The relationship between Lamb weather types and long-term changes in flood frequency, River Eden, UK

Research has found that both flood magnitude and frequency in the UK may have increased over the last five decades. However, evaluating whether or not this is a systematic trend is difficult because of the lack of longer records. Here we compile and consider an extreme flood record that extends back to 1770. Since 1770, there have been 137 recorded extreme floods. However, over this period, there is not a unidirectional trend of rising extreme flood risk over time. Instead, there are clear flood-rich and flood-poor periods. Three main flood-rich periods were identified: 18731904, 19231933, and 1994 onwards. To provide a first analysis of what is driving these periods, and given the paucity of more sophisticated datasets that extend back to the 18th century, objective Lamb weather types were used. Of the 27 objective Lamb weather types, only 11 could be associated with the extreme floods during the gauged period, and only 5 of these accounted for > 80% of recorded extreme floods The importance of these five weather types over a longer timescale for flood risk in Carlisle was assessed, through calculating the proportion of each hydrological year classified as being associated with these flood-generating weather types. Two periods clearly had more than the average proportions of the year classified as one of the flood causing weather types; 19001940 and 19832007; and these two periods both contained flood-rich hydrological records. Thus, the analysis suggests that systematic organisation of the North Atlantic climate system may be manifest as periods of elevated and reduced flood risk, an observation that has major implications for analyses that assume that climatic drivers of flood risk can be either statistically stationary or are following a simple trend. Copyright (c) 2011 Royal Meteorological Society

Reproducibility and relative validity of a food-frequency questionnaire for French-speaking Swiss adults.

BACKGROUND: Due to the distinct cultural and language differences that exist in Switzerland, there is little information on the dietary intake among the general Swiss population. Adequately assessing dietary intake is thus paramount if nutritional epidemiological studies are to be conducted. OBJECTIVE: To assess the reproducibility and validity of a food-frequency questionnaire (FFQ) developed for French-speaking Swiss adults. DESIGN: A total of 23 men and 17 women (43.1+/-2.0 years) filled out one FFQ and completed one 24-hour dietary recall at baseline and 1 month afterward. RESULTS: Crude Pearson's correlation coefficients between the first and the second FFQ ranged from 0.58 to 0.90, intraclass correlation coefficient (ICC) ranged between 0.53 and 0.92. Lin's concordance coefficients ranged between 0.55 and 0.87. Over 80% of participants were classified in the same or adjacent tertile using each FFQ. Macronutrient intakes estimated by both FFQs were significantly higher than those estimated from the 24-hour recall for protein and water, while no significant differences were found for energy, carbohydrate, fats (five groups), and alcohol. De-attenuated Pearson's correlation coefficients between the 24-hour recall and the first FFQ ranged between 0.31 and 0.49, while for the second FFQ the values ranged between 0.38 and 0.59. Over 40 and 95% of participants fell into the same or the adjacent energy and nutrient tertiles, respectively, using the FFQs and the 24-hour recall. CONCLUSIONS: This FFQ shows good reproducibility and can be used determining macronutrient intake in a French-speaking Swiss population in an epidemiological setting.

Cardiovascular effects in patrol officers are associated with fine particulate matter from brake wear and engine emissions

