Critical Hemodynamic Deterioration in an Infant with the Shone Complex and Biliary Atresia Resolving after Liver Transplantation

Objectives: The Shone complex is characterized by serial obstructions of left heart structures. The initial presentation in infancy may be mild with slow progression of the different lesions. We report an infant with the Shone complex including mild mitral valve (MV) stenosis, borderline hypoplasia of the left ventricle, bicuspid aortic valve, and moderately hypoplastic aortic arch (AA). The girl also had heterotaxy syndrome with azygos continuation of the IVC. At the age of 4 months, biliary atresia with liver cirrhosis was diagnosed. So far, the girl was hemodynamically stable and asymptomatic. Together with worsening hepatic function she experienced dramatic hemodynamic deterioration endangering the option of liver transplantation.

Methods: At the age of 5 months, she was admitted with rapidly progressive hepatic failure. Echocardiography revealed an increase of the mean MV Doppler gradient from 3 to 10 mm Hg and an increase in flow velocity in the AA from 3.0 to 3.5 m/s. The patient retained fluid and developed congestive heart failure requiring mechanical ventilation at the age of 152 days. Cardiac cath revealed diastolic dysfunction of the LV (end-diastolic pressure 15 mm Hg) and postcapillary pulmonary hypertension. In the distal AA, we found a gradient of 30 mm Hg. Since balloon dilatation was unsuccessful, we implanted a premounted 8-mm stent reducing the gradient to 12 mm Hg. After improvement of cardiac function reevaluation of the patient revealed that she was able now to tolerate rapid changes of volume and catecholamines.

Result: We decided to perform living liver donation. LT was performed at the age of 162 days (weight, 6 kg) under TEE surveillance of cardiac function particularly during cross clamping and following testing if cross clamping of the hepatic vessels was tolerated without significant hemodynamic compromise. The intra- and postoperative courses were without major complications. Inotropic and vasoactive support could be quickly reduced. Cardiac function showed rapid improvement. ICU stay post-LT was 9 days; discharge from hospital was 26 days after LT.

Conclusion: Hepatic failure due to biliary atresia may result in severe hemodynamic deterioration of a mild congenital left heart lesion due to development of a hyperdynamic state. Although LT under these circumstances is associated with an increased risk, all efforts should be made to proceed, since hemodynamic improvement can be expected following normalization of hepatic function.

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Artikel online veröffentlicht:
21. Februar 2021