Umar S. Boston

Liver cirrhosis in Fontan patients does not affect 1-year post-heart transplant mortality or markers of liver function

BACKGROUND Liver cirrhosis is recognized with long-term follow-up of patients after the Fontan procedure. The effect of liver cirrhosis on the use of heart transplant (HT) and on post-HT outcomes is unknown. METHODS We reviewed Fontan patients evaluated for HT from 2004 to 2012 with hepatic computed tomography (CT) imaging, classified as normal, non-cirrhotic changes, or cirrhosis. The primary outcome was 1-year all-cause mortality, and the secondary outcome was differences in serial post-HT liver evaluation. RESULTS CT imaging in 32 Fontan patients evaluated for HT revealed 20 (63%) with evidence of liver disease, including 13 (41%) with cirrhosis. Twenty underwent HT, including 5 non-cirrhotic and 7 cirrhosis patients. Characteristics at listing between normal or non-cirrhotic (n = 13) and cirrhosis (n = 7) groups were similar, except cirrhosis patients were older (median 17.6 vs 9.6 years, p = 0.002) and further from Fontan (median 180 vs 50 months, p < 0.05). Serial liver evaluation was similar, including aspartate aminotransferase, alanine aminotransferase, bilirubin, albumin, and tacrolimus dose at 1, 3, 6, 9, and 12 months. Overall patient survival was 80% at 1 year, with no difference between cirrhosis and non-cirrhosis patients (86% vs 77%, p = 0.681). Liver biopsies were performed in 7 patients before HT, and all specimens showed architectural changes with bridging fibrosis. CONCLUSIONS Most patients evaluated for HT had abnormal liver findings by CT, with cirrhosis in 41%. One-year mortality and serial liver evaluation were similar between groups after HT. Liver cirrhosis identified by CT imaging may not be an absolute contraindication to HT alone in this population.

Complete repair of Ebstein anomaly in neonates and young infants: A 16-year follow-up

OBJECTIVE The purpose of this study was to review the long-term outcome of patients with Ebstein anomaly who underwent complete repair as neonates and young infants. METHODS Between March 1994 and May 2010, 32 patients (23 neonates and 9 young infants) underwent surgery for Ebstein anomaly. Mean weight was 3.9 ± 2.0 kg (range, 1.9-8.6 kg). The Great Ormond Street Echocardiography score was greater than 1.5 in 22 of the 23 neonates and greater than 1.0 in all infants. All associated cardiac defects were repaired including pulmonary atresia in 15 and ventricular septal defect in 4. Primary outcome measures included (1) early and late survival, (2) freedom from reoperation, (3) durability of tricuspid valve repair, and (4) functional status. RESULTS Early survival was 78.1% (25/32). There was 1 late death. Fifteen-year survival estimate was 74% ± 8%. For patients with pulmonary atresia, early and late survival was 60% ± 12% (9/15) versus 94.1% (16/17) (P < .05), respectively; for those without pulmonary atresia, early and late survival was 60% ± 12% versus 85% ± 10 (P = .06), respectively. The mean follow-up was 5.9 ± 4.5 years (0.1-16 years). A biventricular repair was achieved in 90.6% (29/32) patients. Median preoperative tricuspid regurgitation was 4/4 and at late follow-up, 1/4. Freedom from reoperation at 15 years was 74% ± 10% for patients undergoing biventricular repair. All survivors are in New York Heart Association class I or II. CONCLUSIONS Biventricular repair of Ebstein anomaly in symptomatic neonates is feasible with good early and late survival, especially in those without pulmonary atresia. Tricuspid valve repair is durable, and functional status is excellent.

Successful Bridge Through Transplantation With Berlin Heart Ventricular Assist Device in a Child With Failing Fontan

In patients with failed Fontan circulation, end-stage heart failure can develop or Fontan physiology failure requiring transplantation. Experience with ventricular assist device support for these patients as a bridge to heart transplantation has been limited and often not resulted in successful hospital discharge. We report the successful use of the Berlin Heart EXCOR (Berlin Heart, The Woodlands, TX) ventricular assist device in bridging a child with Fontan circulation and systolic dysfunction to heart transplantation and discharge home.

Continuous Flow Device Support in Children Using the HeartWare HVAD: 1,000 Days of Lessons Learned From a Single Center Experience.

