Assessing pulmonary hypertensive vascular disease in childhood. Data from the Spanish registry.

RATIONALE: There is a lack of knowledge regarding the epidemiology, clinical characterization, and survival in pediatric pulmonary hypertension. OBJECTIVES: To describe the epidemiology, outcomes, and risk factors for mortality in pediatric pulmonary hypertension in Spain. METHODS: We analyzed data from the Spanish Registry for Pediatric Pulmonary Hypertension. From January 2009 to June 2012, a total of 225 patients diagnosed with pulmonary hypertension in 1998 or after were collected from 21 referral and nonreferral centers. We included all Nice etiologies, estimated incidence and prevalence of pulmonary hypertension in the Spanish pediatric population, and analyzed risk factors for mortality (Nice etiologic group, clinical and hemodynamic variables). Patients were classified as follows: group I, pulmonary arterial hypertension (n = 142; 61%); group II, left heart disease (n = 31; 14%); group III, respiratory disease (n = 41; 18%); group IV, thromboembolic pulmonary hypertension (n = 2; 1%); or group V, mostly inherited metabolic diseases (n = 10; 4.5%). Of the patients studied, 31% had multifactorial pulmonary hypertension. MEASUREMENTS AND MAIN RESULTS: Mean age at diagnosis was 4.3 ± 4.9 years (50% < 2 yr). Survival rates at 1 and 3 years were 80 and 74% for the whole cohort, and 89 and 85% for patients with pulmonary arterial hypertension. Independent risk factors for mortality included an etiologic group other than pulmonary arterial hypertension (P < 0.001), age at diagnosis younger than 2 years old (P < 0.001), advanced functional class at diagnosis (P < 0.001), and high right atrial pressure at diagnosis (P = 0.002). CONCLUSIONS: In moderate to severe pediatric pulmonary hypertension, the prognosis is better in pulmonary arterial hypertension than in other Nice categories. In pediatric pulmonary hypertension age at diagnosis younger than 2 years is a risk factor for mortality, in addition to the previously established risk factors.

Implantación de stents en cardiología pediátrica. Resultados iniciales / Use of stents in pediatric cardiology. Initial results

Antecedentes y objetivo: Un stent es un dispositivo útil en el tratamiento de lesiones estenóticas vasculares asociadas a cardiopatías congénitas. En el año 1997 iniciamos nuestra experiencia, cuyos resultados se exponen en este trabajo. Pacientes y método: Se han implantado 17 stents, en 12 niños con edad media de 82 meses (2-168 meses) mediante 13 procedimientos. Ocho pacientes tenían estenosis de ramas pulmonares posquirúrgicas; 3 pacientes recoartaciones de aorta y uno, cardiopatía compleja dependiente del ductus. En los primeros 8 pacientes se efectuó cateterismo derecho y se siguió la técnica habitual de implantación con guía de alto soporte y vaina de Mullins. En los restantes el abordaje fue por vía arterial femoral. Se utilizaron 13 stents de Palmaz; 3 stents Express; y uno, Intrastent. Resultados: El diámetro de las lesiones se incrementó tras el implante desde 3,1 (2,8-5,6) hasta 12 (10-15) mm (p<=0,002), y el gradiente a través de las estenosis descendió desde 32 (21-45) a 8,5 (0-11,5) mmHg (p<=0,002). En el paciente dependiente del ductus permitió la suspensión de prostaglandinas y el alta hospitalaria. Tras un seguimiento medio de 27 meses (1-60) ha habido una oclusión completa tardía de rama pulmonar y una parcial de arteria lobular superior derecha. Un enfermo falleció por causas ajenas al stent (shock cardiogénico previo), el resto están asintomáticos y no precisan medicación. Conclusión: El stent es un dispositivo seguro y eficaz. Puede ser utilizado en una amplia variedad de lesiones estenóticas (AU)

[Use of stents in pediatric cardiology. Initial results]. / Implantación de stents en cardiología pediátrica. Resultados iniciales.

