TIPSS Procedure in the Treatment of a Single Patient After Recent Heart Transplantation Because of Refractory Ascites Due to Cardiac Cirrhosis
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- Fava, M., Meneses, L., Loyola, S. et al. Cardiovasc Intervent Radiol (2008) 31: 188. doi:10.1007/s00270-007-9251-y
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We present the case of a female patient with arrhythmogenic dysplasia of the right ventricle who evolved to refractory heart failure, ascites, and peripheral edema. As a result, heart transplantation was performed. Subsequently, refractory ascites impaired the patient’s respiratory function, resulting in prolonged mechanical ventilation. She was successfully treated with transjugular intrahepatic portosystemic shunt (TIPSS) placement, which allowed satisfactory weaning of ventilatory support.
Trials have been conducted in cirrhotic patients, with promising results. TIPSS leads to a decrease in portal pressure and decompresses the liver.
We report the efficacy of TIPSS in treatment of refractory ascites in a patient with recent heart transplantation and cardiac cirrhosis. To our knowledge, this is the first report of TIPSS placement in a heart transplant recipient.
We present a 30-year-old woman with arrhythmogenic dysplasia of the right ventricle diagnosed 10 years previously. She was admitted to our hospital with evidence of cardiogenic shock. Physical examination showed tense ascites that was thought to be secondary to heart failure. She was placed on the national waiting list for heart transplantation (status one) and, 6 days later, underwent orthotopic heart transplantation.
We recorded a central venous pressure of 15 mm Hg and a portal vein pressure of 60 mm Hg during the procedure, before the TIPSS was placed (pre-TIPSS portosystemic gradient [PSG], 45 mm Hg). The final PSG was 10 mm Hg.
A transjugular hepatic biopsy was done as well, which only showed cirrhosis.
The early maximal filling velocity/late maximal filling velocity (E/A) ratio was >1 in the echocardiograms before and after TIPSS deployment.
The patient was discharged 7 months after transplantation (5 months after TIPSS deployment) because of the need to manage her recurrent pulmonary infections (she was under aggressive immunosuppressant regimen) and her nutritional condition. These complications were not associated with TIPSS.
This patient developed severe ascites that was refractory to medical treatment —in the context of cardiac cirrhosis—even after cardiac transplantation. Ascites threatened ventilatory function and clinical outcome. Her principal manifestation of cirrhosis was ascites, without other symptoms of portal hypertension. The hepatic synthetic function was preserved. The transplanted heart had an adequate adaptation to TIPSS placement and there were no changes in cardiac function or diameters.
In this case we addressed two unreported issues: the use of TIPSS for treatment of refractory ascites secondary to cardiac cirrhosis and the response of cardiac graft to TIPSS deployment. Cardiac cirrhosis is a condition observed infrequently nowadays. It occurs in terminal cardiac function and is characterized by alterations in the hepatic stroma secondary to outflow obstruction due to cardiac failure. Thrombosis of sinusoids, venules, and suprahepatic veins is fundamental in pathogenesis [12, 13].
The clinical hallmark is ascites. The laboratory studies (liver enzymes and bilirrubin) generally are normal, as in our patient . Frequently, the presentation of cardiac cirrhosis is masked by symptoms and signs of right heart failure . In our case hepatic disease was not suspected before transplantation. When ascites is secondary to heart failure it usually resolves early after transplantation, unlike our case .
TIPSS use has been broadly described for treatment of refractory ascites secondary to viral, inflammatory, or ethylic cirrhosis [6–9]. To the best of our knowledge, its use has not been described in cardiac cirrhosis because this disease is observed in patients with severe heart failure, and TIPSS is contraindicated because it may precipitate acute right heart failure from an acute increase in pulmonary arterial pressure . However, in our patient, the transplanted heart made it possible to use TIPSS in this etiology. However, when we considered the use of TIPSS, there was concern about the adaptation of the transplanted heart to the hemodynamic changes induced by TIPSS.
TIPSS deployment creates a hyperdynamic state: there is an increase in cardiac index and a decrease in systemic vascular resistance. In addition, there is an increase in heart rate, pulmonary pressure, pulmonary capillary wedge pressure, right atrial pressure,and end diastolic and end systolic left ventricular volumes. These changes occur 30 min after the procedure [16, 17]. In our patient, pre and post-TIPSS echocardiography showed normal heart function with low pulmonary pressure. The lack of these hyperdynamic changes in our patient after TIPSS deployment could be due to denervation of the graft , however, more studies are necessary. Perhaps animal models would be useful in this regard.
On the other hand, there are new reports that recognize the importance of cardiac function for patient outcome after TIPSS deployment [19, 20]. A recent study  showed that diastolic dysfunction could be a predictor of poor survival in patients with cirrhosis and TIPSS. In this report, those patients with restrictive diastolic dysfunction (i.e., early maximal filling velocity/late filling velocity ratio [E/A] ≤1) had higher mortality than patients without dysfunction. One possible cause suggested by those authors is the reduction in portal vein liver perfusion after TIPSS deployment. It is not compensated by an increase in arterial perfusion due to diastolic dysfunction. This is an important issue considering that the presence of diastolic dysfunction is not infrequent in cardiac graft due to rejection (21).
Our patient had normal diastolic function before and after TIPSS (E/A ratio >1). It might be possible to consider the reduction of the diameter of the stent in order to increase the perfusion from portal vein to hepatic parenchyma if impairment of diastolic function is demonstrated in follow-up.
Pre-TIPSS PSG measurement can be a useful tool for clinicians considering the potential benefit of TIPSS in refractory ascites. One study reported that a higher pre-TIPSS PSG is predictive of a better response regardless of the severity of liver disease and serum creatinine level. The mean PSG in patients with a good response in that report was 20.9 mm Hg, versus 15 mm Hg in those who did not respond . In our case the pre-TIPS PSG was 45 mm Hg, so this therapy seemed to be an adequate alternative for refractory ascites. Our patient had a final PSG of 10 mm Hg. There is a delicate trade-off in post-TIPSS PSG. It has been accepted as a therapeutic goal to achieve a final PSG <12 mm Hg, however, an excess of flow throughout the stent can precipitate right heart failure or encephalopathy . In fact, a post-TIPSS PSG <8 mm Hg has been reported to increase the mortality risk factor by 3 . Therefore, the range of optimal post-TIPSS PSGs would only be between 8 and 12 mm Hg.
In summary, this case illustrates that it is possible to treat refractory ascites due to cardiac cirrhosis after heart transplantation using TIPSS.