Shortness of breath due to portopulmonary hypertension and hepatopulmonary syndrome: diagnostic challenges and complex management approach in frail patients

Abstract

A 60-year-old woman with a background of frailty, non-alcoholic fatty liver disease (NAFLD), cirrhosis and type 2 diabetes mellitus (T2DM), presented with worsening shortness of breath and a drop in oxygen saturation on sitting and standing up. Her chest X-ray demonstrated evidence of upper lobe venous diversion. Given the hypoxia, she had a computed tomography pulmonary angiography (CTPA) to rule out a pulmonary embolism. The only finding from the CTPA was pulmonary hypertension in the absence of any clots in the lungs. An ultrasound of the abdomen confirmed portal hypertension with splenomegaly and a cirrhotic liver, therefore, an initial diagnosis of portopulmonary hypertension and hepatopulmonary syndrome was made.The patient declined an agitated saline contrast echocardiography. Based on frailty she was not deemed to be a suitable candidate for a liver transplant and was discharged with a package of care alongside home oxygen therapy with periodic review in the gastroenterology clinic. She was assessed as stable with no new concerns while on home oxygen and diuretics.This case highlights challenges in diagnosing and managing patients with cirrhosis, portopulmonary hypertension and hepatopulmonary syndrome with a background of complex comorbidities and frailty.

Keywords: cirrhosis; hepatopulmonary syndrome; non-alcoholic fatty liver disease; portopulmonary hypertension; shortness of breath.

Fig 1.

Chest X-ray showing bilateral upper lobe venous diversion (yellow arrows) along with cardiomegaly, both characteristic features of congestive cardiac failure.

Fig 2.

Computed tomography of the chest showing pulmonary outflow tract diameter of 29.6 mm (blue arrow) compared with a smaller aortic root diameter of 26 mm (red arrow), suggestive of probable pulmonary hypertension.

Fig 3.

Ultrasound of the abdomen showing coarse echotexture with irregular outline and hepatomegaly (red arrows) and splenomegaly of 130 mm (yellow arrow).

Fig 4.

2D echocardiography with pressure gradients showing normal left ventricular cavity size with globally mildly increased wall thickness. Left ventricular ejection fraction ∼55%–60%; mild to moderate aortic stenosis; mildly thickened mitral valve cusps with posterior annular calcification noted; trivial mitral regurgitation; trace of aortic regurgitation; trivial tricuspid regurgitation; estimated right ventricular systolic pressure ∼17 mmHg and right atrial pressure; pulmonary valve peak velocity 133.57 cm/second; pulmonary valve acceleration time 109 mseconds; and pulmonary valve peak gradient 7.1 mmHg.