Aamir Jeewa

Presentation, Diagnosis, and Medical Management of Heart Failure in Children: Canadian Cardiovascular Society Guidelines

Pediatric heart failure (HF) is an important cause of morbidity and mortality in childhood. This article presents guidelines for the recognition, diagnosis, and early medical management of HF in infancy, childhood, and adolescence. The guidelines are intended to assist practitioners in office-based or emergency room practice, who encounter children with undiagnosed heart disease and symptoms of possible HF, rather than those who have already received surgical palliation. The guidelines have been developed using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology, and are accompanied by practical Recommendations for their application in the clinical setting, supplemented by online material. This work does not include Recommendations for advanced management involving ventricular assist devices, or other device therapies.

Outcomes of pediatric patients supported by the HeartMate II left ventricular assist device in the United States

OBJECTIVE The HeartMate II (HMII; Thoratec, Pleasanton, CA) continuous-flow left ventricular assist device (LVAD) is an established treatment modality for advanced heart failure in adults. The objective of this study was to evaluate outcomes of pediatric patients supported by the HMII LVAD. METHODS This was a retrospective review of the Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) of patients supported with a HMII from April 2008 to September 2011. The primary cohort comprised pediatric patients aged 11 to 18 years. Outcomes were compared with a group of young adults aged 19 to 39 years who underwent HMII implant during the same period. Ischemic etiologies for heart failure were excluded. RESULTS There were 28 pediatric patients, of whom 19 (68%) were males, 14 (46%) were African American, and 7 (25%) underwent device placement in a pediatric hospital. Competing outcomes analysis showed that at 6 months of follow-up, the composite of survival to transplantation, ongoing support, or recovery was 96% for the pediatric group, which was not significantly different from the young adult group (96%, p = 0.330). The 2 groups had similar INTERMACS profiles but differed in diagnosis, weight, and morbidities. Bleeding complications requiring surgical intervention were more common in the pediatric group. CONCLUSIONS Pediatric outcomes with a HMII LVAD are comparable to that of young adults. As we continue to monitor this growing group, more sophisticated characterization and comparisons will be possible. Also, as technology progress and second- and third-generation devices are introduced, the number of children who will benefit from mechanical support will continue to grow.

Facilitated cardiac recovery in fulminant myocarditis: pediatric use of the Impella LP 5.0 pump

We describe the successful use of the Impella LP 5.0 intracardiac microaxial pump (Abiomed, Danvers, MA) in a 13-year-old boy with fulminant biopsy-proven viral myocarditis. The patient, who previously was in refractory cardiogenic shock despite increasing inotropic and vasopressor support, immediately stabilized after Impella LP 5.0 implantation and was successfully bridged to a full recovery. Months later, he remains completely well, with no intracardiac or peripheral vascular sequelae of the procedure. In carefully selected pediatric patients the Impella may be a beneficial form of temporary mechanical circulatory support for fulminant cardiogenic shock.

Genetic determinants of right-ventricular remodeling after tetralogy of Fallot repair.

Background:Hypoxia-inducible factor (HIF1A) regulates the myocardial response to hypoxia and hemodynamic load. We investigated the association of HIF1A variants with right-ventricular (RV) remodeling after tetralogy of Fallot (TOF) repair.Methods:Children with TOF were genotyped for three single-nucleotide polymorphisms in HIF1A. Genotypes were analyzed for association with RV myocardial protein expression and fibrosis at complete repair (n = 42) and RV dilation, fractional area change, and freedom from pulmonary valve/conduit replacement on follow-up.Results:In 180 TOF patients, mean age at repair was 1.0 ± 0.8 y with follow-up at 9.0 ± 3.5 y; 82% had moderate to severe pulmonary insufficiency. Freedom from RV reinterventions at 5, 10, and 15 y was 92, 84, and 67%, respectively. Patients with more functioning HIF1A alleles had higher transforming growth factor β1 expression and more fibrosis at initial repair as compared with controls (P < 0.05). During follow-up, patients with more functioning HIF1A alleles showed less RV dilation, better preservation of RV function, and greater freedom from RV reinterventions (P < 0.05). This was confirmed in a replication cohort of 69 patients.Conclusion:In children who have had TOF repair, a lower number of functioning HIF1A alleles was associated with RV dilation and dysfunction, suggesting that hypoxia adaptation in unrepaired TOF may influence RV phenotype after repair.

Infectious Complications and Outcomes in Children Supported with Left Ventricular Assist Devices

BACKGROUND Infectious complications constitute a major cause of morbidity and death in adult patients supported with left ventricular assist devices (VADs). The incidence and patient outcomes related to infectious complications in pediatric patients on VAD support remain largely unknown. The aim of this study was to determine the incidence of infection among pediatric VAD recipients and to characterize the microbiology, associated risk factors, and clinical outcome. METHODS We conducted a retrospective record review of all patients undergoing VAD support for ≥2 weeks at Texas Childrens Hospital from June 1999 to December 2011. Infections were categorized as VAD-specific, VAD-related, or non-VAD-related using the International Society for Heart and Lung Transplantation (ISHLT) definitions for VAD infections. RESULTS Fifty-two VADs were implanted in 51 patients; of these, 35 patients (69%) had 92 infections while receiving VAD support. These included 10 VAD-specific infections, 23 VAD-related infections, and 59 non-VAD infections. The overall rate of VAD infections (specific + related) was 8/1,000 days of VAD support. The most common pathogens were Staphylococcus aureus, coagulase-negative staphylococci, Pseudomonas aeruginosa, and Candida spp. Of 8 deaths that occurred during VAD support, 3 (37.5%) were directly related to infections. Continuous-flow VAD (p = 0.0427) and prior cardiac transplantation with rejection (p = 0.0191) were significantly associated with development of VAD infections. CONCLUSIONS Infectious complications are common in pediatric patients undergoing VAD support. VAD infections do not prevent successful cardiac transplantation in children.

