Hybrid stenting of restrictive atrial septum in an infant with hypoplastic left heart syndrome after hybrid stage 1 palliation

AbstractObjectivesPulmonary lymphangiectasia associated with hypoplastic left heart syndrome with an intact or restrictive atrial septum may result from increased left atrial pressure, and is associated with worse outcomes following staged reconstruction due to lung dysfunction and significant hypoxaemia. Our objective was to characterise the incidence of pulmonary lymphangiectasia in cases of early mortality following stage 1 reconstructions.MethodsAn institutional cardiac surgical database was retrospectively searched for patients who died within 30 days following a stage 1 reconstruction between 1 January, 1984 and 31 December, 2013. During that period, 1669 stage 1 procedures were performed. Autopsy lung specimens were reviewed by a paediatric pathologist. Patients who died of suspected technical issues were excluded.ResultsA total of 54 patients were included, and of these seven cases (8.5%) of pulmonary lymphangiectasia were identified. The mean estimated gestational age was 38.2±2.4 weeks, and the mean birth weight was 3.0±0.6 kg. The median interval between surgery and death was 1 day (with a range from 0 to 18 days). The atrial septum was intact in one patient (14.3%), restrictive in three patients (42.9%), and unrestrictive in three patients (42.9%).ConclusionsPulmonary lymphangiectasia may develop in hypoplastic left heart syndrome with or without a restrictive atrial septum. As standard prenatal diagnostic evaluations and treatment methods for pulmonary lymphangiectasia are limited, this may be an important contributor to early and late mortality following stage 1 reconstruction for hypoplastic left heart syndrome.

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Impact of Hybrid Stage 1 Palliation for Hypoplastic Left Heart Syndrome: Histopathological Findings

Pediatric Cardiology ◽

10.1007/s00246-018-1851-6 ◽

2018 ◽

Vol 39 (5) ◽

pp. 1001-1008 ◽

Cited By ~ 1

Author(s):

Takashi Kido ◽

Takaya Hoashi ◽

Masataka Kitano ◽

Masatoshi Shimada ◽

Kenichi Kurosaki ◽

...

Keyword(s):

Hypoplastic Left Heart Syndrome ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Histopathological Findings ◽

Stage 1 ◽

Left Heart Syndrome ◽

Hybrid Stage 1 Palliation

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Incidence and impact of acute kidney injury in patients with hypoplastic left heart syndrome following the hybrid stage 1 palliation

Cardiology in the Young ◽

10.1017/s1047951120004199 ◽

2020 ◽

pp. 1-7

Author(s):

Tyler W. Cunningham ◽

Yubo Tan ◽

Catherine D. Krawczeski ◽

John D. Spencer ◽

Shasha Bai ◽

...

Keyword(s):

Acute Kidney Injury ◽

Prenatal Diagnosis ◽

Hypoplastic Left Heart Syndrome ◽

Lower Incidence ◽

Kidney Injury ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Stage 1 ◽

Left Heart Syndrome ◽

Hybrid Stage 1 Palliation

Abstract Objective: Acute kidney injury leads to worse outcomes following paediatric cardiac surgery. There is a lack of literature focusing on acute kidney injury after the Hybrid stage 1 palliation for single ventricle physiology. Patients undergoing the Hybrid Stage 1, as a primary option, may have a lower incidence of kidney injury than previously reported. When present, kidney injury may increase the risk of post-operative morbidity and mortality. Methods: A retrospective, single centre review was conducted in patients with hypoplastic left heart syndrome who underwent Hybrid Stage 1 from 2008 to 2018. Acute kidney injury was defined as a dichotomous yes (meeting any injury criteria) or no (no injury) utilising two different criteria utilised in paediatrics. The impact of kidney injury on perioperative characteristics and 30-day mortality was analysed. Results: The incidence of acute kidney injury is 13.4–20.7%, with a severe injury rate of 2.4%. Patients without a prenatal diagnosis of hypoplastic left heart syndrome have a higher incidence of kidney injury than those prenatally diagnosed, (40% versus 14.5%, p = 0.024). Patients with acute kidney injury have a significantly higher incidence of 30-day mortality, 27.3%, compared to without, 5.6% (p = 0.047). Discussion: The incidence of severe acute kidney injury after the Hybrid Stage 1 palliation is low. A prenatal diagnosis may be associated with a lower incidence of kidney injury following the Hybrid Stage 1. Though uncommon, severe acute kidney injury following Hybrid Stage 1 may be associated with higher 30-day mortality.

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