Lung volumes, ventricular function and pulmonary arterial flow in children operated on for left-sided congenital diaphragmatic hernia: long-term results

Abolmaali, Nasreddin; Koch, A.; Götzelt, Knut; Hahn, Gabriele; Fitze, Guido; Vogelberg, Christian

doi:10.1007/s00330-010-1723-9

Cited by 20 publications

(4 citation statements)

References 29 publications

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“…Indeed, several reports on lung morphology after neonatal repair of CDH describe an increase in alveolar size27 and chest radiographs are frequently abnormal in CDH survivors 28. A recent study comparing MRI‐based functional pulmonary measurements in 12 CDH patients and healthy matched controls revealed normal volumes on pulmonary function testing and increased ipsilateral lung volume on MRI in CDH 29. Those CDH patients had a smaller cross‐sectional area and lower flow parameters of the left pulmonary artery.…”

Section: Discussionmentioning

confidence: 99%

Lung function in young adults with congenital diaphragmatic hernia; a longitudinal evaluation

Spoel

Zijp

Hop

et al. 2012

Pediatric Pulmonology

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Section: Discussionmentioning

confidence: 99%

Lung function in young adults with congenital diaphragmatic hernia; a longitudinal evaluation

Spoel

Zijp

Hop

et al. 2012

Pediatric Pulmonology

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“…large and small CDH). Also, previous studies using functional lung MRI (DCE, 3He) found less subdivided, enlarged alveoli, and reduced ipsilateral perfusion in patients with CDH [ 22 – 26 , 51 ]. In our study, we show that in patients with a large CDH ipsilateral impairment of ventilation and perfusion is densely clustered (high DDI V and DDI Q ) and overlapping in accordance with the Euler-Liljestrand-effect (high VQD match ).…”

Section: Discussionmentioning

confidence: 92%

Long-term pulmonary outcome of children with congenital diaphragmatic hernia: functional lung MRI using matrix-pencil decomposition enables side-specific assessment of lung function

Streibel,

Willers,

Bauman

et al. 2023

Eur Radiol

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Objectives In patients with congenital diaphragmatic hernia (CDH) the exact functional outcome of the affected lung side is still unknown, mainly due to the lack of spatially resolved diagnostic tools. Functional matrix-pencil decomposition (MP-) lung MRI fills this gap as it measures side-specific ventilation and perfusion. We aimed to assess the overall and side-specific pulmonary long-term outcomes of patients with CDH using lung function tests and MP-MRI. Methods Thirteen school-aged children with CDH (seven with small and six with large defect-sized CDH, defined as > 50% of the chest wall circumference being devoid of diaphragm tissue) and thirteen healthy matched controls underwent spirometry, multiple-breath washout, and MP-MRI. The main outcomes were forced expiratory volume in 1 second (FEV1), lung clearance index (LCI2.5), ventilation defect percentage (VDP), and perfusion defect percentage (QDP). Results Patients with a large CDH showed significantly reduced overall lung function compared to healthy controls (mean difference [95%-CIadjusted]: FEV1 (z-score) −4.26 [−5.61, −2.92], FVC (z-score) −3.97 [−5.68, −2.26], LCI2.5 (TO) 1.12 [0.47, 1.76], VDP (%) 8.59 [3.58, 13.60], QDP (%) 17.22 [13.16, 21.27]) and to patients with a small CDH. Side-specific examination by MP-MRI revealed particularly reduced ipsilateral ventilation and perfusion in patients with a large CDH (mean difference to contralateral side [95%-CIadjusted]: VDP (%) 14.80 [10.50, 19.00], QDP (%) 23.50 [1.75, 45.20]). Conclusions Data indicate impaired overall lung function with particular limitation of the ipsilateral side in patients with a large CDH. MP-MRI is a promising tool to provide valuable side-specific functional information in the follow-up of patients with CDH. Clinical relevance statement In patients with congenital diaphragmatic hernia, easily applicable MP-MRI allows specific examination of the lung side affected by the hernia and provides valuable information on ventilation and perfusion with implications for clinical practice, making it a promising tool for routine follow-up. Key Points • Functional matrix pencil decomposition (MP) MRI data from a small sample indicate reduced ipsilateral pulmonary ventilation and perfusion in children with large congenital diaphragmatic hernia (CDH). • Easily applicable pencil decomposition MRI provides valuable side-specific diagnostic information on lung ventilation and perfusion. This is a clear advantage over conventional lung function tests, helping to comprehensively follow up patients with congenital diaphragmatic hernia and monitor therapy effects.

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“…Flow, acceleration time and cross-sectional area of the left pulmonary artery were reduced. Long-term significance of these changes remains unclear however ( 55 ).…”

Section: Long Term Cardiovascular Outcome In Cdh-phmentioning

confidence: 99%

Diagnosis, management and long term cardiovascular outcomes of phenotypic profiles in pulmonary hypertension associated with congenital diaphragmatic hernia

Chaudhari,

Schmidt Sotomayor,

Maheshwari

2024

Front. Pediatr.

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Congenital diaphragmatic hernia (CDH) is a developmental defect of the diaphragm resulting in herniation of viscera into the chest. This condition is characterized by pulmonary hypoplasia, pulmonary hypertension (PH) and cardiac ventricular dysfunction. PH is a key component of the pathophysiology of CDH in neonates and contributes to morbidity and mortality. Traditionally, PH associated with CDH (CDH-PH) is thought to be secondary to increased pulmonary arterial resistance and vasoreactivity resulting from pulmonary hypoplasia. Additionally, there is increasing recognition of associated left ventricular hypoplasia, dysfunction and elevated end diastolic pressure resulting in pulmonary venous hypertension in infants with CDH. Thus, hemodynamic management of these infants is complex and cautious use of pulmonary vasodilators such as inhaled nitric oxide (iNO) is warranted. We aim to provide an overview of different phenotypic profiles of CDH associated PH and potential management options based on current evidence and pathophysiology.

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Lung volumes, ventricular function and pulmonary arterial flow in children operated on for left-sided congenital diaphragmatic hernia: long-term results

Abstract: Functional MRI detected pulmonary and cardiac findings in the late follow-up of CDH children which may be missed by standard clinical methods and might be relevant for decisions regarding late outcome and treatment.

Cited by 20 publications

References 29 publications

Lung function in young adults with congenital diaphragmatic hernia; a longitudinal evaluation

Lung function in young adults with congenital diaphragmatic hernia; a longitudinal evaluation

Long-term pulmonary outcome of children with congenital diaphragmatic hernia: functional lung MRI using matrix-pencil decomposition enables side-specific assessment of lung function

Diagnosis, management and long term cardiovascular outcomes of phenotypic profiles in pulmonary hypertension associated with congenital diaphragmatic hernia

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