Background: Exposure to fine particulate matter air pollutants (PM2.5) affects heart rate variability parameters, and levels of serum proteins associated with inflammation, hemostasis and thrombosis. This study investigated sources potentially responsible for cardiovascular and hematological effects in highway patrol troopers. Results: Nine healthy young non-smoking male troopers working from 3 PM to midnight were studied on four consecutive days during their shift and the following night. Sources of in-vehicle PM2.5 were identified with variance-maximizing rotational principal factor analysis of PM2.5-components and associated pollutants. Two source models were calculated. Sources of in-vehicle PM2.5 identified were 1) crustal material, 2) wear of steel automotive components, 3) gasoline combustion, 4) speed-changing traffic with engine emissions and brake wear. In one model, sources 1 and 2 collapsed to a single source. Source factors scores were compared to cardiac and blood parameters measured ten and fifteen hours, respectively, after each shift. The "speed-change" factor was significantly associated with mean heart cycle length (MCL, +7% per standard deviation increase in the factor score), heart rate variability (+16%), supraventricular ectopic beats (+39%), % neutrophils (+7%), % lymphocytes (-10%), red blood cell volume MCV (+1%), von Willebrand Factor (+9%), blood urea nitrogen (+7%), and protein C (-11%). The "crustal" factor (but not the "collapsed" source) was associated with MCL (+3%) and serum uric acid concentrations (+5%). Controlling for potential confounders had little influence on the effect estimates. Conclusion: PM2.5 originating from speed-changing traffic modulates the autonomic control of the heart rhythm, increases the frequency of premature supraventricular beats and elicits proinflammatory and pro-thrombotic responses in healthy young men. [Authors]

Quantifying Accuracy Improvement in Sets of Pooled Judgments: Does Dialectical Bootstrapping Work?

Galton (1907) first demonstrated the "wisdom of crowds" phenomenon by averaging independent estimates of unknown quantities given by many individuals. Herzog and Hertwig (2009; hereafter H&H in Psychological Science) showed that individuals' own estimates can be improved by asking them to make two estimates at separate times and averaging them. H&H claimed to observe far greater improvement in accuracy when participants received "dialectical" instructions to consider why their first estimate might be wrong before making their second estimates than when they received standard instructions. We reanalyzed H&H's data using measures of accuracy that are unrelated to the frequency of identical first and second responses and found that participants in both conditions improved their accuracy to an equal degree.

On the dual contrast enhancement mechanism in frequency-selective inversion-recovery magnetic resonance angiography (IRON-MRA)

The susceptibility of blood changes after administration of a paramagnetic contrast agent that shortens T(1). Concomitantly, the resonance frequency of the blood vessels shifts in a geometry-dependent way. This frequency change may be exploited for incremental contrast generation by applying a frequency-selective saturation prepulse prior to the imaging sequence. The dual origin of vascular enhancement depending first on off-resonance and second on T(1) lowering was investigated in vitro, together with the geometry dependence of the signal at 3T. First results obtained in an in vivo rabbit model are presented.

Computed tomography of the cervical spine: comparison of image quality between a standard-dose and a low-dose protocol using filtered back-projection and iterative reconstruction.

OBJECTIVE: To compare image quality of a standard-dose (SD) and a low-dose (LD) cervical spine CT protocol using filtered back-projection (FBP) and iterative reconstruction (IR). MATERIALS AND METHODS: Forty patients investigated by cervical spine CT were prospectively randomised into two groups: SD (120 kVp, 275 mAs) and LD (120 kVp, 150 mAs), both applying automatic tube current modulation. Data were reconstructed using both FBP and sinogram-affirmed IR. Image noise, signal-to-noise (SNR) and contrast-to-noise (CNR) ratios were measured. Two radiologists independently and blindly assessed the following anatomical structures at C3-C4 and C6-C7 levels, using a four-point scale: intervertebral disc, content of neural foramina and dural sac, ligaments, soft tissues and vertebrae. They subsequently rated overall image quality using a ten-point scale. RESULTS: For both protocols and at each disc level, IR significantly decreased image noise and increased SNR and CNR, compared with FBP. SNR and CNR were statistically equivalent in LD-IR and SD-FBP protocols. Regardless of the dose and disc level, the qualitative scores with IR compared with FBP, and with LD-IR compared with SD-FBP, were significantly higher or not statistically different for intervertebral discs, neural foramina and ligaments, while significantly lower or not statistically different for soft tissues and vertebrae. The overall image quality scores were significantly higher with IR compared with FBP, and with LD-IR compared with SD-FBP. CONCLUSION: LD-IR cervical spine CT provides better image quality for intervertebral discs, neural foramina and ligaments, and worse image quality for soft tissues and vertebrae, compared with SD-FBP, while reducing radiation dose by approximately 40 %.

