Diaphragmatic breathing

Intensive care medicine

Extracorporeal membrane oxygenation

Biology

Hypoplasia

Surgery

Fetus

Cardiology

Pulmonary hypoplasia

Hernia

Congenital diaphragmatic hernia

Alternative medicine

Genetics

Heart failure

Hypoplastic left heart syndrome

Bronchopulmonary dysplasia

Pathophysiology

Gestational age

Cardiac function curve

Internal medicine

Pulmonary hypertension

Heart disease

Diaphragmatic hernia

Medicine

Pregnancy

Pathology

Frontiers in Pediatrics

There is growing recognition that the heart is a key contributor to the pathophysiology of congenital diaphragmatic hernia (CDH), in conjunction with developmental abnormalities of the lung and pulmonary vasculature. Investigations to date have demonstrated altered fetal cardiac morphology, notably relative hypoplasia of the fetal left heart, as well as early postnatal right and left ventricular dysfunction which appears to be independently associated with adverse outcomes. However, many more unknowns remain, not least an understanding of the genetic and cellular basis for cardiac dysplasia and dysfunction in CDH, the relationship between fetal, postnatal and long-term cardiac function, and the impact on other parts of the body especially the developing brain. Consensus on how to measure and classify cardiac function and pulmonary hypertension in CDH is also required, potentially using both non-invasive imaging and biomarkers. This may allow routine assessment of the relative contribution of cardiac dysfunction to individual patient pathophysiological phenotype and enable better, individualized therapeutic strategies incorporating targeted use of fetal therapies, cardiac pharmacotherapies, and extra-corporeal membrane oxygenation (ECMO). Collaborative, multi-model approaches are now required to explore these unknowns and fully appreciate the role of the heart in CDH.

The heart in congenital diaphragmatic hernia: Knowns, unknowns, and future priorities