Pulmonary Hypertension Following HPS

An 18-year-old girl with a complex medical history had a large spleen (350 g) removed at age 5 and a liver transplant at age 12 (native liver showed evidence of noncirrhotic portal hypertension). Hepatopulmonary syndrome (dyspnea; PaO2 of 67 mmHg; positive CEE; clubbing; normal chest radiograph, HRCT, and pulmonary angiogram) treated with oxygen was diagnosed at age 14, followed 4 years later by an acute terminal course of chest pain, dyspnea, right upper quadrant pain, and hypoxia. She was given a diagnosis of asthma elsewhere and entered the hospital where she died 6 hours later. At autopsy, the heart showed right ventricular hypertrophy, and the large intrapulmonary arteries showed atheroma, whereas there was none in the aorta. Histologic sections showed the lesion seen below.

plexembolusdil1dil2

Lesion Found at Autopsy 4 Years after Diagnosis of HPS

At a branch point of a pulmonary artery (upper center), a plexus of thin-walled vessels replaces the vessel wall. The plexus, in turn, is surrounded by dilated vessels.

A bone marrow embolus, indicating terminal resuscitation, lies in the parent artery.

Find the plexus of thin-walled vessels.

Find 2 dilated vessels (dilation lesions).

Find the bone marrow embolus.

What is the diagnosis?

Answer

 

The occurrence of pulmonary hypertension following HPS has been documented previously [1,2]. Mal, et al. describe a 46-year-old man with HPS and alcoholic cirrhosis, who had an improvement in his dyspnea and hypoxemia after a period of more than two years. The 99mTcMAA and CEE had become normal, but he had developed pulmonary hypertension (93/38 mmHg vs the previous 28/20) [1]. Another patient, a 53-year-old man with alcoholic cirrhosis, had a diagnosis of HPS made 6 months before liver transplantation. At transplant, the pulmonary artery pressure was noted to be 45/26 mmHg with a pulmonary vascular resistance of 212. At a second transplant three months later because of poor liver function, the patient died when the pulmonary artery pressure and the pulmonary vascular resistance rose to 62/35 and 914, respectively, during reperfusion. Right ventricular hypertrophy and plexiform lesions were found at autopsy [2].

These cases represent a rare complication of HPS. The cause of the transformation to hypertension is obscure.

References: To return to reference section after viewing abstract, click here before clicking on "abstract".

1. Mal H, Brugiere O, Durand F, Fartoukh M, Cohen-Solal A, Fournier M. Pulmonary hypertension following hepatopulmonary syndrome in a patient with cirrhosis. J Hepatol 1999; 31:360-364. Abstract

2. Martinez-Palli G, Barbera J, Taura P, Cirera I, Visa J, Rodriguez-Roisin R. Severe portopulmonary hypertension after liver transplantation in a patient with preexisting hepatopulmonary syndrome. J Hepathol 1999; 31:1075-1079. Abstract

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Plexus of thin-walled vessels

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Dilation lesion

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Dilation lesion

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Bone marrow embolus, indicating resuscitation

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Plexogenic angiopathy indicating pulmonary hypertension, presumed portopulmonary hypertension in this case

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