Finding a Path to the Heart: Right Heart Catheterization in Patients with Right Congenital Diaphragmatic Hernia and Anomalous Venous Anatomy

Children with congenital diaphragmatic hernia (CDH) may require cardiac catheterization for evaluation of pulmonary hypertension. The extent of abnormal systemic venous return complicating access to the right heart in this population has not been well described. We identified children with CDH who underwent cardiac catheterization at the University of California, San Francisco, at less than or equal to 2 years from 2006 to 2025. Non-invasive imaging, catheterization reports, and angiography were reviewed. Nine children with right CDH (three with hepatopulmonary fusion) and twenty-one children with left CDH underwent cardiac catheterization. Initial catheterization occurred at 4 weeks-2 years of age and 1.9-13 kg. The right heart was directly accessed from the inferior vena cava (IVC) via the femoral vein in all patients with left CDH. Patients with right CDH had abnormal venous anatomy precluding direct IVC-right atrial access in several. This was likely secondary to IVC compression at the diaphragm. Fluoroscopy times in the latter group were shorter with superior vena cava access via the internal jugular vein compared to inferior vena cava access via the femoral vein. Abnormal systemic venous anatomy complicating venous access to the right heart should be considered for patients with right CDH, especially those with hepatopulmonary fusion. If not already delineated by non-invasive imaging, initial angiography from the femoral vein can guide the approach to cardiac catheterization.