Hepatopulmonary Syndrome in Children: A 20‐Year Review of Presenting Symptoms, Clinical Progression, and Transplant Outcome

Abstract

Hepatopulmonary syndrome (HPS) in stable patients with cirrhosis can easily be overlooked. We report on the presenting symptoms, disease progression, and outcomes after liver transplantation (LT) in children with HPS. Twenty patients were diagnosed with HPS between 1996 and 2016. The etiologies were as follows: biliary atresia (n = 9); alpha-1-antitrypsin deficiency (n = 2); cryptogenic liver disease (n = 3); and others (n = 6). HPS presentations were as follows; dyspnea (n = 17) and pneumonia (n = 3). For diagnostic confirmation, the following techniques were used: technetium-99m-labeled macroaggregated albumin lung perfusion scan (n = 13) or contrast echocardiogram (n = 7). There were 16 patients listed for LT, with a median age at HPS diagnosis of 10 years and an average wait from listing to LT of 9 weeks. A marked rise in hemoglobin (Hb; median, 125-143.5 g/L) and modest decrease in oxygen saturation (SpO ; median 91% to 88% room air) were evident over this time. Patients' need for assisted ventilation (1 day), pediatric intensive care unit (PICU) stay (3 days), and total hospital stay (20 days) were similar to our general LT recipients-the key difference in the postoperative period was the duration of supplementary O requirement. Hb of ≥130 g/L on the day of LT correlated with a longer PICU stay (P value = 0.02), duration of supplementary O (P value = 0.005), and the need for the latter beyond 7 days after LT (P value = 0.01). Fifteen patients had resolution of their HPS after LT. The 5-, 10-, and 20-year survival rates were unchanged at 87.5%. None had a recurrence of HPS. In conclusion, HPS is a life-threatening complication of cirrhosis which usually develops insidiously. This combined with the often-stable nature of the liver disease leads to delays in diagnosis and listing for LT. Progressive polycythemia extends the need for supplementary O and PICU stay. We advocate screening for HPS with a combination of SpO and Hb monitoring to facilitate earlier recognition, timely LT, and shortened recovery periods.

Impact of hypoxemia on pediatric liver transplantation for hepatopulmonary syndrome

“…The need for MV, the lengths of ICU and hospital stays, and the time required for weaning from supplemental O 2 were significantly longer in G2 than in G1. Similar to the results of other reports, 12,20,23,30…”

“…Al‐Hussaini et al 12 and Warner et al 23 described supplemental oxygen therapy requirement in 55.5% and 69% of patients, respectively, in their series. In the present report, 47.5% of the patients required supplemental O 2 ; it should be noted that 80% of the patients with very severe hypoxemia were on O 2 therapy before transplantation.…”

“…comes from studies in the adult population. A small number of case series address the condition in children with chronic liver disease requiring LT. 4,12,13,[20][21][22][23][24][25][26][27][28][29][30] The number of patients enrolled in these studies ranged from 6 to 17, and the reports did not include comparisons of outcomes between patients with very severe hypoxemia…”

Technical Considerations in Liver Transplantation for Biliary Atresia With Situs Inversus

“…In addition to certain postoperative complications after LT, including biliary leakage, PV thrombosis, and intracranial vascular complications, BASM syndrome–associated HPS appears to be another major cause of increased mortality in this cohort . Although some reports showed that HPS can improve after LT, the pathophysiology is still unknown, and patients with severe HPS in our patient series failed to improve their pulmonary situation. The overall number of BA patients with SI and HPS appears very limited, and the presentation of conclusive studies that provide guidelines for the indications of LT and, more importantly, valid thresholds to avoid inferior outcomes are, therefore, still lacking.…”

50 Years Ago in T J P

“…The mechanisms of hepatopulmonary syndrome are likely to involve endogenous vasodilators and pulmonary vascular remodeling. 4 Fifty years ago, the severe liver disease progressed slowly despite a thorough workup and this patient eventually developed a coma and died. Hepatopulmonary syndrome often resolves after liver transplantation and the availability of transplantation is the major difference from the situation 50 years ago.…”