Impact of Telehealth on Failure to Attend Rates and Patient Re-Engagement in Adult Congenital Heart Disease Clinic.

BACKGROUND: The coronavirus disease 2019 (COVID-19) pandemic paved the way for telehealth consultations. We aimed to determine the impact of telehealth on rates of failure to attend (FTA) in adult congenital heart disease (ACHD) clinics and whether telehealth could re-engage patients with previous FTA face-to-face appointments. METHODS: This was a retrospective audit of a tertiary ACHD clinic over a 12-month pre-telehealth (26 March 2019-17 March 2020) and 12-month post-telehealth implementation period (24 March 2020-16 March 2021). Patients with one or more FTAs during the 24-month study period were included. Our ACHD clinic is run three times per month. Patients with ACHD are offered lifelong follow-up and reviewed annually on average. Re-engagement was defined as two or more consecutive face-to-face FTAs immediately before the telehealth period with subsequent attendance of their telehealth appointment. RESULTS: A total of 359 patients with a total of 623 FTAs were included. Complexity of congenital heart disease was moderate in 56% (202/359) and severe in 19% (69/359) of patients. Overall FTA rate was 18% (623/3,452). FTA rate was significantly lower in the post-telehealth period (15%, 257/1,664) compared with the pre-telehealth period (20%, 366/1,788) (p<0.00001). At study conclusion, 1% of patients had died (5/359). Of the 354 remaining patients, 42% (150/354) were considered lost to follow-up (two or more FTAs including telehealth), 37% (132/354) missed only one clinic appointment, and 20% (72/354) previously considered lost to follow-up had re-engaged in the telehealth period. CONCLUSIONS: Rates of FTA in a tertiary ACHD clinic significantly reduced after the introduction of telehealth consultation. A fifth of patients considered lost to follow-up were re-engaged with telehealth. Additional strategies to further reduce FTA should be explored.

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.

Berlin Heart EXCOR Support in the First Year of Life: A Single Centre Experience.

BACKGROUND: Berlin Heart EXCOR Pediatric Ventricular Assist Device (BHE) (Berlin Heart AG, Berlin, Germany, BHE) is used worldwide for mechanical circulatory support as a bridge to transplantation or recovery for children with end-stage heart failure. The study aim was to evaluate morbidity and mortality of children less than one year old supported with BHE to identify predictors of adverse outcomes. METHODS: Data of all children aged less than one year supported with BHE between 2005 and 2018 at the Royal Children's Hospital, Melbourne were reviewed. Adverse events were defined using PediMACS criteria. RESULTS: Fourteen (14) children under 1 year of age were implanted with BHE at a median age and weight of 0.37 years (IQR 0.09-0.7) and 5.7 kg (IQR 3.5-7.95) respectively. Four (4) patients were neonates, and 10 were older infants. Twelve (12) patients had cardiomyopathy and two, myocarditis. Preoperative extracorporeal membrane oxygenation (ECMO) support was required in six patients for a mean of 9 days (IQR 6-13). Sepsis occurred in five patients (36%) and thromboembolic stroke in two patients (14%). Survival to bridge to transplantation (11) and recovery (1) was achieved in 12 patients (86%). Mortality was 14%. The median duration of BHE support was 110 days (IQR 40-161). Both patients who died were neonates with myocarditis and required surgical re-intervention during BHE support. CONCLUSIONS: BHE provides excellent support as a bridge to transplantation or recovery in infants, with a low incidence of neurological dysfunction. Neonates with myocarditis may be at greater risk for death after BHE implantation.

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.

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.

Identifying low-grade cellular rejection after heart transplantation in children by using gene expression profiling.

Endomyocardial biopsy (EMB) remains the gold standard for detecting rejection after heart transplantation but is costly and invasive. This study aims to distinguish no rejection (0R) from low-grade rejection (1R/2R) after heart transplantation in children by using global gene expression profiling in blood. A total of 106 blood samples with corresponding EMB from 18 children who underwent heart transplantation from 2011 to 2014 were analyzed (18 baseline/pretransplantation samples, 88 EMB samples). Corresponding rejection grades for each blood sample were 0R in 39% (34/88), 1R in 51% (45/88), and 2R in 10% (9/88). mRNA from each sample was sequenced. Differential expression analysis was performed at the gene level. A k-nearest neighbor (kNN) analysis was applied to the most differentially expressed (DE) genes to identify rejection after transplantation. Mean age at transplantation was 10.0 ± 5.4 yr. Expression of B cell and T cell receptor sequences was used to measure the effect of posttransplantation immunosuppression. Follow-up samples had lower levels of immunoglobulin gene families compared with pretransplantation ( P < 3E-5) (lower numbers of activated B cells). T cell receptor alpha and beta gene families had decreased expression in 0R samples compared with pretransplantation ( P < 4E-5) but recovered to near baseline levels in 1R/2R samples. kNN using the most DE gene (MKS1) and k = 9 nearest neighbors correctly identified 83% (73/88) of 1R/2R compared with 0R by leave-one-out cross validation. Using a genomic approach we can distinguish low-grade cellular allograft rejection (1R/2R) from no rejection (0R) after heart transplantation in children despite a wide age range.

