Pulmonary Hypertension in Patients with Congenital Diaphragmatic Hernia: Does Lung Size Matter?

Madenci, Arin L.; Church, John; Gajarski, Robert J.; Marchetti, Kathryn; Klein, Edwin J.; Coughlin, Megan A.; Kreutzmann, Jeannie; Treadwell, Marjorie C.; Ladino-Torres, Maria; Mychaliska, George B.

doi:10.1055/s-0037-1607291

Cited by 11 publications

(6 citation statements)

References 49 publications

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“…However, the same group later demonstrated a relationship between o/e TFLV and severity of persistent pulmonary hypertension in a separate study. 33 …”

Section: Lung Volumementioning

confidence: 99%

Fetal lung MRI and features predicting post-natal outcome: a scoping review of the current literature

Whitby

Gaunt

2023

BJR

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The outcome for infants with fetal lung pathologies not only depends on the nature of the pathology, but the impact it has on the developing lungs. The main prognostic factor is the degree of pulmonary hypoplasia, but this is not detectable pre-natally. Imaging techniques aim to simulate these features with a variety of surrogate measurements, including lung volume and MRI signal intensity. Despite the complexity of the various research studies and lack of consistent methodology, this scoping review aims to summarise current applications, and promising techniques requiring further investigation.

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“…However, the same group later demonstrated a relationship between o/e TFLV and severity of persistent pulmonary hypertension in a separate study. 33 …”

Section: Lung Volumementioning

confidence: 99%

Fetal lung MRI and features predicting post-natal outcome: a scoping review of the current literature

Whitby

Gaunt

2023

BJR

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“…As seen in Fig 1, intrauterine herniation of abdominal organs into the fetal thorax is associated with disrupted organogenesis of the heart and lungs. Pulmonary hypoplasia and pulmonary hypertension (PH) have long been recognized as the key pathophysiological components of CDH and contributors to disease severity and outcomes 4 .…”

Section: Introductionmentioning

confidence: 99%

“…Pulmonary hypoplasia and pulmonary hypertension (PH) have long been recognized as the key pathophysiological components of CDH and contributors to disease severity and outcomes. 4 There is a growing appreciation that postnatal cardiac dysfunction may also be a key component of CDH pathophysiology. 5 Both right and left ventricular function may be affected as a result of fetal and postnatal changes in ventricular development and loading conditions.…”

mentioning

confidence: 99%

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“Heart of the Matter”: Cardiac Dysfunction in Congenital Diaphragmatic Hernia

Gowda

Patel

2023

Am J Perinatol

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Despite advances in caring for neonates with congenital diaphragmatic hernia (CDH), mortality and morbidity continues to be high. Additionally, the pathophysiology of cardiac dysfunction in this condition is poorly understood. Postnatal cardiac dysfunction in neonates with CDH may be multifactorial with origins in fetal life. Mechanical obstruction, competition from herniated abdominal organs into thoracic cavity combined with redirection of ductus venosus flow away from patent foramen ovale leading to smaller left-sided structures may be a contributing factor. This shunting decreases left atrial and left ventricular blood volume which may result in altered micro- and macro- vascular aberrations affecting cardiac development in the prenatal period. Direct mass effect from herniated intra-abdominal contents restricting cardiac growth, and/or reduced left ventricular preload may contribute independently to left ventricular dysfunction in the absence of right ventricular dysfunction and or pulmonary hypertension. With variable clinical phenotypes of cardiac dysfunction, pulmonary hypertension, and respiratory failure in patients with CDH, there is increased need for individualized diagnosis and tailored therapy. Routine use of therapy such as inhaled nitric oxide (iNO) and sildenafil which induces significant pulmonary vasodilation may be detrimental in LV dysfunction whereas in a patient with pure RV dysfunction, they may be beneficial. Targeted functional echocardiography serves as a real time tool for defining the pathophysiology and aids optimization of vasoactive therapy in affected neonates.

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“…These conditions reduce the risk of anesthesia and increase the postoperative survival (4,5). Newborns with mild-to-moderate CDH, based on the lung-to-head ratio (LHR ≥ 1.0), are more likely to meet these physiological standards compared to severe cases (LHR < 1.0) (6). Therefore, one of the main reasons why the optimal timing of surgery remains unclear is that there is no separate study on mild-to-moderate and severe cases, while the optimal timing of surgery for these cases should be different.…”

Section: Introductionmentioning

confidence: 99%

Surgical Timing in Left Mild-to-Moderate Congenital Diaphragmatic Hernia

Liu

Hong

et al. 2021

Iran J Pediatr

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Background: The optimal timing of surgery for left-sided mild-to-moderate congenital diaphragmatic hernia (CDH) remains unknown. Objectives: To determine the optimal timing of surgery for left-sided mild-to-moderate CDH. Methods: Thirty newborns were randomly divided into emergency (EAR) and delayed (DEL) surgery groups. Thoracoscopic repair of CDH was performed within 48 hours after birth in the EAR group and then in the DEL group. Next, the baseline data, primary and secondary endpoints, and adverse reactions were assessed. Results: Differences between the two groups were not significant in terms of the measured lung-to-head ratio (LHR), preoperative pulmonary artery hypertension (PAH)-free/mild PAH ratio, surgery duration, duration of postoperative mechanical ventilation, incidence of postoperative moderate-to-severe PAH, postoperative mortality, and recurrence rate in the follow-up (P > 0.05 for all). Meanwhile, age at surgery (P = 0.001), duration of fasting (P = 0.001), and hospital stay (P = 0.032) were significantly different between the two groups. Conclusions: Timing of thoracoscopy, performed within 85 hours of birth for left-sided CDH repair, does not affect the therapeutic outcomes of children with left-sided mild-to-moderate CDH.

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Pulmonary Hypertension in Patients with Congenital Diaphragmatic Hernia: Does Lung Size Matter?

Abstract: Among infants with isolated CDH, PPLV, and o:eLHR were significantly associated with PHTN severity, especially among patients requiring ECMO. Prenatal lung size may help predict postnatal PHTN and associated therapies.

Cited by 11 publications

References 49 publications

Fetal lung MRI and features predicting post-natal outcome: a scoping review of the current literature

Fetal lung MRI and features predicting post-natal outcome: a scoping review of the current literature

“Heart of the Matter”: Cardiac Dysfunction in Congenital Diaphragmatic Hernia

Surgical Timing in Left Mild-to-Moderate Congenital Diaphragmatic Hernia

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