Pacientes con corazón univentricular sometidos a cirugía de Glenn / Patients with univentricular heart submitted to Glenn surgery

Resumen Objetivo: Describir las características clínicas, quirúrgicas y posquirúrgicas de pacientes univentriculares sometidos a cirugía de Glenn en un centro de referencia cardiovascular. Método: Estudio descriptivo, retrospectivo, llevado a cabo entre enero de 2012 y diciembre de 2016, en pacientes menores de 18 años que consultaron a una clínica de cuarto nivel, con cardiopatías de fisiología univentricular, definidos por ecocardiografía institucional, presentados en junta médico-quirúrgica, operados o no como primer estadio de paliación y seguidos en el programa de ventrículo único de la institución, posterior a un cateterismo cardiaco para ser presentado en junta médica que definió la realización de cirugía de Glenn. De la historia clínica se recolectaron datos de ecocardiogramas diagnósticos, cateterismos cardiacos, descripciones quirúrgicas y evoluciones. Resultados: Se analizaron 88 pacientes univentriculares, de los cuales el 63% eran hombres y el 36% eran mujeres. La anatomía del ventrículo funcional univentricular derecho estuvo presente en el 38.6% y la morfología funcional izquierda en el 61.4%. Entre las características asociadas con la mortalidad se verificó que el 1.1% tuviera insuficiencia moderada de la válvula atrioventricular y que el 3.4% tuviera insuficiencia grave de la válvula atrioventricular. El 38.6% presentó cifras de presión pulmonar elevadas, medidas por cateterismo cardiaco, y el 46% tenían resistencia vascular pulmonar aumentada. Se usó terapia vasopresora antes de la cirugía de Glenn en nueve pacientes; todos recibieron milrinona. La mortalidad posquirúrgica fue del 18%. Conclusiones: Este estudio evidencia que el diagnóstico y la intervención temprana contribuyen a reducir la morbimortalidad en los pacientes con diagnóstico de corazón univentricular, puesto que sin intervención de segundo estadio de paliación sería mortal para la mayoría de ellos. Así mismo, evidencia la importancia de la implementación de un programa integral para la atención de enfermedades cardiovasculares complejas.

Abstract Objective: To describe the clinical, surgical and post-surgical characteristics of univentricular patients undergoing Glenn’s surgery in a cardiovascular reference center. Method: Descriptive, retrospective study from January 2012 to December 2016, in patients under 18 who consulted a fourth level clinic with cardiopathies of univentricular physiology defined by institutional echocardiography, presented at the surgical medical board, operated or not as the first stage of palliation, followed in the single ventricle program of the institution, later performed a cardiac catheterization to be presented at the medical board that defined the performance of Glenn’s surgery. From the clinical history, data of diagnostic echocardiograms, cardiac catheterizations, surgical descriptions, and evolutions were collected. Results: A total of 88 univentricular patients were analyzed, of which 63% were men and 36% women. The anatomy of the right univentricular functional ventricle was present in 38.6% and 61.4% of left functional morphology. Among the characteristics associated with mortality, it was verified that 1.1% had moderate atrioventricular valve insufficiency and 3.4% had severe atrioventricular valve insufficiency in the patients who participated in the study. 38.6% had pulmonary arterial hypertension detected by cardiac catheterization and 46% had increased pulmonary vascular resistance. The use of vasopressor therapy before Glenn’s surgery was present in 9 patients and of them, the whole had milrinone. The postoperative mortality was 18%. Conclusions: This study shows that diagnosis and early intervention reduce morbidity and mortality in patients with a diagnosis of univentricular heart since without intervention of the second stage of palliation would be fatal for the vast majority of patients. It also demonstrates the importance of a comprehensive program for the care of complex cardiovascular pathologies.