Multi-vessel giant coronary artery aneurysms: An unusual cause of chest pain.

We describe an unusual case of multi-vessel giant coronary artery aneurysms complicated by acute coronary syndrome despite escalation of therapy. A 65-year-old man with hypertension and hypercholesterolemia presented to clinic with atypical chest pain over 4 months. Outpatient computed tomography coronary angiography (CTCA) demonstrated giant coronary aneurysms involving all 3 major coronary arteries. Outpatient coronary angiogram findings were in concordance with the CTCA with no definite obstructive coronary disease. Myocardial perfusion imaging was normal. He was commenced on dual antiplatelet therapy (DAPT). At 6 months, he presented with chest pain and non-ST-elevation myocardial infarction. Repeat coronary angiogram demonstrated occluded first septal LAD branch which previously had aneurysmal dilatation. DAPT was changed to long-term oral anticoagulation. He remains well at 18 months. This case highlights the importance of multi-modality imaging in the diagnosis and workup of coronary artery aneurysms and challenges in management; an individualized approach is required.

Fate of Patients With Single Ventricles Who Do Not Undergo the Fontan Procedure.

BACKGROUND: The Fontan procedure, the last of a series of palliative operations for patients born with single ventricles, is associated with a significant late burden of complications. There are other strategies for patients who are suboptimal candidates for Fontan completion; however, the long-term outcomes of these different surgical options have not been clearly elucidated. We performed a systematic literature review to establish the current role of other treatment approaches besides the Fontan procedure. METHODS: The MEDLINE and Embase databases were systematically searched for articles describing the long-term outcomes of patients with single ventricles who have not received the Fontan procedure. RESULTS: A total of 36 articles met all inclusion criteria. There is a scarcity of contemporary data on the non-Fontan cohort. Historical studies provided a significant contribution. CONCLUSIONS: Long-term survival of unoperated patients with single ventricles is possible under the rare conditions of having balanced hemodynamics. As many as half of patients may survive on only a systemic-to-pulmonary artery shunt or bidirectional cavopulmonary shunt for more than 20 years with reasonable functional status. In patients with a failing single ventricle, the bidirectional cavopulmonary shunt is an excellent bridge to heart transplantation and may provide better posttransplant survival than patients with a Fontan circulation. Currently, the Fontan procedure continues to be the best definitive palliation for patients born with single ventricle lesions. However, for those with borderline indications, other strategies should be carefully considered.

The quadricuspid truncal valve: Surgical management and outcomes.

OBJECTIVE: To determine the outcomes of patients with a quadricuspid truncal valve (TV) and durability of TV repair. METHOD: We reviewed 56 patients with truncus arteriosus and a quadricuspid TV who underwent complete repair between 1979 and 2018. RESULTS: TV insufficiency was present in 39 patients (mild, n = 22; moderate, n = 14; and severe, n = 3). Fourteen patients had concomitant TV surgery. Early mortality in patients who had concomitant TV surgery was 14% (2 out of 14 patients) and overall survival was 77.1% ± 11.7% at 15 years. Freedom from TV reoperation was 30.3% ± 14.6% at 15 years. Early mortality in patients who did not undergo concomitant TV surgery was 9.5% (4 out of 42 patients) and overall survival was 74.9% ± 6.9% at 15 years. Progression of TV insufficiency requiring TV surgery occurred in 16.7% (7 out of 42 patients). Freedom from TV reoperation was 77.1% ± 7.8% at 15 years. The most common method of repair was tricuspidization of the TV. Freedom from TV reoperation was 64.3% ± 21.0% at 10 years after tricuspidization and 0% at 6 years after other types of TV surgery. Overall follow-up was 97.6% (41 out of 42 patients) complete for survivors with median follow-up of 16.6 years. At last follow-up there was no TV insufficiency in 16 patients, mild insufficiency in 24 patients, and moderate insufficiency in 1 patient. CONCLUSIONS: More than one-third of patients with a quadricuspid TV require TV surgery. Tricuspidization of the quadricuspid TV appears to be a durable repair option with good long-term outcomes.

