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Surgical Palliation or Primary Transplantation for Aortic Valve Atresia

Published on Apr 1, 2020in The Journal of Thoracic and Cardiovascular Surgery5.261
· DOI :10.1016/j.jtcvs.2019.08.104
Kathryn A. Stackhouse4
Estimated H-index: 4
(BIDMC: Beth Israel Deaconess Medical Center),
Brian W. McCrindle88
Estimated H-index: 88
+ 7 AuthorsGösta B. Pettersson42
Estimated H-index: 42
(Cleveland Clinic)
Abstract
Abstract Objectives To describe surgical pathway progression through adolescence of an inception cohort of neonates with aortic valve atresia managed initially with either surgical palliation or primary transplantation, comparing survival and self-reported health-related quality of life. Methods From 1994–2000, 565 neonates with aortic atresia were admitted to 26 Congenital Heart Surgeons’ Society hospitals and followed annually for vital status. Initial management included surgical palliation (n=453) and primary cardiac transplantation (n=68). PedsQL health-related quality-of-life questionnaires were sent cross-sectionally to a subgroup of 198 patients alive at previous follow-up, with 80 responses. Results Risk of death was initially high for both treatment strategies. However, compared with initial surgical palliation, survival with primary transplantation, including waitlist mortality, was greater and persisted long-term (65% vs 40% at 15 years; P=.002). Survival after secondary transplantation (48% at 9 years) was lower than after primary, 74%. Health-related quality-of-life total score was lower overall than that of the general adolescent population (71±16 vs 84±13; P=.0001; normal=100), but similar to that of adolescents with chronic diseases. It was similar in the surgical palliation and primary transplantation groups (70±16 vs 75±15; P=.3). Surgical palliation patients reported more symptoms (76±15 vs 63±18; P=.02). Conclusions Patients receiving primary heart transplantation for aortic atresia in 1994–2000 experienced better survival, fewer symptoms, and equivalent quality of life compared with those undergoing initial surgical palliation. Notwithstanding limited availability of neonatal and infant donor hearts, primary transplantation may be considered for those neonates with risk factors predictive of exceptionally poor survival after surgical palliation.
  • References (39)
  • Citations (1)
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References39
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#1Brian H. Morray (UW: University of Washington)H-Index: 4
#2E. Albers (UW: University of Washington)H-Index: 5
Last. Yuk M. Law (UW: University of Washington)H-Index: 19
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Neonatal heart transplantation was developed and established in the 1980’s as a durable modality of therapy for complex-uncorrectable heart disease. Patients transplanted in the neonatal period have experienced unparalleled long-term survival, better than for any other form of solid-organ transplantation. However, the limited availability of neonatal and young infant donors has restricted the indications and applicability of heart transplantation among newborns in the current era. Indications fo...
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#1James M. Meza (U of T: University of Toronto)H-Index: 8
#2Edward J. Hickey (U of T: University of Toronto)H-Index: 19
Last. Robert D.B. Jaquiss (Children's Medical Center of Dallas)H-Index: 25
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Background The effect of the timing of stage-2-palliation (S2P) on survival through single ventricle palliation remains unknown. This study investigated the optimal timing of S2P that minimizes pre-S2P attrition and maximizes post-S2P survival. Methods The Congenital Heart Surgeons' Society's critical left ventricular outflow tract obstruction cohort was used. Survival analysis was performed using multiphase parametric hazard analysis. Separate risk factors for death after the Norwood and after ...
9 CitationsSource
#1C.L. Poh (University of Melbourne)H-Index: 1
#2Yves d ’Udekem (University of Melbourne)H-Index: 30
Aim We now know that 20–40% of patients with a single ventricle will develop heart failure after the second decade post-Fontan surgery. However, we remain unable to risk-stratify the cohort to identify patients at highest risk of late failure and death. We conducted a systematic review of all reported late outcomes for patients with a Fontan circulation to identify predictors of late death. Methods We searched MEDLINE, Embase and PubMed with subject terms (“single ventricle”, “Hypoplastic left h...
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#1Richard G. Ohye (UM: University of Michigan)H-Index: 40
#2Dietmar Schranz (University of Giessen)H-Index: 32
Last. Yves d ’Udekem (Royal Children's Hospital)H-Index: 30
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Universally fatal only 4 decades ago, the progress in the 3-stage palliation of hypoplastic left heart syndrome and related single right ventricular lesions has drastically improved the outlook for these patients. Although the stage II operation (hemi-Fontan or bidirectional Glenn) and stage III Fontan procedure have evolved into relatively low-risk operations, the stage I Norwood procedure remains one of the highest-risk and costliest common operations performed in congenital heart surgery. Yet...
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Hypoplastic left heart syndrome (HLHS) with aortic atresia has evolved from a universally fatal disease when Norwood et al performed the first staged surgical palliations in 1979 to one with reasonable intermediate term outcomes. Despite remarkable surgical advances, transplant-free survival in this group of patients at 3 years of age is only 64%. Heart transplantation as a primary treatment for HLHS has been advocated principally by Bailey et al at Loma Linda Children’s Hospital. Although prima...
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#1Waldemar F. Carlo (UAB: University of Alabama at Birmingham)H-Index: 8
#2S.C. West (University of Pittsburgh)H-Index: 3
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Background Pulmonary blood flow during Stage 1 (Norwood) palliation for hypoplastic left heart syndrome (HLHS) is achieved via modified Blalock–Taussig shunt (MBT) or right ventricle to pulmonary artery conduit (RVPA). Controversy exists regarding the differential impact of shunt type on outcome among those who require transplantation early in life. In this study we explored waitlist and post-transplant outcomes within this sub-population stratified by shunt type. Methods Eligible patients were ...
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#1Aparna Kulkarni (Albert Einstein College of Medicine)H-Index: 4
#2Richard Neugebauer (Columbia University)H-Index: 29
Last. Daphne T. Hsu (Albert Einstein College of Medicine)H-Index: 11
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Background Infants with hypoplastic left heart syndrome after palliation have the worst survival among heart transplant recipients. Heart transplantation is often reserved for use in patients with sub-optimal results after palliative surgery. This study characterized outcomes after listing in infants with a single ventricle who had undergone the Norwood procedure and identified predictors of the decision to list for heart transplantation. Methods The public-use database from the multicenter, pro...
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#1Michael E. Mitchell (Children's Hospital of Wisconsin)H-Index: 47
#1Michael E. Mitchell (Children's Hospital of Wisconsin)H-Index: 14
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