The purpose of this study is to provide a single center experience with a continuous flow device in adolescents with end-stage heart failure. A retrospective single center analysis of patients aged 18 years or younger implanted with HVAD (HeartWare Inc, Framingham, MA) between October 2012 and March 2014 was performed. Demographics, preimplant and postimplant clinical data, survival, and adverse events (AEs) were recorded. A matched group of adults based on diagnosis, body surface area (BSA), and time period were used for outcome comparisons. Six adolescents with dilated cardiomyopathy were implanted with the HVAD. Median age and BSA were 13.4 years and 1.45 m2, respectively. All were Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) profile-1 or profile-2. Median days on device were 108 with total patient-days on device of 1,017. Four adolescents were discharged home on device all in New York Heart Association 1. Five underwent transplantation with 100% survival. There were 18 AEs with one AE per 170 days on device. Compared with the adult cohort (n = 5), there was no difference in 1 year survival (p = 0.32). HVAD support in adolescents is highly successful as a bridge to transplantation. It provides early rehabilitation and improvement in quality of life. Morbidity is not negligible but appears comparable with that seen in adults.

Surgical Decision Making in Neonatal Ebstein's Anomaly: An Algorithmic Approach Based on 48 Consecutive Neonates.

Objective: There is currently no consensus of opinion regarding the optimal surgical management of Ebstein’s anomaly (EA) in neonates and young infants. Reported early mortality rates range from 25% to 100%. In this study, we present an algorithm for choosing the best management option for neonates with EA based on analysis of our experience. Patients and Methods: From 1994 to June, 2011, 48 neonates with a diagnosis of EA were managed by the same surgical team. Of these, two died before intervention; the remaining 46 either were managed medically initially (n = 20) or underwent surgical intervention during the neonatal period (n = 26) or early infancy (n = 9). Results: The mean weight was 3.6 ± 1.7 kg (1.9-8.6) and mean follow-up time was 6.3 ± 4.5 years (0.2-16). Of the 20 patients initially managed medically, 11 remain well without intervention and nine required complete repair in infancy, with 100% survival. Of the 26 neonatal operations, 23 (88%) were complete biventricular repairs, 1 Starnes’ palliation, and two Blalock–Taussig shunts (BTSs) ± pulmonary valvotomy. Among those having a two-ventricle repair, anatomic pulmonary atresia (APA) was a risk factor for early mortality (46.1%, 6 of 13) compared with those without pulmonary atresia (EA/no-PA; 10%, 1 of 10), P < .05. Conclusions: Most symptomatic neonates with EA will require early operation. For those with APA and mild tricuspid regurgitation (TR), a modified BTS and reduction atrioplasty may be the best initial option. For those with functional pulmonary atresia and severe TR and pulmonary regurgitation, ligation of the main pulmonary artery and placement of a BTS may provide the best initial palliation. For the rest, either a biventricular repair or a single-ventricle palliation is indicated.

Surgical management of intrapericardial teratoma in the fetus.

Intrapericardial teratomas often present as life-threatening problems when diagnosed during fetal life. They are large lesions that compress the heart and lungs and can result in tamponade if not treated expeditiously. We present a case of a large teratoma that was managed by prenatal pericardiocentesis followed by surgical resection.

Improved survival after heart transplant for failed Fontan patients with preserved ventricular function.

BACKGROUND Patients with a failing Fontan continue to have decreased survival after heart transplant (HT), particularly those with preserved ventricular function (PVF) compared with impaired ventricular function (IVF). In this study we evaluated the effect of institutional changes on post-HT outcomes. METHODS Data were retrospectively collected for all Fontan patients who underwent HT. Mode of failure was defined by the last echocardiogram before HT, with mild or no dysfunction considered PVF and moderate or severe considered IVF. Outcomes were compared between early era (EE, 1995 to 2008) and current era (CE, 2009 to 2014). Management changes in the CE included volume load reduction with aortopulmonary collateral (APC) embolization, advanced cardiothoracic imaging, higher goal donor/recipient weight ratio and aggressive monitoring for post-HT vasoplegia. RESULTS A total of 47 patients were included: 27 in the EE (13 PVF, 14 IVF) and 20 in the CE (12 PVF, 8 IVF). Groups were similar pre-HT, except for more PLE in PVF patients. More patients underwent APC embolization in the CE (80% vs 28%, p < 0.01). There was no difference in donor/recipient weight ratio between eras. There was a trend toward higher primary graft failure for PVF in the EE (77% vs 36%, p = 0.05) but not the CE (42% vs 75%, p = 0.20). Overall, 1-year survival improved in the CE (90%) from the EE (63%) (p = 0.05), mainly due to increased survival for PVF (82 vs 38%, p = 0.04). CONCLUSIONS Post-HT survival for failing Fontan patients has improved, particularly for PVF. In the CE, our Fontan patients had a 1-year post-HT survival similar to other indications.

