PurposeAnalgesia and sedation protocols are reported to reduce the requirement of sedative and analgesic agents, duration of mechanical ventilation, and length of pediatric intensive care unit (PICU) stay. However, these studies often were conducted based on inhomogeneous cohorts. The aim of this study was the evaluation of a nurse‐driven analgesia and sedation protocol in a homogenous population of infants following corrective surgery for tetralogy of Fallot (TOF).Design and MethodsThis retrospective analysis was conducted in a cardiac PICU of a tertiary referral center. Two cohorts of patients who underwent corrective surgery for TOF below the age of 7 months, were retrospectively evaluated before and after implementation of a nurse‐driven analgesia and sedation protocol. We compared peak and cumulative doses of midazolam, morphine, and clonidine, length of PICU stay and time on mechanical ventilation.ResultsA total of 33 patients were included in the preimplementation period and 32 during the postimplementation period. Implementation of the nurse‐driven analgesia and sedation protocol had no effect on time on mechanical ventilation (72 hr [24–141] vs. 49 hr [24–98]), but significantly on length of PICU stay (7 days [5–14] vs. 5 days [4–7]). Cumulative doses of midazolam (7.37 mg/kg [4.70–17.65] vs. 5.0 mg/kg [2.70–9.12]) as well as peak doses of midazolam (0.22 mg·kg−1·hr−1 [0.20–0.33] vs. 0.15 mg·kg−1·hr−1 [0.13–0.20]) and morphine (50.0 µg·kg−1·hr−1 [39.7–79.9] vs. 42.5 µg·kg−1·hr−1 [29.7–51.8]) were significantly reduced. The postimplemantation group showed no increase in postoperative complications and adverse events.Practice ImplicationsThe implementation of a nurse‐driven analgesia and sedation protocol is safe in infants following corrective surgery for TOF. It reduces significantly the length of PICU stay, cumulative and peak doses of midazolam and peak doses of morphine.
One of the major obstacles preventing successful percutaneous pulmonary valve implantation (PPVI) is related to the close proximity of coronary artery branches to the expected landing zone. The aim of this study was to assess the frequency of coronary artery anomalies (CAAs) especially those associated with major coronary branches crossing the right ventricular outflow tract (RVOT) and to describe their relevance for the feasibility of percutaneous pulmonary valve implantation (PPVI). In our retrospective single-center study 90 patients were evaluated who underwent invasive testing for PPVI in our institution from 1/2010 to 1/2020. CAAs were identified in seven patients (8%) associated with major branches crossing the RVOT due to origin of the left anterior descending (LAD) or a single coronary artery from the right aortic sinus. In 5/7 patients with CAAs balloon testing of the RVOT and selective coronary angiographies revealed a sufficiently large landing zone distal to the coronary artery branch. While unfavorable RVOT dimensions prevented PPVI in one, PPVI was performed successfully in the remaining four patients. The relatively short landing zone required application of the “folded” melody technique in two patients. All patients are doing well (mean follow-up 3 years). CAAs associated with major coronary branches crossing the RVOT can be expected in about 8% of patients who are potential candidates for PPVI. Since the LAD crossed the RVOT below the plane of the pulmonary valve successful distal implantation of the valve was possible in 4/7 patients. Therefore these coronary anomalies should not be considered as primary contraindications for PPVI.
Background
Congenital portosystemic shunts (CPSS) are rare vascular malformations and can be classified into extrahepatic and intrahepatic shunts. Extrahepatic CPSS, also termed Abernethy malformations are associated with severe long-term complications including portopulmonary hypertension, liver atrophy, hyperammoniemia and hepatic encephalopathy. We report a hitherto undescribed variant of Abernethy malformation requiring an innovative approach for interventional treatment.
Case presentation
We describe a 31-year-old patient following surgical repair of atrioventricular septal defect at the age of 6 years. In the long-term follow-up he showed persistent pulmonary hypertension which deteriorated despite dual pulmonary vasodilative treatment. When he developed arterial desaturation and symptomatic hyperammoniemia detailed reassessment revealed as underlying cause a hitherto undescribed variant of Abernethy malformation connecting the portal vein with the right lower pulmonary vein. Following interdisciplinary discussions we opted for an interventional approach. Since the malformation was un-accessible to interventional closure via antegrade venous or retrograde arterial access, a transhepatic percutaneous puncture of the portal vein was performed. Temporary balloon occlusion of the malformation revealed only a slight increase in portal venous pressure. Interventional occlusion of the large vascular connection was achieved via this transhepatic approach by successive implantation of two large vascular occluding devices. The postinterventional course was unremarkable and both ammonia levels and arterial saturation normalized at follow-up of 12 months.
Conclusions
Portal vein anomalies should be included in the differential diagnoses of pulmonary hypertension or pulmonary arterio-venous malformations. Based on careful assessment of the anatomy and testing of portal vein hemodynamics interventional therapy of complex Abernethy malformations can be performed successfully in specialized centers.
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