No abstract

Standardization of the diagnostic routine for children with congenital heart disease associated with pulmonary arterial hypertension (PAH-CHD) is crucial, in particular since inappropriate assignment to repair of the cardiac lesions (e.g., surgical repair in patients with elevated pulmonary vascular resistance) may be detrimental and associated with poor outcomes. Thus, members of the Congenital Heart Disease and Pediatric Task Forces of the Pulmonary Vascular Research Institute decided to conduct a survey aimed at collecting expert opinion from different institutions in several countries, covering many aspects of the management of PAH-CHD, from clinical recognition to noninvasive and invasive diagnostic procedures and immediate postoperative support. In privileged communities, the vast majority of children with congenital cardiac shunts are now treated early in life, on the basis of noninvasive diagnostic evaluation, and have an uneventful postoperative course, with no residual PAH. However, a small percentage of patients (older at presentation, with extracardiac syndromes or absence of clinical features of increased pulmonary blood flow, thus suggesting elevated pulmonary vascular resistance) remain at a higher risk of complications and unfavorable outcomes. These patients need a more sophisticated diagnostic approach, including invasive procedures. The authors emphasize that decision making regarding operability is based not only on cardiac catheterization data but also on the complete diagnostic picture, which includes the clinical history, physical examination, and all aspects of noninvasive evaluation.

No abstract

Background: Ideally, vasodilator therapies for pulmonary arterial hypertension (PAH) should have a favorable impact on markers of vascular dysfunction, in addition to their known effects on hemodynamics, cardiac function, and patient's physical capacity. Methods:We analyzed circulating (plasma) markers of endothelial and platelet activation/dysfunction (enzyme-linked immunoassays) in the specific setting of advanced PAH associated with congenital heart disease, during the course of sildenafil and tadalafil therapies. Thirty-one patients were enrolled (age 10-54 years), most of them with chronic hypoxemia and elevated hematocrit. Drugs were administered orally for 6 months (sildenafil [n = 16], 20 mg t.i.d.; tadalafil [n = 15], single daily dose of 40 mg). Measurements were performed at baseline, and 90 and 180 days.Results: Compared to controls, patients had elevated baseline β-thromboglobulin (β-TG, P = .002), P-selectin (P = .027), tissue-type plasminogen activator (t-PA, P = .009), and von Willebrand factor antigen (VWF:Ag, P = .010). Thrombomodulin was importantly reduced (TM, P < .001), while soluble CD40 Ligand was not changed (P = .320). Tadalafil administration was associated with improvement of β-TG (P = .004), t-PA (P = .003) and TM (P = .046) levels, while P-selectin was improved by sildenafil treatment only (P = .034). VWF:Ag improved transiently in the sildenafil group (P = .019). Both therapies were associated with improvement of the physical capacity (functional class and distance walked during the 6-minute test, P < .05), hematocrit and hemoglobin level (P < .05), and health-related quality of life (physical and mental components, P < .05). Conclusion:In PAH associated with congenital heart disease, phosphodiesterase 5 inhibitors seem to have beneficial actions at microcirculatory level, beyond the proposed effects as vasodilators. K E Y W O R D Sbiomarkers, congenital heart disease, endothelial dysfunction, phosphodiesterase 5 inhibitors, platelets, pulmonary hypertension | 247 CLAVÉ et al.

Respiratory virus infections are the main cause of infant hospitalization and are potentially severe in children with congenital heart disease (CHD). Rapid and sensitive diagnosis is very important to early introduction of antiviral treatment and implementation of precautions to control transmission, reducing the risk of nosocomial infections. In the present study we compare different techniques in the diagnosis of respiratory viruses in CHD infants. Thirty-nine samples of nasopharyngeal aspirate were obtained from CHD infants with symptoms of respiratory infection. The Multiplex PCR (Seeplex® RV 12 ACE Detection) driven to the detection of 12 respiratory viruses was compared with the direct immunofluorescence assay (DFA) and PCR, both targeting seven respiratory viruses. The positivity found by DFA, Multiplex and PCR was 33.3%, 51.3% and 48.7%, respectively. Kappa index comparing DFA and Multiplex, DFA and PCR and PCR and Multiplex PCR was 0.542, 0.483 and 0.539, respectively. The concordance between techniques was considered moderate. Both Multiplex PCR (p = 0.001) and PCR (p = 0.002) detected significantly more respiratory virus than DFA. As the performance of the tests may vary, the combination of two or more techniques may increase diagnostic sensitivity favoring the diagnosis of co-infections, early introduction of antiviral therapy and implementation of appropriate measures.

