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325 Outcomes of Lung Transplantation in Children with Idiopathic Pulmonary Arterial Hypertension: St. Louis Children's Hospital Experience
Abstracts
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cant differences between the groups for days on inotropes, days intubated, or length of stay (Table 1). Table 1 Demographics and outcomes in single-lung vs. control pts with CHD who underwent OHT. Single-lung, n⫽17
Controls, n⫽26
Diagnosis - Single 12 (71%) 24 (92%) Ventricle Weight (kilograms) 44.3 (IQR 40.5) 41.5 (IQR 39.9) Age (years) 15.4 (IQR 31.6) 8.5 (IQR 17.5) Days Intubated 3 (IQR 7) 5 (IQR 5) Days on Inotropes 6 (IQR 11) 7 (IQR 12) Length of Stay (days) 17 (IQR 9) 28 (IQR 16)
Significance p⫽.06 p⫽.58 p⫽.30 p⫽.84 p⫽.60 p⫽.09
IQR interquartile range.
Conclusions: Hospital morbidity and long-term survival after OHT in pts with single-lung physiology was excellent, and not different from CHD controls. OHT should be considered a preferred option in these pts. 325 Outcomes of Lung Transplantation in Children with Idiopathic Pulmonary Arterial Hypertension: St. Louis Children’s Hospital Experience B.S. Goldstein,1 C.B. Huddleston,3 S.C. Sweet,2 J. Mao,1 R.M. Grady.1 1 Pediatric Cardiology, Washington University/St. Louis Children’s Hospital, St. Louis, MO; 2Pediatric Pulmonology, Washington University/St. Louis Children’s Hospital, St. Louis, MO; 3Cardiothoracic Surgery, Washington University/St. Louis Children’s Hospital, St. Louis, MO. Purpose: The natural history of idiopathic pulmonary arterial hypertension (IPAH) in patients of all ages is one of relentless progression. For those who fail medical therapy, lung transplantation remains the ultimate palliation. In the United States, IPAH is the second leading indication for lung transplant in all children and first for children ages 1-5 years. In this study, we report our nearly 20 year experience with lung transplantation in children with IPAH. Methods and Materials: We performed a retrospective chart review of children with IPAH listed for lung transplant at our center between 1991 and 2009. Our data reflects a total of 26 patients with ages ranging from 1.9 to 18.9 years. Nineteen of 26 patients were transplanted (bilateral lung n⫽13, single lung n⫽3, heart-lung n⫽3) The impact of pre-transplant variables on survival was evaluated. Results: Median survival for those tranplanted was 5.8 years. One and 5-year survival rates were 95% and 61%, respectively. Survival was independent of age, weight, need for IV inotropes, need for IV vasodilators, transplant type, year of transplant and severity of pulmonary artery pressures. Only one child died prior to discharge after transplant. Seven children (⬃27%) died while waiting for transplant. Compared to the transplanted group, these children had significantly higher incidences of suprasystemic right heart pressures (p⫽0.02) and hemoptysis (p⫽0.006). Conclusions: Our study is the largest to date to look at outcomes for lung transplant in children with IPAH. Median survival compares favorably to that of all pediatric lung transplant recipients, 5.8 yrs vs 4.5 yrs, respectively. No variables we assessed significantly impacted survivial, thus outcome seems more related to factors that influence long-term survival in all transplant recipients, such as rejection and infection. Lung transplantation remains a viable option for children with IPAH, especially those who have developed suprasystemic right heart pressures or hemoptysis despite maximal medical therapy. 326 An Integrated Pediatric Pump-Lung for Cardiac/Cardiopulmonary Support Z.J. Wu,1 B.N. Gellman,2 T. Zhang,1 K. Dasse,2 J.P. Garcia,1 B.P. Griffith.1 1Surgery, University of Maryland Baltimore, Baltimore, MD; 2Levitronix, LLC., Waltham, MA.