Paracorporeal lung assist device: An innovative surgical strategy for bridging to lung transplant in an infant with severe pulmonary hypertension caused by alveolar capillary dysplasia

Alveolar capillary dysplasia is a frequently fatal congenital pulmonary disease during infancy. Lung transplantation (LT) is the only means of survival. We report the case of an infant with alveolar capillary dysplasia who subsequently had development of severe hemodynamic compromise as a result of refractory pulmonary hypertension (PH) and was successfully bridged to LT with a paracorporeal lung assist device (PLAD) in a unique configuration.

Current Trends in the Management of Neonates With Ebstein's Anomaly.

Background: The optimal management strategy for neonates with Ebsteins anomaly is unknown. This analysis was undertaken to assess current trends in the management and prognosis of neonates born with Ebsteins anomaly in the United States, as reflected in an administrative database. Methods: The Pediatric Health Information System database (40 children’s hospitals) was used to review the reported incidence and available data on neonates with Ebsteins anomaly treated in the United States between 2003 and 2007. Primary outcome was hospital survival. Of the 415 patients identified, 257 (62%) did not undergo initial surgical intervention as neonates. Aortopulmonary shunt only was done on 63 patients (15%), single-ventricle palliation on 36 (9%), two-ventricle repair on 16 (4%), heart transplantation in 3 (1%), and a catheter-based intervention or a hybrid palliative approach was applied in 40 (10%). Intergroup comparisons were done using chi-square analyses. Results: Mortality for the entire cohort was 24% (100 of 415). For medically treated patients, this was 22% (56 of 257). For surgically treated and hybrid patients, this was 30% and 23%, respectively (P = NS). Conclusions: The majority of patients born with Ebsteins anomaly currently do not undergo surgical intervention as neonates. Significant early mortality in this group suggests that certain subsets of patients may benefit from earlier surgical intervention. Among the severely symptomatic neonates who do undergo early surgical intervention, the mortality remains high, irrespective of the surgical approach taken. A multicenter trial may be appropriate to identify strategies to optimize care for these critically ill neonates. Further analysis of risk factors for early mortality is warranted.

Incidence of Platelet Dysfunction by Thromboelastography-Platelet Mapping in Children Supported with ECMO: A Pilot Retrospective Study.

Background Bleeding complications are common and decrease the odds of survival in children supported with extracorporeal membrane oxygenation (ECMO). The role of platelet dysfunction on ECMO-induced coagulopathy and resultant bleeding complications is not well understood. The primary objective of this pilot study was to determine the incidence and magnitude of platelet dysfunction according to thromboelastography (TEG®)–platelet mapping (PM) testing. Methods Retrospective chart review of children <18 years old who required ECMO at a tertiary level hospital. We collected TEG®–PM and conventional coagulation tests data. We also collected demographic, medications, blood products administered, and clinical outcome data. We defined severe platelet dysfunction as <50% aggregation in response to an agonist. Results We identified 24 out of 46 children on ECMO, who had TEG®–PM performed during the study period. We found the incidence of severe bleeding was 42% and mortality was 54% in our study cohort. In all samples measured, severe qualitative platelet dysfunction was more common for adenosine diphosphate (ADP)-mediated aggregation (92%) compared to arachidonic acid (AA)-mediated aggregation (75%) (p = 0.001). Also, ADP-mediated percent of platelet aggregation was significant lower than AA-mediated platelet aggregation [15% (interquartile range, IQR 2.8–48) vs. 49% (IQR 22–82.5), p < 0.001]. There was no difference in kaolin-activated heparinase TEG® parameters between the bleeding group and the non-bleeding group. Only absolute platelet count and TEG®–PM had increased predictive value on receiver operating characteristics analyses for severe bleeding and mortality compared to activated clotting time. Conclusion We found frequent and severe qualitative platelet dysfunction on TEG®–PM testing in children on ECMO. Larger studies are needed to determine if the assessment of qualitative platelet function by TEG®–PM can improve prediction of bleeding complications for children on ECMO.