Management of right ventricular outfl ow tract obstruction: Evolution to revolution

Percutaneous pulmonary valves have opened up new avenues of treatment in patients requiring pulmonary valve replacement. The management of tetralogy of Fallot demonstrated the evolution of treatment: from palliativecare to modern day early complete surgical repair. Use of trans-annular patches to treat right ventricle outfl ow obstruction gave rise to signifi cant pulmonary regurgitation. Clinicians considered this a benign condition until,three decades later patients started dying unexpectedly. Surgical pulmonary valve replacement was the only treatment, but these showed dysfunction after some years. Clear guidelines for intervention do not exist. Current guidelines recommend treatment at upper limits of tolerance. Arguments for earlier intervention are presented, but none of the guidelines have been scientifically validated

Right ventricular outfl ow tract revalvulation using the Melody Valve: The next frontier

Congenital heart lesions with abnormal right ventricular outfl ow tract anatomy will require early surgical intervention. Re-intervention rates in these patients are high due to homograft degeneration. Until recently, surgery was the onlytreatment option for right ventricular outfl ow tract dysfunction. Percutaneous pulmonary valve implantation has been introduced as a new therapeutic alternative to prolong conduit life span and to reduce surgical re-intervention rates. Short- and mid-term results have been favourable and showed that it is a safe and effective therapy. These are reviewed with emphasis on the Melody valve

Is Stent Placement Effective for Palliation of Right Ventricle to Pulmonary Artery Conduit Stenosis?

ObjectivesThis study was designed to evaluate the outcome of stent placement (SP) for conduit discrete stenosis using predefined criteria.BackgroundRight ventricle (RV) to pulmonary artery (PA) conduits are often associated with complications, such as stenosis, requiring multiple surgical replacements.MethodsPatients who underwent primary or repeat SP were included. Indications for SP were clinical symptoms and/or RV to systolic blood pressure (SBP) ratio (RV:SBP) >0.65 by echocardiography. Our definition of success was a decrease in RV:SBP by >20%, a final RV:SBP ratio of <0.65, or resolution of symptoms.ResultsStents were placed successfully in 28 of 31 patients (90%), including 3 patients who underwent the procedure solely for symptoms. The RV:SBP ratio decreased (0.75 ± 0.17 vs. 0.52 ± 0.12, p < 0.001), and the conduit diameter increased (postero-anterior 9.1 ± 2.9 vs. 12.0 ± 2.8 mm, lateral 8.3 ± 2.2 vs. 11.6 ± 2.4 mm, p < 0.001). In the 28 patients with successful SP, 8 (29%) remained free from second intervention. In the remaining patients, the median time to re-intervention was 16 months (range 6 to 44 months). Second transcatheter interventions (4 SP, 4 balloon dilation) were successful in 8 of 13 patients. Complications included balloon rupture (n = 4), stent fracture (n = 2), and pseudoaneurysm formation (n = 1).ConclusionsInitial SP has excellent intermediate outcomes, successfully postponing surgical intervention for the majority of patients. Conduit restenosis may be successfully treated with a second transcatheter intervention. On the basis of these data, SP is likely the procedure of choice for patients with a discrete stenosis of the RV to PA conduit

A retrospective analysis of the duration of mechanical ventilation in Scandinavian paediatric heart centres

Aim Early extubation after cardiac surgery shortens paediatric intensive care unit (PICU) length of stay (LOS) and decreases complications from mechanical ventilation (MV). We explored the duration of MV in Scandinavian paediatric heart centres. Methods We retrospectively reviewed the MV duration and PICU LOS of 696 children operated for atrial septal defect (ASD), ventricular septal defect (VSD), tetralogy of Fallot (TOF) or total cavopulmonary connection (TCPC) in four Scandinavian centres in 2015-2016. Neonates (n = 90) were included regardless of heart surgery type. Results Patients with ASD were extubated at a median of 3.25 h (interquartile range [IQR] 2.00-4.83), followed by patients with TCPC (median 5.00 h, IQR 2.60-16.83), VSD (median 7.00 h, IQR 3.69-22.25) and TOF (median 18.08 h, IQR 6.00-41.38). Neonates were not extubated early (median 94.42 h, IQR 45.03-138.14). Although MV durations were reflected in PICU LOS, this was not as apparent among those extubated within 12 h. The Swedish centres had shortest MV durations and PICU LOS. Extubation failed in 24/696 (3.4%) of patients. Conclusion Scandinavian paediatric heart centres differed in the duration of postoperative MV. Deferring extubation up to 12 h postoperatively did not markedly prolong PICU LOS.Peer reviewe