BACKGROUND AND AIM: Balloon-expandable stents are useful in treating vascular stenotic lesions associated with congenital heart defects. We report our experience of this device since 1997. PATIENTS AND METHOD: Seventeen stents were implanted in 12 children in 13 procedures. The mean age of the patients was 82 months (range: 2-168 months). Eight patients had postsurgical stenoses in branch pulmonary arteries, three patients had aortic re-coarctation and one patient had a ductus-dependent complex heart defect. In the first eight patients we performed right catheterization, following the usual technique of percutaneous implantation with super-stiff wire and Mullins sheath. In the remaining patients, vascular access was through the femoral artery. The Palmaz stent was used in 13 patients, Express stents in three and Intrastent in one patient. RESULTS: After stent implantation, the diameter of the narrowings increased from 3.1 mm (2.8-5.6) to 12 mm (10-15) (p < 0.002) and the gradient was reduced from 32 mmHg (21-45) to 8.5 mmHg (0-11.5) (p < 0.002). In the patient with ductus-dependent heart defect, the procedure allowed prostaglandin E1 withdrawal and hospital discharge. After a mean follow-up of 27 months (range: 1-60 months) one occurrence of late complete occlusion of branch pulmonary artery and one case of side-branch partial occlusion (right superior lobe branch) have been detected. One patient died from causes unrelated to the stent (prior cardiogenic shock). The remaining patients are asymptomatic and medication free. CONCLUSION: The stent is a safe and effective device that could be used in a wide variety of stenotic vascular lesions.

[Doppler echocardiographic evaluation of pulmonary hypertension in children]. / Valoración con ecocardiografía Doppler de la hipertensión pulmonar del niño.

INTRODUCTION: We analysed the usefulness of Doppler echocardiography to determine the presence and severity of pulmonary hypertension (PH) in children. METHOD: The whole group consisted of 63 patients, 42 with congenital heart disease that underwent cardiac catheterization (32 of whom had PH = study group) and 21 healthy children. These 21 patients and the remaining 10 without PH at cardiac catheterization made up the control group. All children were studied with Doppler Echocardiography to evaluate the pulmonary flow pattern with the sample volume placed in the pulmonary artery trunk, 1 cm distal to the pulmonic valve. The preejection period (PEP), ejection period (EP), acceleration time (AcT), the indexes PEP/EP, PEP/AcT, AcT/EP and the morphologic pattern of the pulmonary flow (type I: with peak flow velocity at midsystole; type II: with peak flow velocity in early systole; type III: with midsystolic notching) were analysed and quantitative parameters corrected according to hear rate by dividing theirs value by the square root of R-R interval. In the hemodynamic study we analysed the systolic (SPAP), diastolic and mean pulmonary artery pressure, and the mean pulmonary pressure/mean systemic pressure ratio (Pp/Sp). We compared the echocardiographic variables in both, study and control groups, and analysed the hemodynamic and echocardiographic correlation between the variables in question. RESULTS: Pattern I of pulmonary flow was associated with absence of PH and pattern II and III with PH (p < 0.001). The best results of quantitative variables were either corrected AcT (AccT) rather less in the study group than in control group (2.89 +/- 0.56 vs 4.05 +/- 0.56 ms, p < 0.001) and PPE/AcT index, 1.28 +/- 0.3 in the hypertensive group and 0.78 +/- 0.16 in the control group (p < 0.001). The best correlation were AcT with SPAP (r = -0.82) and Act with Pp/Sp ratio (r = -0.84). CONCLUSIONS: We consider that pulmonary flow analysed with Doppler echocardiography is a reliable, suitable and non-invasive method to evaluate PH in children.

[An evaluation of surgical correction in tetralogy of Fallot. A study of 101 cases]. / Evaluación de la corrección quirúrgica en la tetralogía de Fallot. Un estudio de 101 casos.