Heart Failure in Adult Congenital Heart Disease.

As the population of adult congenital heart disease patients ages and grows, so too does the burden of heart failure in this population. Despite the advances in medical and surgical therapies over the last decades, heart failure in adult congenital heart disease remains a formidable complication with high morbidity and mortality. This review focuses on the challenges in determining the true burden and management of heart failure in adult congenital heart disease. There is a particular focus on the need for developing a common language for classifying and reporting heart failure in adult congenital heart disease, the clinical presentation and prognostication of heart failure in adult congenital heart disease, the application of hemodynamic evaluation, and advanced heart failure treatment. A common case study of heart failure in adult congenital heart disease is utilized to illustrate these key concepts.

Determining Expected Research Skills of Medical Students on Graduation: a Systematic Review.

BACKGROUND: Global interest in research skills in undergraduate medical education is growing. There is no consensus regarding expected research skills of medical students on graduation. We conducted a systematic review to determine the aims and intended learning outcomes (ILOs) of mandatory research components of undergraduate medical curricula incorporating the teaching, assessment, and evaluation methods of these programs. METHODS: Using the PRISMA protocol, MEDLINE and ERIC databases were searched with keywords related to "medical student research programs" for relevant articles published up until February 2020. Thematic analysis was conducted according to student experience/reactions, mentoring/career development, and knowledge/skill development. RESULTS: Of 4880 citations, 41 studies from 30 institutions met the inclusion criteria. Programs were project-based in 24 (80%) and coursework only-based in 6 (20%). Program aims/ILOs were stated in 24 programs (80%). Twenty-seven different aims/ILOs were identified: 19 focused on knowledge/skill development, 4 on experience/reactions, and 4 on mentoring/career development. Project-based programs aimed to provide an in-depth research experience, foster/increase research skills, and critically appraise scientific literature. Coursework-based programs aimed to foster/apply analytical skills for decision-making in healthcare and critically appraise scientific literature. Reporting of interventions was often incomplete, short term, and single institution. There was poor alignment between aims, teaching, assessment, and evaluation methods in most. CONCLUSIONS: The diversity of teaching programs highlights challenges in defining core competencies in research skills for medical graduates. Incomplete reporting limits the evidence for effective research skills education; we recommend those designing and reporting educational interventions adopt recognized educational reporting criteria when describing their findings. Whether students learn by "doing", "proposing to do", or "critiquing", good curriculum design requires constructive alignment between teaching, assessment, and evaluation methods, aims, and outcomes. Peer-reviewed publications and presentations only evaluate one aspect of the student research experience.

High prevalence of early arch reobstruction after arch repair in patients with anomalous right subclavian artery.

OBJECTIVES: Having an anomalous right subclavian artery has been quoted to be a risk factor for early and late adverse events. We wanted to determine the rate of adverse outcomes in patients who have undergone arch repair with an associated anomalous right subclavian artery. METHODS: The follow-up of 76 patients, with an anomalous right subclavian artery, who underwent arch repair at a single institution for various indications between 1981 and 2017 was reviewed. RESULTS: There were 12 patient deaths. Twenty-three patients required an aortic arch reintervention (17 surgeries, 2 of which were indicated for bronchial obstruction). At last follow-up, 8 of 54 surviving patients (15%) had arch reobstruction (peak gradient >25 mmHg or reintervention). Freedom from aortic arch obstruction at 10 and 15 years was 51% [95% confidence interval (CI) 36-65%] and 35% (95% CI 19-51%), respectively. Neither the complete resection of the adjacent ridge nor the detachment and reimplantation of the anomalous subclavian vessel seemed to have an impact on the rate of reobstruction [hazard ratio (HR) 1.6, 95% CI 0.77-3.5; P = 0.2 and HR 0.61, 95% CI 0.083-4.5; P = 0.6, respectively]. CONCLUSIONS: Patients with an anomalous right subclavian artery are at risk of arch reobstruction necessitating reintervention but long-term follow-up was unable to demonstrate the mechanism of this obstruction in patients with this anomaly.