The morphological findings in the lung vessels of children with pulmonary arterial hypertension associated with uncorrected congenital cardiac shunts may be qualitatively and quantitatively assessed by lung biopsy in selected cases. Among 297 lung biopsies collected over a period of 11 years two cases were detected presenting the typical findings of pulmonary capillary hemangiomatosis, a rare disorder usually associated with pulmonary veno-occlusive disease. We report the clinical and histological findings and discuss about the impact of this unexpected and not-previously described associated lesion on the prognosis and on the therapeutic strategy in patients with pulmonary hypertension associated with congenital heart disease.

Management of pediatric pulmonary hypertension associated with congenital heart disease (PHT-CHD) is challenging. Some patients have persistently elevated pulmonary artery pressure (PAP) after cardiac surgery, an undesired condition that is difficult to predict. We investigated the value of clinical, hemodynamic, and histopathological data in predicting the outcome in a prospective cohort. Patients with PHT-CHD received sildenafil orally pre- and postoperatively for six months and then were subjected to a catheter study. Thirty-three patients were enrolled (age range = 4.6–37.0 months). Pulmonary vascular resistance (PVR) was 4.9 (range = 3.9–7.2) Wood units × m2 (median with IQR). Twenty-two patients had a ≥ 20% decrease in PVR and pulmonary-to-systemic vascular resistance ratio (PVR/SVR) in response to inhaled nitric oxide (NO). The response was directly related to the degree of medial hypertrophy of pulmonary arterioles ( P < 0.05) (morphometric analysis, intraoperative lung biopsy). Subsequently, five of the non-responders had a ≥ 30% increase in pulmonary blood flow in response to sildenafil (3.0 [2.0–4.0] mg/kg/day). Six months after surgery, PAP and PVR were significantly lower ( P < 0.001 vs. baseline), even in seven patients with Heath-Edwards grade III/IV pulmonary vascular lesions ( P = 0.018), but still abnormal in 12 individuals (>25 mmHg and >3.0 U × m2, respectively). A preoperative PVR/SVR of ≥24% during NO inhalation and a wall thickness of arteries accompanying respiratory bronchioli of ≥4.7 (Z score) were identified, respectively, as risk and protection factors for abnormal postoperative hemodynamics (hazard ratio [95% CI] = 1.09 [1.01–1.18], P = 0.036; and 0.69 [0.49–0.98], P = 0.040, respectively). Thus, in PHT-CHD patients receiving oral sildenafil pre- and post-surgical repair of cardiac lesions, mid-term postoperative outcome is predictable to some extent.

A indústria de alimentos, em especial a de processamento de frutos, produz uma grande quantidade de resíduos agroindustriais. Esses resíduos constituem 65-70% da massa total dos frutos, conforme a espécie do fruto. Uma alternativa que vem crescendo, consiste no aproveitamento destes resíduos como matéria-prima para a produção de alimentos que sejam incluídos na alimentação humana. Este trabalho teve como objetivo propor a elaboração de produtos de panificação com substituições parciais da farinha de trigo por sementes de goiaba em pó. Foram utilizadas três formulações com diferentes percentuais de substituição da farinha de trigo por sementes de goiaba em pó, com níveis de substituição de 5%, 10% e 0% (controle). Como resultado pode-se observar que a preparação bolo doce, formulações com 5% e 10% de substituição, apresentaram médias equivalentes aos termos hedônicos "gostei moderadamente" e "gostei muito". A preparação pizza sabor portuguesa, formulação com 5% de substituição obteve as maiores médias em todos os atributos pesquisadas, cujas médias equivaleram ao termo hedônico "gostei moderadamente". Com isso, pode-se concluir que a adição de pós obtidos de resíduos de frutos como a goiaba é uma alternativa bastante viável para evitar o desperdício e agregar benefícios nutricionais aos produtos de panificação. PALAVRAS-CHAVE:semente de goiaba (Psidium guajava L.) em pó, desenvolvimento de produtos, análise sensorial. PREPARATION AND ACCEPTABILITY OF BAKING PRODUCTS ENRICHED WITH GUAVA SEEDS (Psidium guajava L.) POWDER ABSTRACTThe food industry, in particular fruit processing, produces a large amount of waste. These residues constitute 65-70 % of the total weight of the fruit, depending on the species. An increasingly popular alternative is to make use of this waste as feedstock for the production of foods for human consumption. This work proposes the preparation of bakery products, partially replacing wheat flour by guava seeds powder. Three formulations were used, with three different percentages of replacement of wheat flour by guava seeds powder at levels of 5% substitution, 10% and 0% (control). The results show that the preparation sweet cake formulations with 5% and 10% substitution, showed equivalent to medium hedonic terms "like moderately" and "liked". The preparation portuguese pizza flavor formulation with 5% substitution had the highest mean scores in all the surveyed attributes, equivalent to the hedonic term "like moderately". Thus, we can conclude that the addition of powder made from the residue of fruits like guava is a highly feasible alternative which both avoids wastage and adds nutritional benefits to bakery products.