Tuning In to Sound: Frequency-Selective Attentional Filter in Human Primary Auditory Cortex.

Cocktail parties, busy streets, and other noisy environments pose a difficult challenge to the auditory system: how to focus attention on selected sounds while ignoring others? Neurons of primary auditory cortex, many of which are sharply tuned to sound frequency, could help solve this problem by filtering selected sound information based on frequency-content. To investigate whether this occurs, we used high-resolution fMRI at 7 tesla to map the fine-scale frequency-tuning (1.5 mm isotropic resolution) of primary auditory areas A1 and R in six human participants. Then, in a selective attention experiment, participants heard low (250 Hz)- and high (4000 Hz)-frequency streams of tones presented at the same time (dual-stream) and were instructed to focus attention onto one stream versus the other, switching back and forth every 30 s. Attention to low-frequency tones enhanced neural responses within low-frequency-tuned voxels relative to high, and when attention switched the pattern quickly reversed. Thus, like a radio, human primary auditory cortex is able to tune into attended frequency channels and can switch channels on demand.

Nutritional deficiencies after Roux-en-Y gastric bypass for morbid obesity often cannot be prevented by standard multivitamin supplementation

BACKGROUND: Despite the increasing use of Roux-en-Y gastric bypass (RYGBP) in the treatment of morbid obesity, data about postoperative nutritional deficiencies and their treatment remain scarce. OBJECTIVE: The aim of this study was to evaluate the efficacy of a standard multivitamin preparation in the prevention and treatment of nutritional deficiencies in obese patients after RYGBP. DESIGN: This was a retrospective study of 2 y of follow-up of obese patients after RYGBP surgery. Between the first and the sixth postoperative months, a standardized multivitamin preparation was prescribed for all patients. Specific requirements for additional substitutive treatments were systematically assessed by a biologic workup at 3, 6, 9, 12, 18, and 24 mo. RESULTS: A total of 137 morbidly obese patients (110 women and 27 men) were included. The mean (+/-SD) age at the time of surgery was 39.9 +/- 10.0 y, and the body mass index (in kg/m(2)) was 46.7 +/- 6.5. Three months after RYGBP, 34% of these patients required at least one specific supplement in addition to the multivitamin preparation. At 6 and 24 mo, this proportion increased to 59% and 98%, respectively. Two years after RYGBP, a mean amount of 2.9 +/- 1.4 specific supplements had been prescribed for each patient, including vitamin B-12, iron, calcium + vitamin D, and folic acid. At that time, the mean monthly cost of the substitutive treatment was $34.83. CONCLUSION: Nutritional deficiencies are very common after RYGBP and occur despite supplementation with the standard multivitamin preparation. Therefore, careful postoperative follow-up is indicated to detect and treat those deficiencies.

Chemoradiotherapy in malignant glioma: standard of care and future directions.

