Poorer Self-Reported Physical Health and Higher Anxiety Trait in Young Adults With Previous Coarctation Repair.

BACKGROUND: Little is known about the impact of a coarctation repair on the functional outcomes of young adults. This study aimed to determine (1) the functional and mental health status in young adults with previous coarctation repair, and (2) the impact of late hypertension on their quality of life. METHODS: A cross-sectional study using validated self-reported questionnaires (Short Form 36 version 2 [SF-36v2], Beck Depression Inventory [BDI], and State-Trait Anxiety Inventory [STAI]) was performed in 54 patients aged 15-47 years with previous paediatric coarctation repair. Questionnaire scores were compared to healthy age- and gender-matched controls. Patients' previously published 24-hour blood pressure monitoring results were included. RESULTS: Late hypertension was present in 64% (34/54) at a mean of 29±8 years after coarctation repair. SF-36v2 mean physical component summary score was significantly lower in coarctation patients compared with controls (53.1±6.8 vs 56.0±4.7, p=0.02), but there was no significant difference in mean mental component summary score (p=0.2). SF-36v2 mean role emotional score tended to be associated with 10 mmHg increases in mean 24-hour systolic blood pressure (regression coefficient 4.3 p=0.06). STAI mean trait anxiety score tended to be higher in coarctation patients compared with controls (36.6±9.0 vs 33.5±7.8, p=0.06). There was no significant difference in BDI scores between patients and controls. CONCLUSIONS: Young adults with previous coarctation repair report poorer physical health and tended towards higher anxiety trait compared to healthy controls. Strategies to improve self-reported physical health and anxiety should be explored. Long-term assessment of quality of life outcomes in coarctation patients is warranted.

Major Device-Dependence of Measured Hypertensive Status From 24-Hour Ambulatory Blood Pressure Monitoring After Aortic Coarctation Repair.

BACKGROUND: Twenty-four-hour (24-hr) ambulatory blood pressure monitoring (ABPM) is often considered the gold standard to detect hypertension. We aimed to determine the short-term progression of 24-hour blood pressure after coarctation repair and to compare ABPM between two different devices. METHODS: We performed a cross-sectional study using 24-hour ABPM (Oscar 2) in 47 patients aged 16-48 years with previous paediatric coarctation repair and not on antihypertensive medication. Results were compared to a previous ABPM using paired analyses. A subset (10/47, 21%) had an additional previous ABPM performed using a Spacelabs device. RESULTS: After a mean follow-up of 27±6 years after repair, hypertension and prehypertension on Oscar 2 ABPM was present in 57% (27/47) and 11% (5/47), respectively. Mean follow-up time between Oscar 2 ABPMs was 3.9±1.4 years, and between first Oscar 2 and Spacelabs and between Spacelabs and second Oscar 2 ABPM was 1.4±0.8 and 1.8±0.3 years, respectively. There was no difference in the proportion of hypertensive patients between Oscar 2 ABPMs (55% [26/47] vs. 57% [27/47], p=1.0) but 17 patients (17/47, 36%) had a reclassification of 24-hour ABPM status. Mean 24-hour systolic blood pressure was higher in both Oscar 2 ABPMs compared to Spacelabs (142.4±11.7 vs. 120.4±11.8mmHg, p=0.0001; and 137.4±12.2 vs. 120.4±11.8mmHg, p=0.0001; respectively). CONCLUSION: There was high intra-device reproducibility of 24-hour ABPM results using an Oscar 2 device but poor inter-device reproducibility in patients with repaired coarctation. Device-specific reference values may be required to ensure reliable 24-hour ABPM interpretation.

A Cross-Sectional Study of the Prevalence of Exercise-Induced Hypertension in Childhood Following Repair of Coarctation of the Aorta.

