1. First presentation of portal hypertension complicated by hepatopulmonary syndrome
- Author
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Ceyhun Aksel Oztumer, Mohamed Saleh, Nahima Miah, and Aidan Ryan
- Subjects
Adult ,Liver Cirrhosis ,Male ,medicine.medical_specialty ,Cirrhosis ,medicine.diagnostic_test ,business.industry ,medicine.medical_treatment ,General Medicine ,Liver transplantation ,medicine.disease ,Chronic liver disease ,Pulmonary hypertension ,Liver Transplantation ,Respiratory failure ,Internal medicine ,Liver biopsy ,Hypertension, Portal ,Cardiology ,Medicine ,Portal hypertension ,Humans ,business ,Hepatopulmonary syndrome ,Hepatopulmonary Syndrome - Abstract
Hepatopulmonary syndrome (HPS) is a serious complication of chronic liver disease, characterised by portal hypertension and arterial hypoxaemia due to intrapulmonary vascular dilatation. We report an unusual case in which a 27-year-old man had a first presentation of portal hypertension and cirrhosis complicated by HPS. This patient presented with progressive dyspnoea on exertion and deterioration in mobility, with a type 1 respiratory failure and increased oxygen demand. A bubble echocardiogram showed a possible right-to-left shunt, CT aortogram displayed evidence of portal hypertension and cirrhosis, and liver biopsy findings were consistent with alpha-1 antitrypsin deficiency. The patient’s increased oxygen demand was subsequently treated with continuous positive airway pressure before he was discharged with 8 L home oxygen. With no current established medical therapy for HPS, the patient was assessed for liver transplantation and a decision was made in favour of this.
- Published
- 2023