Inhaled Iloprost as a First-Line Therapy for Persistent Pulmonary Hypertension of the Newborn

PURPOSE: Persistent pulmonary hypertension of the newborn (PPHN) is a potentially fatal disease. Inhaled iloprost, a stable analogue of prostacyclin, has recently been used as a therapeutic option. However, there are no clinical guidelines on the use of iloprost, specifically for neonates. This study aimed to suggest the use of inhaled iloprost as a rescue therapy for PPHN based on our experience.METHODS: The efficacy and adverse events of inhaled iloprost were evaluated prospectively in nine full-term neonates with PPHN. We monitored the following parameters: fraction of inspired oxygen (FiO₂), respiratory severity score (RSS), heart rate, and mean blood pressure.RESULTS: The inhalation dose was 1 to 2 µg/kg initially, and 4 to 8 inhalations per day were applied over 2 to 8 days, except in the case of one neonate who died 2 days after birth. Echocardiographic findings, changes in FiO₂, and RSS improved within the next 7 days in eight of the nine patients. Severe side effects on heart rate and blood pressure were not observed.CONCLUSION: Our experience suggests that inhaled iloprost can be used as a first-line treatment in newborn infants with PPHN when inhaled nitric oxide is not available. To the best of our knowledge, this report is the first prospective case series on the use of inhaled iloprost in PPHN.

Tracheal Bronchus with Persistent Pulmonary Hypertension of the Newborn: A Case Report

Tracheal bronchus is an uncommon anomaly in which an ectopic bronchus originates directly from the supracarinal trachea. It is usually an asymptomatic anatomical variant incidentally found on computed tomography or bronchoscopy. However, it can present with symptoms, such as chronic cough, wheezing, atelectasis, and recurrent pneumonia. We report a case of tracheal bronchus diagnosed in the neonatal period, in which the term baby presented with respiratory distress and persistent pulmonary hypertension of the newborn after birth, but no other congenital anomaly was found on further evaluation.

Clinical Application of Inhaled Nitric Oxide Therapy in Persistent Pulmonary Hypertension of the Newborn

Inhaled nitric oxide (iNO) is recognized as a potent and selective pulmonary vasodilator that does not decrease systemic vascular tone. The therapeutic application of iNO in human was first described in 1991. Subsequent reports showed that iNO therapy was effective to improve oxygenation in infants with persistent pulmonary hypertension of the newborn (PPHN). Owing to its selective pulmonary vasodilator effects, iNO therapy is an important treatment for term newborns with hypoxemic respiratory failure due to PPHN. The Food and Drug Administration of the United States of America first approved iNO in 1999 for use as a medical gas to treat hypoxic respiratory failure associated with clinical or echocardiographic evidence of pulmonary hypertension in term and late preterm neonates. Thereafter, iNO therapy is clinically applied to treat PPHN in term and late preterm neonates without consensus. In this review, we focused on the clinical practice of iNO therapy in PPHN. Based on published studies, we discuss iNO initiation and withdrawal methods, respiratory support devices that complement iNO therapy, and the patient and gas monitoring during iNO therapy.

Prognosis and Side Effects of Inhaled Nitric Oxide Treatment in Persistent Pulmonary Hypertension of the Newborn

Inhaled nitric oxide (iNO) is a potent and selective pulmonary vasodilator agent that improves arterial oxygenation and subsequent clinical outcomes for newborn infants with persistent pulmonary hypertension of the newborn (PPHN). Along with beneficial pharmacological properties, iNO also shows toxicological effects. Although the side effects of iNO have not been fully understood, these need to be thoroughly considered and monitored for the safe and effective clinical use of iNO. This article presents a review of the side effects of iNO and short-term and long-term clinical prognosis in newborn infants > or =34 weeks' gestation with PPHN.