A modified implantation technique of the HeartWare ventricular assist device for pediatric patients

Limitations to our study include the lack of a control group or a different treatment arm. Owing to recurrent, lifethreatening, and refractory bleeding in these patients, we concluded that withholding a therapy that we had previously shown would benefit these patients would be unethical. We also did not assess the relative effect of thalidomide in reduction of LVAD-associated GIAD compared with other therapeutic agents. Other investigators have used agents, including octreotide, to reduce bleeding due to GIAD in LVAD patients, although octreotide is difficult to tolerate due to its mode of delivery (injection) and adverse effects, including nausea and bradycardia. Lenalidomide, a synthetic analog of thalidomide, has been shown to be as potent as the parent drug, with less of the non-hematologic adverse effects, and is another potential treatment option for these patients. In summary, thalidomide can be used safely in patients with LVAD-associated GIAD when prescribed as part of a treatment protocol. These findings must be replicated in multicenter, randomized studies to corroborate our results. Disclosure statement

Pediatric ventricular assist device use as a bridge to transplantation does not affect long-term quality of life.

OBJECTIVE The present study sought to determine the long-term quality of life (QOL) of children who required long-term ventricular assist device (VAD) support as a bridge to transplantation (BTT) compared with children who underwent heart transplantation without VAD support. Currently, 20% of children undergoing heart transplantation have required a VAD as a BTT. Few data have been published assessing how children requiring a VAD as a BTT will fair in terms of their long-term QOL. METHODS The present study used a cross-sectional design, using the Core and Cardiac modules of the Pediatric Quality of Life Inventory survey. In a secondary analysis, the factors associated with worse QOL outcomes among the VAD patients were also investigated. RESULTS At follow-up (median, 4.2 years), between the 21 children who required a VAD as a BTT and 42 who went straight to transplantation, no significant differences were found in the QOL as measured using the Psychosocial Health Summary Score, Physical Health Summary Score, or Total Score in the surveys Core Module, nor were any differences found in the outcomes assessed using the surveys Cardiac Module. Of the patients who required a VAD, only the presence of a neurologic complication was associated with worse QOL, which was demonstrated by decreased Physical Health Summary and Cardiac Communication scores. CONCLUSIONS Over the long term, surviving children who required a long-term VAD as a BTT experience a similar QOL as those who went straight to transplantation.

The Potential to Avoid Heart Transplantation in Children: Outpatient Bridge to Recovery with an Intracorporeal Continuous-Flow Left Ventricular Assist Device in a 14-Year-Old

Pediatric mechanical circulatory support has evolved considerably in the past decade. Improvements in device design and availability have led to increased short-, medium-, and long-term support options for pediatric patients with heart failure. Most pediatric mechanical circulatory support is utilized as a bridge to transplant and as a bridge to recovery in patients with temporary etiologies of heart failure (i.e., myocarditis). Described herein is our recovery program, and we report our experience as an independent pediatric ventricular assist device program with an intracorporeal continuous-flow device employed as an out-of-hospital bridge to recovery for a child with end-stage chronic heart failure.

Outpatient management of a child with bidirectional Glenn shunts supported with implantable continuous-flow ventricular assist device.

Outpatient management of a child with bidirectional Glenn shunts supported with implantable continuous-flow ventricular assist device Iki Adachi, MD, Aamir Jeewa, MD, Sarah Burki, MD, E. Dean McKenzie, MD, and Charles D. Fraser Jr, MD From the Congenital Heart Surgery, Texas Children’s Hospital, Houston, Texas; Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas; Pediatric Cardiology, Texas Children’s Hospital, Houston, Texas; and the Pediatrics, Baylor College of Medicine, Houston, Texas.

Outcomes after percutaneous coronary artery revascularization procedures for cardiac allograft vasculopathy in pediatric heart transplant recipients: A multi-institutional study

BACKGROUND Cardiac allograft vasculopathy is an important cause of long-term graft loss. In adults, percutaneous revascularization procedures (PRPs) have variable success with high restenosis rates and little impact on graft survival. Limited data exist in pediatric recipients of transplants. METHODS Data from the Pediatric Heart Transplant Study (PHTS) were used to explore associations between PRPs and outcomes after heart transplant in patients listed ≤18 years old who received a first heart transplant between 1993 and 2009. RESULTS Revascularization procedures were done in 28 of 3,156 (0.9%) patients; 13 patients had multiple PRPs giving a total of 51 PRPs performed across 15 centers. Mean recipient age at time of transplant was 7.7 ± 6.7 years; mean donor age was 15.9 ± 15.4 years. The mean time to first PRP was 5.7 ± 3.2 years. Vessels involved were left anterior descending artery (41%), right coronary artery (25%), circumflex artery (18%), other coronary branches/unknown (16%). PRPs consisted of 38 (75%) stent implantations and 13 (25%) balloon angioplasties with an overall procedural success rate of 73%. Freedom from graft loss after PRPs was 89%, 75%, and 61% at 1, 3, and 12 months. In addition, patients with transplants from donors >30 years old were found to have less freedom from the need for a revascularization procedure than patients with transplants from younger donors (p < 0.0001). CONCLUSIONS In this large pediatric heart transplant cohort, use of PRPs for cardiac allograft vasculopathy was rare, likely related to procedural feasibility of the interventions. Despite technically successful interventions, graft loss occurred in 39% within 1 year post-procedure; relisting for heart transplant should be considered.