Decreased right ventricular longitudinal strain in children with hypoplastic left heart syndrome during staged repair and follow-up: does it have implications in clinically stable patients?

2020 ◽  
Vol 36 (9) ◽  
pp. 1667-1677
Author(s):  
Roshan D’Souza ◽  
Yan Wang ◽  
Renzo J. C. Calderon-Anyosa ◽  
Andrea E. Montero ◽  
Maalika M. Banerjee ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Hypoplastic Left Heart Syndrome ◽  
Longitudinal Strain ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome

Abstract 7: Intracoronary Autologous Cardiac Progenitor Cell Transfer In Children With Hypoplastic Left Heart Syndrome: 2-year Results Of The Ticap Trial

2014 ◽  
Vol 115 (suppl_1) ◽  
Author(s):  
Shuta Ishigami ◽  
Suguru Tarui ◽  
Michihiro Okuyama ◽  
Daiki Ousaka ◽  
Junko Kobayashi ◽  
...  
Keyword(s):  
Heart Failure ◽  
Cardiac Function ◽  
Right Ventricular ◽  
Hypoplastic Left Heart Syndrome ◽  
Treated Group ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome

Backgrounds: Hypoplastic left heart syndrome (HLHS) is a severe congenital heart malformation. Objective: The aim of this study is to determine whether intracoronary delivery of autologous cardiosphere-derived cells (CDCs) was feasible and safe to treat the children with HLHS. Methods: Four-teen patients with HLHS undergoing staged palliations were prospectively enrolled in this trial between January, 2011, and January, 2012. Seven patients constitutively assigned to receive intracoronary CDCs injection followed by 7 patients allocated to a control group with standard care alone. The primary endpoint was to assess the safety and the secondary endpoint was the preliminary efficacy by assessing the improvements of the right ventricular function during the follow-up. Results: No major complications were reported within 24 months of CDC infusion. Echocardiography showed that improvement of right ventricular ejection fraction (RVEF) was greater in the CDC-treated group (+5.3±3.2%) than in controls (+0.1±3.4%, P=0.01) at 3-month follow up. This cardiac function enhancement was manifested even in long-term observation (+7.8±4.9% vs. +2.2±3.1% at 1 year, P=0.03; +8.8±3.7% vs. +3.4±6.4% at 2 years, P=0.04). The absolute improvements in RVEF between 2 groups was confirmed by using right ventriculogram (RVG: +8.9±7.6% vs. +2.0±2.8% at 1 year, P=0.02; +8.1±6.0% vs. +2.9±3.9% at 2 years, P=0.04). In addition, RVEF on cMRI was also markedly improved in CDC-treated patients from 36.1±7.5% at baseline to 42.7±8.7% at 1 year (P=0.04) and to 42.4±7.6% at 2 years (P=0.047). Heart failure status was reduced in CDC-treated group as shown by significant decrease in Ross Heart Failure Class (2.6±0.8 at baseline vs. 1.4±0.5 at 2 years, P=0.01). Moreover, Z scores for weight-for-age was significantly increased from −4.0±2.7 at baseline to −2.2±1.4 at 2 year (P=0.02), whereas all of these parameters did not change in control subjects. Conclusion: These results of 2-year follow-up of TICAP trial suggest that intracoronary infusion of autologous CDCs is feasible and safe to treat the children with HLHS. This novel therapeutic strategy may impact on cardiac function as well as clinical symptom of heart failure status and somatic growth in long-term outcome.

Congenital Brain Anomalies Associated With the Hypoplastic Left Heart Syndrome

PEDIATRICS ◽  
1990 ◽  
Vol 85 (6) ◽  
pp. 984-990 ◽  
Author(s):  
Tracy A. Glauser ◽  
Lucy B. Rorke ◽  
Paul M. Weinberg ◽  
Robert R. Clancy
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Hypoplastic Left Heart Syndrome ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Brain Anomalies ◽  
Physical Features ◽  
Left Heart Syndrome

This study details the type, frequency, and clinical presentation of developmental brain anomalies in 41 infants with the hypoplastic left heart syndrome encountered during a 52-month interval. Overall, 29% of the infants had either a major or minor central nervous system abnormality. Overt central nervous system malformations, including 3 cases of agenesis of the corpus callosum and 1 case of holoprosencephaly, were seen in 4 infants (10%). Micrencephaly (brain weight at autopsy more than 2 SDs below the mean for age) was found in 27% of the infants. An immature cortical mantle was seen in 21% of the study group. Seven infants (17%) had specific recognizable patterns of malformation. The absence of dysmorphic physical features did not preclude overt or subtle central nervous system malformations. Conversely, the presence of dysmorphic features did not reliably indicate an underlying brain anomaly. Infants who had hypoplastic left heart syndrome as one of multiple nonneurologic malformations were more likely to have micrencephaly than those infants with hypoplastic left heart syndrome as an isolated abnormality. Occurrence of developmental neuropathology was elevated in those infants with hypoplastic left heart syndrome who did not have a recognizable pattern of malformation but who were small for gestational age, microcephalic, or had ocular abnormalities. Infants with hypoplastic left heart syndrome deserve careful genetic, ophthalmologic, and neurologic evaluations, imaging of their intracranial anatomy, and long-term neurologic follow-up.