Pediatric cataracts: clinical aspects, frequency of strabismus and chronological, etiological, and morphological features / Catarata pediátrica: aspectos clínicos, frequência de estrabismo e características cronológicas, etiológicas e morfológicas

Purpose: This study aimed to evaluate the frequency of strabismus and chronological, etiological, and morphological features in patients with pediatric cataracts. Methods: Medical records of pediatric patients were evaluated at the Congenital Cataract Section, Department of Ophthalmology, Federal University of São Paulo, from 2001 to 2011. Patients with congenital cataract or developmental cataract were included. The patients with traumatic cataract, cataract secondary to uveitis, radiation or drugs, aphakic or pseudophakic patients who underwent surgery in another hospital, patients with glaucoma, non-lenticular leukocorias (retinoblastoma, retinopathy of prematurity, prelenticular leukocorias), and lens subluxation were excluded from the study. The following outcomes were evaluated: frequency of chronological, etiological, and morphological features, laterality, and occurrence of associated strabismus. Results: A total of 207 patients were included. One hundred and seventeen patients (56.5%) had congenital cataract and 90 patients (43.5%) had developmental cataract. One hundred and nine patients (52.6%) had unilateral cataract. In terms of morphology, 72 children (33.8%) had zonular cataract and 66 (31.9%) had total cataract. Idiopathic cataract affected 150 patients (72.5%). There were 108 patients (52.2%) with strabismus, mainly secondary esotropia. Conclusion: Idiopathic etiology was the most frequent cause in this group of patients. Zonular cataract was the main morphological type of cataract in the study. Unilateral cataract occurred more frequently in patients with persistent fetal vasculature (PFV). Strabismus presented in 52% of the patients. The current analysis may help establish an earlier and more accurate diagnosis of pediatric cataracts. .

Objetivos: Avaliar as frequências do estrabismo e as características cronológica, etiológica e morfológica das cataratas pediátricas. Método: Estudo retrospectivo dos prontuários de crianças atendidas no Ambulatório de Catarata Congênita do Departamento de Oftalmologia da Universidade Federal de São Paulo no período entre 2001 e 2011. Foram incluídos pacientes com diagnóstico de catarata congênita ou de desenvolvimento. Foram excluídos os pacientes com catarata traumática; secundárias a uveíte, radiação ou medicamentos; pacientes operados em outro serviço; pacientes com glaucoma; leucocorias não cristalinianas (retinoblastoma, retinopatia da prematuridade, leucocorias pré-cristalinianas), e com sub-luxação do cristalino. Foram avaliadas: as frequências cronológicas, etiológicas e morfológicas das cataratas; a lateralidade e a ocorrência de estrabismo associado nestes pacientes. Resultados: Foram incluídos 207 pacientes. Cento e dezessete (56,5%) apresentavam catarata congênita e 90 (43,5%) apresentavam catarata de desenvolvimento. Cento e nove (52,6%) pacientes eram portadores de catarata unilateral. Quanto à morfologia, 72 crianças (33,8%) apresentavam catarata zonular e 66 (31,9%) apresentavam catarata total. A etiologia idiopática foi a mais frequente (72,5%) afetando 150 pacientes. Foram observados 108 pacientes (52,2%) com estrabismo associado, especialmente endotropias secundárias. Conclusões: A etiologia idiopática foi a mais frequente neste estudo. O tipo morfológico zonular foi o mais frequentemente diagnosticado. Cataratas unilaterais ocorreram mais frequentemente em pacientes com persistência da vasculatura fetal. O estrabismo associado ocorreu em 52% dos pacientes. A análise dos resultados deste estudo pode ...

Clinical study on intratracheal instillation of sodium nitroprusside for treating persistent pulmonary hypertension of newborn / 重庆医学

Objective To investigate the therapeutic effect and safety of the intratracheal instillation of sodium nitroprusside for treating persistent pulmonary hypertension of newborn (PPHN) .Methods The intratracheal instillation of sodium nitroprusside was used to 19 cases of PPHN under the mechanical ventilation ,and the pulmonary arterial mean pressure(PAMP) ,arterial mean pressure(AMP) ,transcutaneous oxygen saturation(TcSaO2 ) of the right upper and left lower limb at the basic state ,30 ,60 ,120min after intratracheal instillation of sodium nitroprusside were respectively measured and compared .Results Among 19 cases of PPHN ,17 cases had the primary lung disease .Of 17 cases ,14 cases(82 .35% ) had significant decrease of PAMP after intratracheal instillation of sodium nitroprusside ,which was most significant at 30min after therapy and the difference was statistically significant compared with before therapy [(21 .30 ± 4 .200)mm Hg vs .(30 .30 ± 4 .20)mm Hg ,P0 .05] .Conclusion The intratracheal instillation of sodium nitroprusside is the safe ,effective and economic method for treating PPHN .

