Implantable LENUS pro pump for treprostinil infusion in three pediatric patients.

BACKGROUND: Prostanoid treatment in patients with severe pulmonary arterial hypertension (PAH) has been proven safe and effective. Subcutaneous administration of treprostinil has side effects, which limits their use and acceptance. An implantable pump for continuous intravenous treprostinil infusion has been recently approved. We describe our experience with the implantable pump in three pediatric patients. DESCRIPTION OF CASES: The LENUS pro pump was implanted in three adolescents with severe PAH, who were treated with tadalafil, ambrisentan, and subcutaneous treprostinil. The indication of the Lenus pro pump implantation was the local side effects of subcutaneous treprostinil (pain, inflammation, and local infection) that were not well tolerated and that severely decreased their quality of life. The pump was surgically implanted under general anesthesia.One patient, in functional class IV, suffered postoperative hemodynamic instability and small pneumothorax, requiring an increase in treprostinil dose up to 85 ng/kg/min and a decrease 9 days after the pump implantation. The second patient who was discharged 4 days after surgery with treprostinil at 60 ng/kg/min reported improvement in his quality of life, but the dose requirement increased up to 92 ng/kg/min. After a 21-month follow-up, this patient received a lung transplant. The third patient presented a hematoma at the pump site with no other complications and had a follow-up of 9 months with an improvement in her quality of life. COMMENTS: Implantable pumps for continuous parenteral prostanoid infusion in pediatric patients are an alternative to external pumps, especially when familiar psychological or psychomotor issues hinder the use of external pumps.

Continuous terlipressin infusion as rescue treatment in a case series of children with refractory septic shock.

BACKGROUND: Despite intensive therapy, refractory pediatric septic shock has a high rate of morbidity and mortality. Additional treatments are needed to improve outcomes in such cases. OBJECTIVE: To report the clinical effects of continuous terlipressin infusion as rescue treatment for children with septic shock refractory to high catecholamine doses. METHODS: Sixteen episodes of catecholamine-resistant septic shock were recorded in 15 children (aged from newborn to 15 years) who received compassionate rescue treatment with terlipressin at 6 pediatric intensive care units. Terlipressin treatment consisted of a loading dose (20 µg/kg) followed by continuous infusion at a rate of 4-20 µg/kg/h. Terlipressin was titrated at increases of 1 µg/kg/h to maintain mean arterial pressure (MAP) in normal range for age and to reduce catecholamine dosage. The main outcome was survival of the episode. Secondary outcomes included hemodynamic effects, ischemia, and terlipressin-related adverse events. RESULTS: Terlipressin increased median MAP from 48 (range 42-63) to 68 (45-115) mm Hg 30 minutes after terlipressin administration (p < 0.01). MAP was subsequently sustained, which allowed for the reduction of norepinephrine infusion from 2 µg/kg/min (1-4) at baseline to 1.5 µg/kg/min (0.4-4) at 1 hour, 1.3 µg/kg/min (0-8) at 4 hours, 1 µg/kg/min (0-2) at 12 hours, 0.45 µg/kg/min (0-1.4) at 24 hours, and 0 µg/kg/min (0-0.6) at 48 hours (p < 0.05 vs baseline in all cases). In 8 (50%) of the 16 septic shock episodes the patients survived, 7 (44%) without sequelae. One patient survived with sequelae (minor amputation and mild cutaneous ischemia). Eight patients had signs of ischemia at admission; terlipressin induced reversible ischemia in another 4 patients. Meningococcal infection, prior ischemia, and MAP were risk factors for mortality. CONCLUSIONS: Continuous terlipressin infusion may improve hemodynamics and survival in some children with refractory septic shock. Terlipressin could contribute to tissue ischemia.

Complication of surgery for scoliosis in children with surgically corrected congenital cardiac malformations.

INTRODUCTION: There is a high incidence of scoliosis in patients who have undergone cardiothoracic surgery for correction of congenital cardiac disease, this risk being 10 times higher than in the general population. MATERIALS AND METHODS: So as to analyse the surgical and postoperative complications, we designed a retrospective study to include every child who underwent spinal orthopaedic surgery, and who had previously undergone cardiothoracic surgery because of a congenital cardiac malformation. We excluded those patients who had syndromes associated with the development of scoliosis. RESULTS: We identified 18 patients with surgically treated congenital cardiac disease who had undergone surgery for scoliosis over a period of 7 years. This group came from a total number of 87 patients undergoing spinal fusion over the same period. Of those with congenitally malformed hearts, 61% had acyanotic lesions, with ventricular septal defect being the most frequent single lesion, present in 40%. All the patients needed blood transfusions during the surgery, with aprotinin used in 73% to reduce the bleeding, and inotropes needed for 4 children. During the immediate postoperative period, 1 patient died in the first 24 hours, while 7 (39%) had different complications, pneumonia in 4, pleural effusions in 2, and rhabdomyolysis in the other, as opposed to a rate of complications of 27% in patients without heart disease. CONCLUSION: The surgical and postoperative complications in these patients depend on the specific cardiac lesion. A multidisciplinary team with experience in the treatment of congenitally malformed hearts is essential for appropriate management of these patients.