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

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.

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Developmental outcome of patients with hypoplastic left heart syndrome treated with heart transplantation

ACC Current Journal Review ◽

10.1016/s1062-1458(03)00221-6 ◽

2003 ◽

Vol 12 (3) ◽

pp. 92

Author(s):

L. Ikle ◽

K. Hale ◽

L. Fashaw ◽

M. Boucek ◽

A.A. Rosenberg

Keyword(s):

Heart Transplantation ◽

Hypoplastic Left Heart Syndrome ◽

Developmental Outcome ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Left Heart Syndrome

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

Undersea and Hyperbaric Medicine ◽

10.22462/03.20.2020.13 ◽

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.

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Outcome of infants with hypoplastic left heart syndrome who undergo atrial septostomy before heart transplantation

The American Journal of Cardiology ◽

10.1016/s0002-9149(99)00624-4 ◽

2000 ◽

Vol 85 (1) ◽

pp. 124-127 ◽

Cited By ~ 8

Author(s):

Micheal A Kuhn ◽

Ranae L Larsen ◽

Neda F Mulla ◽

Joyce K Johnston ◽

Richard E Chinnock ◽

...

Keyword(s):

Heart Transplantation ◽

Hypoplastic Left Heart Syndrome ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Atrial Septostomy ◽

Left Heart Syndrome

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Associations Between Day of Admission and Day of Surgery on Outcome and Resource Utilization in Infants With Hypoplastic Left Heart Syndrome Who Underwent Stage I Palliation (from the Single Ventricle Reconstruction Trial)

The American Journal of Cardiology ◽

10.1016/j.amjcard.2015.07.044 ◽

2015 ◽

Vol 116 (8) ◽

pp. 1263-1269 ◽

Cited By ~ 2

Author(s):

Joyce T. Johnson ◽

Lynn A. Sleeper ◽

Shan Chen ◽

Richard G. Ohye ◽

Michael G. Gaies ◽

...

Keyword(s):

Hypoplastic Left Heart Syndrome ◽

Resource Utilization ◽

Single Ventricle ◽

Stage I ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Left Heart Syndrome

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

Critical Care Nurse ◽

10.4037/ccn2017763 ◽

2017 ◽

Vol 37 (2) ◽

pp. 72-88 ◽

Cited By ~ 10

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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