Effectiveness and Safety of Intravenous Iloprost for Severe Persistent Pulmonary Hypertension of the Newborn.

Objective: The aims of this study were to determine the effectiveness (oxygenation), safety (hemodynamic status) and short term outcomes of intravenous iloprost (IVI) administration as a rescue therapy in severe persistent pulmonary hypertension of the newborn (PPHN). Design: Retrospective medical records review. Setting: Tertiary neonatal intensive care unit at Songklanagarind Hospital, Songkhla Province, Hat Yai, Thailand. Participants: Newborns who received IVI as an adjunctive therapy for treatment of severe PPHN, as defined by an oxygen index (OI) of >20 and without response to conventional therapies. Main Outcome Measures: The change of OI and alveolar-arterial oxygen difference before and after commencement of IVI. Results: 33 neonates with severe PPHN at a median gestation of 39 weeks and a baseline OI of 40 (range, 21-101) received IVI. The median OI and alveolar-arterial oxygen difference had a statistically significant decrease after 2 hours of treatment and continued to decline thereafter (P<0.05). All infants received one or more inotropic medications and volume expanders to provide blood pressure support with no statistically significant difference of blood pressure and heart rate before and after IVI treatment. The mortality rate was 15.2%, all of them had initially severe hypoxemia with a median OI of 53.6. Conclusions: IVI may be effective in improving oxygenation and should be considered as a rescue therapy for infants with severe PPHN, especially in a limited resource environment with no inhaled nitric oxide available. Systemic hypotension may be a cause for concern.

A case of persistent pulmonary hypertension of the newborn: Treatment with inhaled iloprost / 소아과

We report a case of a full-term neonate with persistent pulmonary hypertension who developed asphyxia after birth and was treated with iloprost. The neonate had persistent hypoxia and did not respond to supportive treatment. Because inhaled nitric oxide (iNO) was not available in our hospital, inhaled iloprost was administered via an endotracheal tube. This resulted in an immediate elevation of oxygen saturation. Echocardiography revealed the conversion of the right-to-left ductal shunt to the left-to-right one and a decrease of the right ventricular pressure. The use of inhaled iloprost did not cause any significant side effects. Here, we describe our experience where iloprost was used in a neonate with persistent pulmonary hypertension because the standard therapy with inhaled nitric oxide was not available.

Uso de milrinona no tratamento da hipertensão pulmonar persistente do recém-nascido / Milrinone for persistent pulmonary hypertension of the newborn treatment

OBJETIVO: Descrever uma série de casos de recém-nascidos com hipertensão pulmonar persistente grave, que receberam milrinona para promover a vasodilatação pulmonar. MÉTODOS: Análise retrospectiva de prontuários de 28 pacientes com diagnóstico de hipertensão pulmonar persistente do recém-nascido (HPPRN). Após o diagnóstico, todos os pacientes receberam uma dose de ataque de 50mcg/kg de milrinona, seguida por 0,75mcg/kg/min. O índice de oxigenação (IO) foi calculado no início da infusão e 72 horas após o início da medicação. RESULTADOS: Todos os neonatos receberam milrinona e o sildenafil foi associado em 54 por cento. O uso de dopamina assegurou a manutenção da pressão arterial em nível adequado em todos os casos. Sedação contínua, alcalinização e surfactante foram medidas coadjuvantes no tratamento. Durante a internação, sete pacientes (25 por cento) evoluíram a óbito e todos eles apresentaram aumento do IO, com elevação da média de 25 para 38 com a milrinona. Os sobreviventes, com exceção de um neonato, apresentaram redução do IO em uso de milrinona, com queda da média de 19 para 7. CONCLUSÕES: O uso da milrinona parece ser uma alternativa para o tratamento da HPPRN, na ausência do óxido nítrico. A redução do IO com a medicação foi fator determinante da boa evolução dos pacientes. O índice de falha no tratamento com a milrinona nesta casuística foi semelhante ao encontrado na literatura para o uso de óxido nítrico.

