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Long-Term Treatment of Pulmonary Arterial Hypertension with Treprostinil in Patient with Eisenmenger and Down Syndromes
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A7049 - Long-Term Treatment of Pulmonary Arterial Hypertension with Treprostinil in Patient with Eisenmenger and Down Syndromes
Author Block: Z. Gasior, A. Sikora-Puz, K. Banska-Kisiel; Department of Cardiology, Medical University of Silesia, Katowice, Poland.
Treprostinil, a prostacyclin analogue, is a drug that is widely used to treat pulmonary arterial hypertension (PAH). We present the long-term treatment effects in 22-year woman with Down Syndrome and congenital heart defect with PAH.
22-year old woman with diagnosed Down Syndrome and large ventricular septal defect has been addmited to the department of cardiology for treatment of the PAH. In her early childhood, when she was 3 years old, Eisenmenger Syndrome developed.
Five years ago during the hospitalization in department of cardiology she was in WHO class III, with central cyanosis, hematocrit 60%, Sat 02 in capillary blood 75% and NT-proBNP 82 pg/mL. In 6 minute walk test (6MWT) she got the distance 110 meters. Patient has been qualified for treatment with bosentan in dosis 2 x 125 mg. After six months her functional class improved to WHO II and the distance in 6WMT increased to 225 m. Two years later her status deteriorated (WHO class III) and we decided to turn on treatment with subcutaneous infusion of treprostinil using an insulin pump. We were afraid of the patient tolerance because of her moderate intellectual disability. However, very good connection with the patient’s mother made possible to continue the treatment, with gradual escalation of the drug dosis up to 42 ng/kg/min. Unfortunately, 6 months later local pain and inflammation at the infusion site appeared and patient has been referred for intravenous treprostinil administration using an implantable Lenus Pro pump. The drug administration rate has not been changed and the drug reservoir has been refilled every 28 days. The distance in 6MWT gradually increased and is now 380 m. During the last year we were forced to shorten the time between the following refilling from 28 to 24 days. It means that the dosis of the drug has been increased by 17% up to the 49 ng/kg.min.
In conclusion, very good connection between the doctor and mother of the patient with intellectual disabilities allows the effective long-term treatment of PAH using different types of drug administration. An unexpected increase in the drug administration rate during intravenous
pump infusion may be observed, and should be taken into account following the therapeutic proces.
Author Block: Z. Gasior, A. Sikora-Puz, K. Banska-Kisiel; Department of Cardiology, Medical University of Silesia, Katowice, Poland.
Treprostinil, a prostacyclin analogue, is a drug that is widely used to treat pulmonary arterial hypertension (PAH). We present the long-term treatment effects in 22-year woman with Down Syndrome and congenital heart defect with PAH.
22-year old woman with diagnosed Down Syndrome and large ventricular septal defect has been addmited to the department of cardiology for treatment of the PAH. In her early childhood, when she was 3 years old, Eisenmenger Syndrome developed.
Five years ago during the hospitalization in department of cardiology she was in WHO class III, with central cyanosis, hematocrit 60%, Sat 02 in capillary blood 75% and NT-proBNP 82 pg/mL. In 6 minute walk test (6MWT) she got the distance 110 meters. Patient has been qualified for treatment with bosentan in dosis 2 x 125 mg. After six months her functional class improved to WHO II and the distance in 6WMT increased to 225 m. Two years later her status deteriorated (WHO class III) and we decided to turn on treatment with subcutaneous infusion of treprostinil using an insulin pump. We were afraid of the patient tolerance because of her moderate intellectual disability. However, very good connection with the patient’s mother made possible to continue the treatment, with gradual escalation of the drug dosis up to 42 ng/kg/min. Unfortunately, 6 months later local pain and inflammation at the infusion site appeared and patient has been referred for intravenous treprostinil administration using an implantable Lenus Pro pump. The drug administration rate has not been changed and the drug reservoir has been refilled every 28 days. The distance in 6MWT gradually increased and is now 380 m. During the last year we were forced to shorten the time between the following refilling from 28 to 24 days. It means that the dosis of the drug has been increased by 17% up to the 49 ng/kg.min.
In conclusion, very good connection between the doctor and mother of the patient with intellectual disabilities allows the effective long-term treatment of PAH using different types of drug administration. An unexpected increase in the drug administration rate during intravenous
pump infusion may be observed, and should be taken into account following the therapeutic proces.
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