Hypoplastic Left Heart Syndrome

Feinstein, Jeffrey A.; Benson, D. Woodrow; Dubin, Anne M.; Cohen, Meryl S.; Maxey, Dawn; Mahle, William T.; Pahl, Elfriede; Villafañe, Juan; Bhatt, Ami B.; Peng, Lynn F.; Johnson, Beth; Marsden, Alison L.; Daniels, Curt J.; Rudd, Nancy A.; Caldarone, Christopher A.; Mussatto, Kathleen A.; Morales, David L.S.; Ivy, D. Dunbar; Gaynor, J. William; Tweddell, James S.; Deal, Barbara J.; Furck, Anke K.; Rosenthal, Geoffrey L.; Ohye, Richard G.; Ghanayem, Nancy S.; Cheatham, John P.; Tworetzky, Wayne; Martin, Gerard R.

doi:10.1016/j.jacc.2011.09.022

Cited by 426 publications

(179 citation statements)

References 463 publications

(546 reference statements)

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“…Although the Fontan procedure has significantly improved early survival, transplant-free 30-year survival remains at only 60 % [7]. These patients can also experience significant long-term complications including heart failure, protein-losing enteropathy, and plastic bronchitis [8]. The pathophysiology of these complications is poorly understood, but may be linked to limited pulmonary artery growth, low-shear non-pulsatile pulmonary blood flow (PBF), and thrombus formation leading to increased pulmonary vascular resistance (PVR) [18].…”

Section: Introductionmentioning

confidence: 99%

Elevated Low-Shear Blood Viscosity is Associated with Decreased Pulmonary Blood Flow in Children with Univentricular Heart Defects

et al. 2016

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After the Fontan procedure, patients with univentricular hearts can experience long-term complications due to chronic low-shear non-pulsatile pulmonary blood flow. We sought to evaluate hemorheology and its relationship to hemodynamics in children with univentricular hearts. We hypothesized that low-shear blood viscosity and red blood cell (RBC) aggregation would be associated with increased pulmonary vascular resistance (PVR) and decreased pulmonary blood flow (PBF). We performed a cross-sectional analysis of 62 children undergoing cardiac catheterization—20 with isolated atrial septal defect (ASD), 22 status post Glenn procedure (Glenn), and 20 status post Fontan procedure (Fontan). Shear-dependent blood viscosity, RBC aggregation and deformability, complete blood count, coagulation panel, metabolic panel, fibrinogen, and erythrocyte sedimentation rate were measured. PVR and PBF were calculated using the Fick equation. Group differences were analyzed by ANOVA and correlations by linear regression. Blood viscosity at all shear rates was higher in Glenn and Fontan, partially due to normocytic anemia in ASD. RBC aggregation and deformability were similar between all groups. Low-shear viscosity negatively correlated with PBF in Glenn and Fontan only (R2 = 0.27, p < 0.001); it also negatively correlated with pulmonary artery pressure in Glenn (R2 = 0.15, p = 0.01), and positively correlated with PVR in Fontan (R2 = 0.28, p = 0.02). Our data demonstrate that elevated low-shear blood viscosity is associated with negative hemodynamic perturbations in a passive univentricular pulmonary circulation, but not in a pulsatile biventricular pulmonary circulation.

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Section: Introductionmentioning

confidence: 99%

Elevated Low-Shear Blood Viscosity is Associated with Decreased Pulmonary Blood Flow in Children with Univentricular Heart Defects

et al. 2016

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“…Today, interstage mortality ranges between 2% and 20% with almost 75% of deaths occurring suddenly. 2,7,8 In recognition of this, in 2011, the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) focused on improvement in interstage mortality. 9 Today, a large majority of programs use home monitoring during the interstage period.…”

Section: The Interstage Periodmentioning

confidence: 99%

“…Hospital mortality after the Norwood procedure remains the highest among common congenital heart procedures, ranging between 7% and 19%. 2,3 An analysis of resource utilization for common structural heart defects has shown that the Norwood procedure is associated with the highest cost, and the third longest length of stay. 4 …”

mentioning

confidence: 99%

Harnessing Teams and Technology to Improve Outcomes in Infants With Single Ventricle

Shirali

Erickson

Apperson

et al. 2016

Circ: Cardiovascular Quality and Outcomes

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Abstract-Infants with single ventricle require staged cardiac surgery, with stage I typically performed shortly after birth, stage II at 4 to 6 months of age, and stage III at 3 to 5 years of age. There is a high risk of interstage mortality and morbidity after infants are discharged from the hospital between stages I and II. Traditional home monitoring requires caregivers to record measurements of weight and oxygen saturation into a binder and requires families to assume a surveillance role. We have developed a tablet PC-based solution that provides secure and nearly instantaneous transfer of patient information to a cloud-based server, with the capacity for instant alerts to be sent to the caregiver team. The cloudbased IT infrastructure lends itself well to being able to be scaled to multiple sites while maintaining strict control over the privacy of each site. All transmitted data are transferred to the electronic medical record daily. The system conforms to recently released Food and Drug Administration regulation that pertains to mobile health technologies and devices. Since this platform was developed in March 2014, 30 patients have been monitored. There have been no interstage deaths. The experience of care providers has been unanimously positive. The addition of video has added to the use of the monitoring program. Of 30 families, 23 expressed a preference for the tablet PC over the notebook, 3 had no preference, and 4 preferred the notebook to the tablet PC.

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“…With regard to HLHS palliative surgery and the valuable insight that can be gathered experimentally, MCLs can be modified to incorporate the effect of respiration [21] which becomes relevant when these patients receive their second and third surgeries, i.e. the Glenn procedure and the total cavopulmonary connect (or Fontan completion) [22]. A system incorporating a patient-specific 3D-printed Fontan model has indeed been realised [23].…”

Section: Multi-scale Hydrodynamic Setups: Studying Single Ventricle Pmentioning

confidence: 99%

3D Printing Cardiovascular Anatomy: A Single-Centre Experience

Biglino¹,

Capelli²,

Taylor³

et al. 2016

New Trends in 3D Printing

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This chapter presents the experience of the cardiac engineering team within the Centre for Cardiovascular Imaging at Great Ormond Street Hospital for Children (London, UK) in using three-dimensional (3D) printing technology. 3D models can serve different functions towards implementing a patient-specific approach for studying and potentially treating congenital heart disease (CHD). In order to showcase different potential applications, this chapter discusses not only clinical case studies and engineering experiments but also the potential for translation through patients and public involvement and engagement (PPI/E).

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Hypoplastic Left Heart Syndrome

Cited by 426 publications

References 463 publications

Elevated Low-Shear Blood Viscosity is Associated with Decreased Pulmonary Blood Flow in Children with Univentricular Heart Defects

Elevated Low-Shear Blood Viscosity is Associated with Decreased Pulmonary Blood Flow in Children with Univentricular Heart Defects

Harnessing Teams and Technology to Improve Outcomes in Infants With Single Ventricle

3D Printing Cardiovascular Anatomy: A Single-Centre Experience

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