Preoperative Stabilization of Infants With Hypoplastic Left Heart Syndrome Before Stage I Palliation

2016 ◽  
Vol 36 (1) ◽  
pp. 52-59 ◽  
Author(s):  
Amy Donnellan ◽  
Lindsey Justice
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Single Ventricle ◽  
Management Strategies ◽  
Ventilatory Support ◽  
Practice Variation ◽  
Severe Form ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Single Ventricle Physiology ◽  
Left Heart Syndrome

Hypoplastic left heart syndrome is a severe form of congenital heart disease that results in single-ventricle physiology. Although surgical palliation in infants with this syndrome is widely agreed on in practice, variation occurs in both surgical and medical management. Perinatal factors that affect preoperative management include the subtype of the syndrome and the patient’s birth weight, gestational age, and genetic abnormalities. The general aspects of perioperative stabilization and management are monitoring, use of vasoactive infusions, ventilatory support, and nutrition. Management strategies for balancing single-ventricle physiology are aimed at addressing the 3 major causes of desaturation: diminished pulmonary blood flow, low mixed venous oxygen saturation, and pulmonary venous desaturation.

scholarly journals Variation in care for infants undergoing the Stage II palliation for hypoplastic left heart syndrome

2018 ◽  
Vol 28 (10) ◽  
pp. 1109-1115 ◽  
Author(s):  
Aaron Eckhauser ◽  
Sara K. Pasquali ◽  
Chitra Ravishankar ◽  
Linda M. Lambert ◽  
Jane W. Newburger ◽  
...  
Keyword(s):  
Length Of Stay ◽  
Hypoplastic Left Heart Syndrome ◽  
Single Ventricle ◽  
Circulatory Arrest ◽  
Deep Hypothermic Circulatory Arrest ◽  
Practice Variation ◽  
Stage Ii ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome

AbstractBackgroundThe Single Ventricle Reconstruction trial randomised neonates with hypoplastic left heart syndrome to a systemic-to-pulmonary-artery shunt strategy. Patients received care according to usual institutional practice. We analysed practice variation at the Stage II surgery to attempt to identify areas for decreased variation and process control improvement.MethodsProspectively collected data were available in the Single Ventricle Reconstruction public-use database. Practice variation across 14 centres was described for 397 patients who underwent Stage II surgery. Data are centre-level specific and reported as interquartile ranges across all centres, unless otherwise specified.ResultsPreoperative Stage II median age and weight across centres were 5.4 months (interquartile range 4.9–5.7) and 5.7 kg (5.5–6.1), with 70% performed electively. Most patients had pre-Stage-II cardiac catheterisation (98.5–100%). Digoxin was used by 11/14 centres in 25% of patients (23–31%), and 81% had some oral feeds (68–84%). The majority of the centres (86%) performed a bidirectional Glenn versus hemi-Fontan. Median cardiopulmonary bypass time was 96 minutes (75–113). In aggregate, 26% of patients had deep hypothermic circulatory arrest >10 minutes. In 13/14 centres using deep hypothermic circulatory arrest, 12.5% of patients exceeded 10 minutes (8–32%). Seven centres extubated 5% of patients (2–40) in the operating room. Postoperatively, ICU length of stay was 4.8 days (4.0–5.3) and total length of stay was 7.5 days (6–10).ConclusionsIn the Single Ventricle Reconstruction Trial, practice varied widely among centres for nearly all perioperative factors surrounding Stage II. Further analysis may facilitate establishing best practices by identifying the impact of practice variation.

scholarly journals Seasonality of Hypoplastic Left Heart Syndrome and Single Ventricle Heart in Poland in the Context of Air Pollution

2021 ◽  
Vol 10 (15) ◽  
pp. 3207
Author(s):  
Iwona Strzelecka ◽  
Małgorzata Biedrzycka ◽  
Filip Franciszek Karuga ◽  
Bartosz Szmyd ◽  
Katarzyna Batarowicz ◽  
...  
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Single Ventricle ◽  
Single Case ◽  
Winter Season ◽  
Left Heart ◽  
Target Level ◽  
Hypoplastic Left Heart ◽  
Factors Affecting ◽  
Left Heart Syndrome ◽  
Significant Factors