Long-term outcomes following repair of truncus arteriosus and interrupted aortic arch.

OBJECTIVES: We aim to evaluate the long-term outcomes following repair of truncus arteriosus with an interrupted aortic arch. METHODS: We reviewed all children (n = 24) who underwent repair of truncus arteriosus and an interrupted aortic arch between 1979 and 2018 in a single institution. The morphology of the interrupted aortic arch was type A in 5, type B in 18 and type C in 1. RESULTS: The median age at repair was 10 days and the median weight was 3.1 kg. Direct end-to-side anastomosis of the ascending and descending aorta was performed in 16 patients (67%, 16/24), patch augmentation in 5 patients (21%, 5/24) and direct anastomosis with the use of an interposition graft to the descending aorta in 2 patients (8%, 2/24). One patient, the first in the series, underwent interrupted aortic arch repair via subclavian flap aortoplasty prior to truncus repair. A period of deep hypothermic circulatory arrest was used in 16 patients, and isolated cerebral perfusion was used in 8 patients. The early mortality rate was 17% (4 out of 24 patients). There were no late deaths and overall survival was 83 ± 8% [95% confidence interval (CI) 61-93] at 20 years. Freedom from any reoperation was 33 ± 11% (95% CI 14-54) at 5 years and 13 ± 9% (95% CI 2-34) at 10 years. Six patients underwent 10 aortic reoperations. Freedom from aortic arch reoperation was 69 ± 11% (95% CI 42-85) at 10 and 20 years. Follow-up was 95% complete (19/20), with a median follow-up time of 20 years. At last follow-up, no clinically significant aortic arch obstruction was identified in any patient, and all patients were in New York Heart Association Class I/II. CONCLUSIONS: Repair of truncus arteriosus with an interrupted aortic arch with direct end-to-side anastomosis results in good survival beyond hospital discharge. Although the long-term functional state of patients is good, reoperation rates are high.

Reduced Aortic Distensibility is Associated With Higher Aorto-Carotid Wave Transmission and Central Aortic Systolic Pressure in Young Adults After Coarctation Repair.

Background The long-term prognosis of patients with repaired aortic coarctation is characterized by high rates of cardiovascular and cerebrovascular disease related to hypertension, the basis of which remains unclear. To define potential underlying mechanisms, we investigated aortic and carotid arterial biomechanics and wave dynamics, and determinants of aortic systolic blood pressure, in young adults after coarctation repair. Methods and Results Aortic arch and carotid biomechanics, wave intensity and wave power, and central aortic blood pressure, were derived from echocardiography and brachial blood pressure in 43 young adults after coarctation repair and 42 controls. Coarctation subjects had higher brachial and central systolic blood pressure ( P=0.04), while aortic compliance was lower and characteristic impedance (Zc) higher. Although carotid intima-media thickness was higher ( P<0.001), carotid biomechanics were no different. Carotid forward compression wave power was higher and was negatively correlated with aortic compliance ( R2=0.42, P<0.001) and distensibility ( R2=0.37, P=0.001) in coarctation subjects. Aortic wave power and wave reflection indices were no different in control and coarctation patients, but coarctation patients with elevated aortic Zc had greater aorto-carotid transmission of forward compression wave power ( P=0.006). Aortic distensibility was the only independent predictor of central aortic systolic blood pressure on multivariable analysis. Conclusions Young adults following coarctation repair had a less compliant aorta, but no change in carotid biomechanics. Reduced aortic distensibility was related to greater transmission of aortic forward wave energy into the carotid artery and higher central aortic systolic blood pressure. These findings suggest that reduced aortic distensibility may contribute to later cardiovascular and cerebrovascular disease after coarctation repair.

Long-term mortality and cardiovascular burden for adult survivors of coarctation of the aorta.

