Fetal cardiac biometry and function in HIV-infected pregnant women exposed to HAART therapy.

OBJECTIVE: Previous studies have suggested that antiretroviral therapy in human immunodeficiency virus (HIV)-infected pregnant women can cause alterations in cardiac structure and function in fetuses or children, although the results are not very clear. The aim of this study is to assess whether or not these alterations are present in the heart of fetuses of HIV-infected pregnant women, undergoing treatment with highly active antiretroviral therapy (HAART). STUDY DESIGN: We performed a prospective study on 29 consecutive HIV-positive pregnant women treated with HAART and 70 consecutive HIV-negative pregnant women as controls. Fetal cardiac biometry and function was evaluated by echocardiography at 30-32 weeks of gestation. Fetal biometry, estimated fetal weight and umbilical artery Doppler were also measured. RESULTS: We found E/A tricuspid ratio values slightly increased (0.84 vs 0.80, p = 0.03) and diastolic length shortened (49.7 vs 51.7%, p = 0.03) in the HIV-infected group. The rest of the biometric and functional cardiac parameters were not different between both groups. There were no cases of vertical transmission of HIV infection. CONCLUSION: In HIV-infected pregnant women treated with HAART, no significant changes are showed in fetal cardiac parameters.

Alveolar capillary dysplasia with misalignment of the pulmonary veins associated with aortic coarctation and intestinal malrotation.

Alveolar capillary dysplasia with misalignment of the pulmonary veins (ACD/MPV) is a rare and lethal cause of refractory pulmonary hypertension of the newborn. We describe the clinical course of a neonate with refractory pulmonary hypertension diagnosed with ACD/MPV, aortic coarctation and other not previously reported associated malformations.

Safety and tolerability of targeted therapies for pulmonary hypertension in children.

The objective of this study is to evaluate the safety and tolerability of the pharmacological treatment of pulmonary hypertension in pediatric patients. It is a retrospective, longitudinal, observational study on pediatric patients undergoing treatment with pulmonary targeted therapies. 63 patients were included (51% male), with a median age of 3.4 years (IQR, 3.6 months-10 years) and a median weight 13 kg (IQR, 6-30 kg). Congenital heart disease was the etiology of pulmonary hypertension in the majority of cases (n = 33) and 28 patients were in NYHA functional class III-IV. The most commonly used drug was sildenafil (n = 79, 56%), followed by bosentan (n = 27, 23%), and a combination of both (n = 14, 41%). 34 patients had adverse reactions (54%) with an incidence rate of 1.02 per patient per year. The most commonly reported reactions were gastrointestinal symptoms (22%) and spontaneous erections (22%) in males. Nine severe adverse reactions (10%) occurred, requiring eight treatment withdrawal and one hospital admission. Treatment with targeted therapies for pulmonary hypertension is safe in the pediatric population. Severe ADRs were uncommon both in monotherapy and in combination therapy. Combination therapy was associated with a higher rate of ADRs. We observed similar survival rates in children receiving sildenafil doses according to the European Medicines Agency (EMA) recommendations or higher.

Cirugía de arco aortica en neonatos y lactantes con perfusión cerebral selectiva: resultados a corto y medio plazo (AU) / Aortic arch surgery with selective cerebral perfusion in neonates and infants. Short and middle term results

Introducción: La cirugía de arco aórtico en neonatos y lactantes históricamente se realizaba con circulación extracorpórea, hipotermia profunda y parada circulatoria total, que como complicación pueden causar daño cerebral. Las técnicas de perfusión cerebral selectiva (PSC) permiten reducir o eliminar la parada, protegiendo al cerebro que recibe en todo momento sangre oxigenada. Presentamos nuestra experiencia con estas operaciones. Métodos: Estudio retrospectivo de 40 pacientes con reconstrucción quirúrgica de la arco aórtico desde 2002 hasta 2009, empleando técnicas de PSC. Análisis de distintas variables con especial interés en la cirugía realizada y en los resultados a corto-medio plazo. Estudio estadístico con SPSS versión 15.0. Resultados: Edad: 1,3 ± 2,4 meses; peso 3,4 ± 1,7 kg; 55% varones. Cardiopatía asociada en un 90%. Morfología del arco aórtico: hipoplásico 62%; interrumpido 30%; recoartación postquirúrgica en 2 niños. El 82% necesitaron prostaglandinas intravenosas. La anastomosis aórtica fue terminolateral (avance aórtico) en el 77,5%, termino terminal en el 17,5% y aortoplastia con parche en el 5%. La PSC duró 36 ± 11 minutos a 23º C rectales, canulando el tronco braquiocefálico en el 47%, reduciendo los flujos a 30-40 ml/kg/min. La monitorización cerebral se hizo con presión radial derecha y saturación cerebral (INVOS). La mortalidad hospitalaria fue del 10% y la tardía del 2,7%. Durante la estancia hospitalaria concurrieron: lesión del recurrente 38%, reintervención por recoartación precoz 5%, e infarto cerebral 2,8%. El seguimiento medio es de 26 ± 21 meses. En la evolución posterior se recortaron 3 pacientes (8%) precisando 2 de ellos angioplastia percutánea. Conclusiones: La PSC en la reconstrucción del arco aórtico permite al cirujano gran precisión, minimizando el riesgo de injuria cerebral. La mortalidad hospitalaria es aceptable y en cuanto a la morbilidad destaca la lesión del nervio recurrente asociado a la movilización aórtica y a la resección ductal. La evolución posterior es buen estando el 92% de los pacientes libres de recoartación (AU)

