What question did this study set out to answer?

To evaluate clinical outcomes, atrial morphology, left atrial decompression pathways, and pulmonary histopathology in infants with hypoplastic left heart syndrome and an intact atrial septum.

August 22, 2026Journal of the American College of CardiologyOpen Access

The hypoplastic left heart syndrome with intact atrial septum: atrial morphology, pulmonary vascular histopathology and outcome

Q: What is the clinical evidence from this study?

Study design: Observational. Population: Hypoplastic left heart syndrome with intact atrial septum (n=18). Intervention: Intact atrial septum. Primary outcome: Mortality.

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Key result

Intact atrial septum in HLHS linked to high mortality despite intervention, reflecting severe pulmonary vasculopathy.

n=18

Population

18 infants with hypoplastic left heart syndrome and intact atrial septum

Design

Cohort

Authors

JRJack RychikPediatric Cardiology JRJonathan J. RomeDeborah Heart and Lung Center MCMargaret H. CollinsCincinnati Children's Hospital Medical Center

Discussion

Member takes

Implication

Infants with hypoplastic left heart syndrome and intact atrial septum have very poor outcomes despite aggressive surgical intervention, with atrial morphology serving as a marker for the severity of pulmonary vascular disease.

Key Points

To evaluate clinical outcomes, atrial morphology, left atrial decompression pathways, and pulmonary histopathology in infants with hypoplastic left heart syndrome and an intact atrial septum.
Retrospective review of clinical records and echocardiograms for 18 infants (5.7%) with intact atrial septum identified among 316 infants with hypoplastic left heart syndrome over a 6.5-year period at a single center.
Classification of atrial morphology (types A, B, and C), assessment of left atrial decompression pathway obstruction, surgical procedures (Norwood operation or balloon atrial septostomy), and lung histopathology analysis.
Echocardiography categorized 18 infants into three atrial morphology types: type A (large left atrium, n=12), type B (small left atrium with thick muscular septum, n=4), and type C (giant left atrium with severe mitral regurgitation, n=2), with severe pathway obstruction noted in 7 infants (3/12 type A, 4/4 type B).
Surgical intervention via the Norwood operation was performed in 17 infants and emergency balloon atrial septostomy in 1 (who died); early survival occurred exclusively in 6 infants (all type A with unobstructed pathways), of whom 3 died after subsequent cavopulmonary surgery.
Lung histopathology demonstrated severely dilated lymphatics and arterialization of pulmonary veins, which was most severe in infants with type B atrial morphology and marked obstruction to left atrial egress.

Study Design

Type

Observational (n=18)

Multicenter

Structured PICO

Population

18 infants with hypoplastic left heart syndrome and intact atrial septum evaluated at a single center over a 6.5-year period.

Exposure

Norwood operation (n=17) or emergency balloon atrial septostomy (n=1)

Outcome

Mortality/survival and its relationship to atrial morphology, left atrial decompression pathway, and lung histopathologyhard clinical

Cite This Study

Rychik et al. (1999) conducted an observational in Hypoplastic left heart syndrome with intact atrial septum (n=18). Intact atrial septum was evaluated on Mortality. Infants with hypoplastic left heart syndrome and intact atrial septum face high mortality despite aggressive intervention, with atrial morphology indicating pulmonary vascular disease severity.

synapsesocial.com/papers/6a8969804725fca49978452c https://doi.org/10.1016/s0735-1097(99)00225-9

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August 22, 2026Journal of the American College of CardiologyOpen Access

The hypoplastic left heart syndrome with intact atrial septum: atrial morphology, pulmonary vascular histopathology and outcome

View Full Paper

Ask AI

Bookmark

Key result

Intact atrial septum in HLHS linked to high mortality despite intervention, reflecting severe pulmonary vasculopathy.

n=18

Population

18 infants with hypoplastic left heart syndrome and intact atrial septum

Design

Cohort

Authors

JRJack RychikPediatric Cardiology JRJonathan J. RomeDeborah Heart and Lung Center MCMargaret H. CollinsCincinnati Children's Hospital Medical Center

Discussion

Member takes

Implication

Key Points

To evaluate clinical outcomes, atrial morphology, left atrial decompression pathways, and pulmonary histopathology in infants with hypoplastic left heart syndrome and an intact atrial septum.
Retrospective review of clinical records and echocardiograms for 18 infants (5.7%) with intact atrial septum identified among 316 infants with hypoplastic left heart syndrome over a 6.5-year period at a single center.
Classification of atrial morphology (types A, B, and C), assessment of left atrial decompression pathway obstruction, surgical procedures (Norwood operation or balloon atrial septostomy), and lung histopathology analysis.
Echocardiography categorized 18 infants into three atrial morphology types: type A (large left atrium, n=12), type B (small left atrium with thick muscular septum, n=4), and type C (giant left atrium with severe mitral regurgitation, n=2), with severe pathway obstruction noted in 7 infants (3/12 type A, 4/4 type B).
Surgical intervention via the Norwood operation was performed in 17 infants and emergency balloon atrial septostomy in 1 (who died); early survival occurred exclusively in 6 infants (all type A with unobstructed pathways), of whom 3 died after subsequent cavopulmonary surgery.
Lung histopathology demonstrated severely dilated lymphatics and arterialization of pulmonary veins, which was most severe in infants with type B atrial morphology and marked obstruction to left atrial egress.

Study Design

Type

Observational (n=18)

Multicenter

Structured PICO

Population

18 infants with hypoplastic left heart syndrome and intact atrial septum evaluated at a single center over a 6.5-year period.

Exposure

Norwood operation (n=17) or emergency balloon atrial septostomy (n=1)

Outcome

Mortality/survival and its relationship to atrial morphology, left atrial decompression pathway, and lung histopathologyhard clinical

Cite This Study

synapsesocial.com/papers/6a8969804725fca49978452c https://doi.org/10.1016/s0735-1097(99)00225-9

Also Consider

Synapse has enriched 3 closely related papers on similar clinical questions. Consider them for comparative context:

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