OBJECTIVE: To examine the contemporary long-term outcome after coarctation repair. METHODS: This is a retrospective cohort study of 834 patients aged ≥16 years who underwent coarctation repair under single-centre follow-up. Repair was performed at a median age of 3 years (lower-upper quartile: 1 month to 15 years) by surgery in 83% (690/834) and angioplasty/stenting in 17% (144/834). Survival was compared with an age- and gender matched normal population. Other outcomes included arch reintervention, aortic valve intervention, ascending aortic intervention, and residual/re-coarctation and resting hypertension at latest follow-up. RESULTS: After a median follow-up of 27 years (lower-upper quartile: 18-36), there were 38 late deaths (5%, 38/834). Overall survival was 99%, 88% and 65% at 30, 50 and 70 years of age, respectively, significantly reduced compared with a matched normal population (standardised mortality ratio: 3.20, log-rank: p<0.001). Thirty per cent (246/834) required ≥1 arch reintervention, 13% (111/834) an aortic valve intervention and 5% (43/834) an ascending aortic intervention. Freedom from aortic valve and ascending aortic intervention was 83% and 92% at 50 years and 53% and 81% at 70 years of age, respectively. Residual/re-coarctation (gradient ≥25 mm Hg or repair site/diaphragm ratio ≤70%) at latest follow-up was present in 60% (282/474) and resting hypertension in 57% (379/661). CONCLUSIONS: Long-term survival in contemporary adult survivors of coarctation repair is significantly lower than a matched normal population with accelerated decline after the third decade. Nearly 60% of patients eventually develop hypertension, whereas approximately 50% require further invasive cardiovascular treatment by 50 years of age. Our risk-stratifying data may enable personalised follow-up strategies for this common congenital heart condition.

Early Mentoring of Medical Students and Junior Doctors on a Path to Academic Cardiothoracic Surgery.

BACKGROUND: In 2005 the Department of Cardiothoracic Surgery at The Royal Children's Hospital started an early academic mentoring program for medical students and junior doctors with the aim of fostering an interest in academic surgery. METHODS: Between 2005 and 2015, 37 medical students and junior doctors participated in research in the Department of Cardiothoracic Surgery at The Royal Children's Hospital. Each was given an initial project on which to obtain ethics approval, perform a literature review, data collection, statistical analysis, and prepare a manuscript for publication. A search of the names of these former students and doctors was conducted on PubMed to identify publications. RESULTS: A total of 113 journal articles were published in peer-reviewed journals with an average impact factor of 4.1 (range, 1.1 to 19.9). Thirty (30 of 37, 81%) published at least one article. A mean of 4.3 journal articles was published per student or junior doctor (range, 0 to 29). Eleven (11 of 37, 30%) received scholarships for their research. Nine (9 of 37, 24%) have completed or are enrolled in higher research degrees with a cardiothoracic surgery focus. Of these 9, 2 have completed doctoral degrees while in cardiothoracic surgery training. Five will complete their cardiothoracic surgery training with a doctoral degree and the other 2 are pursuing training in cardiology. CONCLUSIONS: A successful early academic mentoring program in a busy cardiothoracic surgery unit is feasible. Mentoring of motivated individuals in academic surgery benefits not only their medical career, but also helps maintain high academic output of the unit.

Impact of arch reobstruction and early hypertension on late hypertension after coarctation repair.

OBJECTIVES: Late hypertension after coarctation repair is associated with high mortality, but its risk factors remain unclear. This study aims to determine early and late postoperative risk factors for late hypertension after coarctation repair. METHODS: A cross-sectional study including transthoracic echocardiogram and 24-h blood pressure (BP) monitoring was performed in 144 patients aged ≥10 years with previous coarctation repair. Median age at repair was 39 days (interquartile range 0-3 years). Early postoperative hypertension was evaluated by calculating the mean of BP measurements taken on the same day before hospital discharge or the need for antihypertensives prior to discharge. Multivariable analyses were performed to adjust for gender, surgical age, and follow-up age. RESULTS: After a mean follow-up period of 22 ± 7 years, 59% (84/142) of patients were hypertensive: 58% (82/142) on 24-h BP monitoring and 1% (2/142) on antihypertensives. Early postoperative hypertension was present in 58% (73/126): 39% (49/126) on BP measurements and 19% (24/126) on antihypertensives. Early and late arch reobstruction (transthoracic echocardiogram peak gradient ≥25 mmHg) was present in 37% (23/62) and 23% (33/144), respectively. On multivariable logistic analysis, early postoperative hypertension and maximum descending arch velocity on echocardiography were associated with late hypertension on 24-h BP monitoring (odds ratio 2.21, 95% confidence interval 1.05-4.66, P = 0.04; and odds ratio 2.28, 95% confidence interval 1.08-4.81, P = 0.03; respectively). CONCLUSIONS: There is a high prevalence of late hypertension after coarctation repair. Arch reobstruction may be a major determinant of late hypertension. Early postoperative hypertension may identify very early in life those at risk of developing late hypertension.

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.