Korean Circ J.  2015 Jan;45(1):77-80. 10.4070/kcj.2015.45.1.77.

Aggravation of Hepatopulmonary Syndrome after Sildenafil Treatment in a Patient with Coexisting Portopulmonary Hypertension

Affiliations
  • 1Division of Cardiology, Heart, Vascular and Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. dukkyung.kim@samsung.com
  • 2Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

Abstract

Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHTN) are complications of portal hypertension and cirrhosis. Their pathophysiological mechanisms clearly differ. HPS is characterized by a defect in arterial oxygenation induced by pulmonary vascular dilatation. In contrast, PPHTN is predominantly due to excessive pulmonary vasoconstriction and vascular remodeling, but is rarely associated with hypoxia. We report a case of a patient who had both HPS and PPHTN at the time of presentation. HPS was aggravated after sildenafil administration for the treatment of PPHTN. We demonstrated increased amount of intrapulmonay shunt after sildenafil challenge by using agitated saline contrast transthoracic echocardiography.

Keyword

Hepatopulmonary syndrome; Pulmonary arterial hypertension; Sildenafil

MeSH Terms

Anoxia
Dihydroergotamine
Dilatation
Echocardiography
Fibrosis
Hepatopulmonary Syndrome*
Humans
Hypertension*
Hypertension, Portal
Oxygen
Vasoconstriction
Sildenafil Citrate
Dihydroergotamine
Oxygen

Figure

  • Fig. 1 A plain chest radiograph, cardiac magnetic resonance image (CMR) and transthoracic echocardiography (TTE). Plain chest radiograph (A) was normal and CMR (B) exhibited no evidence of pulmonary thromboembolism. TTE demonstrated mild tricuspid regurgitation (C) with an increased right ventricular systolic pressure of 53 mm Hg (D).

  • Fig. 2 Saline-contrast transthoracic echocardiogram (SC-TTE). SC-TTE showed right-to-left shunt (A). Thirty minutes after a challenge dose of 50 mg sildenafil orally, SC-TTE showed intrapulmonary shunt aggravation (B). LA: left atrium, LV: left ventricle, RA: right atrium, RV: right ventricle.

  • Fig. 3 Arterial blood gas analysis (ABGA) results before and after sildenafil challenge. ABGA was obtained just before (0 minute) and 30, 60, 90, 240 minute after sildenafil administration; PaO2 was 80.1 mm Hg, 56.1 mm Hg, 72.8 mm Hg, 61.1 mm Hg, and 71.7 mm Hg, respectively.


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