Hemodynamic phenotypes in CDH: TnECHO characteristics and management
| Phenotype | No PHT and normal cardiac function | PHT and normal/ solely RV Dysfunction | PHT with LV or biventricular dysfunction |
| TnECHO characteristics | PDA flow direction: left-to-right; PFO/ASD flow direction: left-to-right | Impaired RV systolic & diastolic function PDA flow direction: Bidirectional/ right-to-left; PFO/ASD flow direction: Bidirectional/ right-to-left RV dilatation | PDA flow direction: Bidirectional/ right-to-left; PFO/ASD flow direction: left-to-right Decreased LV size (fetal hypoplasia and paradoxical IVS movement) Impaired LV (and/or RV) systolic & diastolic functions |
| Hemodynamic Treatment options/ precautions | Close monitoring of haemodynamic status, particularly during peri-operative period – blood pressure, lactate level, serial TnECHO | Use of dobutamine/ milrinone (if normotensive)/ low-dose epinephrine infusion to augment RV systolic function Use of iNO to reduce RV afterload Use of PGE1 to improve systemic flow in case of right-to-left shunting and closing ductus Use of vasopressin/ norepinephrine as vasopressors to improve SVR/PVR ratio | Use of dobutamine/ milrinone (if normotensive)/ low-dose epinephrine infusion to augment systolic function Use of PGE1 to improve systemic flow in case of right-to-left shunting and closing ductus AVOID high-dose dobutamine due to its chronotopic effects which may reduce ventricular filling and exacerbate diastolic dysfunction PRUDENT use of vasoconstrictors to avoid excessive LV afterload AVOID sole use of pulmonary vasodilator like iNO which may cause pulmonary venous congestion |
ASD, atrial septal defect; IVS, interventricular septum; LV, left ventricle; PDA, patent ductus arteriosus; PFO, patent foramen ovale; PGE, prostaglandins; PHT, pulmonary hypertension; PVR, pulmonary vascular resistance; RV, right ventricle; SVR, systemic vascular resistance; TnECHO, targeted neonatal echocardiography.