages of the Norwood sequence.1–4 Only three-quarters of the patients undergoing surgery for hypoplastic left heart syndrome survive after five years, even at the centres reporting the best outcomes for the Norwood procedure.1,5 In addition to the deaths, some patients are unable to progress through the three stages of reconstruction, and may require cardiac transplantation, or have no options for further therapy. There are many causes for these mortalities and morbidities following the Norwood procedure, including elevated pulmonary vascular resistance, cardiac arrhythmias, coronary arterial insufficiency, right ventricular failure, right ventricular volume overload due to shunt-dependent physiology, and tricuspid valvar regurgitation. Many of these factors are interrelated, and may form feedback loops, which serve to propagate their adverse effects on patients with hypoplastic left heart syndrome.