Haemodynamic consequences of targeted single- and dual-site right ventricular pacing in adults with congenital heart disease undergoing surgical pulmonary valve replacement

Aims The purpose of this study was to create an epicardial electroanatomic map of the right ventricle (RV) and then apply post-operative-targeted single- and dual-site RV temporary pacing with measurement of haemodynamic parameters. Cardiac resynchronization therapy is an established treatment for symptomatic left ventricular (LV) dysfunction. In congenital heart disease, RV dysfunction is a common cause of morbidity—little is known regarding the potential benefits of CRT in this setting. Methods and results Sixteen adults (age = 32 ± 8 years; 6 M, 10 F) with right bundle branch block (RBBB) and repaired tetralogy of Fallot (n = 8) or corrected congenital pulmonary stenosis (n = 8) undergoing surgical pulmonary valve replacement (PVR) for pulmonary regurgitation underwent epicardial RV mapping and haemodynamic assessment of random pacing configurations including the site of latest RV activation. The pre-operative pulmonary regurgitant fraction was 49 ± 10%; mean LV end-diastolic volume (EDV) 85 ± 19 mL/min/m2 and RVEDV 183 ± 89 mL/min/m2 on cardiac magnetic resonance imaging. The mean pre-operative QRS duration is 136 ± 26 ms. The commonest site of latest activation was the RV free wall and DDD pacing here alone or combined with RV apical pacing resulted in significant increases in cardiac output (CO) vs. AAI pacing (P < 0.01 all measures). DDDRV alternative site pacing significantly improved CO by 16% vs. AAI (P = 0.018), and 8.5% vs. DDDRV apical pacing (P = 0.02). Conclusion Single-site RV pacing targeted to the region of latest activation in patients with RBBB undergoing PVR induces acute improvements in haemodynamics and supports the concept of ‘RV CRT’. Targeted pacing in such patients has therapeutic potential both post-operatively and in the long term

The management of tetralogy of Fallot after corrective surgery

With the success of contemporary medical and surgical management in congenital heart disease (CHD), adults with repaired CHD now often outnumber their paediatric equivalents. Tetralogy of Fallot (TOF) has paved the way, not only in the management of native CHD, but also in the management of its repaired form. In this review, we discuss the current surveillance and management of adults with repaired TOF, highlighting outcomes related to these practices

Transcatheter ablation of arrhythmias associated with congenital heart disease

The improvement of surgical techniques resulted in significant life prolongation of many young patients with congenital heart disease (CHD). However, as these patients reach adulthood, their risk for late complications associated with surgery is also increased. One of the most difficult challenges associated with CHD is the high incidence of cardiac arrhythmias that arise from either the myocardial substrate created by abnormal physiology (pressure/volume changes, septal patches, and suture lines) or the presence of surgical scar. Catheter ablation is proven to be effective in treating atrial and ventricular arrhythmias in structurally normal hearts, and has also been used to treat arrhythmias in adults with congenital heart disease. In this review we provide an overview about diagnostic challenges, mapping and ablation techniques and outcome of patients undergoing transcatheter ablation procedures

Branching out:CRT beyond current concepts

Patients suffering from heart failure and delay in electrical activation of the heart can be treated with cardiac resynchronization therapy (CRT). This treatment restores the synchronous contraction of the two large cardiac chambers (ventricles) using a pacemaker. In this PhD thesis some relatively unexplored facets of CRT are investigated. One of these facets concerns the effects of CRT on electrical recovery of the cardiac cells (repolarization). A good and more or less simultaneous repolarization is important in the prevention of arrhythmias. Measurements in patients showed that important changes in the part of the electrocardiogram that represents repolarization already occurred within two weeks of starting CRT. Using a computer model, we demonstrated that these changes were indicative of more simultaneous repolarization. Moreover, we found that a bigger change in repolarization was linked to a larger improvement in cardiac function. These results can contribute to (research into) better treatment of patients with heart failure