Presented is a retrospective outcome study of a 15-year single institutional experience with a contemporary cohort of patients with hypoplastic left heart syndrome and complex that underwent a “Giessen Hybrid” stage I as initial palliation. Hybrid approach consisting of surgical bilateral pulmonary artery banding and percutaneous duct stenting with or without atrial septum manipulation was developed from a rescue approach to a first-line procedure. Comprehensive Aristotle score defined pre-operative condition. Fifteen-year follow-up mortality is reported as occurring within the staged univentricular palliation or before and after biventricular repair. Hybrid stage I was performed in 154 patients; 107 should be treated by single ventricle palliation, 33 by biventricular repair (BVR), 7 received heart transplantation, and 7 were treated by comfort care, respectively. Overall 34 children died. The Aristotle score (mean value 18.2 ± 3) classified for univentricular circulations in newborns did not have statistical impact on the outcome. Two patients died during stage I (1.2 %), and the interstage I mortality was 6.7 %, and stage II mortality 9 %, respectively. Stage III was up to now performed in 57 patients without mortality. At 1 year, the overall unadjusted survival of HLHS and variants was 84 % and following BVR 89 %, respectively. The Fifteen-year survival rate for HLHS and variants was 77 %, with no significant impact of birth weight of less than 2.5 kg. In conclusion, Hybrid stage I fulfilled the criteria of life-saving approach. In our institution, Hybrid procedure replaced Norwood-staged palliation with a considerable mid- and long-term survival rate. Considering interstage mortality close surveillance is mandatory.
Background Newborns with hypoplastic left heart (HLH) are usually palliated with the Norwood procedure or a hybrid stage I procedure. Hybrid is our preferred approach. Given the critical relationship between stage I, interstage, and comprehensive stage II or advanced biventricular repair, we hypothesized that appropriate drug treatment is a significant therapeutic cornerstone, especially for the management of the high-risk interstage. Methods We report a single-center observational study addressing the cardiovascular effects of, in particular, oral β-blockers and the additional use of angiotensin-converting enzyme (ACE) and mineralocorticoid inhibitors. Results In total, 51 newborns-30 with HLH syndrome (HLHS) and 21 with HLH complex (HLHC)-with a median bodyweight of 3.0 kg (range 1.9-4.4; nine with bodyweight ≤ 2500 g) underwent an uneventful "Giessen hybrid approach" using a newly approved duct stent. All patients were discharged home with a single, double or triple therapy consisting of ß-blockers, ACE and mineralocorticoid inhibitors; 90% of the patients received bisoprolol, 10% received propranolol, 72% received lisinopril, and 78% received spironolactone. Resting heart rate decreased from 138 bpm (range 112-172; n = 51) at admission to 123 bpm (range 99-139; n = 51) at discharge and 110 bpm before stage II/biventricular repair/ heart transplantation (range 90-140; n = 37) accompanied by favorable bodyweight gain. No side effects were evident. Conclusion In view of drug risk/benefit profiles, as well as the variable morphology and hemodynamics, the highly selective β1-adrenoceptor blocker bisoprolol is our preferred drug for treatment of HLHS/HLHC in the interstage. We avoid using ACE inhibitor monotherapy and exclude potential risks for coronary and cerebral perfusion pressure beforehand.
Objective: Central pulmonary banding has been proposed as a novel alternative for the treatment of left ventricular dilated cardiomyopathy in children. We sought to investigate the effects of central pulmonary banding in an experimental model of doxorubicin-induced left ventricular dilated cardiomyopathy.Methods: Four-month-old sheep (n ¼ 28) were treated with intermittent intracoronary injections of doxorubicin (0.75 mg/kg/dose) into the left main coronary artery. A total dose of up to 2.15 mg/kg of doxorubicin was administered until signs of left ventricular dilation with functional impairment occurred by transthoracic echocardiography evaluation. Animals that survived were treated with surgical central pulmonary banding through a left anterior thoracotomy or sham surgery. Transthoracic echocardiography and pressure-volume loop measurements were used to compare left ventricular function preoperatively and 3 months later. Macroscopic and microscopic histologic examinations followed after hearts were harvested.
Summary Four horses were exercised for 2 h on a treadmill at up to 200 m/min 4 h after feeding. The trial included 3 treatments, control (A) and electrolyte supplementation provided either in the diet at the morning feeding (B) or 1 h before start of exercise (C). The horses were fed 2 meals/day of hay and concentrate (6.5 g dry matter, 13.8 mg Na, 66.6 mg K and 54.2 mg Cl/kg bwt per meal). The supplement was a mixture of grass meal (47.6%), sugar beet syrup (33.3%) and NaCl (19.1%); additional intake: Na 79.7, K 15.5 and Cl 165.6 mg/kg bwt. Hourly urine and blood samples were taken. Water intake and renal water excretion were significantly influenced by supplementary feeding (intake: A 35.5, B 49.9, C 55.6 ml/kg bwt/12 h; excretion: A 9.47, B 15.34, C 7.92 ml/kg bwt/12 h). The urinary Na and Cl excretion was significantly higher in treatment B (Na 24, Cl 55 mg/kg bwt/12 h) and C (Na 12, Cl 44 mg/kg bwt/12 h) as compared to A (Na 5, Cl 16.5 mg/kg bwt/12 h). Renal K output was less influenced by the different treatments, A 43, B and C 61 mg/kg bwt/12 h. However, the renal Na and Cl excretion was lower in relation to intake when horses received the supplement. In these cases, losses of water, Na and Cl via sweat were compensated for at the end of exercise and a positive balance therefore maintained 12 h after feeding. The total plasma protein concentration increased during exercise, indicating a reduction in plasma volume. The Na and K concentrations in plasma during exercise showed minor changes whereas the Cl concentrations decreased significantly by 2.6 (A), 4.0 (B) and 1.4 (C) mmol/l. Changes in plasma Na and Cl in treatments B and C occurred at constant higher levels than in treatment A. The results suggest a positive impact of NaCl suplementation on water and electrolyte metabolism in exercised horses. The increased water intake indicates that supplementary salt intake is more effective about 4 h than 1 h before exercise.
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