Background: Infantil and neonatal aortic arch surgery historically has been performed with deep hypothermia and cirucularoty arrest (DHCA). Neurodevelopment impairment could be a side effect of DHCA. Antegrade cerebral perfusion (ACP) techniques let us reduce or even not use DHCA, so protecting the brain form hypoxic ischemic injury. We revise our experience with these operations. Methods: Retrospective study of 40 patients with aortic arch surgery from 2002 until 2009, using ACP. We analyze operative techniques, surgical results, morbid-morbidity and follow-up. Statistical study with SPSS 15.0. Results: Age: 1,3 ± 2,4 months; weigh: 3,4 ± 1,7 kg; 55% males. Cardipathy associated in 90%. The aortic arch was hypoplastic in 62&, interrupted in 30%, and was restenosed post surgery in 2 patients. Intravenous prostaglandins were used in 82%. Aortic arch anastomosis was termino-lateral in 77,5%, termino-terminal in 17,5% and patch aortoplaty in 5%. ACP duration was 36 ± 11 minutes, 23ºC rectal, with in nominate artery cannulation in 47%, and flow reduced to 30-40 ml/kg/min. Brain monitorization used right radial artery pressure and cerebral saturation (INVOS). Hospital mortality was 10%. Hospital morbidity: Vocal cord paralysis in 38%, recoarctation with reoperation in 5%, and brain stroke in 2,8%. Mean follow-up was 26 ± 21 months, with 2,7% mortality, and 3 patients with recoarctation (8%, percutaneous angioplasty performed in 2 children). Conclusion: Aortic arch surgery with ACP minimize neurologic injury. Hospital mortality is acceptable, but vocal cord paralysis is still an important problem, probably caused during aortic dissection. After hospital stay the evolution is good with 92% patients free of recoarctation (AU)

Tratamientos actuales aplicables a la transposición de las grandes arterias / Current treatment of complete transposition of the great arteries

Las distintas variantes de trasposición completa de grandes arterias (GA) constituyen el 8% del total de cardiopatías congénitas. Además, la TGA es la primera causa de cianosis neonatal de origen cardíaco. Por su frecuencia y homogeneidad, la corrección anatómica de la TGA simples es el principal marcador de calidad de un programa de cirugía cardíaca neonatal. Se incide en aspectos fisiopatológicos, diagnóstico y de manejo pre y postoperatorio y se revisan históricamente las diferentes técnicas quirúrgicas que se han empleado para reparar las TGA simples y complejas, hasta la corrección anatómica (operación de Jatene). Se analiza la mejora en la mortalidad hospitalaria y tardía hasta llegar a la excelencia situación actual, que ha variado el ominosos pronóstico de esta cardiopatía, abandonada a su evolución natural. En cualquier caso, los pacientes portadores de una TGA reparada, aún asintomáticos y sin tratamiento farmacológico, necesitarán revisiones cardiológicos de por vida (AU)

Different forms of transposition of the great arteries are a common form (8%) of congenital heart disease, being the primary cause of neonatal cardiac cyanosis. The anatomic correction of simple TGA is the main quality marker of any programme of neonatal cardiac surgery. We review pathophysiology, the improvent of pre and postoperative care, diagnostic criteria and the different surgical techniques, till the anatomic correction (arterial switch operation). Actually, hospital and late results are excellent, but even asympthomatics patients with a corrected TGA need cardiology follow-up for ever (AU)