Glioma has been considered resistant to chemotherapy and radiation. Recently, concomitant and adjuvant chemoradiotherapy with temozolomide has become the standard treatment for newly diagnosed glioblastoma. Conversely (neo-)adjuvant PCV (procarbazine, lomustine, vincristine) failed to improve survival in the more chemoresponsive tumor entities of anaplastic oligoastrocytoma and oligodendroglioma. Preclinical investigations suggest synergism or additivity of radiotherapy and temozolomide in glioma cell lines. Although the relative contribution of the concomitant and the adjuvant chemotherapy, respectively, cannot be assessed, the early introduction of chemotherapy and the simultaneous administration with radiotherapy appear to be key for the improvement of outcome. Epigenetic inactivation of the DNA repair enzyme methylguanine methyltransferase (MGMT) seems to be the strongest predictive marker for outcome in patients treated with alkylating agent chemotherapy. Patients whose tumors do not have MGMT promoter methylation are less likely to benefit from the addition of temozolomide chemotherapy and require alternative treatment strategies. The predictive value of MGMT gene promoter methylation is being validated in ongoing trials aiming at overcoming this resistance by a dose-dense continuous temozolomide administration or in combination with MGMT inhibitors. Understanding of molecular mechanisms allows for rational targeting of specific pathways of repair, signaling, and angiogenesis. The addition of tyrosine kinase inhibitors vatalanib (PTK787) and vandetinib (ZD6474), the integrin inhibitor cilengitide, the monoclonal antibodies bevacizumab and cetuximab, the mammalian target of rapamycin inhibitors temsirolimus and everolimus, and the protein kinase C inhibitor enzastaurin, among other agents, are in clinical investigation, building on the established chemoradiotherapy regimen for newly diagnosed glioblastoma.

Chronic low-frequency rTMS of primary motor cortex diminishes exercise training-induced gains in maximal voluntary force in humans.

Although there is consensus that the central nervous system mediates the increases in maximal voluntary force (maximal voluntary contraction, MVC) produced by resistance exercise, the involvement of the primary motor cortex (M1) in these processes remains controversial. We hypothesized that 1-Hz repetitive transcranial magnetic stimulation (rTMS) of M1 during resistance training would diminish strength gains. Forty subjects were divided equally into five groups. Subjects voluntarily (Vol) abducted the first dorsal interosseus (FDI) (5 bouts x 10 repetitions, 10 sessions, 4 wk) at 70-80% MVC. Another group also exercised but in the 1-min-long interbout rest intervals they received rTMS [Vol+rTMS, 1 Hz, FDI motor area, 300 pulses/session, 120% of the resting motor threshold (rMT)]. The third group also exercised and received sham rTMS (Vol+Sham). The fourth group received only rTMS (rTMS_only). The 37.5% and 33.3% gains in MVC in Vol and Vol+Sham groups, respectively, were greater (P = 0.001) than the 18.9% gain in Vol+rTMS, 1.9% in rTMS_only, and 2.6% in unexercised control subjects who received no stimulation. Acutely, within sessions 5 and 10, single-pulse TMS revealed that motor-evoked potential size and recruitment curve slopes were reduced in Vol+rTMS and rTMS_only groups and accumulated to chronic reductions by session 10. There were no changes in rMT, maximum compound action potential amplitude (M(max)), and peripherally evoked twitch forces in the trained FDI and the untrained abductor digiti minimi. Although contributions from spinal sources cannot be excluded, the data suggest that M1 may play a role in mediating neural adaptations to strength training.

Subretinal neovascular membranes complicating uveitis : frequency, treatments and visual outcome

