Richard Martinez

Right ventricular pulsus alternans during acute increase in afterload during balloon pulmonic valvuloplasty

Abstract Pulsus alternans is characterized by a regular rhythm with alternation of strong and weak contractions. It is a phenomenon generally ascribed to severe myocardial dysfunction and occurs most commonly in patients with heart failure secondary to increased resistance to left ventricular ejection. It has also been described in coronary artery disease and in idiopathic dilated cardiomyopathy. Although pulsus alternans has more often been described in advanced myocardial disease, it has also been described in acute, transient ischemia. 1 Right ventricular (RV) alternans has been described previously, but usually in patients with congestive heart failure or severe RV dysfunction, or both. 2,3 The present report describes RV pulsus alternans that occurred acutely during balloon pulmonary valvotomy.

Echocardiographic features of the morphologically right ventriculo-arterial junction.

In the normal heart, the morphologically right ventricle supports the pulmonary trunk. The key to echocardiographic evaluation of the junction between these structures is to understand not only the arrangement of the pulmonary valve, but also the complete muscular infundibulum that supports the valvar leaflets, lifting the valvar complex away from the base of the ventricular mass. As explained in the previous review,1 it is the presence of this free-standing muscular infundibular sleeve that makes it possible for the surgeon to remove the pulmonary valve for use as an allograft in the Ross procedure.2 In this review, we will address the echocardiographic features of the junction, considering primarily the situation in which the morphologically right ventricle supports the pulmonary trunk, but making comparisons with the abnormal arrangements in which either the aorta, or both arterial trunks, arise from the right ventricle. As we will see, the basic arrangement of the free-standing complete muscular infundibulum, or conus, is preserved with these abnormal arrangements, but there can be variation depending on the precise arrangement of the inner heart curvature, or ventriculo-infundibular fold, and the morphology can be further changed depending on the relationship of the arterial trunks themselves. The key to analysis, therefore, and also to accurate description, is to analyse separately the way in which the arterial trunks are joined to the ventricular mass, the relationships of the trunks one to the other, and the precise structure of the supporting right ventricular outflow tract, or outflow tracts in the setting of double outlet connection. If each of these features is then described in its own right, avoiding the temptation to make inferences regarding one feature from knowledge of another, then it is possible to avoid many of the persisting controversies relating to nomenclature.

Long-term management of patients with hypoplastic left heart syndrome: the diagnostic approach at All Children's Hospital

Improved survival in children with hypoplastic left heart syndrome has created a sub-population of children and young adults who are living with functionally univentricular physiology. Routine surveillance with comprehensive screening for structural cardiac disease, functional cardiac disease, arrhythmias, thromboembolic disease, and associated dysfunction of end organs is important. Future directives will better define the plans of care for routine surveillance in patients with hypoplastic left heart syndrome.

Long-term management of adults with conotruncal lesions: the diagnostic approach at All Children's Hospital

Improved survival in children with complex congenital cardiac disease, such as conotruncal abnormalities, has created a sub-population of children and young adults who need comprehensive multi-disciplinary long-term follow-up. Routine surveillance with comprehensive screening for structural heart disease, functional heart disease, thromboembolic disease, arrhythmias, and associated end-organ dysfunction is important. Future research will better define the care plans for routine surveillance in patients with conotruncal abnormalities.