Staged Surgical Palliation in Hypoplastic Left Heart Syndrome and Its Variants

Walter, Eva Maria B. Delmo; Hübler, Michael; Alexi-Meskishvili, Vladimir; Miera, Oliver; Weng, Yuguo; Loforte, Antonio; Berger, Felix; Hetzer, Roland

doi:10.1111/j.1540-8191.2008.00759.x

Cited by 21 publications

(9 citation statements)

References 42 publications

(98 reference statements)

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“…While outcomes of staged reconstruction in the present era have become truly spectacular in experienced centers, still a few infants and children fail to respond satisfactorily [16]. Transplantation has occasionally become necessary, and this has occurred after each stage.…”

Section: Clinical Issues In Infant Heart Transplantationmentioning

confidence: 99%

Heart Transplantation for Congenital Heart Disease in the First Year of Life

Chinnock¹,

Bailey²

2011

CCR

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Successful infant heart transplantation has now been performed for over 25 years. Assessment of long term outcomes is now possible. We report clinical outcomes for322 patients who received their heart transplant during infancy. Actuarial graft survival for newborn recipients is 59% at 25 years. Survival has improved in the most recent era. Cardiac allograft vasculopathy is the most important late cause of death with an actuarial incidence at 25 years of 35%. Post-transplant lymphoma is estimated to occur in 20% of infant recipients by25 years. Chronic kidney disease grade 3 or worse is present in 31% of survivors. The epidemiology of infant heart transplantation has changed through the years as the results for staged repair improved and donor resources remained stagnant. Most centers now employ staged repair for hypoplastic left heart syndrome and similar extreme forms of congenital heart disease. Techniques for staged repair, including the hybrid procedure, are described. The lack of donors is described with particular note regarding decreased donors due to newer programs for appropriate infant sleep positioning and infant car seats. ABO incompatible donors are a newer resource for maximizing donor resources, as is donation after circulatory determination of death and techniques to properly utilize more donors by expanding the criteria for what is an acceptable donor. An immunological advantage for the youngest recipients has long been postulated, and evaluation of this phenomenon may provide clues to the development of accommodation and/or tolerance.

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Section: Clinical Issues In Infant Heart Transplantationmentioning

confidence: 99%

Heart Transplantation for Congenital Heart Disease in the First Year of Life

Chinnock¹,

Bailey²

2011

CCR

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“…Staged univentricular palliation, with the right ventricle serving as the systemic pumping chamber, has transformed HLHS from a lethal condition to a survivable one. 1-3 However, perioperative mortality and long-term morbidity remain significant challenges. 4-7 …”

Section: Introductionmentioning

confidence: 99%

Fetal Aortic Valvuloplasty for Evolving Hypoplastic Left Heart Syndrome

et al. 2014

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Background Fetal aortic valvuloplasty (FAV) can be performed for severe mid-gestation aortic stenosis (AS) in an attempt to prevent progression to hypoplastic left heart syndrome (HLHS). A subset of patients has achieved a biventricular (BV) circulation after FAV. The postnatal outcomes and survival of the BV patients, compared to those managed as HLHS, have not been reported. Methods and Results We included 100 patients who underwent FAV for severe mid-gestation AS with evolving HLHS from March 2000 to January 2013. Patients were categorized based on postnatal management as BV or HLHS. Clinical records were reviewed. Eighty-eight fetuses were live-born, and 38 had a BV circulation (31 from birth, 7 converted after initial univentricular palliation). Left-sided structures, namely aortic and mitral valve sizes and LV volume, were significantly larger in the BV group at the time of birth (p-values <0.01). After a median follow-up of 5.4 years, freedom from cardiac death among all BV patients was 96±4% at 5 years and 84±12% at 10 years, which was better than HLHS patients (log-rank p=0.04). There was no cardiac mortality in patients with a BV circulation from birth. All but 1 of the BV patients required postnatal intervention; 42% underwent aortic and/or mitral valve replacement. On most recent echocardiogram, the median LV end-diastolic volume z-score was +1.7 (range: -1.3, +8.2), and 80% had normal ejection fraction. Conclusions Short- and intermediate-term survival among patients who underwent FAV and achieved a BV circulation postnatally is encouraging. However, morbidity still exists, and on-going assessment is warranted.

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“…32, 33 Many other recent studies of the outcomes after S2P have not found the timing of S2P to be associated with outcomes. 14, 16, 34, 35 Notably, risk factors for death or heart transplantation after S2P have not yet been reported in SVR Trial study participants. Schwartz, et al instead reported risk factors for prolonged hospital length of stay after S2P.…”

Section: Discussionmentioning

confidence: 99%

The Optimal Timing of Stage 2 Palliation for Hypoplastic Left Heart Syndrome

et al. 2017

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Background In infants requiring three-stage single ventricle palliation for hypoplastic left heart syndrome, attrition after the Norwood procedure remains significant. The effect of the timing of stage-2-palliation (S2P), a physician-modifiable factor, on long term survival is not well understood. We hypothesized that an optimal interval between the Norwood and S2P that both minimizes pre-S2P attrition and maximizes post-S2P survival exists and is associated with individual patient characteristics. Methods The NIH/NHLBI Pediatric Heart Network Single Ventricle Reconstruction Trial public dataset was used. Transplant-free survival (TFS) was modeled from (1) Norwood to S2P and (2) S2P to three years, using parametric hazard analysis. Factors associated with death or heart transplantation were determined for each interval. To account for staged procedures, risk-adjusted, three-year, post-Norwood TFS (the probability of TFS at three years given survival to S2P) was calculated using parametric conditional survival analysis. TFS from the Norwood to S2P was first predicted. TFS after S2P to three years was then predicted and adjusted for attrition before S2P by multiplying by the estimate of TFS to S2P. The optimal timing of S2P was determined by generating nomograms of risk-adjusted, three-year, post-Norwood, TFS versus the interval from the Norwood to S2P. Results Of 547 included patients, 399 survived to S2P (73%). Of the survivors to S2P, 349 (87%) survived to three-year follow-up. The median interval from the Norwood to S2P was 5.1 (IQR 4.1–6.0) months. The risk-adjusted, three-year, TFS was 68±7%. A Norwood-S2P interval of three to six months was associated with greatest three-year TFS overall and in patients with few risk factors. In patients with multiple risk factors, TFS was severely compromised, regardless of the timing of S2P and most severely when S2P was performed early. No difference in the optimal timing of S2P existed when stratified by shunt type. Conclusions In infants with few risk factors, progressing to S2P at three to six months after the Norwood procedure was associated with maximal TFS. Early S2P did not rescue patients with greater risk factor burdens. Instead, referral for heart transplantation may offer their best chance at long term survival. ClinicalTrials.gov number: NCT00115934.

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Staged Surgical Palliation in Hypoplastic Left Heart Syndrome and Its Variants

Cited by 21 publications

References 42 publications

Heart Transplantation for Congenital Heart Disease in the First Year of Life

Heart Transplantation for Congenital Heart Disease in the First Year of Life

Fetal Aortic Valvuloplasty for Evolving Hypoplastic Left Heart Syndrome

The Optimal Timing of Stage 2 Palliation for Hypoplastic Left Heart Syndrome

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