Pre- and Postoperative Quantitation of Right Ventricular Tissue Doppler Velocities in Infants with Hypoplastic Left Heart Syndrome

2006 ◽  
Vol 23 (4) ◽  
pp. 303-307 ◽  
Author(s):  
Douglas Christensen ◽  
Brian Cardis ◽  
William Mahle ◽  
Reginald Lewis ◽  
Jeryl Huckaby ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Hypoplastic Left Heart Syndrome ◽  
Tissue Doppler ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Ventricular Tissue ◽  
Left Heart Syndrome

Catheter interventions in the staged management of hypoplastic left heart syndrome

2013 ◽  
Vol 24 (2) ◽  
pp. 212-219 ◽  
Author(s):  
Zdenka Reinhardt ◽  
Joseph De Giovanni ◽  
John Stickley ◽  
Vinay K. Bhole ◽  
Benjamin Anderson ◽  
...  
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Left Pulmonary Artery ◽  
Stage Ii ◽  
Stage Iii ◽  
Catheter Intervention ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome ◽  
Fontan Palliation

AbstractAimTo analyse the current practice and contribution of catheter interventions in the staged management of patients with hypoplastic left heart syndrome.MethodsThis study is a retrospective case note review of 527 patients undergoing staged Norwood/Fontan palliation at a single centre between 1993 and 2010. Indications and type of catheter interventions were reviewed over a median follow-up period of 7.5 years.ResultsA staged Norwood/Fontan palliation for hypoplastic left heart syndrome was performed in 527 patients. The 30-day survival rate after individual stages was 76.5% at Stage I, 96.3% at Stage II, and 99.4% at Stage III. A total of 348 interventions were performed in 189 out of 527 patients. Freedom from catheter intervention in survivors was 58.2% before Stage II and 46.7% before Stage III. Kaplan–Meier freedom from intervention post Fontan completion was 55% at 10.8 years of follow-up. Post-stage I interventions were mostly directed to relieve aortic arch obstruction – 84 balloon angioplasties – and augment pulmonary blood flow – 15 right ventricle-to-pulmonary conduit interventions; post-Stage II interventions centred on augmenting size of the left pulmonary artery – 73 procedures and abolishing systemic venous collaterals – 32 procedures. After Stage III, the focus was on manipulating the size of the fenestration – 42 interventions – and the left pulmonary artery −31 procedures.ConclusionInterventional cardiac catheterisation constitutes an integral part in the staged palliative management of patients with hypoplastic left heart syndrome. Over one-third (37%) of patients undergoing staged palliation required catheter intervention over the follow-up period.

Repair of the tricuspid valve in hypoplastic left heart syndrome

2006 ◽  
Vol 16 (S3) ◽  
pp. 21-26 ◽  
Author(s):  
Richard G. Ohye ◽  
Carlen A. Gomez ◽  
Caren S. Goldberg ◽  
Holly L. Graves ◽  
Eric J. Devaney ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Hypoplastic Left Heart Syndrome ◽  
Volume Overload ◽  
Ventricular Volume ◽  
Norwood Procedure ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Arterial Insufficiency ◽  
Three Stages ◽  
Left Heart Syndrome

The outlook for patients with hypoplastic left heart syndrome has dramatically improved over the past two decades. Universally fatal only 25 years ago, since that time outcomes for staged palliation have shown consistent improvement. Recent reports show that eight to nine patients from every ten can now leave the hospital after the Norwood procedure.1Attrition following the Norwood procedure, nonetheless, remains significant, with from five to fifteen percent of patients dying between the first and second stages of the Norwood sequence.1–4Only three-quarters of the patients undergoing surgery for hypoplastic left heart syndrome survive after five years, even at the centres reporting the best outcomes for the Norwood procedure.1,5In addition to the deaths, some patients are unable to progress through the three stages of reconstruction, and may require cardiac transplantation, or have no options for further therapy. There are many causes for these mortalities and morbidities following the Norwood procedure, including elevated pulmonary vascular resistance, cardiac arrhythmias, coronary arterial insufficiency, right ventricular failure, right ventricular volume overload due to shunt-dependent physiology, and tricuspid valvar regurgitation. Many of these factors are interrelated, and may form feedback loops, which serve to propagate their adverse effects on patients with hypoplastic left heart syndrome.

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