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Factors Associated with Early-Childhood Ipsilateral Perfusion Abnormalities among Patients with Congenital
Akila B Ramaraj1, Hannah V Breitschopf2, Kylie I Holden2
1Division of Pediatric General and Thoracic Surgery, Seattle Children's Hospital, Seattle, WA; Department of General Surgery, UConn Health, Farmington, CT.
Insights
Congenital diaphragmatic hernia (CDH) patients exhibit persistent ipsilateral lung perfusion deficits up to five years post-discharge. Defect size is the primary factor influencing these long-term perfusion abnormalities.
Area of Science:
- Pediatric Surgery
- Pulmonary Medicine
- Medical Imaging
Background:
- Congenital diaphragmatic hernia (CDH) is a complex birth defect affecting lung development.
- Long-term respiratory outcomes, particularly lung perfusion, require further investigation.
- Understanding post-discharge perfusion patterns is crucial for patient management and family counseling.
Purpose of the Study:
- To evaluate long-term, postdischarge, ipsilateral lung perfusion patterns in patients with congenital diaphragmatic hernia (CDH).
- To identify factors associated with these perfusion patterns up to five years after discharge.
- To provide insights for counseling families regarding the long-term prognosis of CDH.
Main Methods:
- A multi-center, long-term follow-up database was established for patients with left-sided CDH.
- Ipsilateral lung perfusion was assessed at 2 and 5 years post-discharge.
- Univariable and multivariable generalized linear modeling analyzed perfusion data and associated factors.
Main Results:
- All evaluated CDH patients demonstrated abnormal ipsilateral lung perfusion at 2 and 5 years.
- Median ipsilateral perfusion was significantly lower than normal, with notable differences between low-risk and high-risk defects.
- Ipsilateral perfusion at 2 years and CDH defect stage were significant predictors of 5-year perfusion.
Conclusions:
- Persistent ipsilateral lung perfusion deficits are characteristic of CDH patients long after discharge.
- CDH defect size is the sole identified factor significantly associated with long-term perfusion.
- Pulmonary hypertension at discharge did not correlate with perfusion deficits, warranting further clinical correlation.
Objectives:
To evaluate long-term, postdischarge, ipsilateral lung perfusion patterns and to identify associated factors among patients with congenital diaphragmatic hernia (CDH).
Study Design:
A long-term follow-up database was created to assimilate data across 4 centers, covering patients with left-sided CDH managed as outpatients between 2010 and 2021. Outcomes from discharge to age 5, focusing on ipsilateral lung perfusion at 2 and 5 years, were analyzed using both univariable and multivariable generalized linear modeling.
Results:
Among the 258 patients with left-sided CDH, 213 (83%) and 100 (39%) had 2- and 5-year follow-up data, respectively. Of these, 173 patients (68%) had low-risk (A/B) defects. At discharge, 58 (22.5%) needed supplemental oxygen and 56 (21.7%) had pulmonary hypertension on echocardiogram. Perfusion data were available for 121 patients (47%) at 2 years and 54 (21%) at 5 years. Compared with the normal left lung perfusion index of 45%-50% at 2 years of age, patients with CDH had abnormal ipsilateral perfusion deficits, with a median of 37.0% for the cohort, and a median ipsilateral perfusion of 39.0% for low-risk defects and 31.0% for high-risk defects, an absolute difference of 8.0 (P < .001). In the longitudinal multivariable analysis, only ipsilateral perfusion at 2 years (P < .001) and CDH defect stage (P = .025) were associated significantly with ipsilateral perfusion at 5 years.
Conclusions:
All patients with CDH had abnormal ipsilateral perfusion at 2 and 5 years, with defect size as the only factor associated with perfusion. Pulmonary hypertension was not associated with perfusion. Further clinical correlation is needed. These findings may help when counseling families on long-term outcomes.
