Objective: Diaphragm paralysis owing to phrenic nerve injury can result in significant morbidity in children undergoing surgical management of congenital cardiac defects. Diaphragm plication is the accepted therapy for diaphragm paralysis. We have investigated subdiaphragmatic venous hemodynamics in patients with biventricular and Fontan circulation after diaphragm plication.
Methods: Doppler ultrasound was used to evaluate flows in the hepatic vein, portal vein, and subhepatic inferior vena cava under respiratory monitoring and with a tilt table. Twenty-nine patients with biventricular circulation were studied: 19 with normal diaphragms and 10 after diaphragm plication. Twenty-eight patients with total cavopulmonary connections after the Fontan procedure were also studied: 19 with normal diaphragms and 9 with plicated diaphragms. Inspiratory/expiratory flow ratios in supine and upright positions were calculated to investigate respiratory effects, and upright/supine flow ratios were calculated to assess gravity effects.
Results: In patients with biventricular circulation and normal diaphragms, hepatic venous flow was augmented by inspiration; this effect was reduced in patients with a plicated diaphragm (upright inspiratory/expiratory flow ratios: 2.4 vs 1.4, respectively; P = .01). Portal venous flow was higher during expiration; this effect was lost in patients with a plicated diaphragm (supine inspiratory/expiratory flow ratios: 0.8 and 1.0; P < .05). In Fontan patients with normal diaphragms, hepatic venous flow depended heavily on inspiration. This effect was blunted in patients with a plicated diaphragm (supine inspiratory/expiratory flow ratios: 3.2 vs 2.3; P < .05). Expiratory augmentation of portal flow was absent in Fontan patients with normal diaphragms and reversed in patients a plicated diaphragm (supine inspiratory/expiratory flow ratios: 1.0 vs 1.6; P = .02). Gravity reduced Fontan portal venous flow; having a plicated diaphragm did not alter this effect (upright/supine flow ratios: 0.7 vs 0.7).
Conclusions: In patients with biventricular and those with Fontan circulation with a paralyzed diaphragm, plication does not completely restore normal subdiaphragmatic venous hemodynamics. In Fontan patients with a plicated diaphragm, important inspiration-derived hepatic venous flow is suppressed, and portal venous flow loses its normal expiratory augmentation. These flow dynamics share similarities with those observed in patients with failing Fontan circulation. This suboptimal splanchnic circulation may contribute to early problems of prolonged pleural effusions and ascites and potentially may promote late Fontan failure. Phrenic nerve injury should consequently be avoided at all costs before or at the time of the Fontan operation.