PULMONARY ENDARTERECTOMY IN PEDIATRIC PATIENTS: INSTITUTIONAL EXPERIENCE

BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is rare in children and published experience with pulmonary endarterectomy (PEA) is limited. METHODS: We retrospectively reviewed six patients (<18 years) who underwent seven PEA procedures at our center between December 2011 and September 2025. Clinical characteristics, perioperative findings, hemodynamic outcomes, and follow-up data were analyzed. RESULTS: Median age was 13 years (range, 2-17), and five patients were female. Five had a history of pulmonary embolism, while one presented with hydatid cyst. Risk factors for CTEPH were hydrocephalus with ventriculoperitoneal shunt (n=2), hydatid cyst disease (n=1), infective endocarditis with left ventricular outflow tract obstruction (n=1), and chronic kidney disease with prior COVID-19 infection (n=1). Preoperative mean systolic pulmonary artery pressure (PAP) was 56 ± 23 mmHg, mean pulmonary artery pressure (mPAP) was 39 ± 15 mmHg, and mean pulmonary vascular resistance index (PVRi) was 7.51 ± 4.56 U·m². Most patients were in World Health Organization (WHO) functional class III or IV. All patients underwent PEA, with concomitant procedures required in most cases. Postoperatively, mPAP decreased to 21 ± 4 mmHg and mean PVRi to 2.36 ± 1.36 U·m². Median intensive care unit (ICU) and hospital stays were 3.5 and 9 days, respectively. There was one in-hospital mortality, all other patients recovered without major complications. CONCLUSIONS: PEA is feasible and effective in carefully selected pediatric patients, including those with complex comorbidities. Significant hemodynamic and functional improvements were achieved, supporting the role of PEA as a potentially curative therapy in this rare and challenging group.

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Journal
The Thoracic and Cardiovascular Surgeon
Published
2026-06-17
DOI
https://doi.org/10.1055/a-2875-1852
Primary Topic
Pulmonary Hypertension Research and Treatments
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article
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article

PULMONARY ENDARTERECTOMY IN PEDIATRIC PATIENTS: INSTITUTIONAL EXPERIENCE

Ela Erdem, Koray Ak, Nezih Onur Ermerak, Figen Akalın et al.
The Thoracic and Cardiovascular Surgeon
Pulmonary Hypertension Research and Treatments
article

PULMONARY ENDARTERECTOMY IN PEDIATRIC PATIENTS: INSTITUTIONAL EXPERIENCE

Ela Erdem, Koray Ak, Nezih Onur Ermerak, Figen Akalın, Bedrettin Yıldızeli, Adam Sagir, Çağatay Çetinkaya, Eylem TUNCER
article en

Abstract

BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is rare in children and published experience with pulmonary endarterectomy (PEA) is limited. METHODS: We retrospectively reviewed six patients (<18 years) who underwent seven PEA procedures at our center between December 2011 and September 2025. Clinical characteristics, perioperative findings, hemodynamic outcomes, and follow-up data were analyzed. RESULTS: Median age was 13 years (range, 2-17), and five patients were female. Five had a history of pulmonary embolism, while one presented with hydatid cyst. Risk factors for CTEPH were hydrocephalus with ventriculoperitoneal shunt (n=2), hydatid cyst disease (n=1), infective endocarditis with left ventricular outflow tract obstruction (n=1), and chronic kidney disease with prior COVID-19 infection (n=1). Preoperative mean systolic pulmonary artery pressure (PAP) was 56 ± 23 mmHg, mean pulmonary artery pressure (mPAP) was 39 ± 15 mmHg, and mean pulmonary vascular resistance index (PVRi) was 7.51 ± 4.56 U·m². Most patients were in World Health Organization (WHO) functional class III or IV. All patients underwent PEA, with concomitant procedures required in most cases. Postoperatively, mPAP decreased to 21 ± 4 mmHg and mean PVRi to 2.36 ± 1.36 U·m². Median intensive care unit (ICU) and hospital stays were 3.5 and 9 days, respectively. There was one in-hospital mortality, all other patients recovered without major complications. CONCLUSIONS: PEA is feasible and effective in carefully selected pediatric patients, including those with complex comorbidities. Significant hemodynamic and functional improvements were achieved, supporting the role of PEA as a potentially curative therapy in this rare and challenging group.

The Thoracic and Cardiovascular Surgeon
Üsküdar University (TR), Sağlık Bilimleri Üniversitesi (TR), Marmara University (TR)
Good health and well-being
Openalex Percentile: Top 7%
Pulmonary Hypertension Research and Treatments
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