Management of Hypoplastic Left Heart Syndrome in Low-Resource Settings and the Ethics of Decision-Making.

Hypoplastic left heart syndrome (HLHS) is possibly the most challenging congenital heart defect to confront in any setting. The highly specialized infrastructure and resources needed to treat HLHS is not available in many low-resource settings. However, low-resource settings must not be assumed to be synonymous with low- and middle-income countries as national income is not necessarily indicative of a country's prioritization of healthcare resources. Besides, a low-resource setting may be institution-specific as well as country-specific. We have stratified institutional capabilities for addressing the requirements of treatment for HLHS into five levels based on the capacity for diagnosis, intervention, and post-discharge monitoring. Depending on institutional capabilities, children born with HLHS in low-resource settings experience a spectrum of outcomes ranging from death without diagnosis to the hybrid or Norwood stage 1 palliation. The decision-making is ethically challenging when resources are scarce and economic efficiency must be considered in the context of distributive justice. Even in settings that would be classified as resource-rich where survival after surgery and quality of life afterward keep improving, not every parent would choose surgical intervention for their hypothetical child with HLHS.

Congenital division of the left atrium (cor triatriatum) in the setting of congenitally corrected transposition of the great arteries.

Congenital division of the left atrium (cor triatriatum) and congenitally corrected transposition of the great arteries are both rare congenital cardiac malformations; their coexistence is exceedingly rare with only two previous reports identified in the literature. This combination of lesions is characterized by a pressure-loaded morphologically left ventricle and a propensity for pulmonary edema dependent on the degree of pulmonary venous obstruction caused by the dividing left atrial shelf. The probable impact of this on the natural history and surgical decision making is discussed.

The spectrum of double-outlet right atrium including hearts with three atrioventricular valves.

Double-outlet right atrium (DORA) is characterized by simultaneous right atrial emptying into both ventricles. Ventriculoatrial septal malalignment is the cardinal morphological feature. Three cases are presented to depict two major types of DORA-DORA with a malaligned atrial septum and DORA with a malaligned ventricular septum. We describe two subtypes of each form of DORA: DORA with a malaligned atrial septum presents with either a common atrioventricular (AV) junction (guarded by a common AV valve) or with a single AV junction (due to the absence of the left AV junction). DORA with a malaligned ventricular septum may be associated with a right ventricle (RV) that is adequate for biventricular repair or a severely hypoplastic RV not compatible with biventricular repair. DORA with a malaligned ventricular septum is closely related to typical straddling of the tricuspid valve. Peculiarly, DORA with a malaligned ventricular septum presents three AV valves at the AV junction and is associated with an abnormal disposition of the AV conduction axis. Clear understanding of the morphology of these lesions is important in preventing a surgical misadventure at the crux of the heart.

Late primary arterial switch for transposition of the great arteries with intact ventricular septum in an african population.

The arterial switch operation (ASO) is the optimal management of transposition of the great arteries with intact ventricular septum (TGA-IVS) within the first 3 weeks of life; beyond this age optimal treatment is debatable. The authors adopted a strategy of primary ASO for TGA-IVS in the first 10 weeks of life regardless of left ventricular (LV) status. This report reviews the early outcomes with this management approach. Between August 2006 and December 2009, 22 patients with TGA-IVS underwent the primary ASO. Sixteen of them were less than 21 days old (early switch group) and 6 were between 31 and 66 days old (late switch group). A review of their hospital records was performed to determine outcomes in the 2 groups. Operative variables and postoperative outcomes were recorded. There was 1 hospital death in the early switch group (6.3%) but none in the late group (0%). Temporary mechanical circulatory support was required in 1 patient (6.3%) in the early switch group and in 2 of the 6 (33.3%) in the late switch group. One late death of undetermined cause occurred in the late switch group 8 weeks after discharge. No significant difference could be demonstrated between the 2 groups in terms of operative variables and the measured postoperative outcomes. It is concluded that the age limit for the primary ASO can be extended to at least 10 weeks; temporary mechanical circulatory support may be required as a rescue.

Primary arterial switch operation for transposition of the great arteries with intact ventricular septum--is it safe after three weeks of age?

In patients with transposition of the great arteries with intact ventricular septum (TGA-IVS), late presentation for surgical treatment is not uncommon. Earlier experience suggested an excess mortality for the primary arterial switch operation (ASO) when performed after the third week of life in such patients. The true age limit for the primary ASO, however, remains speculative. A best evidence topic was written to determine the safety of extending the age limit of the primary ASO for TGA-IVS beyond three weeks of age. Eight of 74 relevant papers constituted the best evidence to address the issue. Sufficient level II evidence was found to suggest that in experienced institutions in the current era, the ASO for TGA-IVS may be performed primarily in infants three to eight weeks of age with comparable early outcomes to younger patients. Kang et al. investigated 275 neonates (younger than 21 days) and 105 infants (age range 21-185 days) undergoing the primary ASO for TGA-IVS. They found no significant difference in terms of in-hospital mortality (5.5% vs. 3.8%) or need for mechanical left ventricular (LV) support (3.6% vs. 5.7%) between the younger and older groups, respectively. Of the younger group, 3.8% developed postoperative LV failure leading to death or mechanical LV support. All nine patients older than two months (age range 61-185 days) survived to discharge although two (aged 69 and 86 days) required mechanical LV support for postoperative LV failure. The postoperative course of the late ASO group, however, was significantly prolonged as reflected in the duration of postoperative ventilation (4.9 vs. 7.1 days, P=0.012) and length of postoperative stay (12.5 vs. 18.9 days, P=0.001). In the report of Sarris et al., 52 patients with TGA-IVS who were older than four weeks of age (36 were older than eight weeks) underwent a primary ASO with early mortality comparable with younger patients (2% vs. 3%, respectively). For infants between the ages of two and six months, the strength of the evidence favoring this management approach is limited by the small sample size of reported series and the increased requirement for mechanical circulatory support for postoperative LV failure.