INTRODUCTION: The optimal management of infants with tetralogy of Fallot continues to evolve. We review our series to evaluate the results. PATIENTS AND METHODS: From 1979 to 1992, 101 children with tetralogy of Fallot without pulmonary atresia, were operated on. Infundibular and valvar stenosis were present in 59 cases (58.4%), distal stenosis in 24 (23.7%) and trunk and/or branches hypoplasia in 14 (13.9%). Until 1985, symptomatic infants underwent palliative surgical techniques. Since then, we prefer early repair as elective treatment in all cases, using palliative techniques only in symptomatic infants with inadequate anatomy. Palliative techniques were used in 35 children (34.6%), mean age at surgery was 6.7 +/- 6.7 months; corrective surgery, after palliative technique, in 23 children (22.8%), mean age at surgery was 36.0 +/- 12.9 months and primary correction in 66 children (65.3%), mean age at surgery was 30.7 +/- 20.8 months. For 45 patients (44.6%) the right ventricular outflow tract obstruction was relieved by a transannular patch. RESULTS: Post-repair right ventricular-left ventricular pressure ratio is a usefull index to predict the short and long-term evolution of this cardiopathy. Thus, values were significantly smaller in children without postoperative cardiac failure (0.51 +/- 0.10 vs 0.59 +/- 0.15; p < 0.01), in the survivors (0.53 +/- 0.12 vs 0.72 +/- 0.13; p < 0.001) and in those with better functional status in the follow-up (0.52 +/- 0.12 vs 0.66 +/- 0.13; p < 0.001). Whole mortality was 13% for two-stage correction and 7.6% for primary correction. From 1985 mortality has reduced at 6.7 and 2.3% respectively. The follow-up was completed in 78 children with corrective surgery (96%), with a mean of 43.4 +/- 32.6 months. There were three later deaths. Actuarial survival at six years is 86%. CONCLUSION: We have proved that the optimal treatment in infants with tetralogy of Fallot and suitable size pulmonary vascular tree is the early primary repair. The pressure relation between both ventricles post-repair is a useful index for the outcome.

[Severe aortic stenosis in the neonate and infant under 6 months old]. / Estenosis aórtica severa en el recién nacido y lactante menor de seis meses.

24 patients with diagnosed aortic stenosis are reported. Clinical symptoms started before 6 months of age (between 24 hours and 6 months, average 87 days). In all cases aortic stenosis was the only existing malformation, except in one patient who had a small muscular ventricular septal defect and in another case who had a patent ductus arteriosus. Clinical symptoms were early and notable. In nineteen there was congestive heart failure whose appearance in the first week of like was a bad prognosis. Relationship between severity of aortic stenosis and electrocardiographic findings was closer than in older patients. Death rate was very high in children who had not been operated (5 out of 12). Of children who received surgery only one died out of 12. Treatment, therefore, in this pathology must be aggressive.

[Aortic coarctation in children. Analysis of 100 cases]. / Coartación de aorta en el niño. Análisis de 100 casos.

One hundred clinical records of coarctation of aorta are presented with 78% catheterization and 31% postmortem examination. Surgical treatment was performed in 46%, 8, in the first month of life, 13 from 1 to 6 month, 5, from 6 to 12 month, 9 from 12 to 24 month and 11 older than 2 years. The surgical mortality was 13%. There was recoarctation in 7.5%. The mortality in the patients not operated on was 31%. This mortality was influenced by the severity of associated malformations. The most frequent associated cardiac malformations were left to right shunts followed by left heart pathology. The association with complex cardiopathies was frequent.

[Visceral leishmaniasis in childhood (author's transl)]. / El Kala-azar en la infancia. Estudio en la provincia de Sevilla.

Authors present six cases of kala-azar in children under six years of age observed in the province of Seville, during a three year period. Ethiologic, clinical, evolutive and therapeutic aspects of this illness are analized. Two of the patients presented jaundice, in which a liver-spleen scan was performed and nothing else but the hepato-splenomegaly was found, as it was described in a previous case. Importance of visualizing leishmanias by bone marrow puncture for a true diagnosis is remarked.