RESUME Les membranes néovasculaires (MNV) compliquent diverses pathologies ophtalmiques. Elles sont à l'origine d'une importante baisse de l'acuité visuelle lorsque elles se situent à proximité de la fovéa. A l'heure actuelle, peu de données relatives à leur association aux pathologies inflammatoires de l'oeil (uvéites) existent. Dans ce travail, la fréquence de MNV a été évaluée parmi 643 patients avec uvéite. Leur impact sur l'acuité visuelle ainsi que le pronostic en fonction des différents traitements effectués ont été étudiés. Les dossiers des 643 patients souffrant d'uvéite ont été étudiés. Les patients présentant une MNV ont été classés en trois groupes en fonction de l'importance de l'inflammation intraoculaire: élevée (2+ cellules dans le vitré), moyenne (1/2+ à 1+ cellules dans le vitré) ou absente (0 cellules dans le vitré). L'évolution de l'acuité visuelle fut considérée comme favorable (+VA: maintient de l'acuité visuelle ou gain d'une ou plusieurs lignes de Snellen) ou défavorable (-VA: perte d'une ou plusieurs lignes Snellen). Chez 9 patients, le traitement instauré a consisté, initialement, en l'administration orale de corticostéroïdes (CST) à haute dose qui, dans le cas d'évolution favorable (-FVA ou régression angiographique de la MNV), était arrêtée en doses dégressives. Dans les évolutions défavorables (-VA ou progression angiographique de la MNV), les CST étaient maintenus à dose moyenne en complémentation d'un traitement par thérapie laser (photothérapie dynamique (PDT), thermothérapie transpupillaire (TTT) ou laser Argon). Ce protocole thérapeutique ne fut appliqué chez trois patients en raison de la non disponibilité de PDT ou d'un diagnostic manqué d'uvéite. Douze patients sur 643 avec uvéite ont présenté une MNV. L'impact visuel moyen était de 4.5 lignes de Snellen et le temps moyen de suivi était de 19.5 mois. Deux patients avec inflammation intraoculaire élevée ont évolué favorablement sous CST seuls. Huit patients avec inflammation intraoculaire moyenne ont évolué favorablement sous CST seuls chez trois patients, alors que quatre patients ont nécessité une thérapie laser additionnelle. Le dernier patient ne fut traité que par thérapie laser sans CST (diagnostic manqué d'uvéite). Deux patients sans inflammation intraoculaire ont eu un pronostic défavorable sous CST seuls (pas d'autre alternative thérapeutique). Notre étude a démontré que les MNV sont une complication rare de l'uvéite qui, après traitement adéquat, ont un pronostic visuel relativement favorable. Bien que les CST semblent être la première modalité thérapeutique, les traitements laser devraient être adoptés tôt dans les situations d'inflammation intraoculaire moyenne ou absente.

Distribution of arm velocity and frequency of arm usage during daily activity: objective outcome evaluation after shoulder surgery.

In clinical settings, functional evaluation of shoulder movement is primarily based on what the patient thinks he/she is able to do rather than what he/she is actually performing. We proposed a new approach for shoulder assessment based on inertial sensors to monitor arm movement in the daily routine. The detection of movement of the humerus relative to the trunk was first validated in a laboratory setting (sensitivity>95%, specificity>97%). Then, 41 control subjects and 21 patients suffering from a rotator cuff tear were evaluated (before and after surgery) using clinical questionnaires and a one-day measurement of arm movement. The quantity of movement was estimated with the movement frequency and its symmetry index (SIFr). The quality of movement was assessed using the Kolmogorov-Smirnov distance (KS) between the cumulative distribution of the arm velocity for controls and the same distribution for each patient. SIFr presented differences between patients and controls at 3 month follow-up (p<0.05) while KS showed differences also after 6 months (p<0.01). SIFr illustrated a change in dominance due to the disorder whereas KS, which appeared independent of the dominance and occupation, showed a change in movement velocity. Both parameters were correlated to clinical scores (R(2) reaching 0.5). This approach provides clinicians with new objective parameters for evaluating the functional ability of the shoulder in daily conditions, which could be useful for outcome assessment after surgery.

Sex reversal: a fountain of youth for sex chromosomes?

Nonrecombining Y chromosomes are expected to degenerate through the progressive accumulation of deleterious mutations. In lower vertebrates, however, most species display homomorphic sex chromosomes. To address this, paradox I propose a role for sex reversal, which occasionally occurs in ectotherms due to the general dependence of physiological processes on temperature. Because sex-specific recombination patterns depend on phenotypic, rather than genotypic sex, homomorphic X and Y chromosomes are expected to recombine in sex-reversed females. These rare events should generate bursts of new Y haplotypes, which will be quickly sorted out by natural or sexual selection. By counteracting Muller's ratchet, this regular purge should prevent the evolutionary decay of Y chromosomes. I review empirical data supporting this suggestion, and propose further investigations for testing it.

