Objectives To identify the determinants of nocturnal hypoxemia in children with CF using clinical parameters, polysomnography (PSG), and lung function. Hypothesis Sleep hypoxemia in children with CF is predicted by both apnea hypopnea index (AHI) and percent predicted forced expiratory volume in one second (pFEV1). Design Retrospective case series. Methods Children aged 5‐18 years were included based on (i) a diagnosis of CF; and (ii) availability of concurrent PSG and pFEV1 data. The impact of (i) demographic and clinical parameters; and (ii) PSG and pFEV1, on the total sleep time spent with arterial oxygen saturation below 90% (TSpO2 < 90) was measured using regression analysis. P‐value <0.05 was considered significant. Results The mean age was 11.6 years (95% confidence interval: 9.5, 13.1). Twenty of 35 (57%) were boys and the mean body mass index percentile was 42.1 (31.5, 52.6). The most common ethnicity was white (66%). OSA was diagnosed in 50%. Neither demographic predictors nor clinical variables predicted the severity of hypoxemia (R2 = 0.23, P = 0.09). While pFEV1 and PSG variables accounted for significant proportion of the overall variance in TSpO2 < 90 (R2 = 0.53, P < 0.001), pFEV1 was identified as the single best predictor of sleep hypoxemia. A pFEV1 cut‐off of 53% indicated a sensitivity of 0.80 and a specificity of 0.87 in predicting sleep hypoxemia. Conclusions pFEV1 is the best predictor of sleep hypoxemia in children with CF and referred for PSG. No demographic or clinical predictors of hypoxemia were identified in this population.

Background: Obstructive sleep apnea syndrome (OSA) is a frequent disorder in children. The clinical characteristics of OSA in very young children under 2 years of age, and more particularly, in those born prematurely, and who have respiratory complications such as bronchopulmonary dysplasia (BPD), are not well defined. We therefore retrospectively reviewed our experience in a group of preterm infants with OSAS. Methods: The records of premature infants with BPD followed in the Pediatric Pulmonary Clinic at the University of Chicago who were diagnosed with OSA from 2004 to 2009 were reviewed and analyzed. Results: Twelve children, eight males, and four females with a mean gestational age of 27 weeks were found to have OSA. Mean age at diagnosis was 19 months. Inability to wean nighttime oxygen, the need to resume oxygen after intercurrent respiratory illness, and snoring were the most common presenting symptoms. The apnea–hypopnea index ranged from 1 to 120/h total sleep time (TST; mean: 29). SpO2 nadir ranged from 50 to 91%. Despite adenotonsillectomy (AT), all children had persistent sleep disordered breathing. Conclusion: In preterm infants, while snoring is a frequent symptom, poor weight gain, and inability to wean nighttime oxygen may indicate the need for further investigation for OSA. In the former preterm infant structural changes in the airway may play an important role along with adenotonsillar hypertrophy. A high level of clinical awareness is required to identify OSA in the formerly preterm infant.

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Background Little is known about the impact of hormone therapy on transgender youth with Cystic Fibrosis (CF). This case report describes an 18-year-old affirmed female with CF who was treated with hormone therapy associated in timing with new growth of Pseudomonas aeruginosa in her sputum culture. Discussion We highlight important considerations, including the impact of gender-affirming hormone therapy on overall CF disease course. Evidence supports that females with CF have worse outcomes than males, which are partly attributed to estrogen effects. Pseudomonas aeruginosa is one of the most prevalent pathogens in people with CF. Here, we highlight a transfemale who grows Pseudomonas aeruginosa for the first time since her youth, nearly 1 year after starting estrogen therapy. This is consistent with previous literature of an association between high estrogen levels and Pseudomonas aeruginosa prevalence, but has never been evaluated in a transgender population. Conclusion Through this case, we demonstrate the need for additional research to understand the relationship between gender-affirmative hormone transition and CF care and management.

Objective: Poor nutrition in patients with cystic fibrosis (CF) has been associated with lower lung function and increased morbidity and mortality. Conversely, better nutritional status has been associated with improved pulmonary function and fewer CF-associated complications. There is no consensus regarding appetite stimulant therapy in patients with CF (pwCF). The primary objective of this study was to determine if the use of appetite stimulants was associated with weight changes in pediatric pwCF in the ambulatory care setting. Methods: This was a retrospective study that evaluated 62 pediatric pwCF who received cyproheptadine or mirtazapine for appetite stimulation for at least 6 consecutive months. Weight z-scores were collected for each patient at baseline, 3, 6, and 12 months of therapy, if available. Results: Increase in weight z-score after 3 months of therapy was statistically significant based on both univariable and multivariable models when evaluating the entire cohort. The adjusted mean difference for change in weight z-score was 0.33 (p< 0.001) from baseline to month 3. There was a statistically significant improvement in pulmonary function after 3 and 6 months of therapy. Conclusions: Appetite stimulant therapy is associated with improvement in weight z-score in the first 3 months of treatment. Appetite stimulant therapy was associated with improvement in pulmonary function in the first 3 months of therapy, which supports the relationship between weight gain and improved pulmonary function in pwCF. These findings suggest that appetite stimulants contribute to weight gain in pediatric pwCF, particularly within the first 3 months of therapy.

Introduction Sinus of Valsalva aneurysm (SOVA) may have associated infective endocarditis in which case single aneurysm may drain into adjacent chamber of heart via multiple openings. We report a rare case of congenital SOVA with associated infective endocarditis where intraoperative transesophageal echocardiogram (TEE) helped in localizing two separate openings in the SOVA draining into right ventricle and an associated perforation in the larger rightward cusp of bicuspid aortic valve (BAV) causing severe aortic regurgitation. Case report A 28-year-old male presented with grade III to IV dyspnea with previous history of infective endocarditis. Preoperative transthoracic echocardiogram (TTE) showed calcified BAV with severe aortic valve regurgitation and stenosis, severe tricuspid regurgitation, and pulmonary artery hypertension along with ruptured SOVA arising from right coronary sinus and draining into the right ventricle. Intraoperative TEE confirmed the TTE findings and in addition showed the presence of two jets arising from the SOVA draining into right ventricle, a subaortic membrane, and a perforation in the aortic cusp opening into left ventricle. The ruptured SOVA was repaired using single patch technique and aortic valve was replaced. The completeness of the repair was checked using TEE to exclude failure of closure of additional opening if any and the patient was discharged from hospital after 7 days of uneventful postoperative course. Conclusion Sinus of Valsalva aneurysm may have multiple openings draining into adjacent chamber, particularly if associated with infective endocarditis. Intraoperative TEE plays a crucial role in identification of multiple opening, involvement of adjacent structure, and assessment of completeness of repair. How to cite this article Kumar B, Munirathinam GK, Sharma P, Puri GD, Singh H. Role of Transesophageal Echocardiography in Rupture of Sinus of Valsalva Aneurysm with Associated Infective Endocarditis. J Perioper Echocardiogr 2016;4(2):59-63.

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