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Aggravation of hepatopulmonary syndrome after sildenafil treatment in a patient with coexisting portopulmonary hypertension.

Chung S, Lee K, Chang SA, Kim DK - Korean Circ J (2015)

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Division of Cardiology, Heart, Vascular and Stroke Institute, 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.

No MeSH data available.


Related in: MedlinePlus

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).
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Figure 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).

Mentions: A 58-year-old man with increasing shortness of breath on exertion was referred to our hospital. He had a 13-year history of hepatitis B virus-induced cirrhosis. The patient had suffered from esophageal variceal bleeding in 1999, and was treated by endoscopic variceal ligation. Progressive exertional dyspnea had developed over the past 5 years. On admittance to another hospital a plain chest radiograph (Fig. 1A) was normal and cardiac magnetic resonance image (CMR) (Fig. 1B) exhibited no evidence of pulmonary thromboembolism or intra- and extra-cardiac shunt disease. A right-heart catheterization showed a mean pulmonary artery pressure (PAP) of 35 mm Hg, capillary wedge pressure of 8 mm Hg, cardiac index of 3.63 L/min/m2 and pulmonary vascular resistance (PVR) of 313 dyne · sec/cm5. The patient was diagnosed as PPHTN and was started on diuretics and sildenafil 25 mg thrice daily. However dyspnea worsened despite treatment. The patient stopped taking sildenafil at his own discretion. Ten days later he was referred to our center. At admission, the patient was in World Health Organization (WHO) functional class (FC) III. His respiratory rate was 20/min, pulse rate 41/min, and blood pressure 127/49 mm Hg. Physical examination revealed spider angiomata, palmar erythema, shifting dullness, positive hepatojugular reflux and bilateral pretibial pitting edema. Heart and lung sounds were normal. Serum total bilirubin was elevated (3.3 mg/dL) and serum albumin level was decreased at 3.3 g/dL. Prothrombin time was 16.9 seconds (international normalized ratio 1.37). Liver cirrhosis was staged as Child-Pugh class B. N-terminal pro-B-type natriuretic peptide was 535.3 pg/mL. Arterial blood gas analysis (ABGA) obtained at room air in supine position revealed a resting PaO2 of 84.4 mm Hg and an alveolar-arterial (A-a) oxygen gradient of 22.9 mm Hg. Initial transthoracic echocardiography (TTE) demonstrated normal left-ventricular and right-ventricular function and mild tricuspid regurgitation (Fig. 1C), with an increased right ventricular systolic pressure of 53 mm Hg (Fig. 1D). Albumin macroaggregate lung perfusion scan showed normal perfusion without significant intrapulmonary shunt (IPS). A saline-contrast TTE (SC-TTE) showed delayed appearance of bubbles in the left atrium following five heart cycles, suggestive of IPS (Fig. 2A). Thirty minutes after a challenge dose of 50 mg sildenafil orally, SC-TTE showed IPS aggravation (Fig. 2B) with a PaO2 drop to 56.1 mm Hg. Four hours after the sildenafil challenge, SC-TTE showed IPS improvement with a PaO2 rise to 71.1 mm Hg (Fig. 3). On the next day, the symptoms and PaO2 did not improve after a challenge dose of 5 mcg iloprost inhalation (PaO2 values were obtained from two ABGAs, performed just before and 15 minutes after the start of iloprost inhalation: 89.8 mm Hg and 86.3 mm Hg, respectively). After administration of furosemide and permanent discontinuation of sildenafil, the patient had significant clinical improvement. The patient remained in WHO FC II without further hospitalization at the 7 months follow-up. Living donor liver transplantation was eventually performed due to advanced liver cirrhosis.


Aggravation of hepatopulmonary syndrome after sildenafil treatment in a patient with coexisting portopulmonary hypertension.

Chung S, Lee K, Chang SA, Kim DK - Korean Circ J (2015)

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).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4310984&req=5

Figure 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).
Mentions: A 58-year-old man with increasing shortness of breath on exertion was referred to our hospital. He had a 13-year history of hepatitis B virus-induced cirrhosis. The patient had suffered from esophageal variceal bleeding in 1999, and was treated by endoscopic variceal ligation. Progressive exertional dyspnea had developed over the past 5 years. On admittance to another hospital a plain chest radiograph (Fig. 1A) was normal and cardiac magnetic resonance image (CMR) (Fig. 1B) exhibited no evidence of pulmonary thromboembolism or intra- and extra-cardiac shunt disease. A right-heart catheterization showed a mean pulmonary artery pressure (PAP) of 35 mm Hg, capillary wedge pressure of 8 mm Hg, cardiac index of 3.63 L/min/m2 and pulmonary vascular resistance (PVR) of 313 dyne · sec/cm5. The patient was diagnosed as PPHTN and was started on diuretics and sildenafil 25 mg thrice daily. However dyspnea worsened despite treatment. The patient stopped taking sildenafil at his own discretion. Ten days later he was referred to our center. At admission, the patient was in World Health Organization (WHO) functional class (FC) III. His respiratory rate was 20/min, pulse rate 41/min, and blood pressure 127/49 mm Hg. Physical examination revealed spider angiomata, palmar erythema, shifting dullness, positive hepatojugular reflux and bilateral pretibial pitting edema. Heart and lung sounds were normal. Serum total bilirubin was elevated (3.3 mg/dL) and serum albumin level was decreased at 3.3 g/dL. Prothrombin time was 16.9 seconds (international normalized ratio 1.37). Liver cirrhosis was staged as Child-Pugh class B. N-terminal pro-B-type natriuretic peptide was 535.3 pg/mL. Arterial blood gas analysis (ABGA) obtained at room air in supine position revealed a resting PaO2 of 84.4 mm Hg and an alveolar-arterial (A-a) oxygen gradient of 22.9 mm Hg. Initial transthoracic echocardiography (TTE) demonstrated normal left-ventricular and right-ventricular function and mild tricuspid regurgitation (Fig. 1C), with an increased right ventricular systolic pressure of 53 mm Hg (Fig. 1D). Albumin macroaggregate lung perfusion scan showed normal perfusion without significant intrapulmonary shunt (IPS). A saline-contrast TTE (SC-TTE) showed delayed appearance of bubbles in the left atrium following five heart cycles, suggestive of IPS (Fig. 2A). Thirty minutes after a challenge dose of 50 mg sildenafil orally, SC-TTE showed IPS aggravation (Fig. 2B) with a PaO2 drop to 56.1 mm Hg. Four hours after the sildenafil challenge, SC-TTE showed IPS improvement with a PaO2 rise to 71.1 mm Hg (Fig. 3). On the next day, the symptoms and PaO2 did not improve after a challenge dose of 5 mcg iloprost inhalation (PaO2 values were obtained from two ABGAs, performed just before and 15 minutes after the start of iloprost inhalation: 89.8 mm Hg and 86.3 mm Hg, respectively). After administration of furosemide and permanent discontinuation of sildenafil, the patient had significant clinical improvement. The patient remained in WHO FC II without further hospitalization at the 7 months follow-up. Living donor liver transplantation was eventually performed due to advanced liver cirrhosis.

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Division of Cardiology, Heart, Vascular and Stroke Institute, 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.

No MeSH data available.


Related in: MedlinePlus