[Absent pulmonary valve syndrome with ductal origin of the left pulmonary artery. Diagnosis only by 2-D echo doppler color flow mapping]. / Síndrome de agenesia valvular pulmonar con emergencia ductal de arteria pulmonar izquierda. Papel de la ecocardiografía Doppler color.

A two-month old infant is described with the rare combination of absent pulmonary valve syndrome, ventricular septal defect, pulmonar "anular" stenosis and ductal origin of the left pulmonary artery. The diagnosis that was confirmed in the operating room was made by 2-D echocardiographic Doppler color flow mapping study without the support of cardiac catheterization.

[Balloon pulmonary valvuloplasty in the neonatal period. The clinical and echocardiographic effects]. / Valvuloplastia pulmonar con balón en el período neonatal. Efectos clínicos y ecocardiográficos.

AIM: To analyze the efficacy of balloon pulmonary valvuloplasty (BPV) as the elective treatment for neonatal critical pulmonary valvar stenosis (PVS). MATERIALS AND METHODS: The results of clinical and echocardiographic features before and after the BPV were reviewed in 29 neonates (18+/-12 days of life). Different hemodynamic and 2-D color Doppler echocardiographic were evaluated. The BPV result was classified as favourable if no other balloon or surgical therapy was required to normalise pulmonary flow and achieve a sustained right ventricle-pulmonary artery (RV-PA) Doppler gradient below 40 mmHg. It was considered unfavourable if the neonate died, needed surgery or redilation and/or the RV-PA Doppler gradient was > or =40 mm Hg. The study developed in three phases: pre BPV immediate post BPV until the hospital discharge (14+/-11 days), and in the mid-term follow-up of between 8 and 96 months (51+/-31 months). RESULTS: Mortality was not registered with BPV. The RV/left ventricular systolic pressure decreased from 1.4+/-0.3 to 0.8+/-0.3 (p<0.01) as a consequence of the dilation, and the the systemic oxygen saturation increased from 85 +/-12 to 92+/-6% (p<0.01). The RV-PA Doppler gradient diminished from 86+/-18 to 28+/-16 mm Hg immediately after BPV (p<0.01) and was registered at 13+/-6 mm Hg in the follow-up (n = 24). The RV-PA junction Z value grew from -1.25+/-0.9 before valvuloplasty to -0.51 +/-0.7 at the final echocardiogram (p<0.01). No changes in the tricuspid diameter were detected between both periods of time. Five neonates obtained unsatisfactory results: 4 in the immediate post BPV (systemic-pulmonary artery shunt 2, transannular patch 2), and 1 in the mid-term follow-up (valvectomy + transannular patch). The actuarial curve reflects that 82,7% of the patients were free form reinterventions at 8 years. CONCLUSIONS: BPV is safe and effective to relief PVS in the neonate. The balloon promotes advantageous changes in both, pulmonary annulus and the right ventricle. In addition, the RV-PA Doppler gradient observations in the follow-up, support the expectation that the BPV is a "curative" therapy.

[Percutaneous balloon pulmonary valvuloplasty: the medium-term results in a series of 100 consecutive pediatric patients]. / Valvuloplastia pulmonar percutánea con balón: resultados a medio plazo de una serie de 100 pacientes pediátricos consecutivos.

OBJECTIVE: To study medium-term follow-up in children with pulmonary stenosis (PS) with percutaneous balloon pulmonary valvuloplasty (PBVP). PATIENTS AND METHODS: Medium-term follow-up results of 106 consecutive PBVP procedures in 100 patients of the same health center are presented. The mean age of the patients was 61.3 +/- 5.1 months, 31 of them with severe PS. RESULTS: After PBVP, and once infundibular contraction ceased, the procedure was defined as successful in 85 children. PBVP was repeated in five patients, achieving satisfactory results in 3. Eight patients suffered from re-stenoses and surgery was performed in 7 of them. The other 12 patients in which PBVP failed underwent surgery. Follow-up of these 88 patients showed a cessation in clinical symptomatology at a mean of 10 +/- 0.7 months. Five-year actuarial probability of achieving a normal EKG axis was 61.7 +/- 9.1%. However, neither ECG nor chest radiology were useful in the diagnosis of high residual gradients. Pulmonary regurgitation was observed in 92% of the children, mild in all of them. The residual transpulmonary gradient obtained immediately after PBVP was the only variable that affected long-term results. CONCLUSIONS: Our results confirm the medium-term success of PBVP in the treatment of PS. The five year actuarial probability of restenosis is 12.4%. Mild pulmonary regurgitation is observed in more than 90% of patients.

[Aortico-left ventricular tunnel associated with pulmonary valve stenosis]. / Túnel aorta-ventrículo izquierdo asociado con estenosis valvular pulmonar.

Aortico-left ventricular tunnel is an unusual cardiac anomaly. The main clinical feature is early, severe aortic regurgitation, and surgical management is mandatory. Exceptionally this defect is associated with pulmonary valve stenosis. A case of a newborn with aortico-left ventricular tunnel plus pulmonary valve stenosis is reported. Initially she underwent percutaneous pulmonary valvuloplasty during diagnostic cardiac catheterization and with surgical closure of the tunnel later.

[Diagnostic features of congenital absence of pulmonary valve (author's transl)]. / Agenesia de las sigmoideas pulmonares. Factores diagnósticos.

Diagnostic features of the absent pulmonary valve syndrome are reported. Association of this syndrome with ventricular septal defect, tetralogy of Fallot and tricuspid atresia is stressed. Severe ventilatory problems are frequent during the neonatal period. A exaggerated right ventricular hypertrophy, in comparison with that expected in the usual case of tetralogy of Fallot, is a useful parison with that expected in the usual case of tetralogy of Fallot, is a useful electrocardiographic sign. Complete diagnosis sometimes requires the technique of occlusion aortography sign. Complete diagnosis sometimes requires the technique of occlusion aortography or contrast injections into pulmonary veins.

Univentricular hearts: pathology of the atrioventricular valves.

Anomalies of the atrio-ventricular valves are frequent in cases of univentricular heart with severe symptomatology during the first year of life and in necropsy material. This suggests that they are important determinants of the natural history of the malformation. Radical "corrective" surgery, in the presence of these anomalies, may be less successful than palliative programs based on sound physiological grounds. Under such circumstances, enlargement or creation of an atrial septal defect may be necessary. A careful assessment of the state of the atrio-ventricular orifices has to be included in the cardiac catheterization protocol of cases of univentricular hearts. From a morphogenetic viewpoint, a single ventricular cavity is not the appropriate substrate for two normal atrio-ventricular valves to develop.