Pulse Oximetry Is Insufficient for Timely Diagnosis of Hepatopulmonary Syndrome in Children with Liver Cirrhosis

• Published in: The Journal of pediatrics Vol. 164; no. 3; pp. 546 - 552.e2
• Main Authors: Hoerning, André, MD, Raub, Simon, Neudorf, Ulrich, MD, Müntjes, Carsten, MD, Kathemann, Simone, MD, Lainka, Elke, MD, Stehling, Florian, MD, Hoyer, Peter F., MD, Gerner, Patrick, MD
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01-03-2014
• Subjects: Adolescent; Blood Gas Analysis; Capillaries - chemistry; Child; Child, Preschool; Contrast Media; Early Diagnosis; Echocardiography; Female; Hepatopulmonary Syndrome - diagnosis; Humans; Infant; Liver Circulation; Liver Cirrhosis - complications; Liver Cirrhosis - surgery; Liver Transplantation; Male; Oximetry; Oxygen - blood; Pediatrics; Portoenterostomy, Hepatic - statistics & numerical data; Prospective Studies; Pulmonary Alveoli - metabolism; Sensitivity and Specificity; Severity of Illness Index; Arterial blood gas; ABG; SO 2; CBG; Capillary blood gas; HPS; cTTE; Oxygen saturation; Intrapulmonary vasodilatation; Hepatopulmonary syndrome; IPVD; PΔAaO 2; Alveolar arterial oxygen gradient; Contrast-enhanced transthoracic echocardiography; SO2; PΔAaO2
• ISSN: 0022-3476; 1097-6833
• DOI: 10.1016/j.jpeds.2013.10.070

Objective To prospectively investigate the prevalence of hepatopulmonary syndrome (HPS), the importance of pulse oximetry in diagnosing HPS, and the longitudinal course after liver transplantation in children with cirrhosis referred for liver transplantation. Study design Fifty-six patients aged 1-17 years (mean age, 4.6 ± 5.0 years) with liver cirrhosis were screened for HPS by hyperemic capillary blood gas (CBG) analysis and contrast-enhanced transthoracic echocardiography. Eleven patients were excluded owing to conditions that can produce cardiopulmonary dysfunction, including 5 with cystic fibrosis, 1 with pulmonary arterial hypertension, and 5 with an intracardial shunt. HPS was classified in accordance with the European Respiratory Society Task Force criteria on pulmonary-hepatic disorders. Patient groups were compared for biochemical and clinical characteristics. Results Eighteen children (40%) with cirrhosis were intrapulmonary vasodilatation (IPVD)-positive and had a pulse oximetry oxygen saturation level >98%. Two of these patients (11%) exhibited moderate HPS with an elevated alveolar arterial oxygen gradient >15 mm Hg and PaO2 <70 mm Hg; they died before undergoing liver transplantation. The sensitivity and specificity of CBG analysis for detecting elevated alveolar arterial oxygen gradient in children with IPVD was 94% and 53%, respectively. HPS was associated with late hepatoportoenterostomy ( P < .04). Liver transplantation led to resolution of HPS in all patients. Conclusion IPVD is frequent in children with liver cirrhosis (40%). Pulse oximetry is insufficient for timely HPS diagnosis. Pathological CBG analysis data indicate IPVD in the majority of cases, but are imprecise in children aged <2 years. Contrast-enhanced transthoracic echocardiography and CBG analysis are recommended for evaluation of HPS in children with cirrhosis, regardless of liver synthesis capacity and clinical chemistry data.