BACKGROUND: Exercise-testing may be a more tolerable method of detecting hypertension in children after coarctation repair compared to gold-standard 24-hour ambulatory blood pressure (BP) monitoring (ABPM). This study aims to determine the prevalence of exercise-induced hypertension and end-organ damage in children after coarctation repair, and the effectiveness of exercise-testing compared to 24-hour ABPM in this population. METHODS: Exercise-testing (Bruce protocol), transthoracic echocardiogram, 24-hour ABPM, and pulse wave velocity were performed in 41 patients aged 8 to 18 years with previous coarctation repair. Median age at repair was 13 days. Exercise-testing data were compared to healthy paediatric controls. Hypertension was defined as BP >95th percentile on 24-hour ABPM compared to normalised data, and systolic BP (SBP) arbitrarily >200mmHg on exercise-testing. RESULTS: After 13±3years, 39% (14/36) were hypertensive on 24-hour ABPM and 12% (5/41) on exercise-testing. Coarctation patients had a higher peak exercise SBP and reduced endurance compared to controls (164±26mmHg vs. 148±19mmHg, p=0.003; and 13.0±1.7mins vs. 14.2±2.4mins, p=0.007; respectively). All patients with a peak exercise SBP >190mmHg were hypertensive on 24-hour ABPM. Pulse wave velocity was higher in hypertensive patients on exercise-testing and 24-hour ABPM compared to normotensive patients (p=0.004 and p=0.06; respectively). CONCLUSIONS: Exercise-testing may be a useful tool to detect hypertension in children and young adults after coarctation repair, particularly in those who do not tolerate 24-hour ABPM. Normative peak exercise BP data for age should be obtained to improve the accuracy of exercise-testing in detecting hypertension.

Co-occurrence of collagenous gastrointestinal disease in siblings in early childhood: New insight into a rare condition.

Collagenous gastrointestinal (GI) disease is a rare inflammatory condition characterized by subepithelial collagen deposition and inflammatory cell infiltrates of the GI mucosa, which typically occurs in the stomach in children. There are few published reports of more extensive involvement in children, and descriptions of familial involvement are rare, with no previous reported cases of affected siblings in early childhood. We describe two siblings with contrasting medical backgrounds, who were simultaneously diagnosed with collagenous GI disease in early childhood. Both children demonstrated gastric and colonic involvement on serial endoscopies, however, had distinct patterns of clinical presentation, disease course, and histological progression, providing new insights into the pediatric phenotype of collagenous GI disease and further, its relationship to microscopic colitis. Although rare, this condition should be considered as a differential in children presenting with severe or refractory iron deficiency anemia, chronic non-bloody watery diarrhea, or unexplained nonspecific chronic abdominal pain.

Laryngeal ultrasound detects a high incidence of vocal cord paresis after aortic arch repair in neonates and young children.

OBJECTIVES: To determine the incidence of vocal cord paresis (VCP) after neonatal aortic arch repair/Norwood-type procedure, and the effectiveness of noninvasive laryngeal ultrasound in detecting VCP compared with gold standard invasive nasoendoscopy. METHODS: Fifty-two patients who underwent an arch repair (39 of 52; 75%) or Norwood-type procedure (13 of 52; 25%) via sternotomy between April 1, 2015, and April 30, 2017 underwent laryngeal ultrasound (50 of 52; 96%) and/or flexible fiber optic nasoendoscopy (39 of 52; 75%) at 48 to 72 hours after endotracheal extubation. Primary arch diagnoses were coarctation in 56% (29 of 52), hypoplastic left heart syndrome in 17% (9 of 52), isolated hypoplastic arch in 17% (9 of 52), and interrupted aortic arch in 10% (5 of 52). The median patient age at surgery was 5.5 days (interquartile range, 4.0-12.5 days). Fifteen patients (15 of 52; 29%) required preoperative intubation. RESULTS: Left VCP was present in 59% (23 of 39) of patients on nasoendoscopy and in 59% (27 of 46) of patients on laryngeal ultrasound, and 4 additional patients had inconclusive ultrasound results. There was agreement between the results of nasoendoscopy and conclusive ultrasound in all cases. The overall sensitivity, specificity, positive and negative predictive values, and Cohen's kappa coefficient of laryngeal ultrasound compared with nasoendoscopy for the detection of left VCP were 95%, 88%, 91%, 93%, and 0.83, respectively. On multivariable analysis, preoperative intubation and arch repair techniques other than the Norwood procedure were associated with left VCP (odds ratio, 12.7; P = .03; and 14.1; P = .03, respectively). CONCLUSIONS: There is a high incidence of VCP after arch repair via sternotomy. Laryngeal ultrasound seems to be an effective and noninvasive method for detecting VCP in neonates and young children.