OBJECTIVE: To describe a series of neonates with severe persistent pulmonary hypertension, who received milrinone as the main treatment for pulmonary vasodilatation. METHODS: Retrospective analysis by chart review of 28 neonates with persistent pulmonary hypertension. A dose of 0.75µg/kg/min of milrinone was given, after a loading dose of 50µg/kg. The oxygenation index (OI) was calculated before and 72 hours after the medication. RESULTS: All infants received milrinone and sildenafil was associated to milrinone in 54 percent. The use of dopamine assured normal blood pressure during milrinone treatment in all patients. Continuous sedation, alcalinization and surfactant were additional measures in the treatment. During the hospitalization period, seven (25 percent) patients died and all of them presented an OI increase after milrinone (the average OI rose from 25 to 38). All but one of the 21 surviving patients presented improvement of the OI with milrinone, with a reduction of the mean index from 19 to 7. CONCLUSIONS: Milrinone can be used to treat persistent pulmonary hypertension of the newborn, in the absence of nitric oxide. The reduction of the OI during treatment was associated with clinical improvement. The failure rate for milrinone treatment in this series of cases was similar to that found in the literature regarding nitric oxide.

Treatment of Severe Meconium Aspiration Syndrome of Newborn Piglet by High Frequency Oscillatory Ventilation and Magnesium Sulphate / 中华围产医学杂志

Objective To evaluate the effect of high frequency oscillation ventilation (HFOV) and HFOV+ MgSO 4 on oxygenation, pulmonary and systemic arterial pressure, histologic alterations, and serum magnesium concentration in severe meconium aspiration syndrome(MAS) piglets with persistent pulmonary hypertension(PPH). Methods Newborn piglets were randomized to HFOV group( n =6), HFOV+MgSO 4 group( n =7) and control group( n =5). Piglets of HFOV group and HFOV+MgSO 4 group received an intratracheal a 20% suspension of human meconium. All piglets were given HFOV, Piglet of HFOV+MgSO 4 group was given MgSO 4 intravenously simultaneously. Cardiopulmonary functions, blood gases were monitored and serum magnesium concentration of piglets in HFOV+MgSO 4 group were measured by atomic absorption spectrophotometric assay. Results (1) HFOV and HFOV+ MgSO 4 treatment showed improved oxygenation in MAS model, PaO 2,a/APO 2 increased significantly and A-aDO 2?,Qs/Qt decreased significantly in HFOV group at 30 minute ( P

Effect of Vasodilators on Pulmonary Hypertension of Neonates / 中华围产医学杂志

Objective To evaluate the effect of vasodilators in different therapies on pulmonary hypertension of neonates. Methods Vasodilators were given by i.v infusion in the usual way and the air compressor pump was used as nebulizer to inhale Neb-NTG (20 ?g/kg) 2 times daily for 3 days. The changes of heart rate, breath, blood pressure and pulmonary artery mean pressure (PAMP) were monitored before and after the treatment. Results PAMP of the patients with abnormal heart structure was (31.7?6.4) mm Hg(1 mm Hg=0.133 kPa) while PAMP of the patients with oxygen deficiency was (26.9?4.2) mm Hg. There was a significant difference in PAMP between the two groups ( t=2.14,P 0.05 ). Conclusion Pulmonary artery pressure can be decreased by inhaling Neb-NTG and its therapeutic efficacy maybe equales that of vasodilators IV infusion. The nebulized inhalation of Neb-NTC may be an easy and effective therapy for the treatment of pulmonary hypertension in neonates.