Valvular pathology in infants and children poses numerous challenges to the paediatric cardiac surgeon. Without question, valvular repair is the goal of intervention because restoration of valvular anatomy and physiology using native tissue allows for growth and a potentially better long-term outcome. When reconstruction fails or is not feasible, valve replacement becomes inevitable. Which valve for which position is controversial. Homograft and bioprosthetic valves achieve superior haemodynamic results initially but at the cost of accelerated degeneration. Small patient size and the risk of thromboembolism limit the usefulness of mechanical valves, and somatic outgrowth is an universal problem with all available prostheses. The goal of this article is to address valve replacement options for all four valve positions within the paediatric population. We review current literature and our practice to support our preferences. To summarize, a multitude of opinions and surgical experiences exist. Today, the valve choices that seem without controversy are bioprosthetic replacement of the tricuspid valve and Ross or Ross-Konno procedures when necessary for the aortic valve. On the other hand, bioprostheses may be implanted when annular pulmonary diameter is adequate; if not or in case of right ventricular outflow tract discontinuity, it is better to use a pulmonary homograft with the Ross procedure. Otherwise, a valved conduit. Mitral valve replacement remains the most problematic; the mechanical prosthesis must be placed in the annular position, avoiding oversizing. Future advances with tissue-engineered heart valves for all positions and new anticoagulants may change the landscape for valve replacement in the paediatric population.

BACKGROUND Optimal treatment of right ventricular (RV) dysfunction observed in patients after tetralogy of Fallot (TOF) repair is unclear. Studies of biventricular (BiV) stimulation in patients with congenital heart disease have been retrospective or have included patients with heterogeneous disorders. OBJECTIVE The purpose of this study was to determine the effects on cardiac function of stimulating at various cardiac sites in an animal model of RV dysfunction and dyssynchrony and in eight symptomatic adults with repaired TOF. METHODS Pulmonary stenosis and regurgitation as well as RV scars were induced in 15 piglets to mimic repaired TOF. The hemodynamic effects of various configurations of RV and BiV stimulation were compared with sinus rhythm (SR) 4 months after surgery. In eight adults with repaired TOF, RV and left ventricular (LV) dP/dtmax were measured invasively during SR, apical RV stimulation, and BiV stimulation. RESULTS At 4 months, RV dilation, dysfunction, and dyssynchrony were present in all piglets. RV stimulation caused a decrease in LV function but no change in RV function. In contrast, BiV stimulation significantly improved LV and RV function (P< .05). Echocardiography and epicardial electrical mapping showed activation consistent with right bundle branch block during SR and marked resynchronization during BiV stimulation. In patients with repaired TOF, BiV stimulation increased significantly RV and LV dP/dtmax (P< .05). CONCLUSION In this swine model of RV dysfunction and in adults with repaired TOF, BiV stimulation significantly improved RV and LV function by alleviating electromechanical dyssynchrony.

Commonly used echocardiography variables, such as tricuspid annular plane systolic excursion and tricuspid annular peak systolic velocity, did not sensitively evaluate global RVEF. A global approach, that includes the whole RV and integration of its different components, was more reliable in patients with rTOF.

(1) Early mortality is related to the severity of preoperative left ventricular dysfunction; it may be reduced by a careful use of postoperative cardiac support techniques. (2) Late results are satisfactory and left ventricular function always recovers. (3) Mitral regurgitation improves along with left ventricular function, but recovery may be incomplete and need reoperation. The data suggest that mitral valve surgery is probably not indicated at initial surgery, except in selected cases with a low potential of recovery (severe regurgitation with relatively well-preserved left ventricular function).

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Improvements in cardiac imaging have recently focused a great interest on the right ventricle (RV). In patients with congenital heart disease, the right ventricle (RV) may support the systemic circulation (systemic RV). There are 2 different anatomic conditions providing such physiology: the congenitally corrected transposition of the great arteries (ccTGA) and the TGA surgically corrected by atrial switch. During the last decades, evidence is accumulating that progressive systemic RV failure develops leading to considerable morbidity and mortality. Various imaging modalities have been used to evaluate the systemic RV, but echocardiography is still predominantly used in clinical practice, allowing an anatomic and functional approach of the systemic RV function and the potential associated anomalies. The goal of this review is to offer a clinical perspective of the non-invasive evaluation of the systemic RV by echocardiography.

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