Hypoplastic left heart syndrome (HLHS) and single ventricle (SV) remain a significant cause of cardiac deaths occurring in the first week of life. Their pathogenesis and seasonal frequency are still unknown. Therefore, we attempt to look at the genesis of the HLHS and SV in the context of territorial distribution as well as seasonality. A total of 193 fetuses diagnosed with HLHS and 92 with SV were selected. The frequency was analyzed depending on the year, calendar month, quarter and season (fall-winter vs. spring-summer). The spatial distribution of HLHS and SV in Poland was analyzed. We observed a statistically significant overrepresentation of HLHS formation frequency in March: 27 (14.00%) in comparison to a monthly median of 15 (IQR: 13.75–16.25; p = 0.039), as well as a significantly higher frequency of HLHS in 2007−2009: 65 cases (33.68%) in comparison to the annual mean of 13.79 ± 6.36 (p < 0.001). We noted a higher frequency of SV among parous with the last menstrual period reported in the fall/winter season of 58 vs. 34 in the spring/summer season (p = 0.014). The performed analysis also revealed significant SV overrepresentation in 2008: 11 cases (12.00%) in comparison to the annual mean of 6.57 ± 2.71 (p = 0.016). Every single case of HLHS was observed when the concentration of benzo(a)pyrene and/or PM10 exceeded the acceptable/target level. Our research indicates that both the season and the level of pollution are significant factors affecting the health of parous women and their offspring. The reason why HLHS and SV develop more frequently at certain times of the year remains unclear, therefore research on this topic should be continued, as well as on the effects of PM10 and benzo(a)pyrene exposure.

Cerebral arterial gas embolism in a patient with hypoplastic left heart syndrome treated with emergent hyperbaric oxygen: case report

2020 ◽  
pp. 431-434
Author(s):  
Mark Binkly ◽  
◽  
Matthew Kelly ◽  
Kevin Hardy ◽  
◽  
...  
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Hyperbaric Oxygen ◽  
Single Ventricle ◽  
Case Reports ◽  
Pulmonary Arteries ◽  
Vena Cava ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Small Areas ◽  
Left Heart Syndrome

A 30-year-old female with a history of seizure disorder and hypoplastic left heart syndrome treated with a Norwood procedure in 1986 followed by a modified non-fenestrated Fontan (Left SVC to IVC to pulmonary arteries) with a known baffle leak presented to the emergency department. On day of presentation, the patient became unresponsive, with perioral cyanosis, rightward gaze and a left facial droop near the end of a platelet transfusion. An emergent non-contrast head CT revealed intracranial air in the right MCA distribution. She was taken to the hyperbaric chamber and was treated with a U.S. Navy Table 6 in a multiplace chamber with no extensions. Ten minutes into the treatment patient became more alert and spontaneously asked questions. The following day she was treated with a U.S. Navy Table 5. Patient had repeat CT of the head, which showed resolution of intracerebral gas and small areas of ischemia in right frontal lobe and right caudate. On hospital day five neurologic exam was normal, with 5/5 strength and no residual deficits. Treating the patient was a concern because patient has a single ventricle, in which the pulmonary artery is connected directly to the vena cava. There is very little data regarding the effects of hyperbaric oxygen (HBO2) therapy on single-ventricle physiology. Only two case reports of three pediatric patients treated with HBO2 for CAGE in a similar setting are known. In these cases the patients had improvements in their symptoms following HBO2. These cases and ours indicate HBO2 is feasible and indicated for CAGE in patients with cyanotic congenital heart disease.

scholarly journals Results of heart transplantation following failed staged palliation of hypoplastic left heart syndrome and related single ventricle anomalies

2015 ◽  
Vol 48 (5) ◽  
pp. 792-799 ◽  
Author(s):  
Bahaaldin Alsoufi ◽  
Shriprasad Deshpande ◽  
Courtney McCracken ◽  
Brian Kogon ◽  
Robert Vincent ◽  
...  
Keyword(s):  
Heart Transplantation ◽  
Hypoplastic Left Heart Syndrome ◽  
Single Ventricle ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome

Interstage Home Monitoring After Newborn First-Stage Palliation for Hypoplastic Left Heart Syndrome: Family Education Strategies

2017 ◽  
Vol 37 (2) ◽  
pp. 72-88 ◽  
Author(s):  
Jo Ann Nieves ◽  
Karen Uzark ◽  
Nancy A. Rudd ◽  
Jennifer Strawn ◽  
Anne Schmelzer ◽  
...  
Keyword(s):  
Home Care ◽  
Hypoplastic Left Heart Syndrome ◽  
Single Ventricle ◽  
Early Recognition ◽  
Home Monitoring ◽  
Growth Failure ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Growth And Survival ◽  
Left Heart Syndrome

Children born with hypoplastic left heart syndrome are at high risk for serious morbidity, growth failure, and mortality during the interstage period, which is the time from discharge home after first-stage hypoplastic left heart syndrome palliation until the second-stage surgical intervention. The single-ventricle circulatory physiology is complex, fragile, and potentially unstable. Multicenter initiatives have been successfully implemented to improve outcomes and optimize growth and survival during the interstage period. A crucial focus of care is the comprehensive family training in the use of home surveillance monitoring of oxygen saturation, enteral intake, weight, and the early recognition of “red flag” symptoms indicating potential cardiopulmonary or nutritional decompensation. Beginning with admission to the intensive care unit of the newborn with hypoplastic left heart syndrome, nurses provide critical care and education to prepare the family for interstage home care. This article presents detailed nursing guidelines for educating families on the home care of their medically fragile infant with single-ventricle circulation.

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