Diagnostic and identification in situ of fungal pathogens in superficial mycosis

Fungi are divided in 3 groups in the field of medical mycology. The dermatophytes are filamentous fungi able to grow on keratinized tissues from human or animals. They are the main cause of superficial and cutaneous mycoses of the skin and its appendix (hair and nail). The yeasts, or dimorphic fungi, can be responsible of diverse types of infections (superficial to deep mycoses). The moulds include all Non-dermatophyte Filamentous Fungi (NDF). In medical mycology, the most representative moulds are Aspergillus spp., Fusarium spp. and Mucor spp. Diagnosis of mycosis is currently based on direct mycological examination of biological samples, as well as macroscopic and microscopic identification of the infectious fungus in culture assay. However, culture assays were found to remain sterile in roughly 40% of cases otherwise positive by direct mycological examinations. Additionally, results from culture assays are often difficult to interpret as various NDF are sometimes isolated. This thesis work is composed of three projects focusing on the development of new assays for direct in situ identification of fungi from dermatological samples. Part 1. A Polymerase Chain Reaction - Terminal Restriction Fragment Length Polymorphism assay (PCR-TRFLP) targeting the 28S rDNA was developed to identify dermatophytes and NDF in nails with suspected onychomycosis. This method is faster and more efficient than culture. It further enables the distinction of more than one agent in case of mixed infection. A fast and reliable assay for the identification of dermatophytes and NDF in onychomycosis was found to be highly relevant since onychomycosis with Fusarium spp. or other NDF are weakly responsive or unresponsive to standard onychomycosis treatments with oral terbinafine and itraconazole. Part 2. A nested PCR-sequencing assay targeting the 28S rDNA was developed to identify dermatophyte species in skin and hair samples. This method is especially suitable for tinea capitis where dermatophytes identification is critical for subsequently prescribing the adequate treatment. The challenge presented when performing direct PCR fungi identification in skin and hair differs from that seen in onychomycosis as small amount of material is generally collected, few fungal elements are present in the clinical sample and one dermatophyte among a dozen species must be identified. Part 3. Fusarium spp. is currently isolated from nails with a frequency of 15% of that of dermatophytes in the laboratory of Mycology of the CHUV (2005-2012). The aim of this work was to examine if the intensive use of terbinafine and itraconazole could be a cause of the high incidence of Fusarium nail infections. For that purpose, two different methods, specific PCR and TRFLP, were used to detect both Fusarium spp. and Trichophyton spp. in nails of previously treated or untreated patients. TRFLP assay was found to be less sensitive than classical PCR assays specifically detecting Fusarium spp. or Trichophyton spp. Independently of the detection method used, the prevalence of Fusarium spp. appears not to be higher in patients previously treated by oral standard treatment with terbinafine and azoles which are highly effective to fight Trichophyton spp. in nails. In many cases Fusarium sp. was detected in samples of patients not previously subjected to antifungal therapy. Therefore, these treatments do not appear to favor the establishment of Fusarium spp. after elimination of a dermatophyte in nail infection. - En mycologie médicale, les champignons sont classés en 3 groupes. Les dermatophytes sont des champignons filamenteux capables de se développer dans les tissus kératinisés des hommes et des animaux, ils représentent la principale cause des mycoses superficielles et cutanées de la peau et de ses appendices (ongles et cheveux). Les levures, ou champignons dimorphiques, peuvent être responsables de divers types d'infections (superficielles à profondes). Les moisissures incluent tous les champignons filamenteux non-dermatophytes (NDF), les Aspergillus spp., les Fusarium spp. et les Mucor spp. sont les principales espèces rencontrées. Le diagnostic d'une mycose est basé sur un examen mycologique direct des prélèvements biologiques ainsi que sur l'identification macroscopique et microscopique du champignon infectieux isolé en culture. Cependant, dans environ 40% des cas, l'identification de l'agent pathogène est impossible par cette méthode car la culture reste stérile, bien que l'examen direct soit positif. De plus, la croissance de moisissures et/ou autres contaminants peut rendre l'interprétation de l'examen difficile. Ce travail de thèse est composé de trois projets focalisés sur le développement de nouvelles méthodes d'identification des champignons directement à partir d'échantillons dermatologiques. Projet 1. Une méthode de Réaction en chaîne de polymérase couplée à du polymorphisme de longueur des fragments de restriction terminaux (PCR-TRFLP), en ciblant l'ADN ribosomal 28S, a été développée pour l'identification des dermatophytes et moisissures dans les ongles avec suspicion d'onychomycoses. Cette technique s'est avérée plus rapide et plus efficace que la culture, permettant l'identification de plusieurs champignons en même temps. Posséder une méthode d'identification rapide et fiable des dermatophytes et des NDF dans les onychomycoses a été jugée nécessaire du fait que les Fusarium et d'autres NDF sont peu ou pas sensibles aux traitements oraux standards à la terbinafine et à Γ itraconazole. Projet 2. Une PCR nichée couplée au séquençage d'un fragment de l'ADN ribosomal 28S a été développée afin de différencier les dermatophytes dans la peau et les cheveux. Cette méthode est particulièrement adaptée au cas de tinea capitis, où l'identification du dermatophyte est essentielle afin de prescrire le traitement adéquat. Le problème de l'identification du pathogène fongique dans les cheveux et la peau diffère des onychomycoses car de petites quantités sont prélevées chez les patients, peu d'éléments fongiques sont présents et il faut discriminer un dermatophyte parmi une douzaine d'espèces potentielles. Projet 3. Au laboratoire de Mycologie du CHUV, les Fusarium ont été isolé dans les ongles à une fréquence de 15% pour la période 2005-2012. Le but de ce travail était d'examiner si l'utilisation intensive de terbinafine et d'itraconazole pouvait être une des causes de la forte incidence des infections des ongles par Fusarium. A cet effet, deux méthodes ont été utilisées pour détecter à la fois Fusarium spp. et Trichophyton spp., la PCR spécifique et le TRFLP. Indépendamment de la méthode choisie, il en résulte que la prévalence des Fusarium η'apparaît pas liée à un traitement au préalable des patients avec de la terbinafine ou des azoles, thérapies très efficaces contre les Trichophyton spp. dans les ongles. De plus, il existe de nombreux cas où Fusarium était détecté chez des patients non traités.

Frequency of severe pulmonary hypertension complicating "isolated" atrial septal defect in infancy.

Atrial septal defects (ASDs) are typically asymptomatic in infancy and early childhood, and elective defect closure is usually performed at ages of 4 to 6 years. Severe pulmonary hypertension (PH) complicating an ASD is seen in adulthood and has only occasionally been reported in small children. A retrospective study was undertaken to evaluate the incidence of severe PH complicating an isolated ASD and requiring early surgical correction. During a 10-year period (1996 to 2006), 355 pediatric patients underwent treatment for isolated ASDs either surgically or by catheter intervention at 2 tertiary referral centers. Two hundred ninety-seven patients had secundum ASDs, and 58 had primum ASDs with mild to moderate mitral regurgitation. Eight infants were found with isolated ASDs (6 with secundum ASDs and 2 with primum ASDs) associated with significant PH, accounting for 2.2% of all patients with ASDs at the centers. These 8 infants had invasively measured pulmonary artery pressures of 50% to 100% of systemic pressure. They were operated in the first year of life and had complicated postoperative courses requiring specific treatment for PH for up to 16 weeks postoperatively. The ultimate outcomes in all 8 infants were good, with persistent normalization of pulmonary pressures during midterm follow-up of up to 60 months (median 28). All other patients with ASDs had normal pulmonary pressures, and the mean age at defect closure was significantly older, at 6.2 years for secundum ASDs and 3.2 years for primum ASDs. In conclusion, ASDs were rarely associated with significant PH in infancy but then required early surgery and were associated with excellent midterm outcomes in these patients.