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Children’s Hospital Boston, Cardiology Department, 300 Longwood Ave, Boston, MA 02115
(Email: james.lock{at}cardio.chboston.org; audrey.marshall{at}cardio.chboston.org).
No one rushes to publish poor results. Well-conceived and aptly executed novel approaches to difficult diseases often remain in the realm of anecdote or even folklore when the outcomes are unsatisfactory. In contrast, reports of small, seemingly successful experiments can be identified throughout the history of pediatric cardiology, even when subsequent and largely unreported experiences prove initial results to be misleading [1, 2]. Thus, the study presented by Glatz and colleagues [3] should be applauded, for it documents one institution’s carefully considered and systematic approach to hypoplastic left heart syndrome with atrial restriction (HLHS/IAS) and shares the results of this approach, despite disappointing outcomes.
Although most of the infants in this study had a prenatal diagnosis of HLHS (68%), outcomes were poor overall. A subset of these prenatally diagnosed fetuses were managed using a strategy of prenatal diagnosis coupled with immediate, primarily surgical intervention. In this small group, only 28% (2 of 7) neonates survived. On the basis of the poor survival in this group of neonates, the authors concluded, reasonably, that this anticipatory management approach was a failure. Coming from a hospital that has added significantly to what we understand about this lesion, the description of this institutional approach and its shortcomings provides valuable information to others struggling to find a successful approach to this disease.
In HLHS/IAS, clinical decompensation and demise can occur immediately at birth, irrespective of an early and accurate diagnosis of single-ventricle heart disease, initiation of prostaglandin infusion, and appropriate resuscitation. Improvement in outcomes requires not only accurate fetal diagnosis and expert medical management but also successful intervention to create an adequate left atrial outlet. The lack of a relationship between prenatal diagnosis and survival in the patients described here may be confounded by the effects of surgical septectomy or emergency stage 1 Norwood.
As the authors speculate in their comments, an operation with cardiopulmonary bypass in the setting of severe pulmonary congestion may negate any benefit of prenatal diagnosis. More effective postnatal intervention may be achieved using other techniques such as transcatheter intervention. Alternatively, when fetuses at risk for severe postnatal left atrial hypertension can be reliably identified by fetal echocardiography, the authors have suggested that fetal intervention for left atrial decompression should be considered. Either way, fetal diagnosis may well be much more helpful than appears at first glance.
Like progressive pulmonary venous stenosis, HLHS/IAS is a disease that will require research and innovation for a successful outcome [4, 5]. The likelihood of progress through innovation in the treatment of these rare and highly lethal disease entities is maximized when poor results are published promptly and self-critically. On several fronts, Glatz and colleagues are to be congratulated for this work.
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