[Anomalous origin of the right pulmonary artery from the ascending aorta. Its surgical correction in early infancy]. / Origen anómalo de la arteria pulmonar derecha de la aorta ascendente. Corrección quirúrgica en la primera infancia.

Anomalous origin of the right pulmonary artery from the ascending aorta is a rare congenital malformation associated with a high mortality during the first year of life. Up to 1990, 75 cases have been reported in the literature. We are reporting 2 cases diagnosed and operated before 3 months of age. Repair was facilitated by the use of deep hypothermic circulatory arrest. The duration of follow up is 5 and 6 years, respectively. Pre and postoperative cardiac catheterization pressure data and angiograms are shown. We review the literature, presenting the physiopathological, embryological and surgical features of this rare congenital anomaly.

[Palliative arterial switch]. / Switch arterial paliativo.

We report the first case published in Spain of a palliative anatomic correction associated with aortic arch repair in a neonate with (S,D,L)-transposition of the great arteries, multiple ventricular septal defects, and severe hypoplasia of the right ventricle with subaortic obstruction and hypoplasic aortic arch with coarctation. A one stage palliative surgery on cardiopulmonary bypass was performed with reconstruction of the aortic arch and an arterial switch procedure which obtained a satisfactory result. The principle of this operation is to switch the subaortic obstruction into a subpulmonary obstruction and reconstruct a large natural aortic root from the principal ventricle. The right ventricle-pulmonary artery continuity may promote growth of the right ventricle with the possibility of a future biventricular repair. We conclude that this operation, when used by surgical teams experienced with arterial switch surgery, is the best treatment for the complex newborn group with single ventricles or severe ventricular disbalance, ventriculoarterial discordance and stablished subaortic stenosis.

[Risk factors in the Glenn bidirectional shunt as an intermediate procedure before Fontan correction]. / Factores de riesgo en el shunt de Glenn bidireccional como proceder intermedio antes de la corrección de Fontan.

OBJECTIVE: The bidirectional Glenn shunt is the most common palliation before the Fontan repair, especially in high-risk patients. We studied the influence of certain risk factors in bidirectional Glenn results, with and without an additional source of pulmonary blood flow. METHODS: Between 1993 and 1998 twenty patients (6-53 months of age) underwent a bidirectional Glenn shunt as the intermediate repair for the Fontan procedure. Diagnoses were: 7 cases of double inlet single ventricle, 4 of tricuspid atresia, 3 of unbalanced AV septal defect, 4 of mitral atresia, 1 hypoplastic left heart syndrome and 1 TGA with hypoplasia of the right ventricle. 17 patients had undergone previous operations. Mean preoperative arterial oxygen saturation was 78.5%. In 6 patients an auxiliary source of pulmonary blood flow was added. RESULTS: Hospital mortality was 15%. In 4 patients the bidirectional Glenn failed. By univariate analysis low weight, preoperative functional status and high pulmonary pressure were factors associated with early death. In bidirectional Glenn failure only the duration of ventilatory support was significant. By multivariate analysis, preoperative functional status and pulmonary pressure were significant. Mean postoperative arterial oxygen saturation at a mean follow-up of 10 months was 84%. CONCLUSIONS: Bidirectional Glenn shunt is an effective and low-risk palliation for patients with univentricular hearts. Only low weight and high pulmonary pressure were significant in hospital mortality, and we advise the association of an additional source of pulmonary blood flow in these patients at the start. Early extubation provides correct performance of the shunt.

[Scimitar syndrome: series of 12 cases]. / Síndrome de la cimitarra: serie de 12 casos.

Our experience in 12 cases of pediatric patients with scimitar syndrome is reported. Except for one, all of them presented cardiac or respiratory manifestations. The symptomatology was related to associated defects (3 atrial septal defects and 1 multiple peripheric pulmonary stenosis), degree of pulmonary hypoplasia, size of the right to left shunt and pulmonary hypertension. 3 patients underwent surgical treatment. One of them died during operation and the other two have had a good evolution. Nine patients with later respiratory manifestations have improved their condition progressively without surgical intervention. Therapeutic approach for patients with scimitar syndrome, respiratory manifestations and onset beyond the neonatal period, should be conservative.

[Clinical consensus on respiratory syncytial virus (RSV) infection prophylaxis and the use of palivizumab in paediatric cardiology.]. / Consenso clínico sobre la profilaxis de la infección por virus respiratorio sincitial y el uso del palivizumab en cardiología pediátrica.

BACKGROUND: Following the results of the CIVIC study, the SECPCC proposes to revise its recommendations for the prevention of RSV, taking into account the new evidence, as well as the preventive experience of paediatric cardiologists. For this purpose a structured method of professional consensus has been chosen. OBJECTIVES: To develop a Spanish clinical consensus on preventing infection by RSV under the auspices of the Sociedad Española de Cardiología Pediátrica y Cardiopatías Congénitas. METHODS: Delphi Consensus modified in two rounds. The study was conducted in four phases: 1) constitution of a Scientific Committee for bibliographic review and submission of the recommendations for discussion, 2) constitution of an Expert Panel with 75 representatives in the speciality, 3) postal survey organised in two rounds and intermediate processing of opinions, and issuing of a report to the panellists, and 4) discussion of the results in a face-to-face meeting of the Scientific Committee. RESULTS: Consensus was reached on 54 of the 70 preventive recommendations analysed. With respect to the 16 remaining issues, no consensus was reached, due to differences in professional opinion and the absence of established criteria among the majority of the experts. CONCLUSIONS: A set of recommendations for RSV prophylaxis in cardiology was developed and updated, rated in accordance with the degree of professional consensus on which they were based. These can be considered valid until such time as new scientific information emerges that warrants a further review.

[Congenital epiphyseal chondrodysplasia punctata. Study of 9 cases]. / Condrodisplasia punctata epifisaria congénita. Estudio de nueve casos.

Clinical data of nine cases of chondrodysplasia punctata, eight males and one female are reported. Two males presented with rhizomelic form, characterized by severe symmetrical shortness of humeri and femora, marked metaphyseal changes, severe psychomotor retardation and cataracts. Consanguinity of parents in one of these cases was consistent with homozygote for an autosomal retarded physical development starting early in life. Cataracts were absent, and psychomotor development was normal except in two cases who were severely retarded. These two cases suffered also from congenital cardiomyopathy (one case of aortic coarctation and the other of severe pulmonary hypertension without shunts). These seven cases were diagnosed of Conradi-Hunermann type which is a mild form of disease.