We report a case of an 11-year-old boy with diagnosed but uncorrected tetralogy of Fallot presented to us for brain abscess drainage. The child was managed successfully with scalp block with sedation.
Pulmonary hypertension is a rare and potentially fatal disease in children if left untreated. Emerging therapies, including Bosentan, a dual endothelin receptor antagonist, have shown significant benefits in the adult...Pulmonary hypertension is a rare and potentially fatal disease in children if left untreated. Emerging therapies, including Bosentan, a dual endothelin receptor antagonist, have shown significant benefits in the adult pulmonary hypertension population;however, few studies have assessed the efficacy and safety of endothelin receptor antagonists in infants and young children. Our study was a single-center retrospective analysis of patients less than two years of age with a confirmed diagnosis of pulmonary hypertension and initiated on Bosentan therapy between 2017 and 2020. Twelve cases met eligibility criteria. Demographic data, laboratory data, echocardiographic, and cardiac catheterization data were analyzed. With treatment, there was a statistically significant decrease in mean right ventricular systolic pressure estimated by the tricuspid regurgitation jet (79 ± 23 mmHg reduced to 52 ± 25 mmHg;p < 0.001) N-terminal pro-hormone B-type natriuretic peptide levels (21,071 reduced to 2,037;p < 0.001). Additionally, improvement and eventual normalization of right ventricular function and septal geometry was seen within the first four months of therapy. Patients who underwent cardiac catheterization after therapy initiation (n = 4) demonstrated hemodynamic improvements;however, only the decrease in diastolic pulmonary artery pressure was statistically significant (p = 0.018). No significant differences in hemoglobin, platelet count, or liver function tests were observed between groups. In conclusion, these data suggest that Bosentan may be an effective and relatively safe treatment option for children less than two years of age with pulmonary hypertension. Further long-term randomized control studies are necessary to validate the potential clinical benefit of utilizing this drug therapy in young children.展开更多
目的探讨波生坦对先天性心脏病合并肺动脉高压患儿心肺功能及血浆N末端B型脑钠肽前体(NT-proBNP)水平的影响。方法选择2013年3月至2019年3月山西省儿童医院收治的先天性心脏病合并肺动脉高压患儿300例。按照随机数字表法分为对照组和观...目的探讨波生坦对先天性心脏病合并肺动脉高压患儿心肺功能及血浆N末端B型脑钠肽前体(NT-proBNP)水平的影响。方法选择2013年3月至2019年3月山西省儿童医院收治的先天性心脏病合并肺动脉高压患儿300例。按照随机数字表法分为对照组和观察组,各150例。对照组在基础干预基础上口服枸橼酸西地那非治疗,观察组在基础干预基础上口服波生坦治疗。2组均治疗1个月,观察比较2组患儿治疗前后平均肺动脉压及血浆NT-proBNP水平以及心功能和肺功能指标变化。结果治疗后2组肺动脉压及NT-proBNP均较治疗前下降,且观察组低于对照组[(30±5)mm Hg(1 mm Hg=0.133 k Pa)比(33±5)mm Hg、(126±16)ng/L比(157±18)ng/L],差异均有统计学意义(均P<0.05)。治疗后观察组6 min步行距离、右心室射血分数、第1秒用力呼气容积、用力肺活量、第1秒用力呼气容积占用力肺活量百分比均高于对照组,差异均有统计学意义(均P<0.05)。结论波生坦治疗能够降低无用药禁忌的先天性心脏病合并肺动脉高压患儿肺动脉压力及NT-proBNP水平,有助于改善患儿的心肺功能,促进心肺功能恢复。展开更多
文摘We report a case of an 11-year-old boy with diagnosed but uncorrected tetralogy of Fallot presented to us for brain abscess drainage. The child was managed successfully with scalp block with sedation.
文摘Pulmonary hypertension is a rare and potentially fatal disease in children if left untreated. Emerging therapies, including Bosentan, a dual endothelin receptor antagonist, have shown significant benefits in the adult pulmonary hypertension population;however, few studies have assessed the efficacy and safety of endothelin receptor antagonists in infants and young children. Our study was a single-center retrospective analysis of patients less than two years of age with a confirmed diagnosis of pulmonary hypertension and initiated on Bosentan therapy between 2017 and 2020. Twelve cases met eligibility criteria. Demographic data, laboratory data, echocardiographic, and cardiac catheterization data were analyzed. With treatment, there was a statistically significant decrease in mean right ventricular systolic pressure estimated by the tricuspid regurgitation jet (79 ± 23 mmHg reduced to 52 ± 25 mmHg;p < 0.001) N-terminal pro-hormone B-type natriuretic peptide levels (21,071 reduced to 2,037;p < 0.001). Additionally, improvement and eventual normalization of right ventricular function and septal geometry was seen within the first four months of therapy. Patients who underwent cardiac catheterization after therapy initiation (n = 4) demonstrated hemodynamic improvements;however, only the decrease in diastolic pulmonary artery pressure was statistically significant (p = 0.018). No significant differences in hemoglobin, platelet count, or liver function tests were observed between groups. In conclusion, these data suggest that Bosentan may be an effective and relatively safe treatment option for children less than two years of age with pulmonary hypertension. Further long-term randomized control studies are necessary to validate the potential clinical benefit of utilizing this drug therapy in young children.
文摘目的探讨波生坦对先天性心脏病合并肺动脉高压患儿心肺功能及血浆N末端B型脑钠肽前体(NT-proBNP)水平的影响。方法选择2013年3月至2019年3月山西省儿童医院收治的先天性心脏病合并肺动脉高压患儿300例。按照随机数字表法分为对照组和观察组,各150例。对照组在基础干预基础上口服枸橼酸西地那非治疗,观察组在基础干预基础上口服波生坦治疗。2组均治疗1个月,观察比较2组患儿治疗前后平均肺动脉压及血浆NT-proBNP水平以及心功能和肺功能指标变化。结果治疗后2组肺动脉压及NT-proBNP均较治疗前下降,且观察组低于对照组[(30±5)mm Hg(1 mm Hg=0.133 k Pa)比(33±5)mm Hg、(126±16)ng/L比(157±18)ng/L],差异均有统计学意义(均P<0.05)。治疗后观察组6 min步行距离、右心室射血分数、第1秒用力呼气容积、用力肺活量、第1秒用力呼气容积占用力肺活量百分比均高于对照组,差异均有统计学意义(均P<0.05)。结论波生坦治疗能够降低无用药禁忌的先天性心脏病合并肺动脉高压患儿肺动脉压力及NT-proBNP水平,有助于改善患儿的心肺功能,促进心肺功能恢复。