Purpose: The use of current ECMO systems for pediatric cardiac/cardiopulmonary support is limited by complexity, component failure, thrombus, and bleeding. To address these issues, a miniaturized integrated pediatric pump-Lung (PediPL) has been developed for patients weighing 3-20kg to allow mobility and extended use for 30 days. Methods and Materials: PediPL incorporates a magnetically levitated impeller with uniquely configured hollow fiber membranes into a single unit capable of both pumping and gas exchange. The design of PediPL was optimized using both computational and experimental approaches. In-vivo studies were performed in a veno-arterial configuration between left jugular vein and common carotid artery of 45⬃55kg sheep in the pediatric flow setting. Results: PediPL has a membrane surface of 0.3m2 and a priming volume of 100ml. It is capable of pumping blood up to 2.5L/min against 140mmHg at 3000rpm and delivering up to 125mlO2/min. Three 3-day and three 28-30 day in-vivo studies were completed. An ACT of 150sec was maintained by continuous heparin infusion. PediPL was operated at a flow of 0.3⬃2.0L/min and was capable of delivering 21⬃107mlO2/min. In spite of varying venous blood O2 saturation, the blood was fully oxygen saturated using PediPL. The chronic performance was stable. Blood chemistry data indicated the animals had normal end organ function. Mean plasma free hemoglobin was 8.6⫾3.7mg/dL and the platelet count remained normal. No thrombosis was found in the explanted devices. Conclusions: PediPL proved capable of providing pediatric cardiac/ cardiopulmonary support with excellent biocompatibility. The impact on the hematological, serological and end-organ function was minimal. A plan for a NIH-sponsored investigational device evaluation (IDE) is underway.
327 The UK Berlin Heart Journey J. Cassidy,1 A. Hoskote,2 S. Haynes,1 M. Fenton,2 J. Smith,1 M. Burch,2 R. Kirk,1 T. Hsia,2 A. Hasan,1 A. Karimova.2 1Paediatric Intensive Care Unit, Freeman Hospital, Newcastle upon Tyne, United Kingdom; 2 Cardiac Intensive Care Unit, Great Ormond St Hospital, London, United Kingdom. Purpose: The Berlin Heart (BH) was first used in the UK in November 2004. Outcomes to October 2010 are reviewed. Methods and Materials: Review of all children bridged with the BH in the 2 UK paediatric heart transplant units. Results: Between 2004-10 77 children with end stage cardiac failure received BH support with the intention of bridge to transplant (BTT). The median age was 23 months (range 1 week-16.9 years), median weight 11(3-90) kg and median support duration 39 (1-163) days. 64(83%) survived to transplant or explantation. 55 (71%) were transplanted and 53 survived to discharge. These comprise 29% of the UK paediatric heart transplants in this time. 9(12%) of children had sufficient cardiac recovery on support for explantation – 7 surviving to discharge. Overall survival to discharge was 78%. Half of those treated are ⬍/⫽ 10 kg in weight. Although the median support time is longer (table 2) survival does not differ. 24(31%) required ECMO pre BH support with 75% surviving. 36(47%) required BIVAD support with 79% survival. 33(43%) required LVAD support with 88% survival. 18 children had ongoing renal dysfunction post BH support of whom 8 subsequently died.
S113
cant differences between the groups for days on inotropes, days intubated, or length of stay (Table 1). Table 1 Demographics and outcomes in single-lung vs. control pts with CHD who underwent OHT. Single-lung, n⫽17
Controls, n⫽26
Diagnosis - Single 12 (71%) 24 (92%) Ventricle Weight (kilograms) 44.3 (IQR 40.5) 41.5 (IQR 39.9) Age (years) 15.4 (IQR 31.6) 8.5 (IQR 17.5) Days Intubated 3 (IQR 7) 5 (IQR 5) Days on Inotropes 6 (IQR 11) 7 (IQR 12) Length of Stay (days) 17 (IQR 9) 28 (IQR 16)
Significance p⫽.06 p⫽.58 p⫽.30 p⫽.84 p⫽.60 p⫽.09
IQR interquartile range.
Conclusions: Hospital morbidity and long-term survival after OHT in pts with single-lung physiology was excellent, and not different from CHD controls. OHT should be considered a preferred option in these pts. 325 Outcomes of Lung Transplantation in Children with Idiopathic Pulmonary Arterial Hypertension: St. Louis Children’s Hospital Experience B.S. Goldstein,1 C.B. Huddleston,3 S.C. Sweet,2 J. Mao,1 R.M. Grady.1 1 Pediatric Cardiology, Washington University/St. Louis Children’s Hospital, St. Louis, MO; 2Pediatric Pulmonology, Washington University/St. Louis Children’s Hospital, St. Louis, MO; 3Cardiothoracic Surgery, Washington University/St. Louis Children’s Hospital, St. Louis, MO. Purpose: The natural history of idiopathic pulmonary arterial hypertension (IPAH) in patients of all ages is one of relentless progression. For those who fail medical therapy, lung transplantation remains the ultimate palliation. In the United States, IPAH is the second leading indication for lung transplant in all children and first for children ages 1-5 years. In this study, we report our nearly 20 year experience with lung transplantation in children with IPAH. Methods and Materials: We performed a retrospective chart review of children with IPAH listed for lung transplant at our center between 1991 and 2009. Our data reflects a total of 26 patients with ages ranging from 1.9 to 18.9 years. Nineteen of 26 patients were transplanted (bilateral lung n⫽13, single lung n⫽3, heart-lung n⫽3) The impact of pre-transplant variables on survival was evaluated. Results: Median survival for those tranplanted was 5.8 years. One and 5-year survival rates were 95% and 61%, respectively. Survival was independent of age, weight, need for IV inotropes, need for IV vasodilators, transplant type, year of transplant and severity of pulmonary artery pressures. Only one child died prior to discharge after transplant. Seven children (⬃27%) died while waiting for transplant. Compared to the transplanted group, these children had significantly higher incidences of suprasystemic right heart pressures (p⫽0.02) and hemoptysis (p⫽0.006). Conclusions: Our study is the largest to date to look at outcomes for lung transplant in children with IPAH. Median survival compares favorably to that of all pediatric lung transplant recipients, 5.8 yrs vs 4.5 yrs, respectively. No variables we assessed significantly impacted survivial, thus outcome seems more related to factors that influence long-term survival in all transplant recipients, such as rejection and infection. Lung transplantation remains a viable option for children with IPAH, especially those who have developed suprasystemic right heart pressures or hemoptysis despite maximal medical therapy. 326 An Integrated Pediatric Pump-Lung for Cardiac/Cardiopulmonary Support Z.J. Wu,1 B.N. Gellman,2 T. Zhang,1 K. Dasse,2 J.P. Garcia,1 B.P. Griffith.1 1Surgery, University of Maryland Baltimore, Baltimore, MD; 2Levitronix, LLC., Waltham, MA.
Purpose: The use of current ECMO systems for pediatric cardiac/cardiopulmonary support is limited by complexity, component failure, thrombus, and bleeding. To address these issues, a miniaturized integrated pediatric pump-Lung (PediPL) has been developed for patients weighing 3-20kg to allow mobility and extended use for 30 days. Methods and Materials: PediPL incorporates a magnetically levitated impeller with uniquely configured hollow fiber membranes into a single unit capable of both pumping and gas exchange. The design of PediPL was optimized using both computational and experimental approaches. In-vivo studies were performed in a veno-arterial configuration between left jugular vein and common carotid artery of 45⬃55kg sheep in the pediatric flow setting. Results: PediPL has a membrane surface of 0.3m2 and a priming volume of 100ml. It is capable of pumping blood up to 2.5L/min against 140mmHg at 3000rpm and delivering up to 125mlO2/min. Three 3-day and three 28-30 day in-vivo studies were completed. An ACT of 150sec was maintained by continuous heparin infusion. PediPL was operated at a flow of 0.3⬃2.0L/min and was capable of delivering 21⬃107mlO2/min. In spite of varying venous blood O2 saturation, the blood was fully oxygen saturated using PediPL. The chronic performance was stable. Blood chemistry data indicated the animals had normal end organ function. Mean plasma free hemoglobin was 8.6⫾3.7mg/dL and the platelet count remained normal. No thrombosis was found in the explanted devices. Conclusions: PediPL proved capable of providing pediatric cardiac/ cardiopulmonary support with excellent biocompatibility. The impact on the hematological, serological and end-organ function was minimal. A plan for a NIH-sponsored investigational device evaluation (IDE) is underway.
327 The UK Berlin Heart Journey J. Cassidy,1 A. Hoskote,2 S. Haynes,1 M. Fenton,2 J. Smith,1 M. Burch,2 R. Kirk,1 T. Hsia,2 A. Hasan,1 A. Karimova.2 1Paediatric Intensive Care Unit, Freeman Hospital, Newcastle upon Tyne, United Kingdom; 2 Cardiac Intensive Care Unit, Great Ormond St Hospital, London, United Kingdom. Purpose: The Berlin Heart (BH) was first used in the UK in November 2004. Outcomes to October 2010 are reviewed. Methods and Materials: Review of all children bridged with the BH in the 2 UK paediatric heart transplant units. Results: Between 2004-10 77 children with end stage cardiac failure received BH support with the intention of bridge to transplant (BTT). The median age was 23 months (range 1 week-16.9 years), median weight 11(3-90) kg and median support duration 39 (1-163) days. 64(83%) survived to transplant or explantation. 55 (71%) were transplanted and 53 survived to discharge. These comprise 29% of the UK paediatric heart transplants in this time. 9(12%) of children had sufficient cardiac recovery on support for explantation – 7 surviving to discharge. Overall survival to discharge was 78%. Half of those treated are ⬍/⫽ 10 kg in weight. Although the median support time is longer (table 2) survival does not differ. 24(31%) required ECMO pre BH support with 75% surviving. 36(47%) required BIVAD support with 79% survival. 33(43%) required LVAD support with 88% survival. 18 children had ongoing renal dysfunction